Klippel-Trenaunay syndrome associated with splenomegaly: report of a case

Abstract

We report herein the case of a 19-year-old woman, diagnosed as having Klippel-Trenaunay syndrome at the age of 3 years, who presented to our hospital with severe abdominal pain. Abdominal computed tomography revealed splenomegaly, ascites, and paracentesis confirming an intraabdominal hemorrhage. Thus, an emergency laparatomy was performed for a suspected splenic rupture, and 2.5 L of blood was drained from the abdominal cavity. Splenomegaly was confirmed, and a splenectomy was performed. The patient's postoperative course was complicated by disseminated intravascular coagulation, but she recovered and was discharged 3 weeks following surgery. Pathological examination of the spleen suggested that the splenomegaly was caused by high venous pressure due to splenic vein stenosis. To our knowledge, this is the first reported case of Klippel-Trenaunay syndrome associated with marked splenomegaly.