summary 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. Epidemiology Prevalence estimated at 1% to 4% in the general population 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 login to view 2 more bullets Goldenhar/OculoAuricularVertebral Syndrome login to view 1 more bullet Jarcho-Levin Syndrome/Spondylocostal dysostosis login to view 5 more bullets Klippel-Feil syndrome login to view 1 more bullet Alagille syndrome login to view 1 more bullet Classification Classification of Congenital Scoliosis 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 login to view 5 more bullets 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 login to view 4 more bullets bracing login to view 2 more bullets Operative posterior fusion (+/- osteotomies and modest correction) indications login to view 3 more bullets anterior/posterior spinal fusion +/- vertebrectomy indications login to view 4 more bullets technique login to view 1 more bullet distraction based growing rod construct indications login to view 1 more bullet outcomes login to view 1 more bullet osteotomies between ribs indications login to view 1 more bullet outcomes login to view 1 more bullet 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 Techniques Spinal arthrodesis +/- vertebrectomy/osteotomy in situ arthrodesis, anterior/posterior or posterior alone indications login to view 1 more bullet hemiepiphysiodesis indications login to view 3 more bullets osteotomy osteotomy of bar hemivertebrectomy hemivertebrae with progressive curve causing truncal imbalance and oblique takeoff login to view 1 more bullet patients < 6 yrs. and flexible curve < 40 degrees best candidates spinal column shortening resection indications login to view 3 more bullets Complications 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 > login to view 1 more bullet unilateral unsegmented bar > fully segmented hemivertebra > unincarcerated hemivertebra > incarcerated hemivertebra > unsegmented hemivertebra > block vertebrae login to view 1 more bullet presence of fused ribs increases risk of progression