Long-term surgical outcome of Chiari type-I malformation-related syringomyelia: an experience of tertiary referral hospital

Neurol Res. 2022 Apr;44(4):299-310. doi: 10.1080/01616412.2021.1981104. Epub 2021 Sep 24.

Abstract

Objective: Syringomyelia is a common condition seen in patients with Chiari type-I malformation (CM1). The purpose of this retrospective study was to evaluate the long-term clinical and radiological outcomes of posterior fossa decompression with duraplasty (PFDD) with coagulation of tonsillar ectopia in consecutive surgically treated adult patients with CM1-related syringomyelia (CRS).

Methods: Over 9 years' duration (1993-2001), medical charts of diagnosed patient with CM1 at our neurosurgical center were reviewed retrospectively. This study included adult patients with CM1 who had syringomyelia and underwent PFDD with coagulation of tonsillar ectopia surgery. The differences between the pre- and postoperative syrinx/cord ratio (S/C), the syrinx length, and the regression of herniated cerebellar tonsils on coronal and midsagittal MRIs were evaluated.

Results: A total of 87 surgical procedures (46 primary operations, 7 ventriculoperitoneal shunts, and 34 additional operations) for CRS were performed on 24 males and 22 females. The mean preoperative S/C was 0.59 ± 0.12. The means of regression in herniated cerebellar tonsils on mid-sagittal and coronal images were 11.8 ± 2.3 mm and 10.2 ± 2.2 mm (p < 0.0001), respectively. 35 (76.1%) patients were discharged after showing signs of recovery or improvement. Different complications occurred in 16 (34.8%) patients. Negative correlations were noticed between postoperative recovery/improvement and the long symptoms' duration, the herniated tonsils' extent, S/C, and the persistence of the herniated tonsils on the coronal images.

Conclusion: Early diagnosis of patients with CRS can improve surgical outcomes. Due to its efficacy in resolving clinical symptoms and syrinx cavities, PFDD is still an optimal surgical approach for CRS.

Keywords: Chiari type-I malformation; Chiari type-I malformation-related syringomyelia; long-term outcomes; surgical treatment; syringomyelia.

MeSH terms

  • Adult
  • Arnold-Chiari Malformation / complications
  • Arnold-Chiari Malformation / diagnostic imaging
  • Arnold-Chiari Malformation / pathology
  • Arnold-Chiari Malformation / surgery*
  • Cerebellar Diseases / diagnostic imaging
  • Cerebellar Diseases / pathology
  • Cerebellar Diseases / surgery
  • Cranial Fossa, Posterior / diagnostic imaging
  • Cranial Fossa, Posterior / pathology
  • Cranial Fossa, Posterior / surgery*
  • Decompression, Surgical*
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Middle Aged
  • Neurosurgical Procedures*
  • Outcome Assessment, Health Care*
  • Retrospective Studies
  • Syringomyelia / diagnostic imaging
  • Syringomyelia / etiology
  • Syringomyelia / pathology
  • Syringomyelia / surgery*
  • Tertiary Care Centers
  • Ventriculoperitoneal Shunt
  • Young Adult