Operative treatment for spinal deformities in cerebral palsy

Abstract

The higher the functional impairment, the more likely patients with cerebral palsy (cP) are to develop a scoliotic deformity. This is usually long-sweeping, C-shaped, and progressive in nature, since the causes of the deformity, such as muscular weakness, imbalance, and osteoporosis, persist through adulthood. In contrast to idiopathic scoliosis, not only is the spine deformed, the patient is also sick. This multimorbidity warrants a multidisciplinary approach with close involvement of the caregivers from the beginning. Brace treatment is usually ineffective or intolerable in light of the mostly stiff and severe deformities and the poor nutritional status. The pros and cons of surgical correction need to weighed up when pelvic obliquity, subsequent loss of sitting balance, pressure sores, and pain due to impingement of the rib cage on the ileum become issues. General risks of, for example, pulmonary or urogenital infections, pulmonary failure, the need for a tracheostoma, permanent home ventilation, and death add to the particular surgery-related hazards, such as excessive bleeding, surgical site infections, pseudarthrosis, implant failure, and dural tears with leakage of cerebrospinal fluid. The overall complication rate averages around 25 %. From an orthopedic perspective, stiffness, marked deformities including sagittal profile disturbances and pelvic obliquity, as well as osteoporosis are the main challenges. In nonambulatory patients, long fusions from T2/T3 with forces distributed over all segments, low-profile anchors in areas of poor soft tissue coverage (sublaminar bands, wires), and strong lumbosacropelvic modern screw fixation in combination with meticulous fusion techniques (facetectomies, laminar decortication, use of local autologous bone) and hemostasis can be employed to keep the rate of surgical and implant-related complications at an acceptably low level. Excessive posterior release techniques, osteotomies, or even vertebrectomies in cases of very severe short-angled deformity mostly prevent anterior one- or two-stage releases. Despite improved operative techniques and implants with predictable and satisfactory deformity corrections, the comorbidities and quality-of-life related issues demand a thorough preoperative, multidisciplinary decision-making process that takes ethical and economic aspects into consideration.

Keywords: Cerebral palsy; Neuromuscular; Pelvic obliquity; Scoliosis; Sitting balance.

Fig. 1

A 16-year-old boy with pain, a pressure sore, and marked deformity of the left hemithorax due to pelvic obliquity and subsequent rib-to-pelvic impingement

Fig. 2

An 11-year-old girl with cerebral palsy, loss of sitting balance, and painful rib–pelvic impingement a. Progressive pelvic obliquity (40°) and right convex thoracolumbar stiff curve (120°) with a compensatory left convex thoracic curve b, c. Two-stage (1 week interval) anterior lumbar release and posterior multi-segmental hybrid construct consisting of polyaxial lumbar pedicle screws, iliosacral screws, thoracic sublaminar (Luque) wires, and a proximal claw (pedicle and transverse process hook)