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Answers on p 412.
A 44 year old man was admitted with a history of intermittent jaundice and rigors. Three months previously he had been admitted to another hospital with similar symptoms and half a stone (3.2 kg) weight loss. At this time his problems were thought to be secondary to choledocholithiasis. At operation, gallstones were seen in the common bile duct with an associated lower common bile duct stricture. He underwent cholecystectomy and choledochoduodenostomy. The symptoms initially resolved postoperatively but subsequently recurred on an intermittent basis. He was transferred to our hospital for further management of his problems.
He had a 28 year history of ulcerative colitis, based on barium enema, which had remained quiescent for many years on salazopyrin maintenance treatment. His past medical history included segmental bronchiectasis secondary to childhood whooping cough. He was a life long non-smoker and did not drink alcohol.
He was jaundiced but apart from a right Dupytren's contracture and his cholecystectomy scar, clinical examination was normal.
His liver function tests were abnormal. Serum alkaline phosphatase was 1020 IU/l, aspartate transaminase 128 IU/l, total bilirubin 113 μmol/l. Serum albumin was 33 g/l and the international normalised ratio mildly raised at 1.3. His full blood count and urea and electrolytes were normal. He was started on antibiotics for possible ascending cholangitis. An abdominal ultrasound confirmed gas in the biliary tree consistent with his previous surgery, but there was no evidence of duct dilatation or recurrent stones in the common bile duct. Endoscopic retrograde cholangiopancreatography was attempted. The common bile duct and common hepatic duct were of small calibre but the intrahepatic ducts were not seen, most of the contrast emptying via the choledochoduodenostomy. A percutaneous transhepatic cholangiogram (PTC) was performed (fig 1).
His symptoms settled on cephadrine and he was discharged. He was followed up in the outpatient clinic and remained reasonably well apart from suffering episodic attacks of jaundice and rigors, which responded quickly to courses of antibiotics. Twelve months after admission his albumin and bilirubin were normal and his alkaline phosphatase and aspartate transaminase were stable at 561 IU/l and 151 IU/l respectively. In view of his longstanding pancolitis surveillance colonoscopy was offered but at this time he declined any further investigation of the colon.
The pattern of intermittent jaundice and rigors continued, although between these episodes he remained well. Five years after his initial presentation he agreed to colonoscopic surveillance. Four pedunculated polyps, each between 1 and 2 cm in size, were removed from the mid-ascending and descending colon. Histological review of these specimens revealed low grade dysplasia. His liver function over this period had deteriorated (table 1).
The prothrombin time was prolonged at 17.3 seconds. Full blood count, urea, electrolytes, and glucose were normal. Repeat abdominal ultrasound showed a complex cystic lesion in the left lobe of the liver, freeintraperitoneal fluid, and marked splenic enlargement. Computed tomography confirmed the cystic abnormality in the left lobe to be in continuity with the left main hepatic duct. The appearance of the liver suggested macronodular cirrhosis with associated portal hypertension. Hydatid serology was negative and serum tumour markers (α-fetoprotein, CA 19–9) were normal. His liver function tests had worsened despite there being no clinical evidence of ongoing sepsis (table 1).
- What does the PTC (fig 1) show and given the history of ulcerative colitis what is the most likely diagnosis?
- What is the connection between this hepatological diagnosis and the findings of dysplasia at colonoscopy?
- Given the deteriorating liver function tests, computed tomography findings, and colonoscopic changes how would you manage this case?
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