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Type 1.5 Split Cord Malformation : A New Theory of Pathogenesis
Mengchun Sun1,2, Benzhang Tao1, Tianbao Luo3
1Department of Neurosurgery, the First Medical Center of Chinese PLA General Hospital, Beijing, China.
Journal of Korean Neurosurgical Society
|November 21, 2021
Summary
This study details two cases of type 1.5 split cord malformation (SCM), a rare condition combining features of types I and II SCM. A novel pathogenesis theory involving uneven regression is proposed for this complex spinal cord anomaly.
Area of Science:
- Neurosurgery
- Developmental Biology
- Spinal Cord Anomalies
Background:
- Split cord malformation (SCM) is a congenital anomaly characterized by the division of the spinal cord.
- Type 1.5 SCM represents an overlap between Type I (dorsal septum) and Type II (ventral septum) SCM.
- Understanding the pathogenesis of Type 1.5 SCM is crucial for accurate diagnosis and treatment.
Observation:
- Two patients, a 52-year-old woman and a 9-year-old boy, presented with Type 1.5 SCM.
- Both exhibited hemicords within a single dural sac, with variations in bony septum location (dorsal or ventral) and fibrous extensions.
- Surgical intervention involved septum removal, spinal cord untethering, and filum terminale sectioning.
Findings:
- Microsurgical procedures confirmed extradural partial bony septa and intact dural sacs in both cases.
- Patients experienced no new neurological deficits post-operatively.
- Literature review supports the overlap of Type I and II SCM, validating Type 1.5 SCM as a distinct entity.
Implications:
- Type 1.5 SCM is proposed as a standardized term for overlapping SCM presentations.
- A new pathogenetic theory suggests uneven distribution and regression during development.
- The extent of meninx primitiva condensation may influence the development of bony septa and fibrous extensions in Type 1.5 SCM.
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