PARS PLANA VITRECTOMY WITH INTERNAL LIMITING MEMBRANE REMOVAL IN TYPE 2 IDIOPATHIC MACULAR TELANGIECTASIA Eric J. Sigler, MD, John C. Randolph, MD, Jorge I. Calzada, MD, Steve Charles, MD
Purpose: To report the anatomical and visual outcomes in a series of patients with type 2 idiopathic macular telangiectasia (mactel) and cystic foveal changes treated with pars plana vitrectomy and internal limiting membrane removal. Methods: Five eyes of 3 patients with mactel were treated with 25-gauge sutureless transconjunctival pars plana vitrectomy and internal limiting membrane peeling. Complete clinical examination, including serial spectral-domain optical coherence tomography, was performed preoperatively and postoperatively. Results: Two of 5 eyes had stable vision at 2-year follow-up with continued evidence of foveal cystic spaces on spectral-domain optical coherence tomography. Two eyes experienced significant visual improvement despite continued evidence of foveal anatomical disruption. One eye experienced slight visual decline and full-thickness macular hole formation. Conclusion: Surgical intervention involving pars plana vitrectomy with internal limiting membrane peeling may be a treatment option for select cases of mactel. Pars plana vitrectomy does not seem to alter the underlying disease process in mactel but may stabilize visual decline or lead to improved vision in some patients. RETINAL CASES & BRIEF REPORTS 0:1–6, 2013
From the Division of Vitreoretinal Surgery, Charles Retina Institute, Memphis, Tennessee.
telangiectatic vs. occlusive).4 The telangiectatic form, or type 2, has been reported to occur in 0.1% of the U. S. population,5 have a mean age at diagnosis in the fifth to sixth decade,5,6 typically presenting bilaterally often with asymmetric involvement,1–4 and has no apparent gender predilection.2,4–6 The most common clinical findings in mactel involve nonproliferative changes, including angiographic perifoveal vascular leakage, degenerative cystic spaces, perifoveal telangiectatic vessels most prominent in the temporal macula, and an overall gray sheen to the central macula or loss of central macular transparency.2–4 Proliferative changes can rarely occur and include intraretinal and subretinal neovascularization.3,4 Cystic macular changes,4 lamellar macular holes (LMH),7,8 and the occasional occurrence of full-thickness macular holes (FTMH)9,10 have been reported in mactel and generally are considered poor candidates for surgery because of the underlying degenerative process and chronic vascular leakage. However, there are some reports of attempted surgical treatment of
T
ype 2 idiopathic macular telangiectasia (mactel) is a rare, bilateral retinal disorder involving telangiectatic perifoveal vasculature typically diagnosed in mid to late adult life of unknown etiology. First termed idiopathic juxtafoveal telangiectasis, macular telangiectasia was described by Gass in 19681 and was subsequently classified further by both Gass2,3 and Yannuzzi et al.4 The original classification differentiated forms of idiopathic retinal telangiectasis based on angiography, laterality, morphology, and age at diagnosis. A simplified classification was later proposed based on vascular morphology (aneurysmal vs.
None of the authors have any financial/conflicting interests to disclose. Reprint requests: Eric J. Sigler, MD, Charles Retina Institute, 6401 Poplar Avenue, Suite 190, Memphis, TN 38119; e-mail: [email protected]
1
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LMH and macular hole (MH) in mactel.10–14 The purpose of the present article was to report our experience with anatomical and visual outcomes in a small series of eyes treated with 25-gauge, transconjunctival, pars plana vitrectomy (PPV) with internal limiting membrane (ILM) peeling in mactel involving inner retinal cystic spaces with LMH configuration. Methods All patients and data were treated in accordance with the Declaration of Helsinki. Cases included consecutive patients presenting between October 2009 and January 2012 prospectively evaluated at a single vitreoretinal referral center (Charles Retina Institute, Memphis, TN). This report was exempt from institutional review board approval as a three-patient case series. All patients were diagnosed with mactel and inner retinal cystic changes based on binocular indirect ophthalmoscopy, fundus photography, fluorescein angiography (FA), and spectral-domain optical coherence tomography (SD-OCT) studies (Spectralis; Heidelberg Engineering, Heidelberg, Germany). Patients with foveal inner retinal cystic spaces resulting from mactel and features of LMH configuration present on SD-OCT as well as symptomatic metamorphopsia was considered for surgical intervention only after extensive counseling and guarded prognosis for significant visual improvement. Although we did not intend to specifically exclude patients with concomitant systemic disease, the patients in the present series had no history of hypertension, diabetes, or other systemic or ocular disease. In all patients, surgical technique involved 25-gauge, transconjunctival PPV (Constellation; Alcon, Fort Worth, TX). Complete posterior hyaloid removal and forceps ILM peeling without staining were performed followed by 25% sulfur hexafluoride gas tamponade and postoperative face-down positioning for 7 days. All patients remained phakic throughout the follow-up period presented and had slight progression of preexisting nuclear sclerosis that was not considered visually significant. All procedures were performed by a single surgeon (S.C.). Case Reports Case 1 A 57-year-old white woman with a history of amblyopia in her right eye presented with complaints of metamorphopsia in her left eye. Examination revealed best-corrected Snellen visual acuity (BCVA) of 20/50 in the right eye and 20/30 in the left eye. Fundus examination revealed abnormal foveal reflex, temporal perifoveal
gray sheen, several superficial intraretinal yellow flecks, and telangiectatic vessels crossing the horizontal raphe in the temporal macula of both eyes. Fluorescein angiography demonstrated characteristic temporal perifoveal telangiectatic vessels and intraretinal leakage typical of mactel in both eyes. Imaging is presented in Figure 1. Spectral-domain optical coherence tomography studies demonstrated inner lamellar hyporeflectant foveal cystic spaces in both eyes. After counseling with guarded prognosis for visual improvement, the patient underwent PPV with sulfur hexafluoride (SF6) gas tamponade in the left eye. At 1-month postoperative visit, BCVA was 20/70 and SD-OCT revealed absence of foveal ILM and an area of temporal foveal subretinal hyporeflectant space in the left eye. Serial follow-up examinations demonstrated continued improvement in BCVA and subjective improvement in metamorphopsia. At 6 months of follow-up, BCVA had improved to 20/20, metamorphopsia was resolved, and SD-OCT demonstrated decreased subretinal hyporeflectant spaces, rare amounts of outer retinal tissue within the central fovea, and loss of inner segment– outer segment line. Despite these anatomical findings, BCVA is 20/20 at 24-month follow-up and remains stable at 20/50 in the right eye. No additional treatments were undertaken.
Case 2 A 54-year-old white man presented with complaints of progressive decrease in vision for several years. Examination demonstrated BCVA of 20/25, right eye, and 20/40, left eye. Fundus examination revealed temporal macular gray sheen and telangiectatic vessels crossing the horizontal raphe in both eyes and a central yellow spot in the fovea of the right eye. Imaging is presented in Figure 2. The SD-OCT revealed LMH in the right eye with only ILM and a thin strand of continuous outer retinal tissue. The SD-OCT revealed inner retinal cystic spaces and subfoveal RPE atrophy in the left eye. After counseling with guarded prognosis for visual improvement, the patient underwent PPV with SF6 in the right eye. One-month postoperative visit revealed BCVA of 20/30, right eye, and 20/40, left eye. The SD-OCT examination revealed partial MH closure in the right eye and continued cystic changes in the left eye. Three-month follow-up examination demonstrated BCVA of 20/30, right eye, and 20/50, left eye. Fundus examination was unchanged in the right eye but demonstrated increased size of cystic spaces in the left eye; therefore, the patient underwent PPV with SF6 in the left eye. Follow-up examination 1 month later revealed BCVA of 20/30, right eye, and 20/50, left eye. The SD-OCT demonstrated FTMH in the left eye and small subfoveal cystic spaces in the right eye. The patient remains stable with BCVA of 20/30, right eye, and 20/50, left eye, at 22-month followup. No additional interventions were performed.
Case 3 A 62 year-old white man presented with complaints of gradually progressive decreased central vision and central metamorphopsia for several months before in both eyes. BCVA was 20/70, right eye, and 20/40, left eye. Fundus examination revealed foveal cystic changes in the right eye and telangiectatic vessels in the temporal perifoveal region of both eyes. Imaging is demonstrated in Figure 3. The SD-OCT revealed loss of inner nuclear layer, hyperreflectant midretinal flecks, and outer nuclear layer disruption in the right eye and inner lamellar cyst and thinning of the subfoveal outer nuclear layer in the left eye. After counseling with guarded prognosis for visual improvement, the patient underwent PPV with SF6 OS. One-month follow-up examination revealed BCVA of 20/60 in the left eye and stable in the right eye. The SD-OCT examination demonstrated
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PPV FOR MACTEL
Fig. 1. Case 1: Fluorescein angiography and SD-OCT of a patient with mactel treated with PPV with ILM removal. A. Arteriovenous phase FA of the right eye demonstrating telangiectatic retinal capillaries and central macular intraretinal leakage most prominent in the temporal perifoveal region. B. Arteriovenous phase FA of the left eye demonstrating similar findings as in (A) with pinpoint fluorescein pooling within an intraretinal space. C. The SD-OCT of the right eye at presentation demonstrating inner foveal cystic space and attenuation of the parafoveal inner nuclear layer; there is apparent elevation of the inner segment–outer segment (IS/OS) line temporally; visual acuity = 20/50; D. The SD-OCT of the left eye at presentation demonstrating subretinal hyporeflectant space, IS/OS disruption, inner retinal cystic space, and thickened ILM spanning the inner retinal surface; visual acuity = 20/30; E. The SD-OCT of the right eye at 2-year follow-up without intervention; the inner retinal cystic space persists with marginal decrease in size, there is IS/OS disruption in the previous area of subtle elevation, and vision is stable without intervention. F. Two-year postoperative SD-OCT of left eye after PPV; the epimacular membrane has been removed, despite enlarged subretinal space, IS/OS disruption, and attenuated outer retinal layers; visual acuity = 20/20.
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reduction in the size of the inner retinal cystic space. Six-month follow-up found BCVA to have increased from baseline to 20/40 in the left eye but decreased to 20/80 in the right eye. The patient elected to undergo PPV with SF6 in the right eye. Three-month postoperative visit revealed visual improvement of 20/40 in the right eye with decreased cystic spaces on OCT and stable BCVA of 20/40 in the left eye. BCVA remains stable at 26-month follow-up examination. No additional interventions were performed.
Discussion Our results indicate that mixed anatomical and visual results may occur after PPV with ILM peeling for LMH in mactel. Although three of five eyes operated had stable or slight visual decline, two patients experienced significant visual recovery despite SD-OCT evidence of continued anatomical disruption and hyporeflectant spaces within the fovea. No patients experienced dramatic visual loss, additional visual loss, or neovascular events within the mean 2-year follow-up period, and no additional treatments were performed. This seems to confirm previous investigations involving PPV with ILM peeling in mactel patients with MH. Gregori and Flynn13 reported two patients, one of whom experienced visual improvement and MH closure and one in whom the MH initially closed only to reopen with
subsequent visual decline. Sandhu and Steel14 reported a case of mactel associated with fine epimacular membrane that experienced visual improvement in both eyes following PPV and ILM removal and noted incomplete posterior hyaloid separation identified intraoperatively in both treated eyes. In contrast, Rishi et al11 reported a case of LMH formation with vitreomacular traction in mactel that persisted, although decreased in size after PPV with ILM removal and was associated with a decline in BCVA. Charbel Issa et al,12 in perhaps the largest series of MH in mactel, reported PPV in two eyes of six patients with LMH, one of whom had a complete posterior hyaloid detachment at surgery and one in whom vitreomacular attachment was identified intraoperatively. Neither of these patients had complete anatomical closure of the LMH, but the patient with attached posterior hyaloid removed at surgery experienced a slight visual improvement.12 Shukla and Venkatesh15 reported FTMH in mactel that experienced spontaneous closure, and one that closed with improved BCVA following PPV and ILM removal.10 Although mactel may theoretically represent a subset of vitreomacular traction that affects a characteristic area of the temporal macula, this seems unlikely, given the absence of obvious focal vitreomacular traction in the area of leakage in many reports, the predominance
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Fig. 2. Case 2: Fluorescein -angiography and SD-OCT of a patient with mactel treated with bilateral PPV and ILM removal. A. Early arteriovenous phase FA of the right eye demonstrating perifoveal telangiectatic retinal capillaries and central macular intraretinal leakage most prominent in the temporal perifoveal region. B. Mid-arteriovenous phase FA of the left eye demonstrating similar findings as in (A). C. Preoperative SD-OCT of the right eye demonstrating subfoveal inner segment–outer segment (IS/OS) disruption and near-complete absence of foveal tissue resembling LMH without foveal inversion or significant epimacular membrane; a thin strand of inner retinal and outer retinal tissue spans the central defect; visual acuity = 20/25. D. Preoperative SD-OCT of the left eye demonstrating inner retinal cystic space and subfoveal IS/OS disruption; visual acuity = 20/40. E. Post-PPV SD-OCT of the right eye reveals absence of the previously seen inner retinal surface tissue and increased continuity of outer retinal tissue; the area of IS/OS disruption seems unchanged; visual acuity = 20/30. F. Post-PPV SD-OCT of the left eye demonstrates FTMH with slight elevation of the nasal border; visual acuity = 20/50.
of mid and deep retinal changes noted on SD-OCT, and the intraretinal leakage seen angiographically. Although the pathogenesis of mactel remains, to our knowledge, obscure, proposed mechanisms include progressive capillary endothelial cell degeneration, ischemic changes in watershed zones, and a primary neurodegenerative process. Current theories regarding the pathogenesis of idiopathic LMH and FTMH include vitreoretinal traction, opening of foveal cysts resulting from macular edema, and tangential traction caused by an epiretinal membrane.16–18 Koizumi et al9 hypothesized that MH formation in mactel is the result of the separation of neural tissue but that the retained vision in their series precluded the possibility of significant photoreceptor atrophy. This would seem to confirm the visual and anatomical results in cases 1 and 3 in the present series. It is important to note that the definition of “lamellar macular hole” may have significant overlap, as imaged via OCT, with inner lamellar cysts or “empty spaces” that are commonly described in mactel, and previous investigations10–14 have predominantly used time-domain OCT. Although the characteristic findings of epimacular membrane, foveal inversion, and vitreomacular traction may differentiate the two entities, the overlapping morphology and additional factors (such as various resolution of imaging devices) may affect the interobserver report
rate and clinical decision making regarding such phenomena. As previously reported,19 we hypothesize that that the cystic spaces observed in mactel result from chronic capillary endothelial incompetence with waxing and waning amounts of intraretinal fluid resulting in separation of neurosensory tissue elements. The characteristic finding of the predominance of inner retinal separation, non–outer plexiform morphology, often with a thin veil of epiretinal tissue (presumably ILM) in the absence of significant foveal effacement and epiretinal membrane may offer insight into the nature of the underlying pathogenesis and seems pathognomonic for mactel. Subsets of patients may have additional risk factors, including vitreofoveal traction and epimacular membrane, that further contribute to loss of foveal contour and MH formation. Therefore, the relief of these factors may facilitate the reformation of a foveal anatomy compatible with improved vision but does not alter the underlying disease process, which we believe is perifoveal vascular incompetence. The mechanism of visual improvement in the cases demonstrated herein remains obscure but may relate to the achievement of a foveal contour more consistent with decreased metamorphospia, such as case 2, in the right eye. There have been few histologic reports of mactel,20–22 but microscopic findings seem to include
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PPV FOR MACTEL
Fig. 3. Case 3: Fluorescein angiography and SD-OCT of a patient with mactel and bilateral PPV with ILM removal. A. Early arteriovenous phase FA of the right eye demonstrating perifoveal telangiectatic retinal capillaries and central macular intraretinal leakage most prominent in the temporal perifoveal region. B. Mid-arteriovenous phase FA of the left eye demonstrating similar findings as in (A). C. Preoperative SD-OCT of the right eye demonstrating multiple intraretinal cystic spaces, loss of subfoveal inner segment–outer segment (IS/OS) line, and temporal hyperreflectant nodule; visual acuity = 20/80. D. Preoperative SD-OCT of the left eye demonstrating multiple subfoveal cystic spaces and a thin veil of tissue spanning the inner retinal surface, presumably ILM; visual acuity = 20/40. E. PostPPV SD-OCT demonstrating decrease in intraretinal cystic spaces, overall retinal thinning, and continued IS/OS disruption; visual acuity = 20/40. F. Post-PPV SD-OCT at 26 months postoperative examination demonstrates atrophy of all retinal layers and thin subfoveal subretinal hyporeflectant space; visual acuity = 20/40.
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capillary pericyte loss, reduction in macular pigment, depletion of type IV collagen, and Müller cell dropout. As Müller cells become depleted, alteration in tangential forces present within the ILM in the parafoveal region may lead to distortion of remaining photoreceptors, which is one possible mechanism by which ILM removal may accomplish visual improvement. In addition, as blood–retina barrier breakdown leads to intraretinal fluid leakage; ILM removal may remove a diffusion barrier and allow transudation into vitreous, thereby reducing the amount of extracellular fluid. In patients not experiencing visual improvement, or developing progression of retinal cavitation, ILM peeling may remove the only remaining, thin inner retinal tissue leading to the development of a FTMH. Patients with visual decline may also experience progression of underlying degeneration after mechanical manipulation of Müller cells with ILM peeling. Pars plana vitrectomy with ILM peeling may require additional investigation, possibly as a component of a clinical trial. We conclude that surgical intervention may be an option for select cases of mactel after careful consideration, especially in those with LMH or SD-OCT evidence of epimacular membrane. Key words: idiopathic juxtafoveal telangiectasis, idiopathic macular telangiectasia, mactel, IMT, internal limiting membrane peeling, JFT, parafoveal telangiectasia, pars plana vitrectomy.
References 1. Gass JD. A fluorescein angiographic study of macular dysfunction secondary to retinal vascular disease. V. Retinal telangiectasis. Arch Ophthalmol 1968;80:592–605. 2. Gass JD, Oyakawa RT. Idiopathic juxtafoveolar retinal telangiectasis. Arch Ophthalmol 1982;100:769–780. 3. Gass JD, Blodi BA. Idiopathic juxtafoveolar retinal telangiectasis. Update of classification and follow-up study. Ophthalmology 1993;100:1536–1546. 4. Yannuzzi LA, Bardal AM, Freund KB, et al. Idiopathic macular telangiectasia. Arch Ophthalmol 2006;124:450–460. 5. Klein R, Blodi BA, Meuer SM, et al. The prevalence of macular telangiectasia type 2 in the Beaver Dam eye study. Am J Ophthalmol 2010;150:55–62 e52. 6. Aung KZ, Wickremasinghe SS, Makeyeva G, et al. The prevalence estimates of macular telangiectasia type 2: the Melbourne Collaborative Cohort Study. Retina 2010;30: 473–478. 7. Patel B, Duvall J, Tullo AB. Lamellar macular hole associated with idiopathic juxtafoveolar telangiectasia. Br J Ophthalmol 1988;72:550–551. 8. Olson JL, Mandava N. Macular hole formation associated with idiopathic parafoveal telangiectasia. Graefes Arch Clin Exp Ophthalmol 2006;244:411–412. 9. Koizumi H, Slakter JS, Spaide RF. Full-thickness macular hole formation in idiopathic parafoveal telangiectasis. Retina 2007; 27:473–476. 10. Shukla D. Evolution and management of macular hole secondary to type 2 idiopathic macular telangiectasia. Eye (Lond). 2011;25:532–533. 11. Rishi P, Kothari AR. Parafoveal telangiectasia (PFT) has been associated with changes in macular architecture and macular
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12.
13.
14.
15.
16.
RETINAL CASES & BRIEF REPORTS´ 2013 VOLUME 0 NUMBER 0 holes (lamellar and full thickness). Retina 2008;28:184–185; author reply 185–186. Charbel Issa P, Scholl HP, Gaudric A, et al. Macular fullthickness and lamellar holes in association with type 2 idiopathic macular telangiectasia. Eye (Lond) 2009;23:435–441. Gregori N, Flynn HW Jr. Surgery for full-thickness macular hole in patients with idiopathic macular telangiectasia type 2. Ophthalmic Surg Lasers Imaging 2010;41 Online:1–4. Sandhu SS, Steel DH. Comment on macular full-thickness and lamellar holes in association with type 2 idiopathic macular telangiectasia. Eye (Lond) 2010;24:1119. Shukla D, Venkatesh R. Spontaneous closure of full-thickness macular hole in type 2 idiopathic macular telangiectasia. Graefes Arch Clin Exp Ophthalmol 2012;250:1711–1712. Gass JD. Reappraisal of biomicroscopic classification of stages of development of a macular hole. Am J Ophthalmol 1995;119: 752–759.
17. Gaudric A, Haouchine B, Massin P, et al. Macular hole formation: new data provided by optical coherence tomography. Arch Ophthalmol 1999;117:744–751. 18. Smiddy WE, Flynn HW Jr. Pathogenesis of macular holes and therapeutic implications. Am J Ophthalmol 2004;137:525–537. 19. Sigler EJ, Randolph JC, Calzada JI, Charles S. Comparison of observation, inravitreal bevacizumab, or pars plana vitrectomy for non-proliferative type 2 idiopathic macular telangiectasia. Graefes Arch Clin Exp Ophthalmol 2013;251:1097–1101. 20. Green WR, Quigley HA, De la Cruz Z, Cohen B. Parafoveal retinal telangiectasis. Light and electron microscopy studies. Trans Ophthalmol Soc U K 1980;100:162–170. 21. Eliassi-Rad B, Green WR. Histopathologic study of presumed parafoveal telangiectasis. Retina 1999;19:332–335. 22. Powner MB, Gillies MC, Tretiach M, et al. Perifoveal Muller cell depletion in a case of macular telangiectasia type 2. Ophthalmology 2010;117:2407–2416.
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Purpose: To report the anatomical and visual outcomes in a series of patients with type 2 idiopathic macular telangiectasia (mactel) and cystic foveal changes treated with pars plana vitrectomy and internal limiting membrane removal. Methods: Five eyes of 3 patients with mactel were treated with 25-gauge sutureless transconjunctival pars plana vitrectomy and internal limiting membrane peeling. Complete clinical examination, including serial spectral-domain optical coherence tomography, was performed preoperatively and postoperatively. Results: Two of 5 eyes had stable vision at 2-year follow-up with continued evidence of foveal cystic spaces on spectral-domain optical coherence tomography. Two eyes experienced significant visual improvement despite continued evidence of foveal anatomical disruption. One eye experienced slight visual decline and full-thickness macular hole formation. Conclusion: Surgical intervention involving pars plana vitrectomy with internal limiting membrane peeling may be a treatment option for select cases of mactel. Pars plana vitrectomy does not seem to alter the underlying disease process in mactel but may stabilize visual decline or lead to improved vision in some patients. RETINAL CASES & BRIEF REPORTS 0:1–6, 2013
From the Division of Vitreoretinal Surgery, Charles Retina Institute, Memphis, Tennessee.
telangiectatic vs. occlusive).4 The telangiectatic form, or type 2, has been reported to occur in 0.1% of the U. S. population,5 have a mean age at diagnosis in the fifth to sixth decade,5,6 typically presenting bilaterally often with asymmetric involvement,1–4 and has no apparent gender predilection.2,4–6 The most common clinical findings in mactel involve nonproliferative changes, including angiographic perifoveal vascular leakage, degenerative cystic spaces, perifoveal telangiectatic vessels most prominent in the temporal macula, and an overall gray sheen to the central macula or loss of central macular transparency.2–4 Proliferative changes can rarely occur and include intraretinal and subretinal neovascularization.3,4 Cystic macular changes,4 lamellar macular holes (LMH),7,8 and the occasional occurrence of full-thickness macular holes (FTMH)9,10 have been reported in mactel and generally are considered poor candidates for surgery because of the underlying degenerative process and chronic vascular leakage. However, there are some reports of attempted surgical treatment of
T
ype 2 idiopathic macular telangiectasia (mactel) is a rare, bilateral retinal disorder involving telangiectatic perifoveal vasculature typically diagnosed in mid to late adult life of unknown etiology. First termed idiopathic juxtafoveal telangiectasis, macular telangiectasia was described by Gass in 19681 and was subsequently classified further by both Gass2,3 and Yannuzzi et al.4 The original classification differentiated forms of idiopathic retinal telangiectasis based on angiography, laterality, morphology, and age at diagnosis. A simplified classification was later proposed based on vascular morphology (aneurysmal vs.
None of the authors have any financial/conflicting interests to disclose. Reprint requests: Eric J. Sigler, MD, Charles Retina Institute, 6401 Poplar Avenue, Suite 190, Memphis, TN 38119; e-mail: [email protected]
1
Copyright ª by Ophthalmic Communications Society, Inc. Unauthorized reproduction of this article is prohibited.
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LMH and macular hole (MH) in mactel.10–14 The purpose of the present article was to report our experience with anatomical and visual outcomes in a small series of eyes treated with 25-gauge, transconjunctival, pars plana vitrectomy (PPV) with internal limiting membrane (ILM) peeling in mactel involving inner retinal cystic spaces with LMH configuration. Methods All patients and data were treated in accordance with the Declaration of Helsinki. Cases included consecutive patients presenting between October 2009 and January 2012 prospectively evaluated at a single vitreoretinal referral center (Charles Retina Institute, Memphis, TN). This report was exempt from institutional review board approval as a three-patient case series. All patients were diagnosed with mactel and inner retinal cystic changes based on binocular indirect ophthalmoscopy, fundus photography, fluorescein angiography (FA), and spectral-domain optical coherence tomography (SD-OCT) studies (Spectralis; Heidelberg Engineering, Heidelberg, Germany). Patients with foveal inner retinal cystic spaces resulting from mactel and features of LMH configuration present on SD-OCT as well as symptomatic metamorphopsia was considered for surgical intervention only after extensive counseling and guarded prognosis for significant visual improvement. Although we did not intend to specifically exclude patients with concomitant systemic disease, the patients in the present series had no history of hypertension, diabetes, or other systemic or ocular disease. In all patients, surgical technique involved 25-gauge, transconjunctival PPV (Constellation; Alcon, Fort Worth, TX). Complete posterior hyaloid removal and forceps ILM peeling without staining were performed followed by 25% sulfur hexafluoride gas tamponade and postoperative face-down positioning for 7 days. All patients remained phakic throughout the follow-up period presented and had slight progression of preexisting nuclear sclerosis that was not considered visually significant. All procedures were performed by a single surgeon (S.C.). Case Reports Case 1 A 57-year-old white woman with a history of amblyopia in her right eye presented with complaints of metamorphopsia in her left eye. Examination revealed best-corrected Snellen visual acuity (BCVA) of 20/50 in the right eye and 20/30 in the left eye. Fundus examination revealed abnormal foveal reflex, temporal perifoveal
gray sheen, several superficial intraretinal yellow flecks, and telangiectatic vessels crossing the horizontal raphe in the temporal macula of both eyes. Fluorescein angiography demonstrated characteristic temporal perifoveal telangiectatic vessels and intraretinal leakage typical of mactel in both eyes. Imaging is presented in Figure 1. Spectral-domain optical coherence tomography studies demonstrated inner lamellar hyporeflectant foveal cystic spaces in both eyes. After counseling with guarded prognosis for visual improvement, the patient underwent PPV with sulfur hexafluoride (SF6) gas tamponade in the left eye. At 1-month postoperative visit, BCVA was 20/70 and SD-OCT revealed absence of foveal ILM and an area of temporal foveal subretinal hyporeflectant space in the left eye. Serial follow-up examinations demonstrated continued improvement in BCVA and subjective improvement in metamorphopsia. At 6 months of follow-up, BCVA had improved to 20/20, metamorphopsia was resolved, and SD-OCT demonstrated decreased subretinal hyporeflectant spaces, rare amounts of outer retinal tissue within the central fovea, and loss of inner segment– outer segment line. Despite these anatomical findings, BCVA is 20/20 at 24-month follow-up and remains stable at 20/50 in the right eye. No additional treatments were undertaken.
Case 2 A 54-year-old white man presented with complaints of progressive decrease in vision for several years. Examination demonstrated BCVA of 20/25, right eye, and 20/40, left eye. Fundus examination revealed temporal macular gray sheen and telangiectatic vessels crossing the horizontal raphe in both eyes and a central yellow spot in the fovea of the right eye. Imaging is presented in Figure 2. The SD-OCT revealed LMH in the right eye with only ILM and a thin strand of continuous outer retinal tissue. The SD-OCT revealed inner retinal cystic spaces and subfoveal RPE atrophy in the left eye. After counseling with guarded prognosis for visual improvement, the patient underwent PPV with SF6 in the right eye. One-month postoperative visit revealed BCVA of 20/30, right eye, and 20/40, left eye. The SD-OCT examination revealed partial MH closure in the right eye and continued cystic changes in the left eye. Three-month follow-up examination demonstrated BCVA of 20/30, right eye, and 20/50, left eye. Fundus examination was unchanged in the right eye but demonstrated increased size of cystic spaces in the left eye; therefore, the patient underwent PPV with SF6 in the left eye. Follow-up examination 1 month later revealed BCVA of 20/30, right eye, and 20/50, left eye. The SD-OCT demonstrated FTMH in the left eye and small subfoveal cystic spaces in the right eye. The patient remains stable with BCVA of 20/30, right eye, and 20/50, left eye, at 22-month followup. No additional interventions were performed.
Case 3 A 62 year-old white man presented with complaints of gradually progressive decreased central vision and central metamorphopsia for several months before in both eyes. BCVA was 20/70, right eye, and 20/40, left eye. Fundus examination revealed foveal cystic changes in the right eye and telangiectatic vessels in the temporal perifoveal region of both eyes. Imaging is demonstrated in Figure 3. The SD-OCT revealed loss of inner nuclear layer, hyperreflectant midretinal flecks, and outer nuclear layer disruption in the right eye and inner lamellar cyst and thinning of the subfoveal outer nuclear layer in the left eye. After counseling with guarded prognosis for visual improvement, the patient underwent PPV with SF6 OS. One-month follow-up examination revealed BCVA of 20/60 in the left eye and stable in the right eye. The SD-OCT examination demonstrated
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Fig. 1. Case 1: Fluorescein angiography and SD-OCT of a patient with mactel treated with PPV with ILM removal. A. Arteriovenous phase FA of the right eye demonstrating telangiectatic retinal capillaries and central macular intraretinal leakage most prominent in the temporal perifoveal region. B. Arteriovenous phase FA of the left eye demonstrating similar findings as in (A) with pinpoint fluorescein pooling within an intraretinal space. C. The SD-OCT of the right eye at presentation demonstrating inner foveal cystic space and attenuation of the parafoveal inner nuclear layer; there is apparent elevation of the inner segment–outer segment (IS/OS) line temporally; visual acuity = 20/50; D. The SD-OCT of the left eye at presentation demonstrating subretinal hyporeflectant space, IS/OS disruption, inner retinal cystic space, and thickened ILM spanning the inner retinal surface; visual acuity = 20/30; E. The SD-OCT of the right eye at 2-year follow-up without intervention; the inner retinal cystic space persists with marginal decrease in size, there is IS/OS disruption in the previous area of subtle elevation, and vision is stable without intervention. F. Two-year postoperative SD-OCT of left eye after PPV; the epimacular membrane has been removed, despite enlarged subretinal space, IS/OS disruption, and attenuated outer retinal layers; visual acuity = 20/20.
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reduction in the size of the inner retinal cystic space. Six-month follow-up found BCVA to have increased from baseline to 20/40 in the left eye but decreased to 20/80 in the right eye. The patient elected to undergo PPV with SF6 in the right eye. Three-month postoperative visit revealed visual improvement of 20/40 in the right eye with decreased cystic spaces on OCT and stable BCVA of 20/40 in the left eye. BCVA remains stable at 26-month follow-up examination. No additional interventions were performed.
Discussion Our results indicate that mixed anatomical and visual results may occur after PPV with ILM peeling for LMH in mactel. Although three of five eyes operated had stable or slight visual decline, two patients experienced significant visual recovery despite SD-OCT evidence of continued anatomical disruption and hyporeflectant spaces within the fovea. No patients experienced dramatic visual loss, additional visual loss, or neovascular events within the mean 2-year follow-up period, and no additional treatments were performed. This seems to confirm previous investigations involving PPV with ILM peeling in mactel patients with MH. Gregori and Flynn13 reported two patients, one of whom experienced visual improvement and MH closure and one in whom the MH initially closed only to reopen with
subsequent visual decline. Sandhu and Steel14 reported a case of mactel associated with fine epimacular membrane that experienced visual improvement in both eyes following PPV and ILM removal and noted incomplete posterior hyaloid separation identified intraoperatively in both treated eyes. In contrast, Rishi et al11 reported a case of LMH formation with vitreomacular traction in mactel that persisted, although decreased in size after PPV with ILM removal and was associated with a decline in BCVA. Charbel Issa et al,12 in perhaps the largest series of MH in mactel, reported PPV in two eyes of six patients with LMH, one of whom had a complete posterior hyaloid detachment at surgery and one in whom vitreomacular attachment was identified intraoperatively. Neither of these patients had complete anatomical closure of the LMH, but the patient with attached posterior hyaloid removed at surgery experienced a slight visual improvement.12 Shukla and Venkatesh15 reported FTMH in mactel that experienced spontaneous closure, and one that closed with improved BCVA following PPV and ILM removal.10 Although mactel may theoretically represent a subset of vitreomacular traction that affects a characteristic area of the temporal macula, this seems unlikely, given the absence of obvious focal vitreomacular traction in the area of leakage in many reports, the predominance
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Fig. 2. Case 2: Fluorescein -angiography and SD-OCT of a patient with mactel treated with bilateral PPV and ILM removal. A. Early arteriovenous phase FA of the right eye demonstrating perifoveal telangiectatic retinal capillaries and central macular intraretinal leakage most prominent in the temporal perifoveal region. B. Mid-arteriovenous phase FA of the left eye demonstrating similar findings as in (A). C. Preoperative SD-OCT of the right eye demonstrating subfoveal inner segment–outer segment (IS/OS) disruption and near-complete absence of foveal tissue resembling LMH without foveal inversion or significant epimacular membrane; a thin strand of inner retinal and outer retinal tissue spans the central defect; visual acuity = 20/25. D. Preoperative SD-OCT of the left eye demonstrating inner retinal cystic space and subfoveal IS/OS disruption; visual acuity = 20/40. E. Post-PPV SD-OCT of the right eye reveals absence of the previously seen inner retinal surface tissue and increased continuity of outer retinal tissue; the area of IS/OS disruption seems unchanged; visual acuity = 20/30. F. Post-PPV SD-OCT of the left eye demonstrates FTMH with slight elevation of the nasal border; visual acuity = 20/50.
of mid and deep retinal changes noted on SD-OCT, and the intraretinal leakage seen angiographically. Although the pathogenesis of mactel remains, to our knowledge, obscure, proposed mechanisms include progressive capillary endothelial cell degeneration, ischemic changes in watershed zones, and a primary neurodegenerative process. Current theories regarding the pathogenesis of idiopathic LMH and FTMH include vitreoretinal traction, opening of foveal cysts resulting from macular edema, and tangential traction caused by an epiretinal membrane.16–18 Koizumi et al9 hypothesized that MH formation in mactel is the result of the separation of neural tissue but that the retained vision in their series precluded the possibility of significant photoreceptor atrophy. This would seem to confirm the visual and anatomical results in cases 1 and 3 in the present series. It is important to note that the definition of “lamellar macular hole” may have significant overlap, as imaged via OCT, with inner lamellar cysts or “empty spaces” that are commonly described in mactel, and previous investigations10–14 have predominantly used time-domain OCT. Although the characteristic findings of epimacular membrane, foveal inversion, and vitreomacular traction may differentiate the two entities, the overlapping morphology and additional factors (such as various resolution of imaging devices) may affect the interobserver report
rate and clinical decision making regarding such phenomena. As previously reported,19 we hypothesize that that the cystic spaces observed in mactel result from chronic capillary endothelial incompetence with waxing and waning amounts of intraretinal fluid resulting in separation of neurosensory tissue elements. The characteristic finding of the predominance of inner retinal separation, non–outer plexiform morphology, often with a thin veil of epiretinal tissue (presumably ILM) in the absence of significant foveal effacement and epiretinal membrane may offer insight into the nature of the underlying pathogenesis and seems pathognomonic for mactel. Subsets of patients may have additional risk factors, including vitreofoveal traction and epimacular membrane, that further contribute to loss of foveal contour and MH formation. Therefore, the relief of these factors may facilitate the reformation of a foveal anatomy compatible with improved vision but does not alter the underlying disease process, which we believe is perifoveal vascular incompetence. The mechanism of visual improvement in the cases demonstrated herein remains obscure but may relate to the achievement of a foveal contour more consistent with decreased metamorphospia, such as case 2, in the right eye. There have been few histologic reports of mactel,20–22 but microscopic findings seem to include
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PPV FOR MACTEL
Fig. 3. Case 3: Fluorescein angiography and SD-OCT of a patient with mactel and bilateral PPV with ILM removal. A. Early arteriovenous phase FA of the right eye demonstrating perifoveal telangiectatic retinal capillaries and central macular intraretinal leakage most prominent in the temporal perifoveal region. B. Mid-arteriovenous phase FA of the left eye demonstrating similar findings as in (A). C. Preoperative SD-OCT of the right eye demonstrating multiple intraretinal cystic spaces, loss of subfoveal inner segment–outer segment (IS/OS) line, and temporal hyperreflectant nodule; visual acuity = 20/80. D. Preoperative SD-OCT of the left eye demonstrating multiple subfoveal cystic spaces and a thin veil of tissue spanning the inner retinal surface, presumably ILM; visual acuity = 20/40. E. PostPPV SD-OCT demonstrating decrease in intraretinal cystic spaces, overall retinal thinning, and continued IS/OS disruption; visual acuity = 20/40. F. Post-PPV SD-OCT at 26 months postoperative examination demonstrates atrophy of all retinal layers and thin subfoveal subretinal hyporeflectant space; visual acuity = 20/40.
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capillary pericyte loss, reduction in macular pigment, depletion of type IV collagen, and Müller cell dropout. As Müller cells become depleted, alteration in tangential forces present within the ILM in the parafoveal region may lead to distortion of remaining photoreceptors, which is one possible mechanism by which ILM removal may accomplish visual improvement. In addition, as blood–retina barrier breakdown leads to intraretinal fluid leakage; ILM removal may remove a diffusion barrier and allow transudation into vitreous, thereby reducing the amount of extracellular fluid. In patients not experiencing visual improvement, or developing progression of retinal cavitation, ILM peeling may remove the only remaining, thin inner retinal tissue leading to the development of a FTMH. Patients with visual decline may also experience progression of underlying degeneration after mechanical manipulation of Müller cells with ILM peeling. Pars plana vitrectomy with ILM peeling may require additional investigation, possibly as a component of a clinical trial. We conclude that surgical intervention may be an option for select cases of mactel after careful consideration, especially in those with LMH or SD-OCT evidence of epimacular membrane. Key words: idiopathic juxtafoveal telangiectasis, idiopathic macular telangiectasia, mactel, IMT, internal limiting membrane peeling, JFT, parafoveal telangiectasia, pars plana vitrectomy.
References 1. Gass JD. A fluorescein angiographic study of macular dysfunction secondary to retinal vascular disease. V. Retinal telangiectasis. Arch Ophthalmol 1968;80:592–605. 2. Gass JD, Oyakawa RT. Idiopathic juxtafoveolar retinal telangiectasis. Arch Ophthalmol 1982;100:769–780. 3. Gass JD, Blodi BA. Idiopathic juxtafoveolar retinal telangiectasis. Update of classification and follow-up study. Ophthalmology 1993;100:1536–1546. 4. Yannuzzi LA, Bardal AM, Freund KB, et al. Idiopathic macular telangiectasia. Arch Ophthalmol 2006;124:450–460. 5. Klein R, Blodi BA, Meuer SM, et al. The prevalence of macular telangiectasia type 2 in the Beaver Dam eye study. Am J Ophthalmol 2010;150:55–62 e52. 6. Aung KZ, Wickremasinghe SS, Makeyeva G, et al. The prevalence estimates of macular telangiectasia type 2: the Melbourne Collaborative Cohort Study. Retina 2010;30: 473–478. 7. Patel B, Duvall J, Tullo AB. Lamellar macular hole associated with idiopathic juxtafoveolar telangiectasia. Br J Ophthalmol 1988;72:550–551. 8. Olson JL, Mandava N. Macular hole formation associated with idiopathic parafoveal telangiectasia. Graefes Arch Clin Exp Ophthalmol 2006;244:411–412. 9. Koizumi H, Slakter JS, Spaide RF. Full-thickness macular hole formation in idiopathic parafoveal telangiectasis. Retina 2007; 27:473–476. 10. Shukla D. Evolution and management of macular hole secondary to type 2 idiopathic macular telangiectasia. Eye (Lond). 2011;25:532–533. 11. Rishi P, Kothari AR. Parafoveal telangiectasia (PFT) has been associated with changes in macular architecture and macular
Copyright ª by Ophthalmic Communications Society, Inc. Unauthorized reproduction of this article is prohibited.
6
12.
13.
14.
15.
16.
RETINAL CASES & BRIEF REPORTS´ 2013 VOLUME 0 NUMBER 0 holes (lamellar and full thickness). Retina 2008;28:184–185; author reply 185–186. Charbel Issa P, Scholl HP, Gaudric A, et al. Macular fullthickness and lamellar holes in association with type 2 idiopathic macular telangiectasia. Eye (Lond) 2009;23:435–441. Gregori N, Flynn HW Jr. Surgery for full-thickness macular hole in patients with idiopathic macular telangiectasia type 2. Ophthalmic Surg Lasers Imaging 2010;41 Online:1–4. Sandhu SS, Steel DH. Comment on macular full-thickness and lamellar holes in association with type 2 idiopathic macular telangiectasia. Eye (Lond) 2010;24:1119. Shukla D, Venkatesh R. Spontaneous closure of full-thickness macular hole in type 2 idiopathic macular telangiectasia. Graefes Arch Clin Exp Ophthalmol 2012;250:1711–1712. Gass JD. Reappraisal of biomicroscopic classification of stages of development of a macular hole. Am J Ophthalmol 1995;119: 752–759.
17. Gaudric A, Haouchine B, Massin P, et al. Macular hole formation: new data provided by optical coherence tomography. Arch Ophthalmol 1999;117:744–751. 18. Smiddy WE, Flynn HW Jr. Pathogenesis of macular holes and therapeutic implications. Am J Ophthalmol 2004;137:525–537. 19. Sigler EJ, Randolph JC, Calzada JI, Charles S. Comparison of observation, inravitreal bevacizumab, or pars plana vitrectomy for non-proliferative type 2 idiopathic macular telangiectasia. Graefes Arch Clin Exp Ophthalmol 2013;251:1097–1101. 20. Green WR, Quigley HA, De la Cruz Z, Cohen B. Parafoveal retinal telangiectasis. Light and electron microscopy studies. Trans Ophthalmol Soc U K 1980;100:162–170. 21. Eliassi-Rad B, Green WR. Histopathologic study of presumed parafoveal telangiectasis. Retina 1999;19:332–335. 22. Powner MB, Gillies MC, Tretiach M, et al. Perifoveal Muller cell depletion in a case of macular telangiectasia type 2. Ophthalmology 2010;117:2407–2416.
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