Q04.2
BillableHoloprosencephaly
HCC Category Mapping
V28HCC 182 — Spinal Cord Disorders/Injuries
0.282V24HCC 72 — Spinal Cord Disorders/Injuries
0.464ESRDHCC 72 — Spinal Cord Disorders/Injuries
0.000RxHCCHCC 155 — Myelitis and Encephalomyelitis
0.000What This Code Means
A birth defect where the brain fails to properly divide into two hemispheres, resulting in a single brain structure instead of two separate halves.
Coding Tips
- •Specify the severity level if documented (alobar, semilobar, or lobar)
- •This is often associated with facial abnormalities and other congenital anomalies
Clinical Significance
Holoprosencephaly is a severe brain malformation where the cerebrum fails to divide into hemispheres, causing significant neurological and developmental disabilities. This condition requires intensive multidisciplinary care and has variable prognosis depending on severity.
Documentation Requirements
- ✓Documentation of failed brain hemisphere division on neuroimaging
- ✓Specific type of holoprosencephaly if identified (alobar, semilobar, lobar)
- ✓Associated craniofacial abnormalities documentation
- ✓Neurological examination findings and developmental assessment
- ✓Seizure history and neurological complications
- ✓Endocrine dysfunction assessment (hypothalamic-pituitary)
- ✓Associated genetic testing results if performed
- ✓Comprehensive care plan and prognosis documentation
Commonly Confused Codes
Q04.0 — Corpus callosum malformation involves different interhemispheric structureQ04.1 — Arhinencephaly specifically affects olfactory structuresQ04.3 — Other brain reduction deformities don't involve hemisphere divisionQ75.0 — Craniosynostosis affects skull, not brain divisionQ87.0 — Malformation syndromes may include but are broader diagnoses