G11.5
BillableHypomyelination - hypogonadotropic hypogonadism - hypodontia
HCC Category Mapping
V28HCC 200 — Cerebellar Ataxia and Other Degenerative Diseases of Nervous System
0.262V24HCC 72 — Spinal Cord Disorders/Injuries
0.464ESRDHCC 72 — Spinal Cord Disorders/Injuries
0.000What This Code Means
Hypomyelination-hypogonadotropic hypogonadism-hypodontia is a rare genetic syndrome characterized by underdeveloped nerve insulation, hormone deficiency, and missing or underdeveloped teeth.
Coding Tips
- •Code each component of the syndrome separately if they are clinically significant and documented
- •Document any associated neurological symptoms or developmental delays
Clinical Significance
Hypomyelination-hypogonadotropic hypogonadism-hypodontia (4H syndrome) is an extremely rare genetic leukodystrophy affecting white matter formation, hormonal function, and dental development. This multisystem condition requires coordination across neurology, endocrinology, and dentistry, representing substantial complexity and cost in patient management.
Documentation Requirements
- ✓Brain MRI demonstrating hypomyelination pattern
- ✓Endocrine evaluation documenting hypogonadotropic hypogonadism
- ✓Dental examination documenting hypodontia (missing or underdeveloped teeth)
- ✓Genetic testing results (POLR3A or POLR3B mutations) if available
- ✓Neurological examination documenting cerebellar signs and motor dysfunction
- ✓Developmental history and current functional status
Commonly Confused Codes
G11.6 — Leukodystrophy with vanishing white matter disease: different leukodystrophy with distinct MRI patternE23.0 — Hypopituitarism: captures the hypogonadotropic component but misses neurological featuresG11.8 — Other hereditary ataxias: less specific and should not be used when 4H syndrome is confirmedE75.25 — Metachromatic leukodystrophy: different type of leukodystrophy with different clinical features
Code Hierarchy
└G11Hereditary ataxia└G11.5Hypomyelination - hypogonadotropic hypogonadism - hypodontia
└G11.5Hypomyelination - hypogonadotropic hypogonadism - hypodontia