HISTOLOGIC AND IMMUNOHISTOCHEMICAL FEATURES OF A VITREOUS CYST P. Kumar Rao, MD,*† Arghavan Almony, MD,* Gaurav K. Shah, MD,*† Blake Cooper, MD,‡ Morton E. Smith, MD*
Purpose: To report the histologic and immunohistochemical features of a congenital vitreous cyst. Methods: Interventional case report with immunohistochemical correlation. A 58-yearold woman with known vitreous cyst since childhood had an enlarging visual floater. The vitreous cyst was removed by pars plana vitrectomy and central vision was restored. Results: Pathology confirmed a vitreous cyst derived from neural and glial tissue and consistent with retinal origin. Conclusions: Congenital vitreous cysts, derived from neural and glial tissue, can be safely removed using pars plana vitrectomy. RETINAL CASES & BRIEF REPORTS 3:60 – 61, 2009
From the *Department of Ophthalmology & Visual Sciences, Washington University School of Medicine, St. Louis; †Barnes Retina Institute, St. Louis, MO; and ‡University of Kansas Hospital, Kansas City.
“floater” was in her visual axis. Examination revealed a posterior vitreous detachment with a large, free-floating vitreous cyst (Figure 1). Peripheral retinal examination with depression techniques revealed scattered, discrete posterior and midperipheral chorioretinal scars, circumferential lattice degeneration, and normal pars plana and pars plicata. The patient was disturbed by the floater and associated visual impairment and elected to undergo pars plana vitrectomy with removal of the vitreous cyst. The cyst was removed using a blunt 20-gauge cannula and active suction technique. Hematoxylin and eosin staining revealed a multilayer cellular cyst wall. The cells were composed of pink cytoplasm with indistinct borders and the spindle nuclei appeared bland with a single
V
itreous cysts, first reported by Tansley in 1899, are rare and can be divided into two general categories: acquired cysts associated with surgery, trauma, degenerative or inflammatory ocular conditions (including retinitis pigmentosa and uveitis), and parasites; and congenital cysts.1 In 1909, Durst used the following characteristics to distinguish a congenital cyst from an acquired one: 1) existence of associated congenital anomalies; 2) absence of signs of old or recent inflammation; 3) absence of vessels; and 4) presence of amblyopia and squint.2 We report a patient with a symptomatic congenital vitreous cyst, describing surgical management and histologic and immunohistochemical features of the cyst. Case Report A 58-year-old woman reported an increasingly large, mobile “floater” which had been intermittently symptomatic since childhood. She had undergone uneventful cataract extraction and posterior chamber lens placement several years before presentation. Best-corrected vision was 20/30 but decreased to 20/70 when the The authors have no financial interest in this article. Reprint requests: P. Kumar Rao, MD, Barnes Retina Institute, 1600 S. Brentwood Blvd, Suite 800, St. Louis, MO 63144; e-mail: [email protected], [email protected]
Fig. 1. Examination reveals a large, free-floating vitreous cyst.
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FEATURES OF A VITREOUS CYST
small nucleolus (Figure 2A). Immunohistochemistry demonstrated the cells were positive for neuron specific enolase which identifies neural elements and glial fibrillary acidic protein (GFAP) which identifies glial elements (Figure 2, B and C). No cells stained for cytokeratin. While Alcian blue material, consistent with vitreous, was found in the wall of the cyst, there was no Alcian blue positive material in the cyst lumen.
Discussion Pigmented congenital cysts are thought to originate from the pars ciliaris epithelium whereas nonpigmented cysts are believed to be remnants of the hyaloid vascular system.3 In their description of a pigmented vitreous cyst, Orellana and colleagues described a single cuboidal layer of nonpigmented and pigmented cells with large melanin granules. They concluded that the cyst was consistent with pigment epithelium origin.1 Nork and Millecchia described the histologic features of another pigmented cyst composed of a single layer of pigmented cells with mature and immature melanosomes. Stains for actin and carbonic anhydrase were positive but negative for GFAP leading them to conclude that the cyst represented a choristoma of the hyaloid system.4 AsiyoVogel reported the histologic findings of a vitreous cyst removed from a patient who developed the cyst after multiple retinal detachment repair procedures.5 Like the case described by Asiyo-Vogel, our specimen also suggests neuronal elements with gliosis on immunohistochemistry; however, our patient did not have an antecedent history of vitreoretinal surgery or trauma. Our specimen may be consistent with glial remnants of Bergmeister papilla.
References 1.
2. 3. Fig. 2. A, Hematoxylin and eosin staining reveal a multilayer cellular cyst wall. The cells are composed of pink cytoplasm with indistinct borders and the spindle nuclei appear bland with a single small nucleolus. B, Immunohistochemistry confirms positive neuron specific enolase and C, glial fibrillary acidic protein.
4. 5.
Orellana J, O’Malley RE, McPherson AR, Font RL. Pigmented free-floating vitreous cysts in two young adults. Electron microscopic observations. Ophthalmology 1985;92:297–302. Francois J. Pre-papillary cyst developed from remnants of the hyaloid artery. Br J Ophthalmol 1950;34:365–368. Bayraktar Z, Kapran Z, Ozdogan S. Pigmented congenital vitreous cyst. Eur J Ophthalmol 2004;14:156–158. Nork TM, Millecchia LL. Treatment and histopathology of a congenital vitreous cyst. Ophthalmology 1998;105:825–830. Asiyo-Vogel MN, El-Hifnawi E, Laqua H. Ultrastructural features of a solitary vitreous cyst. Retina 1996;16:250–254.
Purpose: To report the histologic and immunohistochemical features of a congenital vitreous cyst. Methods: Interventional case report with immunohistochemical correlation. A 58-yearold woman with known vitreous cyst since childhood had an enlarging visual floater. The vitreous cyst was removed by pars plana vitrectomy and central vision was restored. Results: Pathology confirmed a vitreous cyst derived from neural and glial tissue and consistent with retinal origin. Conclusions: Congenital vitreous cysts, derived from neural and glial tissue, can be safely removed using pars plana vitrectomy. RETINAL CASES & BRIEF REPORTS 3:60 – 61, 2009
From the *Department of Ophthalmology & Visual Sciences, Washington University School of Medicine, St. Louis; †Barnes Retina Institute, St. Louis, MO; and ‡University of Kansas Hospital, Kansas City.
“floater” was in her visual axis. Examination revealed a posterior vitreous detachment with a large, free-floating vitreous cyst (Figure 1). Peripheral retinal examination with depression techniques revealed scattered, discrete posterior and midperipheral chorioretinal scars, circumferential lattice degeneration, and normal pars plana and pars plicata. The patient was disturbed by the floater and associated visual impairment and elected to undergo pars plana vitrectomy with removal of the vitreous cyst. The cyst was removed using a blunt 20-gauge cannula and active suction technique. Hematoxylin and eosin staining revealed a multilayer cellular cyst wall. The cells were composed of pink cytoplasm with indistinct borders and the spindle nuclei appeared bland with a single
V
itreous cysts, first reported by Tansley in 1899, are rare and can be divided into two general categories: acquired cysts associated with surgery, trauma, degenerative or inflammatory ocular conditions (including retinitis pigmentosa and uveitis), and parasites; and congenital cysts.1 In 1909, Durst used the following characteristics to distinguish a congenital cyst from an acquired one: 1) existence of associated congenital anomalies; 2) absence of signs of old or recent inflammation; 3) absence of vessels; and 4) presence of amblyopia and squint.2 We report a patient with a symptomatic congenital vitreous cyst, describing surgical management and histologic and immunohistochemical features of the cyst. Case Report A 58-year-old woman reported an increasingly large, mobile “floater” which had been intermittently symptomatic since childhood. She had undergone uneventful cataract extraction and posterior chamber lens placement several years before presentation. Best-corrected vision was 20/30 but decreased to 20/70 when the The authors have no financial interest in this article. Reprint requests: P. Kumar Rao, MD, Barnes Retina Institute, 1600 S. Brentwood Blvd, Suite 800, St. Louis, MO 63144; e-mail: [email protected], [email protected]
Fig. 1. Examination reveals a large, free-floating vitreous cyst.
60
61
FEATURES OF A VITREOUS CYST
small nucleolus (Figure 2A). Immunohistochemistry demonstrated the cells were positive for neuron specific enolase which identifies neural elements and glial fibrillary acidic protein (GFAP) which identifies glial elements (Figure 2, B and C). No cells stained for cytokeratin. While Alcian blue material, consistent with vitreous, was found in the wall of the cyst, there was no Alcian blue positive material in the cyst lumen.
Discussion Pigmented congenital cysts are thought to originate from the pars ciliaris epithelium whereas nonpigmented cysts are believed to be remnants of the hyaloid vascular system.3 In their description of a pigmented vitreous cyst, Orellana and colleagues described a single cuboidal layer of nonpigmented and pigmented cells with large melanin granules. They concluded that the cyst was consistent with pigment epithelium origin.1 Nork and Millecchia described the histologic features of another pigmented cyst composed of a single layer of pigmented cells with mature and immature melanosomes. Stains for actin and carbonic anhydrase were positive but negative for GFAP leading them to conclude that the cyst represented a choristoma of the hyaloid system.4 AsiyoVogel reported the histologic findings of a vitreous cyst removed from a patient who developed the cyst after multiple retinal detachment repair procedures.5 Like the case described by Asiyo-Vogel, our specimen also suggests neuronal elements with gliosis on immunohistochemistry; however, our patient did not have an antecedent history of vitreoretinal surgery or trauma. Our specimen may be consistent with glial remnants of Bergmeister papilla.
References 1.
2. 3. Fig. 2. A, Hematoxylin and eosin staining reveal a multilayer cellular cyst wall. The cells are composed of pink cytoplasm with indistinct borders and the spindle nuclei appear bland with a single small nucleolus. B, Immunohistochemistry confirms positive neuron specific enolase and C, glial fibrillary acidic protein.
4. 5.
Orellana J, O’Malley RE, McPherson AR, Font RL. Pigmented free-floating vitreous cysts in two young adults. Electron microscopic observations. Ophthalmology 1985;92:297–302. Francois J. Pre-papillary cyst developed from remnants of the hyaloid artery. Br J Ophthalmol 1950;34:365–368. Bayraktar Z, Kapran Z, Ozdogan S. Pigmented congenital vitreous cyst. Eur J Ophthalmol 2004;14:156–158. Nork TM, Millecchia LL. Treatment and histopathology of a congenital vitreous cyst. Ophthalmology 1998;105:825–830. Asiyo-Vogel MN, El-Hifnawi E, Laqua H. Ultrastructural features of a solitary vitreous cyst. Retina 1996;16:250–254.
