Agenesis of the gallbladder in symptomatic adults. A case and review of the literature.

Agenesis of the gallbladder, with normal bile ducts, is a rare congenital condition occurring in 13 to 65 per 100,000 population, probably from failure of the gallbladder bud to develop or vacuolize in utero. Adults are usually asymptomatic. We report a 40-year-old woman with gallbladder agenesis, found at operation for presumed gallbladder disease. We review 44 similar cases: dyspepsia was the predominant symptom in 15 of 44 patients (34%); 24 of 44 (54%) had symptoms suggestive of biliary colic, and 12 of 44 (27%) had jaundice. Common duct stones were found in eight of 12 patients who were jaundiced, but not in any other. A familial tendency was reported in five series. Our patient and one other had another congenital anomaly. Ultrasound was performed on five patients: three had a small, shrunken gallbladder and in two the gallbladder was absent. Ultrasound may suggest the diagnosis if absence of the gallbladder is demonstrated in conjunction with another congenital anomaly.