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 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 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 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 Techniques 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 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 > 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
Technique Guide Technique guides are not considered high yield topics for orthopaedic standardized exams including ABOS, EBOT and RC. Hemivertebra Excision Lindsay Andras David L. Skaggs Spine - Congenital Scoliosis
QUESTIONS 1 of 14 1 2 3 4 5 6 7 8 9 10 11 12 13 14 Previous Next (OBQ20.136) An 18-month-old male is referred to your clinic by his pediatrician for spinal asymmetry that was noticed during his most recent follow-up. Radiographs are obtained during the visit and are depicted in figure A. What is the most appropriate next step in treatment? QID: 215547 FIGURES: A Type & Select Correct Answer 1 Initiate TLSO bracing 2% (17/889) 2 Referral for echocardiogram and renal ultrasound 74% (662/889) 3 Hemivertebrectomy and long thoracolumbar fusion 4% (33/889) 4 VEPTR rods 1% (5/889) 5 Observation with frequent clinic follow-up 19% (165/889) L 2 Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (SBQ13PE.82) A renal ultrasound should be obtained in a patient with which of the following diagnoses? QID: 5235 Type & Select Correct Answer 1 Congenital pseudoarthrosis of the clavicle 6% (279/4908) 2 Accessory navicular 0% (20/4908) 3 Congenital scoliosis 86% (4198/4908) 4 Charcot-Marie-Tooth Disease 7% (340/4908) 5 Congenital curly toe 1% (47/4908) L 2 Question Complexity B Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ12.14) The parents of a 14-month-old boy bring their child into your office. They state the child has reached developmental milestones at appropriate ages, but noticed he was leaning to one side when standing or walking. A radiograph was obtained demonstrating a non-flexible 40-degree curve with multiple vertebral anomalies, highlighted by a convex segmented hemivertebra associated with a concave unilateral bar. After ensuring that the patient has no other associated anomalies in other organ systems, an MRI of the spine revealed no intraspinal abnormalities. What treatment would you recommend to the family? QID: 4374 Type & Select Correct Answer 1 Continued observation with annual follow up 10% (501/5019) 2 Risser Casting 7% (328/5019) 3 VEPTR 4% (212/5019) 4 Instrumentation with growing rods without fusion 10% (494/5019) 5 Excision of the hemivertebra with short segment posterior instrumented fusion 69% (3446/5019) L 3 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK This is an AAOS Self Assessment Exam (SAE) question. Orthobullets was not involved in the editorial process and does not have the ability to alter the question. If you prefer to hide SAE questions, simply turn them off in your Learning Goals. (SAE07PE.72) Figures 31a and 31b show the radiograph and MRI scan of an otherwise normal 3-month-old infant who has a spinal deformity. MRI reveals no intraspinal anomalies. What is the next step in management? QID: 6132 FIGURES: A B Type & Select Correct Answer 1 Posterior spinal fusion with instrumentation 1% (10/910) 2 Anterior-posterior hemiepiphysiodesis 2% (16/910) 3 Brace management 6% (59/910) 4 Cardiac and renal evaluation 77% (701/910) 5 Hemivertebrectomy and fusion 13% (118/910) L 2 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic This is an AAOS Self Assessment Exam (SAE) question. Orthobullets was not involved in the editorial process and does not have the ability to alter the question. If you prefer to hide SAE questions, simply turn them off in your Learning Goals. (SAE07PE.44) A 4-month-old infant is referred for evaluation of congenital scoliosis. The child has no congenital heart anomalies, and a renal ultrasound shows that he has one kidney. Examination reveals mild scoliosis and a large hairy patch on the child’s back. Neurologic evaluation is normal for his age. A clinical photograph and radiograph are shown in Figures 19a and 19b. Initial management should consist of QID: 6104 FIGURES: A B Type & Select Correct Answer 1 referral to a plastic surgeon to remove the hairy patch. 0% (3/902) 2 MRI of the entire spine. 91% (817/902) 3 physical therapy and repeat evaluation and radiographs in 1 year. 4% (33/902) 4 anterior and posterior fusion of the anomalous regions of the spine to prevent deformity. 1% (11/902) 5 voiding cystourethrography. 4% (34/902) L 1 Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic (OBQ07.220) Figure A demonstates different anatomic patterns in congenital scoliosis. Which pattern has the worst prognosis and is an indication for surgery. QID: 881 FIGURES: A Type & Select Correct Answer 1 Illustration 1 7% (247/3692) 2 Illustration 2 1% (39/3692) 3 Illustration 3 1% (41/3692) 4 Illustration 4 3% (118/3692) 5 Illustration 5 87% (3223/3692) L 1 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic (OBQ05.116) A 2-year-old girl presents to the office for evaluation of spinal deformity. A radiograph is shown in Figure A. What additional work-up should be done? QID: 1002 FIGURES: A Type & Select Correct Answer 1 HLA-B27 1% (32/3281) 2 Echocardiography 90% (2953/3281) 3 Bone scan with SPECT images 2% (54/3281) 4 Skeletal survey 5% (170/3281) 5 Electromyogram 2% (53/3281) L 1 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic (SBQ04PE.21) An 8-month-old male presents for evaluation of congenital kyphosis. All of the following support early in situ posterior fusion EXCEPT? QID: 2206 Type & Select Correct Answer 1 50-degree curve 21% (389/1897) 2 Failure of formation 10% (190/1897) 3 Bar formation 13% (241/1897) 4 Neurological weakness 12% (231/1897) 5 Block vertebrae 44% (836/1897) L 5 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK
All Videos (3) Podcasts (1) Login to View Community Videos Login to View Community Videos 12th International Congress on Early Onset Scoliosis - 2018 A hybrid technique of posterior osteotomy with short segmental fusion and dual growing rod technique for severe rigid congenital scoliosis - Jianguo Zhang, MD (ICEOS 2018, #15) Spine - Congenital Scoliosis B 2/13/2019 246 views 2.5 (2) Login to View Community Videos Login to View Community Videos 12th International Congress on Early Onset Scoliosis - 2018 Paper #33 Improvement of Functional Outcome Using 6-minute walk in Patients with Congenital Scoliosis Treated by Growth Friendly Surgery; Five Years Follow-up Study - Noriaki Kawakami, MD - (ICEOS 2018, #93) Spine - Congenital Scoliosis D 2/10/2019 35 views 3.0 (1) Login to View Community Videos Login to View Community Videos 12th International Congress on Early Onset Scoliosis - 2018 Paper #51 Congenital Scoliosis of the Pediatric Cervical Spine - Amer Samdani, MD (ICEOS 2018, #125) Spine - Congenital Scoliosis A 2/10/2019 89 views 4.3 (3) Spine ⎜ Congenital Scoliosis Spine - Congenital Scoliosis Listen Now 18:6 min 10/18/2019 635 plays 4.5 (2)
13th International Congress on Early Onset Scoliosis - 2019 Spinal Deformity, Back Pain, Clonus in 5F (C101324) John T. Smith Spine - Congenital Scoliosis E 11/1/2019 3019 16 7 Congenital scoliosis and tethered cord (C2121) Eric Shirley Spine - Congenital Scoliosis E 1/31/2015 879 1 6