Expansion thoracoplasty improves respiratory function in a rabbit model of postnatal pulmonary hypoplasia: a pilot study.

STUDY DESIGN: Using a rabbit model of postnatal pulmonary hypoplasia, we investigated how expansion thoracoplasty affected growth of the spine and lungs. OBJECTIVE: By constricting the hemithorax in a growing rabbit to create postnatal pulmonary hypoplasia, we quantified how expansion thoracoplasty affects lung volume, respiratory function, alveolar morphology, and spine growth. SUMMARY OF BACKGROUND DATA: In children with thoracic insufficiency syndrome, expansion thoracoplasty of the constricted hemithorax improves respiratory function and controls scoliosis. We hypothesize that expansion thoracoplasty of the constricted hemithorax improves pulmonary hypoplasia by allowing the lung to expand, improving respiratory function and stimulating lung growth. METHODS: Postnatal pulmonary hypoplasia and scoliosis were induced in 5-week old rabbits by constricting left ribs 3 to 8. Expansion thoracoplasty through the fused ribs was performed at 10 weeks. These were compared with Disease rabbits allowed to grow with a constricted left hemithorax and Normal rabbits. Spine and thoracic deformity, right and left lung volumes were measured on reconstructed 3-dimensional computed tomography images and functional residual capacity measured by plethysmography. At maturity, lungs were excised and quantitative histology performed to measure alveolar air fraction and surface density. RESULTS: Expansion thoracoplasty of the constricted left hemithorax improved scoliosis but increased left lung volumes only moderately compared with Disease rabbits. For Disease and Thoracoplasty Treated rabbits, a compensatory increase in the volume of the right lung maintained total lung volumes equivalent to Normal. Alveolar air space fraction was greater in Disease rabbits, suggestive of emphysema improved by expansion thoracoplasty. Capillaries adjacent to the alveoli were prominent in Thoracoplasty Treated rabbits. CONCLUSION: Expansion thoracoplasty reduces scoliosis and increases the volume of the constricted hemithorax, but the relative increase in the ipsilateral lung volume is small since compensatory hypertrophy of the contralateral lung also occurred. Expansion thoracoplasty may improve respiratory function by increasing alveolar capillaries and preventing emphysematous changes.

Predicting inadequate long-term lung development in children with congenital diaphragmatic hernia: an analysis of longitudinal changes in ventilation and perfusion.

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.