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Developmental Abnormalities of the Bile Duct and Foregut

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Diagnosis of Liver Disease

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Abstract

Extrahepatic biliary atresia, paucity of intrahepatic bile ducts, viral hepatitis, and malformations of bile ducts are the main causes of neonatal cholestasis. Biliary atresia occurs due to inflammation and destruction of extrahepatic bile duct systems in utero and during the perinatal period. Malformations of bile ducts are associated with abnormal remodeling of embryonic bile duct plate and fibrocystic lesions are developed, and they include congenital hepatic fibrosis [1], Caroli’s disease [2], microhamartoma [3], and choledochal cyst, and they carry an increased risk of bile duct carcinoma. Differing from other cystic lesions, ciliated hepatic cyst arising from embryonic foregut is detected incidentally in adults and should be differentiated from bile duct lesions [4]. Infantile and adult types of hereditary polycystic disease cause renal failure, and hepatic failure is rare [5, 6].

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Abbreviations

ADPKD:

Autosomal dominant polycystic kidney disease

ADPLD:

Autosomal dominant polycystic liver disease

ARPKD:

Autosomal recessive polycystic kidney disease

CHF:

Congenital hepatic fibrosis

Erb-B2:

Erythroblastic oncogene B2

Gd-EOB-DTPA-MRI:

Gadolinium ethoxybenzyl diethylenetriaminepentaacetic acid magnetic resonance imaging

MEK:

MAP (Mitogen-Activated Protein) / ERK (Extracellular Signal-Regulated Kinase) kinase

mTOR:

Mechanistic target of rapamycin

SOL:

Space-occupying lesion

Srk kinase:

S-receptor kinase

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Author information

Authors and Affiliations

  1. Kyoto, Japan

    Masaki Iwai MD, PhD

  2. Hyogo, Japan

    Takahiro Mori MD, PhD

  3. Hong Kong, China

    Wilson M. S. Tsui MD, FRCPath

Authors
  1. Masaki Iwai MD, PhD
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  2. Takahiro Mori MD, PhD
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  3. Wilson M. S. Tsui MD, FRCPath
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Corresponding author

Correspondence to Wilson M. S. Tsui MD, FRCPath .

Editor information

Editors and Affiliations

  1. Department of Gastroenterology & Hepatology, Tokyo Women’s Medical University, Shinjuku-ku, Tokyo, Japan

    Etsuko Hashimoto

  2. Department of Gastroenterology/Hepatology, Stanford University School of Medicine, Palo Alto, CA, USA

    Paul Y. Kwo

  3. Department of Pathology and Laboratory Medicine, Dartmouth–Hitchcock Medical Center, Lebanon, NH, USA

    Arief A. Suriawinata

  4. Department of Pathology, Caritas Medical Center, Kowloon, Hong Kong, China

    Wilson M.S. Tsui

  5. Division of Gastroenterology, Hepatology & Internal Medicine, Iwai Clinic, Kamigyo-ku, Kyoto, Japan

    Masaki Iwai

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Iwai, M., Mori, T., Tsui, W.M.S. (2019). Developmental Abnormalities of the Bile Duct and Foregut. In: Hashimoto, E., Kwo, P., Suriawinata, A., Tsui, W., Iwai, M. (eds) Diagnosis of Liver Disease. Springer, Singapore. https://doi.org/10.1007/978-981-13-6806-6_10

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  • DOI: https://doi.org/10.1007/978-981-13-6806-6_10

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  • Online ISBN: 978-981-13-6806-6

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