Q07.03
BillableArnold-Chiari syndrome with spina bifida and hydrocephalus
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 combining brain tissue extending into the spinal canal, an opening in the spine, and excess fluid in the brain, causing multiple neurological complications.
Coding Tips
- •This code requires documentation of all three components: Arnold-Chiari, spina bifida, AND hydrocephalus
- •Carefully review imaging and clinical notes to confirm all three conditions are present before assigning this code
Clinical Significance
This represents the most complex form of Arnold-Chiari syndrome, combining brain herniation, open spine defects, and increased intracranial pressure. This combination requires the most intensive multidisciplinary management, multiple surgical interventions, and carries the highest risk of severe neurological complications.
Documentation Requirements
- ✓MRI confirmation of cerebellar tonsillar herniation
- ✓Documentation of spina bifida with anatomical location
- ✓Evidence of hydrocephalus and increased intracranial pressure
- ✓Comprehensive neurological deficit assessment
- ✓Multiple surgical intervention history
- ✓Shunt function and complications
- ✓Functional status and quality of life measures
- ✓Complex care coordination documentation
Commonly Confused Codes
Code Hierarchy
└Q07Other congenital malformations of nervous system└Q07.0Arnold-Chiari syndrome└Q07.03Arnold-Chiari syndrome with spina bifida and hydrocephalus
└Q07.03Arnold-Chiari syndrome with spina bifida and hydrocephalus