Numbers
- Spinal intradural arachnoid cysts are rare pathologic entities in adult populations.
- Across the literature review cohort of 293 cases, the mean patient age was 51.4 years (ranging from 19 to 91 years).
- Sex distribution is nearly equal, consisting of approximately 54% men and 46% women.
- Cysts of primary (idiopathic) origin represent the vast majority of cases (87%), whereas secondary cysts account for 13%.
Pathophysiology
- Pathology
- CSF-filled sacs contained by the arachnoid mater.
- The degree of communication with the surrounding CSF space is variable with some cysts freely communicating and others not at all
- The exact etiopathogenesis of spinal intradural arachnoid cysts remains unclear.
- Neurologic symptoms develop due to
- Neuronal compression of spinal cord
- Alterations in cerebrospinal fluid (CSF) flow dynamics.
- Pulsatile wall movements in cysts can contribute to the deterioration of spinal cord symptoms.
- In secondary cases, a preceding inflammatory arachnoidopathy leads to extensive arachnoidal scarring, altered CSF dynamics, and potential vascular myelopathy due to disturbed spinal cord blood flow.
Aetiology
- Primary aetiology:
- Considered idiopathic or congenital
- Secondary aetiology:
- Presumed to result from a preceding inflammatory arachnoidopathy.
- The most common predisposing factors for secondary cysts include:
- Spinal trauma (40%)
- Iatrogenic causes, such as prior intradural spinal surgery or myelography (28%)
- Central nervous system hemorrhage (16%)
- Meningitis (16%)
Location
- Thoracic: 80%
- T5/T6 being the most common levels.
- Cervical: 15%
- Lumbar: 5%
Clinical Features
- Lesions typically present insidiously with progressive neurologic symptoms.
- The most common presenting symptoms include:
- Pain (68% overall; specifically thoracic back pain and low back pain)
- Sensory changes (58%)
- Weakness (52%)
- Gait difficulty (42%)
- Incontinence / bowel or bladder dysfunction (24%)
- The average duration of symptoms prior to initial presentation is approximately 26 to 28 months.
Radiology
- MRI
- focal buckling/compression of the spinal cord or a fluid structure with signal features similar to CSF.
- Cysts span an average of 3.5 vertebral levels (range 1–17 levels).
- CT myelography is also used in a subset of patients (24%).
- Position Relative to Cord:
- The majority of cysts are positioned dorsal to the spinal cord (90%), though some are ventral (9%) or lateral (1%).
- Associated Findings:
- A syrinx is observed in 35% of cases
- Focal cord signal changes are observed in approximately 28% to 32% of patients.
cyst. C and D, Case 18 with a T3-8 ventral arachnoid cyst.
Risk Factors
- Risk factors for developing secondary intradural arachnoid cysts include:
- Prior history of spinal trauma
- Previous spinal/intradural surgeries
- Past central nervous system hemorrhages
- Preceding central nervous system infections (such as meningitis)
- Patients with secondary cysts face higher risk factors for poor surgical outcomes, including increased risk of radiographic progression and need for repeat surgery.
Management
Conservative
- Indications
- Asymptomatic patients
- Symptoms do not correlate with the cyst's anatomical location
Surgery
- Indications
- Patients exhibiting neurological symptomatology correlated with spinal cord compression on imaging.
- Surgical Approaches:
- Open microsurgical approaches (e.g., wide thoracic laminectomies or laminotomies) using intraoperative ultrasound for localization.
- Cyst fenestration or excision is the primary surgical strategy in 98% of cases.
- Alternative interventions include cyst marsupialization or shunting procedures (e.g., cystoperitoneal or cystosubarachnoid shunts).
- Endoscopic fenestration strategies are also described, offering reduced operative time and shorter hospital stays.
Outcome
- Overall Results:
- Primary surgical management yields clinical improvement or complete resolution of preoperative symptoms in 86% of patients.
- Surgical fenestration resulted in a statistically significant reduction in thoracic back pain (P = .034).
- Primary vs. Secondary Outcomes:
- Primary Cysts: Have significantly better outcomes, with a 92% rate of symptomatic improvement or resolution.
- Secondary Cysts: Demonstrate significantly worse outcomes, with only 44% reporting symptomatic improvement and 56% experiencing no change.
- Complications & Recurrence:
- Secondary cysts have a significantly higher rate of radiographic progression compared to primary cysts (21% vs. 8%; P = .032).
- Secondary cysts require repeat surgical intervention more than twice as often as primary cysts (16% vs. 6%; P = .041).