title: Clinical presentation of extrahepatic portal vein obstruction: 10-year experience at a tertiary care hospital in Pakistan.
abstract: OBJECTIVE
  To evaluate the clinical presentation, possible etiological factors, management and outcome of patients in our hospital with extrahepatic portal vein obstruction (EHPVO).
  
  
  METHODS
  This study included patients with EHPVO followed up in our department during last 10 years. Patients of cirrhosis with EHPVO were excluded. Patients' clinical presentation, etiology of EHPVO, management and outcome results were analyzed.
  
  
  RESULTS
  Of 30 patients, 19 (67.9%) were males. Median age was 12 years. Of 14 patients who underwent liver biopsy 9 had histological activity index stage of 1/6. History of omphalitis and pulmonary tuberculosis was present in one case each. Of 22 patients with the available thrombophilia profile, nine patients had a deficiency of protein C, five patients had a deficiency of protein S, one each had reduced level S of anti-thrombin III and factor V mutation. The predominant presenting symptom was hematemesis (15 patients, 53.6%). Seven patients (25%) had splenomegaly. Three patients (10.7%) had no esophageal varices on endoscopy. Three patients underwent splenectomy due to severe pancytopenia. Endoscopic retrograde cholangipancreatography was performed in four patients (14.3%) due to portal biliopathy. Common bile duct stenting was performed in all four patients. Of them, one patient underwent splenorenal shunt operation for indication of hemobilia. One patient died at the age of 40 years, due to cholangitis and sepsis.
  
  
  CONCLUSIONS
  Results from this study show that the anticoagulant deficiency is a common cause of EHPVO in our setup. Hematemesis is a common presenting symptom. Some of these patients have symptomatic portal biliopathy.
authors.affiliation: Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
authors.forename: Farina M
authors.initials: FM
authors.lastname: Hanif
authors.affiliation: Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
authors.forename: Ghous Bux
authors.initials: GB
authors.lastname: Soomro
authors.affiliation: Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
authors.forename: Sara Nazir
authors.initials: SN
authors.lastname: Akhund
authors.affiliation: Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
authors.forename: Nasir Hassan
authors.initials: NH
authors.lastname: Luck
authors.affiliation: Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
authors.forename: Syed Mudassir
authors.initials: SM
authors.lastname: Laeeq
authors.affiliation: Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
authors.forename: Zaigham
authors.initials: Z
authors.lastname: Abbas
authors.affiliation: Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
authors.forename: Syed Mujahid
authors.initials: SM
authors.lastname: Hassan
authors.affiliation: Department of Pathology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
authors.forename: Muhammed
authors.initials: M
authors.lastname: Mubarak
keywords: Extra hepatic portal vein obstruction; portal biliopathy; variceal bleed