Title

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

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