A Case of Biliary Atresia With Heterotopia Cartilage at Porta Hepatis

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  • Torikai Motofumi
    Department of Neonatology, Perinatal Medical Center, Kagoshima City Hospital Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University
  • Matsukubo Makoto
    Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University
  • Harumatsu Toshio
    Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University
  • Onishi Shun
    Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University
  • Yamada Koji
    Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University
  • Kawano Takafumi
    Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University
  • Yoshioka Takako
    Department of Pathology, National Center for Child Health and Development
  • Muraji Toshihiro
    Department of Pediatric Surgery, Kirishima Medical Center
  • Kaji Tatsuru
    Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University Clinical Training Center, Kagoshima University Hospital
  • Ieiri Satoshi
    Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University

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Other Title
  • 肝門部結合織に異所性軟骨組織を認めた胆道閉鎖症の1例
  • カンモンブ ケツゴウショク ニ イショセイ ナンコツ ソシキ オ ミトメタ タンドウ ヘイサショウ ノ 1レイ

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Abstract

<p>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.</p>

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