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Subconjunctival Silicone Oil After Vitreoretinal Surgery
Letters to The Journal
Vol. 115, No.1
Multiple mitochondrial DNA deletions have been found in several different neurologic syndromes, including variants of chronic progressive external ophthalmoplegia and recurrent myoglobinuria.v" Both autosomal dominant and autosomal recessive inheritance patterns have been noted, but all patients had ragged red fibers on skeletal muscle biopsy specimens and harbored multiple mitochondrial DNA deletions with similar molecular features. Despite the wide variety of accompanying signs and symptoms, the only prominent gastrointestinal feature was dysphagia in one pedigree.' Myo-neuro-gastrointestinal encephalopathy syndrome should be added to the growing list of neurologic syndromes associated with multiple mitochondrial DNA deletions. We predict that further human diseases will be pathogenetically linked to multiple mitochondrial DNA deletions and that most will have chronic progressive external ophthalmoplegia as an integral feature. However, a number of other organ systems may be involved as well. Our case is unique among cases of multiple mitochondrial DNA deletions because of the association with the myo-neuro-gastrointestinal encephalopathy syndrome and the lack of ragged red fibers in skeletal muscle. The myo-neuro-gastrointestinal encephalopathy syndrome exhibits noteworthy genetic heterogeneity, and determination of the relative frequency of multiple mitochondrial DNA deletions in patients with this syndrome awaits more extensive studies.
References 1. Threlkeld, A. B., Miller, N. R., Golnik, K. c., Griffin, J. W., Kuncl, R. W., Johns, D. R., Lehar, M., and Hurko, 0.: Ophthalmic involvement in myoneuro-gastrointestinal encephalopathy syndrome. Am. J. Ophthalmol. 114:322, 1992. 2. Lauber, J., Marsac, c., Kadenbach, B., and Seibel, P.: Mutations in mitochondrial tRNA genes. A frequent case of neuromuscular diseases. Nucleic Acids Res. 19:1393, 1991. 3. Johns, D. R., and Hurko, 0.: Preferential amplification and molecular characterization of junction sequences of a pathogenetic deletion in human mitochondrial DNA. Genomics 5:623, 1989. 4. Zeviani, M.: Nucleus-driven mutations of human mitochondrial DNA. J. lnher. Metab. Dis. 15:456,1992. 5. Suornalainen, A., Majander, A., Haltia, M.,
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Somer, H., Lonnqvist. J., Savontaus, M.-L., and Peltonen, L.: Multiple deletions of mitochondrial DNA in several tissues of a patient with severe retarded depression and familial progressive external ophthalmoplegia. J. Clin. Invest. 90:61, 1992.
Subconjunctival Silicone Oil After Vitreoretinal Surgery Amy K. Hutchinson, M.D., Antonio Capone, M.D., and Hans E. Grossniklaus, M.D.
Department of Ophthalmology, Emory University School of Medicine. Inquiries to Hans E. Groseniklaus, M.D., L. F. Montgomery Eye Pathology Laboratory, Emory Eye Center, 1327 Clifton Rd. N.E., Atlanta, GA 30322. Complications associated with the use of intraocular silicone oil have been well documented, and extravasation of the silicone oil into the subconjunctival space has been reported.P The clinicopathologic features of this complication, however, have not been well documented. A 57-year-old man was examined for severe proliferative vitreoretinopathy and a second recurrence of a retinal detachment in his right eye. He had had an extracapsular cataract extraction and a posterior chamber intraocular lens implant, placement of a scleral buckle, and a vitrectomy for a recurrent retinal detachment. Examination showed visual acuity to be hand motions in the right eye. Ophthalmoscopy showed fixed retinal folds, a prominent area of proliferative vitreoretinopathy, and several large retinal breaks. The patient underwent a second vitrectomy with retinal membrane dissection, photocoagulation, removal of the intraocular lens and silicone oil injection. Two months postoperatively, visual acuity in the right eye was 20/400 and the retina remained attached; however, a diffuse bulbar conjunctival subepithelial infiltrate was noted. The infiltrate coalesced over a period of several weeks to form transparent, firm subepithelial droplets (Fig. 1). The patient underwent removal of the in traocular silicone oil and excision of the in vol ved conjunctiva. Histopathologic examination of the conjunctival specimen showed fibrovascular tissue infiltrated with chronic inflammatory cells including foreign body giant cells. Vacuolated spaces were identified that corresponded to extruded silicone oil (Fig. 2). Ultrastructural
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January, 1993
AMERICAN JOURNAL OF OPHTHALMOLOGY
Fig. 1 (Hutchinson, Capone, and Grossniklaus). Top, External examination shows bulbar conjunctival subepithelial accumulations of transparent material. Bottom, Closer inspection discloses the droplike appearance of the material.
examination confirmed the presence of intracellular and extracellular accumulations of silicone oil droplets. We have seen another patient with subconjunctival silicone oil after retinal detachment surgery. As with our first patient, the lesion in our second patient initially appeared as a diffuse subepithelial mass infiltrate that coalesced into transparent droplets, suggesting a pattern in the clinical evolution of subconjunctival silicone oil. There are several cystic and noncystic subepithelial bulbar conjunctival masses in the clinical differential diagnosis of subconjunctival silicone oil. The cystic entities include epithelial inclusion cyst, lymphangiectasia, Leber's lymphangiectasia hemorrhagica, and cystic amelanotic nevi. Noncystic subepithelial masses that may be similar in clinical appearance to subepithelial silicone oil include lymphoma, myxoma, vascular tumors, and other rare lesions." The clinical history and findings from external examination can help exclude these lesions.
Fig. 2 (Hutchinson, Capone, and Grossniklaus). Top, The conjunctival biopsy specimen exhibits extracellular vacuoles (asterisk) and occasional foreign body giant cells (arrow) (toluidine blue, x 160). Bottom, Ultrastructural features of the biopsy specimen include endothelial-lined (end) vascular channels, extracellular erythrocytes (rbc), and silicone oil droplets (sil) (X 1,900).
References 1. Federman, J. L., and Schubert, H. D.: Complications associated with the use of silicone oil in 150 eyes after retinovitreous surgery. Ophthalmology 95:870,1988. 2. Honda, Y., Ueno, 5., Miura, M., and Yamaguchi, H.: Silicone oil particles trapped in the subretinal space. Complications after substitution of the vitreous. Ophthalmologica 192:1, 1986. 3. Grossniklaus, H. E., Green, W. R., Luckenbach, M., and Chan, C. c.: Conjunctival lesions in adults. A clinical and histopathologic review. Cornea 6:78, 1987.
Vol. 115, No.1
Multiple mitochondrial DNA deletions have been found in several different neurologic syndromes, including variants of chronic progressive external ophthalmoplegia and recurrent myoglobinuria.v" Both autosomal dominant and autosomal recessive inheritance patterns have been noted, but all patients had ragged red fibers on skeletal muscle biopsy specimens and harbored multiple mitochondrial DNA deletions with similar molecular features. Despite the wide variety of accompanying signs and symptoms, the only prominent gastrointestinal feature was dysphagia in one pedigree.' Myo-neuro-gastrointestinal encephalopathy syndrome should be added to the growing list of neurologic syndromes associated with multiple mitochondrial DNA deletions. We predict that further human diseases will be pathogenetically linked to multiple mitochondrial DNA deletions and that most will have chronic progressive external ophthalmoplegia as an integral feature. However, a number of other organ systems may be involved as well. Our case is unique among cases of multiple mitochondrial DNA deletions because of the association with the myo-neuro-gastrointestinal encephalopathy syndrome and the lack of ragged red fibers in skeletal muscle. The myo-neuro-gastrointestinal encephalopathy syndrome exhibits noteworthy genetic heterogeneity, and determination of the relative frequency of multiple mitochondrial DNA deletions in patients with this syndrome awaits more extensive studies.
References 1. Threlkeld, A. B., Miller, N. R., Golnik, K. c., Griffin, J. W., Kuncl, R. W., Johns, D. R., Lehar, M., and Hurko, 0.: Ophthalmic involvement in myoneuro-gastrointestinal encephalopathy syndrome. Am. J. Ophthalmol. 114:322, 1992. 2. Lauber, J., Marsac, c., Kadenbach, B., and Seibel, P.: Mutations in mitochondrial tRNA genes. A frequent case of neuromuscular diseases. Nucleic Acids Res. 19:1393, 1991. 3. Johns, D. R., and Hurko, 0.: Preferential amplification and molecular characterization of junction sequences of a pathogenetic deletion in human mitochondrial DNA. Genomics 5:623, 1989. 4. Zeviani, M.: Nucleus-driven mutations of human mitochondrial DNA. J. lnher. Metab. Dis. 15:456,1992. 5. Suornalainen, A., Majander, A., Haltia, M.,
109
Somer, H., Lonnqvist. J., Savontaus, M.-L., and Peltonen, L.: Multiple deletions of mitochondrial DNA in several tissues of a patient with severe retarded depression and familial progressive external ophthalmoplegia. J. Clin. Invest. 90:61, 1992.
Subconjunctival Silicone Oil After Vitreoretinal Surgery Amy K. Hutchinson, M.D., Antonio Capone, M.D., and Hans E. Grossniklaus, M.D.
Department of Ophthalmology, Emory University School of Medicine. Inquiries to Hans E. Groseniklaus, M.D., L. F. Montgomery Eye Pathology Laboratory, Emory Eye Center, 1327 Clifton Rd. N.E., Atlanta, GA 30322. Complications associated with the use of intraocular silicone oil have been well documented, and extravasation of the silicone oil into the subconjunctival space has been reported.P The clinicopathologic features of this complication, however, have not been well documented. A 57-year-old man was examined for severe proliferative vitreoretinopathy and a second recurrence of a retinal detachment in his right eye. He had had an extracapsular cataract extraction and a posterior chamber intraocular lens implant, placement of a scleral buckle, and a vitrectomy for a recurrent retinal detachment. Examination showed visual acuity to be hand motions in the right eye. Ophthalmoscopy showed fixed retinal folds, a prominent area of proliferative vitreoretinopathy, and several large retinal breaks. The patient underwent a second vitrectomy with retinal membrane dissection, photocoagulation, removal of the intraocular lens and silicone oil injection. Two months postoperatively, visual acuity in the right eye was 20/400 and the retina remained attached; however, a diffuse bulbar conjunctival subepithelial infiltrate was noted. The infiltrate coalesced over a period of several weeks to form transparent, firm subepithelial droplets (Fig. 1). The patient underwent removal of the in traocular silicone oil and excision of the in vol ved conjunctiva. Histopathologic examination of the conjunctival specimen showed fibrovascular tissue infiltrated with chronic inflammatory cells including foreign body giant cells. Vacuolated spaces were identified that corresponded to extruded silicone oil (Fig. 2). Ultrastructural
110
January, 1993
AMERICAN JOURNAL OF OPHTHALMOLOGY
Fig. 1 (Hutchinson, Capone, and Grossniklaus). Top, External examination shows bulbar conjunctival subepithelial accumulations of transparent material. Bottom, Closer inspection discloses the droplike appearance of the material.
examination confirmed the presence of intracellular and extracellular accumulations of silicone oil droplets. We have seen another patient with subconjunctival silicone oil after retinal detachment surgery. As with our first patient, the lesion in our second patient initially appeared as a diffuse subepithelial mass infiltrate that coalesced into transparent droplets, suggesting a pattern in the clinical evolution of subconjunctival silicone oil. There are several cystic and noncystic subepithelial bulbar conjunctival masses in the clinical differential diagnosis of subconjunctival silicone oil. The cystic entities include epithelial inclusion cyst, lymphangiectasia, Leber's lymphangiectasia hemorrhagica, and cystic amelanotic nevi. Noncystic subepithelial masses that may be similar in clinical appearance to subepithelial silicone oil include lymphoma, myxoma, vascular tumors, and other rare lesions." The clinical history and findings from external examination can help exclude these lesions.
Fig. 2 (Hutchinson, Capone, and Grossniklaus). Top, The conjunctival biopsy specimen exhibits extracellular vacuoles (asterisk) and occasional foreign body giant cells (arrow) (toluidine blue, x 160). Bottom, Ultrastructural features of the biopsy specimen include endothelial-lined (end) vascular channels, extracellular erythrocytes (rbc), and silicone oil droplets (sil) (X 1,900).
References 1. Federman, J. L., and Schubert, H. D.: Complications associated with the use of silicone oil in 150 eyes after retinovitreous surgery. Ophthalmology 95:870,1988. 2. Honda, Y., Ueno, 5., Miura, M., and Yamaguchi, H.: Silicone oil particles trapped in the subretinal space. Complications after substitution of the vitreous. Ophthalmologica 192:1, 1986. 3. Grossniklaus, H. E., Green, W. R., Luckenbach, M., and Chan, C. c.: Conjunctival lesions in adults. A clinical and histopathologic review. Cornea 6:78, 1987.