Combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) is a rare, benign tumor, but there are no established managements for CHRRPE. A patient with CHRRPE who is treated successfully by the new combination therapy was described. A 32-year-old man was diagnosed as having CHRRPE after evaluation with ophthalmoscopy, fluorescein angiography (FA), and optical coherence tomography (OCT). First performed intravitreal triamcinolone acetonide (IVTA) (4 mg/0.1 mL) and laser photocoagulation to treat the vascular component. There was a rapid and good response after that therapy, but a recurrence 3 months later. To relieve the glial component, we simultaneously combined vitrectomy with IVTA and laser photocoagulation. After the combination therapy, there were no recurrences or complications. A combination therapy of vitrectomy, laser photocoagulation, and intravitreal triamcinolone could be considered as a possible management for CHRRPE with the vascular and glial components.
Vitrectomy, Laser Photocoagulation, and Intravitreal Triamcinolone for Combined Hamartoma of the Retina and Retinal Pigment Epithelium
From the Department of Ophthalmology, Gachon University Gil Hospital, Incheon, Korea.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Dong Heun Nam, MD, PhD, Department of Ophthalmology, Gachon University Gil Hospital, # 1198, Kuwol-dong, Namdong-ku, Incheon 405-760, Korea.
Accepted: July 20, 2009
Posted Online: March 09, 2010
Combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) is a rare, benign condition, characterized by varying amounts of pigmentation, retinal vascular tortuosity, and epiretinal membrane related to vitreomacular traction.1–3 It contains a mixture of neuroseonsory retina, retinal pigment epithelium, retinal vessel, and glial tissue. Subretinal and intraretinal exudation derived from the vascular component of the lesion, or vitreomacular traction associated with epiretinal membrane formation may result in visual loss. There are no established managements for CHRRPE. Some studies demonstrated visual improvement after surgical removal of the epiretinal membrane of the lesion,4,5 and another study suggested photodynamic therapy with Verteporfin for the vascular leakage from the lesion.6 However, controversy exists concerning the benefits of those treatment modalities. We introduce a combination therapy involving vitrectomy and epiretinal membrane peeling for the glial component, and intravitreal triamcinolone acetonide (IVTA) and laser photocoagulation for the vascular component in a patient with CHRRPE.
A 32-year-old man was referred to our department with decreased vision in the right eye. Best-corrected visual acuity (BCVA) in decimal was 0.3 in the right eye and 1.0 in the left eye by Snellen chart. The left eye was normal. Funduscopic examination of the right eye revealed a slightly elevated, grayish white lesion with macular folds involving the posterior pole and the disc (Fig. 1A). Fluorescein angiography (FA) demonstrated early marked tortuosities of the macular vessels with late profound leakage (Fig. 2A). Optical coherence tomography (OCT) showed an elevated, hyperreflective lesion with cystoid macular edema (CME) (Fig. 3A). The central foveal thickness was 421 μm in the right eye and 162 μm in the left eye. The maximal thickness of the extrafoveal lesion was 461 μm. At the first examination, we have not chosen more invasive modalities such as vitrectomy and epiretinal membrane peeling, but less invasive modalities such as IVTA and laser photocoagulation. We just performed IVTA (4 mg/0.1 mL) and macular laser photocoagulation to treat vascular leakage and cystoid macular edema. One month after the treatment, BCVA had increased to 0.6 in the right eye. FA and OCT showed minimal leakage and CME, and the central foveal thickness was 298 μm. The maximal thickness of the extrafoveal lesion was 347 μm (Fig. 3B). Three months after the treatment, however, BCVA was aggravated to 0.2. FA and OCT revealed recurrence of vascular leakage and CME. The central foveal thickness and the maximal thickness of the extrafoveal lesion were 446 μm and 486 μm, respectively. (Fig. 3C). Therefore, we planned a combination therapy involving vitrectomy with membrane peeling for the tractional epiretinal membrane, and IVTA and laser photocoagulation for the vascular leakage and macular edema. During 23-gauge sutureless vitrectomy, there was no posterior vitreous detachment, and we detached the posterior hyaloid membrane and peeled the epiretinal membrane involving macular and peripapillary area. We realized that the epiretinal and internal limiting membranes could be easily peeled from the retina, even though faint bleeding (Fig. 4A). The endo-laser photocoagulation was applied to the membrane-peeled area. At the end of surgery, IVTA (4 mg/0.1 mL) was done. Histopathologically, the lesion was mixture of fibrocyte and macrophage without any tissue from retina (Fig. 4B). Twelve months after the combination therapy, the vision (0.6) was stable and there were no specific adverse events (Fig. 1B). FA and OCT showed the absence of vascular leakage and hyperreflective membrane. The central foveal thickness and the maximal thickness of the extrafoveal lesion were 238 μm and 276 μm respectively (Figs. 2B and 3D).
Figure 1. (A) At the First Examination, Fundus Photograph Showing a Slightly Elevated, Grayish White Lesion with Macular Folds Involving the Posterior Pole and the Disc. (B) At the Last Examination, Fundus Photograph Showing Successful Removal of the Epiretinal Membranes and Laser Photocoagulation Scars.
Figure 2. (A) At the First Examination, Fluorescein Angiogram Showing Severe Vascular Tortuosity and Leakage. (B) At the Last Examination, Fluorescein Angiogram Showing Absence of Vascular Leakage.
Figure 3. (A) At the First Examination, Optical Coherence Tomogram (OCT) Showing an Elevated, Hyperreflective Lesion with Cystoid Macular Edema (CME). The Central Foveal Thickness (CFT) and the Maximal Thickness of the Extrafoveal Lesion (EFTMax) Were 421 μm and 461 μm, Respectively. (B) One Month After Intravitreal Triamcinolone Acetonide (IVTA) and Laser Photocoagulation (LP), OCT Showing Absence of CME. CFT Was Reduced to 298 μm and EFTMax Was 347 μm. (C) Three Months After IVTA and LP, OCT Showing Recurrence of CME. CFT and EFTMax Were 446 μm and 486 μm Respectively. (D) Twelve Months After Vitrectomy, IVTA, and LP, OCT Showing Absence of CME and Hyperreflective Membrane. CFT and EFTMax Were Reduced to 238 μm and 276 μm.
Figure 4. (A) Intraoperative Photograph Showing the Relieved Epiretinal Membrane from the Retinal Surface. (B) Light Microscopic Photograph Showing the Mixture of Fibrocyte and Macrophage Without Any Tissue from Retina.
There are two clinically significant components (vascular and glial) in CHRRPE, related to visual loss and symptom. In our opinion, it would be great if improved just with less invasive management for the vascular component, without surgical approach for the glial. IVTA and macular laser photocoagulation has become an increasingly used therapy for diabetic macular edema, because of rapid improvement and maintenance of vision.7 Recently, bevacizumab, a monoclonal antibody against vascular endothelial growth factor (VEGF), has been widely used as an anti-angiogenic therapy. The anti-angiogenic activity of TA may be mediated not through a VEGF pathway but through the ability to inhibit inflammation.8 TA has also known to inhibit the proliferation of RPE cells, glial cells, inflammatory cells and fibroblasts significantly.9 Therefore, TA could be more beneficial than bevacizumab in CHRRPE. In our case, there was a rapid and good response after IVTA and laser photocoagulation, but a recurrence 3 months later. We realized that the epiretinal and internal limiting membranes could be easily peeled from the retina. These results suggest that non-surgical management such as IVTA and laser photocoagulation might be enough for the vascular component in CHRRPE, and the glial component was surgically extrinsic to the retina. To relieve the traction component, we combined vitrectomy and membrane peeling. There was no recurrence and complication during the first year after the combination therapy. A combination therapy of vitrectomy, laser photocoagulation, and intravitreal triamcinolone could be considered as a possible management for CHRRPE with the vascular and glial components.
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