JEFFREY J. METER, M.D., Orthopaedic Resident
ROBERT STANTON, M.D., Pediatric Orthopaedic Attending
January 16, 1996
CLINICAL CASE PRESENTATION
THE ALFRED I. DUPONT INSTITUTE
A condition which is defined by a tubular cavity which contains fluid within the spinal cord.
Heavy birthweight, protracted labor, traumatic delivery
Symptoms - Headache - worsens with cough, sneeze, strain. Common in communicating form. Neckache. Body/joint pains - worsens with straining. Often multiple misdiagnoses - CTS, cubital tunnel... Numbness - may replace pain
Signs - Horner's syndrome, Nystagmus, Muscle wasting, LE spasticity, Charcot UE joints, Pes Cavus, Short neck, Low hairline, Limb length inequality, Hand/foot asymmetry, Diplopia, Giddiness, Dysphagia, Dysphonia, Salivation Disorder, Sexual dysfunction, Abnormal pain & temperature sensibility, Asymmetric abdominal reflexes
Rapidly progressive scoliosis, Upper thoracic curve, Left thoracic curve, High double thoracic curve, Cervical bony , nomalies, Erosion of cervical bodies, Widened spinal canal, Basilar invagination, Cervical ribs
Arnold-Chiari malformation, Klippel-Feil, Myelomeningocele
Two American studies quote the incidence of scoliosis in syringomyelia to be about 60% If a child develops symptoms of scoliosis before age 16 the eventual incidence of scoliosis is 82%, if later than age 16 symptoms develop, the incidence is 48%.
Suspect if C5 canal is 6mm greater than body. If it is 4mm there is a 3:1 probability of syrinx.
If C5 canal is wider than C6 body - suspect.
The cystic lesions are typically located dorsal to the central canal. In this position they are presumed to impinge on the medial nuclear groups - ventromedial and dorsomedial, and thus affect the anterior horn cells, thereby affecting the muscles of the trunk in those segments.
Initial treatment involves draining the cyst, observing the spine, and fusing the spine with progression. Risks of surgery are substantial, with possible neurologic progression.