Abstract
To evaluate the effects of congenital dialphragmatic hemia (CDH) and pulmonary hypoplasia on subsequent lung development, we performed lung Xenon 133 ventilation and M.A.A. - Tc 99m perfusion scintigraphies (LVPS) in 16 patients (pts) 2-3 months of age who had had surgical repair of a left CDH within the first 12 hours of life. 10 of these 16 pts were reevaluated at 1-2 years and again at 5-6 years in 6 of them. On the initial lung scintigraphies, ventilation (V) to the hernia side was decreased in 8/16 pts and Δ[VR-VL] averaged 12.6 ± 13.7. In 9/16 pts, there was a trapping of Xenon 133 at the left lung base. Perfusion (P) to the hernia side was decreased in 11/16 pts and Δ[PR-PL] averaged 26.2 ± 23.2. Correlation between V and P was good : r = 0.70, p < 0.01. After 1 year, V to the hernia side became normal except in 2/10 pts (ΔV = 5.2 ± 4.9), but P to the hernia side remained low in 9/10 pts (ΔP = 21.2 ± 7.9). After 5 years, V was normal in all of the 6 pts studied (ΔV = 2.6 ± 3.1); however P to the hernia side was still reduced in 5/6 pts (ΔP = 18.6 ± 5.4). These results show a progressive inprovemsnt and normalization of V and a persisting reduction of P to the lung of the hernia side, suggesting a primary vascular pulmonary hypoplasia in CDH. LVPS is a unique ncn invasive method that provides quantitative and qualitative informations on P and V, which are very helpful in the follow-up of repaired CDH, during the first years of life when physiologic studies are most difficult to obtain.
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Demarquez, J., Lambert, B., Lamireau, T. et al. LUNG VENTILATION AND PERFUSION SCINTIGRAPHY IN THE FOLLOW-UP OF REPAIRED CONGENITAL DIAPHRAGMATIC HERNIA. Pediatr Res 26, 508 (1989). https://doi.org/10.1203/00006450-198911000-00056
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DOI: https://doi.org/10.1203/00006450-198911000-00056