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Neurociruga

2011; 22: 453-456

Meduloblastoma: una presentacin infrecuente en la recurrencia local

R. Garcia-Armengol; A. Guilln-Quesada* y J.M. Costa-Clar*

Servicio de Neurociruga. Hospital Universitario Son Dureta. Palma de Mallorca. Servicio de Neurociruga*. Hospital San Joan de Du.
Esplugues de Llobregat. Barcelona.

Resumen Introduccin

Introduccin. El meduloblastoma es un tumor con El meduloblastoma es uno de los tumores ms comunes


un elevado potencial de recurrir y de metastatizar. de la fosa posterior en la infancia; ocasiona cerca del 30%
Caso clnico. Presentacin de un caso de medulo- de las neoplasias ubicadas en este lugar en la poblacin
blastoma de fosa posterior en un nio de 6 aos peditrica. Es un tumor invasivo maligno que pertenece
intervenido en 3 ocasiones y tratado con quimiotera- al grupo de tumores primitivos neuroectodrmicos6. La
pia y radioterapia con una recurrencia local que se recurrencia de este tumor ya sea recurrencia local como
extenda extracranealmente a nivel de la musculatura metastsica, es la norma, presentndose con una gran varie-
laterocervical del cuello. dad de formas clnicas, muchas de ellas extremadamente
Conclusin. Aunque las metstasis extracraneales raras3.
del meduloblastoma no es un fenmeno aislado, la En la presente nota clnica presentamos el caso de un
extensin extracraneal del meduloblastoma es un fen- nio de 6 aos de edad con una recurrencia local muy poco
meno poco frecuente en la recurrencia local. frecuente, extendindose extracranealmente a tejidos blan-
dos del cuello e infiltrando la musculatura laterocervical.
PALABRAS CLAVE: Meduloblastoma. Metstasis sist-
micas. Recurrencia. Caso clnico

Meduloblastoma: infrequent onset in local recu- Varn de 6 aos de edad, que en diciembre del 2005 es
rrence diagnosticado e intervenido quirrgicamente en Ecuador
de un meduloblastoma del vermis cerebeloso. En enero
Summary del 2006 en una RM cerebral de control, se objetiva una
importante masa residual, por lo que se decide reintervenir
Introduction. Meduloblastoma is high potential to quirrgicamente, quedando restos tumorales. Se realiza
recur and spread tumour. quimioterapia posterior y radioterapia en la fosa posterior,
Case report. One case posterior fosse meduloblas en el neuroeje y una sobreimpresin en el lecho tumoral.
toma in six years old boy who received 3 surgeries, En marzo del 2007, se objetiva nuevo crecimiento tumoral,
chemotherapy and radiotherapy, with local recurrence por lo que se decide nueva intervencin quirrgica. En el
and extracranial extension into neck laterocervical TC de crneo de control no se visualizan masas captantes
musculature. de contraste. Posteriormente se realiza quimioterapia
Conclusion. Although meduloblastoma extraneural adyuvante. En un examen de control, se objetiva una masa
metastases are not isolated phenomenon, meduloblas- dura y fija en la zona cervical posterior y lateral izquierda.
toma extracraneal extension is uncommon phenomenon Se realiza una resonancia magntica cerebral y cervical
in local recurrence. que muestra una tumoracin slida en la fosa posterior
que se extiende desde el margen inferior e izquierdo de la
KEY WORDS: Meduloblastoma. Systemic metastases. cisterna magna, y del cuarto ventrculo al espacio epidural.
Recurrence. Dicha lesin envuelve el hueso intracraneal, y a travs de la
craniectoma occipital media previa al hueso extracraneal
confluyendo los dos componentes a travs del foramen
magno y descendiendo por la regin cervical posterolateral
Recibido: 25-05-09. Aceptado: 25-04-10 hasta nivel de C3 y C4, entrando en la cadena espinal acce-

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Garcia-Armengol y col 2011; 22: 453-456

Figura 1. Imagen de tomografa con contraste donde se visualiza la lesin envolviendo el hueso de fosa posterior y saliendo
extracranealmente.

Figura 2. Imgenes de resonancia magntica potenciadas en T2 donde se visualiza la lesin extendindose a planos muscu-
lares de la musculatura laterocervical del cuello.

soria. (Fig. 1) Hay una segunda lesin de partes blandas lgicamente sin focalidades neurolgicas. En la exploracin
cervicales posteriores adyacentes a la lesin occipital de fsica presentaba, una masa dura palpable y fija a nivel de
23 mm y caractersticas similares a la lesin previamente la zona cervical posterior y lateral izquierda, dolorosa a la
descrita. Impresin diagnstica: tumor de fosa posterior palpacin. Se decide intervenir quirrgicamente. Se realiza
con extensin extracraneal cervico-occipital compatible una ampliacin de la craniectoma de la fosa posterior, y se
con recidiva local. (Fig. 2) practica una exresis subtotal de la tumoracin intracraneal
El paciente se mantena clnicamente estable, y neuro- que invade la duramadre, hueso peristico y musculatura

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Meduloblastoma: una presentacin infrecuente en la recurrencia local 2011; 22: 453-456

esternomastoidea. La anatoma patolgica concluye que se la anaplasia est claramente implicada en la diseminacin
trata de un meduloblastoma. La parte extracraneal presenta fuera del sistema nervioso central5.
anaplasia. El caso clnico que presentamos corrobora el hecho de
la diseminacin a travs del abordaje quirrgico, y apoya
Discusin sta hiptesis diagnstica. Aunque esta implantacin
directa tumoral es un hecho poco frecuente, (hemos encon-
Les presentamos el caso clnico de un paciente con una trado tan solo 3 caso publicados en la literatura)1,4,7,8, es una
recurrencia local de un meduloblastoma. Originalmente complicacin evitable, y debemos considerarla.
dicho tumor se localizaba a nivel cerebelar, pero con las Las metstasis subcutneas del meduloblastoma,
sucesivas cirugas y tratamientos coadyuvantes, ha pre- deberan incluirse en el diagnstico diferencial, cuando
sentado una diseminacin que se extiende hacia planos un paciente ha recibido una craneotoma previa por un
extracraneales e infiltra hueso y musculatura laterocervi- meduloblastoma, ya que, como se ha visto en este trabajo,
cal. como en los publicados previamente en la literatura, la
El motivo de presentar este caso clnico, est moti- recurrencia de ste a nivel subcutneo, aunque rara, es
vado porque, si bien el meduloblastoma tiene una gran posible.
capacidad de metastatizar y de recurrir (la mayora de
meduloblastomas recurren a los 2 aos de la primera resec- Bibliografa
cin y de un tratamiento neurooncolgico, tanto a nivel del
sistema nervioso, a travs del lquido cefalorraqudeo, como 1. Colak, A., Belen, D., Onol, B., Saglam, S.: Adult medu-
a nivel extraneural, va hematgena, como a travs de los lloblastoma presenting as a large neck tumor. Case report. J
ganglios linfticos)2, la recurrencia local fuera del sistema Neurosurg Sci. 1993; 37: 171-173.
nervioso central, es un hecho poco frecuente, encontrado 2. Duffner, P., Cohen, M.E.: Extraneural metastases in
en la literatura en muy pocas ocasiones. El meduloblastoma cildhood brain tumor. Ann Neurol 1981; 10: 261-265.
es el tercer tumor cerebral en frecuencia, que se presenta 3. Eberhart, C.G., Cohen, K.J., Tihan, T., Goldthwaite,
con metstasis extraneurales despus del glioblastoma P., Burger, P.C.: Medulloblastoma with systemic metasta-
multiforme. Las metstasis en los tumores cerebrales fuera ses: evaluatin of tumor histopathology and clinical beha-
del sistema nervioso son raras, debido por un lado, a que vior un 23 patients. J Pediatr Hematol Oncol 2003; 25:
el tejido cerebral no se puede implantar y crecer en otros 198-203.
tejidos, y tambin motivado a que los tumores cerebrales 4. Galarza, M., Sosa, F.P.: Pure subcutaneous seeding from
malignos tienen una elevada agresividad, y los pacientes medulloblastoma. Pediatr Neurol. 2003; 29: 245-249.
mueren antes de producirse las metstasis5. A pesar de 5. Kochbati, L., Bouaouina, N., Hentati, D., Nasr, C.,
ello, el 5% de las metstasis del meduloblastoma son Besbes, M., Benna, F., Boussen, H., Maalej, M.: Medulloblas-
sistmicas, principalmente, de localizacin sea, que son toma with extracentral nervous system metastases: clinical
las ms comunes (77%), seguido de los ganglios linfticos presentation and risk factors. Cancer/Radiothrapie 2006; 10:
(33%), pulmn (17%), msculo (13%) e hgado (10%)5. 107111.
Galarza4 publica en el ao 2003 un caso clnico de un 6.Koeller, K.K., Rushing, E.J.: Medulloblastoma: a com-
meduloblastoma diseminado a nivel del tejido subcutneo prehensive review with radiologic-pathologic correlation.
abdominal, no explicable por una diseminacin hematgena Radiographics 2003; 23: 1613-1637.
ni linftica sino ms bien producida por la infiltracin de las 7. McComb, J.G., Davis, R.L., Isaacs, H. Jr, Landing, B.H.:
clulas tumorales durante el abordaje quirrgico. Creemos Medulloblastoma presenting as neck tumors in 2 infants. Ann
que como en nuestro caso, la extensin local del tumor en Neurol. 1980; 7: 113-117.
una localizacin tan poco frecuente como la musculatura 8. Topham, E.J., Francis, N., Bunker, C.B.: Medulloblas-
laterocervical o a nivel subcutneo, est producida por la toma metastasizing to the skin. Br J Dermatol. 2006; 154: 554-
implantacin de las clulas malignas en el zona cervical 555.
en la reseccin tumoral. Como se hipotetiza en el articulo
de Galarza4 es posible que exista la posibilidad de una ruta Garcia-Armengol, R.; Guilln-Quesada, A.; Costa-Clar,
quirrgica, dnde las clulas malignas puedan contaminar J.M.: Meduloblastoma: una presentacin infrecuente en la
los instrumentos quirrgicos durante la intervencin con recurrencia local. Neurociruga 2011; 22: 453-456.
la posibilidad de extenderse a otros tejidos, implantarse
y crecer. Tambin es posible que la quimioterapia y la Correspondencia: Roser Garcia-Armengol. Servicio de Neuro-
radioterapia, puedan alterar el sistema inmune y promover ciruga. Hospital Universitario Son Dureta (Palma de Mallorca).
el crecimiento tumoral extracraneal2. Tambin se puede C/ Andrea Doria, 55. 07014 Palma de Mallorca.
producir una progresin del grado histolgico tumoral3, y E-mail: rga36560@hotmail.com

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Garcia-Armengol y col 2011; 22: 453-456

Comentario al trabajo Meduloblastoma: una presenta- quimioterapia adyuvantes, haya desarrollado una hidro-
cin infrecuente en la recurrencia local de R. Armengol cefalia obstructiva extraventricular. La derivacin (cat-
y cols. ter peritoneal) sigue el mismo trayecto retromastoideo y
suboccipital hasta el tringulo posterior del cuello por lo
Los autores presentan el caso poco frecuente de reci- que es plausible que la implantacin se haya producido por
diva local extracraneal de un meduloblastoma. La primera va licuoral siguiendo el trayecto de la misma. La nica
consideracin sobre el caso clnico que nos ocupa es la metstasis subcutnea de nuestra serie de meduloblastomas
importancia de la reseccin quirrgica en una primera ins- se produjo en la fosa supraclavicular, sobre el catter de
tancia. El pronstico del meduloblastoma, an tratndose una derivacin ventrculo peritoneal previa, sin implantes
de un tumor altamente agresivo, est claramente relacio- abdominales, por lo que cabe suponer que dicha disemi-
nado con el grado de reseccin obtenida durante la primera nacin estaba producida por el paso de LCR y clulas
ciruga. La experiencia del cirujano, la localizacin tumoral tumorales pericatter.
(existencia o no de invasin del suelo del IV ventrculo por Las metstasis extracraneales del meduloblastoma
el tumor) y la edad del paciente (probablemente por la son muy inhabituales pero deben tenerse en cuenta en el
posibilidad de aadir radioterapia adyuvante al tratamiento diagnstico diferencial de cualquier masa subcutnea que
quirrgico) son variables que se han relacionado definitiva- aparezca en un paciente operado en el transcurso de su tra-
mente con el pronstico y la supervivencia de los pacientes tamiento adyuvante y evolucin posterior.
afectados por estos tumores1.
En otro orden de cosas, adems de los factores rela- Bibliografa
cionados en la discusin con las metstasis extracraneales
(hematgena, linftica o manipulacin quirrgica) debe 1. Correlation of neurosurgical subspecialization with out-
tenerse en cuenta la posibilidad de diseminacin a lo comes in children with malignant brain tumors. Albright, A.L.,
largo del trayecto de la derivacin ventriculoperitoneal. Si Sposto, R., Holmes, E., Zeltzer, P.M., Finlay, J.L., Wisoff,
bien los autores no comentan la existencia o no de dicha J.H., Berger, M.S., Packer, R.J., Pollack, I.F. Neurosurgery.
derivacin en este paciente en concreto, cabe suponer que 2000 Oct; 47: 879-85; discussion 885-887.
un meduloblastoma de fosa posterior, con tres interven-
ciones previas, diseminacin en cisternas, radioterapia y J. Hinojosa
Madrid

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Neurociruga
2011; 22: 457-460

Fatty filum with different histological features. Case report

Y. Izci; S. Pusat and O. Onguru*

Departments of Neurosurgery and Pathology*. Gulhane Military Medical Academy. Turkey.

Summary nstico y manejo apropiados. Un nio de 3 aos de edad


se present con marcas cutneas y moderada debilidad
Split cord malformation and fatty filum are com- en piernas sugestivos de malformacin espinal. La reso-
pletely different clinical entities and thought to arise nancia magntica revel duplicacin de la mdula de
via different pathophysiologies. Recognition of these tipo II y siringomielia T12-L1, que se asoci con anclaje
distinct lesions in the same patient is important for medular y filum terminale lipomatoso. El paciente fue
appropriate diagnosis and management. sometido a una laminotoma T12-L1 para la elimina-
A 3 year-old boy presented with skin lesions and cin de la banda fibrosa entre las dos hemimdulas y
mild leg weakness suggestive of spinal malformation. laminotoma L4-L5 con seccin del filum lipomatoso. El
Magnetic resonance imaging revealed type II split cord estudio anatomopatolgico del filum confirm la pre-
malformation at T12-L1, syringomyelia at T8-T10 levels sencia de hueso, grasa, y clulas epiteliales ciliadas, que
associated with tethered cord and fatty filum terminale. se asociaron con proliferacin meningotelial.
The patient underwent a T12-L1 laminotomy for the Informamos un caso excepcional de diastematomielia
removal of fibrous band between the 2 hemicords and tipo II que coexiste con un filum lipomatoso con patrn
L4-L5 laminotomy for transection of the fatty filum. histolgico diferente. En nuestra opinion, este patrn
Histopathological examination of the filum confirmed histolgico correspondiente al filum lipomatoso no ha
the presence of bone, fat, and ciliated epithelial cells sido documentado previamente, y ello suscita cuestio-
associated with meningothelial proliferation in the same nes sobre una posible relacin, sea de asociacin o cusal,
specimen. entre estas dos patologas.
We report an unusual case of type II split cord
malformation coexisting with a fatty filum which PALABRAS CLAVE. Diastematomielia. Filum lipomatoso.
have different histological patterns. To the best of our Anclaje medular. Nio.
knowledge, this histological appearance of a fatty filum
has not yet been reported and this raises the question of Introduction
a possible associative or causative relationship between
these distinct pathologies. Split cord malformation (SCM) is a form of closed
neural tube defect in which the spinal cord is longitudina-
KEY WORDS. Split cord malformation. Fatty filum. lly split by a fibrous band or a bone spicule. Type II SCM
Tethered cord. Child (Diplomyelia) consists of two hemicords housed in a single
dural tube separated by a fibrous median septum2. Patients
Filum lipoatoso con caractersticas histolgicas diferen- with SCM may have multiple causes of tethering associated
tes. Caso clnico with their congenital anomaly such as myelomeningocele,
intradural lipoma, and fatty filum2,8,9.
Resumen The fatty filum involves fatty infiltration of the whole
length or of part of the filum terminale. It is the simplest
La diastematomielia y el filum lipomatoso son enti- form of conditions causing tethered cord syndrome (TCS)
dades clnicas completamente diferentes y cuya patoge- and the filum terminale can be thickened, normally with
nia se considera diferente. El reconocimiento de estas lipomatous tissue1,3. The fatty filum may be associated with
lesiones en el mismo paciente es importante para el diag- SCM, suggesting a common timing of pathogenesis during
development.
Recibido: 12-06-10. Aceptado: 17-09-10 A rare case of diplomyelia associated with a fatty filum

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Izci et al 2011; 22: 457-460

Figure 1. (A) Preopera-


tive sagittal T1-weig-
hted MRI of the patient
showing syringomyelia
at T8-T10 level and
conus ending at L4.
The white arrow shows
the hyperintense fatty
filum. (B) Axial T2-
weighted MRI section
shows 2 hemicords that
are separated by a thin
fibrous band at T12
level. (C) The filum is
hyperintense in axial
MRI sections.

is presented. Bone, fat, and meningoepithelial proliferation vation of the underlying neural structures.
associated with ciliated epithelial cells were found in the The histological examination of the removed speci-
histological examination of the filum. These histological men consisted of a non-encapsulated piece of fatty tissue
findings and possible pathogenetic mechanisms are discus- measuring 10.60.6 cm. The cut surface showed a soft
sed. yellow area. Pathological examination of the resected

Case report

A 3-year-old boy presented with mild leg weakness and


cutaneous lesions on the back. Physical examination revea-
led a dimple, and red lesions with a hairy patch. The child
had normal power in his lower limbs. Both knee and ankle
reflexes were present. He had a normal anal reflex. Spinal
magnetic resonance (MR) images revealed a syringomyelia
at T10-T12, a fibrous band dividing the spinal cord at T12
level and a low-lying conus with tethering of the cord at
L5-S1, associated with the presence of fatty tissue at the
lower end of the terminal filum (Fig. 1). Results of electro-
physiological and urodynamic studies were normal.
The child underwent surgical exploration. The fibrous
band between the 2 hemicords was removed via a T12-L1 Figure 2. (A) Intraoperative view of the hemicords separa-
laminotomy and the conus medullaris was released by ted by a fibrous band at T12-L1 level; (B) Intraoperative
cutting the fatty filum terminale via an L4-L5 laminotomy view of the fatty filum at L5 level after laminotomies and
(Fig. 2). A part of the fatty filum was resected with preser- dural openings.

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Fatty filum with different histological features. Case report 2011; 22: 457-460

Figure 3. (A): histopathological examination of the filum shows bone and fat tissues associated with meningothelial prolife-
ration and fibrous tissue. (B): bone, F: Fat, MP: Meningothelial proliferation, Fi: Fibrous tissue, V: Vessel (H&E x40); (B)
Ciliated epithelial cell bundles are obvious in the filum after magnification. The black arrow shows the cilia of the epithelial
cells. E: Epithelial cell tuft (H&E x200).

tissue demonstrated an area with different cytoarchitecture. were completely different to those of the normal filum.
There was a complex cellular arrangement with sheets of Atrophy of the caudal neural tube is responsible for the
meningothelial proliferation, bone, fat tissue and fibrosis. formation of the filum terminale, which connects the future
In some areas there were ciliated epithelial cells (Fig. 3). conus medullaris with the coccygeal medullary vestige, a
Postoperatively the patient began, mobilization and was mixed tissue containing ependymal cells, neurons, and glial
discharged without any complication. cells embedded in fibrofatty fibrolipomatous tissue in the
central area between the coccyx and the end of the dural
Discussion sac. A fatty filum seems to occur due to a congenital error
in the canalization of the caudal bud of the spinal cord. An
Diplomyelia and fatty filum terminale are separate aberrant accumulation of mesenchymal precursor cells
conditions presumed to have different underlying patho- from the pluripotent cell mass of the caudal eminence is
physiological characteristics. Although fatty filum is a thought to lead to an abnormal retrogressive differentiation
well-known coexisting pathology within the split cord of the secondary neural cord10,12,15. The exact mechanism
malformations, this intriguing association with different is not well understood, but one could propose that faulty
histological patterns has not been previously reported in cell movements may prevent the migration of the mesoder-
the pediatric literature. mal cells to their normal positions, subsequently leading
The unified theory proposes that all SCMs originate to distal cord dysfunction. These changes may cause the
from one basic ontogenetic error occurring around the formation of adhesions of the fatty filum to the adjacent
time when the primitive neurenteric canal closes5. This structures and the traction effect on the spinal cord without
basic error is the formation of an accessory neurenteric by preventing the normal flexibility and elasticity thus pre-
canal through the midline embryonic disc that maintains disposing to cord tethering10.
communication between yolk sac and amnion and enables Although it has been reported that a filum terminale
continued contact between ectoderm and endoderm within that is either fatty or thick might have a tethering effect
the canal. Thus, according to the unified theory, both Type on the spinal cord, which causes symptoms of tight spinal
I and II SCMs originate from the same basic embryoge- cord syndrome, it has also been reported that a normal
netic mechanism, the only difference between them being thickness filum terminale can have a tethering effect if its
the presence or absence of meninx precursor cells in the natural architecture is changed due to various factors. In
endomesenchymal tract14. SCMs are usually associated general, the diagnosis of TCS is based on clinical findings
with other pathologies such as TCS13,17, and the fatty filum and serial neurological examinations, with radiological
is considered as one of the causes of this syndrome. In our investigations playing a secondary role. The fat within the
case, we found Type II SCM associated with a TCS and short, thick filum is discernible by unenhanced computed
fatty filum. But the histological characteristics of the filum tomography or MR imaging4. MR is excellent for detecting

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Izci et al 2011; 22: 457-460

diplomyelia and for showing hyperintense fat within the 6. Fontes, R.B., Saad, F., Soares, M.S., de Oliveira, F.,
thickened filum. The demonstration of this fat is diagnostic Pinto, F.C., Liberti, E.A.: Ultrastructural study of the filum
of an abnormal filum terminale. We detected diplomyelia terminale and its elastic fibers. Neurosurgery 2006; 58: 978-
and fatty filum on the preoperative MR imaging and plan- 984.
ned surgery in the light of these findings. We also obtained 7. Gamble, H.J.: Electron microscope observations upon
filum samples for histopathological examination. the conus medullaris and filum terminale of human fetuses. J
The normal filum is composed largely by loose colla- Anat 1971; 110: 173-179.
gen fibers, but it also contains small blood vessels and 8. Izci, Y., Gurkanlar, D., Gnl, E.: An unusual type of
occasional small nerve fascicles18. In addition, remnants of split cord malformation. J Clin Neurosci 2007; 14: 383-386.
the central canal lined with ependymal cells may also be 9. Izci, Y., Gonul, M., Gonul, E.: The diagnostic value of
detected in some specimens6,7,11. But in a thickened filum, skin lesions in split cord malformations. J Clin Neurosci 2007;
elevated numbers of collagen bundles, scattered nests of 14: 860-863.
ependymal cells, glial cells, large clumps of fat cells, and 10. Karabatsou, K., Crooks, D., Williams, D., Buxton,
areas of hyalinization are frequently observed throughout N.: Combination of myxopapillary ependymoma and fatty
the connective tissue. Ciliated epithelial cells, meningothe- filum in a child with tethered cord syndrome. Case report. J
lial proliferation and bone tissue are not frequently found in Neurosurg Pediatr 2008; 1: 386-388.
a thickened filum. Our case emphasizes the need to obtain 11. Miller, C.: The ultrastructure of the conus medullaris
a full histopathological examination of the tissues resected and filum terminale. J Comp Neurol 1968; 132: 547-566.
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the associated lesions may alter the long-term prognosis in dermoid in a low lying conus tethered by a fatty filum embr-
these patients by using with the appropriate treatment and yological implications. Acta Neurochir (Wien) 2007; 149:
follow-up with sequential MR imaging studies. 1173-1175.
In conclusion, TCS must be evaluated carefully with 13. Ozturk, E., Sonmez, G., Mutlu, H., et al.: Split cord
appropriate radiological techniques. Structure of the spinal malformation and accompanying anomalies. J Neuroradiol
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tial diagnosis. double spinal cord malformations. Neurosurgery 1992; 31:
451-480.
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