[Pulmonary hypoplasia: An analysis of cases over a 20-year period]

Abstract

Introduction: Pulmonary hypoplasia is the most frequent congenital anomaly associated with perinatal mortality.

Material and methods: A retrospective and descriptive review was conducted on cases of patients diagnosed with pulmonary hypoplasia between 1995 and 2014 in a tertiary university hospital. An analysis was made of the prenatal imaging, clinical manifestations, post-natal diagnostic tests, treatment and management, long-term follow up, and survival data.

Results: A total of 60 cases were identified, all of them with prenatal imaging. Sixteen patients required foetal surgery. Congenital diaphragmatic hernia was the most frequent diagnosis. Main clinical presentation was respiratory distress with severe hypoxemia and high requirements of mechanical ventilation. Mortality rate was 47% within first 60 days of life, and 75% for the first day of life. Pneumonia and recurrent bronchitis episodes were observed during follow-up. They had a lung function obstructive pattern, and their quality of life and exercise tolerance was good.

Conclusions: High neonatal mortality and significant long-term morbidity associated with pulmonary hypoplasia requires an early diagnosis and a specialised multidisciplinary team management.

Keywords: Agenesia pulmonar; Congenital diaphragmatic hernia; Hernia diafragmática congénita; Hipoplasia pulmonar; Lung hypoplasia; Pulmonary agenesis; Pulmonary hypoplasia.