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April 1996

Aortic Dissection in a 5-Year-Old Girl With Marfan's Syndrome

Author Affiliations

Children's Hospital of Wisconsin 9000 W Wisconsin Ave, Suite 721 Milwaukee, WI 53226

Arch Pediatr Adolesc Med. 1996;150(4):440-442. doi:10.1001/archpedi.1996.02170290106020

Marfan's syndrome is an autosomal dominant, heritable disease with variable connective tissue manifestations. The abnormal elastic tissue predisposes the aorta to cystic medial degeneration, which can result in dissection, rupture, and aneurysm formation. This complication usually presents between 30 and 50 years of age.1 We present a case of aortic dissection in a 5-year-old girl with Marfan's syndrome and discuss the value of noninvasive imaging.

Report of a Patient. A five-year-old girl with Marfan's syndrome presented to the emergency department complaining of intermittent, mild lower-back pain for 2 weeks, which had become severe and incapacitating a few hours prior to admission. On initial examination, the patient was normotensive (blood pressure, 115/80 mm Hg), with a heart rate of 85 beats per minute, a grade II/VI systolic murmur, and peripheral pulses of 1+ throughout.

Her medical history indicated that she was not severely affected, and the suggestion of Marfan's syndrome