Autor(es): Neves, C ; Ramalho, C ; Azevedo, I ; Fragoso, AC ; Rocha, G ; Guimaraes, H
Data: 2019
Identificador Persistente: https://hdl.handle.net/10216/154160
Origem: Repositório Aberto da Universidade do Porto
Assunto(s): Pulmonary sequestration; Neonatal Intensive Care Unit; pulmonary malformation; lung; prenatal diagnosis; newborn
Background: Pulmonary Sequestration (PS) is a rare congenital malformation, with few cases reported. Thus, the prenatal and postnatal natural history of PS and the best management approach have not been fully characterized. The aim of this study is to evaluate the experience with PS in our level III hospital, to improve our practice. Methods: A retrospective review of all cases with PS admitted to our hospital between January 1996 and December 2016 was performed. Results: Fifteen cases of PS were identified. Antenatal ultrasonography evaluation suggested the existence of 3 (20%) PS, 3 (20%) Congenital Pulmonary Airway Malformations (CPAM), 3 (20%) cases with no established diagnosis between CPAM and PS and 1 (6.7%) neuroblastoma. All newborns were admitted to Neonatal Intensive Care Unit and 7 (46.7%) became symptomatic. When postnatal X-ray and/or computer tomography scan were performed, the lesions corresponded to 12 (80%) PS, 1 (6.7%) CPAM, 1 (6.7%) suprarenal mass and 1 (6.7%) congenital diaphragmatic hernia. Eight (53.3%) cases had concomitant abnormalities. An invasive postnatal intervention was performed in 5 of 7 (71.4%) symptomatic patients and in 4 of 8 (50%) asymptomatic ones. Two patients underwent embolization. Surgery was performed in 8 (53.3%) cases (thoracotomy in 3 and thoracoscopy in 5). Respiratory morbidity was reported in 2 asymptomatic cases conservatively managed. Final diagnosis, based on histopathological examination or postnatal imaging when surgery wasn't applied, was: 9 (60%) extralobar sequestrations and 3 (20%) intralobar sequestrations; in 3 (20%) cases, it wasn't possible to confirm the type of lesion. Conclusion: PS was associated to a high rate of congenital abnormalities. Concordance between prenatal and postnatal findings was poor. According to our and other series, treatment of asymptomatic PS is controversial. Nevertheless an elective surgery should be preferred to prevent the risks of an urgent surgery. The outcome was generally good.
