The authors relate an uncommon case of combined hamartoma of the retina and retinal pigment epithelium associated with optic coloboma

J Rasmussen

M Lind

O Frank

English

PubMed

Lind, Morten

Online Research Database In Technology

The Use of Flow Models for Automated Plant Diagnosis

Eduardo Damasceno

null Pereira Damasceno

null Galvarro Vianna

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© 2011 Damasceno and Damasceno, publisher and licensee Dove Medical Press Ltd. This is an Open Access article which permits unrestricted noncommercial use, provided the original work is properly cited.Clinical Ophthalmology 2011:5 353–354Clinical Ophthalmology Dovepresssubmit your manuscript | www.dovepress.comDovepress 353C A s e  r e P O rTopen access to scientific and medical researchOpen Access Full Text ArticleDOI: 10.2147/OPTH.S15272Combined hamartoma of the retina  and retinal pigment epithelium associated  with optic colobomaNadyr Antonia Damascenoeduardo F DamascenoCorrespondence: eduardo F Damasceno rua do Bispo 230/102, rio de Janeiro,  rJ, Brazil 20261-066 Tel +55 21 3563 4467 Fax +55 21 2568 7728 email e_damasceno@yahoo.comAbstract: The authors relate an uncommon case of combined hamartoma of the retina and retinal pigment epithelium associated with optic coloboma.Keywords: combined hamartoma of the retina and retinal pigment epithelium, benign tumor, visual loss, optic nerve colobomaIntroductionCombined hamartoma of the retina and retinal pigment epithelium is a benign tumor that may involve pigment epithelium, and vascular and glial tissue of the retina to varying degrees.1 It may cause significant visual loss and simulate choroidal melanoma and other intraocular tumors.2Case reportThe purpose of this report is to describe a rare case of combined hamartoma associated with optic coloboma in the fellow eye. A 24-year-old man presented with visual acuity of 20/20 in the right eye and 20/400 in the left eye as per the Snellen chart. In the fundus of the right eye, there were two nonpigmented nodular lesions, one placed close to the optic disc and the other in the temporal retina (Figure 1 above). In the left eye, there was a large excavation placed inside the optic disc tissue (Figure 1 below).Fluorescein angiography showed hyperfluorescence in the right eye’s lesions  (Figure 1 above). The same evaluation in the left eye demonstrated optic disc coloboma (Figure 1 below). In the ocular ultrasound, the lesion close to the optic disc in the right eye was elevated and dense (Figure 2).DiscussionCombined hamartoma of the retina and retinal pigment epithelium has been  occasionally associated with systemic disease such as neurofibromatosis and tuber-ous sclerosis. There were various ocular abnormalities associated with combined hamartoma such as choroidal neovascularization, secondary epiretinal membrane with macular hole formation, X-linked juvenile retinoschisis, optic nerve head pits, and optic nerve head drusen.1 Combined hamartoma are almost always a solitary, unilateral tumor, and located close to the optic disc in 76%, in the macula in 17%, and in the peripheral retina in only 7%.3 Visual acuity varies with the location of the lesions, secondary macular distortion (epiretinal membrane, macular hole, choroidal neovascularization), and  vitreous hemorrhage. The nodular lesions of posterior pole  Clinical Ophthalmology downloaded from https://www.dovepress.com/ by 137.108.70.14 on 15-Jan-2020For personal use only.Powered by TCPDF (www.tcpdf.org)                               1 / 1Clinical OphthalmologyPublish your work in this journalSubmit your manuscript here: http://www.dovepress.com/clinical-ophthalmology-journalClinical Ophthalmology is an international, peer-reviewed journal covering all subspecialties within ophthalmology. Key topics include: Optometry; Visual science; Pharmacology and drug therapy in eye diseases; Basic Sciences; Primary and Secondary eye care; Patient Safety and Quality of Care Improvements. This journal is indexed on PubMed Central and CAS, and is the official journal of The Society of Clinical Ophthalmology (SCO). The manuscript management system is completely online and includes a very quick and fair peer-review system, which is all easy to use. Visit http://www.dovepress.com/ testimonials.php to read real quotes from published authors.Clinical Ophthalmology 2011:5submit your manuscript | www.dovepress.comDovepress DovepressDovepress354Damasceno and Damascenocould vary different amounts of retinal pigment, vascular tissue, and glial  components. In this case, the combined hamartoma had a low pigmentation and was associated with an optic coloboma. The main reason for this association is considered incidental and makes this an uncommon case. The literature reports little manifestations involving the optic disc.4 As recommended by the literature, the patient was observed through  periodical follow-up.5DisclosureNo conflicts of interest were declared in relation to this paper.References1. Elliot D, Schachat AP. Combined hamartoma of the retina and retinal pig-ment epithelium. In Ryan SR, editor. Retina. St. Louis, MO: Mosby Ed. 2001:640–646.2. Schachat AP, Shields JA, Fine SL, et al. Combined hamartomas of the  retina and retinal epithelium. Ophthalmology. 1984;91(12):1609–1615.3. Font RL, Moura RA, Shetlar DJ, Martinez JA, Mcpherson AR. Combined hamartoma of sensory retina and retinal pigment epithelium. Retina. 1989;9(4):302–311.4. Harper CA, Gole GA. Combined hamartoma of the retina and RPE: an unusual cause of the dragged disc appearance. Aust NZ J Ophthalmol. 1986;14(3):235–238.5. Avitabile T, Franco L, Reibaldi M, Pulvirenti M. Combined pigment epithelial and retinal hamartomas: long-term follow-up of three cases. Can J Ophthalmol. 2007;42:318–320.Figure 1 Color retinography – right and left eye and fluorescein angiography – late phase – right and left eye.Figure 2 B ultrasound – nodular lesion – right eye. Clinical Ophthalmology downloaded from https://www.dovepress.com/ by 137.108.70.14 on 15-Jan-2020For personal use only.Powered by TCPDF (www.tcpdf.org)                               1 / 1

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