J Pediatr Surg

. 2022 Jan 13;S0022-3468(22)00033-1. doi: 10.1016/j.jpedsurg.2021.12.048. Online ahead of print. https://pubmed.ncbi.nlm.nih.gov/35090718/

Risk stratification helps identify congenital diaphragmatic hernia (CDH) infants in need of formal neurodevelopmental assessment: Observations from a structured, interdisciplinary long-term follow-up clinic

Mahdi Hassan ¹, Dylan Patel ¹, Kathryn LaRusso ¹, Louise Koclas ², Megan Smith-Morin Ot ³, Adam J Shapiro ⁴, Ana Sant’Anna ⁵, Gabriel Altit ², Sabrina Beauseigle ¹, Pramod S Puligandla ⁶Affiliations expand

• PMID: 35090718
• DOI: 10.1016/j.jpedsurg.2021.12.048

Abstract

Background/purpose: Neurodevelopmental delay (NDD) affects congenital diaphragmatic hernia (CDH) infants. Initial assessment by experienced developmental pediatricians, supported by Bayley-3 tests, is a viable pathway for NDD identification and surveillance. We risk stratified CDH infants to observe differences in incidence and type of NDD based on disease severity.

Methods: Patients from a CDH long-term follow-up database started in 2012 were reviewed (REB#2019-4583). Risk stratification into low, moderate, and high-risk cohorts was performed using the CDH Study Group Mortality Prediction Score. Patients requiring ECLS, supplemental oxygen at 30 days and patch repair were also considered high-risk (i.e. usual clinical criteria). Post-discharge NDD assessments by developmental pediatricians and occupational therapists (Bayley-3) were analyzed for all patients >18months. NDD incidence and type per risk group was determined using descriptive statistics.

Results: Of 102 CDH patients included for study, 26% (27/102) had NDD. Risk stratification identified 2(2%), 7(7%), and 18(18%) patients with NDD in the low, moderate and high-risk groups, respectively. Language delay (2 low; 6 moderate; 10 high) was the most prevalent. Three patients had both expressive and receptive language delay. Motor deficits were observed almost exclusively in the high-risk group.

Conclusion: Based on our experience, NDD affects one-quarter of CDH infants. Risk stratification helped identify infants at increased risk of NDD. While language delays predominated across all risk groups, multiple deficits occurred in higher risk cohorts. These patients should receive structured NDD assessment as part of an optimal interdisciplinary CDH care pathway.

Keywords: Congenital diaphragmatic hernia; Disease severity; Long-term surveillance; Neurodevelopmental outcomes; Risk stratification.

Copyright © 2022. Published by Elsevier Inc.