Pathogenesis of congenital cystic adenomatoid malformation of the lung

Moerman, Philippe; Fryns, Jean‐Pierre; Vandenberghe, Kamiel; Devlieger, Hugo; Lauweryns, Joseph M.

doi:10.1111/j.1365-2559.1992.tb00401.x

Cited by 95 publications

(73 citation statements)

References 13 publications

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“…Pre-or postnatal total regression of the lesion was observed in 64.3% of cases, a possibility in all 3 types of CCAM even if it is more common in type II. This appears to be explained by a transitory obstruction of the upper respiratory tract or by a compensatory growth of space-occupying healthy lung tissue around the lesion [5,6,18]. A prenatal reduction in the dimensions of the lesion, however, does not necessarily indicate that no surgical intervention is required, as was experienced in 2 of the 4 neonates operated (1 case of type-I and 1 of type-III CCAM).…”

Section: Discussionmentioning

confidence: 98%

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

Santis

Masini²,

Noia³

et al. 2000

Fetal Diagn Ther

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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.

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Section: Discussionmentioning

confidence: 98%

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

Santis

Masini²,

Noia³

et al. 2000

Fetal Diagn Ther

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“…Its etiology and pathogenesis remain unknown. It may occur as a sporadic nonhereditary lesion or in association with certain genetic syndromes such as trisomy 18 and hereditary renal dysplasia [4]. Several factors are involved in lung development and a malfunction of any of them may produce a congenital anomaly such as CCAM.…”

Section: Introductionmentioning

confidence: 99%

Late-Onset Congenital Cystic Adenomatoid Malformation of the Lung

Luján

Bosque²,

Mirapeix³

et al. 2002

Respiration

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Background: Congenital cystic adenomatoid malformation of the lung (CCAM) is an embryonic developmental anomaly of an unknown etiology usually diagnosed antenatally by imaging techniques. A minority of cases may not be identified by prenatal imaging techniques and may go unnoticed for the first 6 months of their extrauterine life. Due to its rarity, physicians are unlikely to suspect the condition. Objectives: To highlight the embryology, clinical symptomatology, diagnostic procedures, therapeutic approach and clinical follow-up of a series of 12 patients with late-onset CCAM. Methods: An observational study which offers the description of the clinical presentation, diagnostic methods, treatment and follow-up of 12 patients with late-onset CCAM. Setting: A 600-bed teaching hospital in a reference area of 350,000 inhabitants. Patients: 12 patients from 1983 to 1999. Results: Twelve diagnosed cases of late-onset CCAM. Mean age at diagnosis: 6.7 years (range: 6 months to 23 years). Clinical presentation: 9 out of 12 (75%) with repeated lung infections, 2 out of 12 (16%) chance finding, and 1 case (8%) with pneumothorax. On pathological examination, 7 were found to be CCAM type I and 4 CCAM type II according to Stocker’s classification; 1 patient is currently awaiting surgery. The diagnostic method of choice nowadays is a computed tomography (CT) scan performed in the 7 more recent cases; in the former 5 cases an isotopic lung scan was done (and in 2 of them a bronchography was also performed). Treatment: 11 patients were operated: 8 lobectomies, 2 segmentectomies and 1 localized resection. Mean follow-up: 8 years (range: 6 months to 16 years). Complications: One reintervention due to a reappearance of the lesion in the patient who underwent localized resection of the CCAM. No cases of malignancy were found. Conclusions: Late-onset CCAM is an infrequent illness which requires a high level of clinical suspicion. It usually presents in the form of repeated infections. The most frequent pathological forms are type I and II (Stocker). The diagnostic method of choice is the CT scan. The recommended treatment is radical surgery of the lesion once diagnosis has been established. Malignancy and relapses are very infrequent when radical surgery is not postponed.

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“…CCAM results from a branching abnormality in lung development and is comprised of cystic and adenomatoid overgrowth of terminal bronchioles and airspaces accompanied by lack of communication between the lesion and the tracheobronchial tree due to an absent or atretic segmental bronchus. Pathogenesis is thought to involve localized defi ciency in the bronchopulmonary budding and branching process, causing bronchial atresia and possible complete involution [13] .…”

Section: Discussionmentioning

confidence: 99%

Congenital Cystic Adenomatoid Malformation in the Fetus: A Hypothesis of Its Development

Kulwa

Tharakan²,

Baxi³

2005

Fetal Diagn Ther

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We present a case of congenital cystic adenomatoid malformation of the lung diagnosed at 34 weeks of gestation in the setting of polyhydramnios. The fetus had CCAM in the L lung, with mediastinal shift to the right and ascites. The neonate underwent drainage of cysts and subsequent left lung lobar resection with improvement in respiratory function. The pathology of CCAM is reviewed in detail. We speculate the role of alcohol as a teratogen through retinoic acid at 8–10 weeks of gestation when fetal lungs are actively developing.

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Pathogenesis of congenital cystic adenomatoid malformation of the lung

Cited by 95 publications

References 13 publications

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

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

Late-Onset Congenital Cystic Adenomatoid Malformation of the Lung

Congenital Cystic Adenomatoid Malformation in the Fetus: A Hypothesis of Its Development

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