Choroidal Hemangioma:

Central scotoma in the right eye

Greg Zablocki, Curtis Hagedorn, MD and Kevin D. Brady, MD

January 23, 2007, updated Feb 2, 2007

Chief Complaint: Central scotoma in the right eye.

History of Present Illness: This 56-year-old female presented presented for evaluation with reduced vision and an oval-shaped central scotoma in the central visual field of the right eye for two months.

Past Ocular History: History of cataract extraction in both eyes - Right eye 6 years ago and left eye 13 years ago.

Medical History: None

Medications: None

Family History: Noncontributory

Social History: Noncontributory

Ocular Exam:

Figure 1: Flesh-colored lobular sub-retinal lesion superior to the optic nerve, OD.
Figure 2
2A: Early phases of flourescein angiograph with hyperflourescence within the lesion in a coarse vascular pattern 2B: Marked dye persistance within the lesion 20 minutes after injection
2C: Optical coherence tomography (OCT) section through the macula demonstrating sub-retinal fluid (red arrow) prior to treatment. Visual acuity 20/70.

Course: The patient's examination, FFA and OCT were all consistent with choroidal hemangioma. The patient underwent photodynamic therapy employing the technique described in the literature (Michels, 2005). After infusion of 6mg/m2 of verteporfin (Visudyne) intravenously the lesion was illuminated for 163 seconds with the activating laser. Two months later vision had improved from 20/70 to 20/30 and the sub-retinal fluid had resorbed clinically and on OCT (Figure 3)

Figure 3: OCT of the right macula eight months after treatment showing complete resorption of fluid - visual acuity 20/30.

Discussion

Choroidal hemangioma are vascular hamartomas that are present in two distinct forms: a discrete or circumscribed form, and a diffuse form. The diffuse form is typically associated with Sturge-Weber syndrome and discrete lesions are more common in non-syndromic cases. Discrete lesions are typically located near and temporal to the optic disc and are solitary. On examination their most distinguishing feature is their color, usually orange-red or orange-yellow, although other variations have been noted. Symptoms associated with the lesion are usually absent until adulthood. The visual defects that are observed are due to retinal degeneration overlying the tumor as well as serous retinal detachments caused by the lesion.

Fluorescein angiography and ultrasonography are helpful in making the diagnosis of a discrete choroidal hemangioma. On angiography, the lesion will quickly develop diffuse hyperfluorescence and in the arteriovenous phases the initially mottled pattern of fluorescence becomes more confluent. A "coarse vascular pattern" of spotty hyperfluorescence is often described. In later stages, compartmentalized accumulation of dye occurs in the outer retina. Hypofluorescence is also sometimes observed outlining the lesion, correlating to melanocytes that have been pushed to the margin of the tumor. Hemangioma also have a characterstic ultrasonography presentation. The lesions are usually dome shaped with a high internal reflectivity of regular structure. Also, spontaneous vascular movements are not present. In cases where choroidal hemangiomas are associated with increased pigmentation, the highly refective echographic signature of the lesion is helpful in distinguishing from choroidal melanomas, which have a characteristic low to medium reflectivity.

Photocoagulation has long been the accepted method for treating discrete choroidal hemangiomas that are associated with serous retinal detachment. Extent of treatment is limited to the amount needed to resolve the serous fluid. Recently, photodynamic therapy has shown promise in its efficacy and safety in treating symptomatic hemangiomas (Michels, 2005 and Vincuna-Kojchen 2006) and was the method chosen in this case. Additional long-term studies are underway, but intial results are promising. Other therapies have been tried, including low-dose external beam irradiation, proton therapy, and plaque radiotherapy for certain cases of choroidal hemangioma with mixed results.

Diagnosis: Choroidal Hemangioma

EPIDEMIOLOGY

  • Rare; exact incidence is unknown
  • Likely congenital
  • Nearly all cases have been in Caucasians
  • No gender preference

SIGNS

  • Orange-red, flesh-colored, or pinkish lesion
  • Usually near the optic disc
  • Unilateral and solitary is the norm; more than one tumor is rare
  • Tumors are usually amelanotic and transilluminate readily
    • Unusual to show retinal pigment epithelial changes that are seen in melanomas
  • FFA: Hemangioma rapidly develop diffuse, coarse pattern of hyperfluorescence
  • Ultrasonography: lesion is elevated and has a regular, high internal reflectivity

SYMPTOMS

  • Many choroidal hemangioma cause absolutely no symptoms and are found on routine ophthalmoscopy
    • Those that do become symptomatic usually do so in adulthood
  • Induced hyperopia
  • Metamorphopsia
  • Blurred or decreased vision due to serous retinal detachment or retinal degeneration overlying the tumor

TREATMENT

Observation may be all that is indicated in non-vision threatening lesions

Treatment is usually indicated with serous retinal detachments and vision loss:

  • laser photocoagulation used to be the only treatment option
  • photodynamic Therapy (PDT) has shown great promise and has been successful in the majority of cases
    • an average of two treatments is needed to resolve serous fluid
  • low dose radiation, proton, and plaque therapy are being studied

If diffuse hemangioma, work-up for Sturge-Weber Syndrome should be initiated. Glaucoma may need to be addressed

Differential Diagnosis:

References

  1. Baumal C. Ophthalmic Ultrasonography. In: Vander J, Gault J. Ophthalmology Secrets. Philidelphia, PA: Hanley & Belfus, Inc; 1998:40-41.
  2. Frau E, et al. Low-dose proton beam therapy for circumscribe choroidal hemangiomas. Arch Ophthalmol. 2004;122(10):1471-5.
  3. Kivela T, et al. Stereotactic radiotherapy of symptomatic circumscribed choroidal hemangiomas. Ophthalmology. 2003;110(10):1977-82.
  4. Michels, et al. Verteporfin therapy for choroidal hemangioma: a long-term follow-up. Retina. 2005;25(6):697-703.
  5. Sanborg G. Choroidal Hemangioma. In: Ryan S, Schachat A, eds. Retina. Vol 1. St. Louis, MO: The C.V. Mosby Company; 1989:757-766.
  6. Spalton D, Hitchings R, Hunter P, eds. Atlas of Clinical Ophthalmology. 2nd ed. London, England: Wolfe Publishing; 1994:9.20-9.21
  7. Vincuna-Kojchen J, et al. Applicaton of the standard photodynamic treatment protocol for symptomatic circumscribed choroidal hemangioma. Ophthalmologica. 2006;220(6):351-5.

Suggested Citation Format: Zablocki G, Hagedorn C, Brady KD. Choroidal Hemangioma: Central scotoma in the right eye. EyeRounds.org. January 23, 2007; Available from: http://webeye.ophth.uiowa.edu/eyeforum/65-Choroidal-Hemangioma-Photodynamic-Therapy.htm.