Outcomes of scapulothoracic fusion in facioscapulohumeral muscular dystrophy: A systematic review

Background Facioscapulohumeral muscular dystrophy (FSHD) is a rare condition associated with selective weakness of the muscles of the upper arm, face, and shoulder girdle, negatively affecting daily activities. Scapulothoracic arthrodesis may restore shoulder function and improve quality of life. The purpose of this review is to evaluate the outcomes and complications of scapulothoracic arthrodesis in FSHD patients. Methods Medline, Pubmed, and Embase were systematically searched. Studies were included if they described scapulothoracic arthrodesis in FSHD with follow-up, and outcomes were adequately reported. Thirteen eligible articles reported the outcomes of 199 arthrodesis in 130 patients. Results The mean gain of shoulder forward elevation and abduction were 45° (p < 0.05) and 40° (p < 0.05), respectively. There was an overall cosmetic satisfaction and improved performance of daily activities. There is limited and heterogeneous data on changes in pulmonary function, but such changes are clinically insignificant. The rate of complications was 41% of which 10% were serious, requiring an intervention or re-admission. The most common complications were hardware failure (8%), non-union (6%), and pneumothorax (5%). Discussion Scapulothoracic arthrodesis improved cosmesis, performance of daily activities and shoulder motion with no clinically significant loss of pulmonary function. The complication rate is high, and some are potentially serious.

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Letter to the Editor Regarding “Non-Union After Multiple Lumbar Fusion Surgeries in a Patient with Facioscapulohumeral Muscular Dystrophy: A Case Report”

World Neurosurgery ◽

10.1016/j.wneu.2020.05.072 ◽

2020 ◽

Vol 141 ◽

pp. 527 ◽

Cited By ~ 1

Author(s):

İlker Eren ◽

Cüneyt Şar ◽

Mehmet Demirhan

Keyword(s):

Case Report ◽

Muscular Dystrophy ◽

Lumbar Fusion ◽

Facioscapulohumeral Muscular Dystrophy ◽

Non Union ◽

Letter To The Editor

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Cardiac Involvement in Facioscapulohumeral Muscular Dystrophy (FSHD)

Frontiers in Neurology ◽

10.3389/fneur.2021.668180 ◽

2021 ◽

Vol 12 ◽

Author(s):

Allison Ducharme-Smith ◽

Stefan Nicolau ◽

C. Anwar A. Chahal ◽

Kirstie Ducharme-Smith ◽

Shujah Rehman ◽

...

Keyword(s):

Muscular Dystrophy ◽

Atrioventricular Block ◽

Cardiac Involvement ◽

Case Reports ◽

Shoulder Girdle ◽

Facioscapulohumeral Muscular Dystrophy ◽

Reduced Ejection Fraction ◽

Cardiac Abnormalities ◽

Conduction Abnormality ◽

Conduction Abnormalities

Background: Facioscapulohumeral muscular dystrophy (FSHD) is one of the most common muscular dystrophies and predominantly affects facial and shoulder girdle muscles. Previous case reports and cohort studies identified minor cardiac abnormalities in FSHD patients, but their nature and frequency remain incompletely characterized.Methods: We reviewed cardiac, neurological and genetic findings of 104 patients with genetically confirmed FSHD.Results: The most common conduction abnormality was complete (7%) or incomplete (5%) right bundle branch block (RBBB). Bifascicular block, left anterior fascicular block, complete atrioventricular block, and 2:1 atrioventricular block each occurred in 1% of patients. Atrial fibrillation or flutter were seen in 5% of patients. Eight percent of patients had heart failure with reduced ejection fraction and 25% had valvular disease. The latter included aortic stenosis in 6% (severe in 4% and moderate in 2%) and moderate aortic regurgitation in 8%. Mitral valve prolapse (MVP) was present in 9% of patients without significant mitral regurgitation. There were no significant associations between structural or conduction abnormalities and age, degree of muscle weakness, or size of the 4q deletion.Conclusions: Both structural and conduction abnormalities can occur in FSHD. The most common abnormalities are benign (RBBB and MVP), but more significant cardiac involvement was also observed. The presence of cardiac abnormalities cannot be predicted from the severity of the neurological phenotype, nor from the genotype.

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