A Case of Choledocholithiasis and Intestinal Malrotation in an Adolescent with Repaired Gastroschisis
A 17-year-old girl visited Chungbuk National University Hospital, presenting with fever and abdominal pain that had developed 7 days previously. She had undergone an operation for gastroschisis repair at birth. At the time of admission, her vital signs were as follows: blood pressure, 120/80 mm Hg; pulse rate, 70 beats per minute; respiration rate, 18 breaths per minute; and body temperature, 37.5℃. She appeared emaciated and acutely ill. Her body mass index was 18.1 (height, 151 cm; and weight, 41.3 kg). A physical examination revealed icteric sclera, a very thin abdominal cavity, and a longitudinal surgical scar at the midline. Tenderness was noted in the upper abdominal area, and normal bowel sounds were heard. There was no particular finding in the limb or neurological examinations. Laboratory analysis revealed a white blood cell count of 27,000 cells/mm3; hemoglobin level, 12.3 g/dL; platelet count, 277,000 cells/mm3; aspartate aminotransferase level, 108 IU/L; alanine aminotransferase level, 144 IU/L; alkaline phosphatase level, 476 IU/L; total protein level, 6.5 g/dL; albumin level, 3.7 g/dL; total bilirubin level, 14.1 mg/dL; direct bilirubin level, 9.2 mg/dL; amylase level, 592 U/L; lipase level, 825 U/L; blood urea nitrogen level, 5.0 mg/dL; and creatinine level, 0.5 mg/dL. These data were compatible with diagnoses of acute cholangitis and acute biliary pancreatitis. An abdominal computed tomography scan revealed a round calcifying lesion near the pancreas (), and the absence of the retroperitoneal duodenum and the anterior position of the spleen with respect to the left kidney. The anterior and left-sided position of the superior mesenteric vein compared with that of the superior mesenteric artery was observed in the abdominal computed tomography scan (). The coronal view revealed the distorted architecture of the liver and a high-attenuation lesion at the extrahepatic bile duct (). Results of a barium examination revealed a right-sided duodenojejunal flexure and malpositioning of the bowel, with the small bowel on the right side and the colon on the left side (). Intestinal malrotation could be diagnosed based on these findings. ERCP was performed by using a lateral-view endoscope in the usual way. After approaching the ampulla, the endoscope was pushed against the duodenal wall to maintain the papilla in the correct position, and a selective bile duct cannulation confirmed the presence of a 12-mm round stone in the common bile duct. With a guidewire-assisted technique, the single dark brown stone was removed by using a Dormia basket after the endoscopic sphincterotomy (). The procedure lasted for 15 minutes. A cholangiogram obtained through an endoscopic nasobiliary drainage tube revealed a distorted biliary system, which was characterized with diffuse dilatation of the intrahepatic and extrahepatic biliary trees, downward-positioned gallbladder and a right intrahepatic biliary tree, and a horizontally located common bile duct. The abnormal orientation of the duodenum suggested intestinal malrotation (). Clinical symptoms and liver function tests normalized after the ERCP procedure. The patient has been monitored for 3 years and is in a normal state.