Bilateral congenital diaphragmatic hernia with absent pleura and pericardium. Birth Defects Res A Clin Mol Teratol 2005 Sep;73(9):624-7
Date
07/08/2005Pubmed ID
16001446DOI
10.1002/bdra.20173Scopus ID
2-s2.0-26444596646 (requires institutional sign-in at Scopus site) 10 CitationsAbstract
BACKGROUND: Bilateral congenital diaphragmatic hernia is a rare form of diaphragmatic hernia. Independently, pericardial defects are an extremely rare phenomenon. In the case presented, we provide the first complete description of an infant with bilateral congenital diaphragmatic hernia with complete agenesis of the pericardium and inferior parietal pleura.
CASE: A male infant was born at 38 weeks of gestation with a prenatal diagnosis of left-sided congenital diaphragmatic hernia. After 1 week of aggressive management, the patient was taken to the operating room for repair. Intraoperatively, the patient was found to have absence of the diaphragm bilaterally, no pleura inferiorly, and no pericardium. A biological mesh was used to construct a diaphragm. At 6 months of age, the patient is growing normally, requiring only supplemental oxygen without pressure support.
CONCLUSIONS: Embryologically, this anomaly represents complete lack of development of the pleurocardial folds, pleuroperitoneal folds, and transverse septum, which is previously unreported.
Author List
St Peter SD, Shah SR, Little DC, Calkins CM, Sharp RJ, Ostlie DJAuthor
Casey Matthew Calkins MD Professor in the Surgery department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
Abnormalities, MultipleFemale
Follow-Up Studies
Hernia, Diaphragmatic
Hernias, Diaphragmatic, Congenital
Humans
Infant, Newborn
Male
Pericardium
Pleura
Pregnancy
Prenatal Diagnosis
Time Factors
Treatment Outcome
