Aortic dissection is a rare entity in childhood and a high index of
suspicion is required for prompt management. It should be considered in
children with Marfan syndrome complaining of chest pain.
A 15 years male was hospitalized with a month long
history of recurring episodes of severe left sided chest pain, five days
history of cough and fever. The pain was piercing in nature, radiating to
the neck, left shoulder and back, associated with profuse sweating and
occasionally with vomiting. His father had expired two months ago after a
similar brief illness at the age of 38 years.
Examination revealed an asthenic built child with
height of 162 cm, arm span 174 cm, arm span-to-height ratio 1.074 and
US:LS ratio 0.82. He had high arched palate, arachnodactly, positive wrist
and thumb signs. There were signs of consolidation over left base.
Cardiovascular system examination revealed a short diastolic murmur in
aortic space. Skiagram revealed a massive consolidation of left lower
zone. Clinical and radiological features of consolidation resolved
following antibiotic therapy. However repeat radiological evaluation
revealed an underlying mediastinal widening. High resolution computerized
tomography revealed a large aortic aneurysm (Fig. 1). Color
Doppler revealed aortic dilatation just after the origin of left
subclavian artery and a large intimal flap just before the origin of
superior mesenteric artery.
![](images/corr-2.jpg) |
Fig. 1 High resolution CT revealing a
large aortic aneurysm arising from aortic knuckle and extending till
the celiac axis with a large posterolateral dissecting part in the
left paraspinal gutter. |
The catastrophic cardiovascular events in Marfan
syndrome, most often a result of aortic aneurysm and dissection, result in
an average age expectancy of 32 years [1]. Several studies have validated
the role of beta-blockers in retarding aortic root dilatation and
preventing aortic dissection/rupture [2]. Therapy with calcium channel
blockers, angiotensin inhibitors and angiotensin II receptor antagonists
have also shown similar effects [3]. Successful surgical intervention has
revolutionized the management of these patients, resulting in median
survival of 61 years [4].
The patient showed symptomatic improvement following
beta blockers and was referred for surgical correction, which was however
denied on grounds of extremely high operative risk. The patient is
surviving after two years of follow up.
References
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McKusick VA. Life expectancy and causes of death in Marfan syndrome .N
Engl J Med. 1979;300:772-7.
2. Ladouceur M, Fermanian C, Lupoglazoff JM, Edouard T,
Dulac Y, Acar P, et al. Effect of beta blockade on ascending aortic
dilatation in children with Marfan syndrome. Am J Cardiol. 2007;99:406-9.
3. Yetman AT. Cardiovascular pharmacotherapy in
patients with Marfan syndrome. Am J Cardiovasc Drugs. 2007;7:117-26.
4. Finkbohmer R, Johnston D, Crawford ES, Coselli J,
Milewicz DM. Marfan syndrome: long-term survival and complications after
aortic aneurysm repair. Circulation. 1995;91:922-39.
5. Yazici M, Soydinc S, Davutoglu V, Akdemir I, Dinckal
MH. Large ascending aneurysm and severe aortic regurgitation in a
7-year-old child with Marfan syndrome. Int J Cardiovasc Imaging.
2004;20:263-7.