Cystic Adenomatoid Malformation of the Lung: Prenatal Diagnosis, Prognostic Factors and Fetal Outcome

Abstract

Aim: To evaluate 14 cases of cystic adenomatoid malformation (CAM) of the lung with regard to antenatal management and fetal outcome. Results: Intrauterine shunts were placed in 3 fetuses to avoid pulmonary hypoplasia. Prenatal sonography showed that the lesions had a tendency to resolve in 4 cases. Of the 9 infants who survived, 5 were treated surgically (lobectomy). Conclusions: The antenatal evolution of these lesions is highly variable; the lesions may even resolve spontaneously, therefore the diagnosis of CAM types II and III should be followed by a period of observation. Intrauterine drainage is recommended in cases of CAM type I if unfavorable prognostic factors are encountered.

Prenatal diagnosis and outcome of echogenic fetal lung lesions

“…Attempts at fetal therapy in hydropic fetuses with CAM are summarized in Table 62–4, 6–8, 39, 56, 71, 77, 84–87, 92–105. Essentially, there were 50 cases treated by placement of a thoracoamniotic shunt or thoracentesis; among these, there were 17 (34%) intrauterine or neonatal deaths and 33 (66%) survivors.…”

Outcome of congenital cystic adenomatoid malformation of the lung after antenatal diagnosis

“…Whether to perform surgery on asymptomatic newborns with CCAM remains controversial among pediatric surgeons [12,13]. Some authors suggest early surgical intervention in an effort to prevent episodic infection and malignant transformation [5,13–15]. However, it is reasonable to delay surgery in asymptomatic patients regardless of their chest radiograph results [16] even though resection of pulmonary lesions is associated with a low morbidity and mortality rate [17–19].…”

Chronic inflammation in congenital cystic adenomatoid malformations. An underestimated risk factor?

“…There are no disputes in the literature concerning those infants who are symptomatic at birth and require immediate surgical intervention. In cases of asymptomatic newborns, conservative management is suggested and elective operation recommended, according to different authors, at a variable age ranging from 6 to 18 months of life [2,3], to avoid superimposed infections of the malformed areas and development of occult malignant transformation. Reports of pleuropulmonary blastoma in infants and young children and bronchoalveolar carcinoma in older children and adults, may suggest the appropriateness of such a precocious surgical approach [4,5].…”