BRMS is an uncommon cause of recurrent jaundice and conjugated hyperbilirubinemia in children. Because its presentation may mimic that of a choledochal cyst, the correct diagnosis is frequently made intraoperatively at a planned choledochal cystectomy. However, identification of this entity prior to surgery is important because early surgery may carry excess morbidity and mortality.
An article to be published on August 14, 2008 in the World Journal of Gastroenterology reports this case. The research team led by Ryan W. Himes from Texas Children's Hospital presented a patient with BRMS in whom therapeutic endoscopy was used successfully as an adjunct to chemotherapy and radiotherapy.
Authors presented the case of a 3-year-old with BRMS in whom endoscopic retrograde cholangiopancreatography (ERCP) was successfully used to obtain a tissue specimen for diagnosis and to place a stent to relieve the biliary obstruction while chemotherapeutics were employed, obviating the need for surgery and its attendant risks of morbidity and mortality. The technique allowed them to obtain tissue for diagnostic purposes and to place a biliary stent to relieve obstruction. Standard chemotherapy and radiotherapy regimens were employed to shrink the tumor. The patient is free-of-disease with one year of follow-up. They concluded that ERCP is an effective alternative to surgery for BRMS in some patients.