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Adolescent Boy With Choroidal Mass

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A boy aged 13 years was referred for choroidal mass of the right eye. Visual acutiy was 20/60 OD and 20/20 OS. Intraocular pressures and anterior segment examination findings were normal. Fundus examination revealed orange, subretinal mass with associated subretinal fluid in the posterior pole. The lesion was followed closely and throughout a series of visits, the lesion and fluid continued to progress, the visual acuity worsened to 20/200, and the tumor mass extended closer to the fovea (Figure 1). The mass measured 15 mm in basal diameter with a thickness of 6.4 mm on B-scan ultrasonography.

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Circumscribed choroidal hemangioma

B. Iodine 125 plaque brachytherapy

Circumscribed choroidal hemangioma (CCH) is a benign, vascular tumor considered to be congenital in nature. It is classically described as an orange-red tumor, usually in the posterior pole.1 Circumscribed choroidal hemangioma is usually diagnosed when it is causing visual disturbance from exudative retinal detachment. Circumscribed choroidal hemangioma can be treated by a variety of methods including photodynamic therapy (PDT), laser photocoagulation, cryotherapy, external beam radiotherapy, stereotactic radiotherapy, proton beam radiotherapy, episcleral plaque radiotherapy, and transpupillary thermotherapy PDT.2

The results of PDT for CCH are very favorable; however, the exact treatment regimen of PDT for CCH varies depending on lesion size and location. In 2 studies of PDT in CCH, tumor thickness ranged from 1.9 mm to 5.9 mm (smaller than in this patient) and thicker lesions are less responsive to PDT or require more sessions.2,3 Diffuse choroidal hemangiomas and those associated with Sturge-Weber syndrome can achieve similar thicknesses, but it is unusual for true circumscribed lesions to be this thick. Additionally, placement of an intravenous catheter, infusion of verteporfin, and multiple sessions of laser with a slitlamp delivery system would be challenging for a 13-year-old patient (choice A).

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Article Information

Corresponding Author: Brian P. Marr, MD, Department of Ophthalmology, Edward S. Harkness Eye Institute, Columbia University Irving Medical Center, 635 W 165th St, New York, NY 10032 (bpm2133@cumc.columbia.edu).

Published Online: May 16, 2019. doi:10.1001/jamaophthalmol.2019.1177

Conflict of Interest Disclosures: Dr Marr reports other support from Aura Biosciences and Castle Biosciences outside the submitted work. No other disclosures were reported.

Additional Contributions: We thank the patient’s family for granting permission to publish this information.

References
1.
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(12):2237-2248. doi:10.1016/S0161-6420(01)00812-0PubMedGoogle ScholarCrossref
2.
Singh  AD, Kaiser  PK, Sears  JE, Gupta  M, Rundle  PA, Rennie  IG.  Photodynamic therapy of circumscribed choroidal haemangioma.  Br J Ophthalmol. 2004;88(11):1414-1418. doi:10.1136/bjo.2004.044396PubMedGoogle ScholarCrossref
3.
Gupta  M, Singh  AD, Rundle  PA, Rennie  IG.  Efficacy of photodynamic therapy in circumscribed choroidal haemangioma.  Eye (Lond). 2004;18(2):139-142. doi:10.1038/sj.eye.6700597PubMedGoogle ScholarCrossref
4.
Mandal  S, Naithani  P, Venkatesh  P, Garg  S.  Intravitreal bevacizumab (avastin) for circumscribed choroidal hemangioma.  Indian J Ophthalmol. 2011;59(3):248-251. doi:10.4103/0301-4738.81051PubMedGoogle ScholarCrossref
5.
Sagong  M, Lee  J, Chang  W.  Application of intravitreal bevacizumab for circumscribed choroidal hemangioma.  Korean J Ophthalmol. 2009;23(2):127-131. doi:10.3341/kjo.2009.23.2.127PubMedGoogle ScholarCrossref
6.
Schilling  H, Sauerwein  W, Lommatzsch  A,  et al.  Long-term results after low dose ocular irradiation for choroidal haemangiomas.  Br J Ophthalmol. 1997;81(4):267-273. doi:10.1136/bjo.81.4.267PubMedGoogle ScholarCrossref
7.
Harbron  RW, Feltbower  RG, Glaser  A, Lilley  J, Pearce  MS.  Secondary malignant neoplasms following radiotherapy for primary cancer in children and young adults.  Pediatr Hematol Oncol. 2014;31(3):259-267. doi:10.3109/08880018.2013.838723PubMedGoogle ScholarCrossref
8.
López-Caballero  C, Saornil  MA, De Frutos  J,  et al.  High-dose iodine-125 episcleral brachytherapy for circumscribed choroidal haemangioma.  Br J Ophthalmol. 2010;94(4):470-473. doi:10.1136/bjo.2009.160184PubMedGoogle ScholarCrossref
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