A 21-year-old male, with known short bowel syndrome (42 cm jejunum with colon, no ileocecal valve) secondary to surgical therapy of mid-jejunal atresia and total parenteral nutrition (TPN) dependence, was… Click to show full abstract
A 21-year-old male, with known short bowel syndrome (42 cm jejunum with colon, no ileocecal valve) secondary to surgical therapy of mid-jejunal atresia and total parenteral nutrition (TPN) dependence, was evaluated in the Emergency Department with a one-day history of acute, severe, epigastric pain. He reported no nausea, vomiting, or diarrhea. His past medical history was significant for multiple abdominal surgeries (jejunal atresia repair, tapering jejunoplasty following stricture at the jejuno-colonic anastomosis), cholelithiasis (Fig. 1a), infections of central venous catheters, small bowel bacterial overgrowth, and acute pancreatitis. His medications included calcium carbonate, cholecalciferol, ciprofloxacin, fluticasone, loperamide, multivitamins, omega 3-6-9 fatty acids, potassium chloride, rifaximin, and ferrous sulfate. On examination, the patient was afebrile and had normal vital signs and abdominal examination. Laboratory values were notable for lipase 20 U/L, total bilirubin 1.6 mg/dL, AST 57 U/L, ALT 63 U/L, and alkaline phosphatase 244 U/L. Abdominal ultrasound revealed a 1.9-cm non-obstructing gallstone near the neck of the gallbladder, without common bile duct (CBD) dilation at 6 mm. After receiving hydromorphone and a ‘‘GI Cocktail’’ (antacid, viscous lidocaine, and anticholinergic), his pain improved, and he was discharged to home. The next day, he developed severe abdominal pain only minimally improved with medications. He was admitted and shortly thereafter became febrile. He was placed on piperacillin and tazobactam and vancomycin due to concern for ascending cholangitis. On hospital day 2, total bilirubin peaked at 4.2 mg/dL, he grew two forms of Escherichia coli from his central venous catheter, and he was switched to meropenem. On hospital day 3, he developed fluid-responsive hypotension, which was also treated with low-dose norepinephrine. A repeat abdominal ultrasound, with the patient changing positions, identified that the stone was immobile (Fig. 1b). Magnetic resonance cholangiopancreatography (MRCP) showed an impacted gallstone within the gallbladder neck with mass effect on the adjacent CBD, but no evidence of intrahepatic duct dilation (Fig. 2). Stent placement via endoscopic retrograde cholangiopancreatography (ERCP) with cholecystectomy versus immediate surgery was discussed. A joint decision was made by the pediatric surgical and gastroenterological services to proceed directly to laparoscopic surgery. At laparoscopy, it took 45 min to find the gallbladder, due to adhesions from previous operations. Eventually, the gallbladder was freed; after opening the fundus to identify the cystic duct, a cholangiogram identified that there were no stones in the CBD. A large stone was identified in the gallbladder that was externally compressing the CBD. Surgical findings and pathology results were consistent with the diagnosis of Mirizzi syndrome, Type I (Fig. 3). Postoperatively, the patient was intermittently febrile overnight while & Zachary M. Sellers [email protected]
               
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