Splenectomy and proximal lieno-renal shunt in a factor five deficient patient with extra-hepatic portal vein obstruction

A 16-year old boy had gastro-esophageal variceal bleeding, splenomegaly and hypersplenism. During preoperative workup prolonged prothrombin time and activated partial thromboplastin time were detected, which on further evaluation turned out to be due to factor V deficiency. Proximal lieno-renal shunt and splenectomy were successfully performed with transfusion of fresh frozen plasma during and after the surgical procedure. At surgery there was no excessive bleeding. The perioperative course was uneventful and the patient is doing well on follow up.Surgical portal decompressive procedures can be safely undertaken in clotting factor deficient patients with portal hypertension if meticulous surgical hemostasis is achieved at operation and the deficient factor is adequately replaced in the perioperative period.Congenital deficiency of factor V (parahemophilia) is a rare inherited disorder of coagulation which appears to follow an autosomal recessive or partially dominant pattern [1-4]. Although the clinical features are rather mild compared with hemophilia, any surgical procedure undertaken without replacement therapy is uniformly complicated by excessive hemorrhage [1,2,5]. As factor V concentrate is not available, fresh frozen plasma (FFP) is the only available source of factor V at the present time. We report a boy with extra-hepatic portal venous obstruction (EHPVO), portal hypertension and upper gastrointestinal (UGI) hemorrhage who also had factor V deficiency. Although many congenital anomalies such as cleft palate, mental retardation, short stature, syndactyly, atrial septal defect, ventricular septal defect, coarctation of aorta, duplication of the genitourinary collecting system have been associated with congenital deficiency of factor V [1-4], the simultaneous occurrence of factor V deficiency with EHPVO has not been described till date. EHPVO is a relatively common cause of portal hypertension in India, especially in the younger age group [6,7]. Many causes,