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[Adenomatous cystic pulmonary malformations: presentation of 26 cases]
J Canals-Riazuelo1, J Boix-Ochoa, J L Peiro
1Departamento de Cirugía Pediátrica, Hospital Universitario Materno-Infantil Vall d'Hebrón, Barcelona.
Insights
Cystic adenomatoid malformations of the lung (CAM) present with neonatal distress or recurrent infections. Surgical intervention is necessary, with no observed long-term pulmonary function alterations in patients.
Area of Science:
- Pediatric Surgery
- Thoracic Surgery
- Congenital Lung Abnormalities
Context:
- Review of 26 cases of congenital cystic adenomatoid malformations (CAM) treated between 1967-1991.
- Two distinct clinical presentations: neonatal respiratory distress and post-infancy recurrent pulmonary infections.
- Pathologic classification based on embryological development into 3 types.
Purpose:
- To analyze diagnostic imaging value and patterns in CAM.
- To investigate factors influencing disease course based on age of presentation.
- To highlight the importance of differential diagnosis in neonates.
Summary:
- Surgical treatment is indicated for all verified cases of CAM.
- Pathologic study confirmed the necessity of surgical intervention.
- Post-operative follow-up revealed no significant alterations in pulmonary function.
Impact:
- Establishes surgical necessity for CAM.
- Provides insights into diagnostic approaches and differential diagnoses.
- Demonstrates favorable long-term pulmonary outcomes post-surgery.
Abstract:
We present our experience of 26 cases of cystic adenomatoid malformations of the lung (CAM), treated in the Department of Pediatric Surgery in our hospital between 1967-1991. There were two clinical pictures: one neonatal severe respiratory distress and successive repeated pulmonary infection appearing after the patient's first year of life and requiring both urgent diagnosis and treatment. Embryological development determines the pathologic classification of this entity in 3 types. Basic examinations by image are analyzed, bearing in mind their diagnostic value and the patterns they show. After analysing all the conditioning factors, no explanation has been found to the different course that this affectation (< 1 month and > 1 year of age respectively). Normally, neonatal mortality is closely related to other malformations, particularly to cardiovascular ones. Differential diagnosis is very important in the neonatal period, especially with regard to diaphragmatic hernia, lobar emphysema and pulmonary cysts. All these cases have been verified and classified by means of a pathologic study, which has shown the need for surgical operation. In the follow up of the patients no alteration has been noticed in the pulmonary function.