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Average 4.1 of 33 Ratings
Technique guide are not considered high yield topics for orthopaedic standardized exams including the ABOS, EBOT and RC.
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?
Continued observation with annual follow up
Instrumentation with growing rods without fusion
Excision of the hemivertebra with short segment posterior instrumented fusion
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The clinical presentation is consistent with congenital scoliosis with a convex segmented hemivertebra (failure of formation) associated with a concave unilateral bar (failure of separation). The most appropriate treatment is excision of the hemivertebra with short segment posterior instrumented fusion.
In congenital scoliosis the risk of progression is determined by the morphology of vertebrae. The worst prognosis comes when there is a unilateral unsegmented bar with contralateral hemivertebra. This morphology universally progresses, and therefore surgical fusion is required regardless of age and deformity.
Akbarnia et al. reviews treatment options for very young children with spinal deformities. He states that a hemivertebrectomy is an effective treatment for congenital scoliosis, and that it is most commonly done through a combined anterior and posterior approach. While a posterior only approach is possible, the author cautions that one must watch closely for deformity progression and the development of the crankshaft phenomenon.
Holte et al. retrospectively reviewed the results of anterior and posterior hemivertebra excision or wedge resection plus arthrodesis in 37 patients with congenital scoliosis. The average curved decreased from 54 degrees preoperatively to 33 degrees post operatively. Minimal progression of the curve was detected over 12 years of follow up.
King et al. reported on nine patients with progressive congenital scoliosis, including one patient who was only 12 months old. All patients underwent a posterior only, transpedicular anterior hemiepiphysiodesis of at least one hemivertebra and posterior fusion. They reported that curve progression was stopped in all cases, and four of the nine patients had a correction in the curve of 10 degrees or more.
Illustration A shows classification of the types of congenital scoliosis. Illustration B shows a radiograph of a hemivertebrae with a contralateral unsegmented bar.
Answer 1--Continued observation with annual follow up. This answer is wrong, because the patient has vertebral anomalies that will progress without intervention.
Answer 2--Risser Casting. This answer is incorrect because while Risser casting is used to treat young children with scoliosis, it is unlikely to be effective in patients with a convex segmented hemivertebra (failure of formation) associated with a concave unilateral bar (failure of separation)
Answer 3--VEPTR--The primary indication for VEPTR insertion is patients with scoliosis and Thoracic Insufficiency Syndrome--a disorder defined by the Scoliosis Research Society as "the inability of the thorax to support normal respiration or lung growth." While VEPTR may be indicated for other patients with congenital scoliosis, answer 5 avoids the morbidity associated with frequent lengthening and results in less fusion levels.
Answer 4--Instrumentation with growing rods without fusion--Similar to the VEPTR, the growing rod may be used in patients with progressive congenital scoliosis. Again, answer 5 avoids the morbidity associated with frequent lengthening and results in less fusion levels.
Akbarnia BA, Blakemore LC, Campbell RM Jr, Dormans JP.
Instr Course Lect. 2010;59:407-24. PMID: 20415395 (Link to Abstract)
Holte DC, Winter RB, Lonstein JE, Denis F.
J Bone Joint Surg Am. 1995 Feb;77(2):159-71. PMID: 7844121 (Link to Abstract)
Holte, JBJS 1995
King AG, MacEwen GD, Bose WJ.
Spine (Phila Pa 1976). 1992 Aug;17(8 Suppl):S291-4. PMID: 1523515 (Link to Abstract)
King, SPINE 1992
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Average 3.0 of 20 Ratings
Figure A demonstates different anatomic patterns in congenital scoliosis. Which pattern has the worst prognosis and is an indication for surgery.
Congenital scoliosis is caused by anatomic anomalies of the vertebral bodies. The different type of anomalies can be divided into failure of formation (hemivertebrae, wedge vertebrae, butterfly vertebrae) and failure of segmentation (block vertebrae, bar body). The worst situation is when there is failure of formation with contralateral failure of segmentation. An example of this is a hemivertebrae with a contralateral bar body as depicted in Illustration 5. This has the worst prognosis and is an indication for surgery.
Hedequist D, Emans J
J Am Acad Orthop Surg. 2004 Jul-Aug;12(4):266-75. PMID: 15473678 (Link to Abstract)
Hedequist, JAAOS 2004
Average 4.0 of 19 Ratings
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?
Bone scan with SPECT images
The radiograph demonstrates congenital scoliosis with defects in formation (resulting in the hemivertebrae) and segmentation. Basu et al assessed the incidence of intraspinal anomalies and other organic defects associated with different types of congenital spinal deformity using MRI, echocardiography, renal ultrasound, and a thorough clinical assessment. Intraspinal abnormalities were found in 37% of patients - more commonly in those with congenital kyphosis and scoliosis resulting from mixed and segmentation defects. Cardiac defects were detected in 26% and urogenital anomalies in 21% of patients. Based on their finding, they argue magnetic resonance imaging and echocardiography should be an essential part in the evaluation of patients with congenital spinal deformity. Renal ultrasound should be considered, although a complete spine MRI may detect renal pathology as well.
Basu PS, Elsebaie H, Noordeen MH
Spine. 2002 Oct;27(20):2255-9. PMID: 12394903 (Link to Abstract)
Basu, SPINE 2002
Average 3.0 of 27 Ratings
HPI - 18 month old girl with scoliosis
The curve measures 63 degrees and has been stable for 6 months. How would you treat this at this time?