Juvenile Xanthogranuloma Treatment & Management

  • Author: Theodore Curtis, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Jan 26, 2010
 

Medical Care

  • Pharmacotherapy: Topical, subconjunctival, intralesional, and systemic corticosteroids are used for intraocular and orbital juvenile xanthogranuloma (JXG). Orbital lesions may respond to intralesional steroid injections. Iris lesions are treated with topical prednisolone or subconjunctival steroids. These are followed by sub-Tenon steroids if no improvement occurs in several weeks.
  • Radiotherapy: Low-dose radiation may be the treatment of choice for diffuse uveal lesions, especially if glaucoma is present, or if there is poor response to steroid treatment. Typically, 100-200 cGy is administered per dose over a 2- to 3-week period. The total is usually kept under 500 cGy, but higher doses are used for poorly responsive tumors.
  • Antimetabolites are sometimes used as adjuvants to radiotherapy.
  • Appropriate glaucoma medications should be used in the setting of hyphema and increased intraocular pressure.
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Surgical Care

  • Occasionally, surgery is indicated for localized iris lesions involving less than one quadrant. However, the risks to normal ocular structures may outweigh the benefits.
  • The surgeon should be prepared to deal with massive amounts of bleeding, which can occur with iridectomy in this setting.
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Consultations

  • Discussion with a pediatric dermatologist or radiation oncologist may be helpful to document the extent of the disease and plan treatment.
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Diet

  • No dietary restrictions are indicated for this disorder.
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Activity

  • Standard activity restrictions should be put in place for patients who develop spontaneous hyphema. These restrictions generally include bed rest with bathroom privileges and elevation of the head of the bed 30° from the horizontal.
  • Small children should have a protective shield over the eye at all times to prevent rubbing.
  • Eye protection, especially during sports, should be prescribed for older children who have known ocular tumors.
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Contributor Information and Disclosures
Author

Theodore Curtis, MD  Assistant Professor, Department of Ophthalmology, University of Colorado; Consulting Staff, Rocky Mountain Lions Eye Institute

Theodore Curtis, MD is a member of the following medical societies: American Academy of Ophthalmology and American Association for Pediatric Ophthalmology and Strabismus

Disclosure: Nothing to disclose.

Coauthor(s)

David T Wheeler, MD  Associate Professor, Departments of Ophthalmology and Pediatrics, Oregon Health & Science University

David T Wheeler, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Specialty Editor Board

Gerhard W Cibis, MD  Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas, Kansas City

Gerhard W Cibis, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and American Ophthalmological Society

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD  Associate Professor 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, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

J James Rowsey, MD  Former Director of Corneal Services, St Luke's Cataract and Laser Institute, Florida

J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Pan-American Association of Ophthalmology, Sigma Xi, and Southern Medical Association

Disclosure: Nothing to disclose.

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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