scholarly journals Surgery for Combined Hamartoma of the Retina and Retinal Pigment Epithelium

2021 ◽  
pp. 778-783
Author(s):  
Charlotte Maria van der Sommen ◽  
Saskia Helena Margaretha van Romunde ◽  
Koen-Willem Adriaan van Overdam

There is no consensus on whether and when surgical treatment is indicated for combined hamartoma of the retina and retinal pigment epithelium (CHRRPE). We aim to discuss the benefits of surgical intervention and techniques that may improve the outcome. A 24-year-old man experienced progressive visual loss for 6 months in his left eye due to CHRRPE. At presentation, visual acuity was 1.3 LogMAR and fundoscopy revealed extensive tractional pre- and epiretinal membranes, subretinal exudation, and a vasoproliferative tumor in the inferior periphery. A complete vitrectomy was performed, while paying special attention to vitreous shaving at the vitreous base and removal of vitreoschisis-induced vitreous cortex remnants (VCR) from the retinal surface posterior to the vitreous base. Tractional membranes and internal limiting membrane were peeled, and the vasoproliferative tumor was excised. Silicone oil tamponade was removed 11 weeks after surgery. No intra- or postoperative complications occurred. Visual acuity improved to 0.8 LogMAR and remained stable for 48-month follow-up. Vitreoretinal surgery can prevent complications that occur with CHRRPE. In addition, visual function may improve even if the initial visual acuity is low. Timely and complete vitrectomy with extensive membranectomy and detection and removal of VCR is recommended to avoid complications in challenging CHRRPE.

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
José L. Sánchez-Vicente ◽  
Miguel Contreras-Díaz ◽  
Trinidad Rueda ◽  
Enrique Rodríguez de la Rúa-Franch ◽  
Fredy E. Molina-Socola ◽  
...  

Purpose. To describe the case of spontaneous resolution of epiretinal membrane in a patient with Combined Hamartoma of the Retina and Retinal Pigment Epithelium (CHR-RPE), in the clinical context of Gorlin Syndrome (GS).Methods. Observational case report of a 12-year-old female patient is presented. The diagnosis of CHRRPE was made by OCT and fundus examination, which showed a mound of disorganized tissue originating from retina and retinal pigment epithelium. Epiretinal membrane (EM) was also detected. Genetic study was performed to confirm the diagnosis of GS.Results. The patient was observed for 39 months, showing spontaneous resolution of the traction caused by the EM and improvement in visual acuity (VA), which was 20/80 at initial presentation, rising to 20/40 after follow-up period.Conclusions. The presence of EM in CHR-REP is a cause of reduction of visual acuity. Management of this condition is controversial; however, we would like to highlight that spontaneous resolution of the traction caused by EM is possible, resulting in recovery of VA.


2017 ◽  
Vol 27 (2) ◽  
pp. 249-252 ◽  
Author(s):  
Stefano De Cillà ◽  
Micol Alkabes ◽  
Paolo Radice ◽  
Elisa Carini ◽  
Carlos Mateo

Purpose To describe a case series including 4 patients undergoing direct transretinal aspiration of subfoveal perfluorocarbon liquid (PFCL) and internal limiting membrane (ILM) peeling after macula-off retinal detachment surgery. Methods Four patients who had undergone vitreoretinal surgery due to primary rhegmatogenous retinal detachment were further treated because of retained subfoveal PFCL. Direct transretinal aspiration of PFCL through a self-sealing foveal retinotomy was performed in all cases using a 41-G needle placed on the top of the bubble. The ILM was peeled off prior to and after PFCL removal in 2 cases, respectively. Optical coherence tomography (OCT) scans were obtained preoperatively and postoperatively to assess the status of the macula. Results Subfoveal PFCL was successfully removed in all cases. Two patients had silicone oil tamponade at the time of the second surgery, which was temporarily removed in both cases and then reapplied in one. Best-corrected visual acuity improved in all cases. No postoperative macular hole was observed by OCT. Conclusions Direct transretinal aspiration of subfoveal PFCL with a 41-G cannula combined with conventional ILM peeling is a safe and effective technique to avoid long-term damage to the retinal layers with good functional outcomes. Performing the ILM peeling immediately before or after the PFCL aspiration does not seem to influence anatomic results.


Retina ◽  
2009 ◽  
Vol 29 (6) ◽  
pp. 825-830 ◽  
Author(s):  
AARON D. COHN ◽  
POLLY A. QUIRAM ◽  
KIMBERLY A. DRENSER ◽  
MICHAEL T. TRESE ◽  
ANTONIO CAPONE

2005 ◽  
Vol 15 (2) ◽  
pp. 289-291 ◽  
Author(s):  
D. Tognetto ◽  
C. Haritoglou ◽  
A. Kampik ◽  
G. Ravalico

Purpose To describe the occurrence of massive macular edema and visual loss after indocyanine green-assisted (ICG) macular pucker surgery. Methods/Results A 74 years old female presented with a macular pucker and a hypertrophy of the retinal pigment epithelium (RPE) in her left eye. The preoperative visual acuity (VA) was 20/100. Surgery consisted of cataract extraction, lens implantation and standard pars plana vitrectomy with peeling of epiretinal tissue followed by the removal of the internal limiting membrane (ILM) remnants stained using a 0.05% ICG solution. One day after surgery, VA was counting fingers. There was an extensive macular edema and retinal thickening with hyperfluorescence during fluorescein angiography and pronounced autofluorescence using ICG filters. During follow up, the macular edema resolved completely, but VA decreased to 20/800 at six months postoperatively. There was a central scotoma and unstable fixation seen during microperimetry. Discussion This case report indicates that ICG might come into contact with bare retina if injected following removal of epiretinal membranes. Whether the observed RPE hypertrophy might have contributed to the pathogenesis of the adverse effect described remains hypothetical.


2008 ◽  
Vol 18 (2) ◽  
pp. 304-308 ◽  
Author(s):  
S.C.B. Teoh ◽  
E.J. Mayer ◽  
R.J. Haynes ◽  
R.H.B. Grey ◽  
A.D. Dick ◽  
...  

Purpose To report the management and outcome of retinal reattachment surgery in retinochoroidal coloboma. Methods Four patients with retinochoroidal colobomata presented to the Bristol Eye Hospital (a UK tertiary referral center for vitreoretinal surgery) with retinal detachment. INTERVENTION. All were type II colobomatous detachments (three patients with type IIB, one patient with type IID). All eyes underwent vitrectomy with endolaser and/or cryotherapy and three eyes underwent scleral buckling. Two eyes had internal tamponade with gas (SF6, C3F8) while the other two had silicone oil. Endolaser was applied over healthy retinal pigment epithelium. Results At last follow-up, all (100%) remained attached, with no recurrences. Three patients achieved visual acuity of 6/120 or better and were able to perform satisfactory near work with appropriate magnifiers. The last patient began with hand movement vision and retained similar vision but subjectively felt more navigational. Conclusions Good anatomic and functional outcomes can be achieved in this patient group with combined vitrectomy with or without scleral buckling surgery. Endolaser retinopexy is effective over healthy RPE at the margin of the coloboma combined with either gas or oil internal tamponade.


2017 ◽  
Vol 27 (2) ◽  
pp. e32-e34 ◽  
Author(s):  
Andrea Mazzaferro ◽  
Adriano Carnevali ◽  
Ilaria Zucchiatti ◽  
Lea Querques ◽  
Francesco Bandello ◽  
...  

Purpose To evaluate the optical coherence tomography angiography (OCT-A) features of a peripapillary intrachoroidal cavitation (ICC) in a patient with high myopia. Methods A 67-year-old woman with ICC underwent visual acuity testing, refraction, slit-lamp biomicroscopy, dilated fundus examination, and OCT-A. The main findings are described in this case report. Results Best-corrected visual acuity was 20/20 in both eyes. Fundus examination revealed in the right eye a macular scar, a tilted disc along with a peripapillary staphyloma, and an orange-yellowish lesion on the inferior border of the disc. Structural OCT B-scan showed ICC as an intrachoroidal hyporeflective space located below the normal plane of the retinal pigment epithelium adjacent to the optic nerve head. Optical coherence tomography angiography showed the cavitation as a hyporeflective area, devoid of detectable flow from the choriocapillaris and large choroidal vessels layers, suggesting the choroid, including the residual hyperreflective tissue in the outer aspect of the retinal pigment epithelium/Bruch membrane, to be avascular in ICC. Conclusions Optical coherence tomography angiography demonstrated the absence of choroidal and choriocapillary network.


2020 ◽  
pp. 112067212092686
Author(s):  
Sebile Çomçalı ◽  
Pınar Topcu Yılmaz ◽  
Cemal Çavdarlı ◽  
Mehmet Numan Alp

Introduction Iodine deficiency is a leading cause of preventable physical and mental retardation. Potassium iodate is used for iodine supplementation to prevent iodine deficiency. We herein report a case of toxic retinopathy following intentional ingestion of potassium iodine. Case Presentation A 41-year-old male presented with a 5-day history of blurred vision in both eyes. His visual acuity (VA) was hand motion and his pupillary reactions were sluggish bilaterally. The fundus examination revealed bilaterally diffuse retinal pigment epithelium atrophy and secondary pigmentary changes at the posterior pole, but his peripheral fundus was relatively spared. Choroidal thinning, punctate hyperreflective dots along the retinal pigment epithelium layer, and outer retinal atrophy were the optical coherence tomography findings, which were consistent with widespread areas of retinal pigment epithelium window defects observed on fundus fluorescein angiography. The visual evoked potential test showed no response in the right eye and revealed a delay in the latency and a decrease in the amplitude of the P100 wave in the left eye. Wave b responses of the photoreceptors could not be observed in the patient’s electroretinogram. After a vitamin supplementation protocol consistent with the literature, at the 4-month follow-up visit his visual acuity had improved to 0.3 in the right eye and counting fingers in the left eye. Conclusion Potassium iodate toxicity is a cause of serious retinal and choroidal damage and results in severe vision loss. Hydration, hemodialysis, and antioxidants can be helpful to minimize the complications.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Xin Wang ◽  
Ting Zhang ◽  
Rui Jiang ◽  
Gezhi Xu

Abstract Background To report the structure and visual outcomes of pars plana vitrectomy (PPV) for laser-induced full-thickness macular holes (MHs). Methods This retrospective study enrolled 10 patients who underwent vitrectomy for MHs caused by laser injury. Best corrected visual acuity (BCVA), macular spectral-domain optical coherence tomography (OCT) and OCT angiography (OCTA) were used for assessment. Results Four patients were injured by unexpected expose of an yttrium aluminum garnet (YAG) laser, and six patients were accidentally injured by a handheld laser. The MH minimum diameters (MDs) ranged from 55 to 966 μm (mean = 548.00 ± 286.10 μm), and BCVA ranged from 20/400 to 20/50 (mean = logMAR 0.87 ± 0.29) preoperatively. All 10 eyes underwent PPV, internal limiting membrane (ILM) peeling, and gas tamponade. All eyes demonstrated closure of the MH with different degrees of discontinuity of the outer layer of the retina, and four eyes exhibited serious retinal pigment epithelium (RPE) destruction. Postoperative BCVA values were significantly improved (mean = logMAR 0.55 ± 0.33; P = 0.032, t = 2.234). The mean BCVA of the destroyed RPE group was significantly worse than that of the non-destroyed RPE group both before and after surgery (P = 0.019; Wilcoxon signed rank test). Further, OCTA indicated choroidal ischemia in the laser-induced MHs. Conclusion Vitrectomy can be successful in closing laser-induced full-thickness MHs and improving visual acuity. However, If RPE/choroid is involved in laser damage in addition to the outer retinal layer, this may indicate poor visual prognosis.


Choroideremia is X-linked chorioretinal dystrophy characterized by progressive degeneration of the choroid, retinal pigment epithelium (RPE), and retina. The disease is caused by mutations in the CHM gene which is known to be related to membrane transportation protein in the retina and RPE. Male-affected cases have nyctalopia and progressive reduction in visual acuity. Female-affected cases are carriers. This disease is considered incurable, although new promising treatments have been recently introduced such as gene therapy, stem cells, small molecules, and retinal prosthesis.


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