MASSIVE COATS DISEASE–LIKE TUMOR AFTER RETINAL DETACHMENT Stefan Schrader, MD,* Christine Kusserow, MD,* Philipp Steven, MD,* Maya Mu¨ller, MD,* Hans Hoerauf, MD,† Horst Laqua, MD*
Purpose: To describe two patients with peripheral Coats disease–like tumor reaction years after surgical repair for rhegmatogenous retinal detachment. Methods: Retrospective review of two clinical cases. Results: Patient 1 presented with visual deterioration caused by a retinal angiomatous tumor and lipid exudates originating from telangiectatic vessels on the scleral buckle 8 years after vitrectomy and encircling band surgery for a giant retinal tear. In Patient 2, a vascularized retinal mass was detected during vitrectomy for a vitreous hemorrhage 24 years after scleral buckling procedures. Both patients were highly myopic and had no history of scleral perforation or angiomatous proliferation before surgery. They underwent vitrectomy and removal of the vascularized tumor. Histologic examination revealed wide lacunae filled with erythrocytes, connective tissue strands with inflammatory cells, and capillaries that showed thickened multilamellar basement membranes. Conclusion: Secondary Coats disease–like tumor reaction may occur as a late complication years after successful retinal detachment surgery and may be caused by a chronic ischemic and inflammatory stimulus induced by prominent buckling elements in highly myopic eyes. RETINAL CASES & BRIEF REPORTS 3:170 –173, 2009
From *University Eye Hospital Luebeck, Luebeck, Germany, and †University Eye Hospital Goettingen, Goettingen, Germany.
gery causing severe visual deterioration and present interesting histologic findings. Case Reports
T
he formation of retinal telangiectatic and aneurysmal blood vessels with intraretinal and subretinal exudation is known as Coats disease. Although most cases are diagnosed at a young age, Coats disease may also occur in adulthood. Light and electron microscopic analyses for adults show identical histologic features. The deficiency of healthy viable retinal vascular endothelial cells supports the hypothesis that the ocular changes in this syndrome are directly related to a primary retinal vascular defect leading to abnormal permeability.1 Vasoproliferative retinal tumors presenting as peripheral telangiectases and exudation with Coats disease–like features are known as idiopathic and occur secondary to retinitis pigmentosa, uveitis, and long-standing retinal detachment and after conventional buckling surgery.2–5 We describe two patients with huge peripheral Coats disease–like tumors years after successful retinal detachment sur-
Case 1 In 1983, a 12-year-old myopic (⫺3.0 diopters) boy underwent pars plana vitrectomy, encircling band surgery, and silicone oil tamponade on the left eye for a macula-off retinal detachment caused by a giant retinal tear. One year later, uncomplicated pars plana lensectomy and silicone oil removal were performed. The retina remained attached, and visual acuity was 20/100. In 1993, small angiomatous lesions and microaneurysms were detected on the temporal buckle (Fig. 1, a and b). The circumferential buckle appeared prominent, no treatment was performed, and regular follow-up visits were suggested. In 1996, the patient presented with severe vision loss to counting fingers. Slit-lamp examination revealed neovascularization of the iris and massive flare and cells in the anterior chamber. View of the fundus was rarely possible, and a large temporal tumor mass in the anterior vitreous cavity obstructing the optical axis was seen (Fig. 2a). Intraocular pressure was normal. Ophthalmoscopy demonstrated a huge tumor based on the superotemporal buckle in the area of the former giant tear. Subretinal lipid exudation extended into the macular area (Fig. 2b). The surface of the yellowish tumor showed multiple telangiectatic and angiomatous changes. Examination of the right eye was unremarkable; no retinal vascular abnormalities or signs of ischemic ophthalmopathy were seen. Vitrectomy including ab interno resec-
Reprint requests: Hans Hoerauf, MD, Georg-August-University, Department of Ophthalmology, Robert-Koch-Strasse 40, 37075 Goettingen, Germany; e-mail: [email protected]
170
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Fig. 1. Case 1. Nine years after pars plana vitrectomy, encircling band surgery, silicone oil tamponade, and silicone oil removal combined with lensectomy, the retina was stably attached (a), but small angiomatous lesions and microaneurysms were detected on the temporal buckle (b; arrowheads). Inset is showing a higher magnification of the multilamellar structure.
tion of the angiomatous mass and silicone oil tamponade was performed, and the encircling band was transected. Postoperatively, a retinal detachment in the temporal quadrant occurred that was caused by a small break. It was treated successfully with a radial sponge. Due to a secondary macular pucker, vitrectomy and membrane peeling were performed 1 year later. During the further 6-year follow-up, the retina stabilized, and rubeosis iridis regressed and finally disappeared completely. Subretinal lipid exudates slowly reabsorbed (Fig. 3, a– c), and visual acuity increased to 20/400. Silicone oil was removed without complications in 1999. Since then, no tumor or telangiectatic vessel recurrence has been observed, and the retina has remained stably attached.
Case 2 A 65-year-old myopic (⫺6.75 diopters) woman underwent three scleral buckling procedures with encircling band surgery and cryocoagulation in 1974 on the right eye for a rhegmatogenous retinal detachment. Uncomplicated phacoemulsification and posterior chamber lens implantation were performed in 1993, and visual acuity was 20/100. In 2003, visual impairment due to vitreous hemorrhages occurred. Three years later, the patient was referred to our hospital with a dense vitreous hemorrhage and rubeotic secondary glaucoma. Intraocular pressure was 34 mm Hg, and visual acuity was light perception. Slit-lamp examination revealed aggressive neovascularization of the iris and uveal ectropion. Ultrasound analysis revealed a dense equatorial mass and vitreous hemorrhage. Examination of the left eye was unremarkable; in particular, no areas of retinal nonperfusion or vascular abnormalities could be identified. During vitrec-
Fig. 2. Case 1. In 1996, the patient presented with severe vision loss due to a large temporal tumor mass in the anterior vitreous obstructing the optical axis (a). Ophthalmoscopy showed a huge tumor based on the superotemporal buckle in the area of the former giant tear. Subretinal lipid exudation extended into the macular area (b).
tomy, a huge yellowish tumor with superficial angiomatous changes was observed on the inferonasal prominent circumferential buckle. Ab interno resection of the tumor, endolaser coagulation, and silicone oil tamponade were performed. Intraoperative specimens of the aspirated tumor mass were obtained and analyzed by means of light and electron microscopy. Postoperatively, rubeosis regressed, and intraocular pressure decreased to normal ranges.
Histology Light microscopy of specimens from Patient 2 revealed large amounts of erythrocytes within lacunae of fibrinous condensations and inflammatory cell aggregates consisting of lymphocytes and plasma cells. Sparse capillaries were found surrounded by fibroblastic tissue and inflammatory cells (Fig. 4, a and b). Electron microscopy of the capillaries showed signs of degenerative changes, thickened multilamellar basal membranes infiltrated with lipid and fibrin exudates, and cellular residuals. Open lumina were filled with erythrocytes, granulocytes, or lymphocytes. Some capillaries were occluded by thrombi; some fibrin deposits were found within the capillary walls (Fig. 5, a and b).
Discussion Peripheral telangiectatic lesions of variable size and so-called vasoproliferative tumors of the ocular fundus with a Coats disease–like clinical appearance are a well known clinical entity. They are described in the literature
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Fig. 3. Case 1. During follow-up after vitrectomy, ab interno resection of the angiomatous mass, and silicone oil tamponade, the retina stabilized, rubeosis iridis regressed, and subretinal lipid exudates slowly reabsorbed (4 weeks [a], 2 months [b], and 1 year [c] after vitrectomy and silicone oil tamponade).
as primary lesions and as secondary reactions associated with different retinal diseases or long-standing retinal detachment.2,4,6 Some reports about Coats disease–like tumors after conventional buckling surgery for rhegmatogenous detachment also exist.2,3 In the current cases, huge peripheral vascularized tumors with deposition of lipoid material developed as a late complication many years after successful retinal detachment surgery. In Case 1, it is well documented that the tumor originated from characteristic peripheral vessel changes with telangiectatic dilation and aneurysm formation slowly growing over several years, suggesting that localized peripheral telangiectases are an acquired variant secondary to longterm changes after scleral buckling surgery with an identical underlying pathogenetic process, in contrast to primary congenital retinal telangiectasia. Due to the large diameter of the tumor, no cryocoagulation was possible. Laser coagulation would have been possible only on the anterior surface of the tumor, increasing the risk of exudation. In Case 2, vitreous hemorrhage was present, which impeded noninvasive treatment modalities. Interestingly, both of our patients were highly myopic, had undergone circumferential buckle surgery with a 3.5-mm silicone band, and were operated on many years
ago. In both patients, the buckling element appeared very prominent, and tumor growth occurred on the buckle. The association with the most peripheral retinal vessels and the temporal quadrant were discussed as predisposing factors.7 In one of our patients, the tumor was localized on the inferonasal buckle. As in Coats disease, the lesions were located between the equator and the ora serrata. Gray and Gregor3 described chronic outer retinal ischemia and sluggish retinal blood flow as causative factors in two patients with long-standing retinal detachment. Gottlieb and Fammartino2 described an association with the site of previous subretinal drainage and deep scleral suture and suspected disruption of Bruch membrane as the initiator of the process. Most of the affected patients were myopic. As described in other reports of retinal telangiectases, rubeosis was seen in both of our patients,8 which is usually considered to be an indicator of retinal ischemia. Lincoff and Stopa8 reported markedly diminished ocular blood flow after the placement of encircling bands, which may induce ischemia in the choroid and the ciliary body. Therefore, reduced retinal, choroidal, or ciliary body circulation may be involved in the pathogenesis. Interestingly, in both of our patients, rubeosis iridis was not observed
Fig. 4. Case 2. Light microscopic images (original magnification, ⫻40) showing (a) hematoxylin– eosin and (b) periodic acid–Schiff staining of the tumor. Large lacunae are surrounded by connective tissue and filled with large numerous erythrocytes. Within the fibroblastic tissue, small capillaries and aggregates of inflammatory cells (arrowheads) were found.
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Fig. 5. Case 2. Electron microscopic images of tumor capillaries showing degenerative changes, infiltration of the wall structures with lipid and fibrin exudates and cellular residuals (a), and a thickened multilamellar basal membrane (b). Inset is showing a higher magnification of the multilamellar structure.
before development of the retinal tumor and regressed after resection. This may support the hypothesis that the tumor itself stimulates neovascularization. Whether the stimulus for rubeosis formation is mediated by the affected retina or vascular endothelial growth factor production inside the tumor itself is speculative. Histologically, the various forms of telangiectases are very similar. In contrast to other reports,6,7 we could not find huge spindle cell masses of glial nature, but we were able to demonstrate interesting vascular features. The appearance of multilamellar basement membranes indicates the existence of inflammatory stimuli to the capillaries. Endothelial cell necrosis and regeneration are assumed to be the reason for multilamellar membrane formation, which are typically found in Wegener disease.9 In an electron microscopic study of Coats disease, Tripathi and Ashton10 described the appearance of dilated retinal vessels with thickened walls located in the area of primary Coats disease lesions. The vessel walls consisted of basement membrane–like material infiltrated with exudates and blood corpuscles. These results are similar to our findings and are discussed as possible primary abnormal endothelial permeability, which results in leakage into the vessel wall leading to remodeling of the wall structure and aneurysmal or telangiectatic changes. High buckling elements used in retinal detachment surgery may produce scleral necrosis, particularly in highly myopic eyes with thin sclera, and may act as chronic ischemic and inflammatory stimuli. This in turn may lead to secondary endothelial breakdown and a common clinical appearance like primary Coats disease with telangiectatic blood vessel formation followed by exudation, spontaneous bleeding, tumor formation, and consecutive organization. Although the exact pathogenesis of this Coats disease–like reaction remains unclear, our histologic findings indicate the existence of a chronic vascular
inflammatory process. When peripheral telangiectatic changes after retinal detachment surgery occur, they should be followed closely. Early treatment, preferably with laser photocoagulation, cryocoagulation, or photodynamic therapy, should be considered to prevent massive exudation and tumor development. Key words: Coats disease–like tumor, retinal detachment, vasoproliferative retinal tumor, peripheral telangiectasis. Acknowledgments The authors are grateful to Drs. U. Schlo¨tzer-Schrehardt and Holbach from the Department of Ophthalmology at the University of Erlangen (Erlangen, Germany) for histologic examinations and their helpful comments.
References 1.
2. 3. 4.
5. 6. 7.
8. 9.
10.
Apple DJ, Gieser DK. Pathologische Befunde bei einem Erwachsenen mit Morbus Coats. Klin Monatsbl Augenheilkd 1981;179:336–339. Gottlieb F, Fammartino JJ. Retinal angiomatous mass. A complication of retinal detachment surgery. Retina 1984;4:152–157. Gray RH, Gregor ZJ. Acquired peripheral retinal telangiectasia after retinal surgery. Retina 1994;14:10–13. Laqua H, Wessing A. Peripheral retinal telangiectasis in adults simulating a vascular tumor or melanoma. Ophthalmology 1983;90:1284–1291. Shields JA, Decker WL. Presumed acquired retinal hemangiomas. Ophthalmology 1983;90:1292–1300. Heimann H, Bornfeld N. Vasoproliferative tumours of the retina. Br J Ophthalmol 2000;84:1162–1169. Irvine F, O’Donnell N. Retinal vasoproliferative tumors: surgical management and histological findings. Arch Ophthalmol 2000;118:563–569. Lincoff H, Stopa M. Cutting the encircling band. Retina 2006;26:650–654. Gindre D, Peyrol S. Fibrosing vasculitis in Wegener’s granulomatosis: ultrastructural and immunohistochemical analysis of the vascular lesions. Virchows Arch 1995;427:385–393. Tripathi R, Ashton N. Electron microscopical study of Coat’s disease. Br J Ophthalmol 1971;55:289–301.
Purpose: To describe two patients with peripheral Coats disease–like tumor reaction years after surgical repair for rhegmatogenous retinal detachment. Methods: Retrospective review of two clinical cases. Results: Patient 1 presented with visual deterioration caused by a retinal angiomatous tumor and lipid exudates originating from telangiectatic vessels on the scleral buckle 8 years after vitrectomy and encircling band surgery for a giant retinal tear. In Patient 2, a vascularized retinal mass was detected during vitrectomy for a vitreous hemorrhage 24 years after scleral buckling procedures. Both patients were highly myopic and had no history of scleral perforation or angiomatous proliferation before surgery. They underwent vitrectomy and removal of the vascularized tumor. Histologic examination revealed wide lacunae filled with erythrocytes, connective tissue strands with inflammatory cells, and capillaries that showed thickened multilamellar basement membranes. Conclusion: Secondary Coats disease–like tumor reaction may occur as a late complication years after successful retinal detachment surgery and may be caused by a chronic ischemic and inflammatory stimulus induced by prominent buckling elements in highly myopic eyes. RETINAL CASES & BRIEF REPORTS 3:170 –173, 2009
From *University Eye Hospital Luebeck, Luebeck, Germany, and †University Eye Hospital Goettingen, Goettingen, Germany.
gery causing severe visual deterioration and present interesting histologic findings. Case Reports
T
he formation of retinal telangiectatic and aneurysmal blood vessels with intraretinal and subretinal exudation is known as Coats disease. Although most cases are diagnosed at a young age, Coats disease may also occur in adulthood. Light and electron microscopic analyses for adults show identical histologic features. The deficiency of healthy viable retinal vascular endothelial cells supports the hypothesis that the ocular changes in this syndrome are directly related to a primary retinal vascular defect leading to abnormal permeability.1 Vasoproliferative retinal tumors presenting as peripheral telangiectases and exudation with Coats disease–like features are known as idiopathic and occur secondary to retinitis pigmentosa, uveitis, and long-standing retinal detachment and after conventional buckling surgery.2–5 We describe two patients with huge peripheral Coats disease–like tumors years after successful retinal detachment sur-
Case 1 In 1983, a 12-year-old myopic (⫺3.0 diopters) boy underwent pars plana vitrectomy, encircling band surgery, and silicone oil tamponade on the left eye for a macula-off retinal detachment caused by a giant retinal tear. One year later, uncomplicated pars plana lensectomy and silicone oil removal were performed. The retina remained attached, and visual acuity was 20/100. In 1993, small angiomatous lesions and microaneurysms were detected on the temporal buckle (Fig. 1, a and b). The circumferential buckle appeared prominent, no treatment was performed, and regular follow-up visits were suggested. In 1996, the patient presented with severe vision loss to counting fingers. Slit-lamp examination revealed neovascularization of the iris and massive flare and cells in the anterior chamber. View of the fundus was rarely possible, and a large temporal tumor mass in the anterior vitreous cavity obstructing the optical axis was seen (Fig. 2a). Intraocular pressure was normal. Ophthalmoscopy demonstrated a huge tumor based on the superotemporal buckle in the area of the former giant tear. Subretinal lipid exudation extended into the macular area (Fig. 2b). The surface of the yellowish tumor showed multiple telangiectatic and angiomatous changes. Examination of the right eye was unremarkable; no retinal vascular abnormalities or signs of ischemic ophthalmopathy were seen. Vitrectomy including ab interno resec-
Reprint requests: Hans Hoerauf, MD, Georg-August-University, Department of Ophthalmology, Robert-Koch-Strasse 40, 37075 Goettingen, Germany; e-mail: [email protected]
170
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Fig. 1. Case 1. Nine years after pars plana vitrectomy, encircling band surgery, silicone oil tamponade, and silicone oil removal combined with lensectomy, the retina was stably attached (a), but small angiomatous lesions and microaneurysms were detected on the temporal buckle (b; arrowheads). Inset is showing a higher magnification of the multilamellar structure.
tion of the angiomatous mass and silicone oil tamponade was performed, and the encircling band was transected. Postoperatively, a retinal detachment in the temporal quadrant occurred that was caused by a small break. It was treated successfully with a radial sponge. Due to a secondary macular pucker, vitrectomy and membrane peeling were performed 1 year later. During the further 6-year follow-up, the retina stabilized, and rubeosis iridis regressed and finally disappeared completely. Subretinal lipid exudates slowly reabsorbed (Fig. 3, a– c), and visual acuity increased to 20/400. Silicone oil was removed without complications in 1999. Since then, no tumor or telangiectatic vessel recurrence has been observed, and the retina has remained stably attached.
Case 2 A 65-year-old myopic (⫺6.75 diopters) woman underwent three scleral buckling procedures with encircling band surgery and cryocoagulation in 1974 on the right eye for a rhegmatogenous retinal detachment. Uncomplicated phacoemulsification and posterior chamber lens implantation were performed in 1993, and visual acuity was 20/100. In 2003, visual impairment due to vitreous hemorrhages occurred. Three years later, the patient was referred to our hospital with a dense vitreous hemorrhage and rubeotic secondary glaucoma. Intraocular pressure was 34 mm Hg, and visual acuity was light perception. Slit-lamp examination revealed aggressive neovascularization of the iris and uveal ectropion. Ultrasound analysis revealed a dense equatorial mass and vitreous hemorrhage. Examination of the left eye was unremarkable; in particular, no areas of retinal nonperfusion or vascular abnormalities could be identified. During vitrec-
Fig. 2. Case 1. In 1996, the patient presented with severe vision loss due to a large temporal tumor mass in the anterior vitreous obstructing the optical axis (a). Ophthalmoscopy showed a huge tumor based on the superotemporal buckle in the area of the former giant tear. Subretinal lipid exudation extended into the macular area (b).
tomy, a huge yellowish tumor with superficial angiomatous changes was observed on the inferonasal prominent circumferential buckle. Ab interno resection of the tumor, endolaser coagulation, and silicone oil tamponade were performed. Intraoperative specimens of the aspirated tumor mass were obtained and analyzed by means of light and electron microscopy. Postoperatively, rubeosis regressed, and intraocular pressure decreased to normal ranges.
Histology Light microscopy of specimens from Patient 2 revealed large amounts of erythrocytes within lacunae of fibrinous condensations and inflammatory cell aggregates consisting of lymphocytes and plasma cells. Sparse capillaries were found surrounded by fibroblastic tissue and inflammatory cells (Fig. 4, a and b). Electron microscopy of the capillaries showed signs of degenerative changes, thickened multilamellar basal membranes infiltrated with lipid and fibrin exudates, and cellular residuals. Open lumina were filled with erythrocytes, granulocytes, or lymphocytes. Some capillaries were occluded by thrombi; some fibrin deposits were found within the capillary walls (Fig. 5, a and b).
Discussion Peripheral telangiectatic lesions of variable size and so-called vasoproliferative tumors of the ocular fundus with a Coats disease–like clinical appearance are a well known clinical entity. They are described in the literature
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Fig. 3. Case 1. During follow-up after vitrectomy, ab interno resection of the angiomatous mass, and silicone oil tamponade, the retina stabilized, rubeosis iridis regressed, and subretinal lipid exudates slowly reabsorbed (4 weeks [a], 2 months [b], and 1 year [c] after vitrectomy and silicone oil tamponade).
as primary lesions and as secondary reactions associated with different retinal diseases or long-standing retinal detachment.2,4,6 Some reports about Coats disease–like tumors after conventional buckling surgery for rhegmatogenous detachment also exist.2,3 In the current cases, huge peripheral vascularized tumors with deposition of lipoid material developed as a late complication many years after successful retinal detachment surgery. In Case 1, it is well documented that the tumor originated from characteristic peripheral vessel changes with telangiectatic dilation and aneurysm formation slowly growing over several years, suggesting that localized peripheral telangiectases are an acquired variant secondary to longterm changes after scleral buckling surgery with an identical underlying pathogenetic process, in contrast to primary congenital retinal telangiectasia. Due to the large diameter of the tumor, no cryocoagulation was possible. Laser coagulation would have been possible only on the anterior surface of the tumor, increasing the risk of exudation. In Case 2, vitreous hemorrhage was present, which impeded noninvasive treatment modalities. Interestingly, both of our patients were highly myopic, had undergone circumferential buckle surgery with a 3.5-mm silicone band, and were operated on many years
ago. In both patients, the buckling element appeared very prominent, and tumor growth occurred on the buckle. The association with the most peripheral retinal vessels and the temporal quadrant were discussed as predisposing factors.7 In one of our patients, the tumor was localized on the inferonasal buckle. As in Coats disease, the lesions were located between the equator and the ora serrata. Gray and Gregor3 described chronic outer retinal ischemia and sluggish retinal blood flow as causative factors in two patients with long-standing retinal detachment. Gottlieb and Fammartino2 described an association with the site of previous subretinal drainage and deep scleral suture and suspected disruption of Bruch membrane as the initiator of the process. Most of the affected patients were myopic. As described in other reports of retinal telangiectases, rubeosis was seen in both of our patients,8 which is usually considered to be an indicator of retinal ischemia. Lincoff and Stopa8 reported markedly diminished ocular blood flow after the placement of encircling bands, which may induce ischemia in the choroid and the ciliary body. Therefore, reduced retinal, choroidal, or ciliary body circulation may be involved in the pathogenesis. Interestingly, in both of our patients, rubeosis iridis was not observed
Fig. 4. Case 2. Light microscopic images (original magnification, ⫻40) showing (a) hematoxylin– eosin and (b) periodic acid–Schiff staining of the tumor. Large lacunae are surrounded by connective tissue and filled with large numerous erythrocytes. Within the fibroblastic tissue, small capillaries and aggregates of inflammatory cells (arrowheads) were found.
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Fig. 5. Case 2. Electron microscopic images of tumor capillaries showing degenerative changes, infiltration of the wall structures with lipid and fibrin exudates and cellular residuals (a), and a thickened multilamellar basal membrane (b). Inset is showing a higher magnification of the multilamellar structure.
before development of the retinal tumor and regressed after resection. This may support the hypothesis that the tumor itself stimulates neovascularization. Whether the stimulus for rubeosis formation is mediated by the affected retina or vascular endothelial growth factor production inside the tumor itself is speculative. Histologically, the various forms of telangiectases are very similar. In contrast to other reports,6,7 we could not find huge spindle cell masses of glial nature, but we were able to demonstrate interesting vascular features. The appearance of multilamellar basement membranes indicates the existence of inflammatory stimuli to the capillaries. Endothelial cell necrosis and regeneration are assumed to be the reason for multilamellar membrane formation, which are typically found in Wegener disease.9 In an electron microscopic study of Coats disease, Tripathi and Ashton10 described the appearance of dilated retinal vessels with thickened walls located in the area of primary Coats disease lesions. The vessel walls consisted of basement membrane–like material infiltrated with exudates and blood corpuscles. These results are similar to our findings and are discussed as possible primary abnormal endothelial permeability, which results in leakage into the vessel wall leading to remodeling of the wall structure and aneurysmal or telangiectatic changes. High buckling elements used in retinal detachment surgery may produce scleral necrosis, particularly in highly myopic eyes with thin sclera, and may act as chronic ischemic and inflammatory stimuli. This in turn may lead to secondary endothelial breakdown and a common clinical appearance like primary Coats disease with telangiectatic blood vessel formation followed by exudation, spontaneous bleeding, tumor formation, and consecutive organization. Although the exact pathogenesis of this Coats disease–like reaction remains unclear, our histologic findings indicate the existence of a chronic vascular
inflammatory process. When peripheral telangiectatic changes after retinal detachment surgery occur, they should be followed closely. Early treatment, preferably with laser photocoagulation, cryocoagulation, or photodynamic therapy, should be considered to prevent massive exudation and tumor development. Key words: Coats disease–like tumor, retinal detachment, vasoproliferative retinal tumor, peripheral telangiectasis. Acknowledgments The authors are grateful to Drs. U. Schlo¨tzer-Schrehardt and Holbach from the Department of Ophthalmology at the University of Erlangen (Erlangen, Germany) for histologic examinations and their helpful comments.
References 1.
2. 3. 4.
5. 6. 7.
8. 9.
10.
Apple DJ, Gieser DK. Pathologische Befunde bei einem Erwachsenen mit Morbus Coats. Klin Monatsbl Augenheilkd 1981;179:336–339. Gottlieb F, Fammartino JJ. Retinal angiomatous mass. A complication of retinal detachment surgery. Retina 1984;4:152–157. Gray RH, Gregor ZJ. Acquired peripheral retinal telangiectasia after retinal surgery. Retina 1994;14:10–13. Laqua H, Wessing A. Peripheral retinal telangiectasis in adults simulating a vascular tumor or melanoma. Ophthalmology 1983;90:1284–1291. Shields JA, Decker WL. Presumed acquired retinal hemangiomas. Ophthalmology 1983;90:1292–1300. Heimann H, Bornfeld N. Vasoproliferative tumours of the retina. Br J Ophthalmol 2000;84:1162–1169. Irvine F, O’Donnell N. Retinal vasoproliferative tumors: surgical management and histological findings. Arch Ophthalmol 2000;118:563–569. Lincoff H, Stopa M. Cutting the encircling band. Retina 2006;26:650–654. Gindre D, Peyrol S. Fibrosing vasculitis in Wegener’s granulomatosis: ultrastructural and immunohistochemical analysis of the vascular lesions. Virchows Arch 1995;427:385–393. Tripathi R, Ashton N. Electron microscopical study of Coat’s disease. Br J Ophthalmol 1971;55:289–301.
