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Patients with vascular Ehlers–Danlos syndrome (vEDS) have a defect in the formation of type III collagen. This defect puts patients at risk of vascular rupture, uterine rupture, and bowel perforations. The segmental absence of intestinal musculature is a rare histopathologic finding, wherein there is a lack of a muscularis propria layer in the intestinal wall. Although typically documented in the literature in neonates or adults, it can be seen in children of other ages. This is a case report of a patient who exhibits both rare entities, which has not been described in the literature to date.

Treatment of choledochal cysts (CC) includes Roux-en-Y hepaticojejunostomy or choledochoduodenostomy. Postoperative adverse events include stenosis, obstruction, and cholangitis (1). We report 2 CC cases postcholedochoduodenostomy managed by endoscopic retrograde cholangiopancreatography (ERCP) and intraductal endoscopy (Video 1, Supplemental Digital Content, http://links.lww.com/MPG/B286).Case 1: A 4-year-old girl, 15 months postcholedochoduodenostomy for type I CC presents with intermittent epigastric pain. Laboratory reports notable for alanine aminotransferase (ALT) of 174U/L and gamma-glutamyl transferase (GGT) of 170U/L. Hepatobiliary iminodiacetic acid scan was abnormal and magnetic resonance cholangiopancreatography was suggestive of obstruction. Biliary access was obtained via dilating catheters and ERCP-guided balloon dilation with subsequent stent placement. The stent was removed 2 months later with normal labs at 6 months.Case 2: A 15-year-old male 4 years post-choledochoduodenostomy for type IVA CC presents with RUQ pain, and history of recurrent cholangitis and residual cyst with stenosis. Laboratory reports were notable for ALT of 215U/L, GGT of 431U/L, and conjugated bilirubin of 0.8 mg/dL. ERCP with intraductal endoscopy was used to remove stones from the biliary tree (Fig. 1). Laboratory reports normalized within 6 weeks with a plan for surgical revision. Despite similar stenosis rates between Roux-en-Y hepaticojejunostomy and choledochoduodenostomy (2), choledochoduodenostomy may be preferred due to maintenance of bowel integrity and easier endoscopic access. Further research is needed to evaluate differences in the role of each operation and postoperative endoscopic management.