Spinal fusion in a patient with Fukuyama congenital muscular dystrophy

Hino, Kaori; Fukuda, Mitsumasa; Morino, Tadao; Ogata, Tadanori; Ito, Masanori; Ishii, Eiichi

doi:10.1016/j.braindev.2017.02.003

Cited by 2 publications

(2 citation statements)

References 10 publications

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“…In our case, the right main bronchus was compressed by the right clavicle and vertebra because of severe scoliosis. Although spinal fusion surgery may be considered for correcting mild scoliosis in FCMD patients, it is not applicable for cases of severe scoliosis . In such cases, corpectomy may be considered to alleviate the compression .…”

Section: Discussionmentioning

confidence: 99%

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Compression of the respiratory tract in Fukuyama congenital muscular dystrophy

Mitsutake

Matsumoto

Hatano

et al. 2018

Neurology & Clinical Neurosc

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We report the case of a 16-year-old boy in whom Fukuyama congenital muscular dystrophy (FCMD) had been genetically diagnosed. He required mechanical ventilation with tracheotomy due to the respiratory muscular weakness. Despite the respiratory supports, he subsequently had repeated temporal respiratory failure.Chest computed tomography revealed that the right main bronchus was compressed by the right clavicle and vertebra. An adjustable-length and flexible tracheostomy tube and high positive end-expiratory pressure were useful to keep the right bronchus patent. In FCMD, the respiratory tract may be occluded by severe scoliosis, which is improved by airway management.

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Section: Discussionmentioning

confidence: 99%

“…Although spinal fusion surgery may be considered for correcting mild scoliosis in FCMD patients, it is not applicable for cases of severe scoliosis. 4 In such cases, corpectomy may be considered to alleviate the compression. 5 Alternatively, stent placement may also be considered to recanalize the respiratory tract.…”

Section: Discussionmentioning

confidence: 99%

Compression of the respiratory tract in Fukuyama congenital muscular dystrophy

Mitsutake

Matsumoto

Hatano

et al. 2018

Neurology & Clinical Neurosc

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Orthopedic manifestations of congenital muscular dystrophy subtypes in children: Emerging signatures need consolidation: a scoping review

El-Sobky,

Abdulhady,

Mahmoud

et al. 2024

JMSR

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Our objective was to screen the literature on congenital muscular dystrophy (CMD) children/adolescents regarding the extent/nature of reporting orthopedic manifestations/deformities and to assess its appropriateness in informing clinical practice/research. We searched PubMed for original research on orthopedic surgical/non-surgical manifestations of CMD. Eligible articles needed to focus on orthopedic manifestations/deformities as one of the study objectives with no restrictions on study designs/types or search period. Eight hundred and thirty articles were initially identified and screened. Following the exclusion of 501 articles for disagreeing titles/abstracts, 329 were available for eligibility assessment. Two hundred and fifty-five articles were excluded for reasons. Of one hundred articles, 15 were captured manually and 11 through pre-submission searches, with 1078 patients included in the final analysis. The most common subtype was Laminin alpha-2 (LAMA2)-relatedCMD. Orthopedic manifestations of CMD are generally underreported and insufficiently detailed. There is reliable evidence that accurate reporting of orthopedic manifestations can be a valuable clinical supplement to the complex differential diagnosis process in collagen VI-related CMD, LAMA2-related-CMD, LMNA-related-CMD, and SEPN1-related CMD (SELENON). For alpha dystroglycan-related CMD, there is insufficient information to delineate a subtype-specific pattern. There is emerging evidence that reporting spine surgery outcomes may facilitate orthopedic decision making. The greatest clinical/research utility was provided by articles with longitudinal, comprehensive, and correlative reporting of larger cohorts. Detailed reporting of the orthopedic phenotype of CMD in future research may further uncover its diagnostic potential.

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Spinal fusion in a patient with Fukuyama congenital muscular dystrophy

Cited by 2 publications

References 10 publications

Compression of the respiratory tract in Fukuyama congenital muscular dystrophy

Compression of the respiratory tract in Fukuyama congenital muscular dystrophy

Orthopedic manifestations of congenital muscular dystrophy subtypes in children: Emerging signatures need consolidation: a scoping review

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