eMedicine Specialties > Ophthalmology > Retina

Retinopathy, Hemoglobinopathies: Follow-up

Author: Brian A Phillpotts, MD, Former Vitreo-Retinal Service Director, Former Program Director, Clinical Assistant Professor, Department of Ophthalmology, Howard University College of Medicine
Coauthor(s): Hon-Vu Q Duong, MD, Ophthalmologist, Department of Ophthalmology, Westfield Eye Center; Michael J Shapiro, MD, Associate Professor, Vitreoretinal Service, Director, Eye Trauma Services, Department of Ophthalmology, University of Illinois at Chicago; Vitreoretinal Consultant, Pediatric Genetics and Birth Defects Clinic; Oswaldo Castro, MD, Director of Center for Sickle Cell Disease, Professor, Department of Internal Medicine, Howard University; Richard G Fiscella, RPh, MPH, Clinical Associate Professor, Departments of Pharmacy and Ophthalmology, University of Illinois at Chicago
Contributor Information and Disclosures

Updated: Sep 8, 2009

Follow-up

Further Inpatient Care

  • Inpatient care often is not needed, except for noncompliant patients in sickle cell crisis or for patients with a hyphema who are unable to comply with follow-up visits.

Further Outpatient Care

  • Monitor medication dose and adverse effects.
  • Enroll patient in local sickle cell clinic.
  • Ophthalmologic follow-up care is determined by the proliferative stage.
    • Stages I and II - Follow-up care every 6-12 months
    • Stages III and IV
      • Follow-up care is usually within 1 week after laser surgery to rule out retinal detachment.
      • After the first follow-up visit, monthly follow-up visits are advocated to confirm and monitor the regression of the neovascularization.
      • Insufficient treatment requires further laser treatments at the time of follow-up care.
    • Stage V - Per retinal specialist consultation

Complications

Prognosis

  • The prognosis is fair to good if consistent follow-up care is maintained with both an internist/hematologist and an ophthalmologist.

Patient Education

  • Patients with sickle cell disease should be well informed of their current and potential long-term complications.
  • Encourage patients to enroll at a local or regional sickle cell clinic.
  • Provide patients with information regarding a local support group.
  • Encourage parents to seek genetic counseling prior to starting a family.

Miscellaneous

Medicolegal Pitfalls

  • Early detection and treatment may help decrease the retinal complications.

Special Concerns

  • Blood transfusion
    • In the past, exchange transfusion was advocated prior to scleral buckling and/or vitrectomy. Today, this is not universally performed.
    • Consider the risk-to-benefit ratio, not only the possibility of contracting hepatitis secondary to transfusion but also transfusion reaction, septicemia, and AIDS.
    • Consult a hematologist.
    • The patient requires adequate preoperative, intraoperative, and postoperative hydration and correction of acidosis.
    • Transfusions may increase the likelihood of a clinically significant IOP elevation in the setting of a hyphema.
  • Hyphema
    • The presence of hyphema in sickle cell disease should be managed aggressively because patients with sickle cell disease tend to have a poorer prognosis with the same degree of hyphema than patients without sickle cell disease.
    • A relatively small hyphema may lead to severely elevated IOP due to clogging of the trabecular meshwork. Even patients with sickle trait are susceptible to this complication.
    • Patients with sickle cell disease are predisposed to optic nerve damage and/or central retinal artery occlusion with mild increases in IOP.
    • Closely monitor and treat IOP, even when pressure is in the mid 20s (mm Hg).
    • All black patients with hyphema, even those without a past history of sickle cell disease or trait, should be tested for the disease or trait.
    • Rebleeding is a potentially devastating complication, usually occurring 2-6 days after the initial bleed. The chance of rebleeding has been reported as high as 64%.
    • Nasrullah et al demonstrated secondary hemorrhage in 9 of 14 patients.2 This was significantly (P >0.005) different from the 0% rate in 57 eyes of African American sickle cell trait-negative and white children.
    • Rebleeding can be treated prophylactically with oral and/or topical steroids and/or aminocaproic acid (Amicar). Therapy is aimed at keeping the IOP low without exacerbating hypoxia or acidosis.
    • Maintain an IOP lower than 25 mm Hg.
    • Paracentesis or anterior chamber washout is advised for increased IOP.
    • When to surgically intervene should be determined on a case-by-case basis. Be sure to consider the following:
      • Length of time between onset of symptoms and presentation
      • IOP at presentation (ie, 26-50 mm Hg)
      • Prior history of optic nerve disease and response to medical therapy during a previously similar episode
    • Prognosis is good if the IOP is controlled within the first 24 hours.
  • Hyphema therapy
    • Topical beta-adrenergic antagonists are the mainstay of therapy for IOP control.
    • Oral steroids, topical steroids, and/or aminocaproic acid (Amicar) all reduce the incidence of rebleeds. This is not as well documented in sickle cell hyphemas because many studies exclude sickle hyphemas.
    • Avoid topical epinephrine, whenever possible, because it causes vasoconstriction and exacerbates the sickling process.
    • If possible, avoid miotic agents that may increase inflammation.
    • Avoid hyperosmotic agents because they may increase hemoconcentration and viscosity.
    • Avoid carbonic anhydrase inhibitors that may lead to hemoconcentration, systemic acidosis, and an elevated level of ascorbic acid; they may cause further sickling. Methazolamide is theoretically preferable to acetazolamide in such patients because intraocular and systemic acidoses are lower. Some clinicians believe that topical dorzolamide is also reasonable before systemic carbonic anhydrase inhibitors.
    • Bed rest with the head elevated at least 45o is recommended.
    • Frequent follow-up care or hospitalization is recommended.
  • Scleral buckling
    • Scleral buckling seldom is used without combined vitrectomy because rhegmatogenous retinal detachment in sickle cell disease is usually secondary to tractional membranes.
    • The following measures decrease complications (ie, anterior segment ischemia):
      • Avoid disinsertion and/or aggressive manipulation of the recti muscles.
      • Preoperative partial exchange transfusion increases the amount of normal hemoglobin (Hb A), thereby increasing the Hb-O2 carrying capacity.
      • Provide intraoperative and postoperative fluids and oxygen therapy.
      • Administer cycloplegics (parasympathomimetics only).
      • Administer local anesthesia, stellate ganglion block, and papaverine.
      • Decrease IOP.
 


More on Retinopathy, Hemoglobinopathies

Overview: Retinopathy, Hemoglobinopathies
Differential Diagnoses & Workup: Retinopathy, Hemoglobinopathies
Treatment & Medication: Retinopathy, Hemoglobinopathies
Follow-up: Retinopathy, Hemoglobinopathies
References
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Further Reading

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Keywords

hemoglobin retinopathy, proliferative retinopathy, sickle cell disease, sickle cell hemoglobinopathy, sickle cell retinopathy, homozygous sickle cell disease, sickle cell C disease, sickle cell-thalassemia disease, SS disease, SC disease, S-Thal disease

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Contributor Information and Disclosures

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, MD is a member of the following medical societies: American Academy of Ophthalmology, American Diabetes Association, American Medical Association, and National Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Hon-Vu Q Duong, MD, Ophthalmologist, Department of Ophthalmology, Westfield Eye Center
Hon-Vu Q Duong, MD is a member of the following medical societies: American Academy of Ophthalmology
Disclosure: Nothing to disclose.

Michael J Shapiro, MD, Associate Professor, Vitreoretinal Service, Director, Eye Trauma Services, Department of Ophthalmology, University of Illinois at Chicago; Vitreoretinal Consultant, Pediatric Genetics and Birth Defects Clinic
Michael J Shapiro, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Oswaldo Castro, MD, Director of Center for Sickle Cell Disease, Professor, Department of Internal Medicine, Howard University
Disclosure: Nothing to disclose.

Richard G Fiscella, RPh, MPH, Clinical Associate Professor, Departments of Pharmacy and Ophthalmology, University of Illinois at Chicago
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

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Steve Charles, MD, Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine; Adjunct Professor of Ophthalmology, Columbia College of Physicians & Surgeons; Clinical Professor Ophthalmology, Chinese University of Hong Kong
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; OptiMedica Ownership interest 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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