High-yield overview
Pediatric Spine | CP, DMD, SMA | Pelvic Obliquity | Long Constructs
90%Prevalence in GMFCS V
PelvisMust include in fusion
PulmonaryFunction declines 4% / year (DMD)
InfectionHigher risk than AIS
ETIOLOGY
Upper Motor Neuron
PatternCerebral Palsy, Friedreich's Ataxia, Syringomyelia
TreatmentSpasticity management + Surgery
Lower Motor Neuron
PatternSMA, Polio, Myelomeningocele
TreatmentFlaccidity + Surgery
Myopathic
PatternDuchenne MD
TreatmentEarly surgery (Cobb greater than 20-30)
Critical Must-Knows
- Definition: Scoliosis caused by neuropathic or myopathic disorders leading to trunk muscle imbalance.
- Pattern: Typically long, C-shaped thoracolumbar curve with pelvic obliquity.
- Goal: A straight spine over a level pelvis to allow comfortable sitting (and nursing care).
- Pre-Op Optimization: Nutrition (Pre-albumin), Pulmonary function, Seizure control, Bowel regimen.
- Fixation: Generally T2/T3 to Pelvis (Galveston / Iliac screws).
Clinical Pearls
- "In Duchenne (DMD), fuse EARLY (Cobb greater than 20°) to preserve lung function. Don't wait for 50°.
- "Pelvic Obliquity causes ischial pressure sores on the 'down' side.
- "GMFCS Level correlates directly with scoliosis risk (Level I less than 5%, Level V = 90%).
- "Beware of 'Malignant Hyperthermia' in myopathic conditions.
Cardiopulmonary Risk
Duchenne Cardiomyopathy
Fatal Risk. DMD patients have progressive cardiomyopathy. Must have detailed Echo/Cardiology review. Ejection Fraction determines eligibility.
Pulmonary Reserve
FVC less than 30%. Patients with forced vital capacity less than 30% predicted need post-op ventilation planning and have high risk of pneumonia/death.
Nutritional Status
Albumin / Transferrin. Malnutrition is rampant (G-tubes often needed). Poor nutrition = Infection + Wound Breakdown.
Seizure Control
Valproate. Ensure levels are therapeutic. Post-op seizures can break rods!
At a Glance: AIS vs Neuromuscular
| Feature | Adolescent Idiopathic | Neuromuscular |
|---|---|---|
| Curve Pattern | Structural S-shape (Right thoracic) | Long C-shape (Thoracolumbar) |
| Pelvic Obliquity | Rare | Common (Needs fixation) |
| Progression | Stops at maturity | Continues after maturity |
| Surgical Goal | Cosmesis + Prevent progression | Sitting balance + Nursing care |
| Fusion Levels | Selective (save motion) | Long (T2-Pelvis) |
Mnemonic
NUTRITIONPre-operative Checklist
| N | Nutrition Albumin greater than 3.5, TLC greater than 1500 |
| U | Urine Rule out UTI |
| T | Tone Baclofen pump? Spasticity? |
| R | Respiratory PFTs, Sleep study |
| I | Infection Skin, teeth, ears clearance |
| T | Team ICU, Peds, Resp, Dietician |
| I | Imaging Traction films, Pelvic incidence |
| O | Osteopenia Dexa? Bisphosphonates? |
| N | Neurology Seizure control |
| N | Nutrition Albumin greater than 3.5, TLC greater than 1500 | R | Respiratory PFTs, Sleep study | I | Imaging Traction films, Pelvic incidence |
| U | Urine Rule out UTI | I | Infection Skin, teeth, ears clearance | O | Osteopenia Dexa? Bisphosphonates? |
| T | Tone Baclofen pump? Spasticity? | T | Team ICU, Peds, Resp, Dietician | N | Neurology Seizure control |
Hook:NUTRITION is the most commonly missed optimization factor.
Mnemonic
BEACHClinical Assessment
| B | Balance Sitting balance / Head control |
| E | Etiology CP vs DMD vs SMA |
| A | Associations Seizures, cognitive, nutrition |
| C | Curve Flexible vs Rigid |
| H | Hips Dislocation risk |
| B | Balance Sitting balance / Head control | C | Curve Flexible vs Rigid |
| E | Etiology CP vs DMD vs SMA | H | Hips Dislocation risk |
| A | Associations Seizures, cognitive, nutrition |
Hook:Don't forget the BEACH when assessing these kids.
Mnemonic
SITSurgical Goals
| S | Sitting Balance Head centered over pelvis |
| I | Ischial Pressure Level pelvis to prevent ulcers |
| T | Torso Height Maximize space for lungs/abdomen |
| S | Sitting Balance Head centered over pelvis |
| I | Ischial Pressure Level pelvis to prevent ulcers |
| T | Torso Height Maximize space for lungs/abdomen |
Hook:The goal is to help them SIT comfortably.
Overview and Epidemiology
Neuromuscular Scoliosis (NMS) is a spinal deformity associated with a heterogeneous group of disorders including Cerebral Palsy (CP), Duchenne Muscular Dystrophy (DMD), Spinal Muscular Atrophy (SMA), and Spina Bifida.
Key Characteristics:
- Onsent: Often early (less than 10 years).
- Use: Most patients are wheelchair-bound (non-ambulatory).
- Progression: Unlike idiopathic scoliosis, NMS curves continue to progress after skeletal maturity due to loss of trunk control and gravity.
- Impact: Severe curves compromise sitting balance (hands needed for support), cause pelvic obliquity (pressure sores), and restrict pulmonary function (pneumonia risk).
Pathophysiology and Biomechanics
Pathomechancis:
- The primary driver is muscle weakness (DMD/SMA) or spasticity (CP).
- Unlike AIS (rotation driver), NMS is often a collapsing deformity under gravity.
- Pelvic Obliquity: The "foundation" of the spine acts like a tilted table.
- Causes: Suprapelvic (spine curve) or Infrapelvic (hip contracture).
- Consequence: Pressure ulcers on the low side, hip dislocation on the high side.
Classification
Gross Motor Function Classification System (CP)
The risk of scoliosis directly correlates with GMFCS level.
| Level | Description | Scoliosis Risk |
|---|---|---|
| I | Walks without limits | Low (less than 5%) |
| II | Walks with limitations | Low-Mod |
| III | Walks with handheld mobility | Moderate |
| IV | Self-mobility with limitations (Wheelchair) | High (~50%) |
| V | Transported in manual wheelchair (No head control) | Very High (greater than 90%) |
Clinical Assessment
Pre-operative Assessment
AskHistory
- Function: Can they sit? Do they use hands for support?
- Pain: Is the curve painful? (Rare in NMS, look for hip dislocation).
- Pulmonary: History of pneumonia? ICU admissions? CPAP?
- Seizures: Frequency? Medications?
- Nutrition: Feeding tube (PEG)? Weight loss?
Look/FeelExamination
- Sitting Balance: Does the patient list to one side?
- Pelvic Obliquity: Palpate iliac crests sitting. Check for ischial sores.
- Hip Contractures: Hip flexion contracture greater than 20 deg exacerbates lumbar lordosis/kyphosis.
- Windswept Hips: One hip abducted, one adducted.
- Skin: Inspect back and buttocks for breakdown.
PlanSurgical Decision
- Cobb Angle: Surgery typically indicated for Cobb greater than 50 degrees (CP) or greater than 20-30 degrees (DMD).
- Pelvic Obliquity: If greater than 15 degrees, strictly consider fusion to pelvis.
Hip Dislocation
Always check the hips. Pelvic obliquity drives the "high" hip into adduction, leading to subluxation/dislocation. A dislocated hip can be the primary source of pain, not the spine.
Investigations
Radiographic Parameters
| View | Parameter | Significance |
|---|---|---|
| AP Spine (Sitting) | Cobb Angle | Magnitude of deformity. Greater than 50 deg usually operative. |
| AP Spine (Sitting) | Pelvic Obliquity | Angle of pelvis relative to horizontal. Drives extensions to pelvis. |
| Lateral Spine | Kyphosis/Lordosis | NMS often kyphotic (collapsing). |
| Bending/Traction Films | Flexibility | Determines if anterior release is needed (rare nowadays with pedicle screws). |
Blood Work (Optimization):
- Albumin / Pre-albumin: Marker of nutrition. Albumin greater than 3.5 g/dL desired.
- Total Lymphocyte Count: greater than 1500.
- Hematocrit: Optimize pre-op.
- Coagulation Profile: Valproate can affect platelets/factors.
Differential Diagnosis
Not every curved spine in a child with disability is "simple" neuromuscular scoliosis. Distinguish the collapsing C-curve from mimics that change management.
Distinguishing NMS From Look-Alikes
| Condition | Curve / Clue | Key Differentiator |
|---|---|---|
| Adolescent Idiopathic Scoliosis | Structural right thoracic S-curve, normal neurology | No underlying neuromuscular disease; stops at maturity; rarely needs pelvis |
| Congenital Scoliosis | Short, sharp curve; hemivertebra/bar on X-ray | Vertebral anomaly from birth; screen for VACTERL, cardiac and renal anomalies |
| Syndromic Scoliosis (e.g. Marfan, NF1) | Variable curve plus systemic features | Dystrophic ribs/scalloping (NF1), arachnodactyly/lens (Marfan); dural ectasia |
| Syringomyelia / Cord Pathology | Atypical (left thoracic), painful, or rapidly progressive | Abnormal abdominal reflexes; MRI mandatory for atypical curves |
| Painful / Antalgic List (e.g. dislocated hip, osteoid osteoma) | Postural lean, not a fixed structural curve | Resolves when underlying pain treated; hip dislocation is the classic NMS trap |
When to Order an MRI
An atypical curve (left thoracic), a painful curve, rapid progression, abnormal abdominal reflexes, or a new neurological sign mandates whole-spine MRI to exclude a syrinx, tethered cord, or intraspinal tumour before attributing the deformity to the underlying neuromuscular condition.
Management Algorithm
Algorithm
Role of Bracing
- Controversial / Limited Use.
- Bracing does NOT halt progression in neuromuscular curves.
- Purpose: To provide sitting support ("Soft brace" or TLSO) to delay surgery until larger size reached.
- Risk: Pressure sores, restrictive lung defect (compresses ribs).
Wheelchair Modifications
- Custom molded seat backs.
- Wheelchair modifications.
- Lateral trunk supports.
- Tilt-in-space mechanisms.
Bracing is palliative at best.
Surgical Technique
The "Unit Rod" vs Pedicle Screws
Unit Rod (Luque-Galveston):
- Historic gold standard.
- Pre-bent U-shaped rod with sublaminar wires.
- Legs of rod driven into ilium (Galveston technique).
- Pros: Cheap, distributed force (less pullout). Cons: Wire passage risk, limited lordosis control.
All-Pedicle Screw Constructs:
- Modern standard.
- Iliac Screws or S2-Alar-Iliac (S2AI) screws for pelvic fixation.
- Pros: Better correction, 3-column fixation, no canal entry. Cons: Pullout in osteoporotic bone.
Levels:
- Upper: T2 or T3 (prevent proximal kyphosis).
- Lower: Pelvis (if obliquity greater than 15 deg or non-ambulatory). L5 (if ambulatory and pelvis level).
Pelvic fixation is mandatory for obliquity.
Infection Prevention
NMS has 5-10x higher infection rate than AIS. Vancomycin Powder in the wound. Betadine Irrigation. Optimize Nutrition. Minimize OR Traffic.
Complications
Complication Profile
| Complication | Rate | Management / Prevention |
|---|---|---|
| Deep Infection (SSI) | 5-15% (High) | Debridement, Antibiotics, Remove hardware if chronically infected. Prevention is key. |
| Pulmonary Failure | Variable | Prolonged intubation common. Pre-op PFTs mandatory. |
| Pseudoarthrosis | 5-10% | Poor bone stock. Use aggressive grafting. |
| Implant Prominence | Common | Thin patients. Use low profile screws. Cut rod ends flush. |
| Superior Mesenteric Artery Syndrome | Rare | Loss of mesenteric fat pad after straightening. Watch for vomiting. |
Postoperative Care and Rehabilitation
Recovery Pathway
Days 1-3ICU
- Ventilator wean (crucial in DMD).
- Pain control (epidural or PCA).
- Fluid balance.
Days 4-7Ward
- Mobilize to wheelchair.
- Resume bowel regimen.
- Check fitting of wheelchair (back support might need modification).
Day 7+Discharge
- Home when feeding tolerated and pain controlled.
Outcomes
Quality of Life:
- Parents report high satisfaction primarily due to ease of nursing care (transfers, bathing) and improved sitting tolerance.
- Correction of pelvic obliquity is the most impactful factor for sitting.
Controversies & Areas of Uncertainty
Quality of Evidence
There are no randomised trials of spinal surgery in NMS (Cochrane, DMD). Practice rests on case series and physiological reasoning, so the magnitude of benefit on survival and quality of life is genuinely uncertain.
Fuse to Pelvis or Stop at L5?
Pelvic fixation reliably corrects obliquity but adds operative time, blood loss and the risk of S2AI/iliac complications. In ambulant CP with a level pelvis, stopping short of the pelvis can preserve motion; in non-ambulant patients with obliquity, the pelvis is included.
Unit Rod vs All-Screw
All-pedicle-screw and S2AI constructs dominate modern practice, but no high-level trial proves superiority over the Unit Rod, which remains a cost-effective, well-validated option (Tsirikos: 68% curve and 71% obliquity correction).
Timing in DMD
Earlier surgery preserves a respiratory window, but glucocorticoids have reduced curve incidence/severity, so fewer boys now reach surgical thresholds and some are managed without fusion. The classic "fuse early, by 20-30 degrees" rule is being re-examined in the steroid era.
Growing Constructs in SMA
Magnetically controlled growing rods reduce repeat lengthening surgeries but carry implant-related and infection risks; intrathecal nusinersen access must be planned around any spinal construct.
BMP and Graft Choice
Osteopenic, low-volume local bone drives interest in BMP and allograft, but BMP is off-label in children and its safety profile in this population is not established.
Evidence Base
GMFCS Level and Scoliosis Risk (Total Population)
Persson-Bunke M, Hägglund G, Lauge-Pedersen H, et al • Spine (Phila Pa 1976) (2012)
Key Findings:
- Prospective total-population CP registry of 666 children aged 4-18 years
- 28% had clinical scoliosis (17% mild, 11% moderate/severe)
- Risk of scoliosis rose with GMFCS level and age
- GMFCS IV-V: ~50% risk of moderate/severe scoliosis by age 18; GMFCS I-II: almost no risk
Clinical Implication: Base scoliosis surveillance on GMFCS level and age; GMFCS IV-V children need close monitoring from a young age.
Limitation: Observational registry cohort; single national surveillance programme (Sweden).
Posterior Fusion Slows Respiratory Decline in DMD
Velasco MV, Colin AA, Zurakowski D, Darras BT, Shapiro F • Spine (Phila Pa 1976) (2007)
Key Findings:
- 56 DMD patients; %FVC tracked before and after posterior spinal fusion
- Whole-cohort rate of FVC decline fell from 4% per year pre-op to 1.75% per year post-op (p less than 0.0001)
- Paired subgroup: decline fell from 8.0% to 3.9% per year
- Fusion did not reverse decline but significantly slowed it
Clinical Implication: Fusion blunts the rate of pulmonary deterioration in DMD; it preserves trajectory rather than restoring lost FVC, supporting earlier intervention.
Limitation: Retrospective; FVC is a surrogate; no randomised control arm.
No RCT Evidence for DMD Scoliosis Surgery (Cochrane)
Cheuk DKL, Wong V, Wraige E, Baxter P, Cole A • Cochrane Database Syst Rev (2013)
Key Findings:
- Systematic review seeking RCTs of spinal surgery for DMD scoliosis
- 47 relevant studies screened; none met inclusion (all case series, no randomised/quasi-randomised trials)
- No evidence-based recommendation could be made for or against surgery
- Benefits on survival, respiratory function and quality of life remain unproven by high-level evidence
Clinical Implication: The case for DMD spinal surgery rests on case-series and physiological reasoning, not RCTs; counsel families about genuine uncertainty of benefit.
Limitation: Absence of trials reflects ethical/practical barriers to randomisation, not proven lack of benefit.
Major Complications After Spinal Fusion in Cerebral Palsy
Yaszay B, Bartley CE, Sponseller PD, et al • Spine Deform (2020)
Key Findings:
- Prospective multicentre database of 257 CP patients with minimum 2-year follow-up
- 78 patients (30%) had at least one major complication; overall 36% complication rate
- Deep infection 4.7% perioperatively plus 3.1% delayed; prolonged ventilation 8.2%
- Spine-related reoperation rate 14%; 11 patients (4.3%) died during follow-up
Clinical Implication: Quantify the high complication burden during consent; pulmonary and wound complications dominate the early course.
Limitation: Observational; complication definitions vary between centres.
Unit Rod Instrumentation for CP Scoliosis
Tsirikos AI, Lipton G, Chang WN, Dabney KW, Miller F • Spine (Phila Pa 1976) (2008)
Key Findings:
- Retrospective series of 287 CP children treated with Unit Rod instrumentation to the pelvis
- Scoliosis corrected from mean 76 to 25 degrees (68%); pelvic obliquity from 17 to 5 degrees (71%)
- Deep wound infection 4.2% early and 2.5% late; three perioperative deaths
- Caregiver satisfaction 96%
Clinical Implication: Segmental pelvic fixation reliably corrects both curve and pelvic obliquity with high caregiver satisfaction; this large series anchors the historical Unit Rod standard.
Limitation: Retrospective single-institution cohort; no direct comparison with all-screw constructs.
Sacral Alar-Iliac (S2AI) Pelvic Fixation
Jain A, Brooks JT, Kebaish KM, Sponseller PD • JBJS Essent Surg Tech (2016)
Key Findings:
- Describes the S2-alar-iliac technique: screw one-third in sacral ala, two-thirds in ilium, in line with cephalad anchors
- Low-profile, in-line entry avoids the offset connectors and prominence of classic iliac screws
- Referenced pediatric series reported ~70% pelvic obliquity and ~67% coronal Cobb correction
- Screws of 9 mm or greater outer diameter recommended to prevent breakage
Clinical Implication: S2AI fixation gives biomechanically robust, less prominent pelvic anchorage and is the modern workhorse for fusion to the pelvis in NMS.
Limitation: Technique paper with a small referenced case series; not a comparative trial.
Surgery in Severe DMD With FVC Under 30%
Takaso M, Nakazawa T, Imura T, et al • Int Orthop (2010)
Key Findings:
- 14 DMD patients with forced vital capacity under 30% predicted
- Pre-operative inspiratory muscle training raised %FVC before surgery
- All-screw constructs corrected coronal curve and pelvic obliquity, maintained long-term
- Mean %FVC decline 3.6% per year after surgery; high patient and parent satisfaction
Clinical Implication: Even very-low-FVC DMD patients can undergo surgery with acceptable risk in expert centres after respiratory optimisation, though this remains a high-risk group requiring careful selection.
Limitation: Small single-centre series; selected patients; no control group.
Clinical Decision Scenarios
Use these scenarios to practise clinical reasoning and management decisions
CLINICAL SCENARIOStandard
CLINICAL PROMPT
"A 12-year-old boy with GMFCS V Cerebral Palsy presents with a 60 degree thoracolumbar neuromuscular scoliosis and pelvic obliquity. He is finding it hard to sit in his wheelchair."
CLINICAL Q&A
Q1:What are the goals of surgery for this patient?
The primary goals are to: 1. Level the pelvis (correct pelvic obliquity) to prevent pressure sores. 2. Restore a straight spine centered over the pelvis to improve sitting balance. 3. Maximize thoracic volume (prevent contact between ribs and pelvis). 4. Ease nursing care.
Q2:What levels would you fuse?
Long fusion is standard. Upper instrumented vertebra (UIV) typically T2 or T3 to prevent proximal kyphosis. Lower instrumented vertebra (LIV) must be into the Pelvis (Iliac/S2AI) to correct and control the pelvic obliquity.
Q3:What pre-operative nutritional marker would you check?
Albumin and Pre-albumin/Transferrin. Albumin less than 3.5 g/dL is a significant risk factor for wound dehiscence and deep infection. Optimising nutrition (often via PEG) is mandatory before surgery.
KEY CLINICAL POINTS
Goals: Sitting Balance, Pelvic Level
Fusion: T2 to Pelvis
Nutrition is critical
COMMON PITFALLS
Stopping fusion at L5 (will fail)
Ignoring nutrition
FURTHER QUESTIONS
"How do you assess pelvic obliquity on X-ray?"
CLINICAL SCENARIOStandard
CLINICAL PROMPT
"A 14-year-old boy with Duchenne Muscular Dystrophy (DMD) has a 25 degree scoliosis. His FVC is 50% predicted."
CLINICAL Q&A
Q1:The curve is only 25 degrees. Do you observe?
No. In DMD, the recommendation is early surgery once the curve is definitely progressive (greater than 20-30 degrees) and while pulmonary function is still permissible (FVC greater than 30-40%). Waiting for the curve to reach 50 degrees usually means the lungs have deteriorated to a point where surgery is too risky.
Q2:What specific cardiac pathology must be checked?
DMD cardiomyopathy. They develop dilated cardiomyopathy. An Echocardiogram and cardiology clearance are strict requirements. Low Ejection Fraction may contraindicate surgery.
Q3:What anesthesia consideration is critical in myopathic patients?
Malignant Hyperthermia is a risk. Depolarizing muscle relaxants (Succinylcholine) are contraindicated as they can trigger massive rhabdomyolysis and hyperkalemia.
KEY CLINICAL POINTS
DMD = Early Surgery
Lung function window
Cardiomyopathy check
COMMON PITFALLS
Treating DMD like AIS (waiting for 50 deg)
Forgetting the heart
FURTHER QUESTIONS
"What anesthesia precautions are needed in myopathic patients?"
CLINICAL SCENARIOAdvanced
CLINICAL PROMPT
"You performed a T2-Pelvis fusion on a CP patient. 3 weeks post-op, the wound is dehiscencing and draining serous fluid."
CLINICAL Q&A
Q1:What is your management steps?
This is a deep infection until proven otherwise. 1. Admit. 2. Bloods (CRP/ESR/WCC). 3. Contrast CT (check for deep collection). 4. Urgent surgical washout (Debridement). 5. Deep cultures. 6. IV antibiotics. VAC dressing may be needed. Do NOT just give oral antibiotics.
Q2:The hardware is exposed. Do you remove it?
Not initially. If the fusion is not solid (it won't be at 3 weeks), removing hardware leads to loss of correction and 'floppy' spine. We attempt to retain hardware with aggressive debridement and VAC therapy. Removal is a last resort for chronic persistent biofilm infection.
Q3:What organism are you worried about in NMS?
Polymicrobial and Gram Negative organisms (Pseudomonas, E. Coli) are much more common in NMS (from bowel/incontinence) compared to Staph Aureus/Cutibacterium in AIS.
KEY CLINICAL POINTS
Aggressive washout
Retain hardware if possible
Gram negative coverage
COMMON PITFALLS
Antibiotics without washout
Premature hardware removal
FURTHER QUESTIONS
"How does nutritional status impact this complication?"
MCQ Practice Points
DMD Threshold
Q: What is the operative threshold for scoliosis in Duchenne Muscular Dystrophy? A: Cobb angle greater than 20-30 degrees (much lower than the 45-50 degrees for AIS).
Fusion Levels
Q: In a non-ambulatory CP patient with pelvic obliquity, where should the fusion stop distally? A: The Pelvis (Iliac/S2AI screws). Stopping at L5 or S1 has a very high failure rate.
Infection Risk
Q: How does the infection rate of NMS surgery compare to AIS? A: It is significantly higher (5-15% vs less than 1%).
Malignant Hyperthermia
Q: Which neuromuscular condition is associated with Malignant Hyperthermia? A: Duchenne Muscular Dystrophy (and Central Core Disease). Succinylcholine is contraindicated.
Spondylolisthesis Association
Q: Is spondylolisthesis common in NMS? A: No, spondylolisthesis is associated with walking (repetitive stress). NMS patients usually have long kyphoscoliotic C-curves.
Cord Tethering
Q: In which NMS condition is cord tethering most common? A: Myelomeningocele (Spina Bifida). Almost all have tethered cords.
Guidelines, Registries & Global Practice
Global Epidemiology:
- Scoliosis affects roughly a quarter of all children with cerebral palsy and the large majority of GMFCS IV-V (non-ambulant) children, with prevalence and severity rising with GMFCS level and age (Persson-Bunke, total-population data).
- Essentially all boys with Duchenne muscular dystrophy who lose ambulation develop scoliosis; historically up to 90% before routine glucocorticoid use. Long-term daily corticosteroids have markedly reduced both the incidence and severity of DMD scoliosis, so fewer patients now reach surgical thresholds.
- Spinal muscular atrophy (SMA) types I-II carry the highest and earliest scoliosis burden of the flaccid neuromuscular disorders.
Side-by-Side Guidance (where recommendations differ):
| Body | Position relevant to NMS |
|---|---|
| DMD Care Considerations (TREAT-NMD / international consensus) | Annual spine surveillance once non-ambulant; daily glucocorticoids reduce scoliosis incidence; consider surgery for progressive curves with attention to cardiac and respiratory status |
| SMA Standards of Care (international consensus) | Spine monitoring from diagnosis; growth-friendly instrumentation for early-onset curves; account for nusinersen intrathecal access when planning fusion |
| AAOS / POSNA (North America) | Emphasise pre-operative optimisation (nutrition, pulmonary, cardiac) and fusion to the pelvis for non-ambulant patients with pelvic obliquity |
| BSCOS / BOA (UK) | Centralised paediatric spinal services; structured cerebral palsy hip and spine surveillance pathways feed early referral |
| Cochrane (DMD) | No randomised evidence; surgery decisions individualised with explicit discussion of uncertainty |
Registry & Database Evidence:
- Multicentre prospective databases (e.g. the Harms Study Group / CP spine cohorts informing Yaszay 2020) provide the best available complication benchmarks: roughly a one-in-three major complication rate and a 14% spine-related reoperation rate after CP fusion.
- Population-based CP surveillance registries (Sweden's CPUP, and similar UK/Australian/North American programmes) drive GMFCS-based screening intervals.
High- vs Limited-Resource Practice Variation:
- High-resource settings: all-pedicle-screw and S2AI constructs, intra-operative neuromonitoring, cell salvage and tranexamic acid, dedicated paediatric ICU, and multidisciplinary pre-optimisation.
- Limited-resource settings: cost-effective segmental constructs (Unit Rod / sublaminar wire systems remain valuable), selective rather than routine neuromonitoring, and greater reliance on careful patient selection where ICU and blood-bank capacity are constrained.
- Referral principle everywhere: structured hip and spine surveillance pathways frequently detect the curve before it becomes symptomatic, enabling timely referral to a spinal service.
Clinical summary
Optimization Checklist
- •Nutrition (Albumin greater than 3.5)
- •Lungs (FVC greater than 30%)
- •Heart (Echo for DMD)
- •Seizures (Controlled)
- •Bowels (Regimen)
Surgical Principles
- •Fuse T2 to Pelvis
- •Correct Pelvic Obliquity
- •Use TXA (High blood loss)
- •Vancomycin powder (Infection)
Condition Specifics
- •CP: GMFCS V most at risk
- •DMD: Fuse early (greater than 20 deg)
- •SMA: Growing rods often needed
- •Myelomeningocele: Latex allergy / Tethering