Peds Spine

Question 1

Atlantoaxial Rotatory Displacement (AARD)

Kid comes in with torticollis:
- tilted head after recent hx of infection.
- torticollis after a trauma

Answer 1

Xrays, get CT scan,
Soft collar if less than 1 week.

pediatric cervical spine rotatory instability caused by C1-C2 subluxation or facet dislocation. The most common presentation is a young child who presents with torticollis. Grisel’s syndrome is the condition of AARD following a respiratory infection, retropharyngeal abscess, or recent head/neck surgery. Diagnosis is made by dynamic CT scan of the cervical spine. Treatment can be a soft collar, halter traction, halo traction, or surgical fusion depending on the chronicity of the condition. If nonoperative modalities fail, the condition has been present for > 3 months, or the patient has neurologic deficits, then posterior C1-C2 fusion is indicated

AAOS: Cervical traction, usually initiated with a Halter device, is the most appropriate initial treatment. Halo traction may eventually be required, especially in long-standing cases, because of the inability of a child’s skin to handle the traction weight and difficulty maintaining Halter traction at increased weights. The CT scans show an atlantoaxial rotatory fixation of C1 on C2, which can happen after acute trauma. Given the long-standing nature of the problem, a soft cervical collar will be of no benefit but may be useful in acute cases associated with upper respiratory tract infections. Sternocleidomastoid release is used in cases of congenital muscular torticollis, a condition ruled out by this child’s history. Posterior cervical fusion is reserved for children who have failed nonsurgical treatment such as traction.

Question 2

Congenital Muscular Torticollis
what is it? which what does the head tilt and chin rotate? What do you tell parents?

Answer 2

*most common cause of torticollis in infancy
* abnormal contraction of the sternocleidomastoid muscle
* Head tilts toward SCM
* palpable neck massfrom a contracted sternocleidomastoid muscle with the chin rotated towards the contralateral side.
* cervical rotational deformity with chin rotation away from the affected side and head tilt towards the affected side
* Painless! incomparison to AARD
* Typically resolved in 1 year with passive stretching (92-95%)
* bipolar release if fails to resolve after 1 year

Dx: U/S may be used to r/o other causes
Associated w/ packaging disorders: DDH, Meta Adductus, calc valgus
Passive stretching: lateral tilt away from affected side, rotate towards affected side

Question 3

AIS

left sided curve

Answer 3

Left: get an MRI to r/o cyst or syrinx
(right side is common)

Question 4

AIS

RIsk Factors for curve progession

Answer 4

Curve Magnitude:
* Before skelatal Maturity: >25, progress
* After: >50 thoracic, progress @ 1-2 per year; >40 lumbar, progress @ 1-2 per year

Remaining Skeletal grow:
* younger age, tanner score <3 for females
* risser 0-1 (risser 0 first 2/3 of pubertal growth; correlates with greatest velocity of linear growth)
* open tririadiates
* peak growth velocity is best predictor of curve progression

Curve Type:
* Thoracic progresses more than lumbar

Question 5

AIS

Adams forward bend,scoliometer

Answer 5

7 degrees correlates to 20 deg coronal plane curve

Question 6

AIS

Indications for MRI for AIS

Answer 6

atypical curve pattern (left thoracic curve, short angular curve, apical kyphosis)
rapid progression
excessive kyphosis
structural abnormalities
neurologic symptoms or pain
foot deformities
asymmetric abdominal reflexes
a syrinx is associated with abnormal abdominal reflexes and a curve without significant rotation

Question 7

AIS

Bracing for AIS:
Who gets a brace:
How often does the brace need to be worn?
Brace types?
Skeletal Maturity defined as?

Answer 7

• Bracing for patients with adolescent idiopathic scoliosis (AIS) is indicated for patients with curve magnitudes between 25°-45° who remain skeletally immature (Risser 0, 1, or 2)
• • 16-23 hours/day until skeletal maturity or surgical intervention deemed necessary (actual wear minimum 12 hours required to slow progression)
• apex above T7: Milwaukee brace (cervicothoracolumbosacral orthosis)
• apex below T7: TLSO, Boston, Charleston style (night time)
• Risser 4; <1cm change in height over 2 visits 6 months apart; 2 years postmenarchal

Question 8

AIS

Tx for skeletally mature patients w/ AIS

Answer 8

Skeletally mature patients (Risser 4 or 5) with 25-45° curves are treated with observation, as the risk of curve progression is minimal.

In all patients, curves >45-50° are treated with surgical stabilization, as curves in excess of 50° are associated with a high risk of continued worsening throughout adulthood.

Question 9

AIS

Who gets a TLSO for AIS?

Answer 9

TLSO is the mainstay of treatment for patients with adolescent idiopathic scoliosis (AIS) who are at a** risk of progression** (ie. Risser stage 0, curve magnitude 25-45 degrees) with an apex at or below T7.

Question 10

The most common deformity in AIS patients is a

Answer 10

right thoracic deformity with hypokyphosis (normal thoracic kyphosis is approximately 20-50 degrees). Therefore scoliosis with associated kyphosis deformity raises consideration for total spine MRI to evaluate the neural axis.

Question 11

AIS

Risser Staging and relation to bracing for AIS

Answer 11

The treatment of adolescent idiopathic scoliosis (AIS) depends on the magnitude and location of curve as well skeletal maturity of the patient. For curves less than 20 degrees, observation is appropriate until skeletal maturity, with closer intervals during times of peak growth. Curves between 20-25 and 45 degrees in patients who are Risser stage 0,1 or 2 are best treated with bracing to stop progression. Curves with an apex at T7 or below are typically treated with a Boston brace. Curves over 50 degrees generally warrant a discussion about surgery to prevent progression past maturity.

Question 12

Treat of AIS: observation for? Bracing for? What kind of brace?

Answer 12

The treatment of adolescent idiopathic scoliosis (AIS) depends on the magnitude and location of curve as well skeletal maturity of the patient.
Observation: curves less than 20 degrees, observation is appropriate until skeletal maturity, with closer intervals during times of peak growth.

Bracing:** Curves between 20-25 and 45 degrees** in patients who are Risser stage 0,1 or 2 are best treated with bracing to stop progression.

Curves with an apex at T7 or below are typically treated with a Boston brace. Curves over 50 degrees generally warrant a discussion about surgery to prevent progression past maturity.

Question 13

Risser Sign

Answer 13

0: 0 covers the first 2/3rd of the pubertal growth spurt and correlates with the greatest velocity of skeletal linear growth.

Risser I: is given when the ilium calcification measures 25%. As this stage the velocity of linear skeletal growth is on a descending slope.

Risser II: the greater trochanteric apophysis unites with the femur and Ilium calcification measures 50%. There is usually 3 cm of sitting growth remaining and no further growth in the lower extremities.

Risser III: Ilium calcification measures 75%. There is usually 2 cm of sitting growth remaining.

Risser IV: Ilium calcification is almost complete. There is usually 1 cm of sitting growth remaining.

Risser V marks complete skeletal maturation.

Question 14

AIS: AAOS

Brace wear time ??? correlates to % of success

Answer 14

90-93% success when the brace was worn for at least 12.9 hours per day.

AAOS: The efficacy of brace treatment for patients with adolescent idiopathic scoliosis is controversial because its effectiveness remains unproven. One of the challenges is patient noncompliance with prescribed bracing regimens. A recent study investigated curve progression based on actual brace wear using a temperature sensor to accurately assess brace wear. The total hours of brace wear correlated with lack of curve progression with a dose-response effect noted. Curves did not progress in 82% of patients who actually wore the brace more than 12 hours per day. For those who wore the brace for fewer than 7 hours per day, curves progressed in 69%. Prescribed bracing regimens (eg, 16 hours/day or 23 hours/day) had no effect on actual brace wear or curve progression.

Question 15

AAOS AIS

A 13-year-old girl with adolescent idiopathic scoliosis is otherwise healthy with a normal neurologic examination and she began her menstrual cycle 3 months ago. Standing radiographs show a high left thoracic curve from T1-T6 that measures 29 degrees, a right thoracic curve from T7-L1 that measures 65 degrees, and a left lumbar curve from L1-L5 that measures 31 degrees, correcting to 12, 37, and 10 degrees, respectively, on bending films. Her Risser sign is 1. What is the most appropriate management?

Answer 15

PSF of R thoracic curve

The patient has typical adolescent idiopathic scoliosis with a right thoracic curve. This represents a Lenke-1B curve pattern; therefore, only treatment of the thoracic curve is required. The proximal thoracic and thoracolumbar curves are very flexible. The patient is Risser 1 and has just started her menstrual cycles; therefore, she is at significant risk for further curve progression. Bracing is not appropriate for a curve of this magnitude and will not halt the progression of this curve, nor will vertebral body stapling stop this curve. Vertebral body stapling is sometimes useful in small thoracic curves of less than 35 degrees and skeletally immature patients. Anterior and posterior spinal fusion is not required because the patient has no other risk factors, such as neurofibromatosis nor is she at risk for crankshaft. Anterior fusion is an option, but it is not listed.

Question 16

AIS AAOS

What risk factor is most associated with progression of idiopathic scoliosis to a curve requiring surgery?

Answer 16

Magnitude of cuve >30 at peak height velocity
The magnitude of the curve at the time of the peak height velocity is the most prognostic sign in relationship to surgery. More than 70% of curves that measure more than 30 degrees at this time are likely to reach surgical range

Question 17

Bracing Indications from SRS

Answer 17

Scoliosis Research Society criteria: 25° to 45° curve, Risser grade 0 to 2, Sanders stage 4 or less

Question 18

Indications for surgical management are based on curve magnitude, clinical deformity, risk of progression, and curve pattern

Answer 18

In skeletally immature patients, thoracic curves greater than 45° to 50° should be corrected.

Curves greater than 50° with documented progression despite skeletal maturity also should be corrected.

Thoracolumbar curves smaller than 40° may cause considerable clinical deformity and may be corrected in skeletally immature patients.

Question 19

For juvenile scoliosis patients with more than a 20-degree Cobb angle, there is an approximately ??% prevalence of a neurologic abnormality.

Answer 19

For juvenile scoliosis patients with more than a 20-degree Cobb angle, there is an approximately 20% prevalence of a neurologic abnormality. Therefore, recommendations for work-up include an MRI scan of the entire spine.

Question 20

Skeletally immature patient, worsening kyphosis, whats the diagnosis and treatment?

Answer 20

The radiograph shows excessive thoracic kyphosis (normal 20 degrees to 50 degrees) with multiple contiguous vertebral wedging and end plate irregularity, all consistent with the diagnosis of Scheuermann’s kyphosis. The patient is skeletally immature; therefore, there is the potential for progression of the kyphotic deformity. Extension bracing has shown efficacy in the treatment of Scheuermann’s kyphosis that measures 50 degrees to 74 degrees, and has actually reduced the curvature permanently in some patients. A thoracolumbosacral orthosis may be used if the apex of kyphosis is at T7 or lower. Indications for surgical treatment are controversial, but spinal fusion most likely should not be considered for a painless kyphosis measuring less than 75 degrees

Question 21

Back Pain at night, relieved w/ nsaids, DX work up and tx

Answer 21

The CT scan shows a classic “target” lesion, and the bone scan has intense uptake at the site of the osteoid osteoma. The child has had a 2-year history of pain that even wakes her from sleep, so observation and anti-inflammatory drugs is not a preferred treatment. Bracing will not help with the discomfort because the pain is not mechanical in nature. MRI would not be needed in addition to the studies already completed. The osteoid osteoma is close to the spinal cord so radiofrequency ablation is not preferred. Surgical removal and biopsy is the treatment of choice.

Question 22

tx for congenital hemivertebral

Answer 22

Hemivertebra excision and limited fusion

resection for hemivertebra-related congenital scoliosis who have a progression of their deformity. Because of the progression, observation is not appropriate for this patient’s deformity. Bracing has not been shown to alter the progression of congenital scoliosis. The “growing rod” technique is also not effective in preventing progression related to hemivertebra. Distraction instrumentation carries an increased risk of neurologic complications in children with congenital spine deformities. Progression after posterior arthrodesis alone can occur through the so-called “crankshaft phenomenon.”

Question 23

Congenital scoliosis is abnormal vertebral development that typically occurs at ____ weeks of gestation. As a result, ???? can form, which can lead to asymmetric growth of the spinal column and progressive deformity. The ???? also develop during weeks ???? of gestation, which may be affected by the same process causing the vertebral abnormalities. As such, patients with congenital scoliosis should be evaluated for ????. % of other Anomalies

Answer 23

Congenital scoliosis is abnormal vertebral development that typically occurs at **4 to 6 weeks **of gestation. As a result, **hemivertebra or block vertebrae **can form, which can lead to asymmetric growth of the spinal column and progressive deformity. The heart and kidneys also develop during weeks 4-6 of gestation, which may be affected by the same process causing the vertebral abnormalities. As such, patients with congenital scoliosis should be evaluated for cardiac and renal defects with an echocardiogram and renal ultrasound.
60% of other anomalies found

Congenital scoliosis is associated with other anomalies 60% of the time. These anomalies can appear independently, or as part of the VACTERL syndrome (vertebral anomalies, anorectal atresia, tracheoesophageal fistula, and renal and vascular anomalies). Other associated orthopedic conditions include clubfoot, developmental dysplasia of the hip, limb hypoplasia, Sprengel’s deformity, Klippel-Feil syndrome, foot asymmetry, vertical tali, leg atrophy and pes cavus.

Question 23

Congenital anomalies of the vertebral column are associated frequently with other organ system problems. In addition to radiographs of the spine, what other screening tests should be ordered?

Answer 23

Renal U/S, Echo, MRI spine
Approximately 60% of patients with congenital anomalies of the spine have other associated findings. The spine develops around the same time as the cardiovascular system, the genitourinary system, and the musculoskeletal system. Around 20% of patients with congenital scoliosis have an associated urologic abnormality. Approximately 25% of patients with congenital scoliosis have an associated cardiac defect. Spinal cord abnormalities in one study occurred in approximately 37% of patients with congenital scoliosis.

Question 23

Renal anomalies are found in as many as ??? of patients with congenital scoliosis, so a renal ultrasound should be obtained

Answer 23

1/3rd

Question 23

Congenital scoliosis needs following work up

Answer 23

• MRI prior to surgery (Tethered cord, syringomyelia, chiarri)
• Renal U/S or MRI
• ECHO

Question 23

What is Early Onset Scoliosis and what is the concern?

Answer 23

• Scoliosis before the age of 10, dx encompasses a large group of disorders.
• Main concern is pulmonary function.
• RF contributes to higher mortality in untreated EOS

EOS is defined as any spinal deformity with onset before age 10 years. The condition encompasses a heterogeneous group of diagnoses based on the etiology of the deformity,25,26 including idiopathic, syndromic, neuromuscular, and congenital scoliosis (Resource 1). Because spinal growth often is coupled with growth of the thorax and lungs, EOS frequently is associated with thoracic insufficiency syndrome, or the inability of the thorax to support normal respiration and lung growth. In normal lung, development of the alveoli is complete by age 2 years, with airway enlargement continuing until age 5 years and alveolar hyperplasia continuing until age 8 years. In children with unmanaged EOS, respiratory failure is well documented and contributes to higher mortality rates compared with rates in the general population. Respiratory failure is caused by intrinsic (ie, failure of alveolar multiplication) and extrinsic (ie, rib and chest wall deformities) factors. In EOS, intrinsic failure because of chest deformity prevents hyperplasia and development of the lung tissue, whereas the extrinsic rib and chest wall deformity leads to loss of compliance or functional incompetence of the chest wall. Because of the risk of thoracic insufficiency syndrome, management of EOS focuses on maintaining growth of the thorax and lung volume.

Question 24

Non surgical management of EOS: Casting

Answer 24

Serial cast immobilization is used to manage EOS. In patients with curves less than 60° who undergo cast immobilization before age 19 months, curvature is reduced to less than 10° at maturity. Serial casts are applied under general anesthesia and are changed every 8 to 12 weeks

Question 25

In mgmt of EOS w/ growing rods what outcomes has been seen overtime with subsequent lengthening?

Answer 25

rowing rod lengthening procedures to treat early-onset scoliosis, detailed measurements of the amount of distraction force required and length gained during the procedure were taken. The forces required to distract the spine increased over time, and mean length achieved with each distraction decreased. By the fifth lengthening, required distraction forced had doubled vs baseline, and fewer than 8 mm of growth was achieved with each subsequent lengthening.

Question 26

In infantile idiopathic scoliosis, which of the following factors suggests progression?

Answer 26

Rib overlap of the apical vertebra:
Rib overlap of the apical vertebral body or a rib vertebral angle difference of greater than 20 degrees indicates that the curve is likely to progress.

Infantile idiopathic EOS is diagnosed in patients younger than 4 years. The cause of the deformity is unknown, but more than 90% of cases resolve without management. Signs of progression are a RVAD greater than 20° and/or a phase 2 rib at the apex of the curve.

Infantile idiopathic scoliosis occurs more commonly in boys, with a 3 to 1 male to female ratio. Neural axis abnormalities, hip dysplasia, and congenital heart disease are all associated with the condition; spontaneous correction frequently occurs. Curve progression can be predicted by the rib vertebral angle difference or the phase of the rib head. Rib overlap of the apical vertebral body or a rib vertebral angle difference of greater than 20 degrees indicates that the curve is likely to progress. Gender, family history, and age at presentation have not been found to be risk factors for progression.

Question 27

What are the reasons to obtain an MRI of entire spine cord in scoliosis presentation?

Answer 27

These include her young age and the presence of a left-sided curve. For juvenile scoliosis patients with more than a 20-degree Cobb angle, there is an approximately 20% prevalence of a neurologic abnormality. Therefore, recommendations for work-up include an MRI scan of the entire spine.

Question 29

DMD scoliosis:
How is it different from idiopathic?
Benefits to bracing?
Tx:
Poor outcomes associated with?
Periop considerations:

Answer 29

• progresses with general kyphosis and scoliosis with varying degrees of pelvic obliquity as opposed to lordosis normally seen in idiopathic scoliosis
• bracing contraindicated
• Early PSF w/ instrumentation curve 20-30 in non ambulator patient, curves >40 pelvic obliquity >10, tx early befor pulmonary function declines
• FVC drops less than 35 percent, poor outcomes, reduced reserve, increase risk of ventilatory comprose and need for support.
• Peri-op: cardiac, pumonary work up

Question 30

Scoliosis in SMA manifests as

Answer 30

** long, C-shaped thoracolumbar curves which are highly flexible. These curves have high progression rates despite bracing**. The natural history of this scoliosis is progressive sitting imbalance which requires use of the upper extremities to maintain an upright sitting position. Thus the primary surgical goal of correction is to obtain fusion in a position that will improve sitting balance and free the patient’s upper extremities for other tasks.

Question 31

treatment of scoliosis in DMD

Answer 31

Patients with DMD commonly develop scoliosis at an early age, and often require early surgery (before skeletal maturity) to prevent further complications such as pulmonary compromise. Typically surgery should be done for **curves greater than 20°, but before a curve reaches 30°, especially if the patient is already non-ambulatory, has a declining FVC (an FVC below 35% is an indication for surgery), and if rapid progression of the curve is noted on serial examinations. Surgery can be delayed until the curve reaches 40° if the patient’s respiratory function is responding well to steroid treatments.

Unlike adolescent idiopathic scoliosis, scoliosis in Duchenne’s muscular dystrophy is treated early; spinal fusion for a 40-degree deformity is not unusual. Although hematology and neurology consults usually are not necessary prior to surgery, every child should have a comprehensive cardiac evaluation, including an EKG and an echocardiogram because cardiomyopathy is part of the pathologic spectrum of Duchenne’s muscular dystrophy requiring preoperative assessment and intervention.