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). DESIGN: A retrospective study from April 1978 to April 1997. SETTING: Centre hospitalier de la Sagamie, Chicoutimi, Que. PATIENTS: A review of the records of patients having ARSACS who were identi fied in the registry of the Neuromuscular Diseases Clinic at the Centre hos pitalier de la Sagamie revealed 26 patients who received surgical orthopedi c treatment. Initially, the patients were offered conservative treatment, w hich consisted of physiotherapy sessions, the wearing of an ankle-foot orth osis or serial casting. When this was unsuccessful, foot surgery mas consid ered. RESULTS: During the: Study period, 49 orthopedic procedures were done, incl uding 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 arth rodesis 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 surgic al procedures are triple arthrodesis with percutaneous lengthening of the A chilles tendon and adductor and psoas tenotomies combined with neurectomy o f the obturator nerve for perineal hygiene.