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Q1: What is the most likely cause of her jaundice and why was this suspected clinically?
Bile duct obstruction by a pancreatic tumour is suggested from the history of painless progressive jaundice associated with weight loss. Furthermore in the presence of jaundice and a palpable gallbladder, Courvoisier's law* states that the diagnosis is unlikely to be due to cholelithiasis because previous cholecystitis would have caused fibrosis and shrinkage of the gallbladder (the only exception being the presence of simultaneous stones in both the common bile duct and cystic duct). A tumour of the pancreatic head was confirmed on abdominal computed tomography.
Q2: What complication of PTC has occurred?
The thoracic radiograph (see p 787) showed the presence of fluid in the right pleural cavity. The pleural aspirate was bile stained suggesting an iatrogenic biliary pleural fistula; this is a rare complication of PTC. It results from the inadvertent passage of the cholangiography needle through the costodiaphragmatic recess of the pleural cavity and into the liver thus creating an abnormal communication between the two. The pleural cavity provides a low pressure, low impedance reservoir for the drainage of bile from the high pressure obstructed biliary tree.
Q3: What is the treatment of this complication?
In the absence of distal biliary obstruction, spontaneous closure of the fistula may be achieved with several weeks of percutaneous biliary drainage.1 Octreotide, a somatostatin analogue that suppresses gastrointestinal secretions by inhibiting the release of pancreatic and gastrointestinal hormones, may be given as an adjunct to reduce biliary output.2 However, early active intervention has been suggested for this complication if the biliary tree remains obstructed3 and may involve:
Surgery with the aim of relieving the bile duct obstruction. This may require a biliary enteric anastomosis, excision of the fistulous tract, repair of the diaphragmatic defect, and drainage of the subphrenic space.4 In a series of 15 patients treated surgically, all had resolution of symptoms with no postoperative mortality.4
Combined endoscopic percutaneous techniques to decompress the common bile duct and the re-establishment of normal bile flow either by biliary stent placement,5 sphincterotomy,6 or balloon dilatation7 thus allowing the biliary pleural fistula to heal spontaneously. All reported cases have been successfully treated by these techniques,5-7 but due to the rarity of the condition, there has been no large series comparing outcome with surgery.
Management should also include parental nutrition, correction of electrolyte abnormalities with particular attention to possible hyponatraemia due to the loss of the sodium in the bile, chest physiotherapy, and treatment of sepsis with intravenous antibiotics.
PTC provides direct access to the proximal biliary tree when ERCP has failed. In this case, PTC was performed to relieve biliary pressure resulting from an obstructed stent. In experienced hands PTC is relatively safe since dilated ducts are easily punctured percutaneously, enabling excellent visualisation of the upper biliary tree. However, complications occur in up to 69% of patients, with cholangitis occurring in 47%, haemobilia in 7%, and biliary pleural fistula in 2.5%.8
A biliary pleural fistula may be a congenital abnormality or it may be secondary to thoracoabdominal trauma, suppurative complications of biliary tract obstruction, or parasitic liver infections.1 Diagnosis is confirmed either by aspiration of bile from the pleural space or cholangiography demonstrating the fistulous tract. Biliary pleural fistulas are usually associated with a high morbidity and mortality because of the risk of biliary mediastinitis and sepsis, from which this patient subsequently succumbed. Early diagnosis with prompt correction of the biliary obstruction is therefore required for the management of this rare complication.
Biliary pleural fistula.