A Rare Course of Chiari Malformation With Large Syringomyelia Presenting at 54 Years Old

Masato Tanaka1,2, Sneha Sharma3, Kushal H Gori1

  • 1Orthopedic Surgery, Okayama Rosai Hospital, Okayama, JPN.

Cureus
|March 6, 2025
PubMed

Insights

This study reports a rare case of Chiari malformation type 1 (CM1) with large syringomyelia in a 54-year-old man. Surgical intervention led to significant recovery, demonstrating effective treatment for this rare condition.

Area of Science:

  • Neurosurgery
  • Neurology
  • Radiology

Background:

  • Chiari malformation type 1 (CM1) is a congenital neurological condition typically presenting in younger individuals.
  • Symptoms often include headaches, hypalgesia, and temperature sensation loss, posing a significant burden.
  • Late-onset CM1, especially with extensive syringomyelia, is exceptionally rare.

Observation:

  • A 54-year-old male presented with low back pain, lower extremity weakness, sensory deficits, and incontinence.
  • Cervical MRI revealed CM1 with a large syringomyelia extending from C1 to T11.
  • The patient exhibited a widened cervical canal due to chronic spinal cord expansion.

Findings:

  • Surgical treatment involved foramen magnum decompression and syringosubarachnoid shunting using O-arm navigation.
  • The patient experienced substantial recovery of muscle strength and sensory function within one year.
  • Cervical Japanese Orthopedic Association (JOA) scores improved significantly from 11/17 to 16/17.

Implications:

  • This case highlights that CM1 with significant syringomyelia can occur in older adults.
  • Foramen magnum decompression is effective even in long-standing syringomyelia cases.
  • O-arm navigation offers excellent outcomes for complex CM1 and syringomyelia surgeries.