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Management of Advanced Retinopathy of Prematurity in the Older Patient
Management of Advanced Retinopathy of Prematurity in the Older Patient J. DAVID BRADFORD, MD, MICHAEL T. TRESE, MD
Abstract: The authors studied 11 eyes of 10 patients who complained of a recent decrease in vision and whose eyes had the clinical appearance of stage 5 retinopathy of prematurity (RaP) as diagnosed by the presence of a dense, opaque retrolenticular membrane. Patients ranged in age from 4 to 33 years, and birth weights ranged from 680 to 1077 g. All eyes underwent vitrectomy, lensectomy, and membrane peeling and were found intraoperatively to have areas of retina that were attached (stage 48). Preoperatively, a reproducible visual-evoked potential waveform appeared to better predict this retinal configuration and potential retinal function than did the contact 8 scan ultrasound of the globe. Postoperatively, seven (63.6%) eyes demonstrated an improvement in vision. Vitreous surgery may be beneficial in selected eyes of older patients with advanced Rap to clear media opacities and to uncover areas of functional, attached retina, which may allow these patients to more effectively use the vision they possess. Ophthalmology 1991; 98: 11 05-1108
Since the conception of neonatal intensive care units, the survival rate of low birth weight infants has increased. This increased survival rate has resulted in an apparent increase in the incidence of retinopathy of prematurity (ROP). Many of these infants have had progression of their ocular disease to the more severe stages of ROP without receiving ocular therapy, such as cryotherapy, because organized strategies for examining these low birth weight infants and for treating advanced ROP have only been available in the past few years. A population of older patients exists who have had untreated advanced ROP but who have not lost all vision. Frequently, the ophthalmologist is asked to evaluate these older patients with advanced ROP who either complain of a reduction in vision or whose
Originally received: October 28, 1990. Revision accepted: March 1, 1991. From the Department of Ophthalmology, Beaumont Eye Institute, William Beaumont Hospital, Royal Oak, and the Department of Ophthalmology, Children 's Hospital, Detroit. Dr. Bradford is currently affiliated with the Retina Service. Department of Ophthalmology, University of Arkansas for Medical Sciences. Utile Rock . Presented in part at the American Academy of Ophthalmology Annual Meeting, Atlanta, Oct/Nov 1990. Reprint requests to Michael T. Trese, MD, Associated Retinal Consultants, 3535 W Thirteen Mile Rd, Suite 632, Royal Oak, MI 48073.
caretakers notice a reduction in the patient's visual function. Before this reduction in vision occurs, they may have had a level of vision that had helped them to ambulate. These patients generally present with a white, opaque retrolenticular membrane and are commonly described as having stage 5 ROP (total retinal detachment).l However, intraoperatively they are often found to have some area of functional-appearing retina that is attached. Therefore, despite their appearance as stage 5 ROP, they actually have stage 4B ROP (partial retinal detachment with macular involvement).l This study describes the preoperative evaluation and rationale for treatment of this group of older patients with advanced ROP.
PATIENTS AND METHODS The surgical records of one of the authors (MTT) from his practice at William Beaumont Hospital in Royal Oak, MI, and Children's Hospital in Detroit, MI, were retrospectively reviewed. Ten patients who were born prematurely with low birth weight and who were 4 years of age or older at the time of examination and surgery were identified. Between April 1988 and January 1989, 11 eyes (6 right eyes, 5 left eyes) underwent an initiallensectomy, vitrectomy, and membrane peeling procedure for advanced stages ofROP consisting of a white retrolenticular 1105
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Table 1. Profiles of Older Patients Who Underwent Vitreous Surgery for Advanced Retinopathy of Prematurity Age (yrs)/Sex/Eye
Birthweight (g)
4/M/OD
879
2 3 4 5 6 7
7/M/OD 16/F/OD 12/F/OS 17/F/OS 33/F/OS 30/F/OD
680 992 992 964 1077 964
8 9 10 11
9/M/OS 7/M/OD 7/M/OS 9/F/OD
907 800 800 Unknown (Adopted)
Eye No.
Preoperative Procedures
Cryo PK
Cryo x2
Cryo
Operating Procedures
Retinal Attachment
Visual Improvement
Glaucoma
Follow-up (mos)
(1) L, V, MP (2) V, MP, AFE L, V, MP L, V, MP L, V, MP L, V, MP, SB L, V, MP (1) L, V, MP (2) V, MP, AFE (3) V, MP, AFE L, V, MP, SB, Ret L, V, MP L, V, MP (1) L, V, MP, SB (2) V, MP, SO
Attached
Improved
Not present
9
Attached Attached Attached Attached Phthisis Unknown
Improved Improved Improved Improved NLP Not improved
Present Not present Present Not present Present Not present
17 22 12 14 15 18
Attached PA Attached Unknown
Improved NLP Improved Not improved
Present Not present Not present Not present
13 6 6 7
00 = right eye; L = lensectomy; V = vitrectomy; MP = membrane peeling; AFE = air-fluid exchange; Cryo = peripheral retinal cryotherapy; PK = penetrating keratoplasty; OS = left eye; SB = scleral buckle; NLP = no light perception; Ret = retinotomy; PA = peripheral attachment; SO =
silicone oil injection.
membrane and the clinical appearance of stage 5 ROP.I Ages of the patients at the time of surgery ranged from 4 to 33 years. Birth weights ranged between 680 and 1077 g. Patients were followed for 6 to 22 months. All patients presented with the recent onset of decreased vision and had the clinical appearance of stage 5 ROP as diagnosed by an opacity behind the lens obscuring the view of the retina. I Three eyes had previously undergone peripheral retinal cryotherapy for proliferative ROP. One eye had undergone penetrating keratoplasty before vitreous surgery for corneal decompensation and opacification. None of the patients had undergone previous vitreous surgery or scleral buckling procedures. Nine patients had visual acuity oflight perception, one patient had visual acuity of light perception with projection vision, and one patient had visual acuity of hand motions. All patients underwent a preoperative visual-evoked potential and contact B scan ultrasound examination of the globe. Before being considered for surgical intervention, each patient had to demonstrate a reproducible visual-evoked potential waveform. All patients underwent a closed vitrectomy, lensectomy, and membrane peeling procedure using a two-port dissection technique as previously described in other reportS?,3 Briefly, a lensectomy was performed using a 23gauge infusion needle and a vitreous cutter entering the eye through the iris root in the horizontal meridians. The vitreous cutter was then removed, and the fluid infusion was replaced with sodium hyaluronate infused through an infusion-light pipe. Residual lens capsule was removed with intraocular forceps, if possible. The retrolenticular membrane was incised with two 21-gauge needles in a reverse cutting fashion. Membrane-peeling-cutting scissors were used to radialize the retrolenticular membrane and then divide the anterior loop traction. Sodium hyaluronate was added using minimal pressure to gently open the funnel of retina, allowing safer and more complete dissection 1106
of epiretinal tissue. As the posterior pole was approached, the 20-gauge infusion-light pipe using sodium hyaluronate infusion and a contact lens were used to aid in visualization of the retina during dissection of the epiretinal tissue. When dissection was completed, the sodium hyaluronate was exchanged for air. In addition to the vitreous surgery, 2 patients also underwent a scleral buckling procedure at the initial vitrectomy for retinal tears found intraoperatively associated with partial retinal detachment. Also, one patient underwent a scleral buckling procedure and retinotomy for a rhegmatogenous retinal detachment associated with severe retinal traction from proliferative vitreoretinopathy and ROP (Table 1).
RESULTS Preoperatively, all eyes were diagnosed with advanced ROP in a stage 5 configuration as determined by the appearance of an opacity in the retrolenticular space. In all cases, contact B scan ultrasound of the globe gave the appearance of a total retinal detachment, which appeared to confirm the clinical stage of ROP. Before being considered for surgical management, all patients had to demonstrate a reproducible visual-evoked potential waveform preoperati vel y. After the completion of a lensectomy and removal of the retrolenticular opacities, it became apparent intraoperatively that islands of functional-appearing retina were attached to the retinal pigment epithelium at the base of and in between folds of retina posteriorly or in the far periphery making these eyes actually a stage 4B configuration (Fig 1). I The intraoperative findings accounted for the reproducible visual-evoked potential waveform found preoperatively. However, ultrasound of
BRADFORD AND TRESE
•
ROP IN THE OLDER PATIENT
Fig 1. Diagrammatic representations of retinal configurations found in eyes of older patients with advanced stages of ROP with dense retrolenticular opacities. Left. islands of attached retina in the periphery. Right. islands of attached retina posteriorly.
the globe was not as predictive of this stage 4B configuration. Postoperatively, seven (63.6%) eyes maintained the same visual acuity by objective testing as was found before surgery; however, these patients reported subjective improvement in their vision. One eye became phthisical and visual acuity of no light perception developed (case 6), and one eye developed visual acuity of no light perception despite peripheral retinal attachment (case 9). The other two eyes had no improvement in visual acuity subjectively, but maintained visual acuity of bare light perception. The development of glaucoma was the major complication after surgery. Four (36.4%) eyes developed glaucoma, and one of these eyes that also had glaucoma preoperatively became phthisical (case 6). Retinal attachment posterior to the equator was present at the last follow-up visit in seven (63.6%) eyes and peripherally in one eye. The retinas of two eyes could not be evaluated because of corneal opacification secondary to corneal blood staining and corneal edema (cases 7 and 11). Four eyes required additional surgical management after the initial vitrectomy, membrane peeling, and len sectomy. One patient (case 1) developed a recurrent tractional retinal detachment 5 months after surgery, secondary to the development of recurrent epiretinal membrane formation, and required an additional vitrectomy, membrane peeling, and air-fluid exchange to achieve retinal reattachment posterior to the equator. A vitreous hemorrhage developed in case 8 after a retinotomy was performed to relieve severe retinal traction at the time of initial vitreous surgery. The vitreous hemorrhage was cleared with an air-fluid exchange, and the retina was attached at the last follow-up visit. Two of the eyes in which a retinal tear was found intraoperatively required additional surgical procedures-two additional vitrectomies with air-fluid exchanges were performed in one eye (case 7) and a vitrectomy, membrane peeling, and silicone oil injection in another eye (case 11). Both eyes developed corneal opacification due to corneal blood staining and corneal edema with associated visual loss. These retinas could not be visualized.
DISCUSSION Although the majority of older patients with ROP are unlikely to be surgical candidates, there are some patients who can benefit from vitreoretinal surgery later in life. Standard scleral buckling procedures have been used successfully to manage rhegmatogenous retinal detachments associated with regressed ROP. 4 •5 Also, vitreous surgery, often in conjunction with scleral buckles, has been used to manage complicated, late-onset rhegmatogenous retinal detachments associated with regressed ROP. 5 These eyes may have posterior retinal breaks, significant vitreoretinal traction, or proliferative vitreoretinopathy, which necessitates the use of vitreous surgery to help reattach these retinas. Our current study describes the use of vitreous surgery to remove vitreous opacifications and allow attached areas of functional retina to be uncovered. All patients in this study had dense opaque retrolenticular membranes before surgery that obscured the view of the retina giving them the clinical appearance of stage 5 ROP.I All patients had visual acuity of at least light perception and a reproducible visual-evoked potential waveform preoperatively but demonstrated a gradual reduction in visual acuity. Ultrasound of the globe gave the appearance of a total retinal detachment. Intraoperatively, however, these eyes were found to have islands of functional-appearing retina that. were attached to the retinal pigment epithelium at the base of and in between folds of retina posteriorly or in the far periphery making these eyes actually a stage 4B configuration (Fig 1).1 Various protocols have been proposed for evaluating young eyes with advanced ROP preoperatively to help identify those eyes that might achieve a beneficial visual outcome from vitreous surgery. Some authors believe that contact B scan ultrasound of the globe is useful in determining the extent and configuration of retinal detachments. 6 - 8 In our study, the contact B scan ultrasound of the globe gave the appearance of a total retinal detachment, but intraoperatively these eyes were actually found to have areas of attached retina in the base of retinal folds. This disparity between preoperative ultrasound findings and the intraoperative retinal configurations may reflect 1107
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the lack of sensitivity of contact B scan ultrasound to accurately predict in all cases the complex anatomical configurations in advanced ROP. Another method, visualevoked potential, has been used to help identify those eyes with severe ROP that might demonstrate a positive visual response after vitreous surgery.8 In our study, all patients had a reproducible visual-evoked potential waveform before surgery. In our experience, the visual-evoked potential was more predictive of the actual retinal configuration (stage 4B) and potential retinal function than was the contact B scan ultrasound of the globe. In this study, 7 of the 11 eyes (63.6%) showed retinal reattachment posterior to the equator and 1 eye developed partial retinal reattachment in the periphery. Seven of the 11 eyes (63.6%) had visual acuity oflight perception and a subjective improvement in visual acuity after vitreous surgery. An additional two patients maintained bare light perception but had a subjective decrease in visual acuity. These anatomical and visual results compare favorably with the results after vitreous surgery in younger eyes with stage 5 ROP. Anatomical retinal reattachment in these younger eyes has been reported to occur in 33% to 48% of eyes, whereas, functional visual acuity has been attained in 11% to 31% ofeyes. 9- " The developmeIltofglaucoma was the major postoperative complication. Four of the II eyes developed a postoperative elevation of intraocular pressure that required treatment. Phthisis developed in one of these eyes, which was one of the four eyes that had decreased visual acuity. The goal of vitreous surgery in these older patients is to uncover areas of functional , attached retina. Reattachment of the retina oftentimes occurs after vitreous surgery, but many months may be required to reabsorb the subretinal fluid due to the high viscosity and accumulation of cholesterol crystals in the subretinal fluid. Certainly the areas of longstanding retinal detachment have developed photoreceptor degeneration and subretinal membranes limiting the visual potential of these eyes. 12-14 Reattaching retina without photoreceptors cannot in any way contribute to an increase in visual function. Therefore, it is important to attempt to preserve areas of attached retina that can have functioning photoreceptor-retinal pigment epithelial units and intact neuronal connections throughout the visual pathway. By performing a media clearing procedure, we may allow these patients to more effectively use the vision that they possess. A second potential impact of vitreous surgery in these older eyes with advanced ROP may be to release vitreous traction on areas of the retina. This may reduce the number of eyes that develop phthisis because of ciliary body detachment and/or combined rhegmatogenous and tractional retinal detachments, which are unlikely to be able to be repaired. If this is the case, which could be proven only by a randomized prospective clinical trial, then these patients may retain vision for longer periods of time than patients who do not undergo vitreous surgery. Certainly, it is difficult for a sighted population to appreciate the potential benefits of .visual acuities of only 1108
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light perception and projection. However, those patients who have had this low level of vision over a lifetime appear to be able to learn techniques to use this low level of vision to augment their lifestyles. These same patients appear severely disabled when this low level of vision is completely lost. It is clear that we need to strive to better understand the minimal visual acuities that are useful to patients and to learn ways of intervening at an earlier ti.me in the process of advanced ROP when larger areas of the neurosensory retina may be able to be salvaged, thereby improving the patient's visual function. We suggest that, in older patients, the term clinical stage 5 ROP be used to describe eyes that have opalescent retrolenticular tissue completely obscuring the view of the retina until retinal function can be established by clinical electrophysiologic examination.
REFERENCES 1. The International Cornrnittee for the Classification of Late Stages of Retinopathy of Prematurity. An international classification of retinopathy of prematurity. II. The classification of retinal detachment. Arch Ophthalmol 1987; 105:906-12. 2. Trese MT. Two-hand dissection technique during closed vitrectomy for retinopathy of prematurity [Letter]. Am J Ophthalmol 1986; 101: 251-2. 3. Trese MT. Surgical results of stage V retrolental fibroplasia and timing of surgical repair. Ophthalmology 1984; 91 :461-6. 4. Greven C, Tasman W. Scleral buckling in stages 4B and 5 retinopathy of prematurity. Ophthalmology 1990; 97:817-20. 5. Sneed SR. Pulido JS, Blodi CF, et al. Surgical managernent of lateonset retinal detachrnents associated with regressed retinopathy of prernaturity. Ophthalrnology 1990; 97:179-83. 6 Shapiro DR , Stone RD. Ultrasonic characteristics of retinopathy of prematurity presenting with leukokoria. Arch Ophthalmol1985; 103: 1690-2. 7. deJuan E Jr., Shields S, Machemer R. The role of ultrasound in the management of retinopathy of prematurity. Ophthalmology 1988; 95: 884-8. 8. Clarkson JG, Jacobson SG, Frazier-Byrne S, Flynn JT. Evaluation of eyes with stage-5 retinopathy of prematurity. Graefes Arch Clin Exp Ophthalmol1989; 227:332-4. 9. Machemer R. Closed vitrectomy for severe retrolental fibroplasia in the infant. Ophthalmology 1983; 90:436-41. 10. Trese MT. Visual results and prognostic factors for vision following surgery for stage V retinopathy of prematurity. Ophthalmology 1986; 93:574-79. 11. Zilis JD, deJuan E, Machemer R. Advanced retinopathy of prematurity. The anatomic and visual results of vitreous surgery. Ophthalmology 1990; 97:821-6. 12. Foos RY. Chronic retinopathy of prematurity. Ophthalmology 1985; 92:563-74. 13. deJuan E Jr., Machemer R, Flynn JT, Green WR. Surgical pathoanatomy in stage 5 retinopathy of prematurity. In: Flynn JT, Phelps DL, eds. Retinopathy of Prematurity: Problem and Challenge. New York: Alan R. Liss, Inc. , 1988; vol. 24, pp 281-286. (Birth defects original article series; v. 24, no. 1). 14. Foos RY . Pathologic features of retinopathy of prematurity. In: Flynn JT, Phelps DL, eds. Retinopathy of Prematurity: Problem and Challenge. New York: Alan R. Liss, 1988; 73-85. (Birth defects original article series; v. 24, no. 1).
Abstract: The authors studied 11 eyes of 10 patients who complained of a recent decrease in vision and whose eyes had the clinical appearance of stage 5 retinopathy of prematurity (RaP) as diagnosed by the presence of a dense, opaque retrolenticular membrane. Patients ranged in age from 4 to 33 years, and birth weights ranged from 680 to 1077 g. All eyes underwent vitrectomy, lensectomy, and membrane peeling and were found intraoperatively to have areas of retina that were attached (stage 48). Preoperatively, a reproducible visual-evoked potential waveform appeared to better predict this retinal configuration and potential retinal function than did the contact 8 scan ultrasound of the globe. Postoperatively, seven (63.6%) eyes demonstrated an improvement in vision. Vitreous surgery may be beneficial in selected eyes of older patients with advanced Rap to clear media opacities and to uncover areas of functional, attached retina, which may allow these patients to more effectively use the vision they possess. Ophthalmology 1991; 98: 11 05-1108
Since the conception of neonatal intensive care units, the survival rate of low birth weight infants has increased. This increased survival rate has resulted in an apparent increase in the incidence of retinopathy of prematurity (ROP). Many of these infants have had progression of their ocular disease to the more severe stages of ROP without receiving ocular therapy, such as cryotherapy, because organized strategies for examining these low birth weight infants and for treating advanced ROP have only been available in the past few years. A population of older patients exists who have had untreated advanced ROP but who have not lost all vision. Frequently, the ophthalmologist is asked to evaluate these older patients with advanced ROP who either complain of a reduction in vision or whose
Originally received: October 28, 1990. Revision accepted: March 1, 1991. From the Department of Ophthalmology, Beaumont Eye Institute, William Beaumont Hospital, Royal Oak, and the Department of Ophthalmology, Children 's Hospital, Detroit. Dr. Bradford is currently affiliated with the Retina Service. Department of Ophthalmology, University of Arkansas for Medical Sciences. Utile Rock . Presented in part at the American Academy of Ophthalmology Annual Meeting, Atlanta, Oct/Nov 1990. Reprint requests to Michael T. Trese, MD, Associated Retinal Consultants, 3535 W Thirteen Mile Rd, Suite 632, Royal Oak, MI 48073.
caretakers notice a reduction in the patient's visual function. Before this reduction in vision occurs, they may have had a level of vision that had helped them to ambulate. These patients generally present with a white, opaque retrolenticular membrane and are commonly described as having stage 5 ROP (total retinal detachment).l However, intraoperatively they are often found to have some area of functional-appearing retina that is attached. Therefore, despite their appearance as stage 5 ROP, they actually have stage 4B ROP (partial retinal detachment with macular involvement).l This study describes the preoperative evaluation and rationale for treatment of this group of older patients with advanced ROP.
PATIENTS AND METHODS The surgical records of one of the authors (MTT) from his practice at William Beaumont Hospital in Royal Oak, MI, and Children's Hospital in Detroit, MI, were retrospectively reviewed. Ten patients who were born prematurely with low birth weight and who were 4 years of age or older at the time of examination and surgery were identified. Between April 1988 and January 1989, 11 eyes (6 right eyes, 5 left eyes) underwent an initiallensectomy, vitrectomy, and membrane peeling procedure for advanced stages ofROP consisting of a white retrolenticular 1105
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VOLUME 98
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NUMBER 7
Table 1. Profiles of Older Patients Who Underwent Vitreous Surgery for Advanced Retinopathy of Prematurity Age (yrs)/Sex/Eye
Birthweight (g)
4/M/OD
879
2 3 4 5 6 7
7/M/OD 16/F/OD 12/F/OS 17/F/OS 33/F/OS 30/F/OD
680 992 992 964 1077 964
8 9 10 11
9/M/OS 7/M/OD 7/M/OS 9/F/OD
907 800 800 Unknown (Adopted)
Eye No.
Preoperative Procedures
Cryo PK
Cryo x2
Cryo
Operating Procedures
Retinal Attachment
Visual Improvement
Glaucoma
Follow-up (mos)
(1) L, V, MP (2) V, MP, AFE L, V, MP L, V, MP L, V, MP L, V, MP, SB L, V, MP (1) L, V, MP (2) V, MP, AFE (3) V, MP, AFE L, V, MP, SB, Ret L, V, MP L, V, MP (1) L, V, MP, SB (2) V, MP, SO
Attached
Improved
Not present
9
Attached Attached Attached Attached Phthisis Unknown
Improved Improved Improved Improved NLP Not improved
Present Not present Present Not present Present Not present
17 22 12 14 15 18
Attached PA Attached Unknown
Improved NLP Improved Not improved
Present Not present Not present Not present
13 6 6 7
00 = right eye; L = lensectomy; V = vitrectomy; MP = membrane peeling; AFE = air-fluid exchange; Cryo = peripheral retinal cryotherapy; PK = penetrating keratoplasty; OS = left eye; SB = scleral buckle; NLP = no light perception; Ret = retinotomy; PA = peripheral attachment; SO =
silicone oil injection.
membrane and the clinical appearance of stage 5 ROP.I Ages of the patients at the time of surgery ranged from 4 to 33 years. Birth weights ranged between 680 and 1077 g. Patients were followed for 6 to 22 months. All patients presented with the recent onset of decreased vision and had the clinical appearance of stage 5 ROP as diagnosed by an opacity behind the lens obscuring the view of the retina. I Three eyes had previously undergone peripheral retinal cryotherapy for proliferative ROP. One eye had undergone penetrating keratoplasty before vitreous surgery for corneal decompensation and opacification. None of the patients had undergone previous vitreous surgery or scleral buckling procedures. Nine patients had visual acuity oflight perception, one patient had visual acuity of light perception with projection vision, and one patient had visual acuity of hand motions. All patients underwent a preoperative visual-evoked potential and contact B scan ultrasound examination of the globe. Before being considered for surgical intervention, each patient had to demonstrate a reproducible visual-evoked potential waveform. All patients underwent a closed vitrectomy, lensectomy, and membrane peeling procedure using a two-port dissection technique as previously described in other reportS?,3 Briefly, a lensectomy was performed using a 23gauge infusion needle and a vitreous cutter entering the eye through the iris root in the horizontal meridians. The vitreous cutter was then removed, and the fluid infusion was replaced with sodium hyaluronate infused through an infusion-light pipe. Residual lens capsule was removed with intraocular forceps, if possible. The retrolenticular membrane was incised with two 21-gauge needles in a reverse cutting fashion. Membrane-peeling-cutting scissors were used to radialize the retrolenticular membrane and then divide the anterior loop traction. Sodium hyaluronate was added using minimal pressure to gently open the funnel of retina, allowing safer and more complete dissection 1106
of epiretinal tissue. As the posterior pole was approached, the 20-gauge infusion-light pipe using sodium hyaluronate infusion and a contact lens were used to aid in visualization of the retina during dissection of the epiretinal tissue. When dissection was completed, the sodium hyaluronate was exchanged for air. In addition to the vitreous surgery, 2 patients also underwent a scleral buckling procedure at the initial vitrectomy for retinal tears found intraoperatively associated with partial retinal detachment. Also, one patient underwent a scleral buckling procedure and retinotomy for a rhegmatogenous retinal detachment associated with severe retinal traction from proliferative vitreoretinopathy and ROP (Table 1).
RESULTS Preoperatively, all eyes were diagnosed with advanced ROP in a stage 5 configuration as determined by the appearance of an opacity in the retrolenticular space. In all cases, contact B scan ultrasound of the globe gave the appearance of a total retinal detachment, which appeared to confirm the clinical stage of ROP. Before being considered for surgical management, all patients had to demonstrate a reproducible visual-evoked potential waveform preoperati vel y. After the completion of a lensectomy and removal of the retrolenticular opacities, it became apparent intraoperatively that islands of functional-appearing retina were attached to the retinal pigment epithelium at the base of and in between folds of retina posteriorly or in the far periphery making these eyes actually a stage 4B configuration (Fig 1). I The intraoperative findings accounted for the reproducible visual-evoked potential waveform found preoperatively. However, ultrasound of
BRADFORD AND TRESE
•
ROP IN THE OLDER PATIENT
Fig 1. Diagrammatic representations of retinal configurations found in eyes of older patients with advanced stages of ROP with dense retrolenticular opacities. Left. islands of attached retina in the periphery. Right. islands of attached retina posteriorly.
the globe was not as predictive of this stage 4B configuration. Postoperatively, seven (63.6%) eyes maintained the same visual acuity by objective testing as was found before surgery; however, these patients reported subjective improvement in their vision. One eye became phthisical and visual acuity of no light perception developed (case 6), and one eye developed visual acuity of no light perception despite peripheral retinal attachment (case 9). The other two eyes had no improvement in visual acuity subjectively, but maintained visual acuity of bare light perception. The development of glaucoma was the major complication after surgery. Four (36.4%) eyes developed glaucoma, and one of these eyes that also had glaucoma preoperatively became phthisical (case 6). Retinal attachment posterior to the equator was present at the last follow-up visit in seven (63.6%) eyes and peripherally in one eye. The retinas of two eyes could not be evaluated because of corneal opacification secondary to corneal blood staining and corneal edema (cases 7 and 11). Four eyes required additional surgical management after the initial vitrectomy, membrane peeling, and len sectomy. One patient (case 1) developed a recurrent tractional retinal detachment 5 months after surgery, secondary to the development of recurrent epiretinal membrane formation, and required an additional vitrectomy, membrane peeling, and air-fluid exchange to achieve retinal reattachment posterior to the equator. A vitreous hemorrhage developed in case 8 after a retinotomy was performed to relieve severe retinal traction at the time of initial vitreous surgery. The vitreous hemorrhage was cleared with an air-fluid exchange, and the retina was attached at the last follow-up visit. Two of the eyes in which a retinal tear was found intraoperatively required additional surgical procedures-two additional vitrectomies with air-fluid exchanges were performed in one eye (case 7) and a vitrectomy, membrane peeling, and silicone oil injection in another eye (case 11). Both eyes developed corneal opacification due to corneal blood staining and corneal edema with associated visual loss. These retinas could not be visualized.
DISCUSSION Although the majority of older patients with ROP are unlikely to be surgical candidates, there are some patients who can benefit from vitreoretinal surgery later in life. Standard scleral buckling procedures have been used successfully to manage rhegmatogenous retinal detachments associated with regressed ROP. 4 •5 Also, vitreous surgery, often in conjunction with scleral buckles, has been used to manage complicated, late-onset rhegmatogenous retinal detachments associated with regressed ROP. 5 These eyes may have posterior retinal breaks, significant vitreoretinal traction, or proliferative vitreoretinopathy, which necessitates the use of vitreous surgery to help reattach these retinas. Our current study describes the use of vitreous surgery to remove vitreous opacifications and allow attached areas of functional retina to be uncovered. All patients in this study had dense opaque retrolenticular membranes before surgery that obscured the view of the retina giving them the clinical appearance of stage 5 ROP.I All patients had visual acuity of at least light perception and a reproducible visual-evoked potential waveform preoperatively but demonstrated a gradual reduction in visual acuity. Ultrasound of the globe gave the appearance of a total retinal detachment. Intraoperatively, however, these eyes were found to have islands of functional-appearing retina that. were attached to the retinal pigment epithelium at the base of and in between folds of retina posteriorly or in the far periphery making these eyes actually a stage 4B configuration (Fig 1).1 Various protocols have been proposed for evaluating young eyes with advanced ROP preoperatively to help identify those eyes that might achieve a beneficial visual outcome from vitreous surgery. Some authors believe that contact B scan ultrasound of the globe is useful in determining the extent and configuration of retinal detachments. 6 - 8 In our study, the contact B scan ultrasound of the globe gave the appearance of a total retinal detachment, but intraoperatively these eyes were actually found to have areas of attached retina in the base of retinal folds. This disparity between preoperative ultrasound findings and the intraoperative retinal configurations may reflect 1107
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the lack of sensitivity of contact B scan ultrasound to accurately predict in all cases the complex anatomical configurations in advanced ROP. Another method, visualevoked potential, has been used to help identify those eyes with severe ROP that might demonstrate a positive visual response after vitreous surgery.8 In our study, all patients had a reproducible visual-evoked potential waveform before surgery. In our experience, the visual-evoked potential was more predictive of the actual retinal configuration (stage 4B) and potential retinal function than was the contact B scan ultrasound of the globe. In this study, 7 of the 11 eyes (63.6%) showed retinal reattachment posterior to the equator and 1 eye developed partial retinal reattachment in the periphery. Seven of the 11 eyes (63.6%) had visual acuity oflight perception and a subjective improvement in visual acuity after vitreous surgery. An additional two patients maintained bare light perception but had a subjective decrease in visual acuity. These anatomical and visual results compare favorably with the results after vitreous surgery in younger eyes with stage 5 ROP. Anatomical retinal reattachment in these younger eyes has been reported to occur in 33% to 48% of eyes, whereas, functional visual acuity has been attained in 11% to 31% ofeyes. 9- " The developmeIltofglaucoma was the major postoperative complication. Four of the II eyes developed a postoperative elevation of intraocular pressure that required treatment. Phthisis developed in one of these eyes, which was one of the four eyes that had decreased visual acuity. The goal of vitreous surgery in these older patients is to uncover areas of functional , attached retina. Reattachment of the retina oftentimes occurs after vitreous surgery, but many months may be required to reabsorb the subretinal fluid due to the high viscosity and accumulation of cholesterol crystals in the subretinal fluid. Certainly the areas of longstanding retinal detachment have developed photoreceptor degeneration and subretinal membranes limiting the visual potential of these eyes. 12-14 Reattaching retina without photoreceptors cannot in any way contribute to an increase in visual function. Therefore, it is important to attempt to preserve areas of attached retina that can have functioning photoreceptor-retinal pigment epithelial units and intact neuronal connections throughout the visual pathway. By performing a media clearing procedure, we may allow these patients to more effectively use the vision that they possess. A second potential impact of vitreous surgery in these older eyes with advanced ROP may be to release vitreous traction on areas of the retina. This may reduce the number of eyes that develop phthisis because of ciliary body detachment and/or combined rhegmatogenous and tractional retinal detachments, which are unlikely to be able to be repaired. If this is the case, which could be proven only by a randomized prospective clinical trial, then these patients may retain vision for longer periods of time than patients who do not undergo vitreous surgery. Certainly, it is difficult for a sighted population to appreciate the potential benefits of .visual acuities of only 1108
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light perception and projection. However, those patients who have had this low level of vision over a lifetime appear to be able to learn techniques to use this low level of vision to augment their lifestyles. These same patients appear severely disabled when this low level of vision is completely lost. It is clear that we need to strive to better understand the minimal visual acuities that are useful to patients and to learn ways of intervening at an earlier ti.me in the process of advanced ROP when larger areas of the neurosensory retina may be able to be salvaged, thereby improving the patient's visual function. We suggest that, in older patients, the term clinical stage 5 ROP be used to describe eyes that have opalescent retrolenticular tissue completely obscuring the view of the retina until retinal function can be established by clinical electrophysiologic examination.
REFERENCES 1. The International Cornrnittee for the Classification of Late Stages of Retinopathy of Prematurity. An international classification of retinopathy of prematurity. II. The classification of retinal detachment. Arch Ophthalmol 1987; 105:906-12. 2. Trese MT. Two-hand dissection technique during closed vitrectomy for retinopathy of prematurity [Letter]. Am J Ophthalmol 1986; 101: 251-2. 3. Trese MT. Surgical results of stage V retrolental fibroplasia and timing of surgical repair. Ophthalmology 1984; 91 :461-6. 4. Greven C, Tasman W. Scleral buckling in stages 4B and 5 retinopathy of prematurity. Ophthalmology 1990; 97:817-20. 5. Sneed SR. Pulido JS, Blodi CF, et al. Surgical managernent of lateonset retinal detachrnents associated with regressed retinopathy of prernaturity. Ophthalrnology 1990; 97:179-83. 6 Shapiro DR , Stone RD. Ultrasonic characteristics of retinopathy of prematurity presenting with leukokoria. Arch Ophthalmol1985; 103: 1690-2. 7. deJuan E Jr., Shields S, Machemer R. The role of ultrasound in the management of retinopathy of prematurity. Ophthalmology 1988; 95: 884-8. 8. Clarkson JG, Jacobson SG, Frazier-Byrne S, Flynn JT. Evaluation of eyes with stage-5 retinopathy of prematurity. Graefes Arch Clin Exp Ophthalmol1989; 227:332-4. 9. Machemer R. Closed vitrectomy for severe retrolental fibroplasia in the infant. Ophthalmology 1983; 90:436-41. 10. Trese MT. Visual results and prognostic factors for vision following surgery for stage V retinopathy of prematurity. Ophthalmology 1986; 93:574-79. 11. Zilis JD, deJuan E, Machemer R. Advanced retinopathy of prematurity. The anatomic and visual results of vitreous surgery. Ophthalmology 1990; 97:821-6. 12. Foos RY. Chronic retinopathy of prematurity. Ophthalmology 1985; 92:563-74. 13. deJuan E Jr., Machemer R, Flynn JT, Green WR. Surgical pathoanatomy in stage 5 retinopathy of prematurity. In: Flynn JT, Phelps DL, eds. Retinopathy of Prematurity: Problem and Challenge. New York: Alan R. Liss, Inc. , 1988; vol. 24, pp 281-286. (Birth defects original article series; v. 24, no. 1). 14. Foos RY . Pathologic features of retinopathy of prematurity. In: Flynn JT, Phelps DL, eds. Retinopathy of Prematurity: Problem and Challenge. New York: Alan R. Liss, 1988; 73-85. (Birth defects original article series; v. 24, no. 1).