Management of Chiari type 0 malformation: A case study and literature review of surgical approaches, clinical and radiological outcomes

Abstract

Background

Chiari malformation type 0 (CM0) is a rare subtype of Chiari malformations characterized by typical Chiari type 1 symptoms without cerebellar tonsillar herniation. There is currently no consensus on surgical treatment for CM0.

Case

We report a 53-year-old female presenting with neck and left upper limb pain since March 2022. Neurological examination was unremarkable, and MRI revealed a long-segment syrinx extending from C2 to T7 without tonsillar herniation, confirming CM0. She underwent foramen magnum decompression (FMD), with intraoperative findings of adequate tonsillar pulsations and satisfactory cerebrospinal fluid (CSF) flow. Postoperatively, the patient reported significant symptom relief and a six-month follow-up MRI showed reduced syrinx size.

FMD is the primary treatment strategy to restore CSF flow and alleviate symptoms, and several adjunct procedures have been described. A literature review of eight studies (33 patients) identified various surgical techniques, including C1 laminectomy, duraplasty, and intradural lysis of adhesions. Despite positive clinical outcomes, a standardized surgical protocol remains lacking. The heterogeneity in CM0 presentation and management necessitates individualized treatment strategies.

Conclusion

CM0 presents significant diagnostic and therapeutic challenges due to its rarity and the variability in patient presentations and surgical outcomes. The standard surgical approach often centers on FMD, typically supplemented with additional techniques such as C1 laminectomy and duraplasty. In this report, we present a case of CM0 in which the patient demonstrated significant symptomatic and radiological improvement following FMD alone without the need for duraplasty. These findings suggest that FMD alone may be sufficient in select cases of CM0.