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

Riedlinger, Wolfram F. J.; Vargas, Sara O.; Jennings, Russell W.; Estroff, Judy A.; Barnewolt, Carol E.; Lillehei, C. Walton; Wilson, Jay M.; Colin, Andrew A.; Reid, Lynne; Kozakewich, Harry P.

doi:10.2350/06-01-0023.1

Cited by 143 publications

(86 citation statements)

References 42 publications

(85 reference statements)

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“…In 75% cases (18 out of 24) cases the areas of hypoattenuation were present ( Fig. 1) which correspond with the findings reported 2,5,8,9 . In 50% cases (12 out of 24) the areas of hypoattenuation occurred together with another type of BPMs, with three bronchopulmonary sequestrations, all being type 1 CPAMs, and with four sequestrations of type 2 CPAMs, which is an additional CT characteristic of the lesion.…”

Section: Resultssupporting

confidence: 78%

Congenital bronchopulmonary malformation: CT histopathological correlation

Kynčl¹,

Kočí²,

Ptackova³

et al. 2016

BIOMED PAP

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Background. This study evaluated the accuracy of postnatal computed tomography (CT) imaging in the identification of congenital bronchopulmonary malformation (BPM) in comparison with histopathological analysis. Methods. CT scans of prenatally diagnosed BPMs from 24 patients with available histology were analysed retrospectively. The CT images were reviewed blinded to histological findings by two radiologists. Specific diagnosis was assigned based on predetermined criteria. The accuracy of CT was evaluated. Results. The agreement rate in CT diagnosis between two radiologists was 100%. In 75% the lesions were located in the lower lobes. An overlap of 71% in CT and histopathological diagnoses was reached. The least matching diagnosis was type 2 CPAM. Conclusion. Contrast enhanced chest CT is very accurate in characterizing the BPM spectrum and provides important information on lesion type and structure.

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Section: Resultssupporting

confidence: 78%

Congenital bronchopulmonary malformation: CT histopathological correlation

Kynčl¹,

Kočí²,

Ptackova³

et al. 2016

BIOMED PAP

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“…41 Several developmental lung lesions, including CPAM types 1-3, have all been associated with in utero airway obstruction and/or atresia as possible pathogenetic causes; mechanisms by which obstruction or atresia result in variable final lesions have yet to be elucidated. [43][44][45] CCAM and CPAM nomenclature and prominent characteristics are summarized in Table 1. Because of overlap among types and great clinical variability within individual lung lesions, assignment of a particular pulmonary lesion to a specific CCAM-CPAM category can be difficult and may not be consistent between different observers.…”

Section: The Differential Diagnosis Of Air-filled Lung Cysts In Earlymentioning

confidence: 99%

Pulmonary cysts in early childhood and the risk of malignancy

Priest

Williams

Hill

et al. 2008

Pediatric Pulmonology

262

215

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Summary. Surgery for congenital and early childhood lung cysts is often dictated by symptoms such as respiratory distress, infection or pneumothorax. Asymptomatic cysts present a therapeutic dilemma: surgical intervention and ''conservative'' observation have advocates. The risk of malignancy in such cysts is considered by some an indication for surgical intervention and is reviewed in this paper. Pleuropulmonary blastoma (PPB) is the most frequent malignancy associated with childhood lung cysts. Although rare, PPB occurs predictably in certain clinical and familial situations. This unique biology of PPB can inform the cyst management decision. The earliest manifestation of PPB is a malignant lung cyst in young children, clinically and radiographically indistinguishable from benign congenital lung cysts. Histopathologic examination differentiates cystic PPB from the benign cystic variants. Surgical excision of cystic PPB (with or without chemotherapy) cures approximately 85-90% of children. If not excised, cystic PPB evolves to cystic/solid or solid high-grade sarcoma (cure rate 45-60%) by age 2-6 years. Numerous reports of ''malignancy in a congenital lung cyst'' are now understood as the characteristic progression of cystic PPB. PPB is genetically determined in many cases. Detailed family history may reveal the hallmarks of PPB in the patient or young relatives: a unique constellation of diseases including lung cysts, cystic nephroma, childhood cancers, stromal sex-chord ovarian tumors, seminomas or dysgerminomas, intestinal polyps, thyroid hyperplasias, and hamartomas. Pneumothorax and multifocal/bilateral lung cysts also characterize PPB. These diagnoses predict that a lung cyst is more likely PPB than a benign congenital cyst. Patients fitting this pattern deserve histologic diagnosis. The genetic basis for this heritable syndrome is unknown but is being actively investigated.

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“…Two groups of patients were identified: (1) patients diagnosed with PBA on postnatal CT scan (n = 16), and (2) patients with the pathologically confirmed diagnosis of PBA independent of post-mandates postnatal monitoring and subsequent characterization with a postnatal chest computed tomography (CT) scan. Peripheral bronchial atresia (PBA) is a newly identified fetal lung lesion which is often asymptomatic at birth and is characterized pathologically by a cystically dilated bronchus, airspace enlargement and the presence of a mucocele and/or abundant mucus distal to the region of atresia [1][2][3][4] . It is characterized on postnatal CT scan as an area of hyperexpansion and hyperlucency consistent with air trapping often without cystic changes.…”

Section: Introductionmentioning

confidence: 99%

“…It is characterized on postnatal CT scan as an area of hyperexpansion and hyperlucency consistent with air trapping often without cystic changes. Traditionally, bronchial atresia is believed to occur more frequently in the left upper lobe although cases exist documenting bronchial atresia in all lobes of the lung [1,[3][4][5] . Unlike congenital cystic adenomatoid malformations (CCAM), which are associated with an increased risk of infection, pneumothorax, and malignant degeneration, PBAs, if asymptomatic, are not believed to be associated with an increased risk of secondary complications [6,7] .…”

Section: Introductionmentioning

confidence: 99%

Prenatal Course and Postnatal Management of Peripheral Bronchial Atresia: Association with Congenital Cystic Adenomatoid Malformation of the Lung

et al. 2008

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Objective: Peripheral bronchial atresia (PBA), a newly identified fetal lung lesion, is often asymptomatic and managed nonoperatively. However, recent studies suggest that bronchial atresia plays a role in the etiology of microcystic maldevelopment present in congenital cystic adenomatoid malformations (CCAM) which require resection to decrease the risk of infection, pneumothorax and malignant degeneration. The purpose of this study was to evaluate the prenatal radiographic and postnatal computed tomography (CT) scan/pathology findings with attention to the pathologic diagnosis of microcystic maldevelopment in infants with the presumptive diagnosis of PBA. Methods: A retrospective review of prenatal and postnatal records of patients diagnosed with fetal lung lesions was performed. Two groups of patients were identified: (1) patients diagnosed with PBA on postnatal CT scan (n = 16), and (2) patients with the pathologically confirmed diagnosis of PBA independent of postnatal CT findings (n = 23). Results: Prenatal ultrasound diagnosis of these lesions included CCAMs, hybrid lesions, bronchopulmonary sequestrations and bronchial atresia. Eleven of the 16 patients in group 1 with the postnatal radiologic diagnosis of PBA underwent surgical resection, 6 of which were found to have microcystic changes consistent with CCAM. Evaluation of the 23 patients in group 2 with pathologically confirmed PBA identified 65% that had evidence of microcystic maldevelopment consistent with the small cyst type of CCAM. Conclusion: Radiographically diagnosed PBA as well as pathologically confirmed PBA is frequently associated with microcystic changes consistent with the small cyst type of CCAM. Thus, operative management should be considered for PBA to decrease CCAM-associated risks.

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

Cited by 143 publications

References 42 publications

Congenital bronchopulmonary malformation: CT histopathological correlation

Congenital bronchopulmonary malformation: CT histopathological correlation

Pulmonary cysts in early childhood and the risk of malignancy

Prenatal Course and Postnatal Management of Peripheral Bronchial Atresia: Association with Congenital Cystic Adenomatoid Malformation of the Lung

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