Multifocal circumscribed choroidal hemangioma: a rare occurrence

Abstract

A 32-year-old Asian-Indian woman presented with painless diminution of vision in her left eye (oculus sinister (OS)) and fundus examination OS revealed a large dome-shaped orangish-red subretinal lesion at the macula along with a smaller lesion on the superotemporal arcade. Indocyanine green angiography and optical coherence tomography features confirmed the diagnosis of multifocal circumscribed choroidal hemangioma (CCH). There was no evidence of Sturge-Weber syndrome. CCH is usually solitary with only one reported case of multifocal CCH in the literature.

Background

Circumscribed choroidal hemangiomas (CCHs) are benign vascular hamartomas of uncertain aetiology which may be diagnosed incidentally on routine ophthalmic examination or on evaluation for visual symptoms.^1–3 Most of the tumours are unilateral with rare exceptions.^4–6 Further, CCHs classically are known to occur as solitary lesions.³ Herein we report an unusual case of multifocal CCH, and review the literature of a similar case.

Case presentation

A 32-year-old Asian-Indian woman presented with painless diminution of vision in her left eye (oculus sinister (OS)). General examination was unremarkable. Best-corrected visual acuity OS was 20/40. Anterior segment was within normal limits and fundus examination OS revealed a large dome-shaped orangish-red subretinal lesion measuring 11×10×5 mm at the posterior pole involving the fovea (figure 1A, white arrow). In addition, a similar lesion measuring 3×3×2 mm along the superotemporal arcade (figure 1A, yellow arrow) was also noted. Examination of the right eye was normal with no evidence of any choroidal lesion.

Figure 1.

(A) Colour fundus photograph of the left eye showing a large dome-shaped orangish-red subretinal lesion measuring 11×10×5 mm at the posterior pole involving the fovea (white arrow) along with a smaller lesion of 3×3×2 mm on the superotemporal arcade (yellow arrow). (B) Both the lesions show hypercyanescence in the early phase. (C, D) Optical coherence tomography scans passing through the superotemporal and macular lesions, respectively, showing a well-defined choroidal lesion with low internal reflectivity and clear spaces evident in the superficial aspect of the lesions. (E, F) Ultrasound B scan through the smaller superotemporal lesion and the larger macular lesion show high echogenicity at the surface. Internal structure of the lesions is heterogenous with high echogenicity.

Investigations

Indocyanine green angiography (ICG) of the left eye showed early hypercyanescence corresponding to both the lesions (figure 1B, white arrow and yellow arrow) with a rapid and uniform increase in the intensity followed by a late wash out. Optical coherence tomography (OCT) scan through the macular lesion showed a normal vitreoretinal interface with a dome-shaped elevation of the retinal layers due to a large hyporeflective choroidal lesion and the posterior limit of the lesion was not visualised (figure 1C). OCT scan through the superotemporal lesion showed a normal vitreoretinal interface, a well-circumscribed uniformly hyporeflective choroidal lesion with superficial hyporeflective spaces (figure 1D). Ultrasonography B scan (USG) of both the lesions showed high surface echoes with heterogenous high internal echoes (figure 1E, F). Thus the clinical, ICG, OCT and USG features confirmed the diagnosis of multifocal CCH. Systemic evaluation did not reveal any features of Sturge-Weber syndrome.

Outcome and follow-up

The patient was followed up over 1 year and her vision remained stable. Stable vision and lack of subretinal fluid warranted no intervention and the patient was asked to review biannually.

Discussion

Choroidal hemangiomas are benign vascular tumours of the uveal tract, which occur as circumscribed or diffuse type with the latter commonly seen with Sturge-Weber syndrome.¹ The aetiology of CCH is uncertain and they are usually diagnosed in adulthood either incidentally or when complications arise. Loss of vision due to these benign tumours may be a result of their subfoveal location, presence of subfoveal serous detachment, secondary retinal pigment epithelial changes such as hyperplasia or metaplasia, retinoschisis and neovascularisation.^{1 2} Histologically these are congenital vascular hamartomas and increase in size of the lesion is believed to be due to increased venous congestion rather than cell proliferation.² Almost always CCHs are unilateral and solitary.^1–3 However, occurrence of bilateral CCH has been reported in literature with no systemic associations.^4–6 Krohn et al documented the presence of two discrete CCH in an otherwise healthy patient among 113 patients with CCH, which is the first and only reported case of multifocal CCH in English literature.³ In comparison to the reported case of multiple CCH,³ the second tumour in our case was much smaller than the main tumour and more evident on ICG and OCT than clinical examination. Since the aetiology of CCH itself is obscure, no clear explanation exists for solitary or multifocal occurrence of the lesions. Whether the presence of multifocal lesions is underreported is debatable. Nevertheless, this case highlights the fact that CCH may not always occur as solitary lesions and a good clinical examination with use of ancillary investigations helps identify multiple lesions when present.

The treatment options for choroidal hemangioma include photodynamic therapy which is the preferred modality, transpupillary thermotherapy, plaque radiotherapy, proton beam radiotherapy and external beam radiotherapy.¹ Enucleation is rarely indicated when the patient has painful blind eye due to secondary effects of the tumour. The lesions can be observed periodically if there are no signs of tumour activity. In our case, though the patient had two lesions, there were no signs of tumour activity in either of the lesions over 1 year follow-up period, and thus periodic observation was advised.

Learning points.

• Although most commonly circumscribed choroidal hemangiomas are solitary, there can be multiple lesions in very rare instances.

• A thorough clinical examination is warranted to note multiple lesions.

• Presence of multiple choroidal tumours can raise the suspicion of choroidal metastasis. However, clinical features combined with ancilliary investigations can differentiate hemangiomas from metastasis in equivocal cases.