Orthopedic management in autosomal recessive spastic ataxia of Charlevoix-Saguenay.
OBJECTIVE
To review the orthopedic management of choice in patients having autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS).


METHODS
A retrospective study from April 1978 to April 1997.


METHODS
Centre hospitalier de la Sagamie, Chicoutimi, Que.


METHODS
A review of the records of patients having ARSACS who were identified in the registry of the Neuromuscular Diseases Clinic at the Centre hospitalier de la Sagamie revealed 26 patients who received surgical orthopedic treatment. Initially, the patients were offered conservative treatment, which consisted of physiotherapy sessions, the wearing of an ankle-foot orthosis or serial casting. When this was unsuccessful, foot surgery was considered.


RESULTS
During the study period, 49 orthopedic procedures were done, including 24 triple arthrodeses; of these, 9 were combined with lengthening of the Achilles tendon. Most triple arthrodeses were done in patients between the ages of 30 and 49 years. The surgical options evolved during the study from Lambrinudi arthrodesis through arthrodesis of the ankle to triple arthrodesis with lengthening of the Achilles tendon.


CONCLUSIONS
As a complement to conservative treatment, surgery has a place in the care of patients with ARSACS. Clinically, the most effective surgical procedures are triple arthrodesis with percutaneous lengthening of the Achilles tendon and adductor and psoas tenotomies combined with neurectomy of the obturator nerve for perineal hygiene.
Bouchard
M
M
Department of Surgery, Université Laval, Quebec. marcbouchard@hotmail.com
Langlois
G
G
D000125:Achilles Tendon / Q000601:surgery; D000293:Adolescent; D000328:Adult; D000843:Ankle Joint / Q000601:surgery; D001174:Arthrodesis; D002648:Child; D002869:Chromosome Aberrations / Q000235:genetics*; D025063:Chromosome Disorders; D002899:Chromosomes, Human, Pair 9; D005260:Female; D005808:Genes, Recessive / Q000235:genetics*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009128:Muscle Spasticity / Q000235:genetics / Q000601:surgery*; D010348:Patient Care Team; D012189:Retrospective Studies; D013132:Spinocerebellar Degenerations / Q000235:genetics / Q000601:surgery*