- See:
- Orthopaedic Manifestations of Myelomeningocele:
- Discussion:
- scoliosis in myelomenigocele may have a multifactorial etiology:
- idiopathic causes;
- myelomeningocele structural defect;
- congenital defects
- paralytic etiology;
- 15-20% of children who have myelomeningocele have congenital vertebral abnormalities in addition to congenital scoliosis;
- over-all incidence of scoliosis in pts who have thoracic-level lesion is 85%;
- all children who are born with an osseous dysraphism at level of T12 or higher, are at risk for the development of a severe scoliotic
deformity for which arthrodesis is needed;
- progression of scoliosis in myelomeningocele is natural;
- progressive scoliosis in myelomeningocele:
- may manifest as lower extremity spasticity;
- MRI is helpful in evaluating these children;
- diff dx:
- tethered cord:
- if curve continues to progress despite neurosurgical release of tethered cord, then curve must be stabilized with spinal fusion;
- non-functioning shunt:
- must be corrected before attempt is made to correct the scoliotic deformity;
- Arnold Chiari malformation:
- syringomyelia:
- hydromyelia:
- hydrocephalus:
- treatment considerations:
- goal is to have a compensated spine of normal height over level pelvis, w/ preservation of normal sagittal alignment;
- documented progression of curve is indication for early fusion, just as in child who does not have myelomeningocele;
- Bracing:
- orthotic treatment is helpful for the more common paralytic curve until the child reaches puberty;
- although bracing does not alter natural history of deformity, it promotes spinal growth & preserves better sitting posture, esp in pts who
have lesion at thoracic level;
- carefully fitted, custommolded, bivalve plastic total-contact thoracolumbosacral orthosis provides maximum support and allows
monitoring of the skin over pressure-sensitive areas;
- custom-molded chair insert for sitting is valuable for the child who has high thoracic-level lesion or severe kyphotic deformity;
- attempts at bracing (TLSO) may fail & require subcutaneous rodding for very young children and fusion later;
- Operative Treatment of Scoliosis:
- preop eval:
- aggressive evaluation of urinary tract, treatment of urinary tract infection, & periop ATB are important in reducing wound infections;
- treatment of the scoliosis without recognition of syringomyelia and Chiari malformation can lead to paraplegia;
- paralytic scoliosis:
- treated w/ anterior arthrodesis combined w/ posterior arthrodesis and secure internal fixation, plus bone grafting;
- luque sublaminar wiring w/ fixation to sacrum;
- curves > 60 deg require initial anterior release & fusion;
- Selection of fusion level;
- it is difficult to decide which levels to include in spinal fusion;
- 55-deg curve that ends at L-3 can probably be corrected satisfactorily w/ anterior/posterior fusion & instrumentation so that sacral
fusion will not be necessary;
- fusion to sacrum:
- arthrodesis to pelvis is often needed to correct severe pelvic obliquity and dysraphism;
- in pts w/ high-level myelomeningocele, it is important to fuse to sacrum if curve is major and cannot be corrected below 15 deg;
- in child w/ low lumbar myelomeningocele who is able to walk, a fusion to sacrum will impair walking ability & confine child to
wheelchair;
- sacrum should not be fused in walking child unless it is absolutely necessary;
- high-level myelomeningocele:
- it is important to fuse to sacrum if curve is major and cannot be corrected below 15 degrees;
- low level myelomeningocele:
- in pt w/ low lumbar myelomeningocele who is able to walk, fusion to sacrum will impair walking ability & confine child to a
wheelchair;
- staged anterior & posterior arthrodesis:
- in young child, it is important to maintain anterior/posterior fusion, otherwise crank shaft phenomenon" may develop w/continued
progression of curve;
- staged anterior & posterior arthrodeses are necessary to achieve adequate correction and solid fusion due to absence of
posterior osseous elements, muscle weakness, & lack of ligamentous support in the dysraphic spine,
- combined anterior & posterior arthrodeses allow for major correction of spinal deformity as well as stabilization of pulmonary
function and improvement of respiration;
- posterior arthrodesis:
- Luque and CD systems may allow isolated posterior arthrodesis w/o need for anterior fusion;
- Complications:
- spinal operations in patients who have myelomeningocele have been associatted w/high incidence of complications such as pseudarthrosis,
loss of correction, & sepsis
Efficacy of surgical management for scoliosis in myelomeningocele: correction of deformity and alteration of functional status.