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Year : 2022  |  Volume : 15  |  Issue : 2  |  Page : 243-244  

Circumscribed choroidal hemangioma in a case of neurofibromatosis type 1

Shri Bhagwan Mahavir, Department of Vitreo Retina Services and Ocular Oncology, Sankara Nethralaya, Medical Research Foundation, Chennai, Tamil Nadu, India

Date of Submission24-Sep-2020
Date of Decision30-Aug-2021
Date of Acceptance06-Apr-2022
Date of Web Publication29-Jun-2022

Correspondence Address:
Dr. Harshit Vaidya
Shri Bhagwan Mahavir, Department of Vitreo Retina Services and Ocular Oncology, Sankara Nethralaya, Medical Research Foundation, No. 41, Old 18, College Rd, Opposite Women's Christian College, Thousand Lights West, Nungambakkam, Chennai - 600 006, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ojo.ojo_369_20

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Keywords: Circumscribed choroidal hemangioma, limbal dermoid, neurofibromatosis type 1

How to cite this article:
Vaidya H, Khetan V. Circumscribed choroidal hemangioma in a case of neurofibromatosis type 1. Oman J Ophthalmol 2022;15:243-4

How to cite this URL:
Vaidya H, Khetan V. Circumscribed choroidal hemangioma in a case of neurofibromatosis type 1. Oman J Ophthalmol [serial online] 2022 [cited 2022 Dec 4];15:243-4. Available from: https://www.ojoonline.org/text.asp?2022/15/2/243/348998

A 55-year-old woman presented with a history of diminution of vision in the right eye for 1 year. She had no known systemic illness. Best-corrected visual acuity in both eyes was 20/30. On external examination, she had multiple neurofibromas involving forearms, back, and face and a large plexiform neurofibroma of the left upper eyelid causing mechanical ptosis along with lower eyelid ectropion and a temporal fossa defect [Figure 1]a. On ocular examination, the left eye had a limbal dermoid and a normal posterior segment [Figure 1]b. The right eye (contralateral eye) had a normal anterior segment, while the posterior segment had a circumscribed choroidal hemangioma (CCH), presenting as a yellowish subretinal elevation about 2 disc diameters (DD) in size, superotemporal to the disc [Figure 1]c. Ultrasonography B-scan of the right eye revealed a retinochoroidal mass (7.47 mm × 7.09 mm × 3.37 mm) superotemporal to the disc along with inferior exudative retinal detachment. Fundus fluorescein angiogram and indocyanine green (ICG) angiography revealed a corresponding area of hyperfluorescence, which increased in intensity with time but without any evidence of dye-washout on ICG [Figure 1]d. We present a rare association of CCH in the case of neurofibromatosis type 1 (NF1). Choroidal abnormalities in NF1 have been documented using ICG angiography, confocal microscopy using infrared light, and recently with near-infrared reflectance and optical coherence tomography (OCT).[1] Hamartomatous changes have been documented in the retina (retinal astrocytoma) as well as choroid (choroidal neurofibromas or choroidal hamartomas) in patients of NF1.[2],[3] A diffusely thickened choroid in NF is thought to occur due to the hamartomatous changes within it. However, recent OCT-based studies have documented a reduction in mean choroidal thickness in such cases.[1] Choroidal nodules (choroidal neurofibromas) are thought to be choroidal ovoid bodies consisting of proliferating Schwann cells.[2] A rare entity of ganglion cell origin known as choroidal ganglioneuronal hamartoma has been described in the literature in patients with NF1.[3] Choroidal hamartomas have been documented in other familial syndromes, such as Cowden's syndrome.[3] The proband of family suspected to have dominantly inherited neurocutaneous syndrome and multiple cavernous hemangioma presented with CCH. Presence of CCH in such cases reflected a variable expression of this disorder.[4] Although hamartomas are a known entity in NF1, CCH (a vascular hamartoma), as observed in our case, is an extremely rare association that must be looked out for in cases of NF1.[5]
Figure 1: (a) External examination showing left upper eyelid plexiform neurofibroma with severe ptosis. (b) Ocular surface of the left eye showing a temporal limbal dermoid. (c) Wide-field fundus photograph (Optos) of the right eye showing circumscribed choroidal hemangioma superotemporal to the disc. (d) Late phase of fundus fluorescein angiogram of the right eye showing hyperfluorescent lesion

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Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Abdolrahimzadeh B, Piraino DC, Albanese G, Cruciani F, Rahimi S. Neurofibromatosis: An update of ophthalmic characteristics and applications of optical coherence tomography. Clin Ophthalmol 2016;10:851-60.  Back to cited text no. 1
Yasunari T, Shiraki K, Hattori H, Miki T. Frequency of choroidal abnormalities in neurofibromatosis type 1. Lancet 2000;356:988-92.  Back to cited text no. 2
Chang I, Juric-Sekhar G, Marshall D, Zhang J. An incidental finding of choroidal ganglioneuronal hamartoma in a patient with neurofibromatosis type 1. Ophthalmic Plast Reconstr Surg 2017;33:S40-2.  Back to cited text no. 3
Sarraf D, Payne AM, Kitchen ND, Sehmi KS, Downes SM, Bird AC. Familial cavernous hemangioma: An expanding ocular spectrum. Arch Ophthalmol 2000;118:969-73.  Back to cited text no. 4
Shields CL, Honavar SG, Shields JA, Cater J, Demirci H. Circumscribed choroidal hemangioma: Clinical manifestations and factors predictive of visual outcome in 200 consecutive cases. Ophthalmology 2001;108:2237-48.  Back to cited text no. 5


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