Ophthalmologic Manifestations of Kaposi Sarcoma Clinical Presentation

  • Author: Jacqueline Freudenthal, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Feb 22, 2010
 

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.
  • 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.
  • Ophthalmic Kaposi sarcoma lesions are found on the eyelids, conjunctiva, caruncle, and lacrimal sac. They rarely are found inside the orbit. An example of eyelid involvement is shown in the example below. Kaposi sarcoma involvement of the eyelid. CourtesyKaposi sarcoma involvement of the eyelid. Courtesy of Gary N Holland, MD, University of California, Los Angeles, Department of Ophthalmology, Jules Stein Eye Institute.
  • 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 commonlyThe 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 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 and HIV-positive.[2]
  • Homosexual males with HIV 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, and 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 Academy of Ophthalmology, American Medical Association, American Society of Cataract and Refractive Surgery, Association for Research in Vision and Ophthalmology, and Phi Beta Kappa

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 and American Society of Retina Specialists

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

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.

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 Institute

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

Disclosure: Allergan Honoraria Speaking and teaching; Allergan Consulting fee Consulting; Alcon Honoraria Speaking and teaching; Inspire Honoraria Speaking and teaching; RPS Ownership interest Other; Vistakon Honoraria Speaking and teaching; EyeGate Pharma Consulting; Inspire Consulting fee Consulting; Bausch & Lomb Honoraria Speaking and teaching; Bausch & Lomb Consulting fee Consulting

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.

References

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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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