There are many variations. These have been divided and grouped into eight separate categories as shown below:
This manifests as a lateral deviation and rotation of the spine with no identifiable cause, but it is usually related to a brain stem, hormonal or proprioception disorder. The adolescent form is most common but there is an Infantific form also a Juvenile form. Right thoracic curves, with apex T7 or T8 are the most common, followed by double major, left lumbar and right lumbar, in that order.
Severe curves [>90 degrees] accompanied by cardiopulmonary dysfunction result in extreme pain and early death.
Afflicted children are first noticed because of a trunk shift, shoulder or pelvic asymmetry, asymmetric umbilical reflex, spinal curvature, asymmetric rib hump and/or limb length inequality.
Inclusion of the iliac crest on radiographs will reveal the degree of skeletal maturity. A lateral radiograph to check for spondylolisthesis is also recommended. An M.R.I. scan is needed if there is excessive Kyphosis, rapid progression, neurologic symptoms, onset before 11 years, of left thoracic/thoracolumbar curves.
Treatment includes careful observation, some for bracing and if needed surgery.
Bracing does not rectify the problem but does halt rapid progression.
Surgical options include instrumentation without fusion for Infantile, also Juvenile forms. There are several options for the Adolescent form but posterior fusion and instrumentation with Harrington distraction rods are the most common. In all forms of surgery for idiopathic scoliosis, great care must be taken to choose the most appropriate/effective fusion level. It varies according to the age of the child, the degree of the curve and also the degree of progression.
Surgical complications include pseudoarthrosis, implant failure [due to early hook cut out and late rod breakage] also infection of the wound, and the appearance of a neurologic deficit that was not apparent preoperatively.
Points To Note Re. Infantile Scoliosis:
- male predominance
- manifests between 2 months and 3 years
- is accompanied by a flattening of the skull and other congenital defects
Points To Note Re. Juvenile Scoliosis
- similar to adolescent scoliosis in terms of symptoms and treatment
- manifests between 3 years and 10 years
- curve progression is a high risk [70% require treatment, 50% require bracing and 50% require surgery.
- fusion needs to be delayed until adolescence.
CONGENITAL SPINE DISORDERS
Congenital spinal disorders are due to a development defect in the mesenchymal anlage during the 4th to 6th week of gestation.
Three basic defect types are noted:
- failure of formation
There are two separate disorders:
in which a failure in formation
Type I gives the worst prognosis for progression and neurological involvement. It is also the most likely to result in paraplegia and requires surgery. Posterior fusion is the most effective in children under 5 years with curves less than 50 degrees. Combined anterior/posterior fusion is more suitable for older children or those with more severe curvature.
Type II. congenital kyphosis is not usually as serious as Type I.but posterior fusing is needed if the curving is progressive.
This is the most common congenital spinal disorder. The best prognosis is a block vertebrae caused by a failure of segmentation. The worst prognosis presents a unilateral unsegmented bar with a contralateral fully segmented hemivertebra, which will require surgery [the deformities do not require surgery unless progression [which isn’t usual] becomes obvious].
Posterior fusion either with or without instrumentation is the most common treatment for progressive curves. If the ‘crankshaft’ phenomenon occurs however [due to continued anterior spinal growth] the anterior/posterior fusion may be required.
This is an A.D. disorder associated with neoplasia and skeletal abnormalities. It is of neural crest origin and is characterised by numerous neurofibromas [soft fibrous swellings that grow from a nerve] and cafe-au-lait spots. It is an uncommon, inherited disease. It manifests in its most serious form bone abnormalities . The spine is the most common site and may reveal vertebral scalloping, enlarged foramina, severe apical rotation, pencilling of transverse processes or ribs, a paraspinal mass and/or short, light curves.
Surgical treatment involves posterior fusion with instrumentation if there is less than 50 degrees kyphosis. Anterior fusion and strut grafting combined with posterior fusion and instrumentation is required if there is more serious kyphosis.
Neurologic involvement is the most common complication of neurofibromatosis. This can be caused by aspects of the deformity itself, by a soft tissue mass, an intraspinal tumour or by dural ectasia. Anterior decompression with strut grafting followed by posterior fusion is required if this happens.
There are four major types:
[information given under the Congenital Spine Disorders section]
This is characterised by increased thoracic kyphosis [more than 45 degrees] with more than 5 degrees anterior wedging at three sequential vertebrae.
Other clinical symptoms include:
- end plate irregularitie
- spondylolysis [20-50%]
- Schmorl’s nodes
- disc narrowing
It is more common in males and usually manifests in adolescence.
Treatment involves bracing [if the curve is progressive] before skeletal growth is completed. If surgical correction is needed [for an adult with more than 65% curve] it usually involves anterior release and interbody fusion followed by posterior fusion with dual rod segmentally attached, compression instrumentation.
Lumbar Scheuermann’s disease is less common than the thoracic variety and usually less severe as there is no vertebral wedging, though irregular end plates, decreased disc height and Schmorl’s nodes are evident.
Postural Round Back
This disorder is similar to other forms of Kyphosis but there are no vertebral body changes and no sharp angulation. Correction of the problem usually involves backward bending and prone hyperextension. Sometimes bracing is required. Surgery is rare.
Kyphosis can be caused by infections, various bone Dysplasias [including Kneist syndrome] neoplasms, spondylitis or trauma. Post laminectomy kyphosis can be sufficiently severe to require early anterior and posterior fusion.
Many children with neuromuscular disorders develop some form of spinal abnormality; scoliosis being the most common. The curving differs from the curves of idiopathic scoliosis in several ways including:
- they involve more vertebrae
- are less likely to have compensatory curves
- they progress more rapidly and often continue after maturity
- they may be associated with other bone deformity, cervical involvement and pelvic obliquity
- there is decreased pulmonary function plus pneumonia and/or atelectasis
- wheelchair bound patients may become bedridden
Orthotic use is advised until age 12 when corrective fusion is required to provide permanent stability.
Children with upper motor neuron disease are usually treated with a body jacket or seat. Orthotics but eventually require fusion if there’s a curve progression of >50 degrees. Children whose affliction is severe may require both anterior and posterior procedures.
LOW BACK PAIN
Acute, persistent back pain in children should always be investigated. It can be associated with osteomyelitis or diskits. Other, probable disorders, include:
- sciatica [herniated nucleus pulposus]. This may require surgical intervention.
- spondylolysis which can result from sports injuries
- a tumour such as osteoid osteoma.
- a spinal cord anomaly
Bone scans are the best method of screening and diagnosing a possible serious cause of back pain.
CERVICAL SPINE DISORDERS
There are many disorders in this category including:
Pseudosubluxation of the Cervical Spine
Intervertebral Disc Calcification Syndrome
Information concerning each disorder is given, as listed, in the ensuing text.
1) Klippel-Feil Syndrome
This is often associated with renal disease, congenital scoliosis, Sprengel’s deformity, brain stem abnormalities, synkenesis, congenital heart disease and/or congenital cervical stenosis. It is characterised by multiple fused cervical segments due to the segmentation failure of cervical somites within the first eight weeks of gestation. In most cases the treatment is conservative but chronic pain may require surgery. Afflicted children should avoid aggressive sports.
2) Atlantoaxial Instability
This is often associated with various osteochondrodystrophies, Down Syndrome, osodontoidium or other disorders. Rotatory Atlantoaxial Subluxation. [which is often caused by retropharyngeal inflammation] may manifest torticollis. If so this is best treated with traction and bracing, early. Traumatic Atlantoaxial Subluxation may also manifest torticollis which should be treated initially with a soft collar for up to a week. If symptoms persist, cervical traction may be required followed by fusion for fixed rotary subluxation.
3) Os Odontoideum
This appears like a type II odontoid fracture. It may represent the residuals of a previous trauma but causes can’t be verified. If radiographs reveal instability of more than 3mm or neurologic symptoms present a posterior C1-C2 fusion is required.
4) Pseudosubluxation of the Cervical Spine
Similar symptoms to those involving pseudosubluxation are common in children over 8 because of the orientation of the facets. The more serious disorder can only be firmly diagnosed if:
- major trauma is evident
- the subluxation with neck extension is reduced
- pain is resolved easily
- the posterior spinolaminar line and the interspinous distances are mal-aligned
- there is anterior swelling.
5) Intervertebral Disc Calcification Syndrome
This disorder is characterised by an increase in E.S.R. reading, low grade fevers, radiographic disc calcification without erosion, decreased R.O.M. and severe pain. The C-spine is most comTmonly involved. It is a self-limiting disorder and treatment is conservative.
6) Basilar Impression/Invagination
This disorder is a bony deformity at the base of the skull which is characterised by paraesthesia [sometimes hydrocephalus] and weakness. It is caused by cephalad migration of the odontoid into the foramen magnum. Operative treatment is sometimes necessary and can include transoral resection of the dens, occipital laminectomy, also occipitocervica fusion and wiring. Other Spinal Abnormalities
- Diastomatomyelia which is a longitudinal cleft in the spinal cord; either cartilaginus, fibrous or osseous bar. It can lead to tethering of the spinal cord with associated neurologic deficits. Sometimes it is necessary to resect