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Background: Rarely occurring in the pediatric and adolescent population, aortic dissection is a condition with many predisposing factors. Previous studies have suggested that congenital cardiovascular disorders are the most common predisposing conditions. Trauma-associated aortic dissection is considered a rare occurrence., Methods: The Statewide Planning and Research Cooperative System database of New York State was used to retrieve cases of aortic dissection in persons < or = 21 years old over a 10-year period. A retrospective analysis for risk or associated conditions was undertaken., Results: Forty-five of a total of 12,142 cases of aortic dissection (0.37%) occurred in persons < or = 21 years old. No patient was younger than 15 years of age. Six of the 45 died (13%). Most patients were male (37 of 45, or 82%). Contrary to previous reports, the most common associated condition was trauma (19 of 45, or 42%), with Marfan syndrome the second most common (11 of 45, or 24%). Ten of 45 adolescent and young adult patients (22%) had no apparent risk factors., Conclusions: Traumatic aortic disruptions must be considered in children and adolescents who survive serious chest trauma. Family members of pediatric and young adult patients with trauma-associated aortic dissection may need to be evaluated for possible risk of aortic dissection., (2009 Wiley Periodicals, Inc.)

Acute aortic dissection in childhood and adolescence is a rare but life-threatening condition with many known risk factors, most of which are heritable. The importance of determining the presence of any predisposing disorders is stressed, since all close relatives can then be screened for any such inherited trait that would make them at risk for a similar catastrophic event. Even with no obvious predisposing disorder, it may still be prudent to evaluate close kin for the presence of aortic root dilatation, since some genetic disorders have no manifestation other than acute aortic dissection secondary to aortic medial pathology. Close monitoring for at-risk family members, including the index patient, may need to be a life-long process.

Summary: Current concepts in the pathophysiology and predisposing conditions of acute aortic dissection 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 diagnosis, 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 [ABSTRACT FROM AUTHOR]

A 15-year-old adolescent male with dissecting aortic aneurysm is presented. His young age, lack of predisposing factors, and fulminant course with rapid progression to death precluded a correct antemortem diagnosis. Review of the literature reveals that most instances of dissecting aortic aneurysm in childhood and adolescence are associated with predisposing conditions, especially congenital cardiovascular anomalies. The clinical picture is generally characteristic. Prompt evaluation and therapy may be lifesaving. [ABSTRACT FROM AUTHOR]