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Etiologic Factors of Acute Aortic Dissection in Children and Younrg Adults

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DOI: 10.1177/000992280003900201 · Source: PubMed

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 Clinical Pediatrics
http://cpj.sagepub.com

Etiologic Factors of Acute Aortic Dissection in Children and Younrg Adults

Charles R. Fikar and Simone Koch
Clin Pediatr (Phila) 2000; 39; 71
DOI: 10.1177/000992280003900201

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http://cpj.sagepub.com/cgi/content/abstract/39/2/71

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 Etiologic Factors of Acute Aortic Dissection
in Children and Younrg Adults
Charles R. Fikar, MD, MSLS
Simone Koch, BS

Summary: Current concepts in the pathophysiology and predisposing conditions of acute aortic dis-
section in children, adolescents, and young adults are presented. Timely diagnosis is required for
this life-threatening condition. Most children and adolescents with aortic dissection have congenital
cardiovascular anomalies. Certain heritable disorders involving connective tissue also predispose to
this disorder. Newer associations include cocaine abuse and weight lifting. To facilitate early diagno-
sis, the salient physical findings of the known predisposing conditions are reviewed. Clinical
presentation and diagnostic imaging of aortic dissection are briefly summarized. Physicians working
in an acute care setting, particularly in the emergency room, should be aware of disorders
predisposing to acute aortic dissection in the pediatric and young adult population. Practitioners
conducting school or college preparticipation sports evaluations can make use of such information
in their assessment of risk for sudden death. Clin Pediatr. 2000;39:71-80

Introduction aortic dissection are summarized age, younger individuals may be

M uuch new information
concerning the subject
of aortic dissection pre-
senting in the pediatric and
young adult population has ap-
peared in the medical literature
since the first author's previous
published review.' This report re-
views current concepts in the
pathophysiology and predispos-
ing conditions of this life-threat-
ening condition with the aim of
assisting the pediatrician in mak-
ing a timely diagnosis. The major
presenting signs and symptoms of
New York College of Podiatric Medicine, New York, NY.
in addition to current informa- affected. Cases have occurred
tion on imaging technology use- during infancy, childhood, and
ful in this condition. Knowledge adolescence,' and most large se-
of the predisposing conditions ries report some patients under
and their major physical findings 20 years of age."4'5 During a 1-year
may also be useful information time interval, two of 5,658 deaths
for practitioners conducting in New York State in the age range
preparticipation sports physical 0-19 years were due to aortic dis-
assessments when screening for section.1 Of a total of 1,085 pa-
risk for sudden death in the child tients reported in two large series
or adolescent athlete.2 of dissecting aortic aneurysm 38
Approximately 2,000 cases of (3.5%) occurred in persons 19
acute aortic dissection occur years of age or younger.'
every year in the United States.3 Prompt diagnosis may be life
Although most of these cases oc- saving because untreated aortic
cur in persons over 19 years of dissection has an estimated mor-
tality rate of from 1% to 2% per
hour during the first 48 hours
from onset.6 Knowledge of the
predisposing conditions in the pe-

diatric population might expedite

Reprint requests and correspondence to: Charles R. Fikar, MD, MSLS, Library Director, the diagnosis of acute aortic dis-
New York College of Podiatric Medicine, 53 East 124 Street, New York, NY 10035.
section in the presenting infant,
2000 Westminster Publications, Inc., 708 Glen Cove Avenue, Glen Head, NY 11545, U.S.A. child, or adolescent. Since early

FEBRUAR1Y 2000 2000CLINICAL

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diagnosis and treatment remain
the essential prerequisites for sur-
vival of patients with this condi-
tion, those disorders predisposing
to aortic dissection in the pedi-
atric and adolescent age groups
are presented along with some of
their more obvious physical diag-
nostic clues. For detailed diagnos-
tic criteria other reference works
need to be consulted, many of
which appear as references in this
report. Timely diagnosis and on-
going evaluation may also prevent
catastrophic consequences for
family members in the many in-
stances where inherited disorders
are responsible for aortic dissec-
tion. Genetic counseling and
prospective ongoing evaluation
and treatment of patients and kin
will therefore be necessary.
Pathophysiology
Aortic dissection occurs when
blood dissects between the mid-
dle and outer two thirds of the
media, creating a blood-filled
channel within the aortic wall.7
This channel has been termed the
"false lumen." The process usually
occurs in the ascending aorta
within 10 cm of the aortic valve
where an intimal tear is usually
observed. This dissecting intra-
mural hematoma may rupture
into the pericardial, pleural, or
peritoneal cavities. Aortic branch
arteries may become either par-
tially or completely occluded. Sta-
tic obstruction occurs when the
dissecting hematoma intersects or
completely enters the opening of
a branch artery. This decreases
the lumen size or may entirely
block the lumen of the branch
artery. Dynamic obstruction in-
volves the complete or partial cov-
ering of the branch artery open-
ing by a flap of the dissected
aortic wall without the hematoma
72 CLINICAL PEDIATRICS
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Fikar, Koch
dissecting into the branch artery. medial degeneration. Table 1 lists
A combination of dynamic and conditions that predispose to aor-
static obstruction may also occur.8 tic dissection and for which docu-
Forward extension of the mented cases in the age range of
hematoma may cause partial or 0-21 years have been found. Some
total vascular obstruction of the of these conditions are known to
carotid, renal, mesenteric, iliac, be associated with aortic medial
or spinal arteries with subsequent weakening. A brief summary of
catastrophic complications. Back- the cause of this weakening, if
ward extension may cause ob- known, is given for each condition
struction of the coronary arteries in the following paragraphs.
or malfunction of the aortic valvu-
lar apparatus. This is the ana-
tomic basis for many of the signs Predisposing Conditions
and symptoms of acute aortic
dissection. Marfan syndrome involves de-
Initiation and propagation of fective synthesis of the lipopro-
most dissecting aortic aneurysms tein fibrillin-I that is present in
is believed to involve the interplay the media of arteries.3 Fibrillin is
of three factors.3 First, genetic fac- the major element of extracellu-
tors, inflammatory processes, hy- lar microfibrils.10 These connec-
pertension, drug or lifestyle tive tissue microfibrils are
choices, as well as certain predis- thought to provide a framework
posing conditions may intrinsi- for the deposition and crosslink-
cally weaken the aortic wall's me- ing of elastin during the forma-
dial layer, which is composed of tion of elastic tissue. Large arter-
elastin, collagen, and smooth mus- ies such as the aorta contain
cle. Second, a tear occurs in the in- approximately 50% elastin by dry
tima that initiates the process of weight.11 Defects in human fib-
dissection. A transverse intimal rillin-1 have been shown to result
and medial tear has been noted in in the Marfan syndrome. The ma-
more than 95% of patients with jority of affected individuals
aortic dissection.9 Third, appro- have mutations that affect the
priate dynamic circulatory forces synthesis, secretion, or matrix in-
are present that can lead to pro- corporation of this molecule.
gressing dissection. The human fibrillin-1 gene is lo-
The aortic media may be in- cated on chromosome 15.12 Aor-
volved by processes that eventu- tic wall weakening is due to frag-
ally lead to loss and fragmenta- mentation of medial elastic
tion of the elastic lamellae within fibers demonstrable on histo-
the media of the vessel wall as well logic examination.13 Almost 90%
as pseudocyst formation due to of the known causes of death in
the accumulation of glycopro- this syndrome are due to cardio-
teins. Abnormal collagen may be vascular disorders, the majority
present. Smooth muscle loss may being aortic root complications
also occur. The ultimate result of such as aortic dissection, rup-
these processes is weakening of ture, or insufficiency.'4 Aortic
the aortic wall.3,6 Although some dissections have occurred in chil-
of these processes may occur as dren and adolescents with Mar-
the result of normal aging, hyper- fan syndrome."'15"16
tension and conditions involving Definitive diagnosis of Marfan
connective tissue maldevelop- syndrome may not be possible in
ment or destruction accentuate the acute care setting when a
FEBRUARY
FEBRUARY 2000
2000.
 Etiologic Factors of Acute Aortic Dissection
child or adolescent is presenting
with severe chest pain and may be
in shock. However, there are
some physical features that may
provide a clue to the diagnosis
and therefore may make suspi-
cion of aortic dissection timelier.
These include upwardly dis-
placed ocular lens, an "asthenic"
body habitus due to muscular un-
derdevelopment and decreased
subcutaneous fat, tall stature, un-
usually long arm span, arachn-
odactyly, and pes planus and pec-
tus excavatum or carinatum.13,17
Type IV Ehlers-Danlos syn-
drome involves the formation of
FEBRUARY 2000
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this condition. Poor healing qual-
ities may lead to skin scarring.
The small joints of the hands and
feet are hyperextensible and
largerjoints may also be involved.
The face may have a thin, pinched
nose; large eyes that are widely
spaced; midface flattening; as well
as thin lips.1920 Patients with this
syndrome typically die prema-
turely, the most serious condi-
tions being rupture of arteries or
internal organs such as spleen,
colon, uterus, and lung. Rupture
or aneurysm formation of the
aorta as well as other arteries can
lead to early death. Pediatric pa-
tients with this condition have
been demonstrated to have acute
aortic dissection.1,20,21
Familial occurrences of aortic
dissection, without stigmata of
any of the well-characterized heri-
table disorders of connective tis-
sue, have been reported. Docu-
mented cases of familial aortic
dissection have occurred in ado-
lescents.1 Familial aortic dissec-
tion, an entity with a pathology
previously termed Erdheim cystic
medial necrosis, is an inherited
disorder thought to involve weak-
ening of the aortic medial layer.
Another name for this entity is an-
nuloaortic ectasia. Examination
of the aortic media reveals loss of
an abnormal Type III procollagen elastic fibers, deposition of mu-
predisposing to a weakening of copolysaccharide-like material in
aortic connective tissue.6 The ma- the media, and cystic medial
jor collagens found in the walls of changes.22 The current preferred
blood vessels are Type I and Type term for this pathologic picture is
III, with the latter predominating cystic medial degeneration.723
in large vessels such as the aorta.11 Many families have been de-
The gene site is termed COL3A1 scribed with an autosomal domi-
and is located on chromosome nant mode of inheritance.1,24 In
2.18 Patients with this condition the majority of these families no
have thin, translucent, parch- specific biochemical abnormality
ment-like skin that is somewhat has been found. The only unify-
hyperextensible and extremely ing factor in most of these fami-
fragile. Veins may be prominently lies is the familial occurrence of
seen because of translucency of aortic dissection, and the previ-
the skin. Marked skin bruising ously described aortic pathology.
may be the first manifestation of In some instances of familial aor-
CLINICAL PEDIATRICS
CLINICAL PEDIATRICS

tic dissection biochemical abnor-
malities of Type III collagen have
been found.
For example, a single base mu-
tation that converted the codon
for glycine-619 in type III procolla-
gen to arginine was demonstrated
in one family having aortic
aneurysms.25 It has been shown that
the first-degree relatives of proband
cases of thoracic aortic aneurysm,
thoracic aortic dissection, or thora-
coabdominal aneurysm are at
higher risk for thoracic aortic
aneurysm and sudden death.
Proband fathers and sisters had a
1.8-fold increased risk of thoracic
aortic aneurysm while proband
brothers had an 1 1-fold increased
risk in one study.26
A familial syndrome of arterial
dissections with lentiginosis has
been described with one patient
22 years of age having had an as-
cending aortic dissection.27 Cystic
medial degeneration was found in
those patients having undergone
surgical resection or autopsy. The
distinctive physical finding in this
family is the presence of large
numbers of uniformly sized pig-
mented maculae of dark-brown to
black color, of between 2 to 4 mm
in diameter. Appearing particu-
larly on the extremities, these ap-
pear on skin areas not exposed to
sunlight as well as areas exposed
to the sun. The authors reporting
this syndrome speculate that a dis-
order of the neural crest could ac-
count for this syndrome since
both melanocytes and the entire
tunica media of the aortic arch
and its branches originate from
cells of the neural crest. It is
known that ablation of a part of
the cranial neural crest in chick
embryos results in disarray of the
elastin fibers of the aortic wall. A
migration disorder of cells of the
neural crest is a possible mecha-
nism in the pathogenesis of coarc-
tation of the aorta and bicuspid
74 CLINICAL PEDIATRICS
74 CLINICAL PEDIATRICS
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Fikar, Koch
aortic valve, both of which
sociated with aortic dissection
and aneurysm.
Intrinsic abnormalities of the
aorta or its valves such as coarcta-
tion of the aorta, bicuspid aortic
valves, congenital aortic valvular
stenosis, and supravalvular aortic
stenosis, singly and in various
combinations, have been associ-
ated with aortic dissection, in the
pediatric population.l28 Most of
the cases of congenital aortic
valve stenosis were in fact bicuspid
or unicuspid valves. Congenital
cardiovascular anomalies are the
most commonly found abnormal-
ities in children and adolescents
presenting with aortic dissection,
with coarctation of the aorta be-
ing the most common entity.i Be-
fore the introduction of surgical
correction, aortic dissection and
rupture occurred in about 23% of
untreated cases of coarctation of
the aorta.29 Note that aortic dis-
section may occur years after suc-
cessful surgical correction of
coarctation.30
Pathologic changes of cystic
medial degeneration have been
found in the aortas of patients
with either bicuspid aortic valves,
coarctation of the aorta, or both.
Bicuspid aortic valve, aortic coarc-
tation, and cystic medial degener-
ation, either individually or in any
combination, may be part of a ba-
sic developmental defect of the
arterial tree,31 as previously men-
tioned in the discussion of the fa-
milial syndrome of arterial dissec-
tions with lentiginosis.27 An
intrinsic weakening of the aortic
wall may predispose patients with
this pattern of anomalies to aortic
dissection.
A 2-year-old boy with supra-
valvular aortic stenosis and
Williams syndrome had an aortic
dissection with dysplasia of the
aortic wall.28 Williams syndrome
can present with physical findings
are as- affecting the face, limbs, denti-
tion, and the musculoskeletal sys-
tem. The patient may have men-
tal deficiencies, hoarse voice,
hyperactive deep tendon re-
flexes, and poor coordination.
The face may have prominent
lips with open mouth, medial eye-
brow flare, depressed nasal
bridge, epicanthal folds, perior-
bital fullness of subcutaneous tis-
sues, blue sclera, a stellate pat-
tern of the iris, anteverted nares,
and long philtrum. The patient
may also have hypoplastic nails,
hallux valgus, lordosis, scoliosis,
kyphosis, partial anodontia, and
enamel hypoplasia.32
Turner syndrome (45, X) is as-
sociated with aortic dissection as
well as with other cardiovascular
abnormalities, such as bicuspid
aortic valve and aortic coarcta-
tion.33 Many of the patients have
been shown to have cystic medial
degeneration on pathologic ex-
amination and may have a pri-
mary mesenchymal defect involv-
ing bone matrix, lymphatic
vessels, as well as the aorta. One
study using echocardiography
demonstrated that 34% of 35 pe-
diatric patients with Turner syn-
drome had evidence of bicuspid
aortic valve,34 which is also associ-
ated with aortic dissection as pre-
viously noted. These female pa-
tients are usually short in stature
and may be developmentally de-
layed. They tend to have a broad
chest with widely spaced nipples
and a webbed neck. Nails may be
narrow, hyperconvex, or deep-set.
There is an increased incidence
of hyperpigmented nevi as well as
a tendency for keloid formation.35
Cases of aortic dissection in per-
sons with Turner syndrome have
occurred in the pediatric popula-
tion.33 Noonan syndrome has
been associated with aortic dissec-
tion,36 but no cases in the pedi-
atric age group could be found.
FEBRUARY 2000~~~~~~~~~~
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 Etiologic Factors of Acute Aortic Dissection
Fibromuscular dysplasia has
been associated with aortic dissec-
tion in the pediatric population.
Fibromuscular dysplasia primarily
affects the medial layer of arter-
ies.37 This is presumed to cause
the weakening that can lead to
aortic dissection in affected areas.
One 18-year-old patient with this
association was found to have a
point mutation in the gene for
Type III collagen. Many condi-
tions may be associated with fi-
bromuscular dysplasia, including
tuberous sclerosis, neurofibro-
matosis, congenital rubella syn-
drome, and others.38 A 3-year-old
child with tuberous sclerosis was
reported to have died of a rup-
tured thoracic aortic aneurysm.39
This aortic aneurysm was of the
dissecting type (Personal commu-
nication, Manuel R. Gomez, MD,
April 8, 1998). Prominent physical
findings of tuberous sclerosis in-
clude abnormalities mainly occur-
ring in the brain and skin. The
characteristic and distinctive le-
sion is adenoma sebaceum. These
can often be mistaken for acne
and consist of angiofibromas of
the cheeks and nasolabial folds.
Large hypopigmented macules
called ash leaf macules appear
most commonly on the trunk.
The shagreen patch, a skin-col-
ored plaque, contains dermal tis-
sue and may be present during
early childhood. These patches
have been described as having the
appearance of "orange peel" or
"pigskin" and are typically located
in the lumbosacral area. Postpu-
bertal patients may have periun-
gual and subungual fibromas.40,41
Pregnancy is a known risk fac-
tor for the development of aortic
dissection in the young female
population. A 20-year-old patient
has been described. About 50% of
the cases of aortic dissection in
women under the age of 40 occur
in the third trimester of preg-
FERUR
FEBRUARY 2000
nancy.1 The hyperdynamic and within the media, especially if the
hypervolemic circulatory state in media is already weakened from
pregnancy may play a causative some pathologic process. Trau-
role. Also, estrogen may inhibit matic shearing and rupture of the
collagen and elastin deposition vasa vasorum might also be an ini-
in the media of the aorta and tiating event for medial dissec-
contribute to the pathogenesis of tion.1,48 It appears that the major-
dissection in pregnancy. Many ity of posttraumatic dissecting
cases of aorticdissection in preg- aortic hematomas usually occur
nant women with Marfan syn- between the media and adventitia
drome have been reported. One of the aorta in contrast to the in-
might intuitively believe that tramedial location in classical
pregnancy in patients already aortic dissection. These subad-
predisposed to aortic dissection ventitial hematomas may rupture
would increase their risk for that or cause luminal compression
event, but this seems to be an just as the medial type of dissec-
area that has not been specifically tion. Ruptures following trauma
studied.42 A recent report of six may be delayed. These subadven-
cases of aortic dissection during titial hematomas differ from me-
pregnancy demonstrated that two dial dissections in that they tend
(33%) were in women with Mar- to involve short segments of the
fan syndrome.43 aorta and generally show no
Many inflammatory condi- opacification of the false lumen
tions that involve the aorta have during aortography. Cases of aor-
been associated with aortic dissec- tic dissection secondary to trauma
tion. An adolescent with giant cell have occurred in children and
aortitis was demonstrated to have adolescents.48-51
had an aortic dissection. Al- Hypertension is the most fre-
though other disorders such as re- quent finding in adults present-
lapsing polychondritis and ing with aortic dissection.5 Al-
Takayasu arteritis have been asso- though some children with aortic
ciated with aortic dissection, no dissection have had hypertension,
examples in the pediatric age most do not. Although chronic
range could be found.' Although hypertension is the most impor-
aortic aneurysms may occur tant risk factor for aortic dissec-
rarely in children with mucocuta- tion in adults, an exact patho-
neous lymph node syndrome genic mechanism has not been
(Kawasaki disease), no instances found. There is disagreement as
of aortic dissection have been re- to the role of chronic hyperten-
ported to date.44 A child with sion in accelerating the process of
cystinosis had an aortic dissection, cystic medial degeneration.552
but hypertension secondary to Dissection of the aorta may oc-
kidney failure may have been the cur as an immediate or long-term
cause of the dissection.45 consequence of balloon angio-
Trauma has been reported to plasty for coarctation of the aorta.
cause acute aortic dissection.4647 The inflating balloon can cause
Blunt chest or abdominal trauma, aortic intimal tears and medial
particularly high-speed accelera- rupture that can lead to aortic dis-
tion or deceleration injuries as in section. Instances of this occur-
automobile accidents, can cause rence have been reported in in-
aortic dissection or rupture. A fants and children. As previously
traumatic tear of the intima mentioned, the aortas of patients
would permit blood to dissect with aortic coarctation are
200CIIA EITIS 7
CLINICAL PEDIATR[CS 75
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thought to be inherently predis-
posed to the development of dissec-
tion. One would therefore expect
such occurrences regardless of the
method of repair, including surgery
and end-to-end reanastomosis.29,53,54
Weight lifting or weight train-
ing has been associated with aor-
tic dissection in young persons,
including adolescents.55 It has
been speculated that the very
high blood pressure generated
during the lifting of weights, par-
ticularly with straining accompa-
nied by a Valsalva maneuver, may
be the cause of an aortic intimal
tear.55,56 Some of the young adults
with this association have had cys-
tic medial degeneration of the
aorta. It is not known whether sev-
eral years of exposure to the tran-
sient great increases in blood
pressure caused by weight lifting
can cause enough critical stresses
on the aorta to induce cystic me-
dial degeneration. Preexisting
medial disease may have made
these persons more vulnerable to
the stresses produced by weight
lifting. As many adolescents lift
heavy weights for exercise or as
training for sports, encouraging
proper breathing technique dur-
ing lifting may be helpful. Pa-
tients with conditions predispos-
ing to aortic dissection, including
those with family histories of cys-
tic medical degeneration, should
probably be strongly counseled to
refrain from weight lifting. Absti-
nence from contact sports might
also be prudent.56'57
Another newer association
with aortic dissection is the use of
cocaine or crack by young per-
sons.58 It has been speculated that
as a result of high blood pressure
and tachycardia induced by the
pharmacologic effects of cocaine,
an environment conducive to the
development and propagation of
an aortic dissection is created.59
Reported patients have been as
76 CLNIA PEITISFERAY20
76 CLINICAL PEDIATRICS
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Fikar, Koch
young as 26 years old. However, a Neurologic manifestations
review of New York State hospital such as altered mental status,
inpatient statistics for the years dysesthesias, weakness, or paraly-
1987 through 1996 revealed the sis also occur quite commonly.
case of a 20-year-old with a dis- Decreased perfusion to the
secting aortic aneurysm with asso- brain, spinal cord, or peripheral
ciated diagnoses of cocaine abuse nerves secondary to full or par-
and drug psychosis with no other tial occlusion of the aorta or its
predisposing medical condition. branch vessels may be causative.
(Personal Communication, Menges- Shock from cardiac tamponade,
Smith, Eta, Biostatistician, Bureau acute heart failure, or hypoperfu-
of Biometrics, State of New York, sion due to hypovolemia from
Department of Health, May 29, acute aortic rupture may also be
1998.) Physical findings of co- contributory."61'62
caine abuse may include nasal Physical signs of acute aortic
septal ulceration or perforation as dissection involve primarily the
well as general debilitation.60 cardiovascular, pulmonary, and
neurologic systems. Signs of or-
thostatic hypotension may be pre-
Clinical Presentation sent. Shock may be due to aortic
medial hemorrhage, aortic rup-
An abrupt onset of severe ture, pericardial tamponade, or
pain, often described as knifelike, acute heart failure. Acute cardiac
tearing, or ripping, is the most failure may be a manifestation of
common presenting symptom of severe aortic valvular insuffi-
aortic dissection. This excruciat- ciency or may occur when full or
ing pain tends to be migratory partial blockage of coronary ves-
and may involve the anterior and sels leads to acute myocardial in-
posterior chest, neck, jaw, shoul- farction. Extremity blood pres-
der, upper and lower back, ab- sure differentials as well as weak
domen, and extremities. The or absent peripheral pulses may
changing location of the pain, be present. Serial examinations
thought to occur as the dissection may prove useful as deficits may
progresses to involve different ar- be transient and new areas of in-
eas of the aorta, may be a valuable volvement may occur with pro-
clue to this diagnosis. Ascending gression of dissection.1'61
aortic involvement typically Percussive dullness of a
causes anterior chest pain, while hemithorax may be due to hem-
neck and/or jaw pain can occur orrhage from aortic rupture. Al-
with aortic arch dissection. Upper tered states of consciousness, syn-
back pain can occur with dissec- cope, paresis, paralysis, seizures,
tion of the descending thoracic absence of deep tendon reflexes,
aorta while abdominal aortic in- and dysesthesias are among the
volvement may cause lower back more common signs involving the
pain. Clinicians need to be aware neurologic system. A more nearly
that it is possible for the pain to comprehensive discussion of the
remain localized to a single spe- physical findings of acute aortic dis-
cific area such as the back. Vaso- section is presented elsewhere.",6'
vagal symptoms including nausea, A rapid general survey of the
vomiting, and diaphoresis may child or adolescent patient pre-
also accompany the onset of dis- senting with severe chest pain
section. Patients typically tend to may reveal one or more of the
be extremely apprehensive."61,62 specific findings suggestive of the
FEBRUARY 2000
 Etiologic Factors of Acute Aortic Dissection
many previously discussed condi-
tions that may predispose to acute
aortic dissection. Finally, al-
though not strictly a part of the
physical evaluation, a brief med-
ical and family history, including
known familial causes of sudden
death, may also be helpful. In-
quiry into possible drug abuse
and weight training may be valu-
able clues.
Once a diagnosis of aortic dis-
section is entertained, appropri-
ate surgical, cardiologic, and radi-
ographic consultations need to be
expeditiously obtained. Routine
laboratory tests are not of much
value in the acute situation, but
electrocardiography may be use-
ful in patients with congenital
heart disorders or hypertension
and may detect myocardial is-
chemia or infarction. Imaging
studies are the primary means to a
rapid and accurate diagnosis and
will be summarized briefly. A
more nearly comprehensive dis-
cussion of imaging modalities is
presented elsewhere.61,63,64
Imaging
Since time is critical to the out-
come, prudence would dictate
that each emergency care center
develop an imaging strategy for
the diagnosis of suspected aortic
dissection. It has been suggested
that a committee consisting of
knowledgeable specialists such as
cardiologists, emergency medi-
cine specialists, cardiovascular
surgeons, radiologists (and the
authors would add pediatricians)
create such an institutional diag-
nostic strategy. Different centers
will have different technologies
and modalities available and need
to choose the method that yields
accurate, fast results with the least
risk to individual patient cate-
gories. Each of the imaging
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FEBRUARY
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modalities has advantages and dis- Computed tomography is
advantages and is suitable to dif- but does require in-
noninvasive
ferent patient care needs and cir- jection of a contrast agent.
cumstances. Managed care would Demonstration of two distinct aor-
also require that this all be done tic lumens and the intimal flap
in a cost-efficient manner.61,63,64 confirms a diagnosis of aortic dis-
In essence, there are four ma- section. The ability to readily de-
jor technologies available to diag- lineate other causes of mediastinal
nose this condition in addition to widening is an advantage of this
the routine chest radiograph. technology. Its sensitivity is low
These include aortography, and this procedure cannot reliably
echocardiography, computed to- identify aortic insufficiency or
mography, and magnetic reso- demonstrate involvement of
nance imaging. Although the branch vessels, including the coro-
chest radiograph commonly nary arteries. Patients usually
demonstrates mediastinal widen- need to be moved out of the emer-
ing and pleural effusions, other gency care setting in order for this
signs are less commonly seen and test to be performed.61,63,64
are usually not diagnostic. For Magnetic resonance imaging
these reasons, one or more of (MRI) also demonstrates the
these four newer technologies is presence of a double aortic lu-
most often selected to yield the di- men and the intimal flap. Partic-
agnosis.61,63,64 ularly with the use of cine-MRI,
Aortography has been the this imaging modality has been
mainstay for imaging of aortic dis- shown to be extremely accurate
section, but newer imaging in the diagnosis of aortic dissec-
modalities are being used in many tion. This modality is noninvasive
institutions. Findings include and requires no contrast agent.
demonstration of the false chan- Its major disadvantages include
nel, an intimal flap (the site of in- the inability to be used in patients
timal tear), and distortion of the with certain types of implanted
true lumen. This procedure has materials and the inability to pro-
the advantage of easily demon- vide information about coronary
strating concomitant aortic insuf- artery involvement. The require-
ficiency and demarcates the ex- ments for relative isolation in an
tent of dissection into aortic side area usually some distance away
branches, including the coronary from the emergency room for a
arteries. Thrombosis of the false significant period of time in a pa-
channel may occur, which will tient who may be critically ill lim-
prevent visualization of this chan- its its usefulness. Improved tech-
nel and also eliminate the demon- nology will undoubtedly alleviate
stration of any intimal flap. This these concerns.61,63,64
and other problems may lead to Echocardiography using bi-
misdiagnoses. This procedure re- plane or multiplane probes, par-
quires injecting contrast material ticularly using the transeso-
to which a reaction may occur. En- phageal approach, is highly
tering an aorta that may be intrin- sensitive and specific for aortic dis-
sically fragile with a catheter is a section. This imaging technology
potential risk. It is the most costly may be rapidly performed at the
of the four modalities to be dis- bedside. Minimally invasive, this
cussed and usually requires that technique requires no contrast
the patient be moved from the agent. Excellent demonstration of
emergency department.61,63,64 pericardial effusions, aortic insuf-
CLINICAL PEDIATRICS
CLINICAL PFDIATRICS
7777

ficiency, and proximal coronary
arterial blood flow is possible. This
modality is the current imaging
method of choice in a growing
number of institutions.63,64
Conclusion
Current concepts in the patho-
physiology and predisposing con-
ditions of acute aortic dissection
were presented. This life-threaten-
ing condition requires the
atricpractitioner to make a timely
diagnosis in order to ensure
prompt intervention. To facilitate
early diagnosis, the salient physi-
cal findings of the known predis-
posing conditions are discussed.
The most common predispos-
ing conditions of aortic dissection
in children and young adults are
cardiovascular anomalies, partic-
ularly coarctation of the aorta and
congenital aortic valvular stenosis
caused by bicuspid or unicuspid
aortic valves. Other frequent pre-
disposing conditions include dis-
orders of connective tissue such as
Marfan syndrome, Ehlers-Danlos
syndrome Type IV, and Turner
syndrome. As discussed, all of
these conditions predispose to
weakening of the aortic media.
Other, less common etiologies are
also presented along with features
that are felt to predispose to aor-
tic medial weakening. Weight lift-
ing and cocaine abuse are new
conditions associated with aortic
dissection in adolescents and
young adults.
Presenting signs and symp-
toms of acute aortic dissection
were discussed. Manifestations pri-
marily involve the cardiovascular,
pulmonary, and nervous systems.
Current imaging technologies
useful in confirming the diagnosis
of aortic dissection are presented.
Echocardiography is currently be-
ing chosen by many institutions
78
CLINIC~~~~~~~~~~~l PEDIATRICS
78 CLINICAL PEDIATRICS
Fikar, Koch
the method of choice, but each in-
stitution needs to determine the
best technology in view of individ-
ual needs and capabilities.
Because many of the predis-
posing conditions are inherited,
practitioners need to ensure that
the families of affected children
or young adults are referred for
appropriate genetic counseling as
well as cardiovascular evaluation
as deemed necessary. Although in
a crisis situation the presenting
pedi- child, adolescent, or young adult
will appropriately be the focus of
attention, the pediatrician or
other primary care physician
needs to ensure that a careful
family medical history is ob-
tained. All close relatives should
be encouraged to undergo appro-
priate diagnostic studies for
ble aortic root enlargement since
many familial instances of aortic
dissection have no associated
physical findings. Practitioners
conducting school or college
preparticipation sports evalua-
tions can make use of the infor-
mation presented in the assess-
ment of risk for sudden death
from acute aortic dissection.
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