Bilateral congenital diaphragmatic hernia with absent pleura and pericardium.
Document Type
Article
Publication Date
9-1-2005
Identifier
DOI: 10.1002/bdra.20173
Abstract
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.
Journal Title
Birth defects research. Part A, Clinical and molecular teratology
Volume
73
Issue
9
First Page
624
Last Page
627
MeSH Keywords
Abnormalities, Multiple; Female; Follow-Up Studies; Hernia, Diaphragmatic; Hernias, Diaphragmatic, Congenital; Humans; Infant, Newborn; Male; Pericardium; Pleura; Pregnancy; Prenatal Diagnosis; Time Factors; Treatment Outcome
Keywords
Diaphragmatic hernia; Congenital hernias; Pericardium; Pleura
Recommended Citation
St Peter, S. D., Shah, S. R., Little, D. C., Calkins, C. M., Sharp, R. J., Ostlie, D. J. Bilateral congenital diaphragmatic hernia with absent pleura and pericardium. Birth defects research. Part A, Clinical and molecular teratology 73, 624-627 (2005).
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