J Korean Ophthalmol Soc.  2020 Apr;61(4):432-438. 10.3341/jkos.2020.61.4.432.

Multiple Retinal Capillary Hemangioma Associated with NeurofibromatosisType 1

• Lee SM
• Kwon JM
• Shin J
• Kwon HJ

• KMID: 2500111
• DOI: http://doi.org/10.3341/jkos.2020.61.4.432

Abstract

Purpose
To report a case of multiple retinal capillary hemangioma associated with neurofibromatosis type 1 and resulting neovascular glaucoma.
Case summary
A 13-year-old boy was admitted with complaint of visual disturbance and dull pain in his left eye which had begun two weeks prior. Lisch nodules were observed in his left iris and corneal opacity with neovascularization of the iris and angle were detected in the same eye. Multiple retinal capillary hemangiomas with increased tortuosity and congestion of feeding retinal vessels were observed on the upper equator of the left retina; in addition, vitreous hemorrhage was observed. There were no other abnormalities except iris mammillations in the right eye. Numerous café au lait macules were observed on the patient’s entire body. He also exhibited axillary freckling. On brain magnetic resonance imaging, T2-weighted signal intensity was increased in the basal ganglia, left thalamus, and cerebellar white matter; however, no vessel abnormalities were observed on magnetic resonance angiography. High intraocular pressure (IOP) persisted despite the use of IOP lowering agents and IOP was normalized after trabeculectomy with mitomycin C administration.
Conclusions
This case shows that multiple retinal capillary hemangiomas can be accompanied by neurofibromatosis type 1, which may result in neovascular glaucoma.

Keyword

Capillary hemangioma; Neovascular glaucoma; Neurofibromatosis; Trabeculectomy