Vision-Threatening Complications of Juxtapapillary Retinal Capillary Hemangioma: A Case of Retinal Artery Occlusion

Introduction

Juxtapapillary retinal capillary hemangioma (JRCH) is a seldom-seen vascular hamartoma located at the optic nerve head or juxtapapillary area. JRCH can present either as an isolated condition or as an ocular manifestation of von Hippel-Lindau disease [1]. JRCH can have different growth patterns [2]. Generally, the visual acuity of patients with JRCH deteriorates over long-term follow-up. Complications are also a significant cause of vision loss, such as exudation, subretinal fluid, macular edema, epiretinal membrane, and exudative or/and exudative tractional retinal detachment. Unlike the previously reported complications, we report a case of branch retinal artery occlusion (BRAO) in the setting of progressive exophytic JRCH, which, to the best of our knowledge, has not been reported until now. We will emphasize the severity of this complication through this case, as well as the treatment we implemented, to provide valuable experience for when clinicians encounter similar situations.

Case Report

A 34-year-old previously healthy woman presented with visual blurring and visual field defects of the left eye for 4 days. There had been no change in the condition over the 4 days, and no treatment had been administered. She had a negative ocular, medical, and family history. On presentation, the best corrected visual acuity (BCVA) was 20/20 in both eyes. Fundoscopy revealed trace vitreous opacity and a red, well-circumscribed, elevated mass about 1 disc diameter in size on the inferior part of the disc, with a small amount of surrounding intraretinal hemorrhage and overlying dilated and tortuous blood vessels (Figure 1A). B-scan ultrasound showed scattered points in the vitreous body of the left eye and hyperechoic protuberance on the optic surface (Figure 1B). Fluorescein angiography (FA) showed that the tumor was filled during the arterial phase, and there was late diffuse leakage. In the early phase, the infratemporal retinal artery straddled the tumor and wrapped around the infratemporal retinal vein (Figure 1C). An optical coherence tomography (OCT) scan indicated that the lesion was situated within the retina and protruded into the vitreous cavity. Exudation in the outer nuclear layer from the vascular lesion was extending toward but not involving the macula (Figure 2A). The sub-structure of the tumor was not clearly displayed on optical coherence tomography angiography (OCTA; Figure 2B). Visual field examination showed that the physiological blind spot was enlarged in the left eye (Figure 2C). Magnetic resonance imaging of the brain and orbits was unremarkable. Neurological and systemic examinations were normal. Exophytic JRCH was diagnosed, and the patient was initially treated with iodized lecithin tablets (JOLETHIN, Daiichi Yakuhin Sangyo Co, Ltd) 3 mg orally twice per day. Invasive treatment was not recommended, given the patient’s good BCVA and visual field.

Unfortunately, 3 days later, the patient noticed a superior visual field defect in her left eye when she got up in the morning. The BCVA of her left eye decreased to 20/25, and fundoscopy found vitreous hemorrhage in front of the optic disc, swelling of the tumor, and macular edema inferiorly, with a cherry red spot (Figure 3A). Repeat visual field testing revealed a new defect superiorly (Figure 3B), which corresponded to macular edema on OCT (Figure 3C) and vascular occlusion of the inferior macula on OCTA (Figure 3D). These examinations were performed after first aid treatment, which included raceanisodamine hydrochloride (an antispasmodic) 10 mg retrobulbar injection, along with low-flow oxygen inhalation, eye massage, sublingual nitroglycerin, intraocular pressure reduction treatment, Ginaton injection 87.5 mg 4 times daily, intravenously guttae, nutritional nerve therapy, and hyperbaric oxygen treatment. After 10 days of treatment, the tumor body shrank, and retinal and intravitreal hemorrhage were almost absorbed (Figure 4A, 4C); however, the superior visual field defect and the vascular occlusion of the inferior macula were not improved (Figure 4B, 4D). A brief timeline of the case is shown in Figure 5.

Discussion

Retinal capillary hemangioblastomas are rare benign retinal tumors composed of specific cells. The clinical course of JRCH is often progressive vision loss, associated with various manifestations. Catastrophic vision loss and even blindness or phthisis bulbi can occur in complicated cases [3]. Endophytic JRCH grows on the surface of the optic disc or retina. Exophytic JRCH presents as a nodular, orange-red lesion that grows in the outer retina layers. Sessile JRCH is relatively flat [2]. Many techniques can assist in providing a differential diagnosis. Basic fundus examinations, such as scanning laser ophthalmoscopy, can be used for rapid screening. B-scan ultrasound can help preliminarily determine the location and nature of the lesion. FA can identify nearly all early tumors. The FA changes include rapid filling of the tumor in the early stage and leakage and enhanced staining of surrounding tissues in the late stage [4]. OCTA can be helpful in tumor localization, quantitative analysis of tumor size and subretinal fluid, diagnosis, and follow-up of the tumor [5–7].

The treatment goals of JRCH are to maintain the best corrected vision and visual field and deal with vision-threatening complications. There are no specific guidelines or ideal effective treatments for JRCH [8,9]. The ultimate goal of treatment is not to obliterate the tumor, but to control the associated sight-threatening complications. Various treatment modalities include laser photocoagulation [1,10], proton beam radiotherapy [3], transpupillary thermotherapy, indocyanine green-enhanced transpupillary thermotherapy [11], anti-angiogenic intravitreal injection [12,13], photodynamic therapy [14–18], and pars plana vitrectomy [19], or a combination of the above treatments [20–23]; however, the most appropriate treatment for this disease remains to be determined. Because the tumor grows on the optic disc, it is almost impossible to destroy the tumor without damage of superficial nerve fibers. Therefore, each treatment has its risks and limitations, and the postoperative BCVA is generally less than 20/40 [9,13,14,18].

In the present case, both the visual acuity and visual field were relatively good at presentation, and since any invasive treatment can lead to tumor rupture, massive vitreous hemorrhage, and rapid deterioration in vision acuity, we preferred conservative treatment and decided to follow-up and observe. The expected outcomes were vitreous hemorrhage absorption, tumor stabilization, and visual field defect recovery. If BCVA further deteriorated or the vitreous hemorrhage worsened, we would have considered more aggressive invasive treatments, such as pars plana vitrectomy. However, unexpectedly, the patient developed BRAO within a short period of time, which had never previously been reported.

There are many complications of JRCH, which most commonly include retinal exudation, vitreous hemorrhage, increased intraocular pressure, neovascularization, and retinal detachment, primarily caused by the compression of the tumor and hemodynamic abnormalities [24,25]. In our case, the patient had vitreous hemorrhage and subsequently developed secondary BRAO, which had not been reported before. By FA, we found that the tumor filling appeared in the arteriovenous phase, suggesting that the tumor was more closely related to the retinal venous system. BRAO may have been caused by the expansion of the tumor for unknown reasons, which led to the increased pressure, venous expansion, and compression of the underlying arterial blood vessels, resulting in the development of BRAO. Meanwhile, FA showed that the perfusion of the inferior artery, especially the inferotemporal artery, was delayed, compared with that of the superior arteries. This finding can serve as a reminder for clinicians of the ophthalmologist risk factors for RAO. Because our patient was in the hospital, the treatment was given less than 5 min after she noticed the visual field defect. Unfortunately, the visual function of the patient had not recovered. The tumor volume was reduced at the 1-week follow-up, and the condition was stable; therefore, close follow-up was planned. Frequent follow-up without therapeutic intervention may be a more suitable management strategy for our case. Not all JRCH will lead to massive vitreous hemorrhage. Conservative treatment rather than invasive approaches may be more beneficial for preserving the patient’s visual function, in the absence of other complications. For this patient, we will conduct long-term follow-up and observation in hopes of gaining experience of the disease’s progression.

Conclusions

BRAO could be a vision-threatening complication of JRCH. The etiology and pathogenesis of RAO in the setting of JRCH needs to be further explored, and the optimal treatment of this condition remains to be studied.