J Korean Orthop Assoc.  2008 Jun;43(3):379-384. 10.4055/jkoa.2008.43.3.379.

Early-Onset Facioscapulohumeral Muscular Dystrophy: Significance of Pelvic Extensor in Sagittal Spinal Imbalance

• Hwang C
• Kang SJ
• Lee SW
• Ahn YJ
• Kim YT
• Lee DH
• Lee CS

• KMID: 2186456
• DOI: http://doi.org/10.4055/jkoa.2008.43.3.379

Abstract

An 11-year-old girl with early-onset facioscapulohumeral muscular dystrophy (FSHD) presented with progressive gait disturbance and lumbar hyperlordosis. The motor power of her pelvic extensor muscles was grade 3. Pelvic tilt and hip flexion were markedly increased as determined by gait analysis. This FSHD case is an impressive example of a patient demonstrating the concept that weak pelvic extensor muscles cannot keep the spine upright and balanced. The most important factor in the development of hyperlordosis is the weakness of the pelvic extensor muscles, and the results of gait analysis exquisitely explain the pathophysiology. The patient stands with her spine hyperextended to maintain upright posture by a compensatory mechanism of relatively strong back extensor muscles. Corrective surgery for lumbar hyperlordosis was not considered as it could eliminate the compensatory lumbar hyperextension, thus making the spine of the patient stoop forward through the hip joint during walking, being caused by the weakness of her pelvic extensor muscles.

Keyword

Facioscapulohumeral muscular dystrophy (FSHD); Sagittal Spinal imbalance (SSI); Lumbar Hyperlordosis; Pelvic Extensor muscle; Gait analysis

MeSH Terms

Child
Gait
Hip
Hip Joint
Humans
Muscles
Muscular Dystrophy, Facioscapulohumeral
Posture
Spine
Walking

Figure

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  Fig. 1 Facial photograph showing bifacial weakness. Note the inability of the patient to close her mouth completely.

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  Fig. 2 Lateral and posterior photographs with the patient standing demonstrate the extremely marked lumbar lordosis with the hands clasped on the posterior hip region for support. This clinical appearance is virtually pathognomonic for early-onset FSHD. Scapular winging is evident in the back view of the patient. Standing radiographs show lumbar hyperlordosis and a horizontal sacrum (arrow).

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  Fig. 3 The deformity of the patient was flexible.

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  Fig. 4 The result of gait analysis. Anterior pelvic tilt increased so severely that it could not be visualized.

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  Fig. 5 The mechanism of hyperlordosis in this case. The weakness of the pelvic extensor muscles brings about secondary hip flexion contracture (A), and compensatory lumbar hyperlordosis follows (B). Although the lumbar deformity can be corrected surgically or with a brace, stooping will become more severe as the weak pelvic extensor muscles are not be able to overcome the stooping of the pelvis and trunk.

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