Early Diagnosis of a Life-threatening Cause of Obstructive Jaundice

Abstract

A 68 years old man who presented to his primary care physician due a 3 weeks history of generalized pruritus and dark urine. Three months prior to this evaluation, his primary care provider noted an isolated elevation of ALT for which she ordered an abdominal CT scan. Study was reported as essentially normal (Fig. 1). His previous medical history included hepatitis C (genotype 2b) s/p interferon alfa 2b - ribavirin therapy in 2002 with SVR, colonic angiodysplasia, obstructive sleep apnea on CPAP, anxiety disorder, schizophrenia and parasitosis: strongyloidiasis/schistosomiasis. His medication profile included buspirone 5mg, mirtazapine 15mg, hydroxyzine 10mg and paroxetine 40mg. On exam was found with jaundice and icteric sclera. Laboratory reports showed ALP 269 U/L, total bilirubin 9.4mg/dL, direct bilirubin 8mg/dl, AST 65IU/L, ALT 68IU/L, albumin 4.5g/dL, INR 1.12, and platelets 170,000/mcL. Echogram of the abdomen demonstrated an intrahepatic biliary dilation of unclear etiology. MRCP demonstrated significant upstream intrahepatic biliary ductal dilatation with enhancing soft tissue at the confluence of the right and left main intrahepatic ducts (Fig. 2). CEA was normal and CA 19-9 was elevated at 189U/mL. ERCP demonstrated high grade stricture with invasion of the right hepatic duct suggestive of klatskin tumor type IIIa (Fig. 3). Successful sphincterectomy, brushing and stent placement performed. Duct brushings were positive for cholangiocarcinoma. Biopsies of the stricture were non-diagnostic. Patient underwent excision of a 1.4 cm lesion extending to proximal and distal margins, currently on adjuvant chemoradiotherapy. A Klatskin tumor is a cholangiocarcinoma involving the biliary hilum at the junction of the right and left main hepatic ducts. Symptoms related to biliary obstruction include jaundice, pruritus, clay-colored stools, and dark urine. Other common symptoms include abdominal pain, weight loss and fever. Liver biochemical tests typically suggest biliary obstruction, with elevations in total bilirubin, direct bilirubin, and alkaline phosphatase. Transaminase levels may initially be normal. If suspected on ultrasound, additional imaging studies (MRI/MRCP, ERCP, EUS) are essential for assisting with diagnosis and for planning management. A tissue diagnosis can be obtained by a variety of means in patients suspected of having cholangiocarcinoma. Surgery provides the only possibility for a cure. Nonetheless, outcomes after resection are poor, particularly with node-positive disease. Postoperative adjuvant therapy is recommended for all patients who have microscopically positive resection margins, as well as for those with node-positive disease. Knowledge of this condition is important, since delay in diagnosis may lead to poor prognosis.Figure: Normal contrast enhanced abdominal CT.Figure: MRCP showing enhancing soft tissue at confluence of the right and left main intrahepatic ducts.Figure: ERCP showing cholangiocarcinoma Klatskin type IIIa.