Semergen.

2012;38(3):167---174

www.elsevier.es/semergen

FORMACIÓN CONTINUADA-ACTUALIZACIÓN EN MEDICINA DE FAMILIA

Enfermedad de Legg-Calvé-Perthes. Revisión actualizada

I.X. Vargas-Carvajal a y Ó.F. Martínez-Ballesteros b,∗

a
Centro de Salud Cervantes, Guadalajara, España
b
Centro de Salud Juan de Austria, Alcalá de Henares, Madrid, España

Recibido el 3 de abril de 2011; aceptado el 8 de septiembre de 2011

Disponible en Internet el 14 de marzo de 2012

PALABRAS CLAVE Resumen La enfermedad de Legg-Perthes-Calvé es un proceso patológico que afecta al des-
Necrosis; arrollo de la cadera del niño. Aunque sabemos que se produce por necrosis aséptica de la cabeza
Cabeza fémur; femoral, las causas que originan tal necrosis se desconocen realmente.
Avascular; La radiología convencional no permite establecer un diagnóstico precoz, por lo que se nece-
Osteonecrosis; sitan técnicas diagnósticas más avanzadas.
Enfermedad; El momento en que se realice el diagnóstico y en consecuencia, el grado de afectación
Legg-Perthes en que se encuentre la cadera en ese momento, determinan el tratamiento a aplicar y las
complicaciones que puedan desarrollar los paciente en el futuro.
El objetivo de este trabajo es que el médico de atención primaria tenga en consideración
esta entidad cuando se encuentre ante un niño con problemas relacionados con la cadera y sepa
realizar un correcto diagnóstico diferencial con las entidades que presentan una sintomatología
similar dado que el pronóstico final dependerá en buena parte del diagnóstico precoz.
© 2011 Elsevier España, S.L. y SEMERGEN. Todos los derechos reservados.

KEYWORDS Legg-Calve-Perthes disease. Updated review

Femur;
Head; Abstract Legg-Calve-Perthes disease affects the development of the child’s hip. Although we
Necrosis; know that is produced by aseptic necrosis of the femoral head, the cause of such necrosis is
Avascular; really unknown.
Osteonecrosis; Conventional radiology does not permit an early diagnosis; for this reason more advanced
Legg-Perthes; diagnostic techniques are needed.
Disease The timing of the diagnosis determines the degree of impact in the hip and has importance
for the type of treatment to be applied, and the possible complications that the patient may
develop.

∗ Autor para correspondencia.

Correo electrónico: oballesteros@terra.es (Ó.F. Martínez-Ballesteros).

1138-3593/$ – see front matter © 2011 Elsevier España, S.L. y SEMERGEN. Todos los derechos reservados.
doi:10.1016/j.semerg.2011.09.012
168
The aim of this work is to make the general practitioner aware of this disease and to take it into
consideration when examining a child with problems related to the hip, and in order to make
a correct differential diagnosis with conditions that have similar symptoms, because the final
outcome will depend on how quickly the diagnosis was established and also the treatment.
© 2011 Elsevier España, S.L. and SEMERGEN. All rights reserved.
Introducción
La enfermedad de Legg-Calvé-Perthes u osteocondritis
deformante juvenil consiste en una necrosis aséptica de la
cabeza del fémur de los niños debida a una alteración idio-
pática de la circulación. También se le suele denominar coxa
plana porque la característica principal es la pérdida de la
esfericidad de la cabeza femoral1---7 .
Con este trabajo queremos recordar esta enfermedad y
sus datos clínico-patológicos más importantes porque con-
sideramos de gran importancia que el médico de atención
primaria sea capaz de sospechar y, en consecuencia, tener
la facultad para diagnosticar este trastorno, ya que de no
hacerlo en edades tempranas de la vida podría conllevar
graves secuelas en la edad adulta, como es la artrosis de
cadera.
Por otra parte, dadas las características particulares de
la medicina de atención primaria, los médicos que en ella
trabajamos somos los que más veces vemos a los pacientes y
podemos apreciar en ellos cambios que a otros especialistas
les llevarían mucho más tiempo ver (número de visitas a su
consulta y tiempo transcurrido entre ellas).
Los médicos de atención primaria podemos tomar con-
tacto con un paciente con enfermedad de Perthes en
2 grupos de edad diferentes y cuyo pronóstico será tam-
bién diferente según la rapidez con que se instaure el
tratamiento. Si el paciente es un niño de corta edad y,
tras examinarle y hacerle las primeras pruebas diagnósti-
cas, sospechamos que se trata de un caso de enfermedad
de Perthes, orientaremos rápidamente el tratamiento y el
pronóstico será bueno. Si por el contrario, vemos a un niño
mayor, o casi adolescente, el diagnóstico también lo podre-
mos enfocar desde atención primaria pero el objetivo del
tratamiento y el pronóstico serán muy distintos.
Etiología y fisiopatología
La cadera del recién nacido está incompletamente desarro-
llada y pasa por diferentes etapas hasta alcanzar la forma
adulta definitiva. La llegada de sangre al cotilo está bien
asegurada. Pero no ocurre así en la cabeza femoral, dado
que está situada profundamente dentro de la articulación.
El único aporte vascular al núcleo cefálico a esas edades es
a través de ramas epifisarias de la arteria circunfleja poste-
rior, vulnerables al transcurrir por la sinovial o al atravesar
la cáscara cartilaginosa que rodea al núcleo óseo capital1 .
La enfermedad de Perthes se produce por impedimento
de la irrigación sanguínea a la cabeza del fémur, lo que pro-
voca la necrosis de algunas partes del hueso haciéndolas
más frágiles y provocando así el aplastamiento de la cabeza
femoral1,6 . A partir de este momento, el hueso muerto es
I.X. Vargas-Carvajal, Ó.F. Martínez-Ballesteros
reabsorbido y se inicia la llegada de nuevos vasos sanguí-
neos a la cabeza femoral. El hueso vuelve a reconstruirse
en un proceso que puede tardar entre 3 y 4 años. Al final,
la nueva cabeza femoral estará formada por hueso duro que
podrá tener una forma igual a la original (esférica y correcta)
o quedar deformada e incongruente con el acetábulo1,3 .
Factores relacionados
Aunque la etiología es desconocida, se han intentado
involucrar distintos factores como responsables de esta
enfermedad. Entre ellos están: la presencia de una posible
sinovitis, alteraciones de la coagulación, trombofilia, talla
baja y retraso en la edad ósea, traumatismos repetidos,
toma de corticoides, nivel socioeconómico bajo, etc. Sin
embargo, ninguno de ellos ha podido ser demostrado1---4,6,7 .
Epidemiología
Se calcula que la enfermedad la sufren entre el 1,0 y el
2,5 de cada 10.000 niños1,3 . Predomina en entre 4 y 5 veces
más en niños que en niñas1---4,7 , pero en estas el pronóstico
es peor2 . La enfermedad se manifiesta entre los 3 y 8 años.
Los pacientes suelen ser delgados, muy activos y de menor
tamaño que sus contemporáneos. Es bilateral en el 10-15%
de los casos1---3,5---7 . La bilateralidad nunca es sincrónica, de
forma que, cuando está en fase de reosificación una cadera,
se inicia en la contralateral7 . Hay mayor incidencia en japo-
neses y esquimales y es poco frecuente en la raza negra,
aborígenes australianos, indígenas de América y en Europa
Central1 .
Clínica
Las primeras manifestaciones de la enfermedad son moles-
tias o dolor a nivel de la cadera, de la ingle, del muslo o
de la rodilla, siguiendo el trayecto del nervio obturador;
acompañadas de una cojera más o menos acentuada. El dolor
suele ser leve e intermitente aumentando con la marcha o
los juegos y desapareciendo totalmente con el reposo1,2,7 .
Con el tiempo el niño pierde movilidad de la cadera sobre
todo para la abducción y la rotación interna. Puede obser-
varse una contractura en flexo de la cadera, con contractura
de los músculos aductores y del psoas ilíaco; así como hipo-
trofia o atrofia del cuádriceps crural, los gemelos y la nalga
lo que puede provocar una cierta dismetría de miembros
inferiores. La palpación profunda anterior y posterior de la
cadera produce dolor1---5,7 . El niño suele estar afebril7 .
Los exámenes de laboratorio no muestran alteraciones
valorables, el hemograma suele estar normal y tan solo
Enfermedad de Legg-Calvé-Perthes. Revisión actualizada
puede haber un aumento discreto de la velocidad de sedi-
mentación y de la proteína C reactiva5,7 .
Cuando el paciente se sale del perfil típico es difícil el
diagnóstico. En estos casos, la enfermedad de Perthes puede
ser descubierta en forma accidental al hacer estudios radio-
gráficos por otras causas1,2 .
Métodos diagnósticos
Radiología convencional
Las imágenes radiográficas son el método más común para
iniciar el estudio de un cuadro doloroso de cadera o pel-
vis, por sospecha de enfermedad de Perthes u otra entidad
nosológica, por ser un método sencillo de lograr, barato y
relativamente fácil de interpretar. En el estudio de enferme-
dad de Perthes se deben tomar imágenes anteroposteriores
y laterales en posición de Lauenstein (en rana)1 .
Desde el punto de vista radiológico, el proceso de isque-
mia y posterior regeneración del hueso se ha dividido en
varias etapas que revelan los cambios anatómicos de la
cabeza y cuello femorales. La identificación de en qué fase
se encuentra el proceso tiene importancia terapéutica y
pronóstica1---3,6 .
Fase inicial o de necrosis
Se produce la interrupción del aporte vascular y necrosis
ósea. En esta etapa la cabeza femoral es muy vulnerable a
las fuerzas que actúan sobre ella.
Radiológicamente existe aumento del espacio articular
(signo de Waldenström)1 secundario a una fractura sub-
condral. Este es el signo radiológico más precoz. Podemos
encontrar un núcleo epifisario en la cabeza.
Fase de fragmentación
Se inicia un proceso de reabsorción del hueso necrótico.
Radiológicamente aparecen densos islotes óseos, los
centrales se condensan y los laterales sufren osteólisis pro-
duciendo una imagen atigrada.
Fase de reosificación
La densidad se desplaza en sentido contrario. La epífisis es
invadida por vasos, se reabsorben los islotes densos y se
forma tejido óseo rarefacto que luego se trabecula. Se inicia
la reparación con desaparición de la osteólisis metafisaria.
Fase final o de curación
Se produce la sustitución completa del hueso necrótico por
hueso de nueva formación.
El hueso neoformado tiene una consistencia más débil por
lo que puede remodelarse de modo que la morfología de la
cabeza femoral se adapte a la forma del cotilo o no. Este pro-
ceso no será definitivo hasta el final de la maduración ósea.
Dado que el resultado final puede ser el de una cabeza femo-
ral deformada, a esta fase se la puede denominar también
fase de deformidad residual1 .
La duración de cada etapa es muy variable, pero en gene-
ral, la etapa de necrosis y fragmentación dura unos 6 meses,
la de reosificación de 18 meses a 3 años y la fase final o de
curación hasta la maduración ósea1 . Para otros autores la
169
fase de fragmentación dura aproximadamente un año y la
fase de reosificación de 3 a 5 años6 .
El inconveniente principal de la radiografía convencio-
nal es su ineficacia para el diagnóstico precoz de la necrosis
ósea8 . Pero una vez los cambios óseos son visibles esta será
de gran utilidad para la clasificación y seguimiento de la
enfermedad1---3,6 , enfocar el tipo de tratamiento1,3 y el esta-
blecimiento de un pronóstico1---3,8 .
Varios autores han intentado establecer clasificaciones
de la enfermedad en función de los hallazgos radiológicos
con objeto de relacionar la fase evolutiva de la enfermedad
con el pronóstico y el tratamiento a realizar.
Entre ellos destaca el trabajo de Catterall et al.9 que
dividen la afectación de la cabeza femoral en 4 tipos en
función de los datos aportados por la radiografía simple.
En el grupo I se afecta la epífisis de la cabeza femoral en
su región anterior. No hay secuestros ni colapso de la epífisis
ni cambios metafisarios. En el grupo II está afectada más
del 50% de la parte anterior de la epífisis. Pueden formarse
colapsos y secuestros. Reacción metafisaria limitada. En el
grupo III está secuestrada la mayor parte de la epífisis con
fragmentación y colapso extenso, formación de secuestros y
osteólisis metafisaria. En el grupo IV se encuentra afectada
la totalidad de la epífisis con cambios metafisarios avanzados
y alteraciones en la placa de crecimiento.
Si bien esta clasificación ha sido durante muchos años ha
sido el punto de referencia en la enfermedad de Perthes, ha
demostrado una gran variabilidad interobservador1,2,8 y un
valor pronóstico limitado2 .
Catterall añadió además el concepto de signos radiológi-
cos de mal pronóstico a los que también denominó «signos
de riesgo cefálico» o «cabeza de riesgo», que hoy día todavía
tienen vigencia1---3,9 . Estos signos son:
• Signo de Gage. Consiste en un defecto transparente en
forma de V que se ubica en la parte externa de la epífisis
y que se puede apreciar en la radiografía anteroposterior.
• Extrusión o subluxación externa de la cabeza femoral del
acetábulo.
• Zona calcificada por fuera de la epífisis cefálica.
• Crecimiento horizontal de la placa de crecimiento.
• Presencia de quistes metafisarios.
Si aparecen 2 o más de estos signos el pronóstico es
malo1,8 .
La cabeza femoral está formada por 3 pilares: medial
(interno), central y lateral (externo). El pilar lateral es el
más importante desde el punto de vista de soporte del peso
corporal. Herring10 estableció una clasificación basada en
los cambios radiológicos del segmento lateral de la cabeza
femoral en fase de fragmentación (tabla 1). Estableció 3
grupos según la altura de dicho segmento, encontrando
una correlación clara con el pronóstico de la enferme-
dad ya que, a mayor altura, mayor protección contra el
derrumbamiento, y cuando correlacionamos los hallazgos
radiológicos con la edad de aparición de la enfermedad el
nivel de predicción es mejor1,2 . Sobre esta clasificación algu-
nos autores han creído conveniente agregar un cuarto grupo
denominado «grupo B/C» para casos limítrofes en los que el
pilar lateral conserva la altura del 50%, pero el segmento
remanente tiene muchas irregularidades radiográficas1,6 . La
170 I.X. Vargas-Carvajal, Ó.F. Martínez-Ballesteros

Tabla 1 Clasificación de Herring

Normal: División funcional de la cabeza del fémur en pilares lateral, central y medial
Grupo A: La altura del pilar lateral es la normal. Puede haber radiolucidez en pilares central y medial pero sin pérdida
de altura del pilar lateral. Buen pronóstico
Grupo B: Pérdida de altura del pilar lateral pero menor del 50% de su altura normal. El segmento central puede estar
más hundido
Grupo C: El colapso del pilar lateral es superior al 50% de su altura normal. Los pilares central y medial pueden haber
perdido su altura normal pero en menor grado que el pilar lateral. Mal pronóstico
Fuente: Elaboración propia.

clasificación del pilar lateral de Herring tiene un mayor valor
pronóstico y menor variabilidad interobservador que la clasi-
ficación de Catterall2,8 , por lo que hoy día es la clasificación
más utilizada.
Sobre esta clasificación algunos autores han creído con-
veniente agregar un cuarto grupo denominado «grupo B/C»
para casos limítrofes en los que el pilar lateral conserva la
altura del 50%, pero el segmento remanente tiene muchas
irregularidades radiográficas1,6 .
Stulberg11 valoró la situación de la cadera en la fase
final. Estableció 5 grados de deformidad de la cabeza femo-
ral, y añadió el concepto de ‘‘congruencia’’ entre ésta y
el acetábulo (tabla 2). También aporta información acerca
del pronóstico de forma que la incidencia de artrosis de
cadera en la edad adulta se incrementa desde la clase I
hasta la clase V tras una media de seguimiento de 40 años.
Sin embargo, la validez de esta clasificación ha sido cuestio-
nada por varios autores porque según su opinión tiene poca
reproductividad interobservador6 .
Con la sospecha clínica y radiológica de enferme-
dad de Perthes, se deberá completar el estudio con
exploraciones más complejas que deberá solicitar un espe-
cialista en ortopedia, pues se salen del ámbito de la
atención primaria. Estas permitirán establecer una estra-
tegia terapéutica. Entre estas técnicas se encuentran las
siguientes1,2,12 :
También es capaz de valorar durante el proceso evolutivo
el grado de revascularización de la cabeza femoral.
• Resonancia magnética. Al igual que la gammagrafía, per-
mite el diagnóstico precoz del proceso, es decir, antes
de que la radiografía convencional muestre alteraciones,
con la ventaja de que proporciona importante información
de la forma de la cabeza y del acetábulo y el grado de
congruencia entre ambos.
• Artrografía. Es de gran utilidad para la visualización del
contorno de la cabeza femoral y especialmente de su
relación con el acetábulo. Permite al cirujano ortopédico
establecer la estrategia quirúrgica. Su indicación funda-
mental es en el diagnóstico y valoración de la cadera «en
bisagra».
La cadera «en bisagra» (fig. 1) es aquella en la que el
margen lateral del cotilo ha deformado una cabeza femo-
ral grande y blanda. Cuando el paciente intenta abducir la
extremidad inferior, la cabeza femoral no rueda dentro del
cotilo sino que hace bisagra en el margen lateral del cotilo
limitando severamente la movilidad.
• Tomografía computarizada. No se utiliza de forma siste-
mática, si bien puede ser de utilidad en el estudio de la
extensión de la afectación ósea y de la estructura tridi-
mensional de la cadera.

• Ultrasonidos. Útiles en la valoración inicial del proceso y Diagnóstico diferencial

para valorar si existe o no sinovitis.
• Gammagrafía con tecnecio-99. Nos permite el diagnós- Con una historia clínica bien realizada, un examen físico
tico precoz antes de que aparezcan cambios radiológicos. cuidadoso y las radiografías simples de cadera suelen ser
Enfermedad de Legg-Calvé-Perthes. Revisión actualizada
Tabla 2 Clasificación de Stulberg
Clase Descripción
I Congruencia esférica
II Congruencia esférica con
pérdida de la forma de la
cabeza femoral menor de 2 mm
III Congruencia no esférica con
pérdida de la forma de la
cabeza femoral mayor de 2 mm
IV Congruencia no esférica
V Incongruencia no esférica
Fuente: Elaboración propia.
Tabla 3 Diagnóstico diferencial de la cadera dolorosa
Enfermedad de Perthes
Edad 3-10 años
Etiología Desconocida
Síntomas Cojera progresiva. Dolor de
cadera, muslo o rodilla. Sin
fiebre
Exploración Limitación de la abducción y
abolición de la rotación interna
Analítica VSG y PCR normales o
discretamente elevadas.
Hemograma normal
Radiología Alteraciones características
según fase evolutiva de la
enfermedad
Tratamiento Reposo, analgésicos y cirugía
según grado de afectación
Duración Meses o años
PCR: proteína C reactiva; VSG: velocidad de sedimentación globular.
Fuente: Elaboración propia.
171
Características Porcentaje de pacientes que
desarrollan artrosis tras 40
años de seguimiento
Articulación de la cadera 0
completamente normal
Cabeza femoral esférica en las 16
radiografías anteroposterior
y lateral (en rana), con una
o más de las siguientes
anomalías: coxa magna
(cabeza femoral más grande
de lo normal), acortamiento
del cuello femoral o cobertura
acetabular anormal
Cabeza femoral no esférica 58
pero tampoco plana. Forma
ovoide o de hongo; con una
o más de las siguientes
anomalías: coxa magna,
acortamiento del cuello
femoral o cobertura acetabular
anormal
Cabeza femoral aplanada con 75
anormalidades en la cabeza y
cuello femoral y del acetábulo
Cabeza femoral aplanada con 78
forma normal del cuello
femoral y del acetábulo
Artritis séptica Sinovitis transitoria
1-10 años 3-12 años
Estafilococos, estreptococos Desconocida
y Kingella kingae (probablemente viral)
principalmente. También virus
y hongos
Afectación general aguda. Cojera aguda. Dolor de
Fiebre 38-39 ◦ C. Cojera aguda cadera y rodilla.
Sin fiebre ni febrícula
Limitación de todos los Rotación interna abolida
movimientos
Aumento de leucocitos, VSG Aumento de leucocitos,
y PCR VSG y PCR
Inicialmente aumento del Normal
espacio articular. En casos
evolucionados: erosiones
subcondrales y osteomielitis
Reposo, antiinflamatorios y Reposo y
antibióticos amplio espectro antiinflamatorios
(ibuprofeno)
Horas o días 5-7 días
172 I.X. Vargas-Carvajal, Ó.F. Martínez-Ballesteros

Ilion
Trocánter mayor
Cadera
“en bisagra”

Cabeza del fémur

Interlínea fémoroacetabular

Trocánter menor

Fémur en posición neutra Fémur en abducción

Figura 1 Cadera «en bisagra». Fuente: Elaboración propia.

suficientes para establecer un diagnóstico inicial en atención
primaria1,7 . Sin embargo, en la fase inicial de la enfermedad
debe realizarse el diagnóstico diferencial con la sinovitis de
cadera y con las artritis sépticas (tabla 3) en las que los sín-
tomas iniciales suelen ser muy similares1,7 . Es especialmente
importante descartar la presencia de una artritis séptica
pues puede destruir la articulación en unas pocas horas7 . El
diagnóstico diferencial debe incluir también la fractura de
cuello femoral, artritis reumatoide juvenil, fiebre reumática
y tumores, pero estas entidades se dan con mucha menor
frecuencia1,5,7 . Cuando ambas caderas están involucradas
hay que descartar la displasia epifisaria, el hipotiroidismo
y la enfermedad de Gaucher1 .
Siempre deberemos solicitar una serie de parámetros
de laboratorio para intentar diferenciar estas entida-
des, que incluirán la realización de un hemograma, la
determinación de la velocidad de sedimentación globular,
proteína C reactiva, factor reumatoide y antiestreptolisi-
nas, todas ellas fácilmente accesibles desde la atención
primaria1,7 .
Evolución y pronóstico
La enfermedad de Perthes es una enfermedad autolimitada,
pero aunque su evolución natural sea hacia la curación, en
atención primaria, si sospechamos que estamos ante una
enfermedad de Perthes, deberemos hacer revisiones perió-
dicamente cada 3 o 4 meses dado que algunos casos evo-
lucionan desfavorablemente y pueden dejar severas
secuelas en la edad adulta, principalmente artrosis de
cadera2,7 , puesto que la capacidad de remodelación de la
cabeza femoral con el acetábulo finaliza alrededor de los
8 años de edad y a partir de esta edad apenas queda poder
de remodelación2,4,6,7,11 .
Aunque el 80% de los pacientes afectados por la enfer-
medad tienen buena evolución hasta la cuarta década de la
vida, entre la cuarta y quinta década existe un riesgo impor-
tante de desarrollar artrosis de cadera13 y en la sexta década
de la vida, la mitad de los pacientes requerirán el empleo
de una prótesis de cadera según un estudio realizado tras
una media de seguimiento de 47,7 años14 .
Diversos autores1,2,4,6---11,15---18 coinciden en que existen
una serie de factores cuya presencia indica una mala evolu-
ción de la enfermedad. Dichos factores son:
• Edad de inicio de los síntomas mayor de 6 años.
• Grado de deformidad de la cabeza femoral.
• Grado de incongruencia de la cabeza femoral con el ace-
tábulo.
• Curso prolongado de la enfermedad.
• Fase de la enfermedad en que se inició el tratamiento.
• Presencia de los signos de riesgo cefálico o «cabeza de
riesgo» descritos por Catterall.
• Grupos III y IV de Catterall.
• Grupo C de la clasificación del pilar lateral de Herring.
• Cadera «en bisagra».
• Cierre precoz de la fisis.
Enfermedad de Legg-Calvé-Perthes. Revisión actualizada
Tratamiento
Puesto que desconocemos la etiología de la enfermedad la
prevención de la misma no es posible1 .
Cualquier paciente con sospecha de padecer enferme-
dad de Perthes debe ser remitido al cirujano ortopeda para
una valoración completa del caso4 . En casos leves, el segui-
miento podremos hacerlo desde atención primaria7 .
El tipo de tratamiento depende de la severidad de
la enfermedad. En los casos leves, y como la evolución
natural es hacia la curación, el tratamiento consiste en
reposo en cama, uso de muletas, restricción de actividades
deportivas y rehabilitación. Para el tratamiento del dolor y la
inflamación son recomendables los analgésicos y el reposo.
En pacientes con dolor intenso y gran pérdida del movi-
miento de la cadera puede ser necesario el ingreso en un
hospital para controlar mejor el reposo y la medicación o
colocar inmovilizaciones para relajar la musculatura. Una
vez ha desaparecido el dolor se puede iniciar terapia física
o hidroterapia1---3 .
En estadios más avanzados, el objetivo principal del tra-
tamiento es intentar que la forma de la cabeza del fémur
se adapte durante su desarrollo, que finaliza a los 8 años de
edad, lo mejor posible a la forma del cotilo (teoría de la
contención), ya que este actuaría como molde de la cabeza
femoral en formación y se lograría así una articulación con-
gruente. De esta manera se consigue minimizar el desarrollo
de deformidades en la cadera que podrían derivar en el des-
arrollo de artrosis en la edad adulta1---3,11 . Esto se puede
conseguir con la utilización de ortesis, tenotomías de los
músculos aductores y psoas ilíaco, y en los casos severos
con cirugía realizando osteotomías femorales y pélvicas1---6 .
El inconveniente de realizar osteotomías es que hay que rea-
lizar una segunda intervención para retirar el material que
se utilizó para fijar la cabeza femoral dentro del cotilo1,3 . En
los casos de contractura importante de los músculos aduc-
tores se puede realizar tratamiento con toxina botulínica
combinada con fisioterapia intensiva2 .
La utilización de ortesis y la restricción del movimiento
pueden provocar problemas psicológicos y sociales en los
niños al compararse con niños de su edad por lo que, si
se decide realizar este tipo de tratamiento, los niños y sus
familias puede que necesiten la atención de un psicólogo1 .
La mejor estrategia para decidir qué tratamiento
aplicar es considerar la edad en que aparecieron los
síntomas combinada con el grado de afectación de la
cabeza femoral en ese momento1,2,6 . Aplicando este
principio podemos establecer las siguientes pautas de
tratamiento:
Edad menor de 6 años
En este grupo de edad los hallazgos se corresponden con
las clases I y II de Stulberg y se obtienen buenos resultados
con tratamientos conservadores. Si en este grupo de edad
encontramos casos compatibles con la clase III de Stulberg
el tratamiento quirúrgico no aporta nada con respecto al
tratamiento conservador por lo que se prefiere este último6 .
En los casos pertenecientes a los grupos I y II de Catte-
rall el tratamiento debe ser conservador. Si pertenecen a los
grupos III y IV, se valorará mediante resonancia magnética
173
si existe o no extrusión lateral de la cabeza femoral, si no
la hay el tratamiento será conservador; pero si existe extru-
sión u otros signos de riesgo el tratamiento tendría que ser
quirúrgico17,19 .
Si los hallazgos encontrados se corresponden con los gru-
pos A y B de la clasificación del pilar lateral de Herring, el
tratamiento será conservador. Pero si se corresponden con
el grupo B/C o C, el tratamiento será quirúrgico2 .
Edad 6 y 8 años
El tipo de tratamiento a realizar en este grupo de edad está
menos claro. Algunos autores afirman que no hay diferencias
entre que sea conservador o quirúrgico6 .
Si los hallazgos encontrados se corresponden con los gru-
pos A y B de la clasificación del pilar lateral de Herring, el
tratamiento será conservador. Pero si se corresponden con
el grupo B/C o C, el tratamiento será quirúrgico2,19 .
Edad mayor de 8 años
En los casos que se corresponden con los grupos B/C y C de la
clasificación del pilar lateral de Herring, y para ciertos auto-
res, también los que corresponden al grupo B se recomienda
tratamiento quirúrgico2,6,19 .
Los pacientes que perteneciendo a este grupo de edad,
presenten hallazgos compatibles con los grupos I y II de Cat-
terall, el tratamiento quirúrgico es aplicable, pero tan solo
es efectivo en el 41% de los casos6 .
En los casos correspondientes a las clases III y IV de Stul-
berg el tratamiento será quirúrgico15 .
Hay estudios que muestran que a partir de los 8 años de
edad el tratamiento quirúrgico no ofrece ventajas sobre el
tratamiento conservador puesto que ya no es posible preve-
nir o revertir la deformidad de la cabeza femoral20 .
Respecto a la cadera «en bisagra», al ser una cadera
deformada y con movilidad comprometida el tratamiento
ha de ser quirúrgico2,12,15 .
Conflicto de intereses
Los autores declaran no tener ningún conflicto de intereses.
Bibliografía
1. Frías Austria R. Legg-Calvé-Perthes disease. Acta Ortop Mex.
2009;23:172---81.
2. Nelitz M, Lippacher S, Krauspe R, Reichel H. Perthes disease:
current principles of diagnosis and treatment. Dtsch Arztebl Int.
2009;106:517---23.
3. Miranda L, Bas T, Martí V. Enfermedad de Perthes. Conceptos
básicos. An Pediatr Contin. 2005;3:317---21.
4. Atanda Jr A, Shah SA, O‘Brien K. Osteochondrosis: com-
mon causes of pain in growing bones. Am Fam Physician.
2011;83:285---91.
5. Cruz M. Tratado de Pediatría. Vol II. 5.a ed. Barcelona: Espaxs;
1983. p. 1209-12.
6. Kim HK. Legg-Calvé-Perthes disease. J Am Acad Orthop Surg.
2010;18:676---86.
7. Muñoz Calvo MT, Hidalgo Vicario MI, Clemente Pollán J. Pediatría
extrahospitalaria. Fundamentos Clínicos Para Atención Prima-
ria. 4.a ed. Madrid: Ergon; 2008. p. 859---81.
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8. Lee DS, Jung ST, Kim KH, Lee JJ. Prognostic value of modified
lateral pillar classification in Legg-Calvé-Perthes disease. Clin
Orthop Surg. 2009;1:222---9.
9. Catterall A. The natural history of Perthes’ disease. J Bone Joint
Surg Br. 1971;53:37---53.
10. Herring JA, Neustadt JB, Williams JJ, Early JS, Browne RH.
The lateral pillar classification of Legg-Calvé-Perthes disease.
J Pediatr Orthop. 1992;12:143---50.
11. Stulberg SD, Cooperman DR, Wallensten R. The natural his-
tory of Legg Calvé Perthes disease. J Bone Joint Surg Am.
1981;63:1095---108.
12. Choi IH, Yoo WJ, Cho TJ, Moon HJ. Principles of treatment
in late stages of perthes disease. Orthop Clin North Am.
2011;42:341---8.
13. Onishi E, Ikeda N, Ueo T. Degenerative osteoarthritis after Pert-
hes’ disease: a 36-year follow-up. Arch Orthop Trauma Surg.
2011;131:701---7.
14. McAndrew MP, Weinstein SL. A long-term follow-up of Legg-
Calvé-Perthes disease. J Bone Joint Surg Am. 1984;66:860---9.
I.X. Vargas-Carvajal, Ó.F. Martínez-Ballesteros
15. Segev E, Ezra E, Wientroub S, Yaniv M, Hayek S, Hemo Y. Treat-
ment of severe late-onset Perthes’ disease with soft tissue
release and articulated hip distraction: revisited at skeletal
maturity. J Child Orthop. 2007;1:229---35.
16. Sharma S, Shewale S, Sibinski M, Sherlock DA. Legg-Calvé-
Perthes disease affecting children less than eight years of age:
a paired outcome study. Int Orthop. 2009;33:231---5.
17. Canavese F, Dimeglio A. Perthes’ disease: prognosis in children
under six years of age. Canavese F, Dimeglio A. J Bone Joint
Surg Br. 2008;90:940---5.
18. Terjesen T, Wiig O, Svenningsen S. The natural history of Pert-
hes’ disease. Acta Orthop. 2010;81:708---14.
19. Wiig O, Terjesen T, Svenningsen S. Prognostic factors and out-
come of treatment in Perthes’ disease: a prospective study of
368 patients with five-year follow-up. J Bone Joint Surg Br.
2008;90:1364---71.
20. Osman MK, Martin DJ, Sherlock DA. Outcome of late-onset Pert-
hes’ disease using four different treatment modalities. J Child
Orthop. 2009;3:235---42.
 LCPD SUPPLEMENT
Salter Osteotomy in Legg-Calvé-Perthes Disease
George H. Thompson, MD
Background: Femoral head containment in Legg-Calvé-Perthes
disease (LCPD) can be either surgical or nonsurgical. The Salter
or innominate osteotomy is a common method of surgical
containment. This is a review of the technique and results of this
osteotomy in LCPD.
Methods: The operative technique is relatively simple but requires
considerable experience to perform correctly. It can be used alone
or in combination with a proximal femoral varus osteotomy. The
indications for a Salter osteotomy are essentially the same as in
any form of containment treatment in LCPD. This includes: age at
clinical onset of 6 to 10 years (perhaps, 5 y in female), more than
one-half capital femoral epiphyseal involvement (Catterall groups
III or IV, Salter-Thompson group B, and lateral pillar groups B,
B/C, and C), and a good range of hip motion before surgery. The
osteotomy alone is usually indicated for younger children with
recent clinical onset and no femoral head deformity or subluxa-
tion. The combined procedure is better suited for older children
and those with subluxation or a deformed femoral head.
Results: Currently, the results of treatment are best determined
at skeletal maturity using the Stulberg et al classification. When
used alone, approximately 90% to 95% of the involved hips will
have achieved a Stulberg et al class I, II, or III result. When
combined with a proximal femoral varus osteotomy, the results
are somewhat less because of the older age at onset and/or the
presence of a deformed hip.
Conclusions: The Salter osteotomy in LCPD is an effective
method of surgical treatment that can alter the natural history of
the disease process. The main advantage of this osteotomy is its
effect on femoral head remodeling during remaining growth.
Key Words: Legg-Calvé-Perthes disease, Salter osteotomy, innomi-
nate osteotomy, proximal femoral varus osteotomy, Catterall
classification, Salter-Thompson classification, lateral pillar classifi-
cation, Stulberg et al classification, Mose circle criteria
(J Pediatr Orthop 2011;31:S192–S197)
S urgical containment of the femoral head in Legg-
Calvé-Perthes disease (LCPD) is currently the most
common treatment. It has been recently demonstrated
From the Division of Pediatric Orthopaedics, Rainbow Babies and
Children’s Hospital, University Hospitals Case Medical Center, Case
Western Reserve University, Cleveland, OH.
Supported entirely by the Division of Pediatric Orthopaedic Surgery,
Rainbow Babies and Children’s Hospital, Cleveland, OH.
The author declares no conflict of interest.
Reprints: George H. Thompson, MD, Rainbow Babies and Children’s
Hospital Division, Pediatric Orthopaedic Surgery, 11100 Euclid
Avenue, Cleveland, OH 44106. E-mail: ght@po.cwru.edu.
Copyright r 2011 by Lippincott Williams & Wilkins
S192 | www.pedorthopaedics.com J Pediatr Orthop
that surgical containment produce better long-term
results than nonsurgical methods of treatment with
respect to sphericity of the femoral head using specific
criteria.1 Associated with improved sphericity will be a
decreased risk for degenerative osteoarthritis in adult-
hood, the major goal of treatment in LCPD.2,3 Surgical
options include: (1) pelvic osteotomies; (2) proximal
femoral varus osteotomies; and (3) combination of both.
This portion of the symposium is dedicated to the Salter
or innominate osteotomy, the most common pelvic
osteotomy performed in LCPD.
PELVIC OSTEOTOMIES
Pelvic osteotomies are classified into 3 categories:
(1) acetabular rotational osteotomies; (2) medial displace-
ment or Chiari osteotomy; and (3) shelf procedures. The
most commonly performed procedures in LCPD are the
acetabular rotational osteotomies, particularly the Salter
osteotomy. Other rotational osteotomies include the
double osteotomy (Sutherland) and triple osteotomies
(Steel, Tonnis). The Chiari medial displacement osteot-
omy and the shelf procedures are usually used for salvage
of a deformed femoral head. Any pelvic osteotomy can be
combined with a proximal femoral osteotomy (varus 110
to 115 degrees), particularly if the femoral head that can
not be contained by a pelvic or proximal femoral varus
osteotomy alone. The shelf procedure can also be
combined with an acetabular rotational osteotomy, such
as the Salter osteotomy, to gain further lateral coverage of
the femoral head.4
The indications for acetabular rotational osteo-
tomies in LCPD, including the Salter osteotomy, are the
same as for any form of containment treatment.5,6 These
include: (1) 6 to 10 years of age at clinical onset (perhaps
5 y in girls); (2) greater than one-half capital femoral
epiphysis (CFE) involvement [Catterall groups III and
IV,7 Salter-Thompson group B,8 and lateral pillar groups
B, B/C border, and C]9; and (3) loss of femoral head
containment (subluxation) on the anteroposterior radio-
graph. The general prerequisites include: (1) a good to full
range of hip motion preoperatively, especially abduction;
(2) no residual irritability; and (3) a minimally deformed
femoral head.
SALTER OSTEOTOMY
The Salter or innominate osteotomy in LCPD is
performed as described for developmental dislocation of
the hip.10 It is designed to gain anterior and lateral
coverage of the femoral head by acetabular rotation.11,12
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J Pediatr Orthop  Volume 31, Number 2 Supplement, September 2011
The anterior and anterolateral region of the hip are the
areas of greatest stress concentration. Containment is
never absolute, as the femoral head is larger than the
acetabulum. The improved coverage is gained at the
expense of posterior coverage of the femoral head.
Although difficult to visualize radiographically, this
osteotomy also displaces the acetabulum 1 to 1.5 cm
medially, thereby decreasing the biomechanical compres-
sion forces across the hip joint.11,12 It also displaces the
femoral head distally by a similar amount improving the
commonly associated lower extremity length discre-
pancy.13 This osteotomy depends on rotation occurring
at the pubic symphysis. Postoperatively, it is desirable to
maintain hip motion to minimize stiffness and enhance
remodeling. The use of crutches and partial weight
bearing in reliable children is recommended. Once
complete healing has occurred the internal fixation
devices, usually 2 or 3 threaded Steinmann pins, can be
removed. The child is then allowed to return to normal
activities, including sports, as tolerated.
Technical Points
The Salter osteotomy is a relatively easy procedure
to perform but requires a precise understanding of its
various steps to achieve the desired results. As a
consequence, appropriate training and experience should
be obtained before attempting this osteotomy or any
other acetabular rotational osteotomy. Some of the key
technical points are as follows:
 The oblique skin incision must be centered half way
between the anterior superior iliac spine and the greater
trochanter. There is a tendency to perform the incision
more proximally toward the iliac crest. Unfortunately,
the hip joint is more distal. A proximal incision will
make the exposure of the hip capsule and sciatic notch
more difficult.
 Recess the iliopsoas at the musculotendinous junction
at the pelvic brim. This will enhance the opening of the
osteotomy site.
 The osteotomy is made from the greater sciatic notch to
just above the anterior inferior iliac spine. The graft is
obtained from the anterior ilium. Retractors designed
by Rang can aide in the exposure of the sciatic notch
and passage of the wire.14
 Opening the osteotomy is enhanced by the use of 2
large Lewin clamps to control the ilium and the
acetabular segment. The foot on the involved side is
placed on the opposite thigh by positioning the
involved hip in a flexed, externally rotated position.
Extending the hip results in the opening of the
osteotomy. The clamps are used to maintain 1.0 to
1.5 cm of anterior displacement of the acetabular
segment and laterally tilting the iliac segment. It is
important to align all 3 medial cortices (ilium, bone
graft, and acetabular segment).
 A large iliac crest bone graft (35 degree apical angle) is
inserted. Two or 3 large threaded Steinmann pins are
used to secure the 3 segments. The size is determined by
the width of the cancellous bone of the ilium. The
r 2011 Lippincott Williams & Wilkins
Salter Osteotomy in LCP
diameter is large and usually varies between 3.2 and
4.0 mm.
 After wound closure, a small folded towel is placed under
the lumbar spine to restore normal or physiologic
lordosis. The final radiograph will now demonstrate the
typical changes of the osteotomy processFincreased
lateral coverage of the CFE, apparent closure or
narrowing of the obturator foramen, increased
prominence of the ischial spine, and distal displacement
of the femoral head of approximately 1.0 to 1.5 cm.
RESULTS
The results of treatment in LCPD have been difficult
to compare due to a lack of standardization in the
preoperative classification of the extent of CFE involve-
ment (Catterall, Salter-Thompson, or lateral pillar
classifications), the age when treatment was performed,
the postoperative radiographic analysis of the shape of
the femoral head at the completion of the disease process
(Stulberg et al or Mose Circle Criteria), and the lack of
matched control groups.15 Currently, the Stulberg et al3
classification is the most widely used and is the best
predictor of degenerative osteoarthritis in adulthood. In
this classification, class I is a spherical femoral head that
is equal in size to the opposite, uninvolved hip; class II, a
spherical femoral head with coxa magna and coxa breva;
class III, a nonspherical (ovoid) femoral head with a
congruent acetabulum; class IV, a flat femoral head with
abnormalities of the femoral neck and acetabulum; and
class V, a flat femoral head and normal acetabulum.
Thus, class I and II are spherical femoral heads, class III,
nonspherical but oval and congruent hips, and class IV
and V are incongruent hips. Class I, II, and III are usually
considered satisfactory results and class IV and V
unsatisfactory results (Figs. 1A–1H). Whether class III
hips are truly a satisfactory result is a debatable point as
these patients have an increased risk for degenerative
osteoarthritis as an older adult. The Mose2 circle criteria
can also be used but is less popular because it is very
restrictive. The sphericity of the femoral head in the
anteroposterior and lateral radiograph is measured using
a transparent template with concentric circles at 2 mm
intervals. If the sphericity is equal in both projections the
hip is rated “good.” Variance of up to 2 mm is rated
“fair,” whereas a variance of 3 mm or more is rated
“poor.” The good and fair results are considered
satisfactory, whereas poor ratings are unsatisfactory.
Salter Osteotomy Alone
In 1980, Salter16 reported the results of his
“innominate” osteotomy in 110 hips treated over a 15
years period compared with 38 hips treated years earlier
by a weight-relieving sling (noncontainment treatment).
All children in both groups were 6 years of age or older at
clinical onset, had extensive CFE involvement (Catterall
group III or IV), and mild subluxation before the head
became significantly deformed. The radiographic results
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Thompson J Pediatr Orthop  Volume 31, Number 2 Supplement, September 2011

FIGURE 1. A, Anteroposterior radiograph of a 6.5-year-old boy with early Legg-Calvé-Perthes disease involving the left hip. There
is Catterall III, Salter-Thompson group B, and lateral pillar group C involvement of the capital femoral epiphysis (CFE). B, Lateral
radiograph demonstrates the intact or viable posterior aspect of CFE. C, Abduction and internal rotation view of the left hip shows
a good range of motion and the ability to contain the left femoral head. D, Anteroposterior radiograph after a left Salter
osteotomy. Observe the prominence of the ischial spine and relative closure of the obturator foramen. E, Two months
postoperatively, the osteotomy is healed and the 2 large internal fixation Steinman pins have been removed. F, One year
postoperatively, the CFE is reossifying and is being well contained. G, Anteroposterior radiograph 11 years postoperatively. The
patient is now 17.5 years of age and skeletally mature. The femoral head is round but there is a mild coxa magna and coxa breva
yielding a Stulberg et al class II result. H, Lateral radiograph.

were assessed by the Mose circle criteria. The radio-
graphic results in the 110 hips were 77% good and 17%
fair or 94% satisfactory results and only 6% poor or
unsatisfactory results. In the 38 hips treated by non-
containment, the results were 37% good and 29% fair or
66% satisfactory results and 34% poor or unsatisfactory.
Salter and Brown17 expanded this series to 159 patients in
1988. Using the same criteria 79% were rated good, 16%
fair (96% satisfactory) and only 4% poor. Clinically,
93% of the patients were asymptomatic.
In 1982, Ingman et al18 compared the results of 38
Salter osteotomies with 33 cases treated by recumbency in
hip spica casts. They used their own clinical criteria and
the Mose circle criteria radiographically to assess their
results. They reported satisfactory results (good and fair)
in 75% of the patients treated by Salter osteotomy

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J Pediatr Orthop  Volume 31, Number 2 Supplement, September 2011
compared with 82% by conservative treatment. However,
when some of these patients plus additional patients were
reevaluated in 1991 by Paterson et al19 the incidence of
satisfactory results increased to 96%. They felt this
improvement was due to femoral head remodeling during
subsequent growth.
Other studies regarding the Salter osteotomy have
shown a similar incidence of satisfactory clinical and
radiographic results.16,18,20–29 Variations in the original
technique have also been performed to try to improve on
the initial results. Canale et al30 and Cottler and
Donahue31 used a trapezoidal rather than a triangular
shape and reported results similar to the Salter osteo-
tomy. Yoon et al32 described a different iliac osteotomy
to produce similar but more stable orientation of the
osteotomy in LCPD. Sanchez Mesa and Yamhure33
developed a percutaneous technique without the need of
a bone graft.
The recent multicenter study group report on LCPD
reported on 438 patients with 451 involved hips.1 Three
hundred forty-five hips in 337 patients were followed until
skeletal maturity. The inclusion criteria for treatment
were the same as described by Salter.5 There were 68 hips
treated by a Salter osteotomy and 52 hips with a proximal
femoral varus osteotomy. The results between these 2
surgical treatment groups were similar and significantly
improved over no treatment (19 hips), range of motion
(77 hips), and brace treatment (129 hips) using the
Stulberg et al classification. In the Salter osteotomy
group, there were 39 hips (57%) and in the proximal
femoral osteotomy group, there were 34 hips (65%) with
Stulberg et al class I and II results. There were 22 hips
(32%) and 13 hips (25%) with Stulberg et al class III hips,
respectively. Thus, 61 of 68 hips (90%) with a Salter
osteotomy had a satisfactory result, as did 47 of 52 hips
(90%) with a proximal femoral varus osteotomy. The
remaining hips in both groups had class IV or V results.
Other studies have compared Salter osteotomy and
proximal varus osteotomy.34,35 Moberg et al34 in 1997
compared Salter osteotomy and proximal femoral varus
osteotomies and found similar results with respect to
femoral head sphericity. However, this study did show
increased femoral head coverage by the center edge angle
after Salter osteotomy.34 Similar findings were reported
by Kitakoji et al35 in 2005.
Few studies have followed their patients until
skeletal maturity.1,36 In addition to the multicenter
study,1 Ishida et al36 in 2004 reported on 32 patients (37
hips) with LCPD treated with a Salter osteotomy and
followed clinically and radiographically until skeletal
maturity. All hips were classified using the Salter-
Thompson and the Catterall classification. They at-
tempted to divide their patients into those younger than
7 years of age and those 7 years of age and older at
presentation making comparisons with other studies more
difficult. The mean age was 7.7 years (range, 4 to 12.7 y).
This is a different comparison due to inclusion of patients
6 years of age or younger who have an inherently good
prognosis. Overall, age was not a significant factor with
r 2011 Lippincott Williams & Wilkins
Salter Osteotomy in LCP
respect to results according to the Mose circle criteria and
the Stulberg et al ratings. There were 16 hips (43%) rated
Mose good, no fair, and 21 poor results (57%). There
were 11 Stulberg et al class I hips, 5 class II hips, 13 class
III hips, 8 class IV hips, and no class V hips. Thus, there
were 29 hips (78%) with satisfactory results and 8 hips
(22%) with unsatisfactory results using this criteria.
Combined Procedures
The use of a combined Salter and proximal femoral
varus osteotomies in LCPD has been performed more
recently.37–41 They are generally used for patients with an
older age at clinical onset (Z9 y of age), laterally
extruded (subluxated), and deformed femoral heads,
and in those patients in which either osteotomy alone
would not provide adequate containment. The results of
these studies are more standardized and due to their more
recent publication allow appropriate comparative anal-
ysis. Olney and Asher37 in 1985 studied 9 patients at a
mean follow-up of 4.2 years (range, 1.7 to 7.7 y). They
used the clinical criteria of Ratliff42 and the radiographic
criteria of Mose2 and Lloyd-Roberts et al43 in evaluating
their results. The Ratliff criteria includes pain, limited
range of motion, and limp. A patient is rated good if none
of these findings are present, fair if 1 was present, and
poor if 2 or 3 were present. Postoperatively, there were 7
good (78%) and 2 fair (22%) clinical results. The 2
patients with fair results had a persistent limp due to
abductor muscle weakness from greater trochanteric
overgrowth. No patient had significant pain or a
functional loss of hip motion. By the Mose circle criteria,
there were 5 satisfactory (3 good and 2 fair) and 4 poor or
unsatisfactory results. The criteria adapted from Lloyd-
Roberts et al43 improved the radiographic results to 8%
or 89% satisfactory results (4 good and 4 fair) and only 1
poor or unsatisfactory results. A good result implies the
femoral head is round, congruous, and has minimal loss
of epiphyseal height. A fair result indicates the femoral
head is not completely spherical but congruous, had no
more than one fifth of the femoral head uncovered, and a
mild loss of epiphyseal height. A poor result had obvious
flattening of the femoral head with loss of congruity,
greater than one fifth lateral extrusion, and secondary
acetabular changes. Similar results were found by
Crutcher and Staheli38 in 1992. They studied 14 patients
using the same preoperative and postoperative clinical
and radiographic criteria with the exception they also
included the Stulberg et al3 rating in their postoperative
radiographic evaluation. The latter demonstrated 7 class
II hips, 6 class III hips (93% satisfactory), and only 1
class IV hip at a mean follow-up of 8 years (range, 5 to
10.5 y). This indicated a significant salvage rate of hips
with a potentially poor prognosis. They observed that 11
of 14 hips had documentable improvement in femoral
head sphericity with growth due to remodeling. Sarassa
et al39 in 2008 reported on 10 older patients treated
with combined osteotomies. Preoperatively there were 4
Catterall III hips and 6 Catterall IV hips. At a mean
follow-up of almost 4 years, there was 1 Stulberg et al
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Thompson J Pediatr Orthop
class I hip, 5 class II hips, 3 class III hips, and only 1 class
IV hip. Most recently, Javid and Wedge40 in 2009
published their results on combined osteotomies in 20
older patients with LCPD. All had the clinical onset of 8
years of age or older including 6 patients who were 10
years of age or older. Surgery was performed at 9 years of
age or older. All had lateral subluxation of the femoral
head and were followed until skeletal maturity. They used
the lateral pillar classification preoperatively and the
Stulberg et al rating at maturity. There were 11 lateral
pillar B, 7 lateral pillar B/C, and only 2 lateral pillar C
hips preoperatively. At skeletal maturity, there were 6
Stulberg et al class II hips, 9 class III hips, and 5 class IV
hips. There were no class I or V hips. The lateral pillar
classification was not particularly predictive of results.
They felt that the results of the combined osteotomies at
skeletal maturity were improved compared with natural
history of untreated hips. Vukasinovic et al41 in 2000
evaluated patients treated with a combined Salter
osteotomy and a proximal femoral shortening osteotomy.
They found similar results, including a better center-edge
angle, in these patients. These results were similar to those
of Moberg et al34 and Kitakoji et al.35
DISCUSSION
The results of the Salter osteotomy have been
demonstrated to alter the natural history of untreated
LCPD. The previous published studies indicate improved
sphericity of the femoral head. This may be related to the
continuous containment of the femoral head compared
with short-term containment for individuals treated with
a proximal femoral varus osteotomy that will correct with
growth. The persistent containment enhances remodeling
of the femoral head, which continues until skeletal
maturity. Improved femoral head blood supply has
demonstrated by Shim et al44 in dogs after a Salter
osteotomy may shorten the healing process. Grzegor-
zewski et al45 observed more normalization of the
acetabulum after surgical containment than with non-
surgical treatment. They concluded that this contributes
considerably to the remodeling of the femoral head. With
the improved sphericity of the femoral head at maturity,
there should be a decreased risk for degenerative
osteoarthritis in adulthood. The effects of the osteotomy
with the exception of the improved acetabular coverage of
the femoral head resolved with time.34,35,46,47 Saleh et al46
demonstrated that the changes in the obturator foramen
resolve within 6 months. Loder47 found only a slight
change in obstetric pelvimetry in female patients after
pelvic osteotomies as mean follow-up of 8.5 ± 5.2 years.
The advantages of the Salter osteotomy and other
acetabular rotational osteotomies compared with a
proximal femoral varus osteotomy include: (1) better
anterior and lateral coverage of the femoral head; (2) it
does not result in further shortening of the femoral neck
(coxa breva); (3) it increases the length of the involved
extremity by approximately 1 cm rather than further
shortening; (4) it will improve the Trendelenberg gait; (5)
S196 | www.pedorthopaedics.com
 Volume 31, Number 2 Supplement, September 2011
easier metal removal; and (6) no risk for pathologic
fracture through 7 holes in the proximal femur.5,6
However, the Salter osteotomy require extensive experi-
ence to be performed satisfactorily. These are technically
demanding procedures. Combined procedures can even
be more challenging.
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36. Ishida A, Kuwajima SS, Laredo Filho J, et al. Salter innominate
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38. Crutcher JP, Staheli LT. Combined osteotomy as a salvage
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39. Sarassa CA, Herrera AM, Carvajal J, et al. Early clinical and
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40. Javid M, Wedge JH. Radiographic results of combined Salter
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older children. J Child Orthop. 2009;3:229–234.
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42. Ratliff AHC. Pseudocoxalgia. J Bone Joint Surg. 1956;38-
B:498–512.
43. Lloyd-Roberts GC, Catterall A, Salamon PB. A controlled study of
the indications for and the results for femoral osteotomy in perthes
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44. Shim SS, Day B, Leung G. Circulatory and vascular changes in the
hip following innominate osteotomy: an experimental study. Clin
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45. Grzegorzewski A, Synder M, Kozlowski P, et al. The role of the
acetabulum in Perthes disease. J Pediatr Orthop. 2006;26:316–321.
46. Saleh JM, O’Sullivan ME, O’Brien TM. Pelvic remodeling after
Salter osteotomy. J Pediatr Orthop. 1995;15:342–345.
47. Loder RT. The long-term effect of pelvic osteotomy on birth canal
size. Arch Orthop Trauma Surg. 2002;122:29–34.
www.pedorthopaedics.com | S197

O. Wiig,
T. Terjesen,
S. Svenningsen
From Ullevål
University Hospital,
Oslo, Norway
! O. Wiig, MD, Orthopaedic
Surgeon
Orthopaedic Centre
Ullevål University Hospital,
NO-0407 Oslo, Norway.
! T. Terjesen, MD, PhD,
Professor of Orthopaedic
Surgery
Orthopaedic Department
Rikshospitalet University
Hospital, NO-0027 Oslo,
Norway.
! S. Svenningsen, MD, PhD,
Orthopaedic Surgeon
Orthopaedic Department
Sørlandet Hospital, Arendal,
4809 Arendal, Norway.
Correspondence should be sent
to Dr O. Wiig; e-mail:
ola.wiig@ulleval.no
©2008 British Editorial Society
of Bone and Joint Surgery
doi:10.1302/0301-620X.90B10.
20649 $2.00
J Bone Joint Surg [Br]
2008;90-B:1364-71.
Received 21 December 2007;
Accepted after revision 27 May
2008
1364
Prognostic factors and outcome of treatment
in Perthes’ disease
A PROSPECTIVE STUDY OF 368 PATIENTS WITH FIVE-YEAR
FOLLOW-UP
This nationwide prospective study was designed to determine prognostic factors and
evaluate the outcome of different treatments of Perthes’ disease.
A total of 28 hospitals in Norway were instructed to report all new cases of Perthes’
disease over a period of five years and 425 patients were reported and followed for five
years. Of these, 368 with unilateral disease were included in the present study. The hips
were classified radiologically according to a modified two-group Catterall classification and
the lateral pillar classification. A total of 358 patients (97%) attended the five-year follow-up,
when a modified three-group Stulberg classification was used as a radiological outcome
measure. For patients over six years of age at diagnosis and with more than 50% necrosis of
the femoral head (152 patients), the surgeons at the different hospitals had chosen one of
three methods of treatment: physiotherapy (55 patients), the Scottish Rite abduction
orthosis (26), and proximal femoral varus osteotomy (71). Of these hips, 146 (96%) were
available for the five-year follow-up.
The strongest predictor of outcome was femoral head involvement of more or less than
50% (odds ratio (OR) = 7.76, 95% confidence interval (CI) 2.82 to 21.37), followed by age at
diagnosis (OR = 0.98, 95% CI 0.92 to 0.99) and the lateral pillar classification (OR = 0.62, 95%
CI 0.40 to 0.98). In children over six years at diagnosis with more than 50% of femoral head
necrosis, proximal femoral varus osteotomy gave a significantly better outcome than
orthosis (p = 0.001) or physiotherapy (p = 0.001). There was no significant difference
between the physiotherapy and orthosis groups (p = 0.36), and we found no difference in
outcome after any of the treatments in children under six years (p = 0.73).
We recommend proximal femoral varus osteotomy in children aged six years and over at
the time of diagnosis with hips having more than 50% femoral head necrosis. The abduction
orthosis should be abandoned in Perthes’ disease.
The treatment of Perthes’ disease has been con- studies on treatment are retrospective, with a
troversial since its description almost simultane- relatively small number of patients and without
ously by Legg,1 Waldenström,2 Calvé3 and a control group. There is a lack of uniformity
Perthes.4 It includes casts, braces, bed rest, concerning the selection of patients, indications
weight-bearing, non-weight-bearing, physio- for treatment, criteria for evaluation and age
therapy, soft-tissue releases, femoral osteotomy, groups. To our knowledge, there has hitherto
pelvic osteotomy and combinations of the been only one multicentre, prospective study
above. The results of containment of the fem- comparing methods of treatment.18
oral head were first reported by Petrie and In 1996, the Norwegian Paediatric Ortho-
Bitenc.5 Containment of the vulnerable and bio- paedic Society started a nationwide prospective
logically plastic femoral head in the acetabulum study on Perthes’ disease in order to gain more
early in the disease was believed to result in a knowledge of its epidemiology, aetiology19 and
more spherical head during the repair process, treatment. A total of 425 children were fol-
thereby resulting in a more congruent joint.6 lowed clinically and radiologically for five
Non-operative containment treatment has years. They underwent one of three treatments
involved different braces,7-11 whereas surgical involving either physiotherapy, the Scottish Rite
containment includes varus osteotomy of the abduction orthosis,10 or femoral varus osteot-
proximal femur,12-14 or various types of innom- omy. The aims of our study were to determine
inate osteotomy15-17 to redirect the acetabulum prognostic factors and evaluate the outcome of
and thereby improve femoral head cover. Most the three forms of treatments.
THE JOURNAL OF BONE AND JOINT SURGERY
 PROGNOSTIC FACTORS AND OUTCOME OF TREATMENT IN PERTHES’ DISEASE
Patients and Methods
This was a prospective multicentre study whereby all 28
hospitals with paediatric orthopaedic services (six univer-
sity hospitals, 16 county and six local) throughout Norway
were instructed to report all new cases of Perthes’ disease
presenting between 1996 and 2000. The diagnosis was
made by the local orthopaedic surgeon on the clinical and
radiological findings. Recruitment was through informed
consent, and the study was approved by the Norwegian
Data Inspectorate and the Norwegian Directorate of
Health and Social Affairs.
Demographic and clinical data were recorded by the
treating surgeon at diagnosis and submitted to the study
group for registration. In order to minimise loss to follow-
up, repeated requests were made by mail and telephone to
the treating surgeons. Bilateral cases were excluded.
At five-year follow-up, the patients and their parents were
asked about the level of function. Walking distance was con-
sidered normal if the child could walk 5 km; otherwise it
was classed as reduced. Sporting activity was classified into
four categories: normal, participation in all activities but
with reduced function, activities such as swimming only and
no participation. Patients who missed the five-year follow-
up were contacted by telephone and radiographs obtained
by direct request to the different hospitals.
After initial assessment the radiographs at diagnosis
and at one and five years were submitted to the study
group, where one of the authors (SS) recorded the classifi-
cation, the relevant measurements and any other changes
on all radiographs. The inter-observer reliability of the
classification and measurements was assessed both within
the study group and between members of the group and
the treating surgeons. We obtained adequate reliability
using our two-group version of the Catterall
classification20 (weighted κ 0.62), the lateral pillar
classification21 (κ 0.70), femoral head cover (intraclass
correlation coefficient 0.95), and the three-group Stulberg
classification (κ 0.70), when assessed by experienced
examiners.22,23
The radiological phases were determined at diagnosis
and characterised by differences in epiphyseal height, width
and structure compared with the normal hip. The fragmen-
tation phase included hips where the necrotic bone
was partly or totally resorbed. Hips with obvious signs of
re-ossification were classified as such.
Based on anteroposterior and Lauenstein projections,24
the diseased hips were classified according to the original
four groups of Catterall.20 Based on this, we combined
groups 1 and 2 (less than 50% necrosis of the femoral
head), and 3 and 4 (more than 50% necrosis), thereby cre-
ating a simpler two-group classification. We also used the
lateral pillar classification of Herring et al,21 whereby hips
were divided into group A, hips with no height reduction of
the lateral pillar of the femoral head; B, with more than
50% height of the lateral pillar maintained; and C, less than
50% height maintained.
VOL. 90-B, No. 10, OCTOBER 2008
1365
The femoral head cover was calculated as the percentage
of that of the femoral head medial to Perkins’ line com-
pared to the width of the femoral head, both measured
parallel to Hilgenreiner’s line.22
The articulotrochanteric distance was measured as the dis-
tance between two lines perpendicular to Perkins’ line, one
through the proximal aspect of the greater trochanter and the
other through the most proximal aspect of the femoral head.
At the five-year follow-up the hips were classified accord-
ing to Stulberg et al,13 as modified by Neyt et al,25 whereby
class I hips are spherical with a normal femoral head, neck
and acetabulum. Class II heads are spherical, with either
coxa magna, a short neck or a steep acetabulum. Class III
hips have ovoid femoral heads and do not fit within 2 mm of
the Mose26 concentric circles in either anteroposterior or
Lauenstein projections. Class IV hips have flat outlines of the
femoral head (at least one-third of the contour of the femoral
head resembles a straight line on at least one projection), but
there is congruency between the femoral head and the
acetabulum. Class V hips have flat femoral heads and a
normal acetabulum (aspherical incongruency).
The hips were also classified according to a three-group
classification23 where group A hips (Stulberg I and II) have a
spherical femoral head, group B (Stulberg III) have an ovoid
femoral head, and group C (Stulberg IV and V) have a flat
femoral head.
All children with less than 50% femoral head necrosis
(Catterall groups 1 or 2) received physiotherapy alone. In
those hips with more than 50% involvement (Catterall
groups 3 or 4), the treatment was dependent on age at diag-
nosis and femoral head coverage. Children six years or older
at diagnosis, received either physiotherapy, Scottish Rite
orthosis, or proximal femoral varus osteotomy, according to
the choice of the local orthopaedic surgeons. These decisions
were based on surgeons’ preferences, treatment philosophy
and local tradition. All patients from the same hospital were
treated by the same method. The number of hospitals choos-
ing physiotherapy, orthosis and osteotomy were 10, 9 and 9,
respectively. Children with hips in the radiological re-
ossification (new-bone formation in the epiphysis) or healing
(fully rebuilt hips where the epiphysis was completely ossified)
phase of the disease received physiotherapy only.
In children under six years at diagnosis, treatment was
dependent on femoral head cover and radiological phase of
the disease. Children whose femoral head cover was good
(> 80%) were treated by physiotherapy alone. Those with
reduced cover (< 80%) in the early or fragmentation phase
were allocated to the treatment groups above, depending
on the hospital where they were treated. The flow of
patients through the different phases of the study is shown
in Figure 1.
Physiotherapy consisted of range of movement exercises
with special emphasis on abduction, internal rotation and
extension, in addition to muscle strengthening exercises.
The Scottish Rite abduction orthosis comprised a pelvic
band, single-axis hinge joints, thigh-lacers, and an
1366 O. WIIG, T. TERJESEN, S. SVENNINGSEN

Enrolment Allocation Five-year follow-up

Physio
Physio
n = 51
n = 55 Lf-up: n = 4

Orthosis Orthosis
> 6 years
n = 26 n = 25
n = 152
Lf-up: n = 1

Osteotomy
Osteotomy n = 70
n = 71
> 50% Lf-up: n = 1
necrosis
n = 323
Physio Physio
n = 126 n = 123
Lf-up: n = 3

Unilateral < 6 years Orthosis

cases n = 171 n = 22
Perthes’
disease n = 368
n = 425
Osteotomy
n = 23

Physio
Exclusions: n = 39
< 50% Physio
Bilateral cases, n = 55 Protocol departure, n = 6:
necrosis n = 38
Reoss. phase, n = 1 Orthosis, n = 1
n = 45 Lf-up, n = 1
Refused consent, n = 1 Osteotomy, n = 5

Fig. 1

Flowchart of 425 patients with Perthes’ disease (n, number of patients; Reoss. phase, hip in reossification phase; Physio, treatment with physiotherapy;
Orthosis, treatment with the Scottish Rite abduction orthosis; Osteotomy, treatment with proximal femoral varus osteotomy; Lf-up, lost to follow-up).

abduction bar that could piston on itself and was con-
nected to the medial part of the thigh cuff by an eccentric
ball-and-socket joint. Hip abduction had to exceed 35° on
the affected side in order to use the orthosis, in which both
hips were abducted approximately 40° with slight flexion.
The orthosis was worn all day and night, except for bath-
ing or swimming (maximum one hour daily), and the
treatment was terminated when there were signs of new
bone formation on both anteroposterior and Lauenstein
projections.
Proximal femoral varus osteotomy was either subtro-
chanteric with a pre-bent plate or intertrochanteric with a
paediatric blade plate, aiming for a neck-shaft angle of
approximately 110° to 115°. Slight external rotation of the
femoral shaft was performed in cases of increased femoral
anteversion. Post-operative immobilisation in spica cast
was at the surgeon’s discretion.
The patients had clinical and radiological follow-up at
one, three and five years after diagnosis. Those undergoing
femoral varus osteotomy were also reviewed two months’
post-operatively.
Statistical analysis. This was performed using SPSS version
13.0 (SPSS Inc., Chicago, Illinois) and SAS (Statistical
Analysis System, version 9.1.3, Cary, North Carolina). For
the evaluation of risk factors regarding outcome, all vari-
ables were run in simple models, only estimating the effect of
single variables one at a time. Outcome was assessed using
the modified three-group Stulberg classification.23 The cate-
gorical data were analysed by Pearson’s chi-squared test and
continuous variables using a one-way analysis of variance
(ANOVA). A multinomial proportional odds logistic regres-
sion analysis was performed with the three-group Stulberg
classification as outcome. The assumption of proportionality
was assessed with a score test. Variables significant at the
0.25 level were included in the multivariate analysis. Vari-
ables were excluded from the multivariate model one at a
time using a backwards stepwise procedure. All variables
removed were tested for confounding factors.27 The odds
ratio (OR) in this setting is defined as the ratio for being in
the same or a lower Stulberg category, given that the value of
the covariate is reduced by one unit. A mixed-model
repeated-measures ANOVA was performed to assess the

THE JOURNAL OF BONE AND JOINT SURGERY

 PROGNOSTIC FACTORS AND OUTCOME OF TREATMENT IN PERTHES’ DISEASE 1367

Table I. Prognostic factors other than treatment associated with radiographic outcome

Stulberg three-group classification

A B C
Variables Number Number (%) Number (%) Number (%)
Two-group Catterall classification
1 44 37 (84) 7 (16) -
2 314 139 (44) 115 (37) 60 (19)

Age at diagnosis (yrs)

<6 197 113 (57) 63 (32) 21 (11)
≥6 161 63 (39) 59 (37) 39 (24)

Lateral pillar classification

A 33 23 (70) 8 (24) 2 (6)
B 262 134 (51) 82 (31) 46 (18)
C 63 19 (30) 32 (51) 12 (19)
In the Stulberg 3-group classification, group A has a spherical, group B an ovoid, and group C
a flat femoral head. In the two-group Catterall classification, 1 is < 50% femoral head necrosis
and 2 is > 50%. In the lateral pillar classification, group A has no height reduction of the lateral
pillar, group B has > 50% of the lateral pillar maintained, and group C has < 50% maintained

change in femoral head cover over time. The underlying
assumptions were checked using Studentised residuals,
Cook’s d and the Covratio statistics. No indications of any
abnormalities were found. All p-values below 0.05 were con-
sidered significant.
Results
There were 324 boys (76%) and 101 girls enrolled in the
study. The mean age at diagnosis was 5.8 years (1.3 to 15.2)
and there was no significant difference in mean ages
between boys (5.8) and girls (5.9). There were 370 uni-
lateral and 55 (13%) bilateral cases. The latter were
excluded along with one patient in the re-ossification phase
and another who refused consent. Of the 368 children
remaining, the left side was affected in 201 (55%) and the
right in 167. A total of 220 (60%) children had been
treated with physiotherapy, 99 (27%) by osteotomy and 49
(13%) with orthosis.
There were 358 patients (97%) who attended the five-
year clinical follow-up examination. Of these, 83 (23%)
had pain or discomfort, mostly in the groin or thigh, and 83
(23%) had a limp. There was no limitation in walking dis-
tance in 304 (85%), whereas 54 (15%) were unable to walk
5 km before they had pain. A total of 269 (75%) patients
said that they could participate in all sports, 79 (22%) had
reduced function, ten (3%) could participate in swimming
only, and one was unable to do any sport. We found a
strong association between sporting and walking ability, as
263 (87%) of the patients with a normal walking distance
could participate in all sports, compared to five (9%) of
those with reduced walking ability (p < 0.0001). There
were no significant differences in walking ability and level
of sporting activity between the treatment groups.
Radiographs of 358 patients were available at the five-
year follow-up. These showed that 176 patients (49%)
were classified as Stulberg groups I or II, 122 (34%) were in
group III, and 60 (17%) in groups IV or V. We found a
strong association between sporting and walking ability in
relation to Stulberg outcome, as hips classified in groups IV
and V had a more limited walking distance (p < 0.001) and
reduced sporting ability (p = 0.002).
There was a highly significant association (p < 0.001)
between the modified two-group Catterall classification and
radiographic outcome at the five-year follow-up (Table I). Of
44 hips, 37 (84%) with femoral head involvement less than
50% resulted in Stulberg groups I to II, whereas none were
in group IV or V. The outcome was worse in hips with more
than 50% femoral head necrosis, where 60 of 314 (19%)
were IV or V. Because of this markedly better prognosis in
hips with femoral head involvement less than 50% they
were omitted from the comparison of treatment methods.
Age at diagnosis was strongly associated with outcome:
the younger the patient at diagnosis, the better the out-
come, as shown in Table I, where the outcome in children
under six years of age was markedly better than in those
aged six and older (p < 0.0001).
We found a significant association (p = 0.001) between
the lateral pillar classification and Stulberg outcome, as
70% of hips classified as A had spherical femoral heads at
five-year follow-up, compared to 51% of B hips and 30%
of C hips (Table I). There was a similar association when
the lateral pillar classification was applied one year
after diagnosis.
The multinomial proportional odds logistic regression
analysis showed that the modified two-group Catterall
classification was the strongest prognostic factor
(OR = 7.76, 95% CI 2.82 to 21.37), followed by age
at diagnosis (OR = 0.98, 95% CI 0.92 to 0.99) and the
lateral pillar classification at the time of diagnosis
(OR = 0.62, 95% CI 0.40 to 0.98, Table II).

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1368 O. WIIG, T. TERJESEN, S. SVENNINGSEN

Table II. Multinomial proportional odds logistic regression

analysis (odds ratio estimates) of prognostic factors in
Perthes’ disease

Prognostic factor OR* 95% CI†

Two-group Catterall classification 7.76 2.82 to 21.37
Age at diagnosis 0.98 0.92 to 0.99
Lateral pillar classification 0.62 0.40 to 0.98
* OR, odds ratio
†
95% CI, 95% confidence interval

Table III. Radiographic outcome in relation to treatment groups in children with more
than 50% femoral head necrosis

Stulberg three-group classification

A B C
Age at diagnosis Treatment Number (%) Number (%) Number (%) Total
≥ 6 years Physiotherapy 17 (33) 14 (27) 20 (40) 51
Orthosis 5 (20) 9 (36) 11 (44) 25
Osteotomy 30 (43) 32 (46) 8 (11) 70
Total 52 (35) 55 (38) 39 (27) 146

< 6 years Physiotherapy 65 (53) 41.0 (33) 17 (14) 123

Orthosis 10 (45.5) 10 (45.5) 2 (9) 22
Osteotomy 12 (52) 9 (39) 2 (9) 23
Total 87 (52) 60 (36) 21 (12) 168

There was no significant association between radio-
logical outcome and the following factors: articulotrochan-
teric distance, gender, county where the patient lived and
duration of symptoms at diagnosis (p > 0.05). The femoral
head coverage at diagnosis was not significantly associated
with radiographic outcome (p = 0.14). After one year the
cover was significantly lower (p = 0.001) in Stulberg classes
IV and V compared to Stulberg classes 1 and 2 (mean cover
79% and 87%, respectively). The femoral head cover in
patients treated with physiotherapy decreased during the
first years of the disease. The mean at diagnosis was 92%
(95% CI 90.8 to 93.2) and decreased by 9% (95% CI 6.7 to
11.6) to 83% (95% CI 81.7 to 84.8) after one year
(p < 0.0001). Three years after diagnosis the femoral head
cover decreased by 4% (95% CI 2.0 to 5.8) to a mean of
79% (95% CI 77.8 to 80.9), which was significantly less
than at one year (p < 0.0001). From three to five years there
was a slight increase in mean cover to 80%, but the
difference was not significant (p = 0.38).
Of the children over six years of age with more than 50%
femoral head necrosis, 146 of 152 (96%) were available for
the five-year follow-up. The number of patients according
to treatment modality was 51 (34%) for physiotherapy,
25 (17%) for orthosis and 70 (48%) for osteotomy (Fig. 1).
The three treatment groups were similar as regards
demographic and clinical data such as age at diagnosis,
gender, duration of symptoms, side affected, presence of a
limp, and pain at diagnosis (all p-values > 0.26). There was
a significantly lower femoral head cover (p = 0.03) at diag-
nosis in the osteotomy group (cover 85%, 95% CI 79.6 to
90.1) than in the orthosis group (92%, 95% CI 86.8 to
96.0), but no significant differences between osteotomy
and physiotherapy (cover 87%, 95% CI 84.6 to 90.3,
p = 0.56) or orthosis and physiotherapy (p = 0.23).
The distribution of hips according to treatment groups
and the modified three-group Stulberg classification is
shown in Table III. Proximal femoral osteotomy obtained
the best radiological results compared to both orthosis
(p = 0.001) and physiotherapy (p = 0.001). There was no
significant difference in outcome between the physio-
therapy group and the orthosis group (p = 0.36).
Of those children under six years with more than 50%
femoral head necrosis, 168 of 171 were available for the
five-year follow-up; 123 (73%) had been treated with
physiotherapy, 22 (13%) with an orthosis and 23 (14%)
with osteotomy (Fig. 1). Radiological outcome is shown in
Table III, and there was no significant difference between
the groups (p = 0.73).
Of those children with less than 50% femoral head necro-
sis regardless of age, 38 of 45 (84%) were treated with
physiotherapy, five (11%) by osteotomy and one (2%) with
an orthosis as a result of a departure from the protocol,

THE JOURNAL OF BONE AND JOINT SURGERY

 PROGNOSTIC FACTORS AND OUTCOME OF TREATMENT IN PERTHES’ DISEASE
probably because the treating surgeons considered these
hips to be in Catterall group 3 rather than group 2. One
child was lost to follow-up. Statistical analysis comparing
treatments could not be performed reliably in this group.
Discussion
Our study was similar to the randomised surgeon design
described by Rudical and Esdaile28 and Herring et al,18 in
which surgeons and hospitals were assigned to the treat-
ment of their choice. Before the study, orthopaedic sur-
geons at each hospital were allowed to choose one of the
three methods according to their preference and local tradi-
tion. We believe this contributed to the elimination of the
patient selection bias and enhanced surgeon compliance.
The study design also eliminated performance bias, as the
surgeons at each hospital chose the treatment with which
they were most familiar, usually their established treatment
for Perthes’ disease. When the study began in 1996, there
was no prospective randomised study evaluating outcome.
The studies available were mostly retrospective case series,
and no firm conclusion could be drawn regarding the effi-
cacy of any treatment. Consequently, we did not think that
offering one of the three treatment methods to patients with
a less favourable prognosis posed an ethical dilemma.
Children with bilateral disease were excluded because
there is evidence to suggest that they have a more benign dis-
ease,29 and that inclusion might positively skew our results.
At the five-year follow-up, 15% of the patients had clo-
sure of one or both of the triradiate cartilages, and 14%
had closure of one or both femoral head physes. Therefore,
a significant proportion were skeletally immature. How-
ever, all hips were completely healed at the last follow-up. A
spherical femoral head (Stulberg class I or II) at the time of
healing may theoretically become slightly oval by skeletal
maturity if there were a partial physeal arrest, thereby
moving the hip to class III. However, we believe that such a
physeal arrest would occur during the disease, deforming
the femoral head before healing. In agreement with
others,11,30,31 we think each patient can be reliably assigned
to a Stulberg class even if the hips are not skeletally mature.
As did Herring et al,18 we found no significant differ-
ences in walking distance and level of sporting activity
between the treatment groups. However, there was a
significant association between Stulberg outcome after five
years and level of activity, as the radiologically worst hips
had the lowest functional level. Longer term follow-up
using walking tests, validated activity questionnaires and
pain scales is required to better understand the level of
association between radiological and clinical outcomes.
Catterall20 found a correlation between his four-group
classification system and the radiological outcome. We and
others32 support this. However, insufficient inter-observer
reliability with the Catterall classification has been
reported,33,34 and Herring et al18 abandoned the classifica-
tion for this reason. We do not entirely agree, because in a
previous study we found that the inter-observer reliability
VOL. 90-B, No. 10, OCTOBER 2008
1369
of the Catterall classification was adequate (κ value 0.62)
when performed by experienced observers.22 With less
experienced examiners, the modified two-group classifica-
tion, with 50% involvement of the femoral head as the
dividing line, was more reliable. In this study the two-group
classification was the strongest prognostic predictor
according to the multinomial proportional odds logistic
regression analysis. Therefore, we recommend the
classification in clinical practice.
Most long-term studies conclude that patient age at diag-
nosis is an important prognostic factor, as younger patients
have a better outcome,13,20,26,35,36 and this was confirmed
by our results. It is believed that the younger the child at the
onset of disease, the more time is available for remodelling
after healing. The ability of the acetabulum to remodel and
conform to the shape of the femoral head seems to diminish
after the age of eight years.37 Herring et al18 found that chil-
dren older than this had worse results than those younger
than eight years. In our study, age at diagnosis was the
second strongest predictor of outcome.
Although a young age is a predictor of good outcome,
studies with a different experience have been published.
Snyder38 found that 32% of patients aged five years or
younger at onset and Catterall hips 3 or 4 had radio-
logically poor results. Likewise, Fabry, Fabry and Moens39
reported 48% poor results according to a modified Stulberg
classification, and Schoenecker, Stone and Capelli40
reported that 24% of children under six years of age with
hips in Catterall groups 3 or 4 had poor results. Our study
did not support these findings, as the poor results in this
group were only 12%, the same as those reported by
Rosenfeld et al.30
As with some previous studies,36-41 we found a signifi-
cant association between the lateral pillar classification and
radiological outcome. Herring et al18 reported that this
classification was the strongest predictor of outcome. Our
results did not confirm this, as the multinomial odds logis-
tic regression analysis showed that this was the third stron-
gest predictor, after the modified two-group Catterall
classification and age at diagnosis.
Increasing degree of femoral head uncovering at diagno-
sis has been linked to a poor prognosis.32,42,43 This was not
supported by our results, as the femoral head cover at
diagnosis was not associated with the radiological out-
come. However, there was a strongly significant association
between cover one year after diagnosis and the five-year
outcome, indicating that good femoral head cover during
the disease predisposes to favourable long-term results,
thereby supporting the containment concept of Salter.6
Catterall20 found that girls had a worse prognosis than
boys, which is confirmed by others.21,32,42 It has been
assumed that this is due to earlier skeletal maturity in girls,
hence a shorter time for remodelling. However, we could
not confirm any relationship with gender.
There was no significant association between articulo-
trochanteric distance at diagnosis and Stulberg outcome.
1370 O. WIIG, T. TERJESEN, S. SVENNINGSEN
Although we obtained good inter-observer reliability using
this measurement,22 it does not seem to be of prognostic
significance.
Herring et al18 reported 51% of the hips in Stulberg
classes I and II, 34% in class III, and 15% in classes IV and
V in children between six and 12 years at diagnosis, treated
with or without operation. We found fewer hips in classes I
and II (35%), a similar number in class III (38%), and more
in classes IV and V (27%) in this age group. Thus, our over-
all results in patients aged over six years were inferior to
those of Herring et al.18
Along with the present study, the only prospective multi-
centre study on the effect of treatment is that of Herring et
al.18 These authors found that children over eight years of
age at diagnosis with hips classified as lateral pillar B or ‘B/C
border’ benefited from surgery rather than bracing or
physiotherapy. Our results are similar, because proximal
femoral varus osteotomy gave significantly better outcomes
than treatment with an orthosis or physiotherapy in children
older than six at diagnosis whose hips had more than 50%
femoral head necrosis. There was no significant difference in
outcome between the orthosis and physiotherapy groups,
which also supports the findings of Herring et al.18
As in our study, several case series have shown favour-
able outcomes after femoral osteotomy.44-46 Some studies
comparing treatment methods show better results from sur-
gery than from other methods,47-49 whereas others have
reported equal results.50-52 However, it is difficult to draw
firm conclusions, as these studies were not prospective,
patient numbers were small and the inclusion criteria,
severity of disease, age groups treated and indications for
surgery varied considerably.
Our results showed that physiotherapy was inferior to
femoral osteotomy. As physiotherapy has to our knowledge
never been proved to have any effect on outcome in Perthes’
disease, we consider this group as representing the natural
history of the disease.
Varying results are reported with the Scottish Rite ortho-
sis. In patients over 5.5 years at diagnosis and with total
epiphyseal involvement, Curtis et al8 reported 84% good or
fair, and 16% poor results according to the Mose25 criteria.
Herring et al18 also had good outcomes with 52% of the
hips in Stulberg classes I and II, and only 17% poor results
in Stulberg class IV and V for patients of all ages. However,
we found that patients over six years at diagnosis with
more than 50% femoral head necrosis obtained inferior
results, with 44% in Stulberg class IV and V. Our results in
this group were worse than those of Martinez et al11 and
Meehan, Angel and Nelson,53 who had 33% and 21% poor
Stulberg outcomes, respectively.
Price, Day and Flynn54 evaluated the psychological
effects of treatment and concluded that patients treated
with bracing had a more significant deficit with regard to
social and sexual behaviour, and academic ability than chil-
dren who had been treated operatively. Also, bracing
created a sense of being different and handicapped. We
think there is sufficient evidence to abandon the orthosis in
the treatment of Perthes’ disease.
Although Catterall20 stated that there was no change
from one group to another during the disease, Dickens and
Menelaus32 and Van Dam et al55 experienced changes in
Catterall groups during the disease when classification had
been applied before the fragmentation stage. Similarly, the
lateral pillar classification was not reliable if applied before
the early fragmentation phase.18 Therefore, it is inadvisable
to decide on treatment at the time of diagnosis. Herring et al21
stated that it was usually possible to determine the lateral
pillar group within six months of onset of the disease.
Joseph et al56 showed that in order for a femoral osteotomy
to succeed, it had to be performed within one year of diag-
nosis. In children treated with physiotherapy we found that
the greatest reduction in femoral head cover was between
the time of diagnosis and the one-year follow-up. This is
probably close to the expected disease progress in untreated
hips. Therefore, we think it reasonable to recommend that
decisions on treatment should be made at the six-month
follow-up, or perhaps earlier if the disorder has already
reached the fragmentation stage at diagnosis.
In conclusion, this study shows that the strongest predic-
tor of outcomes is the modified two-group Catterall classi-
fication, followed by age at diagnosis and the lateral pillar
classification.
We recommend proximal femoral varus osteotomy in chil-
dren above six years of age at diagnosis with hips having more
than 50% femoral head necrosis. The abduction orthosis
should be abandoned in the treatment of Perthes’ disease.
The statistical analyses were performed in collaboration with P. Mowinckel,
MSc.
No benefits in any form have been received or will be received from a com-
mercial party related directly or indirectly to the subject of this article.
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Acta Ortopédica Mexicana 2009; 23(3): May.-Jun: 172-181
Artículo de revisión
Enfermedad de Legg-Calvé-Perthes
Raúl Frías Austria*
Hospital Ángeles del Pedregal
RESUMEN. La enfermedad de Legg-Calvé-Per-
thes (ELCP) es una necrosis de la epífisis femoral
capital en la época de crecimiento, que evoluciona
por etapas consecutivas con diferentes grados de
intensidad y extensión, por sí misma limitada, cau-
sada por isquemia de origen desconocido con evo-
lución compleja, desde su diagnóstico hasta la de-
cisión de tratamiento, que requiere ser entendido,
para que sea eficiente y retarde o evite la apari-
ción de dolor causado por la artrosis que puede
dejar como secuela
Palabras clave: cadera, necrosis, fémur, necro-
sis avascular, osteotomía, isquemia, niño.
Introducción
Definición
Se define como un proceso autolimitado de eventos en
cascada de diferentes grados de necrosis que afecta a la
epífisis femoral capital juvenil, producido por isquemia
de origen desconocido.
Inicia con un período de isquemia, que causará necrosis
de la cabeza femoral inmadura, ésta lleva a su fragmenta-
ción y a fracturas subcondrales por estrés, con que inicia
su período clínico, para pasar a un período de absorción
ósea, reosificación y remodelación de la cabeza femoral,
dejando como secuelas alteraciones de la longitud de la
extremidad, subluxación o deformidades tanto de la cabe-
za femoral como del acetábulo.1
www.medigraphic.com
* Hospital Ángeles Pedregal. Ortopedia Pediátrica.
Dirección para correspondencia:
Raúl Frías Austria
Periférico sur Núm. 3697 Torre Ángeles. Piso 10, Cons. 1010 Col.
Héroes de Padierna Deleg. Magdalena Contreras, CP. 10700,
México, D.F. Tel. 55685546; 55682227 Ext. 4010 y 4011 Fax:
56522835
E-mail: frisat26@gmail.com
172
medigraphic Artemisa
en línea
ABSTRACT. Legg-Calvé-Perthes disease (LCPD)
is a necrosis of the capital femoral epiphysis that
occurs during the growth stage and evolves in con-
secutive stages with varying degrees of intensity
and extension. It is self-limited, caused by ischemia
of unknown origin, and involves a complex course,
from the time of diagnosis to the decision of pro-
viding treatment. The latter needs to be under-
stood for it to be effective and delay or prevent the
occurrence of the pain resulting from the arthrosis
that may occur as a sequela.
Key words: hip, necrosis, avascular necrosis,
osteotomy, ischemia, child.
La enfermedad de Legg-Calvé-Perthes (ELCP) lleva los
nombres de tres médicos de diferentes países que la descri-
bieron casi simultáneamente en 1910; Legg en los Estados
Unidos escribió sobre «una oscura afección de la cadera» de
cinco casos clínicos y radiográficos.2 Calvé en Francia la
describió como «una seudo-coxalgia».3 Y el alemán Perthes
la llamó «artritis deformante juvenil».4 Los tres coincidían
en que no se trataba de una forma de tuberculosis de la ca-
dera y diferían de la opinión de Waldenström que había pu-
blicado la descripción de las caderas de diez chicos con
problemas similares en 1909, llamándola «der obere tuber-
kulöse collumherd»,5 basándose en que todos los pacientes
tuvieron reacciones cutáneas a la tuberculina positivas.
Se le conoce también con el nombre de «Coxa Plana»
por el estudio que publicó Waldeström en 19226 donde
describió la evolución del problema de principio a fin en
22 niños, cuya principal característica fue la pérdida de es-
fericidad de la cabeza femoral, dando a conocer además
una clasificación radiográfica y por este mismo estudio se
le encuentra como «Enfermedad de Waldeström». Otro si-
nónimo es «Necrosis avascular de la cabeza femoral» ya
que Phemister en 19217 publicó la descripción histológica
del padecimiento como una necrosis ósea.
Por la ausencia de algún factor infeccioso también se le
suele llamar «Necrosis aséptica de la cabeza femoral» o de
la «Cadera Infantil o Inmadura». Pero por considerarse que
 Enfermedad de Legg-Calvé-Perthes
el bloqueo vascular es muy importante se le dio el nombre
de «Necrosis isquémica».7,8
Incidencia
La enfermedad de Legg-Calvé-Perthes (ELCP), es un
desorden de la cadera infantil que ataca típicamente entre
los 4 y los 8 años,9 aunque existen reportes de su presencia
desde los 17 meses hasta la maduración esquelética. Se
presenta un caso en cada 4,000 niños, predominando en el
sexo masculino con una relación de 5 niños por cada niña
y en el 10 al 12% su desarrollo es bilateral.10,11
La ELCP, se presenta con mayor incidencia en la po-
blación asiática, predominando en japoneses y esquima-
les, es poco frecuente en la raza negra, en los aborígenes
australianos, en los indígenas de América, así como en la
población de Europa Central,10,11 aunque existen estudios
de que su presencia puede ser diferente en las regiones de
un solo país europeo.12
Etiología
Al revisar la literatura mundial encontramos que se han
realizado muchos estudios que intentan hallar la etiología
de la ELCP sin lograrlo y hasta la fecha continúa siendo
desconocida.8-13,14
Su causa directa es la isquemia de la epífisis femoral
capital que causa necrosis de la cabeza femoral. La ma-
yoría de los autores la asocian con algunos padecimien-
tos que pueden contribuir a la obstrucción del aporte
sanguíneo capital cefálico, como la sinovitis transitoria,
traumatismos, congestión venosa local, aumento de la
viscosidad sanguínea y trombofilia, pero están de
acuerdo en que ocurre un bloqueo de la circulación en
el extremo proximal femoral, sin que ninguna causa es-
tudiada haya podido demostrarse que es la principal de-
terminante.12
La arteria circunfleja medial femoral es la principal
fuente de suplemento sanguíneo para la cabeza femoral en
adultos, pero en los niños este aporte está dado por los va-
sos cervicales laterales ascendentes los que, como demos-
tró Chung,15 son muy vulnerables y fácilmente bloquea-
bles durante el crecimiento, por su posición anatómica en
esta época de la vida.
Factores predisponentes
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Herencia: Algunos autores han mencionado la posibi-
lidad de una mayor asociación de la ELCP entre familia-
res, pero los estudios de Fisher, 16 Wynne-Davies y Gor-
mely17 demostraron que no existe una predisposición
genética o asociación familiar en este padecimiento.
Otros factores: La literatura mundial cita como predis-
ponentes o que facilitan la aparición de la ELCP al creci-
miento rápido desproporcionado y alterado,18 bajo peso al
nacer,19,20 retardo en la maduración esquelética,21 estatura
ACTA ORTOPÉDICA MEXICANA 2009; 23(3): 172-181
corta,18-22 cambios hormonales sistémicos,23,24 tendencia a
la obstrucción hemática o a la formación de trombos san-
guíneos e índice socioeconómico bajo. 24-28 Todos estos
factores se basan en la hipótesis de que un crecimiento y
desarrollo inadecuado o poco armónico puede restringir o
alterar la evolución vascular del fémur proximal, pero
debe asociarse con bajo peso al nacer junto con alteracio-
nes del crecimiento fetal y algunas otras causas al inicio
de la vida para incrementar el riesgo.19,20 Bahmanyar 29 y
otros autores agregan como factor de riesgo al tabaquismo
materno y ambiental durante el embarazo, así como a la
contaminación.30-35
Fisiopatología
Historia natural de la enfermedad
Conocer la historia natural de la enfermedad resulta
muy importante para el médico tratante, ya que el pro-
nóstico del padecimiento dependerá de que la cabeza
femoral permanezca esférica cuando el paciente alcance
la maduración esquelética, así que plantearse el trata-
miento a establecer depende de que el médico sepa qué
le pasará al paciente sin tratamiento y qué factores le
harán tener un resultado adverso. La edad en que inicia
la enfermedad parece tener una influencia importante ya
que si se presenta en menores de 6 años la posibilidad
de tener un mejor resultado es mayor. 9 Pero el tener el
padecimiento por largo tiempo y el gran período para la
maduración esquelética, hacen que se presenten puen-
tes óseos en la fisis, que deforman la cabeza femoral;
también el no realizar la inmovilización adecuada en
los niños mayores que requieren mucha actividad facili-
ta las fracturas de la frágil cobertura cartilaginosa que
aplanan la metáfisis.
Uno de los problemas en conocer la evolución del pa-
decimiento es que no existen estudios adecuados de la his-
toria de la enfermedad, solamente descripciones parciales
a largo plazo como la de Catteral 39 que dividió a los pa-
cientes en: 1. Fase Inicial, en que principia la fragmenta-
ción, es el período en que la cabeza femoral parece estar
deformada. 2. Fase de Curación en que parece ocurrir una
osificación biológica de la cabeza femoral y puede durar
hasta dos años, 3. Fase de Crecimiento en que puede haber
remodelación. Utilizó para la valoración el sistema de gra-
diente de Sundt,59 por un período de 10 años, tiempo que
fue demasiado corto para tener resultados adecuados en el
padecimiento y sus datos no son clasificables como crite-
rios de tratamiento. Stulberg60 también trató de completar
un estudio parecido, sin lograr pronosticar el futuro de la
cadera, para cambiar la alteración en la congruencia arti-
cular que da artrosis en la vida adulta temprana, que lleva
a la necesidad de un reemplazo articular, necesidad que
aumenta conforme avanza la edad y que más frecuente-
mente ocurre cuando la ELCP inicia después de los 8 a 9
años de edad.
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 Raúl Frías Austria
Mose61 desarrolló una escala de esfericidad de la cabe-
za femoral, tratando de pronosticar su futuro que no debe
hacerse antes de que el paciente haya dejado de crecer;61
sin embargo podemos decir en forma general que los
principales factores pronóstico para la mayoría de los au-
tores son: la deformidad de la cabeza femoral, la incon-
gruencia articular de la cadera, edad de inicio, extensión
del involucramiento epifisiario, alteración del crecimien-
to secundario al cierre fisiario prematuro, curso prolon-
gado de la enfermedad, potencial de remodelación, tipo
de tratamiento y fase de la enfermedad en que inició el
tratamiento. 39,59-61
Phemister 7 en 1921 mostró mediante estudios histoló-
gicos que la ELCP es una necrosis ósea de la epífisis fe-
moral capital con diferentes grados de afectación, evolu-
ción y reparación, en que la cobertura del cartílago
articular se encuentra esencialmente sin afectación aun-
que adelgazada y fue confirmado posteriormente en
1928 por Zemansky.36
Los estudios de Waldenström5,6 permitieron dividir para
su comprensión, el curso de la necrosis y reparación en
cuatro estadios característicos: Necrosis (Inicial), Resor-
ción (Fragmentación), Reosificación y Remodelación.
Bowen37 reportó que el tiempo de duración de cada eta-
pa puede variar mucho en cada caso, pero en general la
etapa de necrosis y resorción dura 6 meses, la de reosifica-
ción de 18 meses a tres años y la de remodelación hasta la
maduración esquelética.
Etapa inicial Necrosis
Mediante la realización de biopsias del centro de la
cabeza femoral afectada, Jonsäter38 encontró que en esta
etapa existe isquemia que causa necrosis del hueso y de
la médula ósea; las trabéculas pueden estar destruidas,
aplastadas. Los núcleos de los osteocitos están picnóti-
cos o ausentes, se forma una masa de hueso desorganiza-
do, muerto, que se acumula en la médula ósea sustituyén-
dola, hay zonas ocasionales de hueso sano, pero sin
evidencia de regeneración ósea. Hay un cese temporal de
la osificación endocondral y no hay formación de núcleo
de osificación.
La placa basal de cartílago muestra cambios degenerati-
vos en que predominan áreas en donde hubo necrosis que
llega hasta la unión cartílago-hueso, pudiendo dejar hue-
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cos. La capa de cartílago periférico se adelgaza, ya que
Podemos decir que el período de fragmentación o resor-
hay una continua proliferación celular local que continúa
ción termina cuando el tejido fibrovascular que ha susti-
nutriéndose del líquido sinovial, mientras la fisis de creci-
miento presenta irregularidades, ya que la falta de aporte
sanguíneo da alteraciones en la alineación de sus células,
limitando su aposición en columnas.
En esta etapa la cabeza femoral es muy vulnerable a las
fuerzas que actúan sobre ella, pudiendo deformarla o cau-
sar fracturas subcondrales que hundan el cartílago, aunque
podría conservar su contorno esférico.38
ACTA ORTOPÉDICA MEXICANA 2009; 23(3): 172-181
Etapa de resorción o Fragmentación
En esta segunda etapa, el estrés que causó el problema
continúa ejerciéndose en la cadera y el hueso necrótico se
aplastará y comprimirá, el cartílago puede hundirse, las
fracturas subcondrales han ocurrido, pero los osteoclastos
inician su actividad, participando en una remodelación de
las trabéculas distorsionadas, realizan una sustitución por
infiltración y aposición, empiezan por un fibrocartílago
que rellena los defectos, esto lo hacen de una manera irre-
gular, primero desde la periferia de la epífisis. Este tejido
conectivo con vascularidad invade el hueso necrótico,
mientras los osteoclastos lo reabsorben, reemplazándolo
por un hueso nuevo inmaduro; como se presenta de mane-
ra infiltrativa y por haber repetido períodos de infartos
óseos, suele tener diferentes grados de maduración en el
mismo hueso.39 Esto también causa cambios similares
acompañantes en el cartílago.
Los colapsos óseos, su fragmentación y la reparación
del hueso, causan pérdida de altura de la epífisis y se inicia
una osificación endocondral. El hueso necrótico colapsa-
do se revasculariza, generalmente primero en la región an-
tero-lateral y aparecen zonas osificadas en el cartílago arti-
cular, que en las radiografías se ven como «áreas de
calcificación» que son uno de los signos de riesgo de
Catterall39,40 y en la porción supero-lateral aparece una
protrusión que se llama «signo de Gage», la cual es una
extrusión lateral de la cabeza femoral que sobresale del
acetábulo.
Al principio de la etapa de resorción, el trabeculado de
la metáfisis puede ser esencialmente normal, pero la parte
central de la metáfisis puede contener tejido adiposo, que
en etapas posteriores estas áreas radiolúcidas corresponde-
rán histológicamente a fibrocartílago que dan una osifica-
ción desorganizada, así la fisis puede extenderse sobre la
metáfisis desde el cuello femoral.
La compresión vertical o el estrés continuo sobre la
cabeza femoral que tiene hueso muerto, inmaduro o sus-
tituido por tejido más suave, puede causar colapso del
cartílago que no tiene un cimiento resistente, dando
aplastamiento de la epífisis, generalmente en dirección
antero-lateral y queda una cabeza femoral plana, extrui-
da, deformada o de forma ovalada.
Fase de Reosificación
tuido al hueso necrótico inicia una fase de maduración
ósea (reosificación) de reparación (este período también
puede llamarse de reparación).
Al persistir fuerzas de compresión en la cadera, por con-
tracturas musculares, soporte del peso corporal o alguna
posición inadecuada de la cabeza femoral en el fondo ace-
tabular, ocurren fracturas que colapsan el hueso avascular
patológico, que lentamente, de forma infiltrativa, se susti-
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 Enfermedad de Legg-Calvé-Perthes
tuye por aposición de un hueso viable, dando esclerosis y
condrificación por metaplasia del tejido fibroso reactivo
formado en la zona y facilitando la función de los osteo-
clastos, que reabsorben el hueso necrótico; esto radiográ-
ficamente se ve como un aumento de la radiolucidez.40
La reosificación generalmente inicia en los márgenes
de la epífisis y progresa hasta que la epífisis está comple-
tamente osificada; se trata de una reosificación parafisiaria
y ésta es la razón por la que puede haber la formación de
puentes óseos entre la metáfisis y la fisis, que dan blo-
queos de crecimiento del cuello femoral; de esta forma hay
una reosificación transfisiaria también.
Fase de Remodelación
La revascularización del hueso involucrado en la ELCP
es muy lenta debido a que la destrucción de sus trabéculas
causa compresión y bloqueo de los canales vasculares de
los capilares óseos y medulares; se dice que existe una se-
gunda etapa de necrosis isquémica, por el bloqueo vascu-
lar que da la compresión por impacto, apisonamiento y
acumulación de escombros de hueso necrótico. Pero una
vez que la epífisis se ha reosificado, la cabeza femoral se
va remodelando poco a poco, hasta que el paciente alcan-
za la madurez esquelética.31 La recuperación de la esferici-
dad de la cabeza femoral dependerá del aplanamiento
existente, de la cantidad de extrusión ósea que tenga, de
la concentricidad de los componentes de la cadera y de las
fuerzas biomecánicas que actúen en ella,41,42 pero frecuen-
temente el resultado final puede ser una cabeza femoral y
acetábulo no esféricos (Coxa Plana) o deformados, por lo
que esta etapa también puede llamarse fase de deformidad
residual.
Características clínicas
La presentación de la enfermedad, suele ser muy insi-
diosa y poco clara, inicia comúnmente en chicos de 4 a 8
años de edad. Los padres notan claudicación al caminar,
que muy pocas veces saben cuándo inició; puede ser de
unas semanas a varios meses. Esta cojera es antálgica, de
zancadas y fases de apoyos cortos, que aumenta con el
ejercicio y disminuye con el reposo; pocas veces tiene
como antecedente una sinovitis tóxica; el 70% refieren un
traumatismo previo.43 El dolor suele no ser incapacitante,
localizado en la profundidad de la cadera, pero se irradia
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de la ingle a la cara anterior y medial del muslo, hacia la
Este es un método que debe usarse para la confirmación
rodilla, siguiendo la trayectoria del nervio obturador. Pue-
de sospecha de enfermedad de LCP, utilizando principal-
de existir una discreta atrofia del muslo y la nalga.
Al palpar al paciente podemos encontrar contractura de
los músculos aductores, así como del psoas ilíaco, con mo-
vilización pasiva dolorosa y limitada. Al tratar de palpar la
cabeza femoral se puede causar dolor por la sinovitis exis-
tente. Posteriormente puede desarrollarse contractura en
flexión de la cadera, permaneciendo la pierna en aducción
y rotación lateral; en este momento hay restricción de la
ACTA ORTOPÉDICA MEXICANA 2009; 23(3): 172-181
rotación medial de la cadera, puede acompañarse de signo
de Trendelenburg positivo y discreta asimetría de la lon-
gitud de las piernas. Bowen37-44 reportó que en el 75% de
los casos el diagnóstico se realiza durante la fase de necro-
sis o fragmentación.
Diagnóstico por imágenes
Las imágenes radiográficas son el método más común,
ampliamente usado, sencillo de lograr, barato y conocido
para diagnosticar y evaluar a la ELCP. Se deben de practi-
car si se sospecha éste u otro padecimiento de las caderas o
la pelvis; descartan o ayudan a su clasificación y pronósti-
co; además dan bases para decidir el tratamiento.
Se deben tomar imágenes antero-posteriores (a p) y late-
rales en posición de Lauenstein (rana); estas imágenes son
la base para colocar a la enfermedad en algún estadio de
las diferentes clasificaciones que se usan.
En las radiografías se observan los signos de diagnósti-
co precoz de Catterall:39 Desplazamiento lateral de la ca-
beza femoral, línea de fractura subcondral, aumento de la
densidad epifisiaria, menor tamaño del núcleo epifisiario
en la cadera afectada comparada con la sana. Datos que si
las radiografías se han efectuado muy precozmente, no se
verán y sólo encontraremos características de sinovitis,
que para Waldenström6-45,46 sólo se ve ensanchamiento del
espacio articular, que dura de una a tres semanas y cuando
hay un aumento homogéneo de la opacidad de la cabeza
femoral ya se está en el período de necrosis aséptica o
avascular que dura de algunos meses hasta un año. En la
etapa de regeneración o fragmentación, se distinguen áreas
de rarefacción con aspecto de fragmentos redondeados, ya
que existe tejido fibroso vascular entre el hueso o hueso
inmaduro sin calcificar, el cuello femoral se ensancha y
puede haber extrusión de la cabeza femoral. En la etapa re-
sidual desaparecen las áreas de rarefacción y hay reempla-
zo por hueso normal, por lo que la cabeza femoral recupe-
ra su esfericidad o bien queda aplanada.45-47
Para Tachdjian48 los primeros signos radiográficos son:
1) Núcleo de osificación de la cabeza femoral pequeña
comparada con la contralateral. 2) Línea de fractura sub-
condral en la cabeza femoral. 3) Incremento de la radio-
opacidad de la cabeza femoral.
Gammagrafía ósea
mente Tecnecio-99m en fase ósea. Mostrará una marcada
disminución o deficiencia de la captación del radio-nú-
cleo en la zona de necrosis avascular de la cabeza femoral,
señala disminución del flujo sanguíneo y del metabolismo
óseo en la etapa isquémica, antes de que se desarrollen los
signos radiográficos, diferenciándola de la sinovitis transi-
toria y para algunos autores determina la extensión del in-
volucramiento epifisiario y posteriormente el retorno de la
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 Raúl Frías Austria
vascularidad al hueso afectado como lo ha mencionado
Conway.49 Para Tsao50 también es una herramienta para se-
guir los patrones de revascularización de la cabeza y el
cuello femoral, dándole un valor pronóstico, pero deben
tomarse vistas anteroposteriores y laterales de la cabeza fe-
moral implicada.
Ultrasonido
El ultrasonido es un método de diagnóstico que se ha
usado en la evaluación inicial y para diferenciar otros pro-
blemas, puede encontrarse protrusión de la cápsula articu-
lar que es común en las sinovitis; sin embargo, en la ELCP
debe haber un adelgazamiento de la membrana sinovial
que pocas veces se reporta en los estudios de ultrasonido
convencionales.51,52
Artrografía
La artrografía nos permite valorar la cobertura cartilagi-
nosa de la cabeza femoral, su esfericidad y su centrado en
el acetábulo, ya que cuando la necrosis ha colapsado la
epífisis deformándola, los elementos de la cadera suelen
estar incongruentes; por medio de la artrografía podemos
ver la extensión y los patrones de la deformidad, bordes
protruidos y rangos de movimiento.53
Resonancia magnética
La resonancia magnética muestra signos más tempranos
que la radiografía simple, ya que deja ver cambios produ-
cidos por la isquemia antes de ser visibles radiográfica-
mente, muestra la fisis y el cartílago articular53-56 y al agre-
gar gadolinium, la sensibilidad para detectar los cambios
A B
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C D
ACTA ORTOPÉDICA MEXICANA 2009; 23(3): 172-181
causados por la necrosis es mayor.57 Sales de Gausy58 refie-
re que además es posible ver la coxa magna en forma tridi-
mensional y cuantificar su tamaño, de esta manera estable-
cer un pronóstico en base a porcentaje de crecimiento.
Clasificación
Las clasificaciones son esquemas de imágenes radio-
gráficas utilizadas para diagnosticar, situar la evolución
de la enfermedad, establecer tratamientos y procurar
pronosticar el futuro de la cadera afectada. Pretenden
predecir los resultados y la posibilidad de desarrollar
osteoartritis.
Catterall,39 utilizando vistas radiográficas en posición
antero-posterior y de Louestein (en rana), cuantifica la ex-
tensión de la afectación en cuatro tipos: Grupo I afecta
menos del 50% de la epífisis capital femoral en su región
anterior, sin colapsos o secuestros (Figura 1-A). Grupo II
la epífisis se encuentra afectada en un 50% en su región
central, puede haber formación de secuestros, pero su co-
lumna lateral permanece intacta (Figura 1-B). Grupo III
afecta a la epífisis hasta en un 75%, presenta colapsos y
formación de secuestros, pero ésta integra la porción me-
dial, postero-medial y algunas veces la lateral (Figura 1-
C). Grupo IV en este grupo casi la totalidad de la epífisis
se encuentra afectada (Figura 1-D). Los dos primeros gru-
pos tienen un buen pronóstico y los dos últimos mal pro-
nóstico. La cadera afectada puede cambiar de un grupo a
otro al progresar la enfermedad; sobre todo si se clasifica
antes de la fase de fragmentación puede haber errores entre
observadores y entre observaciones.40 Catterall describió
los «signos de riesgo cefálico» y al estar presentes dos o
más de ellos, el pronóstico es malo: éstos son el signo de
Gage, calcificación lateral de epífisis, reacción (quiste)
Clasificación de Catterall
Figura 1. Para valorar la cabeza
femoral afectada se deben tomar
radiografías con vista en AP y la-
teral en posición de rana. Ver tex-
to para su descripción. (Catterall
A: The natural history of Perthes’
disease. J Bone Joint Surg 1971;
53B: 37).
176
 Enfermedad de Legg-Calvé-Perthes

metafisiaria, subluxación lateral, horizontalización de la otros autores agregaron un grupo B/C para los casos limítro-
fisis y defecto en V de la fisis y metáfisis. fes en que el pilar lateral conserva la altura del 50%, pero el
Salter y Thomson1 hicieron una clasificación basada en segmento remanente tiene muchas irregularidades radiográ-
la descripción de la extensión de la fractura subcondral en ficas.62,63 La altura del pilar lateral se asocia con la severi-
la zona superolateral de la cabeza, vista principalmente en dad de la coxa plana cuando se alcanza la maduración es-
la placa lateral de cadera y visible antes de los signos de quelética y cuando se combina con la edad de inicio de la
Catterall. Se clasifica en dos grupos: A, Menos del 50% de ELCP la predicción es mejor. Domzalski64 ha sugerido re-
extensión en la cúpula superior de la cabeza femoral. B, cientemente la utilización del índice de crecimiento fisiario
Trazo de fractura mayor al 50%. El tipo A tiene un buen cefálico femoral, que nos predice su crecimiento indicando
pronóstico y el B malo.9 Esta fractura sólo es visible en el momento y tipo de tratamiento más adecuado para tener
aproximadamente un 30% de los casos; antes de ella se mejores resultados.
puede observar una imagen de aumento de la densidad en
el borde superior de la epífisis en forma de media luna, co- Diagnóstico diferencial
nocida como signo de uña, que frecuentemente es el primer
signo radiográfico claro. Existen muchos padecimientos que pueden confundir-
Clasificación de Stulberg.59 En esta clasificación se pro- se con la ELCP pero para el ortopedista con experiencia
pone una valoración de la deformidad de la cabeza femoral en pediatría, la historia clínica, el examen físico cuida-
madura, imaginándola tridimensional en imágenes en dos doso y las placas radiográficas le son suficientes para rea-
dimensiones (ap y en rana), valora la forma de la cabeza fe- lizar el diagnóstico; sin embargo, en la fase inicial debe
moral y su congruencia con el acetábulo (Figura 2), la divi- diferenciarse con una sinovitis o con artritis séptica, en
dió en 5 tipos con un valor predictivo en cada caso: Clase I
es una cadera normal con una congruencia acetabular esféri-
ca concéntrica. Clase II cabeza femoral esférica pero grande,
concéntrica con el acetábulo en las dos vistas, pudiendo te-
I-II
ner uno o más de los siguientes datos; coxa magna, cuello
corto, cobertura acetabular anormal. Clase III se caracteriza
por una congruencia articular no esférica, cabeza femoral
ovoide, contorno de hongo (no aplanado), cabeza o coxa
magna, cuello femoral corto, cobertura acetabular anormal. III-IV
Clase IV congruencia femoral no esférica, cabeza femoral
aplanada con anormalidades de la cabeza, cuello y acetábu-
lo. En estos casos los pacientes tienen molestias temprana-
mente y desarrollarán artrosis a los 40 o 50 años. Clase V
franca incongruencia cabeza acetábulo, cabeza femoral
aplanada, pero el cuello y el acetábulo pueden ser normales V
(Tabla 1), se asocia fuertemente con dolor incapacitante y
osteoartrosis antes de los 60 años.59
Herring y cols.62 propusieron una clasificación basada en
las imágenes radiográficas antero-posteriores bien tomadas
de caderas en posición neutra, durante la fase de fragmenta-
ción (aproximadamente a los 6 meses de iniciado el padeci-
miento) y analizan la columna ósea del lado trocantérico de
la cabeza femoral a la que llama «Pilar lateral» del cual Clasificación de Stulberg
toma su nombre. Esta clasificación divide la epífisis femoral
Figura 2. El tipo I y II tiene congruencia esférica, el tipo III y IV
en tres sectores o pilares; lateral, central y medial (Figura su congruencia no es esférica y el tipo V hay incongruencia no es-
3); Grupo A (Figura 3-A) no existen alteraciones en el pilar férica. (Citada por Adam SP, 9 de Sponseller PD, Desai SS, Millis

www.medigraphic.com
lateral, conserva su altura y radio-lucidez normal, aunque
pudiera haber colapso del pilar central o medial. Grupo B
(Figura 3-B). Se mantiene el pilar lateral más del 50% y su
altura total se conserva pero altera su radio-lucidez de den-
sidad ósea. Grupo C (Figura 3-C). Existe pérdida de más del
50% de la altura del pilar lateral, tiene menos radio-lucidez
que en el grupo B. Grupo D (Figura 3-D) existe pérdida de
más del 25% de la altura del pilar lateral. La clasificación
originalmente es de tres grupos, pero algunos autores agre-
garon el grupo D,40 ya que su pronóstico es más malo, pero
MB: Comparison of femoral and innominate osteotomies for the
treatment of Legg-Calvé-Perthes disease. J Bone Joint Surg Am
1988; 70A: 1131-9.
que los cuadros clínicos iniciales son muy similares, por
lo que en la ELCP siempre debe incluirse una serie de
análisis de laboratorio que permitan descubrir esas posi-
bilidades diagnósticas, en que se incluya una biometría
hemática completa con diferencial, velocidad de sedi-
mentación globular, proteína C reactiva, factor reumatoide

ACTA ORTOPÉDICA MEXICANA 2009; 23(3): 172-181 177

 Raúl Frías Austria

Tabla 1. Clasificación de Stulberg.

Tipo Aspecto radiográfico Congruencia

1 Cadera normal Esférica

2 Cabeza femoral esférica, círculos concéntricos en Rx., Ap. y lateral en rana, con uno o más Esférico
de lo siguiente: coxa magna, cuello corto o normal, acetábulo con inclinación anormal
3 Cabeza ovoide o en hongo, coxa magna, cuello normal o corto, acetábulo con inclinación anormal No esférica
4 Cabeza femoral aplanada, otras anormalidades de la cabeza, cuello y acetábulo No esférica
5 Cabeza aplanada, cuello y acetábulo normal No esférica
con incongruencia

Stulberg SD, Cooperman DR, Wallensten R. The Natural History of Legg-Calvé-Perthes’ disease. J Bone Joint Surg Am 1981; 63: 1095.

y antiestreptolisinas, también aspirado del líquido articu-

lar y su análisis. En la ELCP suelen estar los estudios
normales, excepto la velocidad de sedimentación globu-
lar que se encuentra discretamente aumentada.
No se debe olvidar que después de descartar un proceso
infeccioso, también se debe realizar una investigación ge-
nética y de sus condiciones metabólicas.
En la tabla 2, están los padecimientos con los que la
ELCP debe diferenciarse. A B

Tratamiento

El tratamiento de la ELCP continúa siendo controversial

y los autores siempre evocan estudios que se adaptan a su
experiencia, preferencias y escuela, pero la gran mayoría de
ellos están de acuerdo en que no existe una sola manera de
manejarse, que son varios los tipos y principios de trata-
miento y éstos dependen entre otros muchos puntos, de la
edad de inicio del padecimiento y de la extensión de afec-
tación de la epífisis femoral. Predomina el concepto de
mantener a la cabeza femoral contenida dentro del acetábu-
lo, ya que aunque se ejerza una presión intracapsular mayor
a la normal o extraarticular por la acción de músculos y ten-
dones contracturados, sobre una cabeza femoral inmadura,
deformable por la necrosis y las fracturas subcondrales, el
acetábulo tendrá una acción remodeladora por su contorno
esférico hembra, actuando sobre otra esfera macho en su in-
terior (una esfera no aplana a otra interna, a menos que se
inviertan sus contornos o actúe su borde).
Se debe restaurar la movilidad de la cadera lo más cerca-
no a lo normal, con el uso de analgésicos, antiinflamato-
rios, reposo, combinar tracción-abducción, terapia física,
www.medigraphic.com
hidroterapia, limitar o evitar el soporte del propio peso,
uso de yesos moldeados o aparatos y finalmente cirugías,
como tenotomía de aductores y psoas-iliaco.9,65,66
Esta forma de tratamiento está principalmente indicada
en los menores de 6 años, que tengan extensión de la ne-
crosis de la epífisis menor del 50%.
Resulta útil recordar cómo una de las reglas prácticas
para el tratamiento de la enfermedad de LCP que los niños
menores de 6 años al inicio del problema, del 50% al 60%
no requerirán de tratamiento;1,11,66-68 punto también contro-
C D
Clasificación del Pilar Lateral de Herring
Figura 3. Valoración que se realiza en una placa radiográfica AP
de caderas, y examina el extremo lateral de la cabeza femoral, ver
el texto para su explicación. (Herring JA, Neust JB, Williams JJ et
al: The lateral pillar classification of Legg-Calvé-Perthes disease. J
Pediatr Orthop 1992; 12: 143-150).
versial, pero en forma general, deberemos de seguir los es-
quemas radiográficos de clasificación y pronóstico del pa-
decimiento. Los puntos que indican mal pronóstico como
son: Catterall 3 y 4, Salter-Thomson B, Pilar lateral C, sig-
nos de riesgo clínico, signos de riesgo radiográficos a pesar
de la extensión del problema y deformidad cefálica, todo
esto en menores de 8 años. En mayores de 8 años (Catterall
2, 3 y 4 con o sin signos de riesgo, pilar lateral B y C, Salter-
Thomson B) con y sin deformidad, todos ellos requerirán de
tratamiento. Mientras los de buen pronóstico no requerirán
de tratamiento como son Catterall 1 y 2, Salter-Thomson A
y pilar lateral A. Los de pronóstico indeterminado que po-
drían requerir tratamiento: Catterall 2, pilar lateral B. Las
secuelas de la reosificación de la cabeza femoral o del ace-
tábulo, podrían requerir de tratamiento.

ACTA ORTOPÉDICA MEXICANA 2009; 23(3): 172-181 178

 Enfermedad de Legg-Calvé-Perthes
Tabla 2 Diagnóstico diferencial con enfermedad de
Legg-Calvé-Perthes.
Condrólisis
Enfermedad de Gaucher
Hemofilia
Hipotiroidismo
Artritis reumatoide juvenil
Linfoma
Mucopolisacaridosis
Displasia epifisiaria múltiple
Displasia de Meyer
Neoplasias
Displasia de la cadera en desarrollo residual
Osteomielitis del fémur proximal
Artritis séptica
Displasia espondiloepifisiaria
Enfermedad de células falciformes
Sinovitis tóxica
Necrosis aséptica traumática
Tuberculosis
Deslizamiento femoral capital mínimo
Granuloma eosinófilo
Osteoma osteoide
Sinovitis vellonodular pigmentada
Condroblastoma
Las metas principales en el tratamiento en la ELCP son:
prevenir la deformidad de la cabeza femoral y del acetábu-
lo, retirar las causas que afecten el crecimiento de los com-
ponentes de la cadera y prevenir la enfermedad articular
degenerativa, para esto debe contenerse la epífisis femoral
completamente dentro del acetábulo con su fisis desde me-
dial a lateral.
Para lograr la contención se pueden utilizar yesos mol-
deados en forma de botas largas en abducción y rotación
medial y aparatos, como el de Tachdjian 48 y la ortesis
Atlanta Scottish-Rite,11 que deben mantener 30º de abduc-
ción, medibles en una placa radiográfica AP de caderas
con la ortesis puesta. Esta forma de tratamiento suele dar
problemas sicológicos y sociales a los pacientes, así que
la decisión de uso requiere de una valoración cuidadosa
del caso, que incluya visita a un sicólogo y conocer a la
familia, pero Sinigaglia69 menciona que no existe eviden-
cia científica de que esta forma de tratamiento modifique
la historia natural de la enfermedad, el resultado es igual al
sólo manejo sintomático. Por su parte Wiig12 recomienda
abandonar el uso de ortesis.
Otra forma de mantener a la cabeza femoral en la profun-
www.medigraphic.com
didad del acetábulo es la quirúrgica, usando la osteotomía
varizante con o sin desrotación, la cual reduce las fuerzas
que actúan directamente sobre la cabeza femoral aumentan-
do la superficie de apoyo y diseminándolas en ella, dismi-
nuye también la presión intracapsular, incrementa la cober-
tura acetabular, libera la hipertensión venosa intraósea y
mejora el drenaje venoso intraóseo. Está indicada en mayo-
res de 6 años con más del 50% de necrosis,12 en los del gru-
po B y B/C del pilar lateral;63 para Beer70 a largo plazo ten-
drá mejores resultados que otros métodos.
ACTA ORTOPÉDICA MEXICANA 2009; 23(3): 172-181
Antes de realizarla se debe restaurar la movilidad de la
cadera y no debe haber contracturas musculares, debe de-
jarse el ángulo cérvico-diafisiario a 110º.
Sus desventajas son que requerirá de otra cirugía para
el retiro del material de fijación; el uso de un yeso de in-
movilización por 8 semanas o más puede dejar un acorta-
miento de la extremidad o un varus excesivo y la eleva-
ción del trocánter mayor, que da insuficiencia del glúteo,
Trendelenburg, Duchene y marcha bamboleante.
La osteotomía innominada de Salter es otra forma muy
frecuente de mejorar la cobertura acetabular, ya que lo re-
dirige hacia anterior y lateral. Deja la cabeza femoral en
discreta flexión, abducción y rotación medial con respecto
al acetábulo al apoyar el peso corporal y puede corregir
acortamientos discretos de la extremidad.
Las condiciones para su realización son muy similares a
las de la osteotomía proximal femoral, ya que se deben
realizar estudios previos que permitan centrar a la cabeza
femoral en el acetábulo, corregirse todas las contracturas
musculares y restaurarse la movilidad de la cadera.
Está indicada en mayores de 6 años, en los grupos B y
B/C del pilar lateral y en una necrosis ósea mayor del
50%. Tiene las desventajas de requerir de una segunda ci-
rugía para el retiro de los clavillos y de la inmovilización
en yeso por 12 semanas; el costo quirúrgico es alto, latera-
ESTE
liza el DOCUMENTO ES ELABORADO
acetábulo, aumenta la presión en la POR
cabezaMEDI-
femoral
GRAPHIC
por acción de palanca de los músculos abductores y puede
cambiar su contorno.11
Sposeller71 no encontró diferencia entre osteotomía in-
nominada y osteotomía varizante, en lo que estuvieron de
acuerdo Herring63 y Sharma72 quienes encontraron mejores
resultados sólo cuando el manejo quirúrgico se realizó en
mayores de 8 años.
En casos muy severos en que no es posible un centraje
completo de la cabeza femoral en el acetábulo estará indi-
cado realizar una doble osteotomía, una en el iliaco y otra
en la región proximal del fémur, útil cuando se requiere de
un exceso de varo para lograr la cobertura acetabular y fal-
ta de cobertura en la placa de caderas de centraje, en ma-
yores de 8 años y con una extensión de la necrosis mayor
al 50%.73 También en falta de cobertura acetabular des-
pués de un solo procedimiento.
Artroplastías de repisa; este tipo de cirugías están indi-
cadas en la etapa residual, en que ha quedado el acetábulo
displásico, con la cabeza femoral grande y necesita más de
25º de abducción para lograr contención concéntrica, en
caderas dolorosas con varias cirugías.
Las cirugías de repisa logran aumentar la capacidad
acetabular deficiente, evitan la migración supero-lateral de
la cabeza femoral, aumentan el área de apoyo de la cadera
y disminuyen la presión a través de la articulación.74,75 En-
tre las cirugías de repisa ha ganado popularidad la «Artro-
plastía de repisa ranurada para aumento acetabular» publi-
cada por Staheli,74,75 que tiene la ventaja de no requerir
obligatoriamente una cabeza completamente esférica, es
una cirugía extraarticular que no deja rigidez, no daña el
179
 Raúl Frías Austria
cartílago articular, conserva la anatomía de la pelvis, per-
mite extender el acetábulo, cubre la cabeza femoral, no al-
tera la función del glúteo medio y no daña la articulación
sacro-ilíaca.48,74,75)
Domzalski64 realizó un estudio en que evaluó la esti-
mulación del crecimiento lateral acetabular después del
procedimiento de apoyo del borde acetabular en ELCP,
midiendo su profundidad y altura y concluyó que existe
un sobrecrecimiento del acetábulo, pero al realizar un pro-
cedimiento de apoyo a su borde se induce un crecimiento
adicional al acetábulo verdadero (excluyendo a la repisa
creada), a tres años de realizada la cirugía, lo que no suce-
de en las osteotomías varizantes. Este efecto benéfico de
la cirugía de apoyo al borde es una estimulación del creci-
miento acetabular, prevención de la subluxación y resolu-
ción de la repisa después de la reosificación epifisiaria.64
Kim y sus colaboradores76 en su reporte del equipo de
estudio multicéntrico de la Asociación Japonesa de Orto-
pedia Pediátrica encontró que el tratamiento quirúrgico
tiene un mejor resultado que el tratamiento conservador,
sin que pudieran determinar el tratamiento óptimo.76
La ELCP, continúa siendo un reto para los ortopedis-
tas que vemos principalmente niños; debe impulsarse su
estudio, ya que no conocemos la causa y al no saberla no
podemos realizar un tratamiento etiológico que realmen-
te cure; tampoco se puede prevenir; sin embargo, los es-
tudios que presentamos nos dejan un ánimo optimista, ya
que en base a las mediciones radiográficas que realice-
mos, teniendo en cuenta la edad de inicio del padeci-
miento y con ayuda de las clasificaciones radiográficas,
podemos realizar tratamientos lógicos que mantengan la
esfericidad de la cadera o eviten que ésta se pierda, así
podremos retardar o impedir el dolor y la destrucción ar-
ticular que nos lleve a una artroplastía total de cadera en
una edad temprana.
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50. Tsao AK, Dias LS, Conway JJ, Straka P: The prognostic value
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51. Futami T, Kasahara Y, Suzuki S: Utrasonography in transient syno-
vitis and early Perthes’ disease. J Bone Joint Surg 1991; 73B: 635.
52. Eckerwall G, Hochbergs P, Wingstrand H, Egund N: Sonography
and intracapsular pressure in Perthes’ disease: 39 children exami-
ned 2-36 month alter onset. Acta Orthop Scand 1994; 65: 575.
53. Kaniklides C, Lonnerholm T, Moberg A, Sahlstedt B: Legg-
Calvé-Perthes disease: comparison of conventional radiogra-
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Radiol 1995; 36: 434.
54. Pouletaut P, Claude L, Winzenrieth R: Automated analysis of
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55. Hoffinger SA, Henderson RC, Renner JB: Magnetic resonance
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56. Uno A, Hattori T, Noritake K, Suda H: Legg-Calvé-Perthes disea-
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57. Gent E, Antapur P, Fairhurst J: Perthes’ disease in the very
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67. Allen B: Graphic analysis of femoral growth in young children
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70. Beer Y, Smorgick Y, Oron A, Mirovsky Y, Weigl D, Agar G,
Shitrit R, Copeliovitch L: Long-term result of proximal femo-
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2008; 28(8): 819-24.
71. Sponseller PD, Desais S, Millis MB: Comparison of femoral an
innominate osteotomies for the treatment of Legg-Calvé-Per-
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72. Sharma S, Shewale S, Sibinski M, Sherlock DA: Legg-Calvé-
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181
 Formosan Journal of Musculoskeletal Disorders 3 (2012) 111e115

Contents lists available at SciVerse ScienceDirect

Formosan Journal of Musculoskeletal Disorders

journal homepage: www.e-fjmd.com

Review Article

Overview of surgical treatments in Legg-Calvé-Perthes disease

Kuan-Wen Wu a, Ting-Ming Wang b, Shier-Chieg Huang b, Ken N. Kuo b, c, *
a
Department of Orthopedic Surgery, National Taiwan University Hospital, Chu-Tung, Taiwan
b
Department of Orthopedic Surgery, National Taiwan University Hospital, Taipei, Taiwan
c
School of Medicine, Taipei Medical University, Taipei, Taiwan

a r t i c l e i n f o a b s t r a c t

Article history: Since Dr Legg reported the paper entitled “An obscure affliction of the hip joint” in 1910, Legg-Calvé-
Received 31 August 2012 Perthes disease (LCPD) has been recognized. In the intervening 100 years, our understanding of disease
Received in revised form etiology, natural history, treatment options, and factors related to prognosis have expanded, and yet
13 September 2012
many opinions remain without consensus, especially in treatment decisions. During the past 30 years,
Accepted 13 September 2012
Available online 9 November 2012
containment of the femoral head within the acetabulum by conservative or surgical methods has been
popularly accepted as a concept for treatments. Several large and multicenter retrospective studies have
noted three factors related to outcome in children treated for LCPD: age at onset, severity of involved
Keywords:
lateral pillar classification
femoral head, and type of treatment. In patients with onset over the age of 8 years and greater than
Legg-Calvé-Perthes disease lateral pillar B or B/C class, surgical treatment was associated with improved Stulberg outcomes
surgery compared with conservative treatments. Moreover, the decision to apply appropriate surgical methods
should consider the age at surgery, Waldenström stage, and whether the femoral head was containable
in abduction. Relevant studies with evidence-based data regarding the results of different surgical
methods for LCPD are reviewed here, and there are valid descriptions of surgical indications, charac-
teristics, and associations with improved radiographic outcome.
Copyright Ó 2012, Taiwan Orthopaedic Association. Published by Elsevier Taiwan LLC. All rights reserved.

1. Introduction treatments, which involve different abduction braces, may be

Legg-Calvé-Perthes disease (LCPD) is a juvenile hip disorder
characterized by idiopathic osteonecrosis of the femoral epiphysis
that is believed to be related to arterial infarction.1 According to
Waldenström, the disease process commenced with aseptic
necrosis, and was followed by a subchondral fracture, fragmenta-
tion, revascularization, and remodeling.2,3 LCPD predominantly
affects boys between the ages of 4 years and 8 years.4 If the initial
symptoms occur after this age range, the disease is considered late-
onset and believed to have a poorer outcome compared with
children presenting at a younger age.5e7 In the past 100 years, there
have been many publications concerning the indications and
methods of treatment; however, controversy remains. Current
concepts of treatment for LCPD, either nonoperative or operative,
are largely based on the principles of containing the vulnerable and
biologically plastic femoral head in the acetabulum in the early
stage in order to keep a spherical femoral head and congruent joint
during the repair process.7e9 Nonoperative containment
* Corresponding author. School of Medicine, Taipei Medical University, 250
Wuxing Street, Taipei 11031, Taiwan. Tel.: þ886 2 27361661x7215; fax: þ886 2 6638
3879.
E-mail address: kennank@aol.com (K.N. Kuo).
appropriate in younger patients, whereas in older children with
more severe disease, surgical treatments have been suggested to
obtain a better result.8,9 There have been various surgical options
proposed for management. At the fragmentation stage, the child
may present with a subluxated hip due to extruded epiphyseal
segment of the femoral head. If the hip is containable in abduction,
but subluxated in adduction, containment surgery [e.g., femoral
varus osteotomy,10e13 Salter and triple innominate osteotomy,14e18
or shelf acetabuloplasty (SA)19e21] should be contemplated, taking
consideration of the age of onset and the extent of uncoverage of
the femoral head. However, once the disease course proceeds to the
late reossification or healed stage, the enlarged and saddle-shaped
femoral head may become irreducible. In this situation, the surgery
should be altered from the containment of the femoral head
through remodeling to salvage procedures that improve the
acetabular volume. Various surgical techniques have been
addressed for noncontainable LCPD hips, such as SA,19e21 Chiari
osteotomy,22 proximal femoral valgus osteotomy,23e25 articulated
hip distraction,26,27 surgical dislocation, osteochondroplasty,28e30
and combinations of these procedures. This article reviews the
efficacy of all surgical modalities for LCPD and recommends the
appropriate treatment protocols for clinical application.

2210-7940/$ e see front matter Copyright Ó 2012, Taiwan Orthopaedic Association. Published by Elsevier Taiwan LLC. All rights reserved.
http://dx.doi.org/10.1016/j.fjmd.2012.09.001
112 K.-W. Wu et al. / Formosan Journal of Musculoskeletal Disorders 3 (2012) 111e115
2. Surgical indications
The optimal treatment methods for LCPD and its prognosis are
still not fully understood. In the past 10 years, some authors have
tried to standardize the treatment principles for Perthes’ hip. Age at
diagnosis and extent of femoral head involvement (lateral pillar
classification4 or Catterall classification31) are the most common
classifications used to prognose the outcome following treatments.
They also give indication for different types of treatment. In a large
prospective and multicenter study, Herring et al compared the
results of various conservative and operative interventions.9 They
summarized a basic treatment plan for LCPD. All lateral pillar Group
A and Group B hips whose onset occurred at the age of 6 years or
less have an excellent prognosis and require only symptomatic
treatments. Surgical treatment is proposed in all Group C, B/C, and B
hips whose onset occurred after the age of 8 years. Group B hips
whose onset occurs between the ages of 6 years and 8 years have
a variable prognosis, which seems unrelated to treatment
method.8,9 Another large prospective review with medium-term
follow-up by Wiig et al7 yielded similar findings. They suggested
that children aged 6 years and older, with more than 50% femoral
head involvement (Catterall Groups 3 and 4) had a better outcome
if treated with surgery.
3. Treatment options
3.1. Femoral varus osteotomy
Since the preliminary report by Axer10 in 1965, femoral varus
osteotomy (FVO) has become one of the most popular operative
methods for LCPD. Varus osteotomy of the proximal femur aims
to center the femoral head deeply within the acetabulum and
allows correction of the flexion or rotational deformity simulta-
neously. The prerequisites for FVO are similar to those for
innominate osteotomy, which include a good range of motion, hip
congruency, and being able to contain the femoral head in
abduction. The surgery is recommended in the early stage of
fragmentation, when favorable biological and biomechanical
effects may be anticipated. Many studies reported that FVO yields
good long-term results.11e13
The average varus angle correction needed is approximately 25
to 35 , which would yield a shortening of 1 cm to 2 cm. The initial
shortening caused by osteotomy may gradually correct over
a period of years when it was done in children younger than
8 years. The varus angle correction can be limited to 15 in older
children to avoid prolonged or permanent limp. Theoretically, this
temporary shortening has a positive effect by relieving the pressure
over the necrotic head. However, abductor insufficiency, worsened
by the varus osteotomy, is a serious problem. It will preclude
“dynamic containment” of the femoral head and cause Trende-
lenburg gait. Intensive physiotherapy to actively strengthen
abductors may resolve it. In addition, the greater trochanter must
be maintained distal to the level of the femoral head to prevent an
abductor lurch at any time. Preventive trochanteric epiphysiodesis
or distal transfer is probably required simultaneously or at a later
time.13
3.2. Salter osteotomy
Salter osteotomy, which redirects the acetabulum and improves
the anterolateral femoral head coverage, was introduced as
a method for surgical containment of LCPD in 1962.14. The general
indications for Salter osteotomy are the same as for any form of
containment.15,16 These include: (1) age of onset over 6 years old
(perhaps 5 years in girls); (2) more than 50% of the femoral head
affected; and (3) hip subluxation in the weight-bearing position.
Salter osteotomy has been reported to produce better long-term
results than nonoperative methods with respect to Stulberg clas-
sification.9,15 Other studies have compared Salter osteotomy and
proximal FVO.32e34 They found similar results with respect to
femoral head sphericity, but increased femoral head coverage by
the center-edge angle after Salter osteotomy. Potentially, Salter
osteotomy can displace the acetabulum 1 cm medially and distally
as well, thereby decreasing the biomechanical stress over the hip
joint and improving the generally associated leg length discrep-
ancy.35,36 However, cautious clinical examination and radiographic
assessment are necessary before surgery. The prerequisites of Salter
osteotomy include full range of hip motion preoperatively, espe-
cially abduction and reasonable joint congruency.
Occasionally, Salter osteotomy alone may not provide sufficient
head coverage, particularly in older children (age >9 years). The use
of combined Salter and proximal femoral varus osteotomies (to
110e115 varus angle) have been performed recently to contain
a larger and deformed femoral head.18,37 In Herring’s multicenter
study, they concluded that Group C cases tend to have an unfa-
vorable prognosis regardless of age or surgical treatment.9 The
opinions may be questioned because their treatments lacked
combined osteotomies. As Javid and Wedge’s claimed,37 the
combined surgery may change the otherwise “poor” into “fair” hips
and improve the natural history in older children. The other
benefits of combined surgery include decreasing the effect of
increased intra-articular pressure from innominate osteotomy and
compensating the shortening from femoral osteotomy.
3.3. Triple innominate osteotomy
Although FVO and Salter osteotomy have become the most
common methods for surgical containments, there are certain
practical limitations of these two procedures. The degree of FVO
required to contain the femoral head may further shorten the limb
and cause prolonged limp, especially in older children. Further-
more, use of Salter osteotomy may not provide enough acetabular
rotation to cover the femoral head in severe cases, potentially
leading to iatrogenic hinge abduction.38 Because of the above
concerns, advanced containment methods (e.g., Salter plus varus
osteotomy or triple innominate osteotomy) have been developed
for more severe cases.17,18
Some studies have demonstrated that older age and/or exten-
sive femoral head involvement were risk factors for unsatisfactory
results. Sponseller et al32 found that patients older than 10 years
at onset had a poor outcome regardless of surgical treatment.
Herring et al9 also noted that lateral pillar C group at any age had
a poor outcome, even when treated by femoral varus osteotomy or
Salter osteotomy. These prior studies did not include advanced
containment methods in severely deformed hips. Triple innomi-
nate osteotomy is anticipated to achieve better femoral head
containment than could be achieved with Salter osteotomy alone
and to avoid the leg length discrepancy associated with femoral
varus osteotomy. The retrospective review of Wenger et al17
included 40 hips of 39 children with onset ranging in age from
5 years to 13 years who were treated by triple innominate
osteotomy; a significantly increased CE angle with an average of
17.2 was reported. They achieved predictable head containment
in Herring B patients of all ages and Herring C patients of younger
than 8 years. There were no poor results in the Herring B groups
and 80% of patients older than 8 years attained a good outcome. In
Herring C children younger than 8 years, 83% showed either
a good or a fair result. Older children of Herring C (>10 years)
remain less predictable; triple innominate osteotomy can only be
effective in some of them.
 K.-W. Wu et al. / Formosan Journal of Musculoskeletal Disorders 3 (2012) 111e115
3.4. Shelf acetabuloplasty
Lateral SA is indicated for severe cases when redirection osteot-
omy is thought insufficient to produce optimal coverage of the
extruded femoral head. An intraoperative dynamic arthrography is
useful for further confirmation. In severe Perthes disease, laterally
displaced and enlarged femoral head will preclude normal motion
of the hip. This condition, “hinge abduction” , was first proposed
by Quain and Catterall39 to describe the abnormal movement of
the hip resulting from the impingement of the superolateral portion
of a deformed femoral head on the lateral lip of the acetabulum. In
case of fixed hinge abduction, the patient would present with pain,
restricted hip motion, and permanent gait disturbance.
Several studies have shown that SA is a safe and effective
procedure in managing those cases with aspherical congruency or
incongruency with hinge abduction.19e21. Ghanem et al20 reported
a series of 30 severely involved LCPD patients, average age 8.6
years; all patients were pain free and had normal or almost normal
hip motion. Age at surgery, severity of femoral head involvement,
and presence of “head at risk” signs do not appear to affect the final
outcome.20 At the last evaluation, 19 hips (64%) were classified as
Stulberg 1 or 2 and six (20%) as Grade 3. A significant improvement
was noted in the majority of radiographic parameters. Our retro-
spective study21 also revealed improved abduction, internal rota-
tion, and acetabular coverage after treating Perthes’ patients with
an incongruent hip or hinge abduction by using SA. However,
despite these favorable results in LCPD, SA should remain a salvage
procedure and should be reserved for advanced cases. For
containable cases, “anatomical” procedures, such as redirection
innominate osteotomies, remain the primary choice.
3.5. Chiari osteotomy
In addition to SA, Chiari osteotomy is another popular salvage
procedure for children with insufficient femoral head coverage. The
potential advantage of Chiari osteotomy over shelf procedure is the
reduction of joint loading by medialization of the hip, which was
considered an important factor for improving hip congruency and
femoral head remodelling.22 This method has been proposed for
severe LCPD cases, similar to the shelf procedure. Great care must
be taken, and these salvage procedures should not be performed in
Perthes’ patients at the healed stage due to lack of femoral head
remodeling ability.
3.6. Femoral valgus extension osteotomy
In LCPD children at the late healed stage, decreased abduction is
possibly associated with femoral head overgrowth and saddle-
shaped deformity. When an arthrography demonstrates femoral
head deformity with unstable movement and hinge abduction but
becomes stable in adduction and flexion, a valgus and extension
osteotomy may be an effective procedure for unloading of
deformed epiphyseal segment, restoration of joint congruity, and
alleviation of femoroacetabular impingement. The concept of
femoral valgus extension osteotomy depends on redirecting the
more congruent and round anteromedial part of the femoral head
to the neutral position of weight bearing. This rotational and
sagittal correction can improve the gait and hip motion, reduced
pain, and femoral head shape.23e25 It will also resolve the problems
of leg length shortening and provide a more normal abductor
mechanism. However, this procedure is contraindicated in the stiff
hip.
Choi et al25 reviewed 35 hips, with an average age of 9.4 years at
surgery, that underwent valgus osteotomy for hinge abduction.
Postoperative pain relief, improvement in limp, and increased
113
range of hip motion occurred in the majority of cases. The overall
Iowa hip score significantly increased from 71.2 before surgery to
95.2 at the final follow-up. The Stulberg classification was II in 4
(11.4%), III in 22 (62.9%), and IV in 9 (25.7%). Favorable remodeling
can be anticipated especially when valgus osteotomy is performed
before the late reossification/healed stage and at younger age.
3.7. Hip distraction
Arthrodiastasis, or distraction of the joint, has been considered
as an alternative treatment in LCPD beyond conventional surgical
methods. Articulated hip distraction aims to maintain the head in
a properly contained position, reduce the deforming force,
encourage synovial circulation, and provide an ideal environment
for cartilage repair.26,27 This method can be used in late onset LCPD,
and cases with a varying degree of femoral head deformity or joint
subluxation, and not limited by hip stiffness. The advantages
include easy technique, minimal complication rates, and preser-
vation of the original anatomy of the acetabulum and proximal
femur. Although some studies reported comparable results with
other salvage procedures, the long-term benefits are not conclusive
and require further investigation.
3.8. Surgical dislocation and osteochondroplasty
Residual hip deformity secondary to LCPD can be quite complex
and may cause hip instability, femoroacetabular impingement
(FAI), or combinations thereof, which will ultimately predispose to
degenerative joint disease. Dysplastic acetabulum is often caused
by improper remodeling in response to the nonspherical head.
Impingement in LCPD is possibly due to nonspherical head, over-
riding greater trochanter with short neck, or functional retrover-
sion of proximal femur and acetabulum. Although some authors
have shown successful treatment of young patients with advanced
joint degeneration using hip arthroplasty,40,41 others proposed an
alternative method, “surgical dislocation and osteochondroplasty”,
to deal with those who are symptomatic but do not show severe
radiographic degenerative changes.28e30
Recently, Ganz et al42 reported that their surgical approach to
the hip with dislocation and osteochondroplasty of the headeneck
junction can be used in Perthes hip with only a small risk of
osteonecrosis. It is important to accurately assess the source of the
conflict between the femoral headeneck and acetabular edge to
design the surgical plan. In addition to plain film, advanced imaging
modalities such as 3D computed tomography and magnetic reso-
nance imaging can help to delineate the detailed configuration of
the headeneck junction and the damage pattern of cartilage and
labrum after LCPD. Surgical management should start with
correction of the proximal femur deformity, first through a surgical
dislocation approach. Osteochondroplasty is performed for
enlarged femoral head with reduced headeneck offset. Relative
neck lengthening osteotomy is then indicated to correct extra-
articular impingement from high-riding greater trochanter and
short neck. If the osteoplasty is not enough to correct the FAI, an
intertrochanteric osteotomy to realign the proximal femur has to be
considered. After correction of the femoral deformity, it is critical to
evaluate the acetabulum, which may be dysplastic or over-
augmented. These conditions can be improved through a redirec-
tional periacetabular osteotomy or osteoplasty of the acetabular
rim with labrum refixation.28e30
4. Recommended management for children with LCPD
Many treatment protocols depend on the age of onset and
radiographic appearances, including Waldenström classification
114 K.-W. Wu et al. / Formosan Journal of Musculoskeletal Disorders 3 (2012) 111e115
(necrosis, fragmentation, reossification, healing), extent of involved
femoral head (Herring or Catterall classification), and several clin-
ical and radiological “head at risk” signs described by Catterall.31
After making the diagnosis, careful clinical examination for limp
or limitation of range of hip motion will give further information for
consideration. A recommended protocol for treatment based on
age, clinical, and radiographic findings is as follows.
Children younger than 6 years e prognosis is generally good.
 Symptomatic treatment and range-of-motion exercise
 The exception is the child with whole head involvement (Catterall
4); the prognosis can be improved by FVO or Salter osteotomy
Children between 6 and 8 years e prognosis is variable e
require close observation of signs of “head at risk”, which
indicates the need for surgery. Once any signs of head at risk are
observed, a dynamic arthrography under anesthesia is valuable
before deciding the method of treatment.
 The head is contained and stable, without signs of head at risk e
conservative treatment and close monitoring
 Signs of “head at risk” are present, and the head can be contained
in abduction e FVO or Salter osteotomy alone should be used in
moderate cases, while a combined femoral and Salter osteotomy
or triple innominate osteotomy may be necessary in advanced
cases
 The head cannot be contained but is uncovered and hinged e SA
or Chiari osteotomy will provide adequate support
Children older than 8 years e prognosis is often poor, but
advanced or salvage procedures still bring the benefit for
improved femoral head coverage.
 The head can be contained in abduction (fragmentation stage) e
a combined Salter and proximal femoral varus producing osteot-
omy, triple osteotomy, or SA is feasible
 The head cannot be contained with unstable movement and
hinging (late fragmentation or early reossification stage) e SA or
Chiari osteotomy is appropriate
 The established femoral head deformity can cause painful limp,
unstable movement and hinge abduction, but is stable in adduc-
tion and flexion (early healing stage) e a femoral valgus and
extension osteotomy will produce stable movement, improved leg
length, and more normal abductor strength
 The deformed femoral head leads to joint subluxation and hip
stiffness (any stage) e a hip distraction can help to maintain the
head in a proper position, relieve the deforming force and
encourage the cartilage repair
 For correction of residual deformities after LCPD, such as joint
instability and FAI e a surgical hip dislocation with osteochon-
droplasty, neck lengthening, and greater trochanter advancement
can be used to deal with these sequelae of LCPD and prevent early
degenerative joint disease
5. Summary
Despite current nonoperative or operative treatments being
shown to produce a favorable outcome effectively with a spherical
femoral head in LCPD, there is still much to be investigated. The
results of these studies did not answer the question of why treat-
ments produce a satisfactory outcome in some patients but not in
others. One reason is that treatments provide some load-sharing
effects on the necrotic femoral head, but they do not directly
facilitate the impaired bone healing, especially in older children.
Further studies have developed a better understanding of LCPD
pathogenesis regarding increased bone resorption and delayed new
bone formation in combination with serial mechanical loading.
There have been animal studies of femoral head ischemia, which
obtained improved head sphericity after the use of bisphospho-
nates.43,44 It will be interesting to see whether these medical
strategies could alter the pathophysiology of the disorder and yield
a more promising result in the future.
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