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Long-term efficacy of glycopyrrolate on sialorrhea in Goldenhar syndrome: a case report

BACKGROUND: Goldenhar syndrome (GS) is a rare congenital disease characterized by impaired development of different facial structures and deformations of the teeth structures. Sialorrhea, which can cause difficulties in breathing and language impairment, is very common in GS and often difficult to t...

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Autores principales: Della Bella, Gessica, Castelli, Enrico, Vigevano, Federico
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9450236/
https://www.ncbi.nlm.nih.gov/pubmed/36068575
http://dx.doi.org/10.1186/s13052-022-01320-8
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author Della Bella, Gessica
Castelli, Enrico
Vigevano, Federico
author_facet Della Bella, Gessica
Castelli, Enrico
Vigevano, Federico
author_sort Della Bella, Gessica
collection PubMed
description BACKGROUND: Goldenhar syndrome (GS) is a rare congenital disease characterized by impaired development of different facial structures and deformations of the teeth structures. Sialorrhea, which can cause difficulties in breathing and language impairment, is very common in GS and often difficult to treat. This case report highlights the short- and long-term importance of the therapeutic choice – glycopyrronium in oral solution - for the treatment of sialorrhea in children with poly-malformative syndrome, complicated by outcomes of post-hemorrhagic hydrocephalus. CASE PRESENTATION: We report the case of a 6-year-old child with GS, carrying a percutaneous endoscopic gastrostomy after tracheostomy. The child also presented developmental dysfunction of oral motor skills of feeding, complicated by severe sialorrhea, related to the maxillo-facial dysmorphism. Sialorrhea caused several respiratory tract infections and led to an increase in the care burden. Both the inoculations of botulinum toxin and the treatment with scopolamine transdermal patch have shown mild and transient efficacy. The therapeutic choice of glycopyrronium in oral solution was the most suitable for this patient, leading to long-term sialorrhea control. CONCLUSIONS: This clinical experience represents the first long-term efficacy and tolerability evaluation in using glycopyrrolate oral solution in treating drooling in children with GS. The reduction of drooling over time and the lack of clinically relevant adverse events have contributed to the decrease of respiratory tract infections, the development of oral motor skills, and determining a positive psycho-social impact on the patient's quality of life and her family.
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spelling pubmed-94502362022-09-08 Long-term efficacy of glycopyrrolate on sialorrhea in Goldenhar syndrome: a case report Della Bella, Gessica Castelli, Enrico Vigevano, Federico Ital J Pediatr Case Report BACKGROUND: Goldenhar syndrome (GS) is a rare congenital disease characterized by impaired development of different facial structures and deformations of the teeth structures. Sialorrhea, which can cause difficulties in breathing and language impairment, is very common in GS and often difficult to treat. This case report highlights the short- and long-term importance of the therapeutic choice – glycopyrronium in oral solution - for the treatment of sialorrhea in children with poly-malformative syndrome, complicated by outcomes of post-hemorrhagic hydrocephalus. CASE PRESENTATION: We report the case of a 6-year-old child with GS, carrying a percutaneous endoscopic gastrostomy after tracheostomy. The child also presented developmental dysfunction of oral motor skills of feeding, complicated by severe sialorrhea, related to the maxillo-facial dysmorphism. Sialorrhea caused several respiratory tract infections and led to an increase in the care burden. Both the inoculations of botulinum toxin and the treatment with scopolamine transdermal patch have shown mild and transient efficacy. The therapeutic choice of glycopyrronium in oral solution was the most suitable for this patient, leading to long-term sialorrhea control. CONCLUSIONS: This clinical experience represents the first long-term efficacy and tolerability evaluation in using glycopyrrolate oral solution in treating drooling in children with GS. The reduction of drooling over time and the lack of clinically relevant adverse events have contributed to the decrease of respiratory tract infections, the development of oral motor skills, and determining a positive psycho-social impact on the patient's quality of life and her family. BioMed Central 2022-09-06 /pmc/articles/PMC9450236/ /pubmed/36068575 http://dx.doi.org/10.1186/s13052-022-01320-8 Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Case Report
Della Bella, Gessica
Castelli, Enrico
Vigevano, Federico
Long-term efficacy of glycopyrrolate on sialorrhea in Goldenhar syndrome: a case report
title Long-term efficacy of glycopyrrolate on sialorrhea in Goldenhar syndrome: a case report
title_full Long-term efficacy of glycopyrrolate on sialorrhea in Goldenhar syndrome: a case report
title_fullStr Long-term efficacy of glycopyrrolate on sialorrhea in Goldenhar syndrome: a case report
title_full_unstemmed Long-term efficacy of glycopyrrolate on sialorrhea in Goldenhar syndrome: a case report
title_short Long-term efficacy of glycopyrrolate on sialorrhea in Goldenhar syndrome: a case report
title_sort long-term efficacy of glycopyrrolate on sialorrhea in goldenhar syndrome: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9450236/
https://www.ncbi.nlm.nih.gov/pubmed/36068575
http://dx.doi.org/10.1186/s13052-022-01320-8
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