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Syrinx, Chiari, Tether Need for Treatment James M. Drake FRCSC ICEOS Toronto November 2010 The Hospital for Sick Children University of Toronto, Canada Objectives

Learn about the nervous system embryology, and common anomalies associated with early onset scoliosis, their diagnosis and management. Disclosures - none 2,5 yr old female infantile scoliosis Seen in orthopedic clinic HSC July . MRI 3-6 months ordered

Seen at Shriners USA 2 weeks, MRI conus L2-L3 Recommended de-tethering operation by Shriner neurosurgeon Emergency second opinion neurosurgery HSC Exam normal (? Absent lt ankle jerk) 11 ribs, lumbarized S1, filum normal on MRI Recommended observation embryology

amnion Primitive streak forms in the midline of bilaminar disc Cells enter primitive streak: midline cells form notochord,

paramedian cells form mesenchyme yolk sac embryology: neurulation neural folds neural plate thickens

plate flexes midline sinks into neural groove canalization / retrogressive differentiation cell agglomeration, vacuolation and involution

Primary Neurulation Secondary Neurulation Between 13 and 18 weeks' gestation, the Conus Medullaris was situated at the level of the L4 vertebra, or more caudally, in 100% of the fetuses. At term, all fetuses showed the

CM above L2. A distinct ascent of the CM was detected between 13 and 40 weeks' gestation. Zalel Y, Lehavi O, Aizenstein O, Achiron R. Development of the fetal spinal cord: time of ascendance of the normal conus medullaris as detected by sonography. J Ultrasound Med. 2006 Nov;25(11):1397-401; quiz 1402-3. filum lipoma: failure of involution of distal band distal lipomas are caused by failure of canalization

and retrogressive differentiation fetal MR: lipomyelomeningocele skin covered cord enters sac sagittal

coronal axial Tethered Spinal Cord A concept no definitive test Low lying spinal cord under tension Causes low level spinal cord ischemia &

dysfunction (rarely if ever measured) Leg muscle atrophy, weakness, foot deformity, pain, bladder dysfunction Tethered Spinal Cord 10 yr old boy, hairy patch on back Neurologically intact. Still same 5 yrs later

Plays soccer. (9 yr old Conclusions. The study results suggested that spinal cord untethering may be unnecessary in patients with MM who are undergoing scoliosis corrective surgery and do not present with

clinical symptoms of a tethered cord, even though tethering is radiographically demonstrated. MYELOCYSTOCELE 1 Yr of age, rapidly progressive scoliosis Repeat surgery for un-tethering

Tethered Cord and Scoliosis Diverse spectrums of abnormalities Causal relationships tenuous and risks of tethering/de-tethering not well understood Case by case decision making taking into account Degree of congential abnormality

Degree of pre-existing neurlogical impairment Magnitude of correction Age 4 Age 12 6 yr old girl

21 deg curve, Chiari I, Syringomyelia Chari 1 decompression, subdural Then VP shunt. Curve same not braced 4 yr old boy, foot drop, loss of pain/temp Absent abdominal reflexes

13 yr old female, arthrogryposis, scoliosis. Previous history of palatal insufficiency, mechanical. 2 months post op 5 months post op

Eule JM, Erickson MA, O'Brien MF, Handler M. Chiari I malformation associated with syringomyelia and scoliosis: a twenty-year review of surgical and nonsurgical treatment in a pediatric population. Spine (Phila Pa 1976). 2002 Jul 1;27(13):1451-5. CONCLUSION: Early decompression of Chiari I malformation with syringomyelia and scoliosis resulted in improvement or stabilization of the spinal deformity in 5 cases. Each of these patients underwent decompression before 8 years of age

and before the curve was severe. However, this series represents a few patients demonstrating this trend, and further follow-up and investigation are warranted. Childs Nerv Syst. 2006 Oct;22(10):1351-4. Scoliosis in a child with Chiari I malformation and the absence of syringomyelia: case report and a review of the literature. Tubbs RS, Doyle S, Conklin M, Oakes WJ.

CASE REPORT: We report a child with Chiari I malformation and scoliosis who presented with Valsalva-induced headache/neck pain. MRI revealed no syringomyelia or hydrocephalus. Radiographs revealed that her scoliotic curvature was approximately 13 degrees and was a single levoscoliotic curve. This patient underwent a posterior fossa decompression with duraplasty for her Valsalva-induced pain. Postoperatively, she had resolution of her pain and there has been no progression of her scoliosis at 3 years follow-up. PROGNOSIS: Intriguingly, and scattered throughout the medical literature, many have noted cases of scoliosis in patients with only a Chiari I malformation and no syringomyelia. Moreover, experimental studies have induced scoliosis in animals after compression of the dorsal columns. After a review of the medical literature regarding a

potential cause and effect of herniated hindbrain-induced scoliosis in the absence of syringomyelia, this association although rare, does seem plausible. Childs Nerv Syst. 2004 May;20(5):341-8. International survey on the management of Chiari I malformation and syringomyelia. Schijman E, Steinbok P. RESULTS: Of 246 questionnaires distributed, 76 (30.8%) were completed and returned. There was a consensus that no operation should be carried out in asymptomatic patients with a Chiari I malformation, unless there is associated syringomyelia. There was a

consensus that decompression of the Chiari malformation should be performed in patients with scoliosis when syringomyelia is present, and the majority decompressed the Chiari malformation in scoliotic patients even in the absence of syringomyelia. Suboccipital decompression was the standard surgical procedure for Chiari I malformations. The majority of respondents favored routine dural opening at surgery and closure with a pericranial or synthetic patch graft. In the case of a persistent or progressive syrinx after suboccipital decompression, the majority recommended shunting of the syrinx to the subarachnoid space or to the pleural cavity. CONCLUSION: There continues to be much variation in the management of the Chiari I malformation.

3 monthg female Multiple congenital anomoalies Chromosome 4 deletion, Wolf Hirschorn Syn MRI 2 yrs Clinically well Syringomyelia and Scoliosis

Chiari I, syringomyelia, and scoliosis commonly associated, often good response to surgery, especially if early Chairi I and scoliosis without syringomyelia more controversial Smaller syrinxes (dilatation of central canal) in absence of any clear pathogenetic mechanism are very common, usually benign, rarely seem

to require interention_ Whole group CP CP/dystonia

Head injury other Mean 18.40

20.27 15.54 19.77 18.38

Median 11.88 12.26 11.76

5.7 18.48 Table 2 Mean and median annual progression rate of Cobb angle (degrees per year) Development of scoliosis

Patient 1 Patient 3 Patient 4 180 Patient 6 Patient 7

Patient 8 160 Patient 9 Patient 10 Cobb angle

140 Patient 12 Patient 13 Patient 14 120

Patient 16 Patient 18 100 Patient 19 Patient 20

Patient 21 80 Patient 22 Patient 23 60

Patient 24 Patient 25 Patient 27 40 Patient 29

Patient 31 20 Patient 33 Patient 34 Patient 35

0 Patient 37 0 100

200 Age (months) 300 400 Patient 38

Patient 39 Patient 40 Patient 41 Patient 42 Age 9 Pump inserted Age 11

Age 8 Age 10 Age 12 Age 16

Above patient died respiratory failure Scoliosis etiology ? Intrathecal baclofen human model of scoliosis development ?? Do Baclofen Pumps Cause Scoliosis? Yes

Spine 2007 Nov 15;32(24):2745-50. Progression of scoliosis in patients with spastic quadriplegia after the insertion of an intrathecal baclofen pump. No J Pediatr Orthop. 2008 Sep;28(6):684-7. The impact of intrathecal baclofen on the natural history of scoliosis in cerebral palsy.

7 year old neurenteric cyst excised at birth, progressive myelopathy Conclusions Early onset scoliosis often associated with neuro- anatomic abnormalities or pathologies Clear pathogenetic mechanisms are not well understood making decision making difficult

Careful measured approach, often in multidisciplinary setting, provides reasonable and very satisfying approach

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