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Ophthalmologic Manifestations of Kaposi Sarcoma Clinical Presentation

  • Author: Jacqueline Freudenthal, MD; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Jan 08, 2015
 

History

Identify risk factors for Kaposi sarcoma. The clinician should ask about the following:

  • Demographics
  • Immune status
  • Previous skin lesions
  • Previous treatment for Kaposi sarcoma
  • History of opportunistic infections
  • Current medication use

Symptoms of Kaposi sarcoma include the following:

  • Pain
  • Photophobia
  • Recurrent red or bloody eyes
  • Irritation and foreign body sensation
  • Epiphora
  • Dry eyes
  • Mucopurulent discharge
  • Heavy or swollen eyelids
  • Cosmetic disfigurement of the eyelids
  • Eyelashes rubbing against the eyes
  • Inability to close the eyes
  • Visual obstruction
  • Blurred vision
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Physical

Full ocular examination should include the following:

  • Inspect and evert the eyelids and lashes
  • Perform slit lamp biomicroscopy
  • Examine palpebral and bulbar conjunctivae and fornices in detail
  • Palpate the lacrimal gland, and examine for masses
  • Examine both eyes for proptosis in the rare likelihood of orbital involvement; fortunately, intraocular Kaposi sarcoma has never been reported

The lesions are purplish-red to bright-red and highly vascular with surrounding telangiectatic vessels. They may be macular, plaquelike, or nodular. Ophthalmic Kaposi sarcoma lesions are found on the eyelids, conjunctiva, caruncle, and lacrimal sac. They rarely are found inside the orbit (1 case of choroidal involvement).[16]

See the image below.

Kaposi sarcoma involvement of the eyelid. Courtesy Kaposi sarcoma involvement of the eyelid. Courtesy of Gary N Holland, MD, University of California, Los Angeles, Department of Ophthalmology, Jules Stein Eye Institute.

Dugel et al described 3 clinical stages that may help direct therapy. Stage I and II tumors are patchy and flat. These lesions have a thickness of less than 3 mm in vertical height and are younger than 4 months. Stage III tumors are nodular and elevated with a vertical height of greater than 3 mm. They tend to be older than 4 months.[17]

Of ophthalmic Kaposi sarcoma cases, 6-16% are eyelid lesions, and the superior and inferior eyelids tend to be involved equally. Of ophthalmic Kaposi sarcoma cases, 7-18% are conjunctival lesions. Many conjunctival lesions tend to involve the inferior conjunctiva (as is shown in the image below) and fornix.

The inferior conjunctiva is involved more commonly The inferior conjunctiva is involved more commonly than the superior conjunctiva in Kaposi sarcoma. Courtesy of Gary N Holland, MD, University of California, Los Angeles, Department of Ophthalmology, Jules Stein Eye Institute.

Lesions tend to be indolent, but, as the tumor grows, it can alter ocular adnexal structures[14] and the ocular surface. The mass effect of the tumor on the eyelids can cause mechanical ectropion or entropion with trichiasis and lagophthalmos and irregular astigmatism. Ectropion or entropion can result in poor lid apposition, trichiasis, and lagophthalmos. Consequently, the patient may experience epiphora, poor tear clearance and drainage, recurrent corneal abrasions, pain and discomfort, foreign body sensation, dry eyes, and photophobia. Long-standing trichiasis and exposure can result in corneal infection, scarring, and opacification.

Rarely, tumor bulk may block the visual axis by ptosis or direct obstruction. Tumor bulk may even prevent the complete closure of the eyelid.

Conjunctival involvement may present with subconjunctival hemorrhage, injection, and chemosis.

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Causes

Human herpesvirus-8 (HHV-8) DNA or Kaposi sarcoma–associated herpesvirus (KSHV) has been implicated with patients who are HIV-negative or HIV-positive.[18]

Homosexual males with HIV infection are at an increased risk. This risk is markedly increased with the number of partners.

Patients who have had organ transplants and use immunosuppressive agents and steroids are at an increased risk.

Elderly males of Mediterranean or Ashkenazi ancestry are at an increased risk.

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Contributor Information and Disclosures
Author

Jacqueline Freudenthal, MD Co-Investigator, Ophthalmic Consultants Centre, Toronto

Jacqueline Freudenthal, MD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, Canadian Ophthalmological Society

Disclosure: Nothing to disclose.

Coauthor(s)

Kevin Ryan Yuhan, MD Attending Physician, Cornea, Cataract, Refractive and External Diseases, Southern California Permanente Medical Group

Kevin Ryan Yuhan, MD is a member of the following medical societies: Alpha Omega Alpha, American Medical Association, Association for Research in Vision and Ophthalmology, Phi Beta Kappa, American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery

Disclosure: Nothing to disclose.

Timothy T You, MD Consulting Surgeon in Ophthalmology, Private Practice

Timothy T You, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists

Disclosure: Nothing to disclose.

Specialty Editor Board

Simon K Law, MD, PharmD Clinical Professor of Health Sciences, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, American Glaucoma Society

Disclosure: Nothing to disclose.

Christopher J Rapuano, MD Professor, Department of Ophthalmology, Jefferson Medical College of Thomas Jefferson University; Director of the Cornea Service, Co-Director of Refractive Surgery Department, Wills Eye Hospital

Christopher J Rapuano, MD is a member of the following medical societies: American Academy of Ophthalmology, American Ophthalmological Society, American Society of Cataract and Refractive Surgery, Contact Lens Association of Ophthalmologists, International Society of Refractive Surgery, Cornea Society, Eye Bank Association of America

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Cornea Society, Allergan, Bausch & Lomb, Bio-Tissue, Shire, TearScience, TearLab<br/>Serve(d) as a speaker or a member of a speakers bureau for: Allergan, Bausch & Lomb, Bio-Tissue, TearScience.

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, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Acknowledgements

Anastasios J Kanellopoulos, MD Assistant Program Director, Clinical Associate Professor, Department of Ophthalmology, Manhattan Eye, Ear, and Throat Hospital, New York University

Anastasios J Kanellopoulos, MD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, Eye Bank Association of America, and International Society of Refractive Surgery

Disclosure: Nothing to disclose.

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Section of eyelid with Kaposi sarcoma lesion under high magnification. This tissue section demonstrates increased angiogenesis and spindle-shaped cells. Courtesy of Ben Glasgow, MD, University of California, Los Angeles, Department of Ophthalmology, Jules Stein Eye Institute.
Section of eyelid with Kaposi sarcoma lesion under high magnification. This tissue section demonstrates endothelium-lined vascular channels and proliferation of spindle-shaped cells. Increased angiogenesis with erythrocyte extravasation is observed. Courtesy of Ben Glasgow, MD, University of California, Los Angeles, Department of Ophthalmology, Jules Stein Eye Institute.
The inferior conjunctiva is involved more commonly than the superior conjunctiva in Kaposi sarcoma. Courtesy of Gary N Holland, MD, University of California, Los Angeles, Department of Ophthalmology, Jules Stein Eye Institute.
Kaposi sarcoma involvement of the eyelid. Courtesy of Gary N Holland, MD, University of California, Los Angeles, Department of Ophthalmology, Jules Stein Eye Institute.
 
 
 
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