Syringomyelia is a condition where a cyst, called a syrinx, develops in the spinal cord. It most commonly affects the lower cervical spine. It is often associated with abnormalities of the skull or spinal column. The majority of cases are linked to Chiari malformation type 1, where the cerebellar tonsils are displaced into the spinal canal. Symptoms vary depending on the location of the syrinx but can include pain, loss of sensation, muscle weakness or atrophy, and autonomic dysfunction. Diagnosis is made using imaging like MRI. Treatment involves surgery to decompress pressure on the spinal cord like laminectomy with the goal of resolving the syrinx.

1. SYRINGOMYELIA
DR. VAIBHAVI PARMAR (PT)

2. • Syringomyelia is acquired developmental of cavity ( syrinx)
within the central spinal cord.
• The lower cervical segments usually affected, but the
extension occurs upward into brain stem or downwards as far
as filum terminale.
• The cavitation appears to develop in association with
obstruction

3. • Frequently associated developmental abnormalities
– Vertebral column (thoracic scoliosis, fusion of vertebrae, or
klippel-feil anomaly),
– Base of the skull (platybasia, basilar invagination),
– Cerebellum and brain (type I chiari malformation)
• 90 percent of cases of syringomyelia have type I chiari
malformation.

4. TYPES
• Congenital- associated with chiari malformations
• Acquired – spinal cord tumors (usually intramedullary,
Especially hemangioblastoma)
– Traumatic myelopathy
– Spinal arachnoiditis and pachymeningitis
– Secondary myelomalacia from cord compression (Tumor,
spondylosis), infarction, hematomyelia
• Idiopathic

5. Depending on the connection with
fourth ventricle

6. PATHOGENESIS
• BASED ON GARDEN’S “ HYDRODYNAMIC THEORY”
cv junction
anomaly
Impaired CSF
flow from 4th
ventricle to
cisterna magna
Transmission of CSF arterial
pulse to patent central canal
which dilates the canal below
level of compression

8. CLINICAL FEATURES
• Sensory
• Dissociated sensory loss
• In either or both arms, or in a shawl like distribution ,
• dysesthetic pain, a common complaint in syringomyelia,
usually involves the neck and shoulders, but may follow a
radicular distribution in the arms or trunk.
• When the cavity enlarges to involve the posterior columns,
position and vibration senses in the feet are lost;
astereognosis may be noted in the hands.

9. • Motor
• Syrinx extension into the anterior horns of the spinal cord
damages motor neurons (lower motor neuron) and causes
diffuse muscle atrophy that begins in the hands and
progresses proximally to include the forearms and shoulder
girdles. Clawhand may develop.
• Respiratory insufficiency, which usually is related to changes
in position, may occur.

10. Autonomic
• Impaired bowel and bladder functions usually occur as a late
manifestation.
• Sexual dysfunction may develop in long-standing cases.
• Horner syndrome may appear, reflecting damage to the
sympathetic neurons in the intermediolateral cell column.
• Reflex diminshed or absent
• Painless ulcers of the hands are frequent. Edema and
hyperhidrosis can be due to interruption of central autonomic
pathways.
• Neurogenic arthropathies (charcot joint)

11. DIAGNOSIS
• X RAY CERVICAL SPINE
• • 3D CT
• • MRI

12. TREATMENT
• ANALGESICS - FOR HEAD ACHE &
NECK PAIN
• SURGERY – DECOMPRESSIVE SX
• SUBOCCIPITAL AND CERVICAL
DECOMPRESSION.
– LAMINECTOMY AND SYRINGOTOMY
(DORSOLATERAL MYELOTOMY)

13. • Shunts
– Ventriculoperitoneal shunt - indicated if ventriculomegaly
and increased intracranial pressure are present.
– Syringosubarachnoid dorsal root entry zone shunt
– Syringoperitoneal shunt
Fourth ventriculostomy

14. Neuroendoscopic surgery
– A fibroscope inserted through a small myelotomy allows
inspection of the intramedullary cavity.
– This technique is particularly useful in evaluating and
treating multiple septate syrinxes.
– Septa are fenestrated, either mechanically or by laser. Fluid
from the cavity is then shunted into the subarachnoid space.