Title Have I got bile for you Authors

Title: Have I got bile for you?

Authors: Martin Swali & Ian Harris

Presenting author email address: m. a. singh. swali@gmail. com

Clinical Information: 58 M Severe sepsis Required ventilator support in ITU Blood culture – E. Coli Abnormal LFTs (for discussion) Past medical history - T 3 N 0 M 0 mucinoadenocarcinoma of the caecum with an open right hemi-colectomy (Dec 2013). - Post op anastomotic leak & deep pelvic abscess (Jan 2014). - Infero-lateral MI (Jan 2012) EJF 56% - Gallstones has been incidentally identified on CT scan done for the above.

Radiology ITU portable USS: ‘gallbladder wall oedema but nothing to suggest cholecystitis. Gallbladder shows stones. Normal calibre biliary tree’’. (Patient not fit enough for cross-sectional imaging at this point) Day 4 CT Abdomen: ‘Small amount of free fluid is noted around the liver, in the para-colic gutters bilaterally and pelvis. The gallbladder appears unremarkable. Biliary tree normal calibre’.

Clinical Progress Stormy initial week Patient then progressed well Came off ventilation Had suffered acute kidney injury requiring hemofiltration.

Deranged Liver function tests as follows: q Liver function test’s demonstrate unconvincing obstructive picture which spontaneously resolved. LFTs compatible with general condition of the patient with liver failure. q LFTs resolve mirroring the patient clinically improving in ITU. Date T Bili Alk Phos GGT T Prot Alb ALT 12/09/14 9 98 39 67 41 21 25/09/14 96 Admitted 247 116 41 25 84 25/09/14 103 242 118 44 26 98 26/09/14 108 193 105 47 24 113 27/09/14 129 164 79 43 22 508 28/09/14 104 153 55 39 19 373 29/09/14 103 138 55 41 21 317 29/09/14 93 137 55 42 21 293 30/09/14 96 144 61 41 23 231 * Underlined figures denote abnormality according to our local lab ranges. Red boxes denote rising trend. Green boxes falling trend

Further clinical progress Patient continues to improve but develops progressive abdominal distension. CT abdomen:

Ascitic fluid analysis Looked like bile. Proven biochemically. (Ascites: serum ratio of bilirubin > 5)

Postulated source of bile leak 1) Partially concealed perforated duodenal ulcer OR 2) Biliary tree (iatrogenic or otherwise) CT with oral contrast – no leak

Figures: Top Row (Left) axial MRCP demonstrates possible cap upon the gallbladder and ascites (Middle and right) mildly dilated proximal CBD with impacted stone and deflated CBD distally. Bottom Row ERCP demonstrates an obstructive stone within the CBD, a contrast filling biliary system and contrast extravasation from the hepatobiliary system.

Timeline of key investigations… Day 1 ventilated on Critical care USS Abdomen: ‘The gallbladder shows a degree of oedema consistent with the patient's general condition but nothing to suggest cholecystitis. There is some debris within the gallbladder. Normal calibre biliary tree. ’ Day 4 with little improvement CT Abdomen/Pelvis: ‘Small amount of free fluid is noted around the liver, in the para colic gutters bilaterally and pelvis’. ‘The gallbladder appears unremarkable’ [The Patient was making very good progress sufficient to warrant weaning off ventilator support. However, A subsequent CT Abdomen demonstrated increasing volume of ascites and a decision to drain the ascites was taken. ] *An ascitic drain placed on the right side of the abdomen drained dark brown/green fluid raising the suspicion of iatrogenic perforation of the gallbladder/biliary tree. * Day 15 Ascitic tap biochemistry: ‘serum bili 46: ascitic bili 463 (1 -21 normal)’ ‘Ratio ~ 10 (> 5 = bile leak)’ Day 19 CT Abdomen/Pelvis with oral contrast: ‘the appearances of the duodenum radiologically was unremarkable ’ ( show pic) Day 21 am MRCP: ‘a small cap like appearance on gallbladder. A Distal CBD stone was found ’ [Contention as to whether this was a point of rupture or a phrygian cap] Day 21 pm ERCP: ‘contrast leakage from the hepatobiliary system but No obvious point source of rupture. ’ [a stent was inserted]

Final diagnosis: Spontaneous biliary system rupture secondary to an impacted CBD stone causing bilious ascites on the background of an E. Coli sepsis.

Discussion: This was a very rare presentation of a very common problem Bile duct leak is common (secondary to cholecystectomy, cystic duct stump blow out, biliary tree trauma, or gb rupture from cholecystitis). Much rarer is a leak from an obstructive biliary system. Clearly in this case though the gallbladder wall was normal, because of a closed off obstructed biliary system, there was eventually a point of weakness in the gallbladder fundus. This by analogy occurs more commonly in urinary tract obstruction. The Axial MRCP showed the appearances of a cap not typical of phrygian cap. Uncertainty whether the cap represented the site of biliary system rupture. ERCP demonstrated contrast extravasation from the gallbladder fundus.

Key learning points: Persistence with clinical review of the patient regardless of what the scan demonstrates is paramount to a good outcome for the patient. Correlating with biochemistry markers raises the suspicion of the diagnosis albeit retrospectively in this case. Acknowledgment of the past history is important, in this case the patient was previously confirmed to have gallstones on USS of the abdomen in March 2013.

References: • Rupture of the Common Bile Duct; A Rare Cause of Biliary Peritonitis. Journal of Taibah University Medical Sciences 2011; 6(1): 47 -50. • Kohli S, Singhal A, Arora A, Singhal S. Spontaneous Biliary Peritonitis in Children. J Clin Imaging Sci 2013; 3: 25 • Kang SB, Han SB, Min SK, Lee HK. Nontraumatic perforation of bile duct in adults. Arch Surg. 2004; 139: 1083– 7. • Blegen, H. M, Boyer, E. L. Perforation of Choledochus Cyst with Biliary Peritonitis, Lancet 1946, 66: 117. • Howard ER. Spontaneous biliary perforation. In surgery of liver, bile duct and pancrease in children. 2 nd ed. Arnold publisher; 2002; 169 -174.