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Chiari type I malformation with occult tethered cord syndrome in a child: A case report
Yuan Zhou1, Lin Zhu, Yixing Lin
1Department of Neurosurgery, Jinling Hospital, Jinling School of Clinical Medicine, Nanjing Medical University, Jiangsu, China.
Insights
This study presents a case of Chiari type I malformation (CM1) with occult tethered cord syndrome (OTCS) in a pediatric patient. A staged surgical approach involving filum terminale sectioning followed by posterior fossa decompression effectively resolved symptoms and improved imaging findings.
Area of Science:
- Neurosurgery
- Pediatric Neurology
- Spinal Cord Malformations
Background:
- Chiari type I malformation (CM1) and occult tethered cord syndrome (OTCS) are rare congenital spinal cord abnormalities.
- The management of co-occurring CM1 and OTCS in pediatric patients remains controversial, particularly regarding surgical timing and approach.
Observation:
- A 14-year-old presented with complex neurological symptoms including limb pain, numbness, neck and back pain, and urinary frequency.
- Imaging revealed CM1, syringomyelia, and a fatty filum terminale at L2, with the conus medullaris at L1, indicative of OTCS.
Findings:
- A staged surgical strategy was employed: initial sectioning of the filum terminale (SFT) followed by posterior fossa decompression (PFD).
- The patient experienced significant symptom relief, with resolution of pain and numbness, and demonstrated radiological improvement over a 2-year follow-up.
Implications:
- This case highlights the importance of thorough preoperative evaluation for individualized surgical planning in pediatric CM1 and OTCS.
- A staged surgical approach, starting with minimally invasive SFT and followed by PFD, appears beneficial for managing combined CM1 and OTCS, offering positive clinical and imaging outcomes.
Rationale:
Chiari type I malformation (CM1) and occult tethered cord syndrome (OTCS) are considered to be malformations associated with subtle structural abnormalities of the terminal filum. Few studies have reported patients with CM1 and OTCS. Treatment strategy for patients of CM1 associated with OTCS is controversial.
Patient Concerns:
A 14-year-old child was admitted with intermittent pain and numbness in the right upper limb. And he had urinary frequency, neck pain, back pain, and numbness simultaneously. The imaging examinations showed CM1, syringomyelia, small fat in the filum at the level of the L2 vertebral body but the conus medullaris at the aspect of the L1 vertebral body.
Diagnoses:
The child was diagnosed with CM1 associated with OTCS.
Interventions:
Patient underwent sectioning of filum terminale (SFT) under electrophysiological monitoring during the first hospital and posterior fossa decompression (PFD) during the second hospital.
Outcomes:
After first discharge pain of the right upper limb was relieved, but he still felt numbness. And his numbness was relieved after second discharge. The imaging examinations also showed corresponding improvement during the 2-year follow-up period.
Lessons:
For pediatric patients with CM1 and TCS, treatment trouble is not only to choose the staging operation or simultaneous operation but also staging procedures for treatment of 2 lesions. Detailed preoperative evaluation is essential for development of individualized surgical plan. Staging operation of firstly minimally invasive SFT and later PFD may be helpful for such cases owing to its positive effect on both the symptoms and imaging findings.
