Scoliosis is an abnormal side-away curvature of the spine and it is defined as a 3-D deformity of the spine measured on radiographs as lateral(side-way) curvature of the spine >10°. Scoliosis occurs in about 2-3% of the population and girls are more commonly affected than boys, especially adolescent idiopathic scoliosis, which as the name suggests is seen more during 11-18 years of age.
Scoliosis deformity can progress as the child grows, hence 6 monthly or yearly follow-up with the spine surgeon is required to monitor the progression.
Scoliosis Causes
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Idiopathic Scoliosis: most common type, when exact cause unknown, seen most commonly in the age group 11-16.
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Congenital: a birth defect in the spine or spinal cord anomalies
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Neuromuscular: In patients with underlying neurological conditions (eg, cerebral palsy or paralysis) or muscular abnormalities (e.g. Duchenne Muscular Dystrophy)
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Syndromic: Marfan syndrome, osteogenesis imperfecta etc
Evaluation:
Clinical evaluation:
Patients usually present with spinal deformity, shoulder asymmetry, chest wall and back asymmetry, waist asymmetry noticed by parents or school teachers. In cases of severe deformity, a child may present with breathing difficulty.
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Clinical presentation of a typical scoliosis patient
Adams forward bending test is a simple examination that can be used for screening scoliosis in children at school or at clinics especially to identify mild curves (deformity). In this test, the patient is asked to bend forward as if to touch toes, keeping the knees straight. In a normal person, the back is completely straight, whereas if scoliosis is present, their back becomes prominent with one side higher than the other, due to deviation of underlying spine and attached ribs to one side (crooked spine).
Adams Forward bending test
Radiographs:
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X-rays of the whole spine standing PA and lateral view for the curve measurement and
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X-ray Side-bending PA for evaluation of flexibility of the curve (needed only for pre-operative planning).
MRI screening of the whole spine:
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To rule out spinal cord anomalies such as Arnold Chiari Syndrome, Syringomyelia, diastematomyelia and Tethered Cord Syndrome.
CT Scan of the spine:
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Only in the selected case when the deformity is due to complex congenital anomalies or in tethered cord anomalies
Management:
The general goal is to prevent curve progression and a balanced spine. The treatment options depend on the size and progression of the curve, age of the patient and the aetiology of scoliosis.
The three O’s
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Observation: when curve deformity is less than 25 degrees
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Orthotics: when curve deformity is between 25-40 degrees
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Operation: when curve deformity is more than 50 degrees
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For the curve, less than 25° in an immature child, 6 monthly or yearly Observation is recommended.
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Orthotic (braces) is recommended for immature patients with progressive curves between 25-50°. Bracing, however, does not permanently improve or correct the curve but tries to prevent it from worsening.
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Operation: Curve >45-50° requires surgical correction and the type of surgical correction depends on the age of the patient. Instrumented correction and fusion surgery is the gold standard of treatment for adolescent scoliosis while in early-onset scoliosis (age <8-years) surgical correction requires growth rod or growth guidance techniques. Not all children with scoliosis require surgical correction. Moreover, complete correction of the deformity is not necessary and sometimes risky too. In fact, the aim of surgery is to stop progression and obtain a balanced spine.
Brief description of surgical procedures:
Instrumented fusion Vs fusion less surgeries:
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Instrumented fusion
Instrumented fusion is a gold standard surgical procedure for correcting scoliosis deformity. Screws, hooks or wires are put in the vertebrae and slowly connected to rods and with some manoeuvres, deformed spine is corrected. The bone graft is then placed over the back of spinal bones (laminae) to obtain a bony fusion. Fusion simply means fusing together two or more vertebrae so that they heal into a single, solid bone and maintain the deformity in the corrected position.
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Fusionless surgery: Growth rods or growth guidance system
It is performed to manage early-onset scoliosis (EOS), which refers to spine deformity that is present before 10 years of age. These techniques stop the progression of a curve without adversely affecting the future growth of the spine and thorax. This requires 6 monthly small distraction procedures till the age of 11-12 years (by then most of the spine growth is over). After that final instrumented fusion is performed.
Post-operative care:
Postoperative hospital stay includes 1 day in ICU for monitoring and 4-5 days in the room. Most children are able to walk within 2-3 days after surgery and can return to school after 6-8 weeks. However, bending, lifting heavy objects, sitting on the floor or sports activity should not be resumed for the next 4-5 months. With modern equipment and skills, especially the use of intraoperative neuromonitoring devices, scoliosis surgery has become a safe procedure with a complication rate of less than 1%.
Case 1: Adolescent Idiopathic Scoliosis
The 10-year old girl presented with progressive painless deformity of the back of 4 years duration. She had menarche 6-months back. On examination, she had a right-sided flexible scoliosis curve.
Diagnosis: Idiopathic Scoliosis Lenke type 1, Kings type III, cobbs angle 53 degrees.
Surgery: Scoliosis deformity correction by selective thoracic fusion (posterior instrumentation D4-D11)
Case 2: Early onset scoliosis
Painless progressive deformity of the spine since birth in an independent and physically active eight-year-old girl. No neurocutaneous markers were seen. Neurology was normal and there was no limb length discrepancy.
X-Ray evaluation revealed kyphoscoliosis deformity with cobbs measuring 150 degrees. MRI study did not reveal any intraspinal pathology
Case 3: Complex congenital scoliosis
A 16 years old Iraqi boy, presented with painless progressive deformity since birth. Underwent scoliosis (hemivertebra in-situ fusion) correction 1 year back. On evaluation, he had severe thoracolumbar scoliosis. MRI revealed distal tethering of cord and cervical diastematomyelia. He has undergone tethered cord release and cervical diastematomyelia repair followed by revision scoliosis deformity correction by posterior instrumentation T2-ilium and vertebral column resection at T11 level.
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