eMedicine Specialties > Ophthalmology > Retina

Retinopathy, Hemoglobinopathies: Treatment & Medication

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

Treatment

Medical Care

This condition usually is treated by or with an internist/hematologist.

Surgical Care

Since vitreous hemorrhage and retinal detachment account for most visual loss in hemoglobinopathies, the primary goal in treating proliferative sickle retinopathy is to minimize or eliminate neovascularization. Although treatments are not indicated or required for stages I and II, most advocate the treatment of sickle retinopathy for stage III.

The most widely used therapeutic modalities include laser retinal photocoagulation, retinal cryotherapy, and vitrectomy/membranectomy. The most effective therapeutic modality with minimal postoperative complications appears to be scatter laser retinal photocoagulation.

  • Laser photocoagulation
    • Laser retinal photocoagulation is the more commonly practiced therapeutic modality.
    • Although relatively safe, laser photocoagulation complications include preretinal hemorrhage, vitreous hemorrhage, retinal breaks, retinal detachment, and choroidal neovascularization.
    • Different techniques have been advocated in treating proliferative sickle retinopathy, including scatter photocoagulation and feeder vessel photocoagulation.
  • Scatter photocoagulation
    • Scatter photocoagulation appears to be the most efficacious (and therefore the preferred) treatment of sea fan lesions.
    • The desired photocoagulation endpoint is regression of extraretinal fibroneovascular tissue.
    • Localized scatter photocoagulation is effective in treating early proliferative changes, especially neovascular lesions that lie flat against the retina. Once neovascularization invades the vitreous, localized scatter photocoagulation appears to be less effective.
    • Circumferential scatter photocoagulation places laser burns over a retinal zone of one of the following: at least 3 disc diameter areas of the nonperfused retina, as outlined by fluorescein angiography, or the entire avascular retina, as determined by fluorescein angiography or estimated by the distribution of the occluded vessel.
    • Unlike feeder vessel coagulation, scatter photocoagulation is easier to perform, more effective, and safer. This technique reduces the incidence of vitreous hemorrhage.
    • However, in cases where scatter photocoagulation alone does not achieve the desired result (ie, regression of proliferative changes), feeder vessel photocoagulation may be used as an adjunct to induce infarction to the remaining sea fans.
    • Follow-up care is usually within 1 week after laser surgery to rule out retinal detachment from contracture of the neovascular membrane after laser treatment.
    • After the first follow-up visit, monthly follow-up visits are advocated to confirm and monitor the regression of the neovascularization.
    • Insufficient treatment indicated by failure or arrest of the regression of the neovascularization requires further laser treatments at the time of follow-up care.
    • New or recurrent neovascularization in the treated eye is treated in the similar manner.
  • Feeder vessel photocoagulation
    • Obliterating feeder vessels by retinal photocoagulation has been used to cause infarction of peripheral neovascular beds.
    • This technique has been shown to manage proliferative sickle retinopathy effectively, especially in cases where neovascularization has persisted after extensive scatter photocoagulation treatment.
    • Feeder vessel photocoagulation frequently is complicated by the following: vitreous hemorrhage, retinal detachments, choroidal ischemia, choroidal neovascularization, subretinal hemorrhage and/or fibrosis, or macular pucker and hole formation.
    • To reduce complications, the feeder vessel technique has been modified into 2 sessions, permitting reduction in laser power. On initial treatment, laser burns are low intensity; upon follow-up, a second set of low-intensity burns are superimposed on the initial ones.
    • The method has been proposed to decrease the incidence of the Bruch membrane penetration, thereby decreasing the chance for choroidal neovascularization.
    • Scatter photocoagulation is the preferred technique.
    • Feeder vessel photocoagulation seldom is used because of its high complication rate. When used, feeder vessel photocoagulation is usually an adjunct to scatter photocoagulation.
    • Regular follow-up care is needed to detect and treat complications and new neovascularization.
  • Retinal cryotherapy
    • In 1982, Hanscom demonstrated that retinal cryotherapy is useful in treating peripheral retinal ischemia as opposed to directly obliterating these neovascular beds.1 This methodology of "indirect" obliteration of the neovascular bed showed complete regression of abnormal vessels with minimal complications.
    • Cryotherapy often is limited to cases with cloudy ocular media.
    • Single freeze-thaw and triple freeze-thaw have been advocated in treating PSR. However, it appears that triple freeze-thaw is associated with a high rate of complications (eg, proliferative vitreoretinopathy [PVR]) and, therefore, is not recommended.
  • Vitrectomy
    • Indicated in cases of nonresolving vitreous hemorrhage and retinal detachment (stages IV and V).
    • One of the more serious complications that is associated with vitreoretinal surgery is anterior segment ischemia. Other complications include sickling crisis, optic nerve, and macula infarctions.
    • This procedure relieves the internal tractional forces that act on the retina and facilitate retinal photocoagulation.
    • Rhegmatogenous retinal detachment in sickle cell disease usually is secondary to tractional membrane. Usually requiring pars plana vitrectomy, it seldom is treated with scleral buckling alone.
    • The following measures can decrease complications (ie, anterior segment ischemia):
      • Preoperative partial exchange transfusions (rarely required) increase the amount of normal hemoglobin Hb A, thereby increasing the O2 -carrying capacity.
      • Administer local anesthesia, stellate ganglion block, and papaverine administration.
      • Provide cycloplegics (parasympathomimetics only).
      • Decrease IOP.
      • Provide supplemental oxygen therapy.
      • IOP should be kept lower than 25 mm Hg preoperatively, intraoperatively, and postoperatively.

Consultations

  • Retina specialist
  • Internist/hematologist
  • Geneticist

Medication

See Special Concerns and Surgical Care.

Hemostatic agents

Reduce the incidence of rebleeds in the setting of hyphema.


Aminocaproic acid (Amicar)

Inhibits fibrinolysis via inhibition of plasminogen activator substances; to a lesser degree, through antiplasmin activity.
Main problem is that the thrombi that form during treatment are not lysed, and effectiveness is uncertain.

Adult

Oral syrup: 50-100 mg/kg PO q4h for 5 d
Recommended dose: 50 mg/kg PO q4h to a maximum 5 g q4h; better tolerated with fewer severe adverse effects

Pediatric

Not established

Coadministration with estrogens may cause increase in clotting factors, leading to a hypercoagulable state

Documented hypersensitivity; evidence of active intravascular clotting process; since aminocaproic acid can be fatal in patients with DIC, differentiating between hyperfibrinolysis and DIC is important

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Do not administer unless a definite diagnosis of hyperfibrinolysis has been made; caution in cardiac, hepatic, or renal disease

Corticosteroids

Reduce the incidence of rebleeds in the setting of hyphema.


Prednisolone 1% (Pred Forte)

Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing increased capillary permeability.

Adult

1 gtt 4-6 times/d; may adjust dose depending on severity of anterior chamber inflammation

Pediatric

Not established

Documented hypersensitivity; viral, fungal, or tubercular infections

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in hypertension; known to cause cataract formation with long-term use; suspect fungal invasion in any persistent corneal ulceration where a corticosteroid has been used or is in use (obtain fungal cultures when appropriate)


Prednisone (Deltasone, Orasone, Meticorten)

May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.

Adult

20 mg PO bid (40 mg/d) for 5 d

Pediatric

Not established

Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use

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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References

  1. Hanscom TA. Indirect treatment of peripheral retinal neovascularization. Am J Ophthalmol. Jan 1982;93(1):88-91. [Medline].

  2. Nasrullah A, Kerr NC. Sickle cell trait as a risk factor for secondary hemorrhage in children with traumatic hyphema. Am J Ophthalmol. Jun 1997;123(6):783-90. [Medline].

  3. Acheson RW, Ford SM, Maude GH, et al. Iris atrophy in sickle cell disease. Br J Ophthalmol. Jul 1986;70(7):516-21. [Medline].

  4. Aessopos A, Voskaridou E, Kavouklis E, et al. Angioid streaks in sickle-thalassemia. Am J Ophthalmol. May 15 1994;117(5):589-92. [Medline].

  5. Andreoli TE, et al. Disorder of red cells. In: Cecil's Essentials of Medicine. 4th ed. 1997:383-7.

  6. Asdourian GK. Sickle cell retinopathy. In: Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology. Vol 2. 1994:1006-1018.

  7. Beutler E. Disorders of hemoglobin. In: Fauci AS, et al, eds. Harrison's Principle of Internal Medicine. 14th ed. 1998:645-652.

  8. Bunn HF. Pathogenesis and treatment of sickle cell disease. N Engl J Med. Sep 11 1997;337(11):762-9. [Medline].

  9. Charache S. Eye disease in sickling disorders. Hematol Oncol Clin North Am. Dec 1996;10(6):1357-62. [Medline].

  10. Cohen SB, van Houten PA. Hemoglobinopathies. In: Ryan SJ, et al, eds. Retina. 2nd ed. 1994:1465-1472.

  11. Eagle RC. Retina-sickle hemoglobinopathy. In: Eye Pathology: An Atlas and Basic Text. 1999:147, 153-4.

  12. Farber MD, Fiscella R, Goldberg MF. Aminocaproic acid versus prednisone for the treatment of traumatic hyphema. A randomized clinical trial. Ophthalmology. Mar 1991;98(3):279-86. [Medline].

  13. Farber MD, Jampol LM, Fox P, et al. A randomized clinical trial of scatter photocoagulation of proliferative sickle cell retinopathy. Arch Ophthalmol. Mar 1991;109(3):363-7. [Medline].

  14. Fekrat S, Lutty G, Goldberg M. Retina-Vitreous Macula. In: Guyer D, et al. Hemoglobinopathies. Vol 1. 1999:438-458.

  15. Fox PD, Dunn DT, Morris JS, et al. Risk factors for proliferative sickle retinopathy. Br J Ophthalmol. Mar 1990;74(3):172-6. [Medline].

  16. Gagliano DA, Goldberg MF. The evolution of salmon-patch hemorrhages in sickle cell retinopathy. Arch Ophthalmol. Dec 1989;107(12):1814-5. [Medline].

  17. Goldbert MF. Duane's Clinical Ophthalmology. In: Tasman W and Jaeger EA. Sickle Cell Retinopathy. Vol 3. 1990:1-45.

  18. Green WR. Retina: Systemic Diseases with Retinal Involvement. In: Spencer WH. Ophthalmic Pathology: An Atlas and Textbook. Vol 2. 1996:1155-1160.

  19. Jacobson MS, Gagliano DA, Cohen SB, et al. A randomized clinical trial of feeder vessel photocoagulation of sickle cell retinopathy. A long-term follow-up. Ophthalmology. May 1991;98(5):581-5. [Medline].

  20. Jampol LM. Arteriolar occlusive diseases of the macula. Ophthalmology. May 1983;90(5):534-9. [Medline].

  21. Jampol LM. Vitrectomy surgery in proliferative sickle retinopathy. Am J Ophthalmol. Nov 15 1987;104(5):553-4. [Medline].

  22. Jampol LM, Green JL Jr, Goldberg MF, et al. An update on vitrectomy surgery and retinal detachment repair in sickle cell disease. Arch Ophthalmol. Apr 1982;100(4):591-3. [Medline].

  23. Kark JA, Posey DM, Schumacher HR, et al. Sickle-cell trait as a risk factor for sudden death in physical training. N Engl J Med. Sep 24 1987;317(13):781-7. [Medline].

  24. Kimmel AS, Magargal LE, Stephens RF, et al. Peripheral circumferential retinal scatter photocoagulation for the treatment of proliferative sickle retinopathy. An update. Ophthalmology. Nov 1986;93(11):1429-34. [Medline].

  25. Liebmann JM. Management of sickle cell disease and hyphema. J Glaucoma. Aug 1996;5(4):271-5. [Medline].

  26. Lubin BH. Sickle cell disease and the endothelium. N Engl J Med. Nov 27 1997;337(22):1623-5. [Medline].

  27. McLeod DS, Goldberg MF, Lutty GA. Dual-perspective analysis of vascular formations in sickle cell retinopathy. Arch Ophthalmol. Sep 1993;111(9):1234-45. [Medline].

  28. McLeod DS, Merges C, Fukushima A, et al. Histopathologic features of neovascularization in sickle cell retinopathy. Am J Ophthalmol. Oct 1997;124(4):455-72. [Medline].

  29. Morgan CM, D'Amico DJ. Vitrectomy surgery in proliferative sickle retinopathy. Am J Ophthalmol. Aug 15 1987;104(2):133-8. [Medline].

  30. Palmer DJ, Goldberg MF, Frenkel M, et al. A comparison of two dose regimens of epsilon aminocaproic acid in the prevention and management of secondary traumatic hyphemas. Ophthalmology. Jan 1986;93(1):102-8. [Medline].

  31. Pulido JS, Flynn HW Jr, Clarkson JG, et al. Pars plana vitrectomy in the management of complications of proliferative sickle retinopathy. Arch Ophthalmol. Nov 1988;106(11):1553-7. [Medline].

  32. Raichand M, Dizon RV, Nagpal KC, et al. Macular holes associated with proliferative sickle cell retinopathy. Arch Ophthalmol. Sep 1978;96(9):1592-6. [Medline].

  33. Rednam KR, Jampol LM, Goldberg MF. Scatter retinal photocoagulation for proliferative sickle cell retinopathy. Am J Ophthalmol. May 1982;93(5):594-9. [Medline].

  34. Serjeant GR. Sickle-cell disease. Lancet. Sep 6 1997;350(9079):725-30. [Medline].

  35. Siegel D. Diagnosis and treatment of sickle cell retinopathy. J Am Optom Assoc. Nov 1988;59(11):885-8. [Medline].

  36. Slavin ML, Barondes MJ. Ischemic optic neuropathy in sickle cell disease. Am J Ophthalmol. Feb 15 1988;105(2):212-3. [Medline].

  37. Stryer L. Oxygen-transporting proteins: myoglobin and hemoglobin. In: Biochemistry. 3rd ed. 1988:143-173.

  38. To KW, Nadel AJ. Ophthalmologic complications in hemoglobinopathies. Hematol Oncol Clin North Am. Jun 1991;5(3):535-48. [Medline].

  39. van Meurs JC. Evolution of a retinal hemorrhage in a patient with sickle cell-hemoglobin C disease. Arch Ophthalmol. Aug 1995;113(8):1074-5. [Medline].

  40. Yanoff M, Fine BS. Sickle cell disease. In: Ocular Pathology. 4th ed. 1996:377-8.

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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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