VITRECTOMY AND WAGNER'S VITREORETINAL D E G E N E R A T I O N G A R Y C. B R O W N , M.D.,
AND W I L L I A M S. TASMAN,
Philadelphia,
First described by Wagner 1 in 1938, autosomal dominant vitreoretinal degen eration encompasses a multitude of ocu lar signs. Included in the original de scription are vitreous liquefaction and band formation, radial perivascular pig mentation, peripheral retinal pigmenta tion (probably lattice degeneration), peripapillary chorioretinal atrophy, nar row retinal arterioles, cataracts, myopia, and optic atrophy. Since that time, retinal breaks, 2 - 6 perivascular chorioretinal atro phy, 2 and white without pressure 3 have also been described. Retinal detachment is now recognized to be associated often with the disease and has been reported to be present in 47 to 67% of cases. 2 - 4 Redetachments following surgical repair are common. 2 - 6 We describe herein three patients with Wagner's vitreoretinal degeneration who underwent vitrectomy, and suggest guidelines for vitrectomy as a therapeutic adjunct in the treatment of this disease.
M.D.
Pennsylvania nation of the left eye showed a bullous rhegmatogenous retinal detachment extending from the temporal periphery to the optic disk (Fig. 1). Vitreous bands were present, and the patient was thought to have Wagner's vitreoretinal degeneration. Repair of the detached retina was undertaken on April 14 with scleral flaps, diathermy in a scleral bed, transscleral cryopexy, a silicone tire, an encir cling band, and drainage of subretinal fluid. Subretinal fluid accumulation was minimal the first postoperative day, but had increased by the second postoperative day. This condition necessitated reoperation, at which time the original 8-mm im plant was replaced with a 12-mm implant. Once again the retina was flat postoperatively, but redetached within three weeks. At this time there was apparent developing periretinal membrane for mation. Revision of the scleral buckle was unsuc cessfully attempted on May 28, 1976, and on June 5, the patient underwent pars plana vitrectomy with further revision of the scleral buckle. On most recent examination on Oct. 4, 1977, 16 months later, the visual acuity in the left eye was 6/30 (20/100), and the retina was flat (Fig. 2). Case 2—An 11-year-old girl came here in Febru ary 1975 with a two-month history of decreasing
CASE REPORTS Case 1—A 14-year-old boy came to the emergency room April 12, 1976, with a history of loss of vision in the left eye of seven hours' duration. Aside from a -16.00 sphere refractive error in both eyes, the pa tient's history was noncontributory for eye disease. History of family ocular disorders included one sister with a retinal detachment and another sister with vitreous membranes and extensive peripheral retinal pigmentary changes. Visual acuity at the time of examination was R.E.: 6/15 (20/50); L.E.: 6/60 (20/200). The anterior seg ments were noted to be normal bilaterally. Ophthalmoscopic examination of the right eye revealed equatorial lattice degeneration temporally. ExamiFrom the Retina Service, Wills Eye Hospital and Research Institute, Philadelphia, Pennsylvania. This study was supported in part by the Retina Research and Development Foundation, Philadel phia, and the Lions Club of Pennsylvania. Reprint requests to Gary C. Brown, M.D., Wills Eye Hospital, 1601 Spring Garden St., Philadelphia, PA 19130.
Fig. 1 (Brown and Tasman). Case 1. Initial exam ination. Rhegmatogenous retinal detachment with a horseshoe tear and two holes. Note the hypopigmented peripapillary and perivascular areas, perivascular pigmentary deposition, equatorial lattice degeneration, and vitreous bands (one of which is identified by an arrow).
AMERICAN JOURNAL O F OPHTHALMOLOGY 86:485-488, 1978
485
486
AMERICAN JOURNAL OF OPHTHALMOLOGY
Fig. 2 (Brown and Tasman). Case 1. After vitrecto my. A remnant of one of the vitreous bands (arrow) cut during surgery is seen in this wide-angle photo graph.
vision in the right eye. At 5 years of age she had had rapidly decreasing vision in the left eye, the cause of which was undetermined. Family history revealed her mother had decreased vision bilaterally secon dary to retinal detachments, and her two brothers had retinal detachments. Several aunts and uncles had histories of impaired vision, the causes of which were unknown. Examination on Feb. 25, 1975, revealed the visual acuity to be R.E.: finger counting at 6 ft; L.E.: no light perception. Refractive error in the right eye was a -11.50 sphere. Refractive error in the left eye was unobtainable because the eye was phthisical with band keratopathy. The anterior segment of the right eye demonstrated 1 + flare and cells, but other wise was within normal limits. Ophthalmoscopic examination disclosed a total retinal detachment with a 90-degree superonasal giant tear and a 20degree superotemporal tear (Fig. 3). Equatorial and perivascular pigmentary changes were noted, and vitreous bands were present. Because of these findings and the family history, the patient was thought to have Wagner's vitreoretinal degeneration. Repair of the detached retina was attempted with scleral flaps, diathermy to the bed, a silicone plate, an encircling band, and drainage of subretinal fluid. Fluid remained inferiorly postoperatively, and by April 2, 1975, the retina had again detached totally with apparent massive periretinal proliferation. A second attempt at repair was made on April 8 by using a scleral bed with cryotherapy, an encircling band, and subretinal fluid drainage, but apparent massive periretinal proliferation remained (Fig. 4). On May 19, the patient underwent combined lensectomy and open-sky vitrectomy. An 8.5-mm corneal button was removed at the start of the procedure and was replaced upon termination of the
OCTOBER,
1978
Fig. 3 (Brown and Tasman). Case 2. Initial exami nation. Bullous total retinal detachment with two tears and a hole. Vitreous bands and pigmentary changes, as in Case 1, are present.
vitrectomy. On follow-up examinations, the retina has remained flat (Fig. 5), but the visual acuity was, disappointingly, hand motion when the patient was last seen in March 1977. Case 3—A 3-year-old girl was examined by her primary ophthalmologist on April 26, 1976, because
Fig. 4. (Brown and Tasman). Case 2. Previtrectomy. Fundus as it appeared after the second unsuccessful retinal detachment repair. The picture is that of apparent massive periretinal prolif eration.
VOL. 86, NO. 4
WAGNER'S VITREORETINAL DEGENERATION
Fig. 5 (Brown and Tasman). Case 2. After vitrecto my. Two of the smaller vitreous bands (arrows) still remain. The retina was flat at this time and had remained so at the 22-month follow-up examination.
of parental concern over the progressive darkening in color of her right iris. Ocular examination confirmed this finding and also revealed the pres ence of a cataract and total retinal detachment in the right eye. The patient was admitted here, where examination under anesthesia on April 29 confirmed the presence of a posterior subcapsular cataract and a total retinal detachment with two nasal horseshoe tears in the right eye (Fig. 6). A - 8 . 0 0 sphere refrac tive error was noted in the left eye, and vitreous bands were present in both eyes. Ophthalmologic examination of family members revealed her father and a paternal aunt and uncle to have findings classic for Wagner's vitreoretinal degeneration. On May 3, repair of the detachment was attempted with scleral flaps and a silicone tire, transscleral cryotherapy, an encircling band, and subretinal fluid drainage. A small amount of subretinal fluid remained, but on May 10, examination under anesthesis once again disclosed a bullous total reti nal detachment in the right eye with massive periretinal proliferation. The patient underwent re vision of the scleral buckle, lensectomy, and pars plana vitrectomy on June 28, 1976. The retina re mained flat on most recent examination in June 1977. DISCUSSION
Prognosis for successful reattachment of the retina in Wagner's disease is poor. Hirose, Lee, and Schepens 3 reported 17 (52%) of operated eyes to be detached at six months in their series of 33 eyes. Addi-
487
Fig. 6 (Brown and Tasman) Case 3. Fundus on initial examination revealing a bullous total retinal detachment with two nasal horseshoe tears. Equato rial lattice degeneration and vitreous bands are present, as in the other cases.
tionally, of those eyes undergoing three or more retinal detachment repairs, only three of ten (30%) remained attached. Hagler and Crosswell 2 had surgical fail ure in 36% of the eyes of their series of 22 patients with perivascular chorioretinal degeneration, myopia, presenile cataracts, and vitreous veils. Vitrectomy has been reported to be of therapeutic value in patients with retinal detachment and vitreous bands, 7 - 1 1 but to our knowledge, there have been no re ports of this procedure being used specif ically for Wagner's disease. Vitrectomy seems to be a therapeutic adjunct follow ing unsuccessful retinal detachment re pair using conventional scleral buckling and encircling techniques. The retinas of our three patients have now remained attached for 16, 22, and 12 months, re spectively, after vitrectomy. The patient in Case 1 underwent three unsuccessful retinal detachment procedures before successful vitrectomy, whereas the pa tient in Case 2 underwent attempted re pair twice, and in Case 3, once.
488
AMERICAN JOURNAL OF OPHTHALMOLOGY
At no time in these cases was mem brane stripping attempted, although the fundus pictures suggested massive periretinal proliferation. The majority of the gel was removed with the intravitreal bands. We believe that these bands are vitally important in the production of retinal detachment associated with Wag ner's disease. In our cases, they may also have been partially responsible for the appearance of apparent massive periretinal proliferation after unsuccessful reti nal detachment repair. Removal of the bands may explain successful reattachment of the retinas. Whereas scleral buckling and encir cling band procedures comprise initial management, vitrectomy should be con sidered in the therapeutic regime, espe cially in reoperative procedures. SUMMARY
A 14-year-old boy, an 11-year-old girl, and a 3-year-old girl with Wagner's vitreoretinal degeneration had multiple reti nal detachments. Surgical repair by means of conventional scleral buckling and encircling techniques was unsuccess ful. However, after vitrectomy, their reti nas remained flat.
OCTOBER, 1978
REFERENCES 1. Wagner, H.: Ein bisher unbekantes Erbleiden des Auges (Degeneratio hyaloideo-retinalis hereditaria), beobachtet im Kanton Zurich. Klin. Monatsbl. Augenheilkd. 100:840, 1938. 2. Hagler, W. S., and Crosswell, H. H.: Radial perivascular chorioretinal degeneration and retinal detachment. Trans. Am. Acad. Ophthalmol. Otolaryngol. 72:203, 1968. 3. Hirose, T., Lee, K., and Schepens, C : Wagner's hereditary vitreoretinal degeneration and retinal de tachment. Arch. Ophthalmol. 89:176, 1973. 4. Frandsen, E.: Hereditary hyaloideo-retinal de generation (Wagner) in a Danish family. Acta Ophthalmol. 44:223, 1966. 5. Van Balen, A. Th. M., and Falger, E. L. F.: Hereditary hyaloideo-retinal degeneration and palatoschisis. Arch. Ophthalmol. 83:152, 1970. 6. Alexander, R. L., and Shea, M.: Wagner's dis ease. Arch. Ophthalmol. 74:310, 1965. 7. Freeman, H. M.: Vitreous surgery: Current sta tus of vitreous surgery in cases of rhegmatogenous retinal detachment. Trans. Am. Acad. Ophthalmol. Otolaryngol. 77:202, 1973. 8. Machemer, R., and Norton, E. W. D.: A new concept for vitreous surgery. 3. Indications and Results. Am. J. Ophthalmol. 74:1034, 1972. 9. Peyman, G., Huamonte, F. U., and Goldberg, M. F.: One hundred consecutive pars plana vitrectomies using the vitrophage. Am. J. Ophthalmol. 81:263, 1976. 10. Treister, G., and Machemer, R.: Results of vitrectomy for rare proliferative and hemorrhagic diseases. Am. J. Ophthalmol. 84:394, 1977. 11. Peyman, G., Huamonte, F., Goldberg, M., Sanders, D., Nagpal, K., and Raichand, M.: Four hundred consecutive pars plana vitrectomies with the vitrophage. Arch. Ophthalmol. 96:45, 1978.
AND W I L L I A M S. TASMAN,
Philadelphia,
First described by Wagner 1 in 1938, autosomal dominant vitreoretinal degen eration encompasses a multitude of ocu lar signs. Included in the original de scription are vitreous liquefaction and band formation, radial perivascular pig mentation, peripheral retinal pigmenta tion (probably lattice degeneration), peripapillary chorioretinal atrophy, nar row retinal arterioles, cataracts, myopia, and optic atrophy. Since that time, retinal breaks, 2 - 6 perivascular chorioretinal atro phy, 2 and white without pressure 3 have also been described. Retinal detachment is now recognized to be associated often with the disease and has been reported to be present in 47 to 67% of cases. 2 - 4 Redetachments following surgical repair are common. 2 - 6 We describe herein three patients with Wagner's vitreoretinal degeneration who underwent vitrectomy, and suggest guidelines for vitrectomy as a therapeutic adjunct in the treatment of this disease.
M.D.
Pennsylvania nation of the left eye showed a bullous rhegmatogenous retinal detachment extending from the temporal periphery to the optic disk (Fig. 1). Vitreous bands were present, and the patient was thought to have Wagner's vitreoretinal degeneration. Repair of the detached retina was undertaken on April 14 with scleral flaps, diathermy in a scleral bed, transscleral cryopexy, a silicone tire, an encir cling band, and drainage of subretinal fluid. Subretinal fluid accumulation was minimal the first postoperative day, but had increased by the second postoperative day. This condition necessitated reoperation, at which time the original 8-mm im plant was replaced with a 12-mm implant. Once again the retina was flat postoperatively, but redetached within three weeks. At this time there was apparent developing periretinal membrane for mation. Revision of the scleral buckle was unsuc cessfully attempted on May 28, 1976, and on June 5, the patient underwent pars plana vitrectomy with further revision of the scleral buckle. On most recent examination on Oct. 4, 1977, 16 months later, the visual acuity in the left eye was 6/30 (20/100), and the retina was flat (Fig. 2). Case 2—An 11-year-old girl came here in Febru ary 1975 with a two-month history of decreasing
CASE REPORTS Case 1—A 14-year-old boy came to the emergency room April 12, 1976, with a history of loss of vision in the left eye of seven hours' duration. Aside from a -16.00 sphere refractive error in both eyes, the pa tient's history was noncontributory for eye disease. History of family ocular disorders included one sister with a retinal detachment and another sister with vitreous membranes and extensive peripheral retinal pigmentary changes. Visual acuity at the time of examination was R.E.: 6/15 (20/50); L.E.: 6/60 (20/200). The anterior seg ments were noted to be normal bilaterally. Ophthalmoscopic examination of the right eye revealed equatorial lattice degeneration temporally. ExamiFrom the Retina Service, Wills Eye Hospital and Research Institute, Philadelphia, Pennsylvania. This study was supported in part by the Retina Research and Development Foundation, Philadel phia, and the Lions Club of Pennsylvania. Reprint requests to Gary C. Brown, M.D., Wills Eye Hospital, 1601 Spring Garden St., Philadelphia, PA 19130.
Fig. 1 (Brown and Tasman). Case 1. Initial exam ination. Rhegmatogenous retinal detachment with a horseshoe tear and two holes. Note the hypopigmented peripapillary and perivascular areas, perivascular pigmentary deposition, equatorial lattice degeneration, and vitreous bands (one of which is identified by an arrow).
AMERICAN JOURNAL O F OPHTHALMOLOGY 86:485-488, 1978
485
486
AMERICAN JOURNAL OF OPHTHALMOLOGY
Fig. 2 (Brown and Tasman). Case 1. After vitrecto my. A remnant of one of the vitreous bands (arrow) cut during surgery is seen in this wide-angle photo graph.
vision in the right eye. At 5 years of age she had had rapidly decreasing vision in the left eye, the cause of which was undetermined. Family history revealed her mother had decreased vision bilaterally secon dary to retinal detachments, and her two brothers had retinal detachments. Several aunts and uncles had histories of impaired vision, the causes of which were unknown. Examination on Feb. 25, 1975, revealed the visual acuity to be R.E.: finger counting at 6 ft; L.E.: no light perception. Refractive error in the right eye was a -11.50 sphere. Refractive error in the left eye was unobtainable because the eye was phthisical with band keratopathy. The anterior segment of the right eye demonstrated 1 + flare and cells, but other wise was within normal limits. Ophthalmoscopic examination disclosed a total retinal detachment with a 90-degree superonasal giant tear and a 20degree superotemporal tear (Fig. 3). Equatorial and perivascular pigmentary changes were noted, and vitreous bands were present. Because of these findings and the family history, the patient was thought to have Wagner's vitreoretinal degeneration. Repair of the detached retina was attempted with scleral flaps, diathermy to the bed, a silicone plate, an encircling band, and drainage of subretinal fluid. Fluid remained inferiorly postoperatively, and by April 2, 1975, the retina had again detached totally with apparent massive periretinal proliferation. A second attempt at repair was made on April 8 by using a scleral bed with cryotherapy, an encircling band, and subretinal fluid drainage, but apparent massive periretinal proliferation remained (Fig. 4). On May 19, the patient underwent combined lensectomy and open-sky vitrectomy. An 8.5-mm corneal button was removed at the start of the procedure and was replaced upon termination of the
OCTOBER,
1978
Fig. 3 (Brown and Tasman). Case 2. Initial exami nation. Bullous total retinal detachment with two tears and a hole. Vitreous bands and pigmentary changes, as in Case 1, are present.
vitrectomy. On follow-up examinations, the retina has remained flat (Fig. 5), but the visual acuity was, disappointingly, hand motion when the patient was last seen in March 1977. Case 3—A 3-year-old girl was examined by her primary ophthalmologist on April 26, 1976, because
Fig. 4. (Brown and Tasman). Case 2. Previtrectomy. Fundus as it appeared after the second unsuccessful retinal detachment repair. The picture is that of apparent massive periretinal prolif eration.
VOL. 86, NO. 4
WAGNER'S VITREORETINAL DEGENERATION
Fig. 5 (Brown and Tasman). Case 2. After vitrecto my. Two of the smaller vitreous bands (arrows) still remain. The retina was flat at this time and had remained so at the 22-month follow-up examination.
of parental concern over the progressive darkening in color of her right iris. Ocular examination confirmed this finding and also revealed the pres ence of a cataract and total retinal detachment in the right eye. The patient was admitted here, where examination under anesthesia on April 29 confirmed the presence of a posterior subcapsular cataract and a total retinal detachment with two nasal horseshoe tears in the right eye (Fig. 6). A - 8 . 0 0 sphere refrac tive error was noted in the left eye, and vitreous bands were present in both eyes. Ophthalmologic examination of family members revealed her father and a paternal aunt and uncle to have findings classic for Wagner's vitreoretinal degeneration. On May 3, repair of the detachment was attempted with scleral flaps and a silicone tire, transscleral cryotherapy, an encircling band, and subretinal fluid drainage. A small amount of subretinal fluid remained, but on May 10, examination under anesthesis once again disclosed a bullous total reti nal detachment in the right eye with massive periretinal proliferation. The patient underwent re vision of the scleral buckle, lensectomy, and pars plana vitrectomy on June 28, 1976. The retina re mained flat on most recent examination in June 1977. DISCUSSION
Prognosis for successful reattachment of the retina in Wagner's disease is poor. Hirose, Lee, and Schepens 3 reported 17 (52%) of operated eyes to be detached at six months in their series of 33 eyes. Addi-
487
Fig. 6 (Brown and Tasman) Case 3. Fundus on initial examination revealing a bullous total retinal detachment with two nasal horseshoe tears. Equato rial lattice degeneration and vitreous bands are present, as in the other cases.
tionally, of those eyes undergoing three or more retinal detachment repairs, only three of ten (30%) remained attached. Hagler and Crosswell 2 had surgical fail ure in 36% of the eyes of their series of 22 patients with perivascular chorioretinal degeneration, myopia, presenile cataracts, and vitreous veils. Vitrectomy has been reported to be of therapeutic value in patients with retinal detachment and vitreous bands, 7 - 1 1 but to our knowledge, there have been no re ports of this procedure being used specif ically for Wagner's disease. Vitrectomy seems to be a therapeutic adjunct follow ing unsuccessful retinal detachment re pair using conventional scleral buckling and encircling techniques. The retinas of our three patients have now remained attached for 16, 22, and 12 months, re spectively, after vitrectomy. The patient in Case 1 underwent three unsuccessful retinal detachment procedures before successful vitrectomy, whereas the pa tient in Case 2 underwent attempted re pair twice, and in Case 3, once.
488
AMERICAN JOURNAL OF OPHTHALMOLOGY
At no time in these cases was mem brane stripping attempted, although the fundus pictures suggested massive periretinal proliferation. The majority of the gel was removed with the intravitreal bands. We believe that these bands are vitally important in the production of retinal detachment associated with Wag ner's disease. In our cases, they may also have been partially responsible for the appearance of apparent massive periretinal proliferation after unsuccessful reti nal detachment repair. Removal of the bands may explain successful reattachment of the retinas. Whereas scleral buckling and encir cling band procedures comprise initial management, vitrectomy should be con sidered in the therapeutic regime, espe cially in reoperative procedures. SUMMARY
A 14-year-old boy, an 11-year-old girl, and a 3-year-old girl with Wagner's vitreoretinal degeneration had multiple reti nal detachments. Surgical repair by means of conventional scleral buckling and encircling techniques was unsuccess ful. However, after vitrectomy, their reti nas remained flat.
OCTOBER, 1978
REFERENCES 1. Wagner, H.: Ein bisher unbekantes Erbleiden des Auges (Degeneratio hyaloideo-retinalis hereditaria), beobachtet im Kanton Zurich. Klin. Monatsbl. Augenheilkd. 100:840, 1938. 2. Hagler, W. S., and Crosswell, H. H.: Radial perivascular chorioretinal degeneration and retinal detachment. Trans. Am. Acad. Ophthalmol. Otolaryngol. 72:203, 1968. 3. Hirose, T., Lee, K., and Schepens, C : Wagner's hereditary vitreoretinal degeneration and retinal de tachment. Arch. Ophthalmol. 89:176, 1973. 4. Frandsen, E.: Hereditary hyaloideo-retinal de generation (Wagner) in a Danish family. Acta Ophthalmol. 44:223, 1966. 5. Van Balen, A. Th. M., and Falger, E. L. F.: Hereditary hyaloideo-retinal degeneration and palatoschisis. Arch. Ophthalmol. 83:152, 1970. 6. Alexander, R. L., and Shea, M.: Wagner's dis ease. Arch. Ophthalmol. 74:310, 1965. 7. Freeman, H. M.: Vitreous surgery: Current sta tus of vitreous surgery in cases of rhegmatogenous retinal detachment. Trans. Am. Acad. Ophthalmol. Otolaryngol. 77:202, 1973. 8. Machemer, R., and Norton, E. W. D.: A new concept for vitreous surgery. 3. Indications and Results. Am. J. Ophthalmol. 74:1034, 1972. 9. Peyman, G., Huamonte, F. U., and Goldberg, M. F.: One hundred consecutive pars plana vitrectomies using the vitrophage. Am. J. Ophthalmol. 81:263, 1976. 10. Treister, G., and Machemer, R.: Results of vitrectomy for rare proliferative and hemorrhagic diseases. Am. J. Ophthalmol. 84:394, 1977. 11. Peyman, G., Huamonte, F., Goldberg, M., Sanders, D., Nagpal, K., and Raichand, M.: Four hundred consecutive pars plana vitrectomies with the vitrophage. Arch. Ophthalmol. 96:45, 1978.
