Malformación adenomatoidea quística congénita

Abstract

Congenital cystic adenomatous malformationIntroduction: Congenital Cystic Adenomatous Malformation (CCAM) is an infrequent entity due to an alteration in alveolar-pulmonary development. Material and Methods: A descriptive, retrospective study of newborns presenting CCAM in a tertiary care hospital in Madrid, Spain. Results: Seven patients were found. All were full term, normal weight births. Two patients showed respiratory distress at birth. Two chest x-rays were normal. CT scans showed three clear CCAM lesions, four suggestive of hybrid lesions. All were referred to medical centers with pediatric surgery for followup. Discussion: At birth, this pathology may be asymptomatic and appear as a chest x-ray fi nding. Long term management is complicated by infection and malignization. Conservative treatment of asymptomatic patients includes regular follow up of lesions. Surgical treatment is reserved for symptomatic or complicated patients. Conclusions: Prenatal suspicion of CCAM is important since clinical exam and radiology may be normal in neonatal period. Chest CT scans are important in confi rming diagnosis and determining future surgery. More studies are necessary for the proper diagnosis and management of this disorder.

Benign cystic adenomatoid malformation: A case report and literature review

“…Malformations are related to cardiovascular abnormalities in 21% of patients (persistence of the ductus arteriosus, dextrocardia, and among others), rib cage malformations in 16% of patients (pectus excavatum), and other malformations in 5% of patients. These include pyloric stenosis, pulmonary hypoplasia, diaphragmatic hernia, hydrocephalus, jejunal atresia, and Pierre-Robin syndrome that consist of micrognathia and glossoptosis and accompanied by a cleft lip and palate, as well as lesions of the hard and soft palate, high-arched palate, or the presence of hemi-vertebrae 7,12,13 . It is suggested that for cases of adenoid cystic malformation, genitourinary disorders should be investigated due to the possibility of association with alterations at this level 12,13 .…”

“…These include pyloric stenosis, pulmonary hypoplasia, diaphragmatic hernia, hydrocephalus, jejunal atresia, and Pierre-Robin syndrome that consist of micrognathia and glossoptosis and accompanied by a cleft lip and palate, as well as lesions of the hard and soft palate, high-arched palate, or the presence of hemi-vertebrae 7,12,13 . It is suggested that for cases of adenoid cystic malformation, genitourinary disorders should be investigated due to the possibility of association with alterations at this level 12,13 . As for the differential diagnosis in children, diseases such as diaphragmatic hernia, where the presence of abdominal viscera is found in the thoracic space, pulmonary sequestration that can be differentiated from CCAM, because pulmonary sequestration receives the blood supply from an accessory artery of the aorta, which can observed by Doppler ultrasound.…”

“…Classification of congenital cystic adenomatoid malformation5,12 Type I One or more cysts of 2 cm or more, lined with ciliated pseudostratified columnar epithelia, surrounded by elastic tissue, smooth muscle bands, fibrovascular membranes, and cartilage. Alveoli with normal characteristics between the cystic structures.…”

Malformación congénita de la vía aérea pulmonar tipo I: dos formas clínicas de presentación

“…Representan aproximadamente un 10% de todas las malformaciones congénitas, siendo las más frecuentes aquellas que alteran el árbol bronquial, el parénquima pulmonar y las estructuras vasculares. 8 Las lesiones quísticas congénitas del pulmón están presentes en 1 de cada 10,000-35,000 nacimientos y se presentan como un espectro de anomalías. La mayoría comprenden malformaciones adenomatoideas quísticas congénitas, secuestros pulmonares, enfisema lobar congénito y quistes broncogénicos.…”

Manifestaciones radiológicas de malformaciones pulmonares congénitas. Experiencia de tres hospitales en Bogotá

“…La malformación adenomatoidea quística pulmonar (MAQP) es una masa pulmonar congénita de causa desconocida, que resulta de una proliferación hamartomatosa desordenada y adenomatoide de bronquiolos primarios que están en comunicación con el árbol bronquial, y recibe vascularización de la circulación pulmonar (3,4). Según la literatura, los síntomas pueden ser tos, disnea, hemoptisis, dificultad respiratoria o, incluso, los pacientes pueden permanecer asintomáticos; las manifestaciones típicas son dificultad respiratoria en el recién nacido o infecciones respiratorias a repetición en niños mayores.…”