Stasis Hepaticus Sicklemia

Abstract

Intrahepatic Cholestasis(IHC) is a rare complication of sickle cell disease(SCD). While diagnosis is challenging given the overlap of IHC with other conditions of SCD – hepatitis, cholecystitis, hepatic crisis, early recognition of this entity is essential for avoiding fatal outcome. Case Report: A 41 year old black man was brought to ED for blurred vision, productive cough and right upper quadrant (RUQ)abdominal pain. Past medical history: SCD, hypertension, end-stage renal disease on hemodialysis.Vital signs: BP:90/50, HR:98, T:99 F, SpO2:95% RA and physical exam was remarkable for icteric sclera and skin, RUQ tenderness, hepatomegaly. He was treated with IV hydration and narcotics for acute sickling crisis. On the 2nd hospital day he remained hypotensive, became lethargic & febrile with worsening abdominal pain. Labs:leukocytosis, hyperbilirubinemia(serial bilirubin T/D L14.7/13.8, 23/21, 39/39) and a cholestatic pattern on liver panel, coagulopathy, and elevated BUN/Cr. Serologic tests for viral hepatitis and HIV: negative and abdominal ultrasound was unremarkable except for a single gall stone. CT scan showed normal common bile duct.Subsequently, he developed worsening coagulopathy with bleeding from his oral cavity, increasing abdominal pain and worsening mental status(ammonia: 59).These findings led us to the clinical diagnosis of IHC. He was transferred to a tertiary care center for exchange transfusion. However, he underwent exploratory laparotomy for suspected abdominal compartment syndrome. But no evidence of bowel ischemia was noted on fluorescein injection.Despite maximal supportive care, his condition continued to deteriorate and life support was discontinued as requested by his family. Discussion: IHC is due to widespread sickling within the hepatic sinusoids leading to ischemia & ballooning of hepatocytes- intracanalicular cholestasis. Estimated mortality:37%. It is characterised by striking jaundice, renal impairment, coagulopathy, and encephalopathy with RUQ pain, fever, tender hepatomegaly and leukocytosis. Surgery in IHC has been reported to be assosciated with a poor outcome and all survivors have been treated with exchange transfusion and intensive supportive care. IHC must be considered in the differential diagnosis of SCD patients who present with very severe hepatic crisis and go on to develop the characteristic features mentioned above. Vigorous exchange transfusion and correction of coagulopathy appears to be the best therapeutic approach in these cases.