Agenesis of the gallbladder without extrahepatic biliary atresia
R. S. Bennion, J. E. Thompson Jr and R. K. Tompkins
Department of Surgery, Olive View Medical Center, Sylmar, CA.
Agenesis of the gallbladder without extrahepatic biliary atresia is a rare
disorder. At the UCLA-affiliated hospitals, 12 patients were classified in
the following groups: (1) multiple fetal anomaly, (2) asymptomatic, and (3)
symptomatic. All four patients in the multiple fetal anomaly group died of
their other congenital defects. In the three patients in the asymptomatic
group, the absent gallbladder was an incidental finding at autopsy. The
five patients in the symptomatic group underwent operations for symptoms
suggestive of biliary tract disease, with no gallbladder found; all were
symptom free postoperatively. Operative strategy should include a complete
exploration, operative cholangiography, and common bile duct exploration as
necessary. Possible mechanisms responsible for symptoms include primary
duct stones, biliary dyskinesia, or nonbiliary disorders. Computed
tomography, biliary manometry, upper gastrointestinal tract endoscopy, and
endoscopic cholangiography (with or without sphincterotomy) could be
employed if symptoms continue.
