J Korean Soc Plast Reconstr Surg.  2006 Sep;33(5):669-671.

A Case of Pressure Sore in Congenital Insensitivity to Pain with Anhidrosis

• Hwang JH
• Park SH
• Yoo SI
• Noh BK
• Kim ES
• Kim KS
• Lee SY

Abstract

PURPOSE
Congenital insensitivity to pain with anhidrosis(CIPA) is a rare form of autosomal recessive peripheral sensory neuropathy. Patients with CIPA show loss of pain sensation, which leads to corneal ulcers and opacities, self-mutilation of the tongue and fingertips, as well as fractures with subsequent joint deformities and chronic osteomyelitis. The purpose of this report is to highlight the fact that pressure sores also are a potential complication of CIPA.
METHODS
This case report describes a patient presenting with pressure sores resulting from CIPA. A 5-year-old boy was referred to our department for the treatment of a 5x5cm sacral pressure sore as a result of a hip spica cast applied for the treatment of a left hip joint dislocation. He had a history suggesting CIPA such as multiple bony fractures, mental retardation, recurrent hyperpyrexia, anhidrosis, and clubbing fingers due to oral mutilation. A microscopic examination of the sural nerve showed mainly large myelinated fibers, a few small myelinated fibers and an almost complete loss of unmyelinated fibers. After wound preparation for two weeks, the exposed bone was covered with two local advancement flaps.
RESULTS
Two weeks later, complete wound healing was achieved. A 16-month follow-up showed no recurrence. However, the patient presented with a new pressure sore on the left knee due to orthosis for the treatment of the left hip joint dislocation.
CONCLUSION
The early diagnosis of CIPA and special care of pressure sores are important for preventing and treating pressure sores resulting from CIPA.

Keyword

Congenital insensitivity to pain with anhidrosis (CIPA); Pressure sore

MeSH Terms

Child, Preschool
Congenital Abnormalities
Dislocations
Early Diagnosis
Fingers
Follow-Up Studies
Hereditary Sensory and Autonomic Neuropathies*
Hip
Hip Joint
Humans
Hypohidrosis
Intellectual Disability
Joints
Knee
Male
Myelin Sheath
Nerve Fibers, Myelinated
Orthotic Devices
Osteomyelitis
Pain Insensitivity, Congenital*
Pressure Ulcer*
Recurrence
Sensation
Sural Nerve
Tongue
Ulcer
Wound Healing
Wounds and Injuries