Case Presentation:

A 48 y/o Cambodian woman with history of immigrating to the US thirty years pta and “cryptogenic cirrhosis” was transferred to our facility with abdominal pain, nausea, and anorexia. PE revealed jaundice, tachypnea, scleral icterus, ascites and splenomegaly. Initial laboratory data was significant for total bili 42.5 mg/dL, direct bili 39.8 mg/dL, AST 33 u/L, ALT 32 u/L, alk phos 236 u/L, INR 1.9, alb 2.5 g/dL, Cr 1.3 mg/dL, and Hg 10.4 g/dL. CT abdomen revealed extensive esophageal, peri–splenic and retroperitoneal varices, massive splenomegaly, cirrhotic liver, portal vein thrombosis, and bile duct dilation with multiple stones in the CBD and gallbladder. The patient was felt to be septic and was treated with empiric IV antibiotics and IVF. She stabilized and underwent ERCP with biliary stent placement. CBD brushings demonstrated benign ductal epithelial cells with acute and chronic inflammation, but no malignancy. Hepatic and renal function continued to deteriorate and with MELD score of 41, she underwent liver transplantation. Pathologic examination of the explanted liver demonstrated hepatic schistosomiasis compatible with Schistosoma mekongi. She received treatment with praziquantel, but her remote postoperative course was complicated by pericardial tamponade, anoxic encephalopathy and she expired 1 month later.

Discussion:

Liver transplantation has become an accepted treatment option for patients with ESLD. In developed countries, frequent indications include decompensated cirrhosis and HCC. However, in the developing world, schistosomiasis is responsible for significant liver disease in > 200 million people due to the following: adult worms reside in intestinal venous system, lay hundreds of eggs/day which drain to liver via portal circulation, become trapped in hepatic microcirculation inducing inflammation, fibrosis, and ultimately portal hypertension (PH) which classically is termed noncirrhotic portal hypertension (NCPH). Among the entities that cause NCPH are nodular regenerative hyperplasia, hepatoportal sclerosis, congenital hepatic fibrosis, schistosomiasis and various hypercoagulable states. Patients usually have an asymptomatic course but eventually manifest consequences of PH that include splenomegaly, variceal bleeding, ascites, and rarely liver failure. Unfortunately, in the US this entity is not often considered during evaluation for PH and transplantation as in our patient. We will review transmission, life cycle, epidemiology, pathophysiology, clinical presentation, radiologic and pathologic findings of S. mekongi infection. In addition, we will present rarely reported cases of schistosomiasis associated with solid organ transplantation and results of treatment.

Conclusions:

Schistosomiasis is a rare cause of PH and ESLD in the US and is often not considered in the diagnostic evaluation. We present a case of a woman found to have S. mekongi hepatic infection with classic pipestem fibrosis in the explanted liver and review of the current literature.