Orthopedic management in autosomal recessive spastic ataxia of Charlevoix-Saguenay

Article Figures & Tables

Download figure
Open in new tab
Download powerpoint
FIG. 1
Foot deformity in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). Calcaneovarus is associated with plantar arch accentuation, a metatarsus adductus and clawing of the toes.
Download figure
Open in new tab
Download powerpoint
FIG. 2
Management by triple arthrodesis. The subtalar joint is fixed with a cannulated lag screw; the calcaneocuboidal joint is fixed with a staple and the 3 joints are grafted.
Download figure
Open in new tab
Download powerpoint
FIG. 3
Radiograph 8 weeks after a triple arthrodesis performed according to the technique shown in Fig. 2. The arthrodesis appears to be consolidated.

Table I

Surgical Procedures Performed During the Study Period by Year

Procedure	Year													Total procedures
Procedure	1978	1983	1984	1985	1986	1987	1988	1989	1990	1992	1994	1996	1997	Total procedures
Triple arthrodesis only		1		2	2		3	2						10
Triple arthrodesis, extensor digitorum and hallucis longus transfer			3	2										5
Triple arthrodesis and Achilles tendon lengthening					1			2	2	2			2	9
Achilles tendon lengthening only					2	6		2						10
Ankle arthrodesis							1							1
Plantar aponeurectomy	2													2
Pseudarthrosis revision (talonavicular and calcaneocuboidal)									3					3
Correction of claw toes					1	1								2
Staple removal						1	1					1		3
Adductor and psoas muscle tenotomies and obturator nerve neurectomy								2			2			4
Total	2	1	3	4	6	8	5	8	5	2	2	1	2	49

Table II

Surgical Procedures (N = 49) Performed According to Patient Age and Sex (M/F)

Procedure	Patient age, yr
Procedure	10–19	20–29	30–39	40–49	50–59
Triple arthrodesis only	2/M	1/M, 1/F	4/M	2/M
Triple arthrodesis, extensor digitorum and hallucis longus transfer			2/M, 2/F	1/M
Triple arthrodesis and Achilles tendon lengthening		2/M	4/M	3/M
Achilles tendon lengthening only		2/M, 2/F	4/F		2/F
Ankle arthrodesis		1/M
Plantar aponeurectomy	2/M
Pseudarthrosis revision (talonavicular and calcaneocuboidal)			3/M
Correction of claw toes			2/F
Staple removal			1/M	2/M
Adductor and psoas muscle tenotomies and obturator nerve neurectomy				2/F	2/F
Total	4	9	22	10	4