AI Article Synopsis

  • Bilateral choanal atresia (CA) is a rare birth defect often linked with other abnormalities, particularly the CHARGE association, and this study looks at surgical outcomes for children with this condition.
  • The study analyzed 39 children, noting that the rate of restenosis (narrowing of the nasal passage) post-surgery was 31.3% in the CHARGE group and 47.8% in the non-syndromic group.
  • Results suggested that while endoscopic repair was beneficial for both groups, the use of nasal stents increased the need for additional procedures and rates of restenosis; thus, the use of stents should be reconsidered.

Article Abstract

Bilateral choanal atresia (CA) is a rare congenital malformation frequently associated with other anomalies. CHARGE association is closely linked to bilateral CA. The aim of this study was to describe the outcomes of the endoscopic repair in bilateral CA, and to assess the role of postoperative nasal stenting in two cohorts of CHARGE-associated and non-syndromic CA. Thirty-nine children were retrospectively analyzed (16 patients had CHARGE-associated CA). The rate of postoperative neochoanal restenosis was 31.3% in the CHARGE population, and 47.8% in the non-syndromic CA cohort. Data on postoperative synechiae and granulation tissue formation, need for endonasal toilette and dilation procedures, and number of procedures per patient were presented. Stent positioning led to a higher number of postoperative dilation procedures per patient in the non-syndromic cohort ( = 0.018), and to a higher rate of restenosis both in the CHARGE-associated, and non-syndromic CA populations. Children with CHARGE-associated and non-syndromic bilateral CA benefitted from endonasal endoscopic CA correction. The postoperative application of an endonasal stent should be carefully evaluated.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8269040PMC
http://dx.doi.org/10.3390/jcm10132951DOI Listing

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Article Synopsis
  • Bilateral choanal atresia (CA) is a rare birth defect often linked with other abnormalities, particularly the CHARGE association, and this study looks at surgical outcomes for children with this condition.
  • The study analyzed 39 children, noting that the rate of restenosis (narrowing of the nasal passage) post-surgery was 31.3% in the CHARGE group and 47.8% in the non-syndromic group.
  • Results suggested that while endoscopic repair was beneficial for both groups, the use of nasal stents increased the need for additional procedures and rates of restenosis; thus, the use of stents should be reconsidered.
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Background: Three different homozygous loss-of-function mutations of the Forkhead box E1 (FOXE1) gene have been associated with syndromic cleft palate. Here, we screened the entire promoter region to identify the variations in significant consensus motifs affecting FOXE1 transcription.

Method: Genomic DNAs of 35 cleft palate patients, 10 of whom with CHARGE association, 80 unrelated healthy people and 80 unaffected first-degree relatives were analysed by automatic sequencing.

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