TORTICOLLIS CAUSED BY INTERVERTEBRAL DISC CALCIFICATION

TOBENNA OKEZIE, M.D., Orthopaedic Surgery Resident

S. JAY KUMAR M.D., Attending Pediatric Orthopaedic Surgeon

February 21, 1996

CLINICAL CASE PRESENTATION

ORTHOPAEDIC DEPARTMENT

THE ALFRED I. DUPONT INSTITUTE

WILMINGTON, DELAWARE

CASE HISTORY

Patient is a 4 year old male who developed the insidious onset of neck pain and stiffness 4 days prior to admission. Over the intervening period, the patient suffered from worsening of his symptoms which led to increasing irritability and difficulty sleeping. On the day of admission, he was seen by a local pediatrician for severe neck pain. It was observed that his head was in a fixed position just slightly right of midline. The patient's past medical history was remarkable for two recent self-limited episodes of hives and urticaria on his trunk and extremities that was treated with prednisone. There was vague history of remote trauma about one month previously when the patient had been "horsing" around with his brother. The child had no evidence of a viral prodrome, constitutional symptoms or travel history. He was sent to a local hospital where radiographs of the cervical spine demonstrated calcifications in the C3-4 and C5-6 intervertebral disc spaces . Neurological exam was normal. The child's pain was refractory to morphine treatment and he was transferred to A.I. for the management of his intractable pain. On arrival, his physical exam was unchanged. Blood work revealed a WBC count of 12.7K with 75% PMNs and an ESR of 80. Radiographs of spine showed the presence of an addition calcification in his thoracic spine . He was treated with oral valium and a soft cervical collar. Over the ensuing 24 hrs the child demonstrated marked improvement of his symptoms and was switched to motrin. He was subsequently discharged.

TORTICOLLIS CAUSED BY INTERVERTEBRAL DISC CALCIFICATION

Calcification of intervertebral discs is not uncommon in adults and is usually considered a sign of degeneration due to normal aging without specific clinical or anatomic significance.

Pediatric disc calcification was first described by Baron in 1924 and since that time more than 100 cases have been reported. It is slightly more common in boys than girls (7:5 ratio) with an average age of presentation of 8 years (range 8 days to 13 years)

It is most common in the cervical spine, where it is especially symptomatic. Asymptomatic lesions have been detected in the thoracic spine on routine chest radiographs.

ETIOLOGY

This is unclear. Recognized causes of calcification in adult intervertebral disc such as hyperparathyroidism, hemochromatosis and ochronosis have not been implicated in children.

In addition, other causes of calcification in pediatric connective tissues, including hypervitaminosis D and chrondrocalcinosis, have not been found to be associated with calcification of intervertebral discs. There is no evidence available to suggest that a metabolic defect is present.

Pathophysiology appears to involve calcification of the nucleus pulposus. The annular ligament is spared. The calcified nucleus pulposus may herniate anteriorly into the prevertebral soft tissues or posteriorly into the spinal canal. Changes have been observed in vertebral bodies, but there clinical significance is unclear.

There is a history of antecedent trauma in only about (30%) and upper respiratory infection in (15% with a latent period between 5 days and 3 week.

SIGNS AND SYMPTOMS

Onset of symptoms is abrupt, usually between 12 and 48 hours. Common symptoms are neck pain, spasm torticollis and reduced range of motion. Infrequently, dysphagia or long tract signs will be observed.

Fever has been observed in 23% of patients. A significant elevation of white cell count has rarely been observed

XRAYS

The number of calcified discs varied from 1 to 12 (mean 1.69) . Symptomatic calcified discs are most common at C6-7. Radiologic examination shows images of calcium density in the normally radiolucent intervertebral discs. Anterior or posterior protrusion can be observed. The lesions demonstrate high density on CT and low signal intensity on MRI.

TREATMENT

The natural history is typically one of complete clinical radiographic resolution. Two-thirds of patients are free of symptoms within 3 weeks and 95% within 6 months. The radiographs show regression or disappearance of the calcified deposits in 90%. In the absence of compression of the spinal cord conservative management is preferred. The use sedatives analgesics and cervical traction are tailored to the symptoms. The use of a soft cervical collar and avoidance of body contact sports may be prudent.

One case has been reported in which an anterior cervical discectomy was performed after six weeks of unsuccessful conservative management in al child with a severe radiculopathy.

REFERENCES
  1. Caffey, J.: Pediatric X-ray diagnosis
  2. Herring J.A. Cervical disc calcification: Instructional Case Journal of Pediatric Orthopaedics vol 8: 613-616 1988
  3. Loder, R., Hensinger, R.N., Developmental Abnormalities of the Cervical Spine Chapter 17 In The Pediatric spine :Principles and Practice , S.L. Weinstein, Editor Raven Press Ltd., New York 1994
  4. Sonnabend, D.H., Taylor, T.K.F., Chapman, G.K. : Intervertebral disc calcification syndromes in Children JBJS 64-B p.25-31