E-ISSN: 1019-5157
ISSN: 2651-5024
Research
Dysraphic Tethering Syringomyelia in Split Cord Malformation: Proposal of a Novel TerminologyRostral Junctional Segmental Syringomyelia
Metin Orakdogen✉ ,
Orkhan Mammadkhanli ,
Ahmet Tolgay Akinci
Ahmet Tolgay Akinci
Neurosurgery, Trakya Üniversitesi T?p Fakültesi
DOI: 10.5137/1019-5149.JTN.50163-25.4
Article in Press
Corresponding Author:
Metin Orakdogen (morakdogen@gmail.com)
Abstract
Aim
This retrospective study aimed to investigate the radiological and clinical features of syringomyelia in patients with spinal dysraphism accompanied by split cord malformation (SCM). A secondary objective was to propose a specific terminology to describe different syringomyelia patterns.
Material and Methods
We reviewed 35 patients with SCM who were treated for spinal dysraphism and tethered cord syndrome between 2014 and 2020. Radiological and clinical features were categorized into four groups: SCM (+) Syrinx (−), SCM Type II Syrinx (+), SCM Type I Syrinx (+), and Composite SCM Syrinx (+).
Results
In the SCM (+) Syrinx (−) group (n = 5), all patients presented with closed dysraphism and had no neurological deficits, Chiari malformation, or hydrocephalus.
SCM Type II Syrinx (+) cases (n = 20) exhibited rostral, short-segment syrinxes, primarily located at the thoracolumbar junction.
The SCM Type I Syrinx (+) (n = 5) and Composite SCM Syrinx (+) (n = 5) groups demonstrated a higher incidence of open dysraphism, neurological deficits, Chiari malformation, hydrocephalus, scoliosis, and vertebral anomalies.
Conclusion
Type I and composite SCMs are typically associated with open dysraphism, neurological deficits, and complex vertebral anomalies. In contrast, Type II SCMs primarily exhibit short-segment, rostral syrinxes with fewer associated anomalies. Based on these consistent characteristics, we propose the term rostral (proximal) junctional segmental syringomyelia to describe the syringomyelia pattern observed in Type II SCM, under the broader concept of dysraphic tethering syringomyelia.
This retrospective study aimed to investigate the radiological and clinical features of syringomyelia in patients with spinal dysraphism accompanied by split cord malformation (SCM). A secondary objective was to propose a specific terminology to describe different syringomyelia patterns.
Material and Methods
We reviewed 35 patients with SCM who were treated for spinal dysraphism and tethered cord syndrome between 2014 and 2020. Radiological and clinical features were categorized into four groups: SCM (+) Syrinx (−), SCM Type II Syrinx (+), SCM Type I Syrinx (+), and Composite SCM Syrinx (+).
Results
In the SCM (+) Syrinx (−) group (n = 5), all patients presented with closed dysraphism and had no neurological deficits, Chiari malformation, or hydrocephalus.
SCM Type II Syrinx (+) cases (n = 20) exhibited rostral, short-segment syrinxes, primarily located at the thoracolumbar junction.
The SCM Type I Syrinx (+) (n = 5) and Composite SCM Syrinx (+) (n = 5) groups demonstrated a higher incidence of open dysraphism, neurological deficits, Chiari malformation, hydrocephalus, scoliosis, and vertebral anomalies.
Conclusion
Type I and composite SCMs are typically associated with open dysraphism, neurological deficits, and complex vertebral anomalies. In contrast, Type II SCMs primarily exhibit short-segment, rostral syrinxes with fewer associated anomalies. Based on these consistent characteristics, we propose the term rostral (proximal) junctional segmental syringomyelia to describe the syringomyelia pattern observed in Type II SCM, under the broader concept of dysraphic tethering syringomyelia.
Keywords
Filum terminale
syringomyelia
split cord malformation
spinal dysraphism