CONGENITAL COMPLETE ABSENCE (BILATERAL AGENESIS) OF THE DIAPHRAGM - ARARE VARIANT OF CONGENITAL DIAPHRAGMATIC-HERNIA

Citation
Km. Jasnosz et al., CONGENITAL COMPLETE ABSENCE (BILATERAL AGENESIS) OF THE DIAPHRAGM - ARARE VARIANT OF CONGENITAL DIAPHRAGMATIC-HERNIA, American journal of perinatology, 11(5), 1994, pp. 340-343
Citations number
NO
Categorie Soggetti
Pediatrics
ISSN journal
07351631
Volume
11
Issue
5
Year of publication
1994
Pages
340 - 343
Database
ISI
SICI code
0735-1631(1994)11:5<340:CCA(AO>2.0.ZU;2-J
Abstract
Although congenital diaphragmatic hernia is one of the most common con genital anomalies, complete bilateral absence of the diaphragm is a ve ry rare variant, with six cases reported in the literature. A 1040 g b lack male infant was born at 27 weeks' gestation to a 28-year-old blac k woman with a history of minimal prenatal care and cocaine abuse duri ng pregnancy. Apgar scores were 2/1/1/0/1 at 1, 5, 10, 15, and 20 minu tes, respectively and efforts to resuscitate him were unsuccessful. He died at 21/2 hours of age. Autopsy showed complete absence of the dia phragm, pulmonary hypoplasia, and an atrial septal defect of the heart . Cytogenetic studies showed normal male karyotype. We suggest that de creased or interrupted blood supply to the developing diaphragm of thi s infant may have interrupted normal embryogenesis in the early develo pmental stages.