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Paediatric Spinal Problem

Klippel Feil Syndrome

  1. Kippel Feil Syndrome is because of congenital fusion of one or more cervical vertebrae presentings with classical triad of low hair line, short ‘web’ neck (prominence of trapezius muscle), and limited neck motion.

  2. It results from a failure of the normal division of the cervical somites during the 3rd through 8th week of embryogenesis.  

Musculoskeletal system

  1. Scoliosis (60% cases because of fusion of cervical & cervicothoracic junction )
  2. Sprengel’s deformity (50%, it is congenital elevated or undescended scapula)
    Omovertebral bone bridges the cervical spine to the scapula and limits the neck and shoulder motion.
  3. Webbing of neck,facial asymmetry & torticollis
  4. Radiographic findings of congenital cervical vertebral fusion are diagnostic. Spinal canal stenosis, enlargement of foramen magnum
  5. Syndactyly and diffuse or focal upper extremity hypoplasia.
  6. Secondry osteoarthritis, disc degeneration, spinal stenosis.  

Other Findings

  1. Genito — renal anomalies (~ 35%, so USC is recommended)
  2. Congenital heart defects (~15% , so ECHO is recommended)
  3. Ocular anomalies
  4. Deafness
  5. Synkinesis or mirror movements
  6. Brain stem anomalies
  7. Congenital cervical stenosis
  8. Adrenal aplasia  

Congenital (Infantile) Muscular Torticollis

  1. Torticollis or twisted neck is a symptom of cervical spine abnormality. It may be of two types — congenital & secondary. Congenital or infantile torticollis is commonest form of wry neck. It is fibrosis of sternocleido mastoid (SCM) muscle on one side that fails to elongate as child grows. The cause is muscle ischemia from a distorted position in utero (breech) or birth injury.
  2. A history of difficult labour - lump over SCM (sternomastoid tumor) in first few weeks of life, which disappears in few months. At this stage there is neither deformity nor obvious limitation of movements. Deformity does not become apparent until the child is 1- 2 years old.
  3. The head is tilted toward the involved SCM and the chin is rotated towards the contralateral shoulder, producing the ‘Cock robin’ appearance. SCM on involved side may feel tight & hard and a mass or knot can be detected in the body of SCM in first 3 months of life.
  4. There may be asymmetrical development of face (plagiocephaly).  

Spina Bifida & Diastometamyeli

  1. Diastometamyelia
    1. It is congenital malformation of neural axis in which there is sagittal division of the spinal cord or its intraspinal derivatives into two halves by projection of an osseous, fibrocartilagenous, or fibrous spur, that is attached anteriorly to one or more vertebral bodies and posteriorly to dura.
    2. Multiple congenital anomalies of vertebrae as incomplete spinal fusion, spina bifida, myelomeningocoele, & kyphoscoliosis are often present.
    3. Lumbar vertebrae (L1 to L3) are involved in 50% of cases.
    4. Hallmark cutaneous abnormalities, that are found near the midline at the level of lesion include abnormal tufts of hair, dimpling of the skin, illdefined subcutaneous fatty tumors, and cutaneous vascular malformation.
    5. Neurological motor abnormalities in lower limbs are not ordinarily detected at birth. Initial features may occur from infancy to young adulthood.
    6. Radiological hall mark is widening of interpedicular distance and vertical bony spur or spicule. Often the posterior elements are incompletely formed in the region of diastematomyelia (division or reduplication of cord).
  2. Spina Bifida Oculta 
    1. It is failure of fusion of two or more vertebral arches, most commonly noted in L5 & S1 vertebrae.
    2. It is most common congenital anomaly of spinal column and is of no clinical significance in majority.

Caudal Regression Syndrome (Sacral & Lumbosacral Agenesis)

  1. It is severe axial skeletal and neural deficiency characterized by absence of variable amounts of the sacrum & lumbar spine & associated neural elements.
  2. Developmental failure ranges from mere absence of the lower coccygeal segment to complete aplasia of vertebrae below T12 segment.
  3. A higher than normal incidence of diabetes mellitus in mother and concomitant anomalies of viscera, genitourinary, lower gastrointestinal systems and bladder bowel function is often present.

Tethered Cord (Thickened Filum Terminale)

  1. Tethered cord is usually due to presence of tight filum terminle that does not permit the conus medullaris to migrate upwards normally with growth.
  2. Other causes of tethered spinal cord with in thecal sac preventing migration of conus above L2 or L2 level are diastematomyelia, lipomeningocele, scarring secondary to intrathecal procedures such as closure of meningomyelocele sac.
  3. Back pain radiating into posterior thighs, lower limb weakness, muscle (esp hamstring) tightness, foot deformities as equinus or equinocavus and occasionally bladder or bowel problems. Hemangiomas, sacral dimpling or hairy patch may be present.
  4. Radiological hallmark of spinal cord tethering is presence of conus below its normal level of L1 or L2 after age 2 months.  

Vertebra plana


Vertebra plana is collapse & increased density of vertebral body, with normal or increased disc space. Causes are Eosinophilic granuloma (histiocytosis), Ewings’s sarcoma, metastasis, leukemia, tuberculosis and Calves disease (osteochondritis of vertebral body)




Suspensory plaster cast of sayre, hinge or turn buckle cast of Hibbs and Kisser, Milwaukee brace, Boston brace, Charleston nightime bending brace, Wilmington brace and thoraco-lumbo-sacral orthoses (TLSOs) are used to treat idiopathic scoliosis.


Idiopathic Scoliosis: Brace Treatment

  1. Indications
    Brace treatment is restricted to immature children in an attempt to prevent curve progression during further skeletal growth. So it is indicated in
    1. Growing adolescents (Risser 0, 1 or 2) who on presentation, have curves in range of 30-45 degrees or
    2. Who have had documented progression exceeding 5 degrees in curve that initially measured 20-30 degrees
    3. These patient should have deformities that are considered cosmetically acceptable.
    4. Patient should be realistically willing to wear the brace for the prescribed amount of time.
    5. TLSOs are the most commonly used orthoses today, but their use is restricted to patients whose curve apex is at or below T7. Fortunately, this is the case in most idiopathic scoliosis.
  1. Contraindication  
    1. Large curves (>450) in growing adolescents (need surgical treatment).
    2. Extreme thoracic hypokyphosis  
    3. Skeletally mature adolescent (Riser 4 or 5, and in females 2 years post menarchal) 

  1. Treatment plan of Idiopathic Scoliosis

Curve Magnitude (degree)

Risser Sign Grade



1 or 2


30 —45

Brace (begin after 25°)





4 or 5

(when curve >50 degree)

Genetic & Developmental Disorders of Bones

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