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Pulmonary sequestration: an experience in a level III hospital

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Resumo: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.
Assunto:Pulmonary sequestration; Neonatal Intensive Care Unit; pulmonary malformation; lung; prenatal diagnosis; newborn
País:Portugal
Tipo de documento:journal article
Tipo de acesso:Aberto
Instituição associada:Repositório Aberto da Universidade do Porto
Idioma:inglês
Origem:Repositório Aberto da Universidade do Porto
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conditionsOfAccess_str open access
country_str PT
description 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.
documentTypeURL_str http://purl.org/coar/resource_type/c_6501
documentType_str journal article
id 8bc343f1-4b25-4df9-b734-198c6db60747
identifierHandle_str https://hdl.handle.net/10216/154160
language eng
relatedInstitutions_str_mv Repositório Aberto da Universidade do Porto
resourceName_str Repositório Aberto da Universidade do Porto
spellingShingle Pulmonary sequestration: an experience in a level III hospital
Pulmonary sequestration; Neonatal Intensive Care Unit; pulmonary malformation; lung; prenatal diagnosis; newborn
title Pulmonary sequestration: an experience in a level III hospital
topic Pulmonary sequestration; Neonatal Intensive Care Unit; pulmonary malformation; lung; prenatal diagnosis; newborn