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J Pediatr Surg. 2019 Apr;54(4):651-655. doi: 10.1016/j.jpedsurg.2018.04.008. Epub 2018 Apr 14.

Prenatally versus postnatally diagnosed congenital diaphragmatic hernia - Side, stage, and outcome.

Author information

1
Department of Pediatric Surgery, Karolinska Institutet, Stockholm, Sweden.
2
Department of Neonatology, School of Medicine Pontificia Universidad Católica de, Chile.
3
Department of Pediatric Surgery, McGovern Medical School at UT Health and Children's Memorial Hermann Hospital, Houston, TX, US.

Abstract

AIM:

To compare outcomes between prenatally and postnatally diagnosed CDH in a large multicenter database of prospectively collected data and evaluate factors associated with poorer outcome for prenatally diagnosed CDH.

MATERIAL AND METHODS:

We used information from the multicenter, multinational CDH Study Group database on patients born between 2007 and 2015. We compared differences between prenatally and postnatally diagnosed CDH with respect to survival, side, size, ECMO needs, associated major cardiac malformations and liver position.

RESULTS:

3746 cases of CDH were entered in the registry between 2007 and 2015, with an overall survival of 71%. Of those, 68% had a prenatal diagnosis. Survival rates were significantly better in the postnatally diagnosed group, 83 vs 65%. There was a higher proportion of bigger defect sizes, C and D, in the prenatally diagnosed group, but the survival rates were similar when patients were stratified by defect size. The rate of ECMO utilization was higher overall in the prenatally diagnosed group, 33 vs 22%, but it was similar within similar defect sizes. Right-sided defects are more commonly missed at prenatal screening than left-sided CDH, 53 vs 35% (p < 0.0001).

CONCLUSIONS:

Prenatally diagnosed CDH is associated with larger defect sizes compared to those with a postnatal diagnosis, and consequently have higher morbidity and mortality. Right-sided CDH are more often missed at prenatal ultrasound. The increasing rate of prenatal detection requires a clear understanding of accurate risk stratification, in order to counsel families and to provide appropriate perinatal management.

LEVEL OF EVIDENCE:

I for a Prognosis Study - This is a high-quality, prospective cohort study with 99% of patients followed to the study end point (death or discharge).

KEYWORDS:

(CDH); CDH Study Group; Congenital diaphragmatic hernia; Outcome; Postnatal diagnosis; Prenatal diagnosis; Stage classification; Survival

PMID:
29753526
DOI:
10.1016/j.jpedsurg.2018.04.008
[Indexed for MEDLINE]

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