Bilateral Retinoschisis: Prevalence and Clinical Patterns
Retinoschisis is highly likely to present bilaterally, with the vast majority of cases showing bilateral involvement, particularly in hereditary forms like X-linked retinoschisis (XLRS).
Prevalence of Bilateral Involvement
X-Linked Retinoschisis (XLRS)
- XLRS characteristically presents with bilateral disease in virtually all cases, as it is an inherited condition affecting both eyes symmetrically 1, 2, 3.
- Bilateral highly elevated bullous retinoschisis involving the macula has been documented in infants presenting with nystagmus and strabismus 1.
- Bilateral foveal retinoschisis is the typical presentation pattern, even when peripheral retinoschisis may be unilateral 2.
- Case reports consistently demonstrate bilateral foveoschisis with concurrent photoreceptor atrophy in both eyes 3.
Acquired (Senile) Retinoschisis
- Acquired retinoschisis also demonstrates a strong tendency toward bilateral presentation 4.
- In patients with nanophthalmos, bilateral acquired retinoschisis occurs consistently, suggesting shared underlying mechanisms 4.
- The average follow-up of 40 months in documented cases showed retinoschisis cavities remained stationary bilaterally 4.
Symptomatic Retinoschisis-Detachment
- Even in cases requiring treatment, bilateral involvement is documented, with one reported patient requiring intervention in both eyes 5.
- Retinal cryopexy, external drainage, and intravitreal air injection were needed bilaterally in one patient to achieve long-term macular reattachment 5.
Clinical Implications
Key Diagnostic Considerations
- When retinoschisis is identified in one eye, the fellow eye should be carefully examined, as bilateral disease is the rule rather than the exception 1, 2.
- OCT imaging showing neurosensory layer splitting and fluorescein angiography demonstrating absence of leakage help confirm bilateral involvement 2.
- Family history investigation is critical, as XLRS may affect other male family members even when not initially known 1.
Management Approach
- Most retinoschisis cavities remain stable and rarely extend to the posterior pole 2.
- Prophylactic argon laser treatment may be considered for peripheral retinoschisis to avoid potential retinal detachment risk 2.
- Surgical intervention is not usually indicated for bilateral bullous retinoschisis in infants, as spontaneous reattachment typically occurs with better visual prognosis than initial appearance suggests 1.
Important Caveats
- The presence of bilateral disease should prompt genetic counseling, particularly when XLRS is diagnosed 1.
- Hemorrhage within the schisis cavity or vitreous may be present bilaterally in approximately 80% of infantile XLRS cases 1.
- Hyperreflective material on OCT within the inner nuclear and outer plexiform layers serves as a distinctive bilateral feature of XLRS 3.