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Neurodevelopmental Outcome Following Open Repair of Congenital Diaphragmatic Hernia with and without Support of
Rainer Kubiak1, Thomas Schaible2, Christel Weiss3
1Department of Pediatric Surgery, Children's Hospital of Eastern Switzerland, Sankt Gallen, Switzerland; Department of Pediatric Surgery, Helios Klinikum, University of Heidelberg, Pforzheim, Germany.
Insights
Most children with congenital diaphragmatic hernia (CDH) achieve normal neurodevelopment by age six. Extracorporeal membrane oxygenation (ECMO) was linked to early cognitive delays, while surgical factors influenced later outcomes.
Area of Science:
- Pediatric Surgery
- Neonatal Medicine
- Developmental Neuroscience
Background:
- Congenital diaphragmatic hernia (CDH) is a severe birth defect requiring complex medical and surgical management.
- Neurodevelopmental outcomes in CDH survivors are a significant concern, with potential impacts from disease severity and treatment modalities like ECMO.
- Longitudinal data on developmental trajectories in CDH patients are crucial for understanding long-term sequelae.
Purpose of the Study:
- To determine the incidence of neurodevelopmental delays in children with CDH.
- To identify risk factors for neurodevelopmental delay in CDH patients, comparing those treated with and without ECMO.
- To analyze cognitive, language, and motor development from 6 months to 6 years of age.
Main Methods:
- Prospective follow-up of 278 children undergoing open CDH repair (2009-2018).
- Standardized neurodevelopmental assessments (cognitive, language, motor) conducted between 6 months and 6 years.
- Univariable and multivariable logistic regression used to identify risk factors for developmental delay.
Main Results:
- Of 278 patients, 784 assessments were completed; 130 received ECMO support.
- ECMO group showed higher rates of early cognitive delays, but language and motor outcomes were similar.
- Re-operation in the first year was associated with increased cognitive, expressive language, and motor delays at 2-4 years.
- Antenatal tracheal occlusion and abdominal wall patch repair were linked to motor delays at 4 years.
Conclusions:
- The majority of CDH patients achieve normal neurodevelopment by school age.
- Early cognitive delays are associated with ECMO use, while disease severity and surgical factors impact later development.
- Meticulous surgical techniques and standardized neurological follow-up are vital for optimizing neurodevelopmental outcomes in CDH survivors.
Objective:
To identify the incidence and risk factors for neurodevelopmental delay in a longitudinal cohort of children with congenital diaphragmatic hernia (CDH) with and without support with extracorporeal membrane oxygenation (ECMO).
Study Design:
We prospectively followed until March 2022 those children who underwent open CDH repair between 2009 and 2018 at a single institution. Standardized assessments of cognitive, language, and motor development were conducted from 6 months to 6 years of age. Delays were defined using SD thresholds. Univariable and multivariable logistic regression analyses identified risk factors for neurodevelopmental delay.
Results:
Of 278 patients (130 ECMO, 148 non-ECMO), 784 assessments were completed. Cognitive delays were more common in the ECMO group; language and motor outcomes were similar. Most delays were mild. Most children had no delays in any domain by 6 years of age. In multiple regression analyses, risk factors for delays varied at different timepoints. Reoperation during the first year was negatively associated with 2-4-year cognitive function (OR 3.5, 95% CI: 1.3-9.8), expressive language (OR 2.7, 95% CI: 1.0-7.2), and motor delays (OR 2.4; 95% CI: 1.1-5.1). At 4 years, motor delays were associated with antenatal fetoscopic endoluminal tracheal occlusion (OR 4.9, 95% CI: 1.2-20.1) and abdominal wall patch (OR 4.8, 95% CI: 1.7-13.9).
Conclusions:
Most children with CDH achieved normal neurodevelopment by school age. ECMO use was associated with early cognitive delays. Disease severity and surgical factors were associated with increased risk of delays, suggesting that meticulous surgical technique may protect neurodevelopmental outcomes. Timely support and standardized neurological follow-up may help to prevent or reduce long-term problems.
