Effect of intrauterine repair of diaphragmatic hernia on the accompanying pulmonary hypoplasia in the fetal rabbit.

Severe pulmonary hypoplasia precluding the sustenance of life is often found in newborns with prenatally diagnosed congenital diaphragmatic hernia (CDH). In utero repair of the hernia it is thought to be the sole method of salvaging these patients. To study the efficacy and feasibility of in utero repair of CDH, diaphragmatic hernias (DH) were produced successfully in 81 of 90 fetal rabbits by diaphragmatic perforation via a left thoracotomy at 22 days' gestation (term = 31 days). The DHs were repaired successfully in 25 of 50 fetal rabbits at 26 days' gestation. The rabbits with repaired and non-repaired DHs and their litter-mates (the control group) were delivered at 29 days' gestation by cesarean section. Some of the rabbits were killed and subjected to measurements of body and lung weight, determination of the DNA and surfactant (disaturated phosphatidylcholine; DSPC) contents of the lungs, and light and electron microscopic examination of the lung. Some newborn rabbits underwent endotracheal intubation and measurement of pressure-volume curves and pulmonary compliance. The total lung/body weight ratios and total lung DNA contents in the repair group were greater than those in the non-repair Group (P <0.01). There were no differences among the three groups in regard to body weight. When compared with the control group, both the repair and non-repair groups had increased DSPC content (P <0.01 andP <0.05, respectively), although there was no difference between the repair and non-repair groups. Histologically, the thickness of the terminal air spaces was smaller and the size of the lung acini was larger in the repair group than the non-repair group. Electron-microscopically, the number of type 11 lung cells in both the repair and nonrepair groups tended to be larger than that in the control group. When compared with the non-repair group, the repair group showed increased values for pressure-volume curves (P <0.01) and pulmonary compliance (P <0.01). In conclusion, in utero repair of CDHs is effective in improving the hypoplasticity of the lung accompanying this lesion.