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Predicting inadequate long-term lung development in children with congenital diaphragmatic hernia: an analysis of
Melissa J Hayward1, Virginia Kharasch, Catherine Sheils
1Department of Surgery, Children's Hospital Boston, Harvard Medical School, Boston, MA 02115, USA.
Insights
Congenital diaphragmatic hernia (CDH) survivors often develop lung ventilation-perfusion (V/Q) mismatches. Patch repair surgery significantly increases this risk, highlighting the need for long-term follow-up in affected children.
Area of Science:
- Pediatric Pulmonology
- Neonatal Surgery
- Medical Imaging
Background:
- Infants with congenital diaphragmatic hernia (CDH) exhibit varied lung growth post-birth.
- Pulmonary development in CDH patients is often suboptimal.
- Ventilation-perfusion (V/Q) scintigraphy is a key tool for assessing lung function.
Purpose of the Study:
- To evaluate lung development in children with CDH using sequential V/Q scintigraphy.
- To identify perinatal factors associated with progressive V/Q mismatch in CDH patients.
Main Methods:
- Retrospective review of 137 CDH patients (1990-2005).
- Analysis of sequential V/Q scans in 46 patients.
- Comparison of ipsilateral V/Q quotient changes with sex, patch repair, laterality, gestational age, and ECMO use.
Main Results:
- 61% of patients showed abnormal V/Q scans on last study.
- Patch repair increased risk of ipsilateral V/Q mismatch nearly 7-fold (P < .001).
- Extracorporeal membrane oxygenation had a variable impact; other factors were not significant.
Conclusions:
- Many CDH children experience significant, progressive V/Q mismatches.
- Perinatal factors may indicate underlying pulmonary hypoplasia.
- A distinct subgroup of CDH survivors is at risk and requires ongoing monitoring.
Purpose:
Infants born with congenital diaphragmatic hernia (CDH) demonstrate a wide variability in postnatal catch-up lung growth. The goals of this study were to assess the pulmonary development of children born with CDH using sequential ventilation-perfusion (V/Q) scintigraphy and to identify the perinatal factors that correspond to a progressive V/Q mismatch.
Methods:
The records of 137 patients seen between 1990 and 2005 in a multidisciplinary CDH clinic were reviewed. Changes in the ipsilateral V/Q quotient were compared in 46 patients who had 2 or more studies with the following variables: sex, patch repair, laterality, gestational age, and use of extracorporeal membrane oxygenation. An abnormal V/Q quotient was defined as greater than 1.2 (reference range, 0.8-1.2).
Results:
Abnormal V/Q scans were identified in 28 (61%) of the 46 patients at the time of the last V/Q study. Patients who underwent a patch repair had nearly 7 times the risk (P < .001) of developing an ipsilateral V/Q mismatch. The use of extracorporeal membrane oxygenation had a variable effect on the probability of an abnormal V/Q study finding. No other variable was significant.
Conclusions:
Many children with CDH develop significant and progressive V/Q mismatches. Although some perinatal variables appear to be predictive of this phenomenon, they may simply be surrogates for a greater degree of pulmonary hypoplasia present at birth. This subpopulation of CDH survivors is an identifiable group clearly at risk and thus requires long-term follow-up.
