Marfan syndrome with acute abdomen: a case report

Marfan syndrome is an autosomal dominant connective tissue disorder characterized by a combination of clinical manifestations in different organ systems. Patients with Marfan syndrome (MFS) whose lifetimes are extended may be encountered as acute abdomen (appendicitis) cases apart from the obligatory reasons and emergencies arising naturally out of their disease, as in the case reported.

In a 28-year-old Turkish male, arachnodactyly, pectus excavatum, kyphoscoliosis and, according to pulmonary roentgenogram, a density increase in the left apical field were detected. In addition, according to the echocardiographic examination, Ebstein's anomaly, mitral valve prolapse, pulmonary hypertension, and inferior deficiency of mitral, aorta, and tricuspid valves were present. The patient was planned to be operated on with the prediagnosis of acute abdomen.

Taking into consideration the pathologies that may accompany MFS and the probable future complications, the patients must be closely monitored during anesthesia applications and required measures should be taken beforehand.