Congenital adenomatoid disease of the lung: prenatal diagnosis and perinatal management

Abstract

Prenatal ultrasonographic (US) detection of congenital adenomatoid malformation (CAM) was made in 18 fetuses at 17 - 36 weeks' gestation and managed in our institution during a 10-year period (1985-1994). The lesion was left-sided in 13 cases, right-sided in 4, and bilateral in 1. According to Stocker's classification, 12 cases were type I, 4 type II, and 2 type III. The prenatal course was followed with serial US examinations in 13 cases; the size of the lesion was stable in 8 and decreased in 5. Mediastinal shift was usually observed, and amniotic fluid volume was increased in 4 cases. One fetus was aborted. Six infants presented with respiratory distress syndrome and required neonatal surgery; delayed surgery was performed in 9 cases. Spontaneous regression of the lesion was observed on follow-up in 2 cases. Surgery consisted in lobectomy in 8 cases and segmentectomy in 6. The presence of fetal hydrops, type III lesions, and bilateral lung involvement are prenatal factors known to be associated with a poor prognosis. However, this series and a review of the literature suggest that caution should be observed with regard to the initial impression when counseling the parents regarding prognosis.

Malformações pulmonares congênitas

“…(15,21) The combination of CCAM and certain other malformations, such as pulmonary sequestration, has been demonstrated in various studies, raising the hypothesis that type II CCAM and extralobar pulmonary sequestration have the same embryonic origin. (13,15,18,20,27,30,34,35) However, it is much less common for CCAM to be accompanied by malformations such as facial defects, heart defects, neural tube defects, renal dysplasia/agenesis, and omphalocele. (15,27,34) The lesions vary widely in size and can affect an entire lobe or part of it, as well as an entire lung.…”

“…(13,15,18,20,27,30,34,35) However, it is much less common for CCAM to be accompanied by malformations such as facial defects, heart defects, neural tube defects, renal dysplasia/agenesis, and omphalocele. (15,27,34) The lesions vary widely in size and can affect an entire lobe or part of it, as well as an entire lung. The lesion can affect both sides of patients with equal frequency and can affect any lung lobe, although it occurs more frequently in the lower lobes and rarely affects more than one lobe (85-95% in only one lobe; Figure 3), being slightly more common in males than in females.…”

“…(15,26) In patients with CCAM, there is a broad spectrum of variation in terms of the evolution of the condition, which can be as severe as perinatal death (from hydrops fetalis or pulmonary hypoplasia) or as mild as asymptomatic lesions or spontaneous resolution of symptomatic lesions. (22,27,33,34) The most common symptoms are recurrent infections, (4,27,35) and there have been reports of malignant transformation (to of all CCAMs, (5,15,32,39) and can affect an entire lung or, more rarely, both lungs. (5) The cut-off surface of type III CCAM is firm and generally does not present with cystic lesions; when such lesions are present, they are no larger than 0.5 cm in diameter.…”

“…In symptomatic patients, there is consensus that the lesion must be resected, generally by means of pulmonary lobectomy, which avoids residual disease and recurrence in the remaining tissue. (33,34,37,38) One group of authors reported that only 10% of the asymptomatic patients will develop complications and that a conservative approach is an alternative for such patients. (44) Resection of the area of the lesion exclusively is not recommended.…”

“…(27,32) For the diagnosis of CCAM, a CT scan of the chest is mandatory; it determines the size of the cysts, reveals associated anomalies, defines the anatomical extent of the malformation more accurately, and reveals lesions that, on chest X-rays or ultrasound of the chest, appear to be resolved. (27,28,(32)(33)(34) Studies have shown that CCAM can be accompanied by hydrops fetalis and hemodynamic disturbance caused by vascular compression of intrathoracic structures. (4,38) Doppler echocardiography is used to characterize polyhydramnios or hydrops fetalis, as well as allowing the evaluation of lung involvement, mediastinal shift, and associated pulmonary hypoplasia.…”