TY - JOUR
T1 - Hipoplasia pulmonar:
T2 - análisis de la casuística durante 20 años
AU - Delgado-Peña, Yanny Paola
AU - Torrent-Vernetta, Alba
AU - Sacoto, Gabriela
AU - de Mir-Messa, Inés
AU - Rovira-Amigo, Sandra
AU - Gartner, Silvia
AU - Moreno-Galdó, Antonio
AU - Molino-Gahete, José Andrés
AU - Castillo-Salinas, Felíx
N1 - Publisher Copyright:
© 2015 Asociación Española de Pediatría
PY - 2016/8/1
Y1 - 2016/8/1
N2 - 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.
AB - 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.
KW - Congenital diaphragmatic hernia
KW - Lung hypoplasia
KW - Pulmonary agenesis
KW - Pulmonary hypoplasia
UR - http://www.scopus.com/inward/record.url?scp=84949514105&partnerID=8YFLogxK
U2 - 10.1016/j.anpedi.2015.10.008
DO - 10.1016/j.anpedi.2015.10.008
M3 - Artículo
C2 - 26625967
AN - SCOPUS:84949514105
VL - 85
SP - 70
EP - 76
IS - 2
ER -