J Korean Orthop Assoc.  1999 Dec;34(6):1175-1178.

Complication of Lateral Condyle Fracture of Humerus in a Patient with Hereditary Sensory Autonomic Neuropathy Type IV: A Case Report

• Kang HJ
• Sung SY
• Shin SJ
• Park HW

Abstract

Hereditory Sensory Automnomic Neuropathy (HSAN) is a rare disorder. Five types of HSAN have been described by Dyck et al. Congenital insensitivity to pain with anhidrosis is classified HSAN Type IV. The clinical features include inability to sweat, causing defective thermoregulation, and congenital analgesia, which leads to orthopedic complications such as osteomyelitis, fractures, self mutilation and neuropathic arthropathies. Abnormal findings on nerve biopsy, virtual absence of unmyelinated nerve fibers, have been reported. We report a 5-year-old boy who presented with congenital insensitivity to pain, anhidrosis with fracture of lateral condyle of humerus and its complication.

Keyword

Congenital insensibility to pain; Anhidrosis; Fracture; Complication

MeSH Terms

Biopsy
Body Temperature Regulation
Child, Preschool
Hereditary Sensory and Autonomic Neuropathies
Humans
Humerus*
Hypohidrosis
Male
Nerve Fibers, Unmyelinated
Orthopedics
Osteomyelitis
Pain Insensitivity, Congenital
Self Mutilation
Sweat