Neuromuscular Cerebral Palsy


Figure 1


Question 1:

11 year old male with spastic quadriplegic cerebral palsy presents for evaluation of neuromuscular scoliosis.Supine thoracolumbar spine radiograph (Left image Figure 1) and traction ‘push-pull’ radiograph (Right image Figure 1) demonstrate high magnitude neuromuscular scoliosis.The patient has optimized nutrition via g-tube feeds, no history of pneumonia, and is not on any seizure medications.Parents are frustrated with child’s inability to tolerate seating in wheelchair and desire to proceed with surgical intervention.Surgical recommendations include:

A. T10-L5 instrumented posterior spinal fusion with lumbar Ponte osteotomies.
B. T11-L4 Anterior release with anterior vertebral osteotomies and T2-Pelvis instrumented posterior spinal fusion with lumbar Ponte osteotomies
C. T2-L5 instrumented posterior spinal fusion with lumbar Ponte osteotomies
D. T10-L4 anterior instrumented spinal fusion
E. T2-Pelvis instrumented posterior spinal fusion with multiple lumbar Ponte osteotomies

Preferred Reponse: B. T11-L4 Anterior release with anterior vertebral osteotomies and T2-Pelvis instrumented posterior spinal fusion with lumbar Ponte osteotomies

This patient’s caregiver’s primary complaints relate to the remarkable and rigid pelvic obliquity which has progressed to the point of impairing sitting tolerance.  Accordingly, the goal of this procedure is to adequately correct the pelvic obliquity and restore sitting balance for this patient.  The traction ‘push-pull’ radiograph proves this obliquity to be rigid, and it was felt that anterior release and vertebral osteotomy followed by PSF with Ponte osteotomies would be most effective at achieving surgical goals.  Thomson and Banta recommend anterior procedure for curves that to not bend to <50⁰ and for pelvic obliquity that does not level on traction radiograph (J Pediatr Orthop B 2001;10(1):6-9).  Fusions which stop short of the pelvis are only indicated in neuromuscular scoliosis in the absence of pelvic obliquity.  Isolated anterior or posterior short segment thoracolumbar / lumbar fusions are not recommended in neuromuscular scoliosis secondary to risk of frontal or sagittal plane adding on above the thoracolumbar junction. Postoperative radiographs are shown in Figure 2.
 

 
Figure 2


 
   
Figure 3

Question 2:

15 year old male nonambulatory male with spastic quadriplegic cerebral palsy, seizure disorder and body weight below the 5th percentile with progressive neuromuscular thoracolumbar kyphosis presents for second opinion regarding distal junctional kyphosis 1 month following T2-L3 posterior spinal fusion with multiple Ponte osteotomies.Preoperative and 1 month postoperative lateral radiographs (figure 3) demonstrate LIV screw pullout and L3 vertebral chance fracture.This surgical complication could have been avoided via:

A. Postoperative TLSO bracing
B. Staged anterior fusion prior to posterior fusion to L3
C. Initial instrumentation to L5 or the pelvis
D. Both A & B.
E. All of the above

Preferred Response: C. Initial instrumentation to L5 or the pelvis

This patient has several identifiable risk factors which should alert a treating surgeon to the likelihood of severe osteopenia: nonambulatory, low body weight, and antiepileptic medications.  This patient’s instrumentation levels appear to have been selected as if the etiology for the kyphosis were Scheurman’s kyphosis.  In the setting of neuromuscular spine deformity treatment, instrumentation should extend from T2-L5 or the pelvis with multisegmental fixation in order to maximize osseous fixation across osteopenic vertebrae and avoid junctional kyphosis.  Additionally, given the inherent risk associated with neuromuscular spine surgery, it is imperative that all modifiable risk factors be addressed preoperatively in order to optimize patient outcome (i.e.-nutritional optimization potentially including tube feeds, Vitamin D supplementation, etc).  Note that the postoperative lateral radiograph demonstrating the distal junctional kyphosis was taken in a TLSO.  Postoperative radiographs following revision to the pelvis are shown in Figure 4.


Figure 4



View Spinal Fusion Article
Whitaker AT, Sharkey M, Diab M. Spinal Fusion for Scoliosis in Patients with Globally Involved Cerebral Palsy: An Ethical Assessment. J Bone Joint Surg Am. 2015;97:782-7

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