Congenital cystic adenomatoid malformation of the lung with bronchial atresia

Cachia, Richard; Sobonya, Richard E.

doi:10.1016/s0046-8177(81)80204-3

Cited by 31 publications

(15 citation statements)

References 9 publications

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“…This theory was initially proposed more than 30 years ago in a case report of a mixed lesion containing CCAM, BPS, and bronchial atresia [3]. Despite this report and others [4][5][6], however, the specific relationship between bronchial atresia and other congenital lung masses has remained largely unexplored. More recently, pathologists have shown that the diagnosis of bronchial atresia often requires special diagnostic techniques, including specimen bronchography and microdissection [5].…”

Section: Discussionmentioning

confidence: 99%

“…Although the distinct pathological features for each of these lesions have been well described, little is known about their underlying pathogenesis [1,2]. It has been suggested that many, if not all of these defects, may represent a continuum of fetal lung anomalies associated with unrecognized airway obstruction [3][4][5][6]. In the present study, we sought to further explore this concept by determining the prevalence of bronchial atresia within these seemingly disparate lesions.…”

mentioning

confidence: 99%

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Bronchial atresia: the hidden pathology within a spectrum of prenatally diagnosed lung masses

Kunisaki

Fauza

Nemes

et al. 2006

Journal of Pediatric Surgery

146

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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

Bronchial atresia: the hidden pathology within a spectrum of prenatally diagnosed lung masses

Kunisaki

Fauza

Nemes

et al. 2006

Journal of Pediatric Surgery

146

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“…Admittedly, it is well reported that bronchial derangements may go unrecognized if not actively sought. 3,9 In addition, it is unclear if the features seen within the parenchyma of fetal lung in cases of bronchial atresia or obstruction may differ from those reported in postnatal lung. Perhaps, at least a brief period of postnatal life is necessary for mucus accumulation and mucocele formation.…”

Section: Discussionmentioning

confidence: 99%

Fetal Pulmonary Malformations: Defining Histopathology

Kreiger¹,

Ruchelli

Mahboubi

et al. 2006

American Journal of Surgical Pathology

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Although classification schemes have sought to categorize congenital cystic lung malformations, studies including the pathology of pulmonary malformations occurring specifically during the fetal period are limited. To better characterize such histopathology, we reviewed a total of 23 fetal lung malformations seen at the Children's Hospital of Philadelphia from 1996 to 2004. Twenty-one of the 23 fetal pulmonary malformations could be categorized into 1 of 3 groups based upon the predominant histologic features present within each lesion. Group 1 (9/21) demonstrated tubular airspaces lined by columnar epithelium. Group 2 (6/21) contained airspaces lined by cuboidal epithelium and surrounded by smooth muscle with abundant interstitial mesenchyme. Group 3 (6/21) showed a mixture of relatively mature-appearing airspaces lined by flattened epithelium and scattered dilated bronchiole-like structures. Cysts were of variable size but in all cases showed a respiratory-type lining. Gestational ages ranged from 21 5/7 to 38 2/7 weeks. Patients in groups 1 and 2 were generally younger than those in group 3; however, morphology did not seem to correlate entirely with normal stages of fetal lung development, and group 2 lesions in particular were the least akin to normal fetal lung. In 4 cases a systemic vascular supply to a lobe of lung was identified, providing evidence that such vasculature is embryonic in origin. The histopathology of fetal lung malformations highlights the variability seen in such lesions at all ages, and it is hoped that continued investigations will provide further insight into these enigmatic lesions.

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“…For over 50 years, there has been controversy with regard to the etiology and pathogenesis of malfonnations of the lung, particularly those of the cystic variety [2.5-10]. An important contribution to this field has been prenatal ultrasound, which has revealed prenatal onset not only of ELS but also of ILS, CCAM, and LE, and which has, additionally, documented their evolution [11], More recent investigations in this area have been important in clarifying the nature of and the relationship among these lesions [2,7,9,10,[12][13][14]. For example, the parenchymal maldevelopment observed pathologically in CCAM also can occur in ELS and ILS [2,6,7,9,10,12,13,15,16].…”

Section: Introductionmentioning

confidence: 99%

Bronchial Atresia is Common to Extralobar Sequestration, Intralobar Sequestration, Congenital Cystic Adenomatoid Malformation, and Lobar Emphysema

et al. 2006

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Congenital cystic adenomatoid malformation (CCAM), intralobar sequestration (ILS), extralobar sequestration (ELS), and lobar emphysema (LE) are well-accepted entities; however, certain findings are common to all, particularly the parenchymal maldevelopment characterizing CCAM. Isolated reports have described bronchial atresia (BA) in some specimens in all 4 entities, but this finding has not been evaluated in a prospective manner. With the aid of a dissecting microscope, we prospectively examined 47 lung specimens resected during the past 4 years and submitted with the clinical impression of ELS (n=11), ILS (n=11), CCAM (n=20), LE (n=4), and airway-esophageal communication (n=1). Most lesions were detected by prenatal ultrasound and were resected during infancy. The clinical impression and pathologic findings were compared. Pathologic examination revealed atresia of a lobar, segmental, or subsegmental bronchus in 100% of ELS, 82% of ILS, 70% of CCAM, and 50% of LE (those clinically recognized to have BA or minor CCAM) cases. Parenchymal maldevelopment that characterizes CCAM was present in 100% of CCAM cases (as expected by definition) as well as in 91% of ELS, 91% of ILS, and 50% of LE (those with BA) cases. Bronchial atresia is present in all ELS, most ILS and CCAM, and some LE cases, and its detection is greatly enhanced with the dissecting microscope. Bronchial atresia and CCAM nearly always coexist. It may be that both have the same etiopathogenesis with anatomic differences accounted for by aberrant genetic programs or other insults, perhaps modified by time of onset or duration.

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Congenital cystic adenomatoid malformation of the lung with bronchial atresia

Cited by 31 publications

References 9 publications

Bronchial atresia: the hidden pathology within a spectrum of prenatally diagnosed lung masses

Bronchial atresia: the hidden pathology within a spectrum of prenatally diagnosed lung masses

Fetal Pulmonary Malformations: Defining Histopathology

Bronchial Atresia is Common to Extralobar Sequestration, Intralobar Sequestration, Congenital Cystic Adenomatoid Malformation, and Lobar Emphysema

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