Elsevier

Journal of Pediatric Surgery

Volume 45, Issue 9, September 2010, Pages 1759-1766
Journal of Pediatric Surgery

Original article
Neurodevelopmental outcome of infants with congenital diaphragmatic hernia prospectively enrolled in an interdisciplinary follow-up program

https://doi.org/10.1016/j.jpedsurg.2010.03.011Get rights and content

Abstract

Purpose

The purpose of the study was to evaluate the neurodevelopmental outcome in infants with congenital diaphragmatic hernia (CDH).

Methods

Between June 2004 and September 2007, 41 CDH survivors were prospectively enrolled in an interdisciplinary follow-up program. Neurodevelopmental status was evaluated using the Bayley Scales of Infant Development II (prior 2006, n = 9), the Bayley Scales of Infant Development III (after 2006, n = 27), or the Wechsler Preschool and Primary Scale of Intelligence III (children older than 4 years, n = 5). Scores were grouped as average, mildly delayed, and severely delayed by standard deviation intervals (115-85, 71-84, <70), and mixed if average and mildly delayed in either cognitive or language.

Results

Median age at last assessment was 24 months (range, 6-62). Average, mixed, mildly delayed, and severely delayed scores for neurocognitive and language skills were found in 49%, 19%, 17%, and 15%, respectively. Psychomotor scores were normal, mildly delayed, and severely delayed in 46%, 23%, and 31%, respectively. Autism was present in 7%. Abnormal muscle tonicity was found in 51% (49% hypotonic, 2% hypertonic). Multivariate risk factors for borderline or delayed neurodevelopmental, neurocognitive, and/or psychomotor outcome were intrathoracic liver position (P = .02), presence of a right-sided CDH (P = .02), extracorporeal membrane oxygenation need (P < .001), Gore-Tex patch repair (P = .02), O2 requirement at 30 days of life (P < .01), and hypotonicity (P < .01).

Conclusions

The prospective evaluation in an interdisciplinary follow-up program uncovered striking morbidities in neurodevelopmental status in approximately half of the CDH infants. The most common neurologic sequelae are neuromuscular hypotonicity and psychomotor dysfunction. Patient-specific factors are important determinants of adverse neurologic outcome.

Section snippets

Material and methods

This study was approved by The Children's Hospital of Philadelphia Institutional Review Board, Committee for Protection of Human Subjects (2004-5-3779). Informed consent was obtained from the parents or legal guardians.

Patient population

During the study period, overall, 52 CDH survivors were enrolled in our follow-up program. Of those, 41 (79%) infants were at least 6 months and had returned for detailed neurodevelopmental assessment. Patient characteristics and neonatal hospital course are summarized in Table 1. Gestational age at birth was greater then 36 weeks in 34 (83%) children, between 35 and 36 weeks in 2 (5%), and less then 34 weeks in 5 (12%).

Neurodevelopmental outcome

Each child's most recent neurodevelopmental and neurologic assessment (ie,

Discussion

As technology and sophistication of both neonatal and surgical care (eg, permissive hypercapnia, lung-preserving ventilation strategies, high-frequency ventilation, ECMO) have advanced, survival rates of more severely affected CDH children (liver up and an LHR <1.0) have improved [16]. Although improving short-term morbidity and mortality is an important goal, more recent and ongoing research has been focused on neurodevelopmental outcome as an equally important primary outcome in this

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    Presented in part at the 39th Annual Meeting of the American Pediatric Surgical Association in Phoenix, AZ, May 2008.

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