University of Iowa Health Care

Ophthalmology and Visual Sciences

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Choroidal Osteoma:

10-year-old girl presents for annual follow-up. She reports that she recently failed a school screening

Glen M. Bianchi, M.D., Ronald Keech, MD & Richard Olson, MD

February 21, 2005

Chief Complaint: 10-year-old girl presents for annual follow-up. She reports that she recently failed a school screening.

History of Present Illness: However, the patient offers no complaints herself.

PMH: healthy. FH: non-contributory.POH: Limbal Dermoid OS, Retinal Lesion OD.

EXAM

  • Best corrected visual acuities: 20/40 OD, 20/20 OS
  • Stereo Vision: normal
  • Pupils: normal, no RAPD
  • VF: full to CF OU
  • EOM: normal
  • IOP: normal OU
  • SLE: normal
  • DFE: see below
figure 1
OD - age 5. Large area of sub-retinal fluid. OD - age 7. Deep area of calcification enlarges.
Age 5 Age 7

figure 2. OD - age 10. Sharply demarcated area of calcification with subretinal hemorrhage.

Age 10

figure 3
FFA OD - early FFA OD - mid

FFA OD

FFA OD


figure 4. FFA OD - late. FFA denotes staining in areas of calcification and areas of blockage corresponding to the hemorrhage.

FFA OD


figure 5
A-Scan OD B-Scan OD
A-Scan B-Scan
A-Scan echography demonstrates sound attenuation posterior to the lesion (i.e. downward sloping after the second signal peak). B-Scan echography demonstrates shadowing posterior to the highly dense lesion (i.e. dense plaque with absent signal behind the lesion).

Discussion

Choroidal Osteomas

This is an osseous choristoma of the choroid. There is no known treatment. There is a predilection for women, and it is typically found in the second or third decade of life. Choroidal osteomas may be bilateral in 25% of cases. Growth is seen in 40% of cases with long-term follow-up. These lesions may be associated with choroidal neovascular membranes (CNVM), sub-retinal fluid, and sub-retinal hemorrhages.

80% of patients have 20/30 vision or better at presentation. Visual loss may be gradual or acute due to RPE degeneration or CNVM. Dr. Gass estimates that up to 33% of eyes with choroidal osteoma may develop CNVM.

Fluorescein Angiography: Early, patchy hyperfluorescence with intense late staining.

Echography : Densely echogenic (extremely high internal reflectivity of bone), persists at low sensitivities. Marked "Shadowing," or sound attenuation posterior to lesion. "Void" appears behind lesion.

Histology : Mature bone with interconnecting marrow spaces. Lesion is sharply demarcated form choroid.

Diagnosis: Choroidal Osteomas

EPIDEMIOLOGY

  • Rarely familial.
  • Predilection for women.
  • Presents in second and third decade of life.
  • Incidence: rare

SIGNS

  • Bilateral in 25% of cases.
  • About one-third of cases will be associated with a CNVM.
  • Yellow-white lesion, well demarcated.
  • Fluorescein Angiography: Early, patchy hyperfluorescence with intense late staining.
  • Echography : Densely echogenic (extremely high internal reflectivity of bone), persists at low sensitivities. Marked "Shadowing," or sound attenuation posterior to lesion. "Void" appears behind lesion.

SYMPTOMS

  • Mild vision loss in the early stages.
  • Moderate to severe vision loss if associated with RPE atrophy or CNVM.

TREATMENT

  • No treatment available for choroidal osteoma.

Differential Diagnoses

  • Amelanotic Choroidal Melanoma
  • Amelanotic Choroidal Nevus
  • Choroidal Hemangioma
  • Metastatic Carcinoma
  • Organized Submacular Hemorrhage
  • Cicatricial Macular Degeneration

References

  1. Aylward, G.W., et al., A long-term follow-up of choroidal osteoma. Arch Ophthalmol, 1998. 116(10):1337-41.
  2. Shields, C.L., J.A. Shields, and J.J. Augsburger, Choroidal osteoma. Surv Ophthalmol, 1988. 33(1):17-27.
  3. Grand, M.G., et al., Choroidal osteoma. Treatment of associated subretinal neovascular membranes. Retina, 1984. 4(2):84-9.
  4. Gass, J.D., et al., Choroidal Osteoma. Arch Ophthalmol, 1978. 96(3):428-35.
  5. Kida, Y., et al., Choroidal osteoma in an infant. Am J Ophthalmol, 1997. 124(1):119-20.
  6. Fava, G.E., et al., Choroidal osteoma in a 6-year-old child. J Pediatr Ophthalmol Strabismus, 1980. 17(3):203-5.
  7. Mizota, A., R. Tanabe, and E. Adachi-Usami, Rapid enlargement of choroidal osteoma in a 3-year-old girl. Arch Ophthalmol, 1998. 116(8):1128-9.
  8. Katz, R.S. and J.D. Gass, Multiple choroidal osteomas developing in association with recurrent orbital inflammatory pseudotumor. Arch Ophthalmol, 1983. 101(11):1724-7.
  9. Okada, K., et al., Bilateral choroidal osteomas associated with histiocytosis X. Jpn J Ophthalmol, 1996. 40(1):111-5.
  10. Trimble, S.N. and H. Schatz, Choroidal osteoma after intraocular inflammation. Am J Ophthalmol, 1983. 96(6):759-64.
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  13. Teich, S.A. and J.B. Walsh, Choroidal osteoma. Ophthalmology, 1981. 88(7): p. 696-8.
  14. Gass, J.D., New observations concerning choroidal osteomas. Int Ophthalmol, 1979. 1(2): p. 71-84.
  15. Avila, M.P., et al., Bilateral choroidal osteoma with subretinal neovascularization. Ann Ophthalmol, 1984. 16(4):381-5.
  16. Augsburger, J.J., J.A. Shields, and C.J. Rife, Bilateral choroidal osteoma after nine years. Can J Ophthalmol, 1979. 14(4):281-4.
  17. Morrison, D.L., et al., Review of choroidal osteoma: successful krypton red laser photocoagulation of an associated subretinal neovascular membrane involving the fovea. Ophthalmic Surg, 1987. 18(4): p. 299-303.
  18. Rose, S.J., J.F. Burke, and R.J. Brockhurst, Argon laser photoablation of a choroidal osteoma. Retina, 1991. 11(2):224-8.
  19. Burke, J.F., Jr. and R.J. Brockhurst, Argon laser photocoagulation of subretinal neovascular membrane associated with osteoma of the choroid. Retina, 1983. 3(4):304-7.
  20. Shields, J.A., et al., Ocular manifestations of the organoid nevus syndrome. Ophthalmology, 1997. 104(3): p. 549-57.
  21. Traboulsi, E.I., et al., Posterior scleral choristoma in the organoid nevus syndrome (linear nevus sebaceus of Jadassohn). Ophthalmology, 1999. 106(11):2126-30.

Suggested citation format: Bianchi GM, Keech RV, Olson R: Choroidal Osteoma: 10-year-old girl presents for annual follow-up. She reports that she recently failed a school screening. February 21, 2005; Available from: http://www.EyeRounds.org/cases/case12.htm.

last updated: 02-21-2005
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