Aspectos ortopédicos de la Analgia congênita Hipohidrótica

A propósito de dos casos

Abstract

Hypohidrotic congenital analgesia with anhidrosis is an autosomal recessive form of sensory and autonomic neuropathy, not only uncommon, but also difficult to treat. Diag nostic criteria are: universal a bsence of sensitivity to pain, ârhidrosis, bouts of hyperpyrexia from the first days of life, and mental retardation. We report two patients with Hypohidrotic congenital analgesia (HCA): a nine-year old girl and a twelve-year old boy. Both presented fractu res, osteomyelitis, osteoarticular deformities and Charcot arth ropathy. The purpose of our work was to present the diagnostic and therapeutic difficulties of Hypohidrotic congenital analgesia.