Abstract

Aim: Congenital posterolateral diaphragmatic hernia is an anomaly with a mortality rate ranging from 10% to 35% with the latest developments. The aim of our study is to determine the prognostic factors affecting survival in patients with congenital diaphragmatic hernia and evaluate their effects on lung development using ventilation-perfusion scintigraphy.

Material and Methods: The demographic findings, possible prognostic factors and operation outcomes of 55 patients who were operated due to congenital posterolateral diaphragmatic hernia in the Pediatric Surgery Clinic of Baskent University Ankara Hospital and Baskent University Adana Application and Research Center between January 2000-April 2010 and the results of pulmonary ventilationperfusion scintigraphies performed during infancy of 19 of these patients were retrospectively investigated.

Results: The mortality rate of our patients diagnosed with congenital diaphragmatic hernia is %20. The following factors were determined to negatively affect prognosis: Postmaturity (p=0.013), right sided congenital diaphragmatic hernia (p=0.042), defect diameter ≥4 cm (p=0.015), 5^th minute APGAR score <5 (p=0.001), liver herniation in left congenital diaphragmatic hernia (p=0.018), cardiac comorbidities (p=0.048), concomitant pulmonary hypertension (p=0.001), low basal, preoperative and postoperative blood gas pH values (p=0.024, p=0.001, and p=0.000, respectively), high basal, preoperative and postoperative blood gas PaCO₂ values (p=0.014, p=0.001, and p=0.000, respectively). The pulmonary ventilation-perfusion scintigraphy analysis of the patients revealed decreased ipsilateral pulmonary perfusion and ventilation and increased contralateral pulmonary perfusion and ventilation. The ipsilateral pulmonary ventilation of males was significantly lower than that of females (p=0.042) and the usage of patches was found to negatively affect ipsilateral pulmonary perfusion (p=0.039) and ventilation (p=0.039). In left congenital diaphragmatic hernia patients with stomach herniations, pulmonary perfusion was found to decrease ipsilaterally (p=0.014) and increase contralaterally (p=0.014) with respect to the defective side. The contralateral pulmonary ventilation was found to increase in CDH patients with liver herniations (p=0.013).

Conclusion: Upon evaluation of our congenital diaphragmatic hernia cases within 10 years, postmaturity, right-sided defect, defect diameter ≥4 cm, 5^th minute APGAR score <5, liver herniation in the left congenital diaphragmatic hernia, cardiac comorbidities, concomitant pulmonary hypertension, acidosis and hypercapnia were found to negatively affect prognosis. In surviving patients, male gender, patch usage and stomach and liver herniations in the left congenital diaphragmatic hernia were determined to increase pulmonary hypoplasia. Since ambulatory follow-ups of surviving CHD patients reflect the changes in CHD during infancy permanent changes can be determined using ventilation-perfusion.