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

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

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

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

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

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

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

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

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

12/18/2019 Nejat Akalan MD, PhD

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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