Aim: In this study, we analyze our results in the treatment of congenital diaphragmatic hernia (CDH) using conventional ventilatory therapy without ECMO.
Patients and methods: fifty one CDH patients (27 males and 24 females) treated at our institution between 1997 and 2004 were reviewed. Data referred to obstetrics, prenatal diagnosis, newborn care, type of hernia and surgical treatment were analyzed. We recorded ventilatory treatment modalities and the outcome of the patients. We also compared the survival of our series with those expected using the formula proposed by the CDH study group in 2001. We finally analized separately those patients with early clinical presentation and who fulfilled ECMO criteria. Data from necropsies were also recovered when available.
Results: Prenatal diagnosis was made in 58% of the patients. Fifty nine percent were born by c-section. The diaphragmatic defect was left-sided in 42 patients, right in 8 and bilateral in 1. Ten patients needed a prosthetic patch to close the defect. Eighteen out of the 51 patients (35%) died, 11 of them without surgical treatment. Early presentation of clinical picture was evident in 44 patients; among them 46% required high frequency ventilation and 53% nitric oxide therapy. Medium age at operation was 56+/-49 hours. The 7 children with late clinical presentation are alive. Among the 44 remaining patients, 26 are also alive (59,09%), data similar to those expected by the formula (62.39%, p>0.05). Fifteen patients had oxigenation index (IO) over 40, with a stimated survival rate of 48%, a statistically significant lower rate when compared to patients with IO<40. None of the patients with IO>40 survived; in 4 out of the 7 available necropsy studies, a severe lung hypoplasia was found (index lung weight/body weight <0.006). Among the remaining 27 children with IO<40, 24 (90%) are alive.
Conclusions: A survival rate over 90% can be achieved by conventional ventilatory measures in patients with IO<40. In our experience, children with IO>40 are rarely candidates to ECMO therapy because of the associated severe lung hypoplasia confirmed by necropsy studies.