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Conjenital scoliosis

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  • Congenital Scoliosis is a congenital spinal deformity that occurs due to the failure of normal vertebral development during 4th to 6th week of gestation.

  • Diagnosis is made with AP and lateral full spine radiographs. MRI is required to assess for neural axis abnormalities. 

  • Treatment can be observation or surgical management depending on the specific anatomical anomaly, and curve progression.

Etiology

  • Mechanism

    • caused by a developmental defect in the formation of the mesenchymal anlage

  • Causes

    • most cases occur spontaneously 

       

    • maternal exposures

      • diabetes

      • alcohol

      • valproic acid

      • hyperthermia

  • Genetic

    • uncertain

  • Associated conditions 

     

    • may occur in isolation or with associated conditions

    • with associated systemic anomalies, up to 61%

      • cardiac defects - 10%

      • genitourinary defects - 25%

      • spinal cord malformations

    • with underlying syndrome or chromosomal abnormality

      • VACTERL syndrome

        • in 38% to 55%

        • characterized by vertebral malformations, anal atresia, cardiac malformations, tracheo-esophageal fistula, renal, and radial anomalies, and limb defects

      • Goldenhar/OculoAuricularVertebral Syndrome

        • hemifacial microsomia and epibulbar dermoids

      • Jarcho-Levin Syndrome/Spondylocostal dysostosis

        • short trunk dwarfism, multiple vertebral and rib defects and fusion

        • most commonly autosomal recessive

        • often associated with thoracic insufficiency syndrome (TIS)

          • caused by shortening of the thorax and rib fusions

          • result is thorax is unable to support lung growth and respiratory decompensation

      • Klippel-Feil syndrome

        • short neck, low posterior hairline, and fusion of cervical vertebrae

      • Alagille syndrome

        • peripheral pulmonic stenosis, cholestasis, facial dysmorphism

classification

      • Failure of Formation 

      • Fully segmented hemivertebra

      • -has normal disc space above and below

      •  
      •  
      • Semisegmented hemivertebra

      • -hemivertebra fused to adjacent vertebra on one side with disk on the other

      •  
      •  
      • Unsegmented hemivertebra

      • -hemivertebra fused to vertebra on each side

      •  
      •  
      • Incarcerated hemivertebra

      • -found within lateral margins of the vertebra above and below

      •  
      •  
      • Unincarcerated hemivertebra

      • -laterally positioned

      •  
      •  
      • Wedge vertebra

      •  
      • Failure of Segmentation

      • Block vertebra

      • (bilateral bony bars)

      •  
      •  
      • Bar body

      • (unilateral unsegmented bar is common and likely to progress)

      •  
      • Mixed 

      •  
      • Unilateral unsegmented bar with contralateral hemivertebra

      • (most rapid progression)

      •  

Imaging

  • Radiographs

    • recommended views

      • AP and lateral plain films usually sufficient to confirm diagnosis

  • CT

    • indications

      • judicious use recommended due to radiation exposure

      • 3D CT useful to better delineate posterior bony anatomy and define type for surgical planning

  • MRI

    • indications

      • all patients with congenital scoliosis prior to surgery to evaluate for neural axis abnormality (found in 20-40%) including

        • Chiari malformation

        • tethered cord

        • syringomyelia

        • diastematomyelia

        • intradural lipoma

    • technique

      • sedation required in infants so may be delayed if no surgery is planned and no neuro deficits

  • Additional medical studies

    • important to obtain studies for associated abnormalities 

       

      • renal ultrasound or MRI

      • echocardiogram if suspicion for cardiac manifestations

Treatment

  • Nonoperative

    • observation and bracing

      • indications for observation

        • absence of documented progression, ie:

          • incarcerated hemivertebrae

          • nonsegmental hemivertebrae

          • some partially segmented hemivertebrae

      • bracing

        • not indicated in primary treatment of congenital scoliosis (no effectiveness shown)

        • may be used to control supple compensatory curves, but effectiveness is unproven

  • Operative

    • posterior fusion (+/- osteotomies and modest correction)

      • indications

        • hemi-vertebrae opposite a unlateral bar that does not require a vertebrectomy at any age. this otherwise will relentlessly progress until fused.

        • older patients with significant progression, neurologic deficits, or declining respiratory function

        • having many pedicle screws may decrease crankshaft phenomenon adn obviate the need for an anterior fusion.

    • anterior/posterior spinal fusion +/- vertebrectomy

      • indications

        • young patients with significant progression, neurologic deficits, or declining respiratory function

          • girls < 10 yrs

          • boys < 12 yrs

        • patients with failure of formation with contralateral failure of segmentation at any age that requires hemi-vertebrectomy and/or significant correction. This may be done from a posterior approach 

           

      • technique

        • nutritional status of patient must be optimized prior to surgery

    • distraction based growing rod construct

      • indications

        • may be used in an attempt to control deformity during spinal growth and delay arthrodesis

      • outcomes

        • need to be lengthened approximately every 6 months for best results

    • osteotomies between ribs 

      • indications

        • mulitple (>4) fused ribs wit potential for thoracic insufficiency syndrome

      • outcomes

        • long-term follow up is needed to determine efficacy. the downside is this may make the chest stiff and hurt pulmonary function.

    • Hemi-Vertebrectomy - usally done from a posterior approach, particularly with kyphosis.

      • indications - age 3-8 years (younger is difficult to get good anchor purchase)

      • progressive or significant deformity 

Teqniques

  • Spinal arthrodesis +/- vertebrectomy/osteotomy

    • in situ arthrodesis, anterior/posterior or posterior alone

      • indications

        • unilateral unsegmented bars with minimal deformity

    • hemiepiphysiodesis

      • indications

        • intact growth plates on the concave side of the deformity

        • patients less than 5 yrs. with < 40-50 degree curve

        • mixed results

    • osteotomy

      • osteotomy of bar

    • hemivertebrectomy

      • hemivertebrae with progressive curve causing truncal imbalance and oblique takeoff

        • often caused by a lumbosacral hemivertebrae

      • patients < 6 yrs. and flexible curve < 40 degrees best candidates

    • spinal column shortening resection

      • indications

        • deformities that present late and have severe decompensation

        • rigid, severe deformities

        • pelvic obliquity, fixed

Complication

  • Crankshaft phenomenon

    • a deformity caused by performing posterior fusion alone

  • Short stature

    • growth of spinal column is affected by fusion

      • younger patients affected more

  • Neurologic injury

    • surgical risk factors include

      • overdistraction or shortening

      • overcorrection

      • harvesting of segmental vessels

    • somatosensory and motor evoked potentials important

  • Soft-tissue compromise

    • nutritional aspects of care essential to ensure adequate soft tissue healing

Prognosis

  • Dependent on potential for progression and early intervention

  • Progression 

      

    • most rapid in the first 3 years of life

    • anterior failure of formation is rapidly progressive and often results in paralysis; anterior failure of segmentation can be rapidly progressive but rarely results in paralysis

    • determined by the morphology of vertebrae. Rate of progression from greatest to least is:

      • unilateral unsegmented bar with contralateral hemivertebra >

        • greatest potential for rapid progression (5 to10 degrees/year)

      • unilateral unsegmented bar >

      • fully segmented hemivertebra >

      • unincarcerated hemivertebra >

      • incarcerated hemivertebra >

      • unsegmented hemivertebra >

      • block vertebrae

        • little chance for progression (<2 degrees/year)

    • presence of fused ribs increases risk of progression

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