Functional results after carpal tunnel release in mucopolysaccharidosis

Abstract Background Mucopolysaccharidosis consists of a group of diseases caused by the deficiency of lysosomal enzymes, which may lead to the compression of the median nerve in the carpal tunnel due to the accumulation of glycosaminoglycan, resulting in the hand disability. The study purpose is to...

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Main Authors: Giana Silveira Giostri, Camila Deneka Arantes Souza, Alencar Kenji Nagai, Mara Lucia Schmitz Ferreira Santos, José Silvany Pacheco Sampaio, Flavia David João de Masi Nassif
Format: Article
Language:English
Published: BMC 2021-09-01
Series:Orphanet Journal of Rare Diseases
Subjects:
Online Access:https://doi.org/10.1186/s13023-021-01982-3
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spelling doaj-3c3f8ff480944678a309863e447bc7842021-09-12T11:27:15ZengBMCOrphanet Journal of Rare Diseases1750-11722021-09-011611710.1186/s13023-021-01982-3Functional results after carpal tunnel release in mucopolysaccharidosisGiana Silveira Giostri0Camila Deneka Arantes Souza1Alencar Kenji Nagai2Mara Lucia Schmitz Ferreira Santos3José Silvany Pacheco Sampaio4Flavia David João de Masi Nassif5Serviço de Cirurgia da Mão do Hospital Pequeno PríncipeServiço de Cirurgia da Mão do Hospital Pequeno PríncipeServiço de Cirurgia da Mão do Hospital Pequeno PríncipeServiço de Neurologia Pediátrica do Hospital Pequeno PríncipeHospital Universitário CajuruPUC-PRAbstract Background Mucopolysaccharidosis consists of a group of diseases caused by the deficiency of lysosomal enzymes, which may lead to the compression of the median nerve in the carpal tunnel due to the accumulation of glycosaminoglycan, resulting in the hand disability. The study purpose is to present functional results of carpal tunnel release in mucopolysaccharidosis patients. Patients were selected from an enzyme replacement group in the Department of Pediatric Neurology. The legal guardians of the patients were informed about the likely functional change of the hands induced by compression of the median nerve. Clinical evaluation was performed in those patients who received their legal guardians’ consent to participate and was included inspection, assessment of functional level, wrinkle test and the digital pinch function to manipulate small and large objects. Ultrasound and electromyography were performed to confirm the clinical median nerve compression. Bilateral extended opening technique was performed to access the carpal tunnel and analyze the anatomic findings of the median nerve and the flexed tendons of the fingers. After the surgical release of the carpal tunnel, the clinical evaluation was repeated. Subjective observations of the legal guardians were also considered. Results Seven patients underwent bilateral surgical opening of the carpal tunnel; six boys, mean age of 9.5 (5 to 13), five of them presenting Type II mucopolysaccharidosis, 1 Type I and 1 Type VI. The average follow-up was 12 months (10–13 months). The functional results observed included the improvement in the handling of small and large objects in all children who underwent decompression of the median nerve. The comparison between the pre-operative and post-operative functional levels revealed that 2 patients evolved from Level II to IV, 3 from Level III to IV, 1 from Level IV to V and 1 patient remained in Level III. Tenosynovitis around the flexor tendons and severe compression of the median nerve in the fourteen carpal tunnels were observed during the surgical procedure. In 6 wrists, partial tenosynovitis was performed. Conclusions Despite the improvement in the overall function of the children' hands, we cannot conclude that only surgery was responsible for the benefit. Better designed studies are requiredhttps://doi.org/10.1186/s13023-021-01982-3MucopolysaccharidosisCarpal tunnel syndromeMedian nerveNerve compression
collection DOAJ
language English
format Article
sources DOAJ
author Giana Silveira Giostri
Camila Deneka Arantes Souza
Alencar Kenji Nagai
Mara Lucia Schmitz Ferreira Santos
José Silvany Pacheco Sampaio
Flavia David João de Masi Nassif
spellingShingle Giana Silveira Giostri
Camila Deneka Arantes Souza
Alencar Kenji Nagai
Mara Lucia Schmitz Ferreira Santos
José Silvany Pacheco Sampaio
Flavia David João de Masi Nassif
Functional results after carpal tunnel release in mucopolysaccharidosis
Orphanet Journal of Rare Diseases
Mucopolysaccharidosis
Carpal tunnel syndrome
Median nerve
Nerve compression
author_facet Giana Silveira Giostri
Camila Deneka Arantes Souza
Alencar Kenji Nagai
Mara Lucia Schmitz Ferreira Santos
José Silvany Pacheco Sampaio
Flavia David João de Masi Nassif
author_sort Giana Silveira Giostri
title Functional results after carpal tunnel release in mucopolysaccharidosis
title_short Functional results after carpal tunnel release in mucopolysaccharidosis
title_full Functional results after carpal tunnel release in mucopolysaccharidosis
title_fullStr Functional results after carpal tunnel release in mucopolysaccharidosis
title_full_unstemmed Functional results after carpal tunnel release in mucopolysaccharidosis
title_sort functional results after carpal tunnel release in mucopolysaccharidosis
publisher BMC
series Orphanet Journal of Rare Diseases
issn 1750-1172
publishDate 2021-09-01
description Abstract Background Mucopolysaccharidosis consists of a group of diseases caused by the deficiency of lysosomal enzymes, which may lead to the compression of the median nerve in the carpal tunnel due to the accumulation of glycosaminoglycan, resulting in the hand disability. The study purpose is to present functional results of carpal tunnel release in mucopolysaccharidosis patients. Patients were selected from an enzyme replacement group in the Department of Pediatric Neurology. The legal guardians of the patients were informed about the likely functional change of the hands induced by compression of the median nerve. Clinical evaluation was performed in those patients who received their legal guardians’ consent to participate and was included inspection, assessment of functional level, wrinkle test and the digital pinch function to manipulate small and large objects. Ultrasound and electromyography were performed to confirm the clinical median nerve compression. Bilateral extended opening technique was performed to access the carpal tunnel and analyze the anatomic findings of the median nerve and the flexed tendons of the fingers. After the surgical release of the carpal tunnel, the clinical evaluation was repeated. Subjective observations of the legal guardians were also considered. Results Seven patients underwent bilateral surgical opening of the carpal tunnel; six boys, mean age of 9.5 (5 to 13), five of them presenting Type II mucopolysaccharidosis, 1 Type I and 1 Type VI. The average follow-up was 12 months (10–13 months). The functional results observed included the improvement in the handling of small and large objects in all children who underwent decompression of the median nerve. The comparison between the pre-operative and post-operative functional levels revealed that 2 patients evolved from Level II to IV, 3 from Level III to IV, 1 from Level IV to V and 1 patient remained in Level III. Tenosynovitis around the flexor tendons and severe compression of the median nerve in the fourteen carpal tunnels were observed during the surgical procedure. In 6 wrists, partial tenosynovitis was performed. Conclusions Despite the improvement in the overall function of the children' hands, we cannot conclude that only surgery was responsible for the benefit. Better designed studies are required
topic Mucopolysaccharidosis
Carpal tunnel syndrome
Median nerve
Nerve compression
url https://doi.org/10.1186/s13023-021-01982-3
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