Ectopic bronchus: An insufficiently recognized malformation causing respiratory morbidity in VATER association

Hattori, Haruo; Okazaki, Shin; Higuchi, Yoshinori; Yoshibayashi, Muneo; Yamamoto, Tetsuo

doi:10.1002/(sici)1096-8628(19990115)82:2<140::aid-ajmg7>3.0.co;2-0

Cited by 6 publications

(2 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In the neonatal period, shortly after repair, there are enough explanations for these symptoms: thoracotomy itself, pneumothorax, peptic bronchitis, the fistula stump, aspiration, and other. Deficient drainage through the sometimes-misplaced upper-right lobe bronchus causes atelectasis (50). Recurrent fistula may produce discrete or severe symptoms (51).…”

Section: Lung Hypoplasia In Esophageal Atresiamentioning

confidence: 53%

Lung hypoplasia in rats with esophageal atresia and tracheo–esophageal fistula

et al. 2012

View full text Add to dashboard Cite

IntroductIon: survivors of esophageal atresia and tracheo-esophageal fistula (ea-TeF) often suffer chronic respiratory tract disease. ea-TeF results from abnormal emergence of the trachea from the foregut. This study in a rat model tests the hypothesis that primary lung maldevelopment might be a downstream consequence of this defect. results: The lung was hypoplastic in rats with ea-TeF although the histological pattern was normal. Maturation and arteriolar wall thickness were unchanged, but mesenchymal control of airway branching was weakened. This branching was deficient from embryonal day (e13) on in adriamycin-treated explants. dIscussIon: In conclusion, the lungs were hypoplastic in rats with experimental ea-TeF due to defective embryonal airway branching. however, arteriolar wall and respiratory epithelial patterns remained normal. These findings suggest that similarly defective lung development might contribute to chronic respiratory disease in ea-TeF patients. Methods: Pregnant rats received either 1.75 mg/kg i.p. adriamycin or vehicle on e7, e8, and e9. Lungs were recovered at e15, e18, and e2. Lung weight/body weight ratio, total DNa and protein, radial alveolar count, arteriolar wall thickness, lung maturity, and mesenchymal control of airway branching were assessed. e13 lungs were cultured for 72 h and explant airway branching was measured daily. For comparisons, nonparametric tests (*P < 0.05) were used.

show abstract

Section: Lung Hypoplasia In Esophageal Atresiamentioning

confidence: 53%

Lung hypoplasia in rats with esophageal atresia and tracheo–esophageal fistula

et al. 2012

View full text Add to dashboard Cite

show abstract

“… 7 The preauricular tags seen in our case is a rare manifestation in CRS or VACTERL association with only a few reported cases. 8 Popliteal pterigeum is a well-documented abnormality of the CRS, that can be detected on antenatal scans.…”

Section: Discussionmentioning

confidence: 99%

Severe caudal regression syndrome with overlapping features of VACTERL complex: antenatal detection and follow up

et al. 2017

View full text Add to dashboard Cite

Caudal regression is a rare syndrome with a spectrum of structural defects involving multiple organ systems. Spinal anomalies, a charecteristic feature of the entity, can vary from isolated partial agenesis of the coccyx to lumbosacral agenesis with involvement of the thoracic spine in the most severe cases. The aetiology of this syndrome is not well-known. Maternal diabetes, genetic predisposition and vascular hypoperfusion have been suggested as possible causative factors. Severe forms of the disease are commonly associated with cardiac, renal and respiratory problems with overlapping feature of VACTERL complex (vertebral, anorectal, cardiac, tracheoesophageal, renal and limb anomalies). In this case report, we describe imaging appearances of severe caudal regression syndrome, VACTERL complex associated with multisystem anomalies, detected on a screening antenatal scan during second trimester. Some unusual features of the syndrome including sternal anomaly and absent bony hemithorax are highlighted.

show abstract

Bronchial anomalies in VACTERL association

Kanu

Tegay

Scriven

2008

Pediatric Pulmonology

View full text Add to dashboard Cite

VACTERL association is an acronym made of associated defects including vertebral anomalies, anal atresia, cardiac, tracheal-esophageal fistula, and renal/radial limb anomalies. Tracheal bronchus is a condition characterized by ectopic location of the right upper lobe bronchus at the mid to distal trachea. This condition is associated with congenital anomalies and has been reported in one previous case of VACTERL. We report another infant with VACTERL presenting with respiratory complications due to presence of tracheal bronchus. She also had a narrowed segment of her right main stem bronchus.

show abstract

Ectopic bronchus: An insufficiently recognized malformation causing respiratory morbidity in VATER association

Cited by 6 publications

References 13 publications

Lung hypoplasia in rats with esophageal atresia and tracheo–esophageal fistula

Lung hypoplasia in rats with esophageal atresia and tracheo–esophageal fistula

Severe caudal regression syndrome with overlapping features of VACTERL complex: antenatal detection and follow up

Bronchial anomalies in VACTERL association

Contact Info

Product

Resources

About