Abstract
Purpose
To describe a rare case of racemose hemangioma which developed spontaneous macular macroaneurysm (MA) rupture and vitreaous hemorrhage.
Observations
A 29-year-old healthy asian female visited our hospital and a racemose hemangioma was found in the left eye. At presentation, the best corrected visual acuity (BCVA) was 30/20 in her left eye. At 9 years after the first visit, MA-like lesion was noted in the macular area. After that, vitreous and subretinal hemorrhage appeared in the left eye. The patient underwent simultaneous vitrectomy and cataract surgery, but vitreous re-hemorrhage occurred two days after the operation. To avoid re-hemorrhage, silicone oil (SO) tamponade was added in the second vitrectomy. Two years after the second operation, SO was removed and postoperative BCVA in the left eye was 20/200 without re-bleeding in the vitreous.
Conclusions and Importance
Although retinal hemorrhages have been reported in the patients with a racemose hemangioma, in our case the macular MA rupture occurred at 9 years after the first visit. Congenital retinal arteriovenous anastomosis can show a change in vascular shape in some cases, thus it is important to observe carefully.
1
Introduction
A racemose hemangioma is a rare congenital retinal arteriovenous malformation that can occur as an isolated solitary lesion or component of Wyburn-Mason syndrome (retinal racemose hemangioma associated with intracranial vascular malformation). In cases of the latter, the patient may be affected by intracranial hemorrhage or seizures. Furthermore, the severity of ophthalmic findings can vary widely, from a singular well-defined anastomosis limited to one quadrant of the fundus to a tremendous tumor-like mass with dilated arteries and veins.
We recently encountered a patient with a racemose hemangioma who was complicated with a macroaneurysm (MA) rupture in the macula, as well as subretinal and vitreous hemorrhage.
1.1
Case report
When a 29-year-old female visited an ophthalmic clinic for treatment of hordeolum of the left eyelid, a racemose hemangioma was found in the left eye. The patient was referred to our hospital for further examinations.
The first visit to our hospital occurred in 2008 and visual acuity of the left eye was 30/20, while the right eye was unremarkable. In the left eye, the tortuosity and dilation of retinal arteries and veins were severe, making it very difficult to discriminate between those blood vessel types ( Fig. 1 ; Fig. 2 A, B, C). For determination of Wyburn-Mason syndrome, a head MRI examination was performed, but the results were unremarkable.
Two years after the first visit to our hospital, when the patient was 31 years old, retinal dot hemorrhage findings were observed, with the appearance of mild branch retinal vein occlusion (BRVO) in the supratemporal area. Fluorescein angiography (FA) revealed absence of non-perfusion area, thus retinal photocoagulation therapy was not applied and the retinal hemorrhage disappeared naturally within a few months.
At the age of 32 and again at 34 years, the patient became pregnant and naturally delivered the baby without complications each time. During the associated observation periods, she was not complicated with general hypertension, hyper-lipidemia, or other noteworthy conditions. At 9 years after the first visit, MA-like lesion was noted in the macular area, though direct photocoagulation was not applied because it was thought to be unsafe due to proximity to the fovea ( Fig. 2 D, E, F). OCTA findings revealed macroaneurysm (MA) formation ( Fig. 2 -E, yellow arrow). However, because of its small size, we did not think that there was a risk of rupture. Thereafter, vitreous hemorrhage appeared in the left eye and subretinal hemorrhage at the macula was observed through the vitreous hemorrhage.
The patient underwent simultaneous vitrectomy and cataract surgery procedure. Intra-operative observation showed a subretinal hemorrhage around the macular MA as the bleeding origin of the vitreous hemorrhage. Two days after the operation, vitreous hemorrhage recurred and a second vitrectomy was performed. There was no other bleeding origin in the retina except for the MA. In order to avoid re-bleeding in the vitreous space, a silicone oil tamponade was added during the second vitrectomy. During the SO tamponade, visual acuity of the left eye remained at 4/200 ( Fig. 2 G, H, I). Following the rupture, no evidence of MA formation was noted by OCTA observation ( Fig. 2 -I).
Nearly 2 years after the second surgery, the MA gradually changed to a white color and dry condition, thus a third vitrectomy for removal of the SO was performed. During the procedure, epiretinal membrane peeling was done, though part of the membrane remained due to its tight attachment to the macula ( Fig. 2 J, K, L).
Postoperative visual acuity in the left eye was 20/200 without re-bleeding in the vitreous. Vascular tortuosity and dilation of the retina observed in the former examination partially disappeared along with the appearance of vascular sheathing ( Fig. 3 A, B, C).
