title: Focal dermal hypoplasia (Goltz-Gorlin syndrome) associated with obstructive papillomatosis of the larynx and hypopharynx.
abstract: A 14-year-old girl with focal dermal hypoplasia (Goltz-Gorlin-syndrome) presented with dysphagia, hoarseness, inspiratory stridor, intermittent dry cough and a 10% weight loss. Endoscopy showed that these symptoms were caused by papillomatosis of the hypopharynx and the larynx. The papillomatous masses were resected subtotally by endoscopic laser treatment. Residual papillomas were left in the subglottic space but tracheotomy could be avoided. Complete clinical recovery with adequate weight gain as well as, resolution of dyspnoe and dysphagia resulted after the intervention. Histological examination did not show morphological signs of human papilloma virus as an etiological agent.
authors.affiliation: Universitäts-Kinderklinik, Mathildenstrasse 1, 79106 Freiburg, Germany. gordjani@kk1200.ukl.uni-freiberg.de
authors.forename: N
authors.initials: N
authors.lastname: Gordjani
authors.forename: S
authors.initials: S
authors.lastname: Herdeg
authors.forename: U H
authors.initials: UH
authors.lastname: Ross
authors.forename: H
authors.initials: H
authors.lastname: Grimme
authors.forename: M
authors.initials: M
authors.lastname: Kleinschmidt
authors.forename: M
authors.initials: M
authors.lastname: Brandis
mesh_terms: D000293:Adolescent; D000402:Airway Obstruction / Q000209:etiology*; D005260:Female; D005489:Focal Dermal Hypoplasia / Q000150:complications* / Q000473:pathology; D006801:Humans; D007012:Hypopharyngeal Neoplasms / Q000150:complications* / Q000473:pathology / Q000601:surgery; D007822:Laryngeal Neoplasms / Q000150:complications* / Q000473:pathology / Q000601:surgery; D010212:Papilloma / Q000150:complications* / Q000473:pathology / Q000601:surgery