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

Kunisaki, Shaun M.; Fauza, Dario O.; Nemes, Luanne P.; Barnewolt, Carol E.; Estroff, Judy A.; Kozakewich, Harry P.; Jennings, Russell W.

doi:10.1016/j.jpedsurg.2005.10.082

Cited by 148 publications

(97 citation statements)

References 20 publications

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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: 89%

“…One theory is that that these lesions are related to airway obstruction with secondary pulmonary dysplastic changes. Kunisaki 8 and others 5 claimed that many congenital bronchopulmonary malformations may represent a continuum of foetal lung anomalies associated with unrecognized airway obstruction. Therefore, for example, bronchial atresia occurring early in gestation might favour CPAM or a bronchogenic cyst, while obstruction developing later in gestation might predispose the lungs to develop BPS or CLO (ref.…”

Section: Discussionmentioning

confidence: 99%

“…Since bronchial obstruction occurring during early lung development may be an inciting event in the disease pathogenesis of CPAMs (ref. [5][6][7][8] ). Some authors have postulated that an obstructing bronchial mucus plug may transiently induce abnormal pulmonary growth, thereby mimicking a lung malformation until the obstruction is alleviated 14 .…”

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confidence: 99%

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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: 89%

Section: Discussionmentioning

confidence: 99%

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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

265

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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“…Finally, ultrasound will be helpful to find other malformations that may be associated [7]: kidney (bilateral renal agenesis, multicystic dysplasia), cardiac (tetralogy of Fallot, VIC), gastrointestinal (duodenal atresia, imperforate anus, omphalocele) or chest (diaphragmatic hernia, pulmonary sequestration). In our case, the ultrasound findings were suggestive of the disease.…”

Section: Discussionmentioning

confidence: 99%

Antenatal Diagnosis of Cystic Adenomatoid Malformation of the Lung

O¹

2014

Gynecol Obstet

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The Cystic Adenomatoid Malformation (CCAM) of the lung is rare with a frequency estimated between 1/25 000 and 1/35 000 of pregnancies. It consists of a default of alveoli development associated with an abnormal proliferation of terminal bronchioles giving rise to various sizes of cysts. We report a case of cystic adenomatoid malformation type II of the left lower lobe revealed at 22 weeks of gestation by an acute hydramnios. The fetal karyotype was normal. The presence of fetal anasarca and the severity of the adjacent organs compression have justified the pregnancy interruption which has been strongly recommended by the couple. The autopsy confirmed the diagnostic of cystic adenomatoid malformation associated with a complete hypoplasic right lung.

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

Cited by 148 publications

References 20 publications

Congenital bronchopulmonary malformation: CT histopathological correlation

Congenital bronchopulmonary malformation: CT histopathological correlation

Pulmonary cysts in early childhood and the risk of malignancy

Antenatal Diagnosis of Cystic Adenomatoid Malformation of the Lung

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