Preserve or improve gait efficiency Early identification and stabilization or correction of lower extremity deformities

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Sheila Osborne
5 years ago
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1 ORTHOPEDICS Primary Outcomes Maintenance of a stable and balanced spine. Optimize pulmonary function. Avoid restrictive pulmonary disease. Optimize spinal growth. Avoid or facilitate healing of sacral/ischial decubiti Secondary Outcomes Maintenance of plantigrade feet Prevent pressure sores Tertiary Outcomes Preserve or improve gait efficiency Early identification and stabilization or correction of lower extremity deformities INFANCY (THROUGH AGE 1 YEAR) What are the challenges of early onset scoliosis, kyphosis and pulmonary insufficiency syndrome? Which foot deformities merit correction in infancy and what is appropriate treatment? Obtain baseline AP and Lateral scoliosis xrays Orthopedic evaluations Q 3-6 months in first year of life. Perform Ponseti casting or release for clubfoot or congenital vertical talus deformities, Perform neonatal kyphectomy if required to facilitate skin closure Perform spinal fusion for congenital scoliosis with documented progression TODDLER (1-3 YEARS) What is the proper timing for correction of rotational deformities of the femur and/or tibia Are twister cables useful for rotational deformities? What is the role of bracing or Mehta casting for early onset non-congenital scoliosis? Should gibbous deformity be treated surgically? Is Rib to Pelvic distraction vs kyphectomy the optimum treatment for gibbous?

2 Monitor the spine for development or progression of deformity with AP and Lat Scoliosis radiographs q 1-2 years or more frequently in patients with concerns for progression. Initiate treatment for progressive early onset scoliosis that may involve casting, bracing or growing rod placement Perform tendon release/transfers for unbalanced foot deformities such as the calcaneus foot, or equinovarus foot. Surgically correct rotational deformities of the tibia or femur only if they are limiting further motor development. Otherwise, temporize d/t concerns of recurrence if derotational osteotomies are performed too early. Perform fusion in situ for progressive congenital scoliosis PRE-SCHOOL (3-5 YEARS) Clinical questions: Is bracing effective for early onset, non-congenital scoliosis? Is rib-pelvis distraction vs spine based growing rod construct the optimal treatment for progressive scoliosis? Evaluate gait with careful attention to Orthopedic deformities that render gait inefficient Perform derotational osteotomy when rotational abnormality adversely impacts ambulation, particularly for tibial torsion Treat foot deformities with stretching, casting, bracing, soft tissue release or tendon transfers. Obtain Scoliosis xrays Q 1-2 years or more frequently in patients with progression Work with neurosurgery to identify, if present, neurogenic cause of scoliosis progression Consider bracing for progressive non- congenital scoliosis in the degree range Perform fusion in situ for progressive congenital scoliosis SCHOOL AGE (6-11 YEARS) As per previous age group Monitor gait, rotational deformities and foot position. Scoliosis, pelvic obliquity, and hip flexion contractures cause worsening of mobility. Correct foot deformities with soft tissue release, tendon transfer and osteotomy if necessary. Avoid fusion if possible. Correct tibial and femoral rotational deformities when interfering with gait Consider computerized gait analysis if available in low lumbar or sacral level patients with atypical gait abnormaiities for decision making regarding surgery or bracing Monitor for the development of scoliosis/kyphosis. Obtain AP and Lat Scoliosis radiographs Q 1-2 years or more frequently in patients with

3 progressive spinal deformity. Early surgical treatment of scoliosis will not assure continuation of mobility Teach child about fractures and related precaution. TEEN AGE (12-17 YEARS) What is the impact of scoliosis/kyphosis on gait, sitting balance, upper extremity function? What is the relationship between spinal deformity and decubitus ulcer? Which patients benefit from spinal deformity surgery? How can spinal deformity surgery be safely accomplished? In lumbar scoliosis, how high must the fusion extend? Monitor for the development or progression of scoliosis. If the curve has progressed to an operative magnitude,(50 degrees) discuss with the family the risks/benefits of surgical treatment. If family decides against surgical treatment, then future spine radiographs are not necessary Monitor for deterioration of gait and consider treatment of Orthopedic deformities leading to deterioration such as hip/knee contracture or rotational deformities.. Computerized gait analysis may be useful for decision making in low lumbar and sacral level patients Annual evaluation, unless more frequency indicated. Address bone density issues by recommending calcium and vitamin D supplementation. Osteoporosis: occurs more commonly in the lower extremities and may lead to fractures. Bone densitometry is a critical tool. This is particularly important to non-ambulatory patients, and when hormonal supplementation or replacement (HRT) is used. ADULT (18+ YEARS) What is the optimal Orthopedic transition plan What degenerative issues can be expected for specific levels of function (eg knee arthrosis for midlumbar with valgus thrust gait pattern) and what treatments can mitigate against these problems (eg. KAFO s or crutches in the above example Develop an Orthopedic transition plan Counsel the patient about potential orthopedic degenerative problems.

4 RESEARCH GAPS (all age groups) Is bracing effective in treating developmental (non-congenital) scoliosis in patients with Myelomeningocoele? Is Mehta casting effective in non-congenital early onset scoliosis in MMC? What is the impact of Scoliosis/Kyphosis on gait, sitting balance or upper extremity function? The optimal age for repairing congenital kyphosis is unclear. It is also unclear which patients with scoliosis will benefit from sacral-pelvic instrumentation, or one-stage vs. two-stage operation. What is the relationship between specific foot deformities and the development of pressure sores? Does foot deformity surgery alter the risk of pressure sores? What is the role of guided growth surgery for rotational deformities? Is there a specific Orthopedic transition plan that is ideal? REFERENCES Abo El-Fadl, S. "Early Managment of Neurologic Clubfoot using Ponseti Casting with Minor Posterior Release in Myelomeningocele: A Preliminary Report." Journal of Pediatric Orthopaedics, Part B (2016): Journal. Altiok, H. "Kyphectomy in Children with Myelomeningocele." Clinical Orthopaedics & Related Research (2011): Journal. Bas, C. E. "Safety and Efficacy of Apical Resection Following Growth-Friendly Instrumentation in Myelomeningocele Patients with Gibbus: Growing Rod Versus Luque Trolley." Journal of Pediatric Orthopdics (2015): e Journal. Comstock, S. A. "Posterior Kyphectomy for Myelomeningocele with Anterior Placement of Fixation: A Retropective Review." Clinical Orthopaedics & Related Research (2011): Journal. Flynn, J. M. "VEPTR to Treat Nonsyndromic Congenital Scoliosis: A Multicenter, Mid-term Follow-up Study." Journal of Pediatric Orthopaedics (2013): Journal. Garg, S. "Kyphectomy Improves Sitting and Skin Problems in Patients with Myelomeningocele." Clinical Orthopaedics & Related Research (2011): Journal. Khoshbin, A. "The Long-Term Outcome of Patients Treated Operatively and non-operatively for Scoliosis Deformity Secondary to Spina Bifida." Bone and Joint Journal (2014): Journal. Kim, H. J. "Revision Spine Surgery to Manage Pedicatric Deformity." Journal of the American Academy of Orthopaedic Surgeons (2010): Jounal. Malas, B. S. "What Variables Influence the Ability of an AFO to Improve Function and When Are They Indicated?" Clinical Orthopaedics & Related Research (2011): Journal.

5 Margalit, A. "Myelokyphectomy in Spina Bifida: The Modified Fackler or Sagittal Shalla Technique." Operative Techniques in Orthopaedics (2016): Journal. Matar, H. E. "Effectiveness of the Ponseti Method of Treating Clubfoot Associated with Myelomeningocele: 3-9 Years Follow-up." Journal of Pediatric Orthopaedics Part B (2016): Journal. Mehta, V. A. "Safety and Efficacy of Concurrent Pediatric Spinal Cord Untethering and Deformity Correction." Journal of Spinal Disorders and Techiques (2011): Journal. Mercado, E. "Does Spinal Fusion Influence Quality of Life in Neuromuscular Scoliosis?" Spine (Phila Pa 1976) (2007): S Journal. Ouellet, J. A. "Pressure Mapping as an Outcome Measure for Spinal Surgery in Patients with Myelomeningocele." Spine (Phila Pa 1976) (2009): Journal. Park, K. B. "Surgical Treatment of Calcaneal Deformity in a Select Group of Patients with Myelomeningocele." Journal of Bone & Joint Surgery - American Volume (2008): Journal. Patel, J. "Correlation of Spine Deformity, Lung Function, and Seat Pressure in Spina Bifida." Clinical Orthopaedics & Related Research (2011): Journal. Shingade, V. U. "Single Stage Correction for Clubfoot Associated with Myelomeningocele in Older Children: Early Results." Current Orthopaedic Practice (2014): Journal. Sibinski, M. "Quality of Life and Functional Disability in Skeletally Mature Patients with Myelomeningocele-Related Spinal Deformity." Journal of Pediatric Orthopaedics Part B (2013): Journal. Smith, J. T. "Bilateral Rib-Based Distraction to the Pelvis for the Management of Congenital Gibbus Deformity in the Growing Child with Myelodysplasia." Spine Deformity (2016): Journal.. "Treatment of Gibbus Deformity Associated with Myelomeningocele in the Yourn Child with Use of the Vertical Expandable Prosthetic Titanium Rib (VEPTR): A Case Report." Journal of Bone & Joint Surgery - Americal Volume (2010): Journal. Thomason, J. D. "Orthopedic Management of Spina Bifida." Developmental Disabilities Research Reviews (2010): Journal. Wright, J. G. "Hip and Spine Surgery is of Questionable Value in Spina Bifida: An Evidence-based Review." Clinical Orthopaedics and Related Research (2011): Journal.

Orthopedics. Michael Conklin, MD, Chair Sam Rosenfeld, MD Chanka Nanyakara, MD Shyam Kishan, MD

Orthopedics. Michael Conklin, MD, Chair Sam Rosenfeld, MD Chanka Nanyakara, MD Shyam Kishan, MD Orthopedics Michael Conklin, MD, Chair Sam Rosenfeld, MD Chanka Nanyakara, MD Shyam Kishan, MD Outcomes Primary Outcomes Maintenance of a stable and balanced spine. Optimize pulmonary function. Avoid restrictive