Congenital cystic adenomatoid malformation: is there a difference between the antenatally and postnatally diagnosed cases?

Abstract

No significant difference was found between the two groups. Recognition of these lesions antenatally would benefit patients by avoiding delay in making the diagnosis, which can lead to serious complications. CT was successful in accurately diagnosing and grading CCAM lesions.

Malformações pulmonares congênitas

“…(32) One group of authors (39) conducted a detailed study of CCAMs based on 38 cases and divided CCAMs into 3 types, on the basis of the histological characteristics of the malformations. Initially, CCAMs were classified as types I, II, and III, on the basis of the size of their cysts and histological characteristics, (4,5,15,28,30,31) the frequency for types I, II, and III being 50-70%, 20-40%, and approximately 10%, respectively. (32) Subsequently, two other types of CCAM, types 0 and IV, were added to the classification.…”

“…Of all congenital lung malformations, 25-30% are CCAMs, (15) and approximately 30% of all patients with CCAM are at risk of respiratory failure at birth. (27) It has been shown that CCAMs are hamartomatous lesions, (27) with focal dysplasia and anomalous development, (4,28) and are characterized by a multicystic mass of lung tissue with proliferation of bronchial structures and lung tissue showing aberrant, differentiated architecture, with various degrees of cyst formation. (5,26,(29)(30)(31)(32) The possible mechanisms by which the lesion develops and the exact period of time over which it develops have yet to be determined; however, there is evidence that CCAMs occur between gestational weeks 5 and 22 and result in a wide variety of pathological and radiological presentations.…”

“…(15,29,33,37) Patients with symptomatic CCAM can present with a history of recurrent pneumonia, cyanosis, respiratory difficulty, and pneumothorax. (15,28) In the prenatal phase, surgical intervention is still the best treatment option for the microcystic forms, which are associated with hydrops fetalis. However, there are certain contraindications to surgery, including chromosomal abnormalities, multiple pregnancy, presence of other anatomical abnormalities, and presence of maternal or psychosocial factors.…”

“…(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.…”

“…(34) When such associations are diagnosed, the prognosis is poor and fetal intervention or even pregnancy termination can therefore be indicated. (29) Most individuals with CCAM (90%) are asymptomatic in the neonatal period (28) and over long periods of their lives; however, in any given phase of their lives, they can present with symptoms that are suggestive of pulmonary infection. Approximately 30% of cases are diagnosed in the postnatal period, (28) and 15-50% of cases can resolve in the neonatal The histology of congenital lobar emphysema is generally normal, with mild alveolar dilation, without malformation or destruction (5) of alveolar septa.…”