Link: http://www.ncbi.nlm.nih.gov/pubmed/26625967

An Pediatr (Barc). 2015 Nov 25. pii: S1695-4033(15)00405-1. doi: 10.1016/j.anpedi.2015.10.008. [Epub ahead of print]
[Pulmonary hypoplasia: An analysis of cases over a 20-year period].
[Article in Spanish]
Delgado-Pe?a YP1, Torrent-Vernetta A2, Sacoto G2, de Mir-Messa I2, Rovira-Amigo S2, Gartner S2, Moreno-Gald? A2, Molino-Gahete JA2, Castillo-Salinas F2.
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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.

Copyright ? 2015 Asociaci?n Espa?ola de Pediatr?a. Published by Elsevier Espa?a, S.L.U. All rights reserved.

KEYWORDS:
Agenesia pulmonar; Congenital diaphragmatic hernia; Hernia diafragm?tica cong?nita; Hipoplasia pulmonar; Lung hypoplasia; Pulmonary agenesis; Pulmonary hypoplasia
PMID: 26625967 [PubMed - as supplied by publisher] Free full text