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Pregnancy in the marfan syndrome a life threatening combination?


, : Pregnancy in the marfan syndrome a life threatening combination?. Tidsskrift for den Norske Laegeforening 107(10-11): 939-940, 964

The Marfan syndrome is a heritable disorder of connective tissue with clinical manifestations in the skeletal, occular and cardiovascular systems. The condition is associated with increased length of the limbs, thorax deformity, subluxation of the lenses, and aortic root dilatation. A 22 year old woman with typical manifestations of the Marfan syndrome is presented. Her pregnancy was twice complicated by mechanical small bowel obstruction due to a diaphragmatic hernia which recurred after surgical repair. The combination of the Marfan syndrome with pregnancy represents a possible life-threatening condition, and the risk increases with the degree of pre-existing cardiovascular disease.

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