Congenital Cystic Adenomatoid Malformation of the Lung: Antenatal Ultrasound Findings and Fetal-Neonatal Outcome

Abstract

Seventeen cases of congenital cystic adenomatoid malformation of the lung (CCAM) are reported. They were followed up over a period of 1 month to 15 years. Diagnosis was made by prenatal ultrasound. Our purpose was to evaluate the fetal-neonatal outcome and the prognostic elements observable through ultrasound techniques, and to compare all types of CCAM. The outcome observed ranged from total prenatal resolution to postnatal spontaneous regression of the lesion, to complications due to the presence of nonimmune fetal hydrops (NIFH), intrauterine death and the necessity of surgical intervention. In our experience only hydrops represented a negative predictor of outcome since death occurred in all cases with this pathology. In the absence of NIFH, counselling should stress the prevalence of a positive outcome, even in cases of surgical intervention.

Genetic Analysis of Congenital Cystic Adenomatoid Malformation Reveals a Novel Pulmonary Gene: Fatty Acid Binding Protein-7 (Brain Type)

“…Some lesions regress in utero with no residual abnormality at birth (1)(2), others are associated with fetal polyhydramnios, mediastinal shift, and nonimmune hydrops fetalis resulting in the risk of fetal demise (3). Mediastinal shift and hydrops fetalis are indications for fetal intervention.…”

Presumptive Intrahepatic Congenital Cystic Adenomatoid Malformation

“…[16][17][18][19] In our experience, resection of the lung tumor has a greater than 50% fetal survival rate. A large study by Adzick et al 16 of 134 cases of CCAM supports our experience.…”

Diagnóstico ecográfico prenatal y resultados neonatales de la malformación adenomatoidea quística pulmonar

“…Según la EUROCAT (European Surveillance of Congenital Anomalies) la prevalencia de las MAQ es de 0.87/10.000 nacimientos con una prevalencia mayor en varones 18,19 . En nuestra serie el 71% de los nacidos presentó un resultado favorable en la evolución postnatal, el peor pronóstico correspondió a las MAQ tipo III.…”

“…Se trata de una patología congénita pulmonar que se podría detectar ecográficamente desde las 12 semanas 18 , sin embargo, la edad gestacional media del diagnóstico por ultrasonido es alrededor de las 20 semanas 20 , como se muestra en nuestros datos. Un metaanálisis del grupo del King´s College resume experiencia propia, europea y mundial, reportando supervivencia del 97,2%, resolución prenatal en el 29,6% y cirugía postnatal en el 62,7% de un total de 486 fetos con MAQ y sin hidrops 21 .…”