Congenital Diaphragmatic Hernia – The Neonatal Period (Part I)

Abstract

Aim: The aim of the study was to review our experience in the management of newborns with congenital diaphragmatic hernia (CDH). Methods: A retrospective study including all infants with CDH at the Hospital de São João, a center that does not provide ECMO support, for the period from 1997 to 2006. Since 2003, a new treatment protocol has been used. Results: There were 61 newborns (30 male/31 female) with a birth weight of 2800 g (880 – 3770), and a gestational age of 38 weeks (28 – 41); 46 (75 %) were inborn and 42 (69 %) had a prenatal diagnosis of CDH. There were 2 (3 %) chromosomal anomalies, 3 (5 %) with other congenital anomalies and 1 (2 %) with nonimmune hydrops fetalis. The diaphragmatic defect was left sided in 55 (90 %) cases. Corrective surgery was performed in 43 (70 %) patients. New therapies were used: HFOV 13 % (n = 8); inhaled nitric oxide 13 % (n = 8); and sildenafil 7 % (n = 4). We found that systemic arterial hypotension (p = 0.001), the severity of pulmonary hypertension (p = 0.001), prenatal diagnosis (p = 0.006), birth weight (p = 0.022), female gender (p = 0.029), inborn birth (p = 0.030), arterial pH < 7.35 at admission (p = 0.030), right-sided defect (p = 0.033) and pneumothorax (p = 0.033) to be predictive of mortality. The overall survival rate was 43 % (n = 26), and since 2003 this rate has improved to 61 % for term neonates without other congenital or chromosomal anomalies. Conclusions: Our survival rate for infants with CDH has improved over the last ten years, and this improvement is associated with the use of new therapies such as HFOV, inhaled nitric oxide and sildenafil.

Key words

congenital diaphragmatic hernia - inhaled nitric oxide - sildenafil - survival rate

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