Case Report: Life-Threatening Post-operative Hemorrhage in Klippel-Trenaunay Syndrome Associated With Hypofibrinogenemia

Abstract

Klippel-Trenaunay Syndrome (KTS) is a rare congenital disorder, characterized by venous and lymphatic malformations of the skin, soft tissue, and bone, causing limb hypertrophy. Although, a ruptured hemorrhagic corpus luteum is a rare condition in women of reproductive age, it can result in lethal outcomes. Here, we have described a patient with KTS and hypofibrinogenemia who went through recurrent lethal postoperative bleeding due to a ruptured hemorrhagic corpus luteum. This case suggested that conservative therapy might be the first choice and effective therapy for the patients with KTS, who suffered from bleeding complications of surgical therapy.

Keywords: conservative therapy; fibrinogen concentrate; hypofibrinogenemia; klipple-trenaunay syndrome; post-operative hemorrhage; prophylactic transfusion of fibrinogen.

Figure 1

(A,B) Patient with Klippel–Trenaunay syndrome. (A) The left leg was larger in circumference and longer in length than the right leg. (B) Capillary malformation in the left leg. (C,D) Unenhanced computer tomography (CT) showed peritoneal diffuse effusion in pelvis with different mixed attenuation values at the admission to our department (C) and post-operative period (D).