Short answer · Medically reviewed summary · Last updated: 2026-05-08
Congenital Insensitivity to Pain with Anhidrosis (CIPA), also known as Hereditary Sensory and Autonomic Neuropathy type IV (HSAN IV), is classified under ICD-10 code G60.89 (Other hereditary and idiopathic neuropathy). In the older ICD-9 coding system, this condition is typically categorized under 356.9 (Unspecified hereditary and idiopathic peripheral neuropathy). What is the clinical classification of CIPA? CIPA is an extremely rare autosomal recessive disorder characterized by the inability to feel pain, temperature, and the inability to sweat (anhidrosis).
ICD10 code of Congenital Insensitivity To Pain With Anhidrosis (CIPA) and ICD9 code
ICD-10 and ICD-9 codes for Congenital Insensitivity To Pain With Anhidrosis (CIPA), with classification details for clinicians, coders and patients.
Congenital Insensitivity to Pain with Anhidrosis (CIPA), also known as Hereditary Sensory and Autonomic Neuropathy type IV (HSAN IV), is classified under ICD-10 code G60.89 (Other hereditary and idiopathic neuropathy). In the older ICD-9 coding system, this condition is typically categorized under 356.9 (Unspecified hereditary and idiopathic peripheral neuropathy).
What is the clinical classification of CIPA?
CIPA is an extremely rare autosomal recessive disorder characterized by the inability to feel pain, temperature, and the inability to sweat (anhidrosis). Because Congenital Insensitivity to Pain with Anhidrosis affects the peripheral nervous system, these coding designations help clinicians track the neurological and autonomic manifestations of the disease. Managing Congenital Insensitivity to Pain with Anhidrosis requires precise coding for insurance and clinical record-keeping to ensure patients receive multidisciplinary care.
How is Congenital Insensitivity to Pain with Anhidrosis diagnosed?
Diagnosis of Congenital Insensitivity to Pain with Anhidrosis is primarily clinical, often triggered by unexplained fevers in infancy or self-mutilation behaviors. Genetic testing is the gold standard for confirmation, specifically looking for mutations in the NTRK1 gene. Key diagnostic features include:
- Complete absence of pain perception leading to recurrent injuries.
- Anhidrosis (lack of sweat) resulting in recurrent, life-threatening hyperpyrexia.
- Intellectual disability in many, though not all, patients.
- Absent deep tendon reflexes and diminished autonomic responses.
Is Congenital Insensitivity to Pain with Anhidrosis hereditary?
Yes, Congenital Insensitivity to Pain with Anhidrosis is an autosomal recessive condition. This means that for a child to be born with Congenital Insensitivity to Pain with Anhidrosis, both parents must be carriers of a mutation in the NTRK1 gene. Genetic counseling is essential for families to understand the 25% recurrence risk for future pregnancies.
Next steps
- Consult with a pediatric neurologist or a specialist in autonomic disorders to manage systemic symptoms.
- Connect with the 6 members currently sharing their experiences with Congenital Insensitivity to Pain with Anhidrosis on DiseaseMaps.org.
- Maintain a strict environmental temperature control plan to prevent hyperpyrexia.
- Discuss genetic testing for family planning with a certified genetic counselor.
Medical disclaimer: This information is for educational purposes only and does not replace professional medical advice, diagnosis, or treatment.
References
- NIH Genetic and Rare Diseases Information Center (GARD): HSAN IV.
- Orphanet: Hereditary sensory and autonomic neuropathy type 4 (ORPHA:3000).
- OMIM (Online Mendelian Inheritance in Man): #243000 (Neuropathy, Hereditary Sensory and Autonomic, Type IV).
- National Library of Medicine (PubMed): Clinical reviews on NTRK1-related disorders.
