Retina ve Retinal Pigment Epitelin Kombine Hamartomunun Klinik ve Optik Koherens Tomografideki Karakteristik Özellikleri

1. 1Ulucanlar Eye Training And Research Hospital, Ankara, Turkey
2. 2Ulucanlar Eye Education And Research Hospital, Ankara, Turkey

Sorumlu Yazar: OCT is a noninvasive, noncontact technique which provides high-resolution retinal images and has been widely used in the diagnosis of retinal diseases. In our study, our aim was to report and discuss the OCT findings of our cases with CHRRPE. The morphology of the lesion differs according to the type and amount of its contents like RPE, sensory retina, retinal vessels or vitreous and this may lead to difficulties in the differential diagnosis. Also our second case was misdiagnosed several times. Shields et al.3 reviewed the medical records of their 77 cases with CHRRPE. Decreased VA was reported to be the most frequent presenting symptom of the disease among the others like strabismus or irritation. The main causes of decreased VA are the direct involvement of fovea, optic disc or papilla-macular bundle, macular traction by ERM and subretinal and intraretinal exudation from the vessels inside the lesion.7 None of our cases had exudative retinal detachment and CNVM while 13% of cases had serous effusion and 6% of cases had CNVM in Shields et al.3 study. Our first, second and fourth cases also had retinal vessel tortuosity, which was a frequent finding in CHRRPE. FA showed increased retinal venous tortuosity in venous phase in the first and second cases in addition to macular edema in the fourth case. Gass1 divided the cases with CHRRPE into 4 groups according to the location of the lesions as macular, peripheral, papillary or juxtapapillary once. Our first case had juxtapapillary, second case had macular, but third and fourth cases had almost macular lesions. OCT is a very important for accurate diagnosis especially in the differential diagnosis. Shields et al.4 examined 11 consecutive patients who had CHRRPE by using OCT. OCT showed retinal mass with hyper reflective surface and deep shadowing, retinal disorganization, ERM with secondary retinal folds and striae, retinal traction and/or macular edema. Huot et al.5 presented a 17-year-old girl with CHRRPE. OCT showed hyper reflective ERM with traction and retinal folds with distortion of the foveal architecture. In all our cases, OCT showed hyper reflectivity due to ERM and/or fibrotic tissue and deep hypo reflective shadowing. We observed that the adjacent retina was normal in architecture. Ting et al.6 also observed that, the adjacent retina had appeared to be normal and separate from the mass in their cases. Although our first, second and fourth cases had distinct hyper reflectivity; it wasn’t prominent in the third case. This result was thought to be related with the contents of the lesion. Fundoscopy generally reveals similar findings in CHRRPE and choroidal melanoma. Some cases were reported who had undergone enucleation because of prediagnosis of choroidal melanoma but had pathological diagnosis of CHRRPE later.8-9 Only 3% of cases CHRRPE were reported to get an accurate diagnosis in the first visit, by their first ophthalmologists.10 OCT is very important to distinguish these two diseases. OCT mostly shows normal retinal structure in melanoma while hyper reflectivity in inner retinal layers and hypo reflective shadowing in outer retinal layers are observed in CHRRPE. The locations of CHRRPE and choroidal melanoma are thought to cause these different OCT findings.7 In fact, there are some clinical hints to differentiate these two entities. Retinal detachment, subretinal hemorrhage, vitreous hemorrhage and glaucoma are frequently associated with choroidal melanoma but almost never seen in CHRRPE. Furthermore retinal vessel tortuosity is one of the most important sign in CHRRPE but is not seen in choroidal melanoma.7 Also some other diseases like choroidal nevus, optic disc melanocytoma, RPE adenoma and RPE hypertrophy should be kept in mind in the differential diagnosis. As a result CHRRPE is a benign tumor but its differential diagnosis has vital importance in order to avoid unnecessary enucleation or radiation therapy. Another very important point is the mood of the mostly young patients who had misdiagnosed as a malignant tumor and lost their eyes. This report emphasizes the importance of OCT in addition to careful ophthalmic examination in the diagnosis of CHRRPE. Also it can help the clinicians not only in diagnosis and follow-up but also in planning their cases treatments by detecting the onset of associated complications like ERM, CNVM or retinal detachment. Address for Correspondence/Yazisma Adresi: Ufuk Elgin MD, Ulucanlar Eye Training and Research Hospital, Ankara, Turkey Gsm: +90 532 395 72 40 E-mail: kesintunca@yahoo.com Received/Gelis Tarihi: 03.07.2012 Accepted/Kabul Tarihi: 22.01.2013