Congenital morphological disturbances of hair, NEC

This code signifies a birth defect affecting the structure or form of hair, not attributable to other specific genetic syndromes or conditions. It encompasses a range of congenital hair abnormalities where the hair shaft itself is malformed or deviates from normal development.

Apply this code when documentation indicates a patient presents with a congenital hair anomaly, such as pili torti (twisted hair) or monilethrix (beaded hair), that is not part of a broader syndrome. Use when the physician explicitly diagnoses a morphological disturbance of hair present at birth, and no other more specific ICD-10-CM code applies.

• Ensure documentation clearly states the hair disturbance is congenital and not acquired.
• Review the "Excludes1" note carefully; never code this alongside Menkes' kinky hair syndrome (E83.09).
• If the hair anomaly is a manifestation of a known genetic syndrome, code the syndrome first, and then consider if an additional code for the hair disturbance is necessary based on coding guidelines.