J Pediatr Surg

. 2021 Jun 25;S0022-3468(21)00473-5. doi: 10.1016/j.jpedsurg.2021.06.007. Online ahead of print https://pubmed.ncbi.nlm.nih.gov/34274078/

Long-term outcomes of congenital diaphragmatic hernia: A single institution experience

Claire D Gerall ¹, Latoya A Stewart ¹, Jessica Price ¹, Sandra Kabagambe ¹, Shelby R Sferra ¹, Maggie J Schmaedick ¹, Rebecca Hernan ², Julie Khlevner ³, Usha S Krishnan ⁴, Aliva De ⁵, Gudrun Aspelund ¹, Vincent P Duron ⁶Affiliations expand

• PMID: 34274078
• DOI: 10.1016/j.jpedsurg.2021.06.007

Abstract

Background/purpose: As survival rates for patients with congenital diaphragmatic hernia (CDH) increase, long-term sequelae become increasingly prevalent. We present the outcomes of patients who underwent CDH repair at our institution and discuss standardization of follow-up care in our long-term multidisciplinary follow-up clinic.

Methods: A retrospective review of patients followed in multidisciplinary clinic after CDH repair at our institution from January 1, 2005 to December 1, 2020.

Results: A total of 193 patients met inclusion criteria, 73 females (37.8%) and 120 males (62.2%). Left-sided defects were most common (75.7%), followed by right-sided defects (20.7%). Median age at repair was 4 days (IQR 3-6) and 59.6% of all defects required patch repair. Median length of stay was 29 days (IQR 16.8-50.0). Median length of follow up was 49 months (IQR 17.8-95.3) with 25 patients followed for more than 12 years. Long-term outcomes included gastroesophageal reflux disease (42.0%), diaphragmatic hernia recurrence (10.9%), asthma (23.6%), neurodevelopmental delay (28.6%), attention deficit hyperactivity disorder (7.3%), autism (1.6%), chest wall deformity (15.5%), scoliosis (11.4%), and inguinal hernia (6.7%).

Conclusion: As survival of patients with CDH improves, long-term care must be continuously studied and fine-tuned to ensure appropriate surveillance and optimization of long-term outcomes.

Keywords: Congenital diaphragmatic hernia; Gastroesophageal Reflux; Long-Term Outcomes; Pulmonary hypertension.

Copyright © 2021. Published by Elsevier Inc.