Rare Congenital Portal Vein and Splenic Vein Thrombosis with resultant Non-Cirrhotic Portal Hypertension, Hypersplenism and Thrombocytopenia in A Parturient Requiring Cesarean Delivery
Abstract Number: 68
Abstract Type: Case Report/Case Series
Introduction: Non-cirrhotic portal hypertension during pregnancy is a rare event. The pathophysiology involves obstruction to portal venous flow resulting in elevated pressures leading to the development of collateral circulation, variceal bleeding, coagulopathy, ascites, hepatorenal syndrome, hepatic encephalopathy and even splenic artery rupture. Complications can be associated with a mortality rate as high as 18-50%.1 We present, to the best of our knowledge, the first case report on the perioperative anesthetic considerations and management for cesarean delivery in a parturient with non-cirrhotic portal hypertension.
Description: T.W. is a 35 yo G2P1001 at 32 weeks gestational age who presented with hematemesis, worsening thrombocytopenia, and bleeding anemia. A detailed history revealed a childhood diagnosis of portal vein and splenic vein thrombosis with resultant non-cirrhotic portal hypertension requiring multiple esophageal sclerotherapy and banding procedures.
Endoscopy revealed large esophageal varices and stigmata of recent bleeding. Five esophageal bands were placed, octreotide infusion started, and propanolol continued. Despite multiple blood and platelet transfusions, her platelet count and hematocrit continued to decrease to 55,000/mm3&23.7% respectively. The decision was made to proceed with C/S due to the additional presence of significant variable fetal heart rate deceleration and the concern of worsening portal hypertension and variceal bleed. Steroids were administered for fetal lung maturity.
General anesthesia was indicated because of the risk of neuraxial hematoma due to worsening thrombocytopenia. Octreotide infusion was continued, platelet transfusions and antiemetics given prior to transport to the operating room. General anesthesia was induced using rapid sequence induction. Sodium pentothal 400mg and succinylcholine 140mg were given intravenously. She was intubated and anesthesia was maintained with Isoflurane, expiratory concentration 50%, nitrous 50%, oxygen 50%, muscle relaxants and narcotics. An intra-arterial cannula was inserted for monitoring.
A viable infant was delivered with APGAR scores of 5 &6 at 1&5 minutes. The surgery was uneventful, neuromuscular blockade was reversed at the end of the procedure, the patient extubated and monitored in the intensive care unit. She was discharged on post operative day 3 with follow up to the GI clinic.
Discussion:Due to the high mortality rate associated with the potential complications of portal hypertension in the parturient, multidisciplinary management should be implemented early even before conception if possible. Goals include correcting coagulopathy, maintaining hemodynamic stability, perioperative use of beta blocker and octreotide therapy. Prompt decisions should be made regarding the need for endoscopic procedures, as well as the optimal mode of analgesia/anesthesia.
1. Selo-Ojeme et al.J Obstet&Gynecol&Reproductive Biology 2003;124-7