TY - JOUR
T1 - Patterns of malformation in children with congenital diaphragmatic defects
AU - Cunniff, Christopher
AU - Jones, Kenneth Lyons
AU - Jones, Marilyn C.
N1 - Funding Information:
The medical records of the University of California San Diego Medical Center were examined for cases of congen- Supported in part by National Institutes of Health grant No. AM-07318. Submitted for publication June 12, 1989; accepted Aug. 18, 1989. Reprint requests: Christopher Cunniff, MD, Department of Pediatrics, Slot 512, Universityo f Arkansas for Medical Sciences, 4301 W. Markham St., Little Rock, AR 72205. 9/20/16171 ital diaphragmatic hernia or eventration for the period for January 1976 to April 1988; records at the Children's Hospital of San Diego were examined from the period from January 1978 to December 1987. Excluded were cases of hiatal hernia and cases that were identified at autopsy in stillborn infants or abortuses. Information gathered from the records included gestational age and birth weight, the type and location of the hernia, the presence or absence of associated anomalies, and the final outcome when available. Patients were grouped into five categories on the basis of the anatomic location and type of defect: (1) Bochdalek, posterolateral, and atypical hernias (included in one category because the surgical and autopsy descriptions of the defects varied according to the examiner), (2) hernias through the foramen of Morgagni, (3) absent diaphragm, (4) unspecified types, and (5) diaphragmatic eventration.
PY - 1990/2
Y1 - 1990/2
N2 - The medical records of 102 live-born children with a congenital diaphragmatic defect were reviewed to determine the frequency and nature of underlying chromosomal, genetic, and nongenetic patterns of malformation. Overall, 40 children (39%) had a major nonpulmonary malformation, and 14 of these children (14%) had a previously recognized pattern of malformation. A group of 18 children (18%) with cardiac anomalles had an increased mortality rate in comparison with those children without cardiac defects (72% vs 38%). The frequency and severity of nonpulmonary abnormalitles in children with congenital diaphragmatic defects suggest that examination of affected children should include cardiac evaluation, a karyotype when the defect is one feature of a broader pattern of altered development, and a careful evaluation for minor anomalies, which may provide clues to an overall diagnosis.
AB - The medical records of 102 live-born children with a congenital diaphragmatic defect were reviewed to determine the frequency and nature of underlying chromosomal, genetic, and nongenetic patterns of malformation. Overall, 40 children (39%) had a major nonpulmonary malformation, and 14 of these children (14%) had a previously recognized pattern of malformation. A group of 18 children (18%) with cardiac anomalles had an increased mortality rate in comparison with those children without cardiac defects (72% vs 38%). The frequency and severity of nonpulmonary abnormalitles in children with congenital diaphragmatic defects suggest that examination of affected children should include cardiac evaluation, a karyotype when the defect is one feature of a broader pattern of altered development, and a careful evaluation for minor anomalies, which may provide clues to an overall diagnosis.
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U2 - 10.1016/S0022-3476(05)82884-7
DO - 10.1016/S0022-3476(05)82884-7
M3 - Article
C2 - 2299497
AN - SCOPUS:0025020955
VL - 116
SP - 258
EP - 261
JO - Journal of Pediatrics
JF - Journal of Pediatrics
SN - 0022-3476
IS - 2
ER -