eMedicine Specialties > Ophthalmology > Retina

Retinoschisis, Juvenile: Treatment & Medication

Author: Mi-Kyoung Song, MD, Vitreoretinal Surgeon, Eye Associates of New Mexico
Coauthor(s): Kent W Small, MD, Director/President, Macular and Retinal Disease Center; President, Molecular Insight LLC; Consulting Surgeon, Glendale Eye Medical Group
Contributor Information and Disclosures

Updated: Nov 26, 2007

Treatment

Medical Care

No treatment is available to halt the natural progression of schisis formation. However, the use of topical dorzolamide and oral acetazolamide in reducing cystic spaces and foveal thickness with a concomitant increase in visual acuity has been reported.  Currently, gene therapy research on an Rs1h-deficient mouse model of human retinoschisis has shown restoration of expression of retinoschisin protein in photoreceptors and normal ERG configuration. Gene therapy may be a viable therapeutic option in the future.

Treatment of peripheral schisis cavities is generally not indicated because they can typically regress. Laser photocoagulation has been performed to flatten peripheral schisis cavities and to reduce the risk of retinal detachment; however, in many cases, it resulted in retinal detachment. Because of the nonprogressive nature of congenital retinoschisis, prophylactic photocoagulation may not be warranted. Surgical attempts to flatten peripheral schisis cavities in the absence of retinal detachment have not been shown to be beneficial. Therefore, a conservative approach is advocated.

Amblyopia prevention therapy is indicated in cases of hypermetropia or severe retinoschisis or following surgical intervention for vitreous hemorrhage or retinal detachment.

Surgical Care

Surgery can be performed for the management of vision threatening vitreous hemorrhage and retinal detachment.

  • Most patients who develop vitreous or intraschisis hemorrhage do not require treatment. Surgery is indicated if the hemorrhage is dense or if a blood-filled schisis cavity overhangs the macula. Cauterization of the bleeding vessels may be performed at the time of a vitrectomy.
  • Three types of retinal detachments are associated with congenital retinoschisis. Retinal detachment can be caused by a break in both the inner layer and the outer layer of the retinoschisis. Retinal detachment can be caused by a full thickness retinal hole outside of the schisis cavity. The third type of retinal detachment involves a traction detachment via fibrovascular tissue.
  • Retinal detachment repair in congenital retinoschisis can be technically difficult. To repair a retinal detachment associated with a schisis cavity, care must be taken to remove as much of the vitreous attached to the schisis cavity as possible to relieve any tangential traction. Then, a retinotomy site can be made on the schisis cavity overlying the break in the outer retinal layer such that the schisis cavity and the outer retina can be flattened. An inner wall retinectomy is recommended when the cortical vitreous or preretinal fibrosis is densely adherent. Relief of vitreoretinal traction is believed to prevent the rebleeding of unsupported vessels. Internal tamponade can be achieved with a long-acting gas or with silicone oil. Silicone oil can be removed in a few weeks.

Consultations

  • Patients with XJR should be referred to a vitreoretinal specialist for careful examination and follow-up visit.
  • Family members should be examined to rule out any members who remain undiagnosed.
  • Retinal detachments and vitreous hemorrhages associated with this disease should be managed surgically to preserve vision.
  • The gene associated with XJR has been identified. Genetic counseling should be offered to all patients with XJR as well as to potential carriers and family members.

Diet

No reports on any significance of diet with XJR exist.

Activity

No specific restrictions on activity for patients with XJR exist. However, words of caution should be given to limit activities associated with significant impact to the head.

More on Retinoschisis, Juvenile

Overview: Retinoschisis, Juvenile
Differential Diagnoses & Workup: Retinoschisis, Juvenile
Treatment & Medication: Retinoschisis, Juvenile
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Multimedia: Retinoschisis, Juvenile
References
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References

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Further Reading

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Keywords

X-linked juvenile retinoschisis, XJR, congenital cystic retinal detachment, congenital vascular veils in the vitreous, vitreous veils

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Contributor Information and Disclosures

Author

Mi-Kyoung Song, MD, Vitreoretinal Surgeon, Eye Associates of New Mexico
Mi-Kyoung Song, MD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Retina Specialists, and New Mexico Medical Society
Disclosure: Nothing to disclose.

Coauthor(s)

Kent W Small, MD, Director/President, Macular and Retinal Disease Center; President, Molecular Insight LLC; Consulting Surgeon, Glendale Eye Medical Group
Kent W Small, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American College of Physician Executives, American Medical Association, American Medical Informatics Association, American Ophthalmological Society, American Society of Human Genetics, Association for Research in Vision and Ophthalmology, California Medical Association, Macula Society, Pan-American Association of Ophthalmology, and Retina Society
Disclosure: Nothing to disclose.

Medical Editor

Russell P Jayne, MD, Consulting Vitreoretinal Surgeon, The Retina Center at Las Vegas
Russell P Jayne, MD is a member of the following medical societies: American Medical Association, American Society of Cataract and Refractive Surgery, and American Society of Retina Specialists
Disclosure: Nothing to disclose.

Pharmacy Editor

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
Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Managing Editor

Steve Charles, MD, Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine
Steve Charles, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Club Jules Gonin, Macula Society, and Retina Society
Disclosure: Alcon Laboratories Consulting fee Consulting

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
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