1. Growth of the Spinal Cord and when the Intraspinal Pathology should be addressed
Nejat Akalan, MD, PhD
Department of Neurosurgery
Hacettepe University, Ankara

2. Spinal cord growth Embryonic period Asymmetrical Post-natal period
Both the spine and the spinal cord
lengthen by an order of
magnitude during growth
12/18/2019
Nejat Akalan MD, PhD

3. Congenital spinal anomalies and “tethered cord”, definitions
Surgical intervention, why and when?
Intraspinal anomalies associated with scoliosis
Algorithms for treatment
12/18/2019
Nejat Akalan MD, PhD

4. Congenital Spinal Anomalies
Neuroectoderm
Mesoderm
Neural Tissue
Bone
Soft tissue
Occult Spina Bifida
(Closed dysraphism)
Spinal Lipoma
Diastematomyelia
Dermal sinus
Short-fatty filum
Appert Spina Bifida
(Open dysraphism)
(Neural tube defects)
Myelomeningocele
Scoliosis
Hemi vertebra
Butterfly vertebra
Klippel-Feil
Craniovertebral junction
anomalies
12/18/2019
Nejat Akalan MD, PhD

5. Progressive Neurological Impairment Asymptomatic Occult Spina Bifida
(Closed dysraphism)
Spinal Lipoma
Diastematomyelia
Dermal sinus
Short-fatty filum
Progressive
Neurological
Impairment
Neuromusculoskeletal Syndrome
12/18/2019
Nejat Akalan MD, PhD

6. Tethered cord syndrome
Asymptomatic
Progressive
Neurological
Impairment
Tethered cord syndrome
“constellation of symptoms and signs of motor and sensory neuron dysfunction attributable to abnormally increased tension on the spinal cord”
Neuromusculoskeletal Syndrome
12/18/2019
Nejat Akalan MD, PhD

7. Tethered Cord Syndrome
symptoms
signs
radiological features
back pain
bladder dysfunction
leg weakness
atrophy
loss of dtr’s
sensory loss
lipomyelomeningocele
lipoma of the terminal filum
thickened terminal filum
progressive scoliosis
equinovarus
equinovalgus
low-lying conus
12/18/2019
Nejat Akalan MD, PhD

8. Surgery Prophylactic Progressive Neurological Prevent deterioration
Asymptomatic
Prophylactic
Progressive
Neurological
Impairment
Prevent deterioration
Reversal of the symptoms
Neuromusculoskeletal Syndrome
12/18/2019
Nejat Akalan MD, PhD

9. Decision for intervention
Unclear Definition of the Syndrome
Unknown Natural History
Uncertain Pathogenesis
Lack of Accurate Clinical or Diagnostic Tests
Surgery
Decompression and Untethering
12/18/2019
Nejat Akalan MD, PhD

10. 12/18/2019
Nejat Akalan MD, PhD

11. terminal filum, nerve root, dentate ligaments, dura
12/18/2019
Nejat Akalan MD, PhD

12. Spinal Malformations and Scoliosis
“20–58% of cases of congenital scoliosis
associated with intraspinal abnormalities”
Related to spinal malformation
Co-existing pathology
12/18/2019
Nejat Akalan MD, PhD

13. Spinal Malformations and Scoliosis
Occult spinal dysraphism
Vertebral anomalies
Diastematomyelia
(Split-cord malformations)
Failure of formation
Failure of segmentation
Syringomyelia
İdiopathic
Chiari malf.
Appert spinal dysraphism
Myelomeningocele
12/18/2019
Nejat Akalan MD, PhD

14. Decision making Stable vs. progresive Casual relationship Primary aim
Sequence and timing
12/18/2019
Nejat Akalan MD, PhD

15. Diastematomyelia (Split-cord malformations)
SCM I and II
An adhesion between the ectoderm and endoderm leads to an endomesenchymal tract that bisects the spinal cord.
frequent association with
secondary spinal anomalies
12/18/2019
Nejat Akalan MD, PhD

16. Diastematomyelia (Split-cord malformations)
Presentation
Isolated
Segmentation anomalies
Asymptomatic
NMS Syndrome
Scoliosis
12/18/2019
Nejat Akalan MD, PhD

17. Unclear Definition of the Syndrome
Scenario 1
Incidental
Normal N&P Exam.
Isolated SCM I or II
Tethering ?
Axial growth
Age
Unclear Definition of the Syndrome
Unknown Natural History
Uncertain Pathogenesis
Lack of Accurate Clinical or Diagnostic Tests
12/18/2019
Nejat Akalan MD, PhD

18. Scenario 2 Stable Isolated +NMS Syndrome Progression ? 12/18/2019
Nejat Akalan MD, PhD

19. Scenario 3 Stable Prophylactic ? Scoliosis Progressive Type
Localization
12/18/2019
Nejat Akalan MD, PhD

20. Spinal deformity Neural compromise & Correction due to traction SCM
Untethering
12/18/2019
Nejat Akalan MD, PhD

21. Syringomyelia Longitudinal cavitations within spinal cord
Posterior fossa pathology
Chiari malformations
Craniovertebral junction anomaly
Assoc. with spinal dysraphism
Idiopathic
Inflammatory
Traumatic
12/18/2019
Nejat Akalan MD, PhD

22. Syringomyelia Progressive neural impairment Progressive scoliosis
Morbidity during correction
Treatment
Aims to reverse the pathophysiological mechanism
12/18/2019
Nejat Akalan MD, PhD

23. Treatment + Hydrocephalus Shunt Follow-up (MR) 12/18/2019
Nejat Akalan MD, PhD

24. Treatment Hydrocephalus Chiari I-II Shunt Decompression 12/18/2019
Nejat Akalan MD, PhD

25. Decompression + Fusion
Treatment
Craniovertebral junction anomalies
Decompression + Fusion
12/18/2019
Nejat Akalan MD, PhD

26. İdiopatic (?) Syringo-subarachnoid, Syringo-peritoneal shunt
12/18/2019
Nejat Akalan MD, PhD

27. Syringomyelia and scoliosis
Surgery for syrinx
Follow-up
Correction
12/18/2019
Nejat Akalan MD, PhD

28. Defining the casual relationship is the key for appropriate sequence of the surgical approach in complex spinal malformations
There is not enough scientific evidence to validate the contemporary practice in treating joint neurosurgical and orthopedic malformations
12/18/2019
Nejat Akalan MD, PhD