Sunir Garg

BASICS

DESCRIPTION

Age-related (senile) retinoschisis (SR) is an acquired condition characterized by splitting of the retina into 2 separate layers.

EPIDEMIOLOGY

Incidence

Not clearly defined.

Prevalence

• Ranges from 1.65–7% among persons older than 40 years (1)

• Generally equal in male and females

RISK FACTORS

• Preexisting peripheral retinal cystoid degeneration

• Hyperopia

Genetics

Unknown.

GENERAL PREVENTION

None.

PATHOPHYSIOLOGY

• Vision is not significantly affected when the schisis cavity remains in the periphery

• Visual loss occurs predominantly in 2 circumstances:

– The area of retinoschisis extends posteriorly toward, or into, the macula (occurs in 3% of cases).

– An associated rhegmatogenous retinal detachment (RRD) develops.

ETIOLOGY

• Generally develops as a consolidation of intraretinal cysts in an area of peripheral cystoid degeneration

– Begins anterior at the ora serrata and extends posteriorly

– The splitting most often occurs in the outer plexiform layer

COMMONLY ASSOCIATED CONDITIONS

• Hyperopia

• An RRD may develop secondarily.

DIAGNOSIS

HISTORY

• Age-related retinoschisis is usually asymptomatic and is detected on routine examination.

• Some patients may have peripheral visual field loss, flashes and floaters, and central vision loss.

PHYSICAL EXAM

• Dilated funduscopic examination shows:

– A smooth dome-like elevation of the retina that remains fixed in position and does not undulate.

– Often bilateral (one-fifth to half of the cases) but can be asymmetric.

– Most commonly located inferotemporally

– Peripheral cystoid degeneration is seen anterior to the schisis cavity.

– Inner and/or outer retinal holes may be present.

– Fine, irregular white dots may be seen on the retinal surface.

– Sclerotic retinal vessels in the area of schisis

– The inner layer does not collapse on scleral depression.

DIAGNOSTIC TESTS & INTERPRETATION

Imaging

Initial approach

Photographs to document posterior extent of the area of retinoschisis may be useful for comparison on follow up.

Follow-up & special considerations

Asymptomatic and peripheral age-related retinoschisis can be followed every 2–3 years.

Diagnostic Procedures/Other

Visual field testing reveals an absolute scotoma in the areas of schisis.

Pathological Findings

• There are 2 main types of acquired retinoschisis:

– Typical degenerative retinoschisis

– The more common subtype in which the splitting occurs in the outer plexiform layer

– Reticular degenerative retinoschisis is characterized by cystoid spaces and splitting within the nerve fiber layer

Breaks in the outer retina occur in 11–24% of cases, and may lead to rhegmatogenous retinal detachment.

DIFFERENTIAL DIAGNOSIS

• Rhegmatogenous retinal detachment. This is the most important condition to differentiate from age-related retinoschisis. Chronic rhegmatogenous retinal detachments also may appear smooth and domed. The presence of pigment in the vitreous, or a pigmented demarcation line suggests rhegmatogenous detachment.

– In some cases, differentiating these 2 conditions can be difficult. Two other ways to diagnose them are: 1) Laser retinopexy to the cavity will cause a burn in retinoschisis, but not in a retinal detachment, and 2) Optical coherence tomography (OCT) at the edge of the schisis cavity will show a complete neurosensory retinal detachment in a rhegmatogenous detachment, and a splitting of the neurosensory retina with apposition of the photoreceptors and retinal pigment epithelium in cases of retinoschisis.

TREATMENT

SURGERY/OTHER PROCEDURES

• The majority of cases only need observation as they progress slowly, if at all (1).

• For SR that extends into the posterior pole, demarcation with cryotherapy or laser retinopexy can be considered (2). However, most of these may be observed as rarely does age-related retinoschisis reach the macula, and cryotherapy may increase formation of retinal detachment.

• If a frank RRD develops, surgical repair with scleral buckle or pars plana vitrectomy is usually necessary (1,2).

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

• Dilated fundus examination every 1–3 years for uncomplicated SR without holes

• Patients with multiple retinal breaks or significant posterior extension should be followed more closely.

– Should a retinal detachment develop or should there be a question of an associated retinal detachment, prompt referral to a retinal specialist is advised.

Patient Monitoring

Patients should be counseled to return to the ophthalmologist if any changes in vision occur.

PROGNOSIS

• Vision is typically unaffected or only minimally affected if the area of retinoschisis remains peripheral.

• The prognosis is significantly worse should a retinal detachment develop.

COMPLICATIONS

• RRD

• Posterior extension of the retinoschisis