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Congenital laryngeal web

Orpha number ORPHA2374
Synonym(s) -
Prevalence -
Inheritance -
Age of onset -
ICD-10
  • Q31.0
ICD-O -
OMIM
UMLS
  • C0152416
MeSH -
MedDRA
  • 10023871

Summary

Congenital laryngeal web is a rare malformation consisting of a membrane-like structure that extends across the laryngeal lumen close to the level of the vocal cords. The prevalence at birth is less than 1 in 10 000. Manifestations may appear at any age (hoarse or weak voice and frequent upper respiratory infections) but onset usually occurs during infancy (respiratory distress, stridor and an unusual cry). Other larynx anomalies, such as ventral laryngeal cleft and subglottic stenosis, may be associated with this malformation. The malformation is a result of anomalous embryologic development of the larynx, stemming from incomplete resorption of the epithelial layer that normally obliterates the developing laryngeal opening at about the sixth week of gestation. This layer is usually completely eliminated by the tenth week. Since resorption proceeds from the dorsal to the ventral side, laryngeal webs are anteriorly located, leaving a posterior lumen. The pattern of inheritance of this anomaly in multiplex families is compatible with an autosomal dominant mode of inheritance. Chromosomal and cardiovascular anomalies may also be observed in patients with a congenital laryngeal web. Accordingly, patients with a congenital laryngeal web should undergo genetic screening, which should include testing for a chromosome 22q11 deletion, and a thorough cardiovascular evaluation, which should include imaging of the aortic arch. The primary goals of management for congenital laryngeal web are to provide a patent airway and to achieve a good voice quality. However, vocal cords have a tendency for fibrosis and granulation tissue formation after surgical interventions. Traditionally, the treatment of choice for laryngeal web is laryngofissure and placement of a stent or keel. Laser therapy has also been tested. Good voice quality is only achieved in patients with thin, membranous, uncomplicated webs. Treatment for thick webs, with or without associated congenital subglottic stenosis, remains unsatisfactory.


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