The Journal of Pediatrics
Volume 157, Issue 1 , Page 25, July 2010

50 Years Ago in The Journal of Pediatrics:

A Report of 16 Tumors of the Spinal Cord in Children; The Importance of Spinal Rigidity as an Early Sign of Disease

Departments of Neurology, Pediatrics, Neurosurgery, and Human Biology, Stanford University, Palo Alto, California

Article Outline

 

Richardson FL. J Pediatr 1960;57:42-54.

Fifty years ago in The Journal, Richardson described 16 children with tumors involving the spinal cord. “The diagnoses were difficult,” he chronicled. Indeed, for these 1950s children, poliomyelitis was the more typical etiology of spinal symptomatology, and a pediatrician's tools were limited to lumbar puncture and myelography. Richardson lamented the delay to diagnosis, a median of 7 months.

Do we fare better today making this diagnosis? No! One report of 25 children with primary spinal cord tumors found an average of 7.8 months to diagnosis.1 Have we heeded Richardson's lessons? Perhaps not.

Richardson identified the “presence of painful spinal rigidity” in most cases. Specifically, these children often resist flexion at the vertebral column because of pain, preferring instead to flex at their hips or knees. We cannot forget the early manifestations he points out, such as irritability, sudden screaming at night, anorexia, weight loss, constipation, fear of moving about, head tilt, waddling gait, and anhidrosis below the level of the tumor. We could also add the clinical pearl of scoliosis with a left thoracic curvature, a rare finding, but highly indicative of a tumor or occult syrinx. With time, more obvious neurologic findings ensue: paraparesis, absent or increased reflexes, paresthesias, or enuresis.

We still tend to forget the presentation of these tumors. Spinal ependymoma, astrocytoma (glioma), meningioma, neurofibroma, lipomas, dermoid cyst, or other aggressive cancers are found only after misdiagnosis and delay. Richardson remains on target: “Routine investigations, such as x-rays of the vertebral column, are regarded too frequently as satisfactory evidence of the absence of disease….It is not justifiable to delay myelography for weeks or months, during which time the patient may be in considerable pain, until serious deterioration gives additional clinical or neurological signs.” We could simply substitute “magnetic resonance imaging” for “myelography.” Make haste, and don't waste. Painful spinal rigidity and other aforementioned findings merit a sooner rather than later trip to the magnetic resonance imaging suite.

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Reference 

  1. Crawford JR, Zaninovic A, Santi M, Rushing EJ, Olsen CH, Keating RF, et al. Primary spinal cord tumors of childhood: effects of clinical presentation, radiographic features, and pathology on survival. J Neurooncol. 2009;95:259–269

PII: S0022-3476(10)00036-3

doi:10.1016/j.jpeds.2010.01.021

The Journal of Pediatrics
Volume 157, Issue 1 , Page 25, July 2010