O&T SC044: A Child With Deformed Spine: Congenital Deformities And Other Orthopaedic Problems

Question 1

Plane of deformities of Spine

Answer 1

1. Frontal (Coronal plane) (AP / PA)
   - structural deformity: Scoliosis (shoulder level, pelvis level, spine)
   - non-structural deformity: Pain (causing the patient to tilt to one side)
2. Sagittal (Lateral plane)
   - Cervical lordosis (hyper / hypo)
   - Thoracic kyphosis (hyper / hypo)
   - Lumbar lordosis (hyper / hypo)

—> Combined Frontal + Sagittal (Kyphoscoliosis)

3. Axial (Rotational plane)
   - rotational problem (always occur in Scoliosis e.g. in thoracic spine —> rib cage more prominent on one side —> ***bend over —> form a hump at the back)

OSCE:
- Deformities of spine can often be only seen when ***standing (lying cannot see)
- Ask the patient to stand up first (observe deformity) —> walk patient to couch (observe gait) —> lie patient down (P/E)

Question 2

Scoliosis

Answer 2

Radiograph:
- a 3D deformity
- a curve **>10o measured by the Cobb technique (coronal plane)
- standing radiograph of spine
- with **rotational component (seen in axial plane by CT)

Descriptive:
1. Rotation (hump, scoliometer)
2. Truncal shift (extent of shifting of thoracic cage compared with extent of shifting of pelvis —> distance >2 cm between Vertical trunk reference line (VTRL) (mid point between 2 ribs) and Center sacral vertical line (CSVL))
3. Plumb line shift (distance between C7 spinous process from CSVL)
4. Truncal list

Coronal / Truncal balance:
- a vertical line from middle of head to middle of pelvis (head is exactly on top of pelvis regardless of degree of scoliosis in middle)

Early correction in scoliosis important in children:
- **Extorsion / Intorsion of eye maybe fixed
- **Alveoli cannot develop in fixed rib cage —> Consequential **restrictive pulmonary disease (after 8 yo when development finished)
- Other organs involved: **Heart, ***Great vessels

2 Types:
1. **Structural (Intrinsic bony problem)
2. **Non-structural (Not bone problem but give clinical appearance of scoliosis)

Question 3

Structural scoliosis

Answer 3

Scoliosis ***CANNOT be fully corrected by lateral flexion

3 characteristics (∵ prolonged curvature of spine):
1. Soft tissue contracts in the concavity of the curve
2. Structural changes to bone itself
- Wedging of vertebral body (變左梯形)
- Variations in size of laminae, pedicle, transverse processes, ribs (if thoracic spine)
3. ***Fixed rotatory deformity of involved

Question 4

Non-structural scoliosis

Answer 4

• ***Absence of structural changes + rotation of vertebral body
• Scoliosis **CAN be corrected / overcorrected by **lateral flexion

1. Postural
2. Nerve root irritation (e.g. prolapsed IV disc)
3. Inflammatory / Infection
4. Spinal tumours
5. Hysterical
6. Compensatory (of other abnormalities)

Question 5

***Causes of Scoliosis

Answer 5

Structural / Spinal (**have rotational component):
1. **Idiopathic (Dx of exclusion, most common)

2. Congenital (diagnosed by X-ray)
   - e.g. Block vertebrae, Unsegmented bar, Wedge vertebrae, Hemivertebrae
3. ***Neuromuscular (mixture of conditions —> asymmetric pull of different forces acting on spine)
   - CNS: Cerebral palsy (asymmetric spasticity along spine)
   - Spinal cord: Syringomyelia (e.g. in cervical spinal cord)
   - Anterior horn cells: Poliomyelitis
   - Motor nerve roots: SMA
   - Muscles: Duchenne Muscular dystrophy
4. ***Syndromes
   - Neurofibromatosis
   - Osteogenesis imperfecta
   - Marfan
   - Ehlers-Danlos
5. Traumatic
6. Others (Infection, Tumour, Metabolic)

Non-structural / Extra-spinal:
1. **Pelvic obliquity
2. **Leg length discrepancy
3. ***Sciatic (∵ Radiculopathy can lead to painful posture)
4. Postural

Question 6

Kyphosis

Answer 6

Normal contour:
- Cervical lordosis
- Thoracic kyphosis
- Lumbar lordosis

Normal Thoracic kyphosis:
- Normal range 20-40o (huge range)

Question 7

***Causes of Kyphosis

Answer 7

Structural:
1. Congenital kyphosis
- Spine palpable at a particular area

2. TB kyphosis (Infective cause)
3. Ankylosing spondylitis (Inflammatory cause)
   - **Question mark deformity (ascending fusion from SI joint from caudal to cranial) —> compensate by **knee flexion + ***hip extension to look up —> treatment: osteotomy to break bones up and bring spine up straight
4. Scheurmann’s kyphosis
   - Adolescent
   - Sagittal curve >45o
   - Wedging of 5o in >=3 consecutive vertebrae
   - End plate irregularities
   - Progressive —> lead to various problems —> may surgically fuse spine in a better position

Nonstructural:
1. Postural kyphosis
- Non-structural (i.e. nothing intrinsically wrong with bone / not hyperkyphosis)
- Causes: Vertebral collapse / Disc degeneration causing decrease in disc height
- Problem: Back muscle fatigue —> Mechanical musculoskeletal back pain (trying to pull spine back up)
- Usually <60o
- ***Flexible
- May be precursor to osteoporotic spine
- Treat with hyperextension exercises
- Brace after 3 months if exercise fails

Question 8

***Assessment of children with spine

Answer 8

1. Assessment
   - Etiology
   - Severity
   - Prognosis
2. History
   - Parental demographics
   - **Antenatal history
   - **Birth history
   - **Postnatal complications (e.g. cerebral palsy due to hypoxic damage)
   - Onset of deformity
   - **Developmental milestones, progressive weakness
   - Associated medical comorbidities
   - ***Familial history (e.g. syndromal problems such as NF)
3. Examination
   - General examination
   - Balance and motor control
   - Cutaneous manifestations (Syndromal presentations: NF, Tuft of hair (Spina bifida), Blue sclera (OI))
   - Flexibility assessment
   - Neurological examination
4. Investigation
   - Plain radiographs
   - Renal USG
   - Cardiac Echo
   - Respiratory function
   - CT spine
   - MRI spine
5. Treatment

Question 9

Clinical symptoms of “syndrome of contractures”

Answer 9

1. Plagiocephaly
2. Torticollis sinister
3. Abduction contracture of right hip
4. Adduction contracture of left hip

Question 10

Spinal development

Answer 10

• Fetal spine forms between 4th and 5th week of pregnancy (Congenital spine problem —> other organs develop at this period also have problem)
• Sclerotome cells (somites) migrate medially + meet in midline around notochord (neural tube at dorsal, gut at ventral)
• Divided into cranial + caudal halves, fused with adjacent somite
• 3 primary chondrification centres appear for each vertebral segment
• Primary ossification centre of vertebral body appears at 9 weeks

Question 11

Congenital scoliosis

Answer 11

1. Somites did not split to cranial + caudal parts
   —> Failure of segmentation
   —> Block vertebrae, Unsegmented bar
2. One side of somite underwent apoptosis
   —> Failure of formation
   —> Wedge vertebrae, ?Hemivertebrae
3. One side of somite duplicate / split further
   —> Duplication
   —> Hemivertebrae

• Girls:Boys = 2.5:1
• Clinically apparent at birth / within first year of life
• Subsequent deterioration occurs at varying rates, but the condition is always progressive until the child reaches skeletal maturity
• ***2 peaks of deterioration (∵ 2 peaks of growth): 0-5, 10-18 yo
• Children whose curves are detected at a later age tend to have progression during the preadolescent growth spurt

**Associated anomalies:
- 30-60% of children
- Most common:
1. **Spinal cord (e.g. spinal bifida, split cord, myelomeningocele, syringomyelia) —> **MRI
2. **Cardiac system (e.g. valvular problems) —> **Echo
3. **Genitourinary tract (e.g. horseshoe kidney, duplicate kidney, one kidney) —> ***USG
4. Cervical spine
—> ∵ embryologic development of spinal cord coincides with development of many other organ systems

Associated ***intraspinal anomalies common:
1. Tethered cord
2. Diastematomyelia (split into 2)
3. Diplomyelia (duplicated)
4. Syringomyelia
5. Low conus medullaris (occur in 18-38% of neurologically normal patients)

***Genitourinary tract (20-40%):
1. Renal agenesis
2. Ectopic kidney
3. Horseshoe kidney
4. Duplication
5. Urethral anomalies
6. Reflux

Development of ***restrictive lung function (hypoplastic lung) occurs only as curve approaches 90o
- Severe curves produce most severe pulmonary compromise
- due more to hypoplastic lung rather than physical constriction from curve
- alveolae develop in growing child up to 8 yo

Question 12

Investigations

Answer 12

1. Plain radiographs (PA + Lateral: Standing spine, Suspension spine (i.e. Bending))
   - Bony (Osseous) anomalies (e.g. hemivertebrae, wedge vertebrae, bar)
   - Magnitude of curve (Cobb angle)
   - Degree of rotation (Size, shape, position of pedicles —> should be symmetrical)
   - Bending films (see if spine flexible / rigid)
   - Signs of skeletal maturation (tell stage of development)
   —> **Risser sign
   —> **Ring epiphysis
   —> ***Bone age (sequential ossification of physis of phalanges —> distal radius / ulner))
   ——> also tells how much more deterioration the scoliosis can have
2. Renal USG
3. Cardiac Echo
4. Respiratory function
5. CT spine
6. MRI spine

Question 13

Conditions that always deteriorate

Answer 13

1. Hemivertebra with a bar (always progressive)
2. Hemivertebra (depends on balance of growth plates (discs))
3. Hemivertebra at base of spine (usually progressive)
   (vs Block vertebra: does not progress)

—> ***Early treatment warranted instead of waiting till angle get worse

Question 14

Treatment considerations for spinal deformities in young children

Answer 14

1. Rapidly growing spine
   - Early fusion undesirable
2. Maintenance of pulmonary development

Question 15

Natural history of Scoliosis

Answer 15

• 75% patients require treatment
• 84% untreated developed curves >40o by maturity
• Prognosis / Ultimate progression related to:
  —> type of anomaly + anatomical site of curve

Mortality:
- Increased in severe curves >70o
- Mainly affects infantile / juvenile (∵ cardiorespiratory function compromised) but NOT adolescents

Question 16

Management options

Answer 16

3 O’s:
1. ***Observation
- If know the child will not get worse —> monitor until skeletal maturity
- Know child will get worse but too young to treat

2. Orthosis
   - **Casting (use plaster of Paris to mold the spine —> requires Risser table to pull spine straight) —> usually only applicable up to 2 yo
   - **Bracing (immature rib cage can deform with brace) —> for older children
   - Halo-gravity traction (gradual traction pulling child straight, very minimal risk of damage spinal cord vs operative treatment)
3. Operative treatment
   - ***Fusion of 5 yo child (12.5 cm loss of height —> undesirable —> not recommended)
   - Growth guidance
   - Growth modulation

Question 17

Indications for surgery

Answer 17

1. ***Late diagnosis
2. Severe curves (***>45o)
3. ***Imbalance
4. ***Failed brace treatment

Cannot be done too young (∵ cannot put instrument)
—> usually after 5-6 yo

Example:
- Hemivertebrae —> excise extra vertebrae + fuse segment

Question 18

Growing instrumentations

Answer 18

Internal brace
- to achieve + maintain deformity correction during spinal growth
- serial lengthening every 6-12 months

Question 19

Summary

Answer 19

1. Broad categories of spinal deformities (3 planes)
   - Scoliosis
   - Kyphosis
   - Kyphoscoliosis
2. Etiologies of spinal deformities
   - Idiopathic
   - Congenital
   - Neuromuscular
   - Syndromic
3. Associated anomalies in Congenital scoliosis
   - Intraspinal
   - CVS
   - Renal
4. General approach
5. Principles of treatment (3 O’s)
   - Observation
   - Orthosis
   - Operative treatment

Question 20

SpC O/T Seminar: Scoliosis
Scoliosis

Answer 20

• Lateral deviation of spine
• a 3D deformity: a Rotational deformity as well
• Signs:
  1. **Unlevel shoulders (Left vs Right trapezius)
  2. **Humps
• Scapula hump (at Upper thoracic level)
• Rib hump (at Lower thoracic level, hump usually at same level of convexity)
• Loin hump (at Lumbar level, usually opposite side to Rib hump)
  3. **Waist line asymmetry (indented on one side + straight on other side)
  4. **Truncal shift (whether chest is centred on pelvis)
  5. ***Limb length
  6. Scar
  7. Skin changes

Early scoliosis:
- Symptoms are **rare
—> detected by school screening (start at **10 yo) / incidental finding on CXR (e.g. pneumonia)
1. **Adam’s forward bending test
- look from back for humps (i.e. rotational deformity) —> **>1cm difference between 2 sides —> proceed to Moire topography

2. **Moire topography
   - glass panel with contour map pattern —> **>2 line difference between height of 2 sides —> refer to X-ray —> usually curve >20o on X-ray
3. X-ray
   - Definitive test, not done in every person ∵ radiation + costly if screen for everyone
   - ***Cobb angle (worst angle measured on X-ray)
   —> apex of curve (defined as the vertebral body furthest from midline)
   —> angle between 2 most tilted lines from apex

Question 21

Assessment of Scoliosis

Answer 21

Specialist clinic
- Scoliosis >20o from screening programme

1. Assessment (**Etiology + **Severity + ***Prognosis)
   - History
   - P/E
   - Investigations (X-ray) (further investigations only needed if etiology not obvious)
2. Make diagnosis (can be made without further investigations)

Question 22

History taking in Scoliosis

Answer 22

1. Age
   - Directly related to **diagnosis
   —> Infantile scoliosis (0-3 yo)
   —> Juvenile (3-10 yo)
   —> Adolescent (10-18 yo)
   —> Adult (>18 yo)
   - <10 yo: higher chance of congenital etiology, higher association with intraspinal abnormalities
   - **Treatment implications ∵ growth potential
2. Gender
   - Adolescent idiopathic scoliosis (AIS): most common form, more common in ***females
3. Symptoms
   - Symptoms are ***rare (esp. in AIS) —> If pain present —> suspect other (non-idiopathic) causes
4. Birth history
   - **Congenital syndromes (e.g. **Tetralogy of Fallot: associated with congenital spinal abnormalities)
5. **Developmental milestones
   - Delayed milestones
   —> Birth trauma (e.g. brain injury)
   —> **Syndromal disorders (chromosomal disorders / gene defects)
6. ***PMH
   - May provide clues to cause
   —> Malignant primary
   —> Spine trauma
   —> Inflammatory arthritis
   (although uncommon to cause scoliosis)
7. ***Family history
   - AIS run in families
   - Other rare syndromes (e.g. Neurofibromatosis, Marfan syndrome)
8. ***Menarche
   - AIS deteriorates with puberty growth spurt
   - Peak height velocity
   —> 1 year before onset of menarche to 1 year after, growth completed 2 years post-menarche
   —> 6-8cm per year of height gain
   —> 1-2 degree per month (after skeletal maturity: 1-2 degree per year)

Question 23

P/E in Scoliosis

Answer 23

Posture: Standing
Exposure: Spine + Limbs

Standing, Look from back
1. ***Deviation of spine (Describe position with reference to convexity)

2. ***Scapula, Rib, Loin hump
3. Asymmetry (***Waist line)
4. Balance
   - **Shoulder level
   - **Listing (plumb line from C7, normally through buttock cleft, measure distance of line from buttock cleft —> objective measurement of **balance)
   - **Truncal shift (position of chest with reference to pelvis —> produce waist line asymmetry —> cosmetic problem)
5. Adam’s forward bending test
   - Rotational deformity (Scapula hump, Rib hump, Loin hump)
   —> Scoliometer to measure size of hump (Angle of trunk rotation (ATR))
6. Sagittal profile
   - Hypokyphosis (Flattening) of thoracic spine
   - Kyphoscoliosis
   - Acute kyphosis (may suggest acute causes e.g. trauma, infection, malignancy)
7. ROM
   - Side flexion (more flexible —> easier to treat)

Lying
1. **Pelvic obliquity
2. **True Leg length
3. ***Neurological examination (suggest spinal cord anomaly)
- Lower limb
- Superficial abdominal reflexes
4. Skin changes of neurofibromatosis
5. Ligamentous laxity

6. Maturity
   - Secondary sexual characteristics
   - Menarche
   - **Risser sign on X-ray —> Iliac wing epiphysis (calcification start from front to back (stage 1-4) —> more calcification —> skeletally more mature)
   - **Bone age (left hand) —> Hand epiphysis (disappearance from distal to proximal, ulnar to radial)

Question 24

Untreated Scoliosis

Answer 24

Early identification —> Early treatment —> Prevent complications
1. ***Cardiopulmonary compromise
- Occupy chest volume on deviated side
- Crowded ribs on contralateral side —> impair chest wall movement
—> both cause respiratory compromise / failure
—> Cor pulmonale, Pneumonia etc.

2. Back pain
3. ***Truncal imbalance
   - Excessive loading on concavity —> Accelerated degeneration of spine (OA) —> Back pain, Sciatica
4. ***Cosmetic
5. Increased mortality in severe curves (>70o), mainly affect infantile, juvenile scoliosis

Question 25

Adolescent idiopathic scoliosis (AIS)

Answer 25

• Most common scoliosis
• F:M = 4:1
• Age 11-14, Deteriorate during ***Growth spurt
• ***Diagnosis of exclusion

Aims of treatment:
1. Stop curve progression (if identify early)
2. Correction of deformity (if identify late)
- significant curvature / cosmetic deformity / cardiopulmonary issue

Prevalence:
- >10o: 3%
- >20o: 0.5%
- >30o: 0.2%
- >40o: 0.1%
—> Mild curves are more common, Severe curves are rare
—> **NOT all curves progress, **NOT all curves require treatment

Which curve will deteriorate:
1. During adolescent **growth spurt (i.e. A lot of growth remaining)
- **Menarche
- Height
- **Risser sign
- **Ring apophysis
- ***Bone age

2. ***Cobb angle >50o

Question 26

Treatment of AIS

Answer 26

1. Bracing
   Indications:
   - Early diagnosis (i.e. **Mild curves <30o)
   - **Skeletally immature
   - **NOT correct deformity, only **prevent progression
   - Underarm brace
   —> 3 point pressure to keep spine straight
   —> cosmetically more acceptable
   —> apex below T7
2. Surgery
   Indications:
   - **Late diagnosis (i.e. **Severe curves >45o)
   - **Imbalance
   - **Failed brace treatment
   —> check Compliance, Fitting of brace, Take brace off to check again

Aim:
- Arrest progression
- Achieve spinal fusion
- Correct deformity
- Restore balance
- Improve cosmesis

Disadvantage:
- **Stop growth
- **Stiff spine
- Risk of surgery

Post-op course:
- Surgery 4 hours
- Blood loss 500-800ml
- HDU overnight
- Mobilise day 3
- Home day 5-7 post-op
- No external immobilisation
- Return to school 2-3 weeks
- No exercise for first 3 months
- Normal activities by 6 months

Question 27

Summary of Seminar

Answer 27

• Scoliosis is a symptom / sign, NOT a cause
• AIS is most common cause, but diagnosis of exclusion
• AIS progression is associated with growth spurt —> need to assess growth spurt
• Growth spurt assessment
  —> Clinical: Height gain, Menarche (peak height velocity: 1 year before onset, 1 years after, completed by 2 years post-menarche), Secondary sexual characteristics
  —> Radiological for bone age: Hand X-ray, Risser sign
• Treatment for progressive curves
  —> Brace if mild
  —> Surgery if severe