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Complete diaphragmatic absence of pericardium

MedGen UID:
868849
•Concept ID:
C4023259
•
Congenital Abnormality
HPO: HP:0011630

Definition

No pericardium over the diaphragmatic surface of the heart. It is a congenital defect, not the result of a pericardectomy. Pericardium is present on other parts of the heart. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVComplete diaphragmatic absence of pericardium

Recent clinical studies

Etiology

Frequency of sternal variations and anomalies in living individuals evaluated by MDCT.
Vatzia K, Fanariotis M, Makridis KG, Vlychou M, Fezoulidis IV, Vassiou K
Eur J Radiol 2021 Sep;142:109828. Epub 2021 Jun 24 doi: 10.1016/j.ejrad.2021.109828. PMID: 34280594
Incomplete Pentalogy of Cantrell--A Case Report.
Ali MA, Tazmin T, Latif T, Haque SA, Hossain MA, Islam MN, Khan RH, Hoque MA
Mymensingh Med J 2016 Jan;25(1):153-7. PMID: 26931266
A rare case of congenital diaphragmatic hernia with ectopic liver and absent pericardium.
Kamath GS, Borkar S, Chauhan A, Chidanand B, Kashyap N, Warrier R
Ann Thorac Surg 2010 May;89(5):e36-7. doi: 10.1016/j.athoracsur.2010.02.041. PMID: 20417741
Bilateral congenital diaphragmatic hernia with absent pleura and pericardium.
St Peter SD, Shah SR, Little DC, Calkins CM, Sharp RJ, Ostlie DJ
Birth Defects Res A Clin Mol Teratol 2005 Sep;73(9):624-7. doi: 10.1002/bdra.20173. PMID: 16001446

Diagnosis

The complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twins: A case report of a rare congenital anomaly.
Zvizdic Z, Sefic-Pasic I, Mesic A, Terzic S, Vranic S
Medicine (Baltimore) 2021 Apr 9;100(14):e25470. doi: 10.1097/MD.0000000000025470. PMID: 33832161Free PMC Article
Class II pentalogy of Cantrell.
Naburi H, Assenga E, Patel S, Massawe A, Manji K
BMC Res Notes 2015 Jul 29;8:318. doi: 10.1186/s13104-015-1293-7. PMID: 26219662Free PMC Article
A rare case of congenital diaphragmatic hernia with ectopic liver and absent pericardium.
Kamath GS, Borkar S, Chauhan A, Chidanand B, Kashyap N, Warrier R
Ann Thorac Surg 2010 May;89(5):e36-7. doi: 10.1016/j.athoracsur.2010.02.041. PMID: 20417741
Bilateral congenital diaphragmatic hernia with absent pleura and pericardium.
St Peter SD, Shah SR, Little DC, Calkins CM, Sharp RJ, Ostlie DJ
Birth Defects Res A Clin Mol Teratol 2005 Sep;73(9):624-7. doi: 10.1002/bdra.20173. PMID: 16001446
Symptomatic congenital complete absence of the left pericardium. Case report and review of the literature.
Gehlmann HR, van Ingen GJ
Eur Heart J 1989 Jul;10(7):670-5. doi: 10.1093/oxfordjournals.eurheartj.a059545. PMID: 2670568

Prognosis

The complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twins: A case report of a rare congenital anomaly.
Zvizdic Z, Sefic-Pasic I, Mesic A, Terzic S, Vranic S
Medicine (Baltimore) 2021 Apr 9;100(14):e25470. doi: 10.1097/MD.0000000000025470. PMID: 33832161Free PMC Article
Class II pentalogy of Cantrell.
Naburi H, Assenga E, Patel S, Massawe A, Manji K
BMC Res Notes 2015 Jul 29;8:318. doi: 10.1186/s13104-015-1293-7. PMID: 26219662Free PMC Article
Pentalogy of cantrell with complete ectopia cordis in a fetus with asplenia.
Ludwig K, Salmaso R, Cosmi E, Iaria L, De Luca A, Margiotti K, Valentina C, Manara R, Rugge M
Pediatr Dev Pathol 2012 Nov-Dec;15(6):495-8. Epub 2012 Aug 17 doi: 10.2350/12-03-1169-CR.1. PMID: 22900993
A rare case of congenital diaphragmatic hernia with ectopic liver and absent pericardium.
Kamath GS, Borkar S, Chauhan A, Chidanand B, Kashyap N, Warrier R
Ann Thorac Surg 2010 May;89(5):e36-7. doi: 10.1016/j.athoracsur.2010.02.041. PMID: 20417741
Bilateral congenital diaphragmatic hernia with absent pleura and pericardium.
St Peter SD, Shah SR, Little DC, Calkins CM, Sharp RJ, Ostlie DJ
Birth Defects Res A Clin Mol Teratol 2005 Sep;73(9):624-7. doi: 10.1002/bdra.20173. PMID: 16001446

Clinical prediction guides

Frequency of sternal variations and anomalies in living individuals evaluated by MDCT.
Vatzia K, Fanariotis M, Makridis KG, Vlychou M, Fezoulidis IV, Vassiou K
Eur J Radiol 2021 Sep;142:109828. Epub 2021 Jun 24 doi: 10.1016/j.ejrad.2021.109828. PMID: 34280594
The complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twins: A case report of a rare congenital anomaly.
Zvizdic Z, Sefic-Pasic I, Mesic A, Terzic S, Vranic S
Medicine (Baltimore) 2021 Apr 9;100(14):e25470. doi: 10.1097/MD.0000000000025470. PMID: 33832161Free PMC Article

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