Arnold-Chiari syndrome with spina bifida and hydrocephalus

This code identifies a specific type of Arnold-Chiari malformation, a structural defect in the cerebellum, that is complicated by both spina bifida and hydrocephalus. It describes a congenital condition where brain tissue extends into the spinal canal, accompanied by an incomplete closure of the spinal column and an accumulation of cerebrospinal fluid in the brain.

Use this code when documentation clearly indicates a diagnosis of Arnold-Chiari malformation (specifically Type II, also known as Chiari II malformation) in conjunction with both spina bifida and hydrocephalus. This is typically seen in pediatric patients with complex congenital neurological anomalies. The physician's diagnostic statement must explicitly link all three conditions.

• Ensure the documentation specifies the presence of both spina bifida and hydrocephalus in addition to the Arnold-Chiari syndrome.
• Do not use this code if only Arnold-Chiari syndrome with spina bifida (Q07.02) or Arnold-Chiari syndrome with hydrocephalus (Q07.01) is documented.
• Consider additional codes for specific types of spina bifida (e.g., myelomeningocele) or hydrocephalus if further detail is provided.