How an Orthopedic Surgeon Thinks Bert Knuth, MD June

Is he happy? Is he in pain? Am I doing everything he needs? Am I doing everything right? What if I do more? More of what? What does the future hold? Therapy PT OT Speech Developmental Social work Orthotics? Pediatrics Neurology ENT MRI ? Genetics Medical Diagnostics

 Hypotonia- Low muscle tone  Contractures  Hip subluxation/ dislocation  Scoliosis  Polydactyly, Syndactyly

… “for those who have nothing, a little is a lot”… Jacquelin Perry,MD  Priorities ◦ Communication ◦ Activities of daily living- ADLs  Perineal care, feeding, bathing etc. ◦ Mobility  Sitting/ Seating  Walking ◦ Pain free

 92% CHOP cohort  Role of Physical Therapy ◦ Disuse- more is better ◦ Developmental vs age appropriate ◦ Function  Role of Bracing ◦ Temporary support ◦ Prevention of contractures?

 Nonambulatory children with neuromuscular involvement are prone to develop flexion contracture of the hips and knees. Equinus contracture of the ankle can occur.  Physical therapy for gentle range of motion of the joints should be instituted.  The role of surgical release of contractures is controversial as function may not be improved and recurrence is commonplace.

 Common in nonambulatory patients  Proximal muscle weakness predisposes to structural abnormalities which leads to uncoverage of hips.

 Unilateral dislocation can lead to pelvic obliquity and uneven seating pressure.  Bilateral dislocation can accentuate lumbar lordosis.  Management is controversial as pain is inconsistent and treatment is difficult.

 More common in nonambulatory patients  Discovered at an earlier age and progresses more rapidly in nonambulatory patients.  Nonoperative treatment- Bracing ◦ May make sitting easier but usually ineffective in preventing curve progression or altering need for surgery. ◦ A rigid orthosis can further tax a compromised respiratory status ◦ Role of soft TLSO may be tolerated in young children with flexible curves between degrees to allow more time prior to surgery.

 Surgical Treatment- spinal fusion ◦ Goal is to balance trunk over level pelvis to facilitate seating.

Indications are progressive deformity with curve magnitude greater than degrees Preoperative traction? for low vital capacity Posterior spinal fusion for patients who can tolerate surgery Fusion should include entire thoracic and lumbar spine and extend to the pelvis.

 Extra or webbed digits ◦ Extra toes are usually not a functional concern and are only removed for shoe wear difficulties or cosmetic concerns. ◦ Much more aggressive with extra or webbed fingers if function is altered/ decreased