U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Ductal Plate Malformation

MedGen UID:
388754
•Concept ID:
C2673892
•
Congenital Abnormality
Synonyms: Ductal plate malformation; Ductal plate malformations

Definition

A congenital disorder of the intrahepatic bile ducts characterized by abnormal embryonal development of the ductal plates. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDuctal Plate Malformation

Professional guidelines

PubMed

Hepatic Cysts: Reappraisal of the Classification, Terminology, Differential Diagnosis, and Clinicopathologic Characteristics in 258 Cases.
Armutlu A, Quigley B, Choi H, Basturk O, Akkas G, Pehlivanoglu B, Memis B, Jang KT, Erkan M, Erkan B, Balci S, Saka B, Bagci P, Farris AB, Kooby DA, Martin D, Kalb B, Maithel SK, Sarmiento J, Reid MD, Adsay NV
Am J Surg Pathol 2022 Sep 1;46(9):1219-1233. Epub 2022 Jul 4 doi: 10.1097/PAS.0000000000001930. PMID: 35778790
Jaundice revisited: recent advances in the diagnosis and treatment of inherited cholestatic liver diseases.
Chen HL, Wu SH, Hsu SH, Liou BY, Chen HL, Chang MH
J Biomed Sci 2018 Oct 26;25(1):75. doi: 10.1186/s12929-018-0475-8. PMID: 30367658Free PMC Article
Diagnosis and management of polycystic liver disease.
Gevers TJ, Drenth JP
Nat Rev Gastroenterol Hepatol 2013 Feb;10(2):101-8. Epub 2013 Jan 8 doi: 10.1038/nrgastro.2012.254. PMID: 23296249

Recent clinical studies

Etiology

Genetic, Clinicopathological, and Radiological Features of Intrahepatic Cholangiocarcinoma with Ductal Plate Malformation Pattern.
Chung T, Rhee H, Shim HS, Yoo JE, Choi GH, Kim H, Park YN
Gut Liver 2022 Jul 15;16(4):613-624. Epub 2021 Nov 23 doi: 10.5009/gnl210174. PMID: 34810298Free PMC Article
Biliary atresia liver histopathological determinants of early post-Kasai outcome.
Nguyen AP, Pham YHT, Vu GH, Nguyen MH, Hoang TN, Holterman A
J Pediatr Surg 2021 Jul;56(7):1169-1173. Epub 2021 Mar 26 doi: 10.1016/j.jpedsurg.2021.03.039. PMID: 33838902
Cholangiolocellular Carcinoma With "Ductal Plate Malformation" Pattern May Be Characterized by ARID1A Genetic Alterations.
Sasaki M, Sato Y, Nakanuma Y
Am J Surg Pathol 2019 Mar;43(3):352-360. doi: 10.1097/PAS.0000000000001201. PMID: 30520820
New Advances in Polycystic Liver Diseases.
Santos-Laso A, Izquierdo-Sánchez L, Lee-Law PY, Perugorria MJ, Marzioni M, Marin JJ, Bujanda L, Banales JM
Semin Liver Dis 2017 Feb;37(1):45-55. Epub 2017 Feb 15 doi: 10.1055/s-0036-1597817. PMID: 28201848
Biliary atresia revisited.
Kahn E
Pediatr Dev Pathol 2004 Mar-Apr;7(2):109-24. Epub 2004 Mar 4 doi: 10.1007/s10024-003-0307-y. PMID: 14994122

Diagnosis

Congenital hepatic fibrosis: case report and review of literature.
Hasbaoui BE, Rifai Z, Saghir S, Ayad A, Lamalmi N, Abilkassem R, Agadr A
Pan Afr Med J 2021;38:188. Epub 2021 Feb 18 doi: 10.11604/pamj.2021.38.188.27941. PMID: 33995794Free PMC Article
Jaundice revisited: recent advances in the diagnosis and treatment of inherited cholestatic liver diseases.
Chen HL, Wu SH, Hsu SH, Liou BY, Chen HL, Chang MH
J Biomed Sci 2018 Oct 26;25(1):75. doi: 10.1186/s12929-018-0475-8. PMID: 30367658Free PMC Article
Benign biliary diseases.
Bali MA, Pezzullo M, Pace E, Morone M
Eur J Radiol 2017 Aug;93:217-228. Epub 2017 May 27 doi: 10.1016/j.ejrad.2017.05.035. PMID: 28668418
Diagnosis and management of polycystic liver disease.
Gevers TJ, Drenth JP
Nat Rev Gastroenterol Hepatol 2013 Feb;10(2):101-8. Epub 2013 Jan 8 doi: 10.1038/nrgastro.2012.254. PMID: 23296249
Biliary atresia revisited.
Kahn E
Pediatr Dev Pathol 2004 Mar-Apr;7(2):109-24. Epub 2004 Mar 4 doi: 10.1007/s10024-003-0307-y. PMID: 14994122

Therapy

Bile Acids in Polycystic Liver Diseases: Triggers of Disease Progression and Potential Solution for Treatment.
Perugorria MJ, Labiano I, Esparza-Baquer A, Marzioni M, Marin JJ, Bujanda L, Banales JM
Dig Dis 2017;35(3):275-281. Epub 2017 Mar 1 doi: 10.1159/000450989. PMID: 28249268
The limited prognostic value of liver histology in children with biliary atresia.
Czubkowski P, Cielecka-Kuszyk J, Rurarz M, Kamińska D, Markiewicz-Kijewska M, Pawłowska J
Ann Hepatol 2015 Nov-Dec;14(6):902-9. doi: 10.5604/16652681.1171781. PMID: 26436363
How valuable is ductal plate malformation as a predictor of clinical course in postoperative biliary atresia patients?
Arii R, Koga H, Arakawa A, Miyahara K, Lane GJ, Okazaki T, Urao M, Yamataka A
Pediatr Surg Int 2011 Mar;27(3):275-7. doi: 10.1007/s00383-010-2793-0. PMID: 21069347
Neonatal cholestasis: differentiation of biliary atresia from neonatal hepatitis in a developing country.
Poddar U, Thapa BR, Das A, Bhattacharya A, Rao KL, Singh K
Acta Paediatr 2009 Aug;98(8):1260-4. Epub 2009 May 13 doi: 10.1111/j.1651-2227.2009.01338.x. PMID: 19469771
Significance of ductal plate malformation in the postoperative clinical course of biliary atresia.
Shimadera S, Iwai N, Deguchi E, Kimura O, Ono S, Fumino S, Higuchi K
J Pediatr Surg 2008 Feb;43(2):304-7. doi: 10.1016/j.jpedsurg.2007.10.023. PMID: 18280279

Prognosis

Diversity in cell differentiation, histology, phenotype and vasculature of mass-forming intrahepatic cholangiocarcinomas.
Nguyen Canh H, Takahashi K, Yamamura M, Li Z, Sato Y, Yoshimura K, Kozaka K, Tanaka M, Nakanuma Y, Harada K
Histopathology 2021 Nov;79(5):731-750. Epub 2021 Aug 6 doi: 10.1111/his.14417. PMID: 34018212
Biliary atresia liver histopathological determinants of early post-Kasai outcome.
Nguyen AP, Pham YHT, Vu GH, Nguyen MH, Hoang TN, Holterman A
J Pediatr Surg 2021 Jul;56(7):1169-1173. Epub 2021 Mar 26 doi: 10.1016/j.jpedsurg.2021.03.039. PMID: 33838902
Benign biliary diseases.
Bali MA, Pezzullo M, Pace E, Morone M
Eur J Radiol 2017 Aug;93:217-228. Epub 2017 May 27 doi: 10.1016/j.ejrad.2017.05.035. PMID: 28668418
Ductal plate malformation in patients with biliary atresia.
Vuković J, Grizelj R, Bojanić K, Corić M, Luetić T, Batinica S, Kujundžić-Tiljak M, Schroeder DR, Sprung J
Eur J Pediatr 2012 Dec;171(12):1799-804. Epub 2012 Sep 15 doi: 10.1007/s00431-012-1820-7. PMID: 22983023
Significance of ductal plate malformation in the postoperative clinical course of biliary atresia.
Shimadera S, Iwai N, Deguchi E, Kimura O, Ono S, Fumino S, Higuchi K
J Pediatr Surg 2008 Feb;43(2):304-7. doi: 10.1016/j.jpedsurg.2007.10.023. PMID: 18280279

Clinical prediction guides

Genetic, Clinicopathological, and Radiological Features of Intrahepatic Cholangiocarcinoma with Ductal Plate Malformation Pattern.
Chung T, Rhee H, Shim HS, Yoo JE, Choi GH, Kim H, Park YN
Gut Liver 2022 Jul 15;16(4):613-624. Epub 2021 Nov 23 doi: 10.5009/gnl210174. PMID: 34810298Free PMC Article
Biliary atresia liver histopathological determinants of early post-Kasai outcome.
Nguyen AP, Pham YHT, Vu GH, Nguyen MH, Hoang TN, Holterman A
J Pediatr Surg 2021 Jul;56(7):1169-1173. Epub 2021 Mar 26 doi: 10.1016/j.jpedsurg.2021.03.039. PMID: 33838902
Significance of ductal plate malformation in the postoperative clinical course of biliary atresia.
Shimadera S, Iwai N, Deguchi E, Kimura O, Ono S, Fumino S, Higuchi K
J Pediatr Surg 2008 Feb;43(2):304-7. doi: 10.1016/j.jpedsurg.2007.10.023. PMID: 18280279
Molecular and cellular pathogenesis of autosomal recessive polycystic kidney disease.
Menezes LF, Onuchic LF
Braz J Med Biol Res 2006 Dec;39(12):1537-48. doi: 10.1590/s0100-879x2006001200004. PMID: 17160262
What is congenital hepatic fibrosis?
Desmet VJ
Histopathology 1992 Jun;20(6):465-77. doi: 10.1111/j.1365-2559.1992.tb01031.x. PMID: 1607148

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...