Adenomyomatosis of the Gallbladder With Pancreaticobiliary Maljunction in a Child

Abstract

Adenomyomatosis of the Gallbladder With Pancreaticobiliary Maljunction in a ChildAn 8-year-old girl referred for recurrent abdominal pain for 1 year had a normal physical examination except mild tenderness in the right hypochondrium and normal laboratory values such as AST/ALT 23/12 U/L, GGT 15 U/L, total bilirubin 0.9 mg/dL, and lipase 11 U/L. Magnetic resonance cholangiopancreatography (MRCP) showed thickening of the gallbladder wall, multiple heterogeneous hyperintense cysts of the gallbladder fundus, and no dilatation of the bile duct (Fig. 1). Endoscopic retrograde cholangiopancreatography showed pancreaticobiliary maljunction (PBM) not previously seen on MRCP (Fig. 2). The length of the common channel was approximately 1 cm outside duodenal wall (Fig. 2). She was diagnosed with adenomyomatosis of the gallbladder (AMG) accompanied by PBM and underwent total resection of the extrahepatic bile duct and Roux-en-Y hepaticojejunostomy. Histopathology showed typical AMG findings and no malignancy. She remained free of abdominal pain at 5 months after surgery.AMG is relatively common in adults ( 1), but we know of only 4 previously reported pediatric cases (2). Once the diagnosis of PBM is established, immediate prophylactic surgery is recommended irrespective of biliary dilatation because biliary cancers are frequent in adults with PBM (3). AMG with PBM has been reported previously in adults (4) but not in children. Tanno et al (4) reported that chronic inflammation and/or increased intraluminal pressure caused by regurgitation of pancreatic juice into the gallbladder may lead to AMG in patients with PBM. MRCP and/or endoscopic retrograde cholangiopancreatography should be performed to ensure prompt diagnosis of PBM.

Adenomyomatosis of the gallbladder in an adolescent, a very rare condition

“…Although the localized type is most common, in the small group of children observed, the diffuse type was found in most cases, with five described by different authors 1,3,5,8,9 (50 %). Our case of the segmental type would be the third in the available literature.…”

“…6,7 ADMG was first described in 1960 by Jutras, and since then has increasingly been reported in adults, 1,6,8 nevertheless, in children it remains extremely rare. 1,3,9 ADMG is divided into three types: generalized, l o c a l i z e d , a n d s e g m e n t a l . G e n e r a l i z e d adenomyomatosis is a diffuse thickening of the gallbladder wall with intramural cystic spaces.…”

“…Agrusti, Infundibulum with normal wall, et al 11 central portion with wall thickening and microgallstones within the muscular layer, gallbladder fundus with a mild concentric wall thickening and small gallstones Eda, No data. MRCP -thickening of the gallbladder wall, Endoscopic retrograde et al 9 multiple heterogeneous hyperintense cholangiopancreatographycysts of the gallbladder fundus, and pancreaticobiliary no dilatation of the bile duct. maljunction.…”

Adenomiomatosis vesicular en un adolescente, una afección muy rara

“…Desde entonces, se ha reportado cada vez más en los adultos; 1,6,8 sin embargo, en los niños sigue siendo sumamente rara. 1,3,9 La adenomiomatosis vesicular se divide en tres tipos: generalizada, localizada y segmentaria. La adenomiomatosis generalizada es el engrosamiento difuso de la pared vesicular, con espacios quísticos intraparietales.…”

“…El tipo localizado 2,6 y el tipo segmentario 10,11 se mencionaron en dos informes cada uno (20 %). Si bien el tipo localizado es el más frecuente, en el pequeño grupo de niños estudiado, se halló el tipo difuso más comúnmente, con cinco casos descriptos por distintos autores 1,3,5,8,9 (50 %). Nuestro paciente con el tipo segmentario sería el tercero mencionado en la bibliografía.…”