Cornelia de Lange syndrome and congenital diaphragmatic hernia

Cornelia de Lange syndrome and congenital diaphragmatic hernia

There is a known association between Cornelia de Lange syndrome (CdLS) and congenital diaphragmatic hernia (CDH), with CDH being the cause of death in 5%–20% of CdLS cases. We aimed to identify and describe patients with CDLS and CDH. We hypothesized that CdLS would be associated with high-risk CDH...

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Veröffentlicht in:Journal of pediatric surgery 2021-04, Vol.56 (4), p.697-699
Hauptverfasser: Gupta, Vikas S., Khan, Amir M., Ebanks, Ashley H., Lally, Pamela A., Lally, Kevin P., Harting, Matthew T.
Format: Artikel
Sprache:eng
Schlagworte:
Brachmann de Lange syndrome
CDH study group
Congenital diaphragmatic hernia
Cornelia de Lange syndrome
Outcomes
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Zusammenfassung:There is a known association between Cornelia de Lange syndrome (CdLS) and congenital diaphragmatic hernia (CDH), with CDH being the cause of death in 5%–20% of CdLS cases. We aimed to identify and describe patients with CDLS and CDH. We hypothesized that CdLS would be associated with high-risk CDH and poor outcomes. CDH Study Group patients from 1995 to 2019 were included. Those with CdLS were reviewed retrospectively. Rates of repair and outcomes were compared between patients with and without CdLS. We identified 9,251 CDH patients. Of those, 21 had confirmed CdLS. CdLS patients had a lower birth weight (2.2±0.57 kg) than non-CdLS patients (2.9±0.64 kg) (p
ISSN:0022-3468
1531-5037
DOI:10.1016/j.jpedsurg.2020.06.003