Phrygian cap deformity in a pediatric patient: a case report

The overall prevalence of congenital causes of gallstones is not well established. The Phrygian cap is a congenital anomaly of the gallbladder with an incidence of 4%. The literature provides a few case reports on Phrygian cap deformity with or without associated gallstones. This report presents a 15-year-old Hispanic, overweight female patient transferred to our service due to an aborted laparoscopic cholecystectomy. During the initial surgery, there was a suspicion of choledochal/gallbladder cyst. Due to intraoperative and technical difficulties, the patient was referred to our pediatric surgical service. All her prior imaging was unremarkable. A magnetic resonance cholangiopancreatogram revealed mild prominence of the cystic duct with a slightly ovoid in configuration, normal sized intrahepatic and common bile ducts, suspecting a type VI choledochal cyst. Endoscopic retrograde cholangiopancreatography, showed a phrygian cap deformity. The decision was made to proceed with a standard laparoscopic cholecystectomy. This report highlights that while cholecystectomy is curative, additional imaging may be needed to establish anatomy and diagnosis in unusual gallbladder anatomy.