Control the deformity while preserving spinal, thoracic and pulmonary growth
- Early definitive fusion in a young child stunts thoracic height and lung development β thoracic insufficiency syndrome (Campbell): the inability of the thorax to support normal respiration or lung growth
- Serial Mehta (elongation-derotation-flexion) casting can be curative in progressive infantile idiopathic scoliosis if started young β RVAD greater than 20 degrees and phase 2 rib overlap predict progression (Mehta)
- Law of diminishing returns (Sankar): each successive TGR lengthening yields progressively less length as the spine stiffens and autofuses
- VEPTR was designed by Campbell for thoracic insufficiency syndrome β fused ribs and constricted hemithorax (Jeune, Jarcho-Levin, congenital rib fusion with scoliosis) β via opening-wedge expansion thoracostomy
- Aetiology drives strategy: congenital, neuromuscular, syndromic and idiopathic infantile EOS behave differently and fail differently
- βMCGR avoids repeated anaesthetics but does not avoid revisions β metallosis around the actuator, failure to lengthen and actuator pin fracture are recognised
- βGraduation is a decision, not an event: definitive fusion, in-situ retention of the construct, or removal β each has advocates and trade-offs
- βOutcome measures the examiner wants: T1-T12 and T1-S1 height gain, space-available-for-lung ratio, EOSQ-24 quality of life, complications per patient
Definitive fusion before age 8 to 10 arrests T1-T12 growth, producing a short, stiff thorax and restrictive lung disease. Karol showed fusion of 4 or more thoracic levels before age 8 correlates with reduced forced vital capacity in adulthood. The entire growth-friendly strategy exists to avoid this.
A progressive infantile idiopathic curve (RVAD greater than 20 degrees, phase 2 rib) in a child under 2 years should have serial derotational casting before any implant β it is the only potentially curative growth-friendly treatment.
Across all systems, unplanned returns to theatre are the rule: anchor pull-out, rod fracture, deep infection, proximal junctional kyphosis, wound breakdown in thin children. Bess reported complication risk rises with each additional procedure β consent families honestly at the outset.
Rib cradles placed too high, or excessive distraction elevating the first rib, can cause brachial plexus traction injury. Neuromonitoring during implantation and lengthening, and anchor placement at the second rib or below, mitigate this.
Rationale: Why Growth Matters More Than Correction
The child with early-onset scoliosis has two coupled problems: a progressive spinal deformity and a growing respiratory system that depends on a growing thorax.
- Spinal growth: T1-S1 gains roughly 2 cm per year from birth to age 5, slows to about 1 cm per year from 5 to 10, then accelerates again in the adolescent spurt (Dimeglio). Around half of final sitting height is achieved by age 5.
- Thoracic growth: the thorax reaches about 30 percent of adult volume by age 5 and about 50 percent by age 10. An adult T1-T12 height of approximately 18 to 22 cm is regarded as the threshold for adequate lung volume; a fused short thorax cannot reach it.
- Lung growth: alveolar multiplication is most rapid in the first 2 years and largely complete by around age 8; thereafter lungs enlarge mainly by alveolar hypertrophy. Deformity control before age 8 therefore protects irreplaceable alveolar development β this is the biological deadline that frames every decision.
- Thoracic insufficiency syndrome (Campbell): the inability of the thorax to support normal respiration or lung growth β seen with fused ribs, constricted hemithorax, severe early scoliosis, and hypoplastic thorax syndromes (Jeune asphyxiating thoracic dystrophy, Jarcho-Levin spondylothoracic dysostosis). The windswept, rotated thorax reduces the space available for lung on the concavity.
Space-available-for-lung (SAL) ratio: on an upright PA radiograph, measure the height of the concave hemithorax from the apex of the most cephalad rib to the centre of the ipsilateral hemidiaphragm, and divide by the same measurement on the convex side. A ratio below approximately 0.8 to 0.9 indicates significant concave hemithorax constriction and supports thorax-based (VEPTR) treatment.
Untreated progressive EOS carries excess cardiorespiratory mortality in adulthood (Pehrsson's Swedish natural history series), which is why observation of a clearly progressive curve is not an option β but neither is early fusion. Hence: control the curve while the spine and thorax keep growing.
Aetiology Groups and How They Change Strategy
- Typical behaviour
- Onset under 3 years; many resolve (RVAD less than 20 degrees, phase 1 rib); progressive curves respond well to casting
- Strategic implications
- Serial Mehta casting first β potentially curative; implants reserved for casting failures or late presentation
- Typical behaviour
- Failure of formation or segmentation; unilateral unsegmented bar with contralateral hemivertebra is the most malignant pattern; often rib fusions
- Strategic implications
- Casting largely ineffective for structural anomalies; hemivertebra excision for isolated anomalies; VEPTR where fused ribs constrict the hemithorax; see the congenital scoliosis topic for anomaly-specific surgery
- Typical behaviour
- Long sweeping collapsing curves, pelvic obliquity, poor bone, poor soft tissue envelope, high infection risk
- Strategic implications
- Distraction constructs frequently extended to pelvis (rib-to-pelvis VEPTR or growing rods with iliac/S2AI anchors); higher wound and infection burden; unit rod definitive constructs at maturity β see the neuromuscular scoliosis topic
- Typical behaviour
- Marfan, neurofibromatosis, skeletal dysplasias, VACTERL; dystrophic curves progress rapidly; anaesthetic and airway comorbidity
- Strategic implications
- Multidisciplinary work-up mandatory; MCGR attractive to reduce anaesthetic exposure; anchor fixation compromised by dysplastic bone (NF1 dural ectasia, rib penciling)
All EOS patients need whole-spine MRI before instrumentation: neural axis anomalies (syrinx, Chiari, tethered cord, diastematomyelia) occur in roughly one in five infantile idiopathic curves and more frequently in congenital scoliosis.
Non-Operative Growth-Friendly Treatment First
Elongation-derotation-flexion (EDF) casting under general anaesthesia on a specialised frame (Risser or Mehta table) with longitudinal traction, a derotational moulding force applied over the rib prominence (not directly on the apex, to avoid rib deformation), and windows cut anteriorly for chest expansion and posteriorly over the concavity to allow the thorax to derotate into the space.
- Who: progressive infantile idiopathic scoliosis, ideally started before age 2 with curves under approximately 60 degrees β this population can achieve full resolution (Mehta's series showed curve resolution in the majority treated before 2 years).
- Predictors of progression (Mehta, 1972): rib-vertebra angle difference (RVAD) greater than 20 degrees, and phase 2 rib (convex rib head overlapping the apical vertebral body on the PA film). Draw the RVAD: on the apical vertebra, the angle between a perpendicular to the endplate and a line along each rib neck; convex angle subtracted from concave angle.
- Technique points: casts changed every 8 to 12 weeks (more frequently in the youngest); each change is an anaesthetic β balance against progression risk; transition to bracing once correction is held.
- In non-idiopathic EOS: casting rarely cures but is a legitimate delay tactic β buying spinal height and postponing the first implant (each year gained matters).
Operative Systems: The Exam Comparison
- Principle and anchors
- Dual rods; proximal (pedicle screws or hooks, T2-T4) and distal foundations spanning the curve without fusing intervening levels; tandem connectors; surgical lengthening every 6 months
- Advantages
- Powerful correction; long track record; dual rods superior to single (Thompson)
- Key problems
- Repeated anaesthetics and incisions; law of diminishing returns; rod fracture, anchor pull-out, infection, PJK
- Principle and anchors
- Same foundations as TGR but rod contains a magnetically driven actuator; outpatient external remote-controller lengthenings, typically every 1 to 3 months in small increments
- Advantages
- Avoids repeated surgical lengthenings and anaesthetics; more frequent, more physiological distraction; family burden reduced
- Key problems
- Metallosis and tissue titanium debris around actuator (Teoh); failure to lengthen or rod slippage; actuator pin fracture; MRI compatibility caveats; regulatory scrutiny episodes; still needs planned exchanges when actuator stroke is exhausted, plus unplanned revisions
- Principle and anchors
- Vertical expandable prosthetic titanium rib (Campbell); rib-to-rib, rib-to-spine or rib-to-pelvis cradles; opening-wedge expansion thoracostomy for fused ribs; surgical expansions every 6 months
- Advantages
- Only device designed for thoracic insufficiency syndrome; treats the thorax directly; spares the spine of anchors in rib-based constructs
- Key problems
- Rib anchor migration and rib erosion; brachial plexus risk with cephalad anchors; wound breakdown over prominent implants in thin children; modest scoliosis correction compared with spine-based systems
- Principle and anchors
- Limited apical fusion with corrective apical screws; proximal and distal non-locked sliding screws guide growth along the rods
- Advantages
- No scheduled lengthenings β fewer planned operations; apex controlled directly
- Key problems
- Metal-on-metal wear debris at sliding interfaces; unpredictable guidance and add-on deformity; rod exchange when growth outruns rod length
Sankar and colleagues (Spine, 2011) showed that with traditional growing rods, the T1-S1 length gained per lengthening falls progressively with each successive procedure β attributed to progressive spinal stiffening and spontaneous autofusion under a distracted, immobilised construct. Practical consequences: obtain the best correction at index surgery, do not delay the index procedure expecting to "catch up" later, and temper expectations of late lengthenings.
Operative Technique
Indication: progressive EOS (typically greater than 50 to 60 degrees) with substantial growth remaining, failed or unsuitable for casting, without fused ribs demanding thorax-based treatment. Contraindication: active infection, insufficient bone stock for foundations, kyphosis beyond actuator tolerance (MCGR), very small stature precluding actuator (MCGR). Why this over alternatives: spine-based distraction gives more powerful curve correction than VEPTR; MCGR over TGR when reducing anaesthetic exposure is a priority.
- Position: prone on a radiolucent table, chest and pelvic bolsters, abdomen free, head in a padded holder or Mayfield in the very small; all pressure points padded.
- Imaging/equipment: fluoroscopy; multimodal neuromonitoring (SSEP and MEP) mandatory; cell salvage in small children; MCGR external remote controller checked and rods templated for actuator length.
- Preparation: prophylactic antibiotics; tranexamic acid per protocol; whole-spine MRI reviewed preoperatively.
- Approach: two limited midline (or paramedian muscle-splitting) incisions over the planned proximal and distal foundations only β do not expose the intervening spine, to limit autofusion.
- Dissection: subperiosteal exposure confined to foundation levels; proximal foundation typically T2-T4 with 4 anchor points (pedicle screws, or hooks/rib anchors in poor bone β hooks or rib-based proximal anchors may reduce PJK by preserving the posterior tension band); distal foundation 2 levels of pedicle screws below the end vertebra; iliac or S2-alar-iliac screws for pelvic obliquity.
- Reduction/Reconstruction: rods contoured to sagittal profile (limit contour near the MCGR actuator β it must remain straight over the actuator segment), tunnelled submuscularly (submuscular passage has lower infection and rod fracture rates than subcutaneous), connected via tandem connectors (TGR) or as single actuator rods (MCGR); gentle distraction with monitoring stable.
- At-risk structures: spinal cord (monitor throughout, especially during distraction), pleura at proximal thoracic anchors, great vessels with mal-directed screws, skin envelope in thin children.
- Closure: layered, watertight; local antibiotic powder used by many units.
- Aftercare: TGR β surgical lengthening every 6 months, day-case where feasible; MCGR β outpatient magnetic lengthenings every 1 to 3 months, small increments, distraction confirmed by ultrasound or radiograph; brace rarely needed with dual rods.
- Pitfalls: exposing the mid-spine (accelerates autofusion); over-distraction causing PJK or neurological signal change β distract to soft endpoint, never force; MCGR actuator placed where kyphosis contour is needed.
- Salvage of failure: rod fracture β exchange; anchor pull-out β augment or extend foundation; deep infection β debridement with implant retention early, staged exchange if chronic; construct exhausted β proceed to graduation strategy.
Complications Across All Systems
The rule, not the exception. Bess (JBJS Am, 2010, Growing Spine Study Group) reported complications in the majority of growing-rod patients, with risk increasing with each additional procedure and with subcutaneous rod placement. Consent at the outset must frame this as a multi-year surgical programme, not a single operation.
Distraction forces concentrate at the proximal foundation. Prevention: preserve the posterior tension band and interspinous ligaments above the construct, avoid over-distraction, consider hooks or rib anchors proximally, restore sagittal contour. Management: extend proximally, restore kyphosis correction, address pull-out.
Higher in neuromuscular patients and with each reopening. Early deep infection: debride, retain implants, targeted antibiotics. Chronic/recurrent: staged removal and exchange β sometimes forcing early graduation. Thin children with VEPTR need proactive soft tissue planning.
Prolonged distraction and immobilisation cause spontaneous fusion, limiting late lengthening and final correction. Historically, crankshaft phenomenon (continued anterior growth rotating around a posterior-only fusion) complicated isolated posterior fusion in the very young β one more reason early definitive fusion fails.
Implant-specific additions: TGR β rod fracture (commonest at tandem connectors), anchor pull-out. MCGR β metallosis and elevated tissue titanium around the actuator, failure to lengthen, actuator pin fracture, MRI compatibility caveats (image quality artefact and rod behaviour concerns), and episodes of regulatory scrutiny including temporary market suspension in some jurisdictions. VEPTR β rib cradle migration, rib erosion, brachial plexus traction. Shilla β sliding-interface metal debris, adding-on deformity.
Distraction is the moment of neurological risk. Multimodal monitoring (MEP and SSEP) during index surgery and surgical lengthenings; if signals deteriorate, release distraction immediately, restore blood pressure, and re-check before proceeding. MCGR outpatient lengthenings are unmonitored β hence small, frequent increments.
Outcome Measures
- T1-T12 height: proxy for thoracic and lung volume growth; target trajectory towards 18 to 22 cm at maturity; normal growth about 1.4 cm per year in this age range (Dimeglio) β compare achieved gain against this benchmark.
- T1-S1 height: total spinal growth achieved by the programme.
- Cobb angle: control, not necessarily cure; expect the best correction at index surgery.
- Space-available-for-lung ratio: thorax-specific measure, particularly for VEPTR.
- EOSQ-24: validated parent-reported quality-of-life instrument for EOS β increasingly the headline outcome, since radiographs do not capture the burden of repeated surgery.
- Complications per patient and unplanned returns to theatre: report honestly.
RVAD 20 / Phase 2Predictors of infantile curve progression
Hook:Twenty and two: RVAD over twenty or a phase two rib β treat, do not observe.
CASTGrowth-friendly strategy choice
Hook:CAST β because casting really is the first move.
Lengthening Programme and Surveillance
Best correction is obtained at implantation β the law of diminishing returns means later lengthenings add less. Document T1-T12, T1-S1, Cobb, SAL and EOSQ-24 baseline.
Day-case where possible; distract to soft endpoint under neuromonitoring; each return to theatre adds infection and anaesthetic risk.
External remote controller; confirm achieved distraction with ultrasound (avoids radiation) or radiograph; investigate repeated failure to lengthen (rod slippage, actuator failure, autofusion).
MCGR actuator stroke is finite (approximately 28 to 48 mm depending on rod); exchange when consumed, inspect for metallosis.
Definitive fusion, retention in situ, or removal β see below.
Graduation: What To Do at the End of the Programme
When the child approaches skeletal maturity (or the construct is exhausted and the spine autofused), three options exist:
- Definitive posterior fusion: traditional default β formal exposure, implant exchange to a definitive construct, correction of residual deformity. Reality check: the autofused, stiff, previously distracted spine yields limited additional correction, and complication rates at final fusion are meaningful (Flynn's Growing Spine Study Group data).
- Retention in situ ("graduate without fusion"): leave the mature construct undisturbed if alignment is acceptable and the spine is effectively autofused β avoids a large final operation; risk of late rod fracture and slow curve creep requires surveillance.
- Implant removal without fusion: occasionally chosen for infection or prominence; risk of deformity progression after removal even in apparently stiff spines β least favoured for structural curves.
The decision weighs residual deformity, sagittal balance, autofusion extent, implant integrity, infection history and family priorities. There is no consensus β this is a legitimate "discuss the options" viva answer.
Guidelines, Registries & Global Practice
- Global epidemiology: EOS is uncommon relative to adolescent idiopathic scoliosis but disproportionately morbid; congenital and syndromic causes dominate surgical caseloads worldwide, while presentation is later and curves larger in resource-limited settings where casting infrastructure and device access are constrained.
- Society guidance: the Scoliosis Research Society Growing Spine Committee consensus defines EOS as onset before age 10 and endorses growth-friendly strategies over early fusion; the SRS-endorsed EOS classification (Williams β aetiology, Cobb, kyphosis, progression modifier) standardises communication. POSNA and BSCOS practice statements support serial casting as first-line for progressive infantile idiopathic curves. AAOS and NICE have no dedicated EOS implant guidance; NICE and MHRA involvement has centred on MCGR device safety review, including a period of restricted use pending investigation of metallosis and actuator concerns β candidates should know MCGR has faced genuine regulatory scrutiny.
- Registry and study-group evidence: national arthroplasty-style registries do not capture EOS; the field's registry equivalents are the Growing Spine Study Group and the Children's Spine Study Group / Paediatric Spine Study Group, whose pooled multicentre data underpin most complication and outcome figures quoted above.
- Practice variation by resource setting: MCGR cost and controller availability limit use in many health systems; TGR and Shilla (no scheduled lengthenings) remain workhorses where repeated theatre access or magnetic controllers are unavailable; casting programmes are highly cost-effective and exportable, and several global outreach programmes prioritise establishing EDF casting before implant services.
Controversies & Areas of Uncertainty
- MCGR versus TGR overall benefit: MCGR clearly reduces anaesthetic episodes, but total revision burden, metallosis significance, and cost-effectiveness remain debated; randomised data are sparse.
- Graduation strategy: definitive fusion versus retention in situ versus removal β no consensus; observational data suggest many "graduates" do acceptably without formal final fusion, but selection bias is heavy.
- Optimal proximal anchor: screws versus hooks versus rib anchors for PJK prevention β biomechanically argued, clinically unresolved.
- Distraction frequency: more frequent small MCGR distractions are argued to be more physiological; the ideal interval is unknown.
- How much correction matters: whether aggressive Cobb correction improves pulmonary outcomes beyond height and SAL gains is unproven β thoracic growth, not radiographic cosmesis, is the goal.