A 37-year-old woman presented to the University Hospital Gastroenterology Clinic with intermittent abdominal bloating and discomfort for almost 20 months. Discomfort and bloating were not related to meals, defecation or change in position. Symptoms arose spontaneously and lasted several hours at a time. She recalled at least 1–2 episodes per month from the time symptoms started. She had not had any diarrhea, nausea, vomiting, fever or chills since the onset of symptoms. She recalled having elevated liver enzymes during testing at a health fair a year prior to presentation which showed aspartate transaminase (AST) 31 IU/l (range 10–30 IU/l), alanine transaminase (ALT) 40 IU/l (range 6–40 IU/l), alkaline phosphatase (ALP) 128 IU/l (range 33–115 IU/l) and total bilirubin 0.6 mg/dl. She went to her primary care doctor 3 months prior to presentation and was noted to have elevated liver enzymes and bilirubin (total bilirubin 3.2 mg/dl, direct bilirubin 1.9 mg/dl, AST 134 IU/l, ALT 387 IU/l, ALP 344 IU/l).
Ultrasound was done followed by a CT scan. Ultrasound showed a 2.9 × 2.4 × 2.9 cm hypoechoic mass with calcification in the left hepatic lobe with areas of bile duct dilation greatest in the left hepatic lobe. CT scan (fig. ) showed similar findings with a complex cyst in the inferior aspect of the left lobe of the liver with mild dilation of bile ducts of the left hepatic lobe. She was referred to a gastroenterology clinic 6 weeks prior to presentation where repeat testing showed that the liver enzymes and total bilirubin had normalized (total bilirubin 0.7 mg/dl, AST 18 IU/l, ALT 11 IU/l, ALP 71 IU/l). Magnetic resonance cholangiopancreatography (MRCP) was ordered and showed 2 × 2.5 cm cystic left hepatic lobe lesion with two thin internal septations which showed faint enhancement, with possible communication with the intrahepatic bile ducts. There was diffuse mild intrahepatic biliary dilation at the central left and right intrahepatic ducts. No extrahepatic bile duct dilation was noted. At the proximal common hepatic duct, a waist-like narrowing of the duct (focal absence of intraductal biliary signal) was noted. The possibility of a biliary cystadenoma versus Caroli disease versus hydatid cyst was raised. So, she was referred to the University Hospital Gastroenterology Clinic.
CT scan showing biliary cystadenoma. The cystic lesion (black arrow) in segment 4 of the liver with a central focus of high density is seen representing the biliary cystadenoma. A bile duct stent placed during ERCP can also be seen (red arrow).
The patient had no history of intravenous drug use or tattoos or body piercing, no history of excessive alcohol use or obesity, no history of unprotected sex or sex with multiple sexual partners and no history of working with toxic chemicals. She had no prior history of surgery or medical illness. She had no known allergies. She was not using any medications. There was no significant family history of biliary or liver disease.
Her blood pressure was 126/78 mm Hg, heart rate 74/min, respiratory rate 14/min, temperature 98.6° Fahrenheit and oxygen saturation 99% while breathing ambient air. Physical exam demonstrated mild tenderness in the left upper quadrant on deep palpation without any palpable mass, hernia or scars. The rest of the physical exam was within normal limits. Repeat labs done at the clinic at presentation showed total bilirubin 0.3 mg/dl, AST 16 IU/l, ALT 11 IU/l, ALP 37 IU/l, hemoglobin 13.1 g/dl, white cell count 7,100/mm3 (neutrophils 60.8%, lymphocytes 31.4%, monocytes 6.3%, eosinophils 1.2%, basophils 0.3%), INR 1.0, albumin 4.5 g/dl, CA19-9 10 U/ml (normal <37 U/ml). She was instructed to present for follow-up in 3 months and obtain repeat labs if she developed any intercurrent symptoms.
Two months after clinic visit, repeat lab studies showed elevated total bilirubin and liver enzymes (total bilirubin 4.5 mg/dl, direct bilirubin 2 mg/dl, AST 146 IU/l, ALT 361 IU/l, ALP 166 IU/l). This was accompanied by intermittent episodes of itching that started in the abdominal area and back and spread to the wrists, hands and feet. She was treated with a 14-day course of ciprofloxacin (500 mg twice daily) suspecting cholangitis along with ursodiol (500 mg twice daily) with relief of her itching and jaundice. Three months after clinic visit, lab studies showed normal bilirubin and liver enzyme levels (total bilirubin 0.4 mg/dl, AST 18 IU/l, ALT 15 IU/l, ALP 57 IU/l). Six months after clinic visit, lab studies showed repeat elevation of liver enzymes and total bilirubin (total bilirubin 4.0 mg/dl, AST 152 IU/l, ALT 280 IU/l, ALP 180 IU/l) which normalized on testing 2 weeks later. She received a repeat dose of ciprofloxacin 500 mg p.o. twice daily for 14 days and ursodiol 500 mg p.o. twice daily. She had no relief of her pruritus although her jaundice resolved.
Liver function test fluctuations continued for 15–18 months from initial presentation but were not accompanied by other lab abnormalities. Cholestyramine did not help relieve the pruritus either, so endoscopic retrograde cholangiopancreatography (ERCP) was planned. This was done 18 months from her initial presentation to the clinic. She did not have elevation of CA19-9 when checked periodically during her clinical course. ERCP showed dilation of the right and left hepatic ducts and a 3–4 cm oblong filling defect in the common hepatic duct extending into the left hepatic duct (fig. ). The defect did not appear to fill up on ERCP. Biopsy and brushings of the lesion were done. Balloon sweep during ERCP demonstrated slight movement of the filling defect. A biliary sphincterotomy was done during the procedure with removal of sludge and debris. A 10 Fr 5 cm plastic double pigtail stent was placed into the left hepatic duct across the filling defect during ERCP. Cytological analysis of brushings obtained from the lesion showed no evidence of malignancy. Pathological analysis of the biopsies showed normal bile duct mucosa. ERCP was complicated by pancreatitis necessitating hospital admission for 3 days from which she recovered uneventfully.
Appearance of biliary cystadenoma during ERCP. A filling defect extending from the common hepatic duct to the left hepatic duct is seen (arrow).
Three weeks after ERCP an exploratory laparotomy was done with extrahepatic bile duct resection, left hepatic lobectomy and reconstruction of the right hepatic duct with a Roux-en-Y hepaticojejunostomy with cholecystectomy. Pathology showed cystic dilation of the common hepatic and left hepatic duct with no evidence of malignancy. It also showed chronic inflammation of the extrahepatic bile ducts and focal pericholangitis in the liver. The gallbladder was normal on the excised specimen. Histological exam showed mucinous lining of the cystic lesion with ovarian-like stroma suggestive of biliary cystadenoma with no evidence of malignancy (fig. ).
Fig. 3 Histology of biliary cystadenoma: high power view (20×) showing mucinous lining of the cyst (black arrows) along with the ovarian like stroma (red arrows). Epithelial lining is bland with basal nuclei and abundant mucin-filled cytoplasm with no (more ...)
The patient was seen 2 weeks after surgery in the clinic. She was able to eat normally and had minimal pain resulting from surgery. She was scheduled for a repeat MRCP in 1 year's time.