2020 Volume 56 Issue 7 Pages 1133-1138
The patient was a 60-day-old female infant. She presented with pale-colored stools and jaundice from 40 days after birth. She was admitted to our hospital because of suspicion of biliary atresia (BA) by her former doctor. She had jaundice and hepatomegaly as physical examination findings, and her laboratory data showed hyperbilirubinemia and liver dysfunction. Abdominal ultrasonography showed an atrophic gall bladder and the triangular cord sign, and biliary scintigraphy revealed no tracer exacerbation in the intestine. Because BA was strongly suspected, a trial laparotomy was performed at 67 days of age. Because she was diagnosed as having this disease (III-b1-ν) on the basis of intraoperative findings of an atrophic gallbladder, a cordlike extrahepatic bile duct, and a connective tissue of the porta hepatis, the Kasai procedure was performed. Heterotopia cartilages were found around the cholangiole in the resected connective tissue of the porta hepatis by histopathological analysis. Case reports of heterotopia cartilage tissue in BA are occasionally found, and it is speculated that developmental abnormalities and cartilage metaplasia due to inflammation may be the pathogenetic factors. However, the mechanisms underlying these abnormalities are as yet unclarified. We summarized the case reports of this disorder with heterotopia cartilage and reviewed the literature.
