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Sunday 15 April 2018


  • NF - hereditary hamartomatous disorder on neural crest derivation – appear from any organ system in body
  • type 1 and 2
  • classic – Type I – von Recklinghausen – auto dominant – Schwann cell tumors and pigmentation anomalies – spinal deformities
  • central – Type II – auto dominant – less common – b/l acoustic neuromas – no bony/ orthopaedic involvement
Clinical criteria of NF I
  • two of the following
    • minimum 6 cafe au lait spots larger than 1.5cm in post pubertal patient – larger than 5mm in prepubertal
    • 2 or more neurofibromas or 1 plexiform neurofibroma
    • freckling in inguinal/ axillary region
    • optic glioma
    • 2/more iris lisch nodules by slit lamp examination
    • distinct osseous lesion
    • first degree relative with definitive NF
  • spinal deformity in NF two forms
    • non dystrophic
      • mimic idiopathic scoliosis
    • dystrophic
      • short segmented – sharply angulated curve with severe wedging of vertebral bodies – a/c angulation in sagittal plane – characteristic of NF kyphoscoliosis – striking deformity of vertebral body near apex
      • severe rotation of vertebrae – scalloping of vertebral bodies – spindling of transverse process – foraminal enlargement – rotation of the ribs 900 in AP direction appears to be thin
      • functional scoliosis by leg length discrepancy
      • spinal deformity in dystrophic kyphoscoliosis – caused paraplegia ; not in dystrophic scoliosis alone
      • laminectomy when done alone worsens spinal cord compression in kyphoscoliosis
Treating non dystrophic NF
  • same prognosis and evolution as idiopathic curves – except : higher risk of pseudo arthrosis after operative fusion
  • spinal deformity before 7 yrs of age – closely observed for dystrophic changes : modulation – three penciled ribs – combination of 3 dystrophic features – clinical progression to dystrophic disease is certain
  • treated same as idiopathic except watch for modulation
    • curves <20-250 – observation
    • >300 – brace
    • >40-450 – posterior spinal fusion with segmental hook/ screw instrumentation
  • CT myelography / MRI – before surgery to r/o any intraspinal canal NF
Treatment of dystrophic scoliosis
  • brace not accepted in dystrophic curves
  • surgical options decided by presence/ absence of kyphotic component and neurological deficits
  • r/o intraspinal lesions : pseudomeningocele, dural ectasia, intraspinal neurofibromatosis ( dumbbell tumor) – impingement of this lesions on spinal cord after instrumentation
  • Scoliosis w/o kyphosis
    • observed at 6 months interval if curve is <200
    • curve progresses – posterior spinal fusion
    • >400 – primary anterior and posterior spinal fusion – contraindicated in young age, osteoporosis, peculiar anatomical configurations
    • segmental hook/ screw instrumentation
    • spinal fusion mass inspected 1 yr after surgery – sooner if progression in curve occurs
  • kyphoscoliosis
    • combined anterior and posterior spinal fusion
    • smaller scoliosis with kyphosis <400 – posterior spinal fusion with arthrodesis – with periodic checking of fusion mass for progression
    • kyphosis >500 – anterior disc excision – fibular strut bone grafting (vascularized rib graft augmentation) – posterior spinal fusion with instrumentation
    • anterior fusion should extend one or two levels past the both end vertebrae
    • fusion mass should be reexplores at least every 6 months
  • kyphoscoliosis with spinal cord compression
    • cord compression by kyphosis should be differentiated from intra spinal lesion by investigations
    • severe scoliosis with paraplegia w/o significant kyphosis – assumed to have intra spinal lesions unless proved otherwise
    • laminectomy absolute CI – removal of valuable posterior bone available for fusion -aggravates the kyphosis
    • minor compression – halo pelvic traction
    • significant cord compression – anterior cord decompression – anterior strut graft fusion after this – posterior fusion as second stage
    • if intra spinal lesion is anterior causing cord compression – similar procedure
    • if lesion posterior – hemilaminectomy with tumor excision
Post op
  • immobilized in cast/brace until fusion evident on AP, lateral, oblique X ray
  • exploration of fusion mass periodically

  • excessive bleeding
  • pheochromocytoma
  • patients without deficit developing paraplegia
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