Cavernous hemangioma of the disc is a rare hamartoma typically found unilaterally and primarily in females. Typically, ophthalmoscopy reveals clusters of saccular aneurysms filled with dark venous blood. The tumor is known for its asymptomatic nature and minimal growth over several years even in patients who have extensive lesions.
There are other reports in literature to refute the same, quoting recurrent episode of retinal and vitreous hemorrhage causing diagnostic dilemma and visual loss.
We herein present a case of diagnostic and therapeutic dilemma arising from the confounding initial presentation, atypical of the underlying disease. She had a predisposition to develop recurrent hemorrhages from the cavernous hemangioma leading to vision loss and who inadvertently benefited from photodynamic therapy (PDT).
A 32-year-old female presented to us with complaints of sudden onset of blurry vision in her right eye since the very morning. She was on treatment for idiopathic thrombocytopenic purpura (ITP) with a present platelet count of 40,000 cells/mm3 and was on oral steroids. There was no other significant ophthalmic history and this was her first ophthalmic consultation.
She had 20/20 vision in both her eyes though she indicated that her right eye vision was not as clear as that of the left. Anterior segments of both eyes were normal with brisk reacting pupils. Fundus examination of the right eye showed a 1-disc diameter mound of subretinal hemorrhage just above a disc partly encroaching onto the papillomacular bundle; fovea and macula were spared [Fig. 1]. Left eye fundus was normal, a working diagnosis of idiopathic polypoidal choroidal vasculopathy (IPCV) was made, and an indocyanine angiogram (ICG) was performed to confirm the same. ICG showed a single “hot spot” within the subretinal hemorrhage with rest of the choroidal circulation being normal [Fig. 1]. Since the subretinal hemorrhage did not extend beneath the fovea, she was managed conservatively.
She presented 4 days later with a large paracentral scotoma and floaters with a visual acuity of 20/64. Dilated fundus examination revealed a considerably enlarged subretinal hemorrhage extending and nearly involving the fovea [Fig. 2]. Systemic condition was poor with the platelet count dipping down to 32,000 cells/mm3. Although it is not unusual for IPCV to present with recurrent bleeding, underlying ITP in this patient could have prompted the rapidity of its recurrence. This compelled us to intervene with intravitreal anti-vascular endothelial growth factor injection and gas displacement of the subretinal hemorrhage. She was injected with 0.5 mg/0.05 ml of ranibizumab (Lucentis, Genentech USA Inc.,) with 0.3 ml of pure sulfur hexafluoride (SF6) gas and advised to maintain prone position.
A week later the subretinal hemorrhage had cleared considerably from the macular area with marginal vision improvement [Fig. 3]. A month later, submacular hemorrhage had cleared considerably enough to allow PDT with verteporfin for the suspected polyps. Pretreatment fluorescein angiography, however, revealed fluorescein dye showing the presence of a cluster of preretinal saccular lesions with sluggish intralesional circulation resulting in separation of the cellular and plasma component of the blood, the plasma component staining with fluorescein resulting in “fluorescein caps” [Fig. 4]. These features were suggestive of cavernous hemangioma of the optic disc. The underlying ITP may have complicated the usually innocuous cavernous hemangioma of the optic disc with subretinal hemorrhage. We discussed the option of treating the lesion with PDT with the patient and the same was performed with an informed consent.
Standard PDT was performed employing 2 adjacent laser spots to treat the peripapillary lesion, avoiding the optic disc. Full-fluence PDT was employed along with a laser spot of 3.2 mm to treat the lesion above the disc, and a second laser spot of 1.2 mm with half fluence was used to treat part of the lesion over the papillomacular bundle. This resulted in complete regression of the hemangioma at the 2-week follow-up [Fig. 5]. The lesion remained regressed for 4 months with complete vision restoration to 20/20 when she was last reviewed.
Hemangioma of the retina or optic disc is noted to have systemic associations, which may include cerebral hemangioma and cutaneous hemangiomas. Contrary to this, our patient revealed no cutaneous or central nervous system involvement but had a totally unrelated entity of ITP. In addition, there was no family history of any ocular or systemic diseases. Ours is the first to report recurrent subretinal hemorrhage within a short interval in a patient with cavernous hemangioma. Complication possibly being related to the low platelet count and subsequently stabilized with increase in the platelet count.
Misdiagnosis and diagnosis
Cavernous hemangioma is commonly misdiagnosed as proliferative diabetic retinopathy and secondary glaucoma necessitating intravitreal intervention and treatment for glaucoma. In our patient, the lesion was obscured with subretinal hemorrhage, a rare occurrence in a cavernous hemangioma of the optic disc, resulting in a misdiagnosis of IPCV. Fluorescein angiography is essential in diagnosis of cavernous hemangioma. In early frames of the fundus fluorescein angiography (FFA), the vascular channels within the hemangioma remain hypofluorescent. As the angiogram progresses, these vascular channels fill slowly with the dye. In late frames, a blood-fluorescein interface is characteristically observed in the saccular dilations within the tumor. This phenomenon seems to be related to sedimentation of erythrocytes in the inferior aspect of the venous aneurysm, which appears hypofluorescent. These specific FFA features helped us to arrive at the final diagnosis of cavernous hemangioma of the disc.
Fundus changes: Absence of hard exudates in association with cavernous hemangioma is a typical feature. Our case differs in this as there were extensive hard exudates, extending from the superior arcade to the inferior arcade at the temporal edge of the subretinal hemorrhage. The mechanism though not clear may be postulated to the dense hemorrhage, which occurred.
Treatment: As far as the treatment is concerned, not much is available in literature. Laser photocoagulation of the vessels was suggested way back in 1986. Temporary regression of the macular cavernous tumor with visual improvement in response to systemic infliximab infusion (tumor necrosis factor-α antibodies) has been published in recent years. Compared to these, our case opens up a new arena in treating patients with symptomatic hemangioma of the disc or retina safely with PDT.
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Conflicts of interest
There are no conflicts of interest.
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