Jaundice and malaise in a middle aged man

doi:10.1136/bmj.a3175

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Helen J Fellows, specialist registrar in intensive care medicine1,
Harry R Dalton, consultant gastroenterologist and honorary senior lecturer 2

¹Intensive Care Unit, Royal Devon and Exeter Hospital, Exeter EX2 5DW
²Royal Cornwall Hospital and Peninsular College of Medicine and Dentistry, Truro TR1 3LJ

Correspondence to: H R Dalton harry.dalton{at}rcht.cornwall.nhs.uk

A 52 year old man was referred to the jaundice hotline clinic by his general practitioner with symptoms of malaise and lethargy over one year and a seven day history of jaundice. Apart from a laparoscopic cholecystectomy eight years earlier, he had no other relevant medical history and took no drugs. On examination, he was jaundiced but had no other abnormal physical signs.

His laboratory findings were: bilirubin 135 µmol/l (normal range 3-17), alkaline phosphatase 971 U/l (3-110), alanine aminotransferase 154 U/l (3-35), albumin 35 g/l (35-45).

Ultrasound of the liver showed coarse echotexture and no intrahepatic biliary duct dilatation. Views of the common bile duct were poor owing to overlying bowel gas.

The patient went on to have endoscopic retrograde cholangiopancreatography as an outpatient (fig 1⇓).

Fig 1 Results of endoscopic retrograde cholangiopancreatography

Questions

1 What is the diagnosis?
2 What further investigations should he have?
3 What treatment options are available?

Answers

Short answers

1 Primary sclerosing cholangitis with an extrahepatic (common hepatic duct) dominant stricture.
2 Cross sectional imaging, biliary cytology, and tumour markers.
3 Balloon dilation and stenting have shown mixed results, and medical treatment has been disappointing. Orthotopic liver transplantation is the only intervention that has been shown to improve outcome in this condition.

Long answers

1 Diagnosis

Endoscopic retrograde cholangiopancreatography showed beading and ectasia (fig 2⇓; small arrows) as a result of multifocal stricturing and dilatation of …