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Chorioretinopathy, Central Serous

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Retinoschisis, Juvenile




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Author: Brian A Phillpotts, MD, Former Vitreo-Retinal Service Director, Former Program Director, Clinical Assistant Professor, Department of Ophthalmology, Howard University College of Medicine

Brian A Phillpotts is a member of the following medical societies: American Academy of Ophthalmology, American Diabetes Association, American Medical Association, and National Medical Association

Coauthor(s): Ramu Gounder, MD, Staff Physician, Department of Ophthalmology, Howard University Hospital

Editors: Russell P Jayne, MD, Consulting Vitreoretinal Surgeon, The Retina Center at Las Vegas; Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles; Steve Charles, MD, Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine; Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri; Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Author and Editor Disclosure

Synonyms and related keywords: senile retinoschisis, SR, degenerative retinoschisis, acquired retinoschisis, macrocyst, giant cyst of the retina, retinal detachment

Background

Senile retinoschisis (SR) is a primary, acquired, gradual splitting of the retina into 2 distinct layers. This condition is not senile or age related, as it has been reported in patients in the third decade (20-30 y).

Pathophysiology

SR develops from the coalescence of intraretinal microcysts located in an area of peripheral cystoid degeneration near the ora serrata and extends posteriorly and circumferentially. This process leads to the splitting of the retina at the outer plexiform layer or, less commonly, at the inner nuclear layer.

Frequency

United States

The prevalence is high in the normal population (4-22% of individuals older than 40 y).

Mortality/Morbidity

Often, no visual changes occur, but retinal detachment may occur in a small percentage of patients, leading to visual field defects and/or decreased visual acuity.

  • Visual field defects may manifest as the schisis cavity extends posteriorly.
  • The schisis cavity may remain stable or slowly progress over several years.
  • Retinal detachment may result after the development of retinal holes in both the inner layer and outer layer of the schisis cavity or in the outer layer alone (16%).

Sex

The occurrence of SR is approximately equal in males and females.

Age

SR is more common in patients older than 30 years, but it has been reported in patients younger than 30 years.



History

In the initial stages, patients are generally asymptomatic; however, in the advanced stage of the disease, patients may complain of photopsia, floaters, and visual field loss.

Physical

Examination is completed by using indirect ophthalmoscopy with scleral depression. Goldmann lens/slit lamp biomicroscopy examination also may be performed. Examination reveals the following findings:

  • A retinal elevation is present in the middle to the peripheral part of the retina, often in the lower temporal quadrant.
  • The elevated retinal surface is usually smooth, without folds/undulation, and may contain retinal blood vessels.
  • The inner layer may be transparent and difficult to visualize.
  • In advanced cases, the leading edge of the splitting retinal layers usually elevate acutely, resulting in a bullous cystic cavity.
  • The position of the schisis cavity is constant in shape, elevation, and position; it changes with head position without fluid shift.
  • Scleral depression fails to collapse the inner retinal layer.
  • Scleral depression reveals white-without-pressure of the outer retinal layer.
  • The retinoschisis rarely extends into the macular region.
  • SR is not associated with operculated or horseshoe-shaped retinal tears.
  • Retinal detachments secondary to outer retinal layer holes alone tend to be shallow, small, and located around SR.
  • Retinal detachments secondary to inner retinal layer holes alone or combined with outer layer holes tend to be elevated and large to total detachments.
  • SR is commonly bilateral but not necessarily symmetrical.
  • Outer layer holes are usually large, relatively limited in number, and obvious; inner layer holes are usually small, numerous, and not so obvious.

Causes

Preexisting peripheral cystoid degeneration



Chorioretinopathy, Central Serous
Retinal Detachment, Rhegmatogenous
Retinoschisis, Juvenile

Other Problems to be Considered

Vitreous veil
Vitreous opacity
Vitreous base avulsion



Other Tests

  • Fundus drawings
  • Visual field plotting (sharp absolute scotoma in SR; sloped relative to absolute in retinal detachment)
  • Low-power retinal laser test spot to rule out subretinal fluid, ie, retinal detachment (in very difficult cases)

Histologic Findings

Two histologic types of SR have been identified.

Flat type also is termed typical degenerative retinoschisis. Characteristics of this type include splitting at the outer plexiform layer, usually confined anterior to the equator, and not associated with holes.

Bullous type also is termed reticular degenerative retinoschisis. Characteristics of this type include splitting in the nerve fiber layer, often extends posterior to the equator, and often has outer layer holes that may lead to retinal detachment when associated with inner layer holes.



Surgical Care

Surgery does not reestablish neuronal integrity of the split retina; therefore, it does not improve visual loss secondary to SR. Rarely, spontaneous resolution has been reported.

  • Surgery is undertaken to prevent retinal detachment, when inner and outer retinal holes are found without retinal detachment.
  • Surgery is performed if retinoschisis is associated with a retinal detachment.
  • For SR extending into the posterior pole, the following 2 procedures can be performed:
    • Demarcation of the advancing retinoschisis edge with photocoagulation and/or cryotherapy
    • Application of photocoagulation and/or cryotherapy to the entire outer layer with or without subretinal fluid (SRF) drainage to collapse the inner layer of the retinoschisis

Consultations

Vitreoretinal specialist



Further Outpatient Care

  • Patients should have annual follow-up examinations for retinoschisis that is flat, without breaks, and anterior to the equator.
  • Patients should receive follow-up care every 3-6 months for retinoschisis with breaks or with extension posterior to the equator; if patients remain stable, annual visits thereafter are indicated.
  • More frequent follow-up care is required in patients with symptoms of photopsia and floaters.

Complications

  • A low clinical risk exists of retinal detachment or extension into the posterior pole, leading to an absolute field defect.

Prognosis

  • In a large study conducted by Byer, no change in visual acuity occurred over 9 years (from extension of SR or retinal detachment).
    • Generally, SR does not progress. About 3.2-13.5% of cases progress within 9-10 years of diagnosis.
    • Even with anatomic correction, the visual field deficits are permanent.

Patient Education

  • Patients should be informed about symptoms of retinal detachment.



Medical/Legal Pitfalls

  • Retinoschisis needs to be differentiated from a retinal detachment during every follow-up visit. Misdiagnosis of a retinal detachment may result in substantial visual morbidity.

Special Concerns

  • Progression of the schisis cavity border and/or height usually is caused by the extension of the retinal splitting. However, it may be secondary to new inner and/or outer retinal layer hole formation, warranting a thorough examination with scleral depression to rule out subtle early retinal detachment.
  • When repairing a retinal detachment or retinal dialysis that is secondary to an acquired retinoschisis (with inner and outer retinal holes), care must be taken to close the holes in both layers. These holes may not share the same location (ie, the inner layer hole does not overlie the outer layer hole).
  • Retinal detachment is more likely with the following:
    • Multiple holes rather than a single outer hole
    • Multiple superior and posterior inner holes compared with few inferior and anterior holes



  • Byer NE. Long-term natural history study of senile retinoschisis with implications for management. Ophthalmology. Sep 1986;93(9):1127-37. [Medline].
  • Guyer DR, Chang S, Green WR, et al. Vitreoretinal precursors of retinal detachment. In: Lampert R, ed. Retina-Vitreous-Macula. Vol 1. Harcourt Brace & Company;1999:63-77.
  • Hirose T. Retinoschisis. In: Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology. Vol 2. 1996;1071-1084.
  • Sigelman J. Retinal Diseases: Pathogenesis, Laser Therapy, and Surgery. 1984;7:227-241.
  • Tasman W. Disorders of the Peripheral Fundus. 1980;172-176.
  • Vaughan D. General Ophthalmology. 1992;203.
  • Wu G. Retina: The Fundamentals. Harcourt Brace & Company;1995:176-178.

Retinoschisis, Senile excerpt

Article Last Updated: Feb 13, 2007