Congenital diaphragmatic hernia (CDH) is a severe developmental anomaly associated with high mortality and long-term morbidity. Right-sided defects (RCDH) are less common than left-sided (LCDH) with variable outcomes and survival rates reported in the literature that are more frequently worse than for infants with left hernias.
RCDH has been associated with increased severity of pulmonary hypoplasia and increased incidence of pulmonary hypertension. Long-term respiratory, surgical and musculoskeletal morbidity also appears greater in infants with RCDH that tend to have a higher proportion of larger defects. Laterality alone is not an independent predictor of survival but gestational age, being small for gestation, the size of the defect and antenatal assessment of the fetal lung size have been identified as independent predictors of survival. Fetoscopic endoluminal tracheal occlusion (FETO) in the antenatal period improves survival rates in infants with severe left or right CDH and seem to improve the morbidity associated with right-sided defects but it remains an invasive procedure with potential complications. Ongoing studies aim to reduce FETO-associated complications and to develop adjunct therapies, such as stem cells or sildenafil, that could promote fetal lung growth and maturation and target pulmonary hypertension.
As mortality and morbidity of CDH survivors remain high and till results from the above studies become available, following a structured antenatal and postnatal management of CDH patients with standardised follow up of survivors could help improve neonatal outcomes.
Congenital diaphragmatic hernia, Survival, Laterality, Outcome