Scoliosis

 Scoliosis is a common deformity in many types of neuromuscular diseases

 most severe in nonambulatory patients

Frequency

 the prevalence of spinal deformity in the patient with a neuromuscular disorder is much higher than in
the general population

 It ranges from 20% in children with cerebral palsy to 60% in patients with myelodysplasia.

 The prevalence rises to 90% in males with Duchenne muscular dystrophy.

 In general, the greater the neuromuscular involvement, the greater the likelihood and severity of
scoliosis.

Etiology

 Neuropathic:

 upper motor neuron lesions includes diseases such as cerebral palsy, syringomyelia, and spinal cord
trauma
 lower motor neuron lesions includes poliomyelitis and spinal muscular atrophy

 Myopathic

- include muscular dystrophy, and other forms of myopathy.

Pathophysiology

 The pathophysiology is not well understood.

 The evaluation of a patient with neuromuscular scoliosis entails a thorough assessment of all body
systems.

 Accurate diagnosis of the underlying disease entity is essential and may require muscle biopsy.

 Assessing nutritional status and pulmonary function is extremely important

 The orthopedic examination includes assessment of all extremities and joints for contractures.

 Spinal deformity, decompensation, and shoulder balance are documented.

 Ambulatory status is also evaluated, and patients are classified as walkers, sitters, or nonsitters.

Workup

 Laboratory Studies

 Total lymphocyte count

 The total lymphocyte count should be greater than 1500 cells/mm3.

  Hemoglobin

 Total protein

 Albumin: Patients with serum albumin levels greater than 3.5 mg/dL have a much lower incidence
of postoperative wound infection

 Electrolytes

 Serum blood urea nitrogen

 Creatinine

 Transferrin

Imaging Studies

 Supine anteroposterior and lateral spinal radiographs: These are ordered for very young patients and
older patients who cannot sit.

 Upright anteroposterior and lateral spinal radiographs

 Standing upright radiographs should be used for patients who can stand, and sitting
radiographs should be used for patients who cannot stand.

Traction spinal radiographs: These radiographs are obtained to evaluate the flexibility of the curves.

Treatment

 Medical Therapy

 The goal of nonoperative and operative treatment of patients with neuromuscular scoliosis is
the same: to maintain the spine in a balanced position in the coronal and sagittal planes over a
level pelvis.
 -This goal is achieved with a custom molded thoracolumbosacral orthosis (TLSO) and molded
seating supports.
 The aim is to control the curve during spinal growth rather than to correct the spinal deformity.

 Surgical Therapy

 The surgical principles in the management of neuromuscular scoliosis differ from those in
idiopathic scoliosis.

 Fusion is necessary at a younger age, and the fused portion of the spine is longer.

 Fusion to the sacrum is fairly common because many of these children do not have sitting balance
or have pelvic obliquity.

 Combined anterior and posterior fusion is common in the treatment of patients with
neuromuscular scoliosis:

 posterior elements are absent, as in myelodysplasia

  necessary to gain correction in a rigid lumbar or thoracolumbar curve and achieve a spine
fused in balance over a level pelvis

 The instrumentation used is segmental, with either a multiple hook-rod system, with or without the
addition of sublaminar wires, or a Luque rod and sublaminar wires or a unit rod device.

 When fusion to the sacrum is necessary, it can be performed with the Luque-Galveston technique
or with iliac screws

 Hospital stays are usually 7-10 days.

 Modifications in the child's wheelchair should be made as soon as possible to accommodate the
new sitting position.

 The number of hours spent upright each day should be gradually increased.

 The wound should be assessed 3 weeks postoperatively.

 Radiographs should be obtained 6 weeks postoperatively and again 3 and 6 months after surgery.

Complications

 respiratory problems

 ileus

 nutritional problems

 hip problems

 crankshaft phenomenon.

Outcome and Prognosis

 With care in surgical technique and adequate postoperative care, complications can be minimized. The
patient can return to the preoperative functional level with a successful surgical result, which consists
of a solidly fused spine in balance in the coronal and sagittal planes over a level pelvis.

Idiopathic scoliosis

 is the most common type of spinal deformity confronting orthopedic surgeons.

 Its onset can be rather insidious, its progression relentless, and its end results deadly.

 Proper recognition and treatment of idiopathic scoliosis help to optimize patient outcomes.

Etiology

 The precise etiology of idiopathic scoliosis remains unknown, but several intriguing research avenues
exist.

 A primary muscle disorder has been postulated as a possible etiology of idiopathic scoliosis.
  -The contractile proteins of platelets resemble those of skeletal muscle, and calmodulin is an
important mediator of calcium-induced contractility

 An elastic fiber system defect (abnormal fibrillin metabolism) has been offered as one potential
etiologic explanation for idiopathic scoliosis.

 Disorganized skeletal growth, probably with its root cause at a gene locus or group of loci, has been
discussed as a possible etiologic explanation for idiopathic scoliosis.

Pathophysiology

 Much has been written regarding the potential influence of melatonin on the development of
idiopathic scoliosis.

 Some authors have suggested that a posterior column lesion within the central nervous system might
be present in patients who have idiopathic scoliosis

Presentation

 The vast majority of patients initially present due to perceived deformity.

 -Adams forward-bending test (in conjunction with the use of a scoliometer) has been found to
be an effective screening tool.

 Patient's history include information relative to other family members with spinal deformity,
assessment of physiologic maturity (eg, menarche), and presence or absence of pain.

 Physical examination should include a baseline assessment of posture and body contour.

 Shoulder unleveling and protruding scapulae are common.

 In the most common curve pattern (right thoracic), the right shoulder is consistently rotated forward
and the medial border of the right scapula protrudes posteriorly.

 Assessment of lower (and often upper) extremity reflexes should be performed.

 Abdominal reflex patterns should also be assessed.

 The presence or absence of hamstring tightness should be investigated

 screening should be performed for ataxia and/or poor balance or proprioception (ie, Romberg test).

 One or two different methods of measuring leg length will prove valuable, as a significant percentage
of patients presenting with scoliosis have several centimeters of limb-length discrepancy.

 The main treatment options for idiopathic scoliosis may be summarized as "the 3 O's":

 (1) observation

 (2) orthosis
 (3) operative intervention

types
 Infantile Idiopathic Scoliosis:

- -defined by a seemingly arbitrary age limit (<3 y at the time of diagnosis)

- -only type of idiopathic scoliosis whose most common curve pattern is left thoracic.
- only type of scoliosis that is more common in boys.
- more common in European patients or those of immediate European descent.
- spontaneous resolution (20-92%)
- Nonoperative treatment of progressive infantile idiopathic scoliosis predominates and may
involve the use of conventional thoracolumbosacral orthosis (TLSO)–type braces, Milwaukee-
type braces, and even intermittent Risser casting
- If surgical treatment becomes necessary, anterior release and fusion followed by posterior
spinal fusion with instrumentation is considered to be the functional treatment.

 rib vertebral angle difference (RVAD) originally described by Mehta in 1972.

- measurement carried out at the apical vertebra of the curve

 A treatment outline for infantile idiopathic scoliosis may be as follows

 Curves less than 25° with an RVAD less than 20° are preferentially observed and monitored with
spinal radiographs at regular intervals.

 Curves exceeding these parameters are typically braced, with some consideration given to the
value of intermittent Risser casting.

 Surgery is considered for curves not adequately controlled with nonoperative measures.

 Juvenile idiopathic scoliosis

 most closely mimics the epidemiology and demographics of the adolescent version of the
disease.

 It is more common in females, and its most common curve pattern is a right thoracic curve.

 might be considered to be a malignant subtype of adolescent idiopathic scoliosis

 One potential treatment algorithm for juvenile idiopathic scoliosis is as follows:

 Observation for curves less than 25° with follow-up radiographs at regular intervals

 Bracing for curves that range from 25-40° and at least consideration of bracing (based on curve
flexibility) for curves from 40-50°

 Bracing for smaller curves that demonstrate rapid progression to the 20-25° range

 Surgical intervention for inflexible curves that exceed 40° or virtually any curve that exceeds
50°.

 Adolescent idiopathic scoliosis

 most common type of idiopathic scoliosis and the most common type of scoliosis overall.
 Treatment recommendations for adolescent idiopathic scoliosis are driven almost totally by
curve magnitude (the only caveat being that brace treatment is thought to be effective only in
patients who are still growing).

 observation for curves less than 30°, bracing of curves that reach the 30-40° range, and
consideration of surgery for curves that exceed 40°.