Congenital cystic adenomatoid malformation: the experience of a level III Neonatal Intensive Care Unit
Background: Congenital cystic adenomatoid malformation (CCAM) is a rare disease. Because few cases per center are reported each year, there is still uncertainty concerning pathophysiology, natural history of the disease and the best treatment approach. The aim of this paper is to report the experie...
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doaj-5e6e21067ad34f9e8d2161c2be1709122020-11-25T03:23:07ZengHygeia Press di Corridori MarinellaJournal of Pediatric and Neonatal Individualized Medicine2281-06922016-08-0152e050217e05021710.7363/050217339Congenital cystic adenomatoid malformation: the experience of a level III Neonatal Intensive Care UnitNuno Medeiros0Gustavo Rocha1Tiago Henriques-Coelho2Carla Ramalho3Hercília Guimarães4Faculty of Medicine, Porto University, Porto, PortugalNeonatal Intensive Care Unit, Centro Hospitalar de São João, Porto, PortugalFaculty of Medicine, Porto University, Porto, Portugal; Department of Pediatric Surgery, Centro Hospitalar de São João, Porto, PortugalFaculty of Medicine, Porto University, Porto, Portugal; Department of Obstetrics and Gynecology, Centro Hospitalar de São João, Porto, Portugal; Institute of Health Research and Development, Porto University, Porto, PortugalFaculty of Medicine, Porto University, Porto, Portugal; Neonatal Intensive Care Unit, Centro Hospitalar de São João, Porto, PortugalBackground: Congenital cystic adenomatoid malformation (CCAM) is a rare disease. Because few cases per center are reported each year, there is still uncertainty concerning pathophysiology, natural history of the disease and the best treatment approach. The aim of this paper is to report the experience of a level III Neonatal Intensive Care Unit (NICU) in CCAM cases. Methods: We evaluated all cases diagnosed with CCAM admitted to our hospital between January 2000 and December 2014. Results: Twenty-four neonates with prenatal diagnosis of CCAM were identified. The majority of lesions (75%) had microcystic features. Thirteen newborns were admitted to NICU for observation and evaluation. Half of the prenatally diagnosed lesions were confirmed cases of CCAM. The remaining corresponded to 5 cases (20.8%) of in utero spontaneous regression of the lesion, 5 (20.8%) bronchopulmonary sequestration, 1 (4.2%) bronchial atresia and 1 (4.2%) pleuro-pericardial cyst. Two (8.3%) neonates became symptomatic during the neonatal period and surgery was performed in the first week of life. Ten cases remained asymptomatic. Six of these cases (40%) underwent surgery (thoracotomy in 3 cases and thoracoscopy in 3 cases). Respiratory morbidity was reported in 1 symptomatic case and in 1 asymptomatic case that was managed conservatively. No mortality was reported. Histological examination showed definitive features of CCAM (Stocker classification: type I = 1; type II = 4; type III = 2). Conclusion: Management of CCAM in a NICU does not seem necessary in asymptomatic newborns. Concordance of ante-natal and post-natal findings is very variable. Thoracoscopy is a less invasive surgical procedure in the management of these patients. The outcome is good in most patients.https://www.jpnim.com/index.php/jpnim/article/view/402congenital cystic adenomatoid malformation of lungneonatal intensive care unitthoracoscopy |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Nuno Medeiros Gustavo Rocha Tiago Henriques-Coelho Carla Ramalho Hercília Guimarães |
spellingShingle |
Nuno Medeiros Gustavo Rocha Tiago Henriques-Coelho Carla Ramalho Hercília Guimarães Congenital cystic adenomatoid malformation: the experience of a level III Neonatal Intensive Care Unit Journal of Pediatric and Neonatal Individualized Medicine congenital cystic adenomatoid malformation of lung neonatal intensive care unit thoracoscopy |
author_facet |
Nuno Medeiros Gustavo Rocha Tiago Henriques-Coelho Carla Ramalho Hercília Guimarães |
author_sort |
Nuno Medeiros |
title |
Congenital cystic adenomatoid malformation: the experience of a level III Neonatal Intensive Care Unit |
title_short |
Congenital cystic adenomatoid malformation: the experience of a level III Neonatal Intensive Care Unit |
title_full |
Congenital cystic adenomatoid malformation: the experience of a level III Neonatal Intensive Care Unit |
title_fullStr |
Congenital cystic adenomatoid malformation: the experience of a level III Neonatal Intensive Care Unit |
title_full_unstemmed |
Congenital cystic adenomatoid malformation: the experience of a level III Neonatal Intensive Care Unit |
title_sort |
congenital cystic adenomatoid malformation: the experience of a level iii neonatal intensive care unit |
publisher |
Hygeia Press di Corridori Marinella |
series |
Journal of Pediatric and Neonatal Individualized Medicine |
issn |
2281-0692 |
publishDate |
2016-08-01 |
description |
Background: Congenital cystic adenomatoid malformation (CCAM) is a rare disease. Because few cases per center are reported each year, there is still uncertainty concerning pathophysiology, natural history of the disease and the best treatment approach.
The aim of this paper is to report the experience of a level III Neonatal Intensive Care Unit (NICU) in CCAM cases.
Methods: We evaluated all cases diagnosed with CCAM admitted to our hospital between January 2000 and December 2014.
Results: Twenty-four neonates with prenatal diagnosis of CCAM were identified. The majority of lesions (75%) had microcystic features. Thirteen newborns were admitted to NICU for observation and evaluation. Half of the prenatally diagnosed lesions were confirmed cases of CCAM. The remaining corresponded to 5 cases (20.8%) of in utero spontaneous regression of the lesion, 5 (20.8%) bronchopulmonary sequestration, 1 (4.2%) bronchial atresia and 1 (4.2%) pleuro-pericardial cyst. Two (8.3%) neonates became symptomatic during the neonatal period and surgery was performed in the first week of life. Ten cases remained asymptomatic. Six of these cases (40%) underwent surgery (thoracotomy in 3 cases and thoracoscopy in 3 cases). Respiratory morbidity was reported in 1 symptomatic case and in 1 asymptomatic case that was managed conservatively. No mortality was reported. Histological examination showed definitive features of CCAM (Stocker classification: type I = 1; type II = 4; type III = 2).
Conclusion: Management of CCAM in a NICU does not seem necessary in asymptomatic newborns. Concordance of ante-natal and post-natal findings is very variable. Thoracoscopy is a less invasive surgical procedure in the management of these patients. The outcome is good in most patients. |
topic |
congenital cystic adenomatoid malformation of lung neonatal intensive care unit thoracoscopy |
url |
https://www.jpnim.com/index.php/jpnim/article/view/402 |
work_keys_str_mv |
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