summary Spinal Disorders in Cerebral Palsy are thought to be caused by muscle weakness and truncal imbalance and most commonly present with progressive scoliosis. Diagnosis is made with full-length spine radiographs. Treatment can range from observation, bracing, or surgical management depending on the severity of underlying medical conditions, skeletal maturity of the patient, magnitude of deformity, and curve progression. Epidemiology Scoliosis common in children with cerebral palsy overall incidence is 20% the more involved and severe the cerebral palsy, the higher the likelihood of scoliosis spastic quadriplegic at highest risk, especially if no ability to sit independently. for bedridden children incidence approaches 100% spinal deformity is rare in children who are able to ambulate Etiology See Cerebral Palsy General Pathophysiology muscle weakness and truncal imbalance has been implicated as primary etiology (little evidence to support) pelvic obliquity leads to deforming forces on spine and scoliosis Associated conditions Scoliosis in patients with cerebral palsy differs from idiopathic scoliosis in that curves are more likely to progress login to view 1 more bullet curve begins at earlier age curve is a long, stiff C-shaped curve login to view 1 more bullet curve has greater sagittal plane deformity (kyphotic or lordotic) associated with pelvic obliquity, skeletal maturity is delayed in CP bracing is less effective longer fusions to the pelvis are often necessary patients are more medically fragile and a multi-disciplinary approach is often necessary Classification Weinstein classification Group I - double curves with thoracic and lumbar component and minimal pelvic obliquity Group II - large lumbar or thoracolumbar curves with marked pelvic obliquity Evaluation Treatment of cerebral palsy spine disorders requires a careful risk-benefit analysis. Therefore it is important to have a thorough understanding of the medical history and functional status. History clinical history perinatal history growth & development all medical treatment functional status patients nutritional status respiratory function sitting / standing posture upper and lower extremities function communication skills acuity of hearing and vision Physical Exam muscular-skeletal exam motion, tone, and strength hamstring contracture's (lead to decreased lumbar lordosis) hip contractures (lead to excessive lumbar lordosis) spine exam look at flexibility of curve spinal balance and shoulder height pelvic obliquity Imaging Radiographs introduction important just to use same radiographic technique in patients over time login to view 2 more bullets standard AP & lateral look for rib deformity, wedging, and spinal rotation be sure to evaluate for spondylolisthesis on lateral (incidence of 4-21% in patients with spastic diplegia) bending films important to evaluate flexibility of curve use push-pull radiographs or fulcrum bending radiographs if patient can not cooperate MRI preoperative MRI is not routinely performed for patients undergoing spinal deformity surgery indications for MRI include rapid curve progression change in neurologic exam Treatment Nonoperative observation, custom seat and/or bracing, botox injections indications login to view 3 more bullets outcomes login to view 8 more bullets Operative PSF with/without extension to the pelvis indications login to view 4 more bullets indications to extend to pelvis technique login to view 2 more bullets PSF +/- ASF with/without extension to pelvis indicated for login to view 3 more bullets growing rod distraction indications login to view 1 more bullet technique Preoperative Assessment & Planning Overview treatment of cerebral palsy spine disorders is complicated by medical comorbidities all patients should have a thorough multidisciplinary approach Nutritional status increase complications (infection, length of intubation, longer hospital stays) associated with poor nutritional status (weight less than fifth percentile) be sure patient has adequate nutrition before surgery (serum albumin > 3.5 g/dL, consider gastrostomy tube if not) Respiratory status difficult to do formal pulmonary functional capacity testing can use respiratory history, clinical evaluation, and chest radiographs GI evaluation preoperative management of GERD is important in prevention of aspiration pneumonia Neurologic function if patients have seizure disorder (common) be sure it is under control if patient taking valproic acid, obtain bleeding time as these patients may have increased risk of bleeding Techniques Goals of Surgery obtain painless solid fusion with well corrected, well balanced spine with level pelvis decision to proceed with surgery must include careful assessment of family's goals and careful risk-benefit analysis Fusion levels proximal fusion should extend to T1 or T2 (otherwise risk of proximal thoracic kyphosis) distal fusion depends on curve pattern due to long curves in CP often extends to L4 or L5 extend to pelvis whenever pelvic obliquity is > 15° Posterior fixation techniques Luque rod with sublaminar wires technique Unit rod with sublaminar wires technique Pedicle screw fixation technique may provide better correction and eliminate need for anterior surgery Pelvic fixation techniques Galveston Technique technique to fuse to pelvis with goal of a stability and truncal balance and a level pelvis caudal ends of rods are bent from lamina of S1 to pass into the posterosuperior iliac spine and between the tables of the ileum just anterior to the sciatic notch bilateral sacral screws iliosacral screws spinopelvic transiliac fixation Dunn-McCarthy technique (S-contoured rod that wraps over sacral ala) Anterior and Posterior Techniques use of anterior procedures decreasing with improved posterior constructs higher complication rate in anterior surgery in CP spinal deformity than idiopathic scoliosis decrease complication rate if A/P done on same day verses staging procedure (improved nutritional status, decreased blood loss, short length of hospitalization) Preoperative traction may be option in severe and rigid curve Postoperative bracing usually not required may be used in patients with osteoporosis or tenuous fixation Complications Implant failure sometimes may be asymptomatic and not require treatment includes penetration of pelvic limb of unit rod into pelvis Pulmonary complications chronic aspiration pulmonary insufficiency most common complication in recent study pneumonia GI complications GERD poor nutrition and delayed growth Neurologic complications seizures Wound infection more common in CP than idiopathic scoliosis occurs in 3-5% and usually can be treated with local wound debridement alone Death (0-7%) Prognosis Natural history the larger the curve the more likely it is to progress larger curves are associated with pelvic deformity and obliquity some studies show increase incidence of decubitus ulcer in patients with larger curves, other studies did not