CONGENITAL KYPHOSIS

KYPHOSIS In the sagittal plane, the normal spine has four

balanced curves: the cervical spine is lordotic; the thoracic spine is kyphotic (20 to 50 degrees), with the curve extending from T2 or T3 to T12; the lumbar region is lordotic (31 to 79 degrees); the sacral curve is kyphotic

On standing, the thoracic kyphosis and lumbar lordosis are balanced

Kyphosis of 50 degrees or more in the thoracic spine usually is considered abnormal.

ANATOMY ..

DEFINITION …  is abnormal curving of the spine that causes a bowing or rounding of the back, which leads to a hunchback or slouching posture.

CAUSES .. Osteoporosis. Disk degeneration. Cancer and cancer treatments. Scheuermann's disease. Birth defects.

TYPES .. Postural kyphosis: it is commonly attributed to

slouching and is reversible by correcting muscular imbalances. ( By combination of neuromuscular re-education and exercise) .

Scheuermann’s kyphosis: can cause varying degrees of pain. Can affect other area of spin. The cannot consciously correct posture because the apex of the curve is very rigid.

CONT. Congenital kyphosis: Vertebrae may be malformed or fused

together and can cause further progressive kyphosis as the child develops.

Nutritional kyphosis can result from nutritional deficiencies, especially during childhood, such as vitamin D deficiency which causes Rickets, a softening of the bones that results in curving of the spine and limbs under the child’s body weight.

CONGENITAL KYPHOSIS Congenital kyphosis is an uncommon deformity, but

neurological deficits resulting from this deformity are frequent. Congenital kyphosis occurs because of abnormal development

of the vertebrae consisting of a failure of formation or failure of segmentation of the developing segments.

The spine may be either stable or unstable, or it may become unstable with growth.

Spinal deformity in congenital kyphosis usually will progress with growth, and the amount of progression is directly proportional to the number of vertebrae involved, the type of involvement, and the amount of remaining normal growth in the affected vertebrae

Winter et al. described 130 patients with congenital kyphosis of three types.

Type I iscongenital failure of vertebral body formation. Type II is failure of vertebral body segmentation Type III is a combination of both of these conditions McMaster and Singh further subdivided type I

congenital kyphosis into posterolateral quadrant vertebrae, posterior hemivertebrae, butterfly (sagittal cleft) vertebrae, and anterior or anterolateral wedged vertebrae.

CLINICAL AND RADIOGRAPHICEVALUATION

The diagnosis of a congenital spine problem usually is made by a pediatrician before the patient is seen by an orthopaedist.

The deformity may be detected before birth on a prenatal ultrasound examination or noted as a clinical deformity in a neonate.

If the deformity is mild, congenital kyphosis can be overlooked until a rapid growth spurt makes the condition more obvious

Physical examination usually reveals a kyphotic deformity at the thoracolumbar junction or in the lower thoracic spine.

A detailed neurological examination should be done to look for any subtle signs of neurological compromise.

Associated musculoskeletal and nonmusculoskeletal anomalies should be sought on physical examination.

High-quality, detailed anteroposterior and lateral radiographs provide the most information in the evaluation of congenital kyphosis.

Flexion and extension lateral radiographs are helpful indetermining the rigidity of the kyphosis and possible instabilityof the spine

Role of CT Scan: To detect the nature of bony deformity Size of cartilage analge Estimate possible progression

An MRI study should be obtained in most patients because ofthe significant incidence of intraspinal abnormalities.

OPERATIVE TREATMENT The natural history of this condition usually is one of

continued progression and an increased risk of neurological compromise

Therefore, surgery is the preferred method of treatment

If the diagnosis is uncertain or the deformity is mild, close observation may be an option.

Unless compensatory curves are being treated above or below the congenital kyphosis, bracing has no role in the treatment of congenital kyphosis, because it neither corrects the deformity nor stops the progression of kyphosis.

Surgery is recommended for congenital kyphosis.

The type of surgery depends on the type and size of the deformity, the age of the patient, and the presence of neurological deficits.

Procedures include 1. posterior fusion, 2. anterior fusion, 3. combined anterior and posterior fusion,4. anterior osteotomy with posterior fusion.

Fusion can be done with or without instrumentation.

TREATMENT OF TYPE I DEFORMITY

If associated scoliosis: Anterior approach for decompression may need to be on the concavity of the scoliosis to allow the spinal canal to move into the midline.

Laminectomy has no role in the treatment.

<55 degrees / < 5 year >55 degrees / > 5 yearPosterior fusion alone, extending from one level above the kyphotic deformity to one level below

Anterior strut grafting with temporary distraction+Posterior Fusion +/- Instrumentation

Use of skeletal traction (halo-pelvic, halo-femoral, or halo

gravity)to correct the deformity is tempting but is notrecommended because there is a risk of paraplegia

TREATMENT OF TYPE II DEFORMITY

<55 degrees >55 degrees/Late Presentation

Posterior Fusion with instrumentation with 1 vertebrae above and below.

Anterior Osteotomy + Fusion followed by posterior fusion and instrumentation.

COMPLICATIONS OF OPERATIVE TREATMENT

Some of the more frequent complications of treatment of congenital kyphosis are

Pseudarthrosis Progression of kyphosis paralysis

Pseudarthrosis and progression of the kyphotic deformity can be minimized by performing anterior and posterior fusions for deformities of more than 50 degrees

The risk of this complication can be lessened by not attempting to maximally correct the deformity with instrumentation

Instrumentation should be used more for stabilization of rigid deformities instead of correction

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