Management of Late-onset Angle-closure Glaucoma Associated with Retinopathy of Prematurity ANDREW J. MICHAEL, MD, SAMUEL R. PESIN, MD, L. JAY KATZ, MD, WILLIAM S. TASMAN, MD
Abstract: Late-onset angle-closure glaucoma secondary to retinopathy of prematurity (ROP) occurred in ten eyes of ten patients. The age at presentation ranged from 12 to 45 years (mean, 32 years). Eight eyes had nonneovascular mechanisms for the angle closure while two had neovascular angle closure. Treatment of eyes with this form of secondary angle closure included medical management alone, peripheral iridectomy, trabeculectomy, lensectomy, alloplastic tube shunt implantation, and cilioablative procedures. Three eyes required more than one of these treatments. The choice of therapy was based on the initial intraocular pressure and vision, degree of lens opacity and intumescence, presence of anterior segment neovascularization, and the gonioscopic appearance of the anterior chamber angle. The clinical features of this condition are described and a proposed mechanism and therapeutic approach are presented. Ophthalmology 1991; 98:1093-1098
Secondary angle-closure glaucoma is a well recognized complication of retinopathy of prematurity (ROP). Most commonly, this glaucoma occurs in infancy or childhood. \-3 Blodi4 estimated this complication to occur in as many as 30% of eyes in infants with more severe forms of ROP. Various treatments for angle-closure glaucoma occurring in children with ROP have been proposed, including peripheral iridectomy and sector iridectomy, 2 lensectom y alone or with vitrectomy,3.5 and medical management with miotics4 or corticosteroids. 6
Originally received: October 28, 1990. Revision accepted: February 18, 1991. From the Glaucoma and Retina Departments, Wills Eye Hospital, Jefferson Medical College, Thomas Jefferson University, Philadelphia. Dr. Pesin is currently affiliated with Washington University, St. Louis. Presented at the American Academy of Ophthalmology Annual Meeting, Atlanta, Oct/Nov 1990. Reprint requests to L. Jay Katz, MD, Wills Eye Hospital, Ninth and Walnut Sts, Philadelphia, PA, 19107.
However, to our knowledge, only five cases of angleclosure glaucoma complicating ROP presenting in adults have previously been reported. 7- 9 This report describes ten additional cases of this entity, reviews the literature regarding angle-closure glaucoma complicating ROP, discusses the possible mechanisms involved, and suggests a rational approach to the management oflate-onset angleclosure glaucoma in ROP.
MATERIALS AND METHODS This retrospective study is based on the case histories often patients seen at Wills Eye Hospital with late-onset angle-closure glaucoma and ROP. The files were obtained from the Glaucoma and Retina Registry at Wills Eye Hospital. Patients in this series presented between 1984 and 1990 and were examined and treated by one or more of the authors. Initial evaluation of each patient included Snellen visual acuity, manifest refraction, intraocular tension measurement by applanation (pneumotonometry was used in one case in which there was marked corneal
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irregularity), slit-lamp biomicroscopy, indentation gonioscopy, and fundus examination. Records of prior ophthalmic care and most recent spectacle correction were obtained. The choice of therapy was based on the initial examination and response to subsequent therapy.
CASE REPORTS Case 1. A 12-year-old girl with a history of prematurity (26 weeks gestation, birth weight 850 g) and poor vision was seen in consultation for her glaucoma. Best corrected visual acuity was light perception in the right eye and 20/400 in the left. Spectacle correction was -3.00 + 1.50 X 23 in the right eye and -4.00 in the left. Results of slit-lamp examination showed a dense cataract in the right eye and posterior subcapsular opacity in the left. A retrolental glial mass was noted in each eye. Results of gonioscopy showed an open angle with the ciliary body band visible and a flat iris configuration in the right eye, whereas the left angle was very narrow with Schwalbe's line visible and nearly 360 0 of peripheral anterior synechiae without neovascularization. Intraocular pressures (lOPs) were 20 mmHg in the right eye and 42 mmHg in the left. The patient was treated with oral acetazolamide 125 mg twice daily and levobunolol 0.5% twice daily in the left eye. The lOP of the left eye has been maintained at 17 mmHg and the patient's visual acuity has remained unchanged for I year. Case 2. A 45-year-old man with a history of prematurity (birth weight 1350 g) and perinatal oxygen administration was referred for management of his glaucoma. His right eye had been enucleated at age 2 for uncontrolled glaucoma and pain. He had a 2-year history of lOPs in his left eye ranging from 17 to 28 mmHg on dipivefrin 0.1 % twice daily and timolol 0.5% twice daily. Best corrected visual acuity was 20/40 with a spectacle correction of -18.50 + 3.50 X 96. Results of slit-lamp examination showed a shallow anterior chamber and mild lenticular nuclear sclerosis. Intraocular pressure by applanation was 17 mmHg. Results of gonioscopy showed a very narrow occludable angle with only the most anterior trabecular meshwork visible. Indentation gonioscopy demonstrated 60 0 of peripheral anterior synechiae and brought the scleral spur into view elsewhere. Results of fundus examination showed findings of regressed ROP and peripheral retinoschisis. A neodymium:YAG laser peripheral iridectomy was performed, after which lOPs ranged from 14 to 20 mmHg on timolol 0.5% twice daily in the left eye. Results of subsequent gonioscopic examination showed a deeper angle configuration, with scleral spur visible without indentation and no worsening of the peripheral anterior synechiae. Visual acuity, automated visual fields, optic disc and fundus appearance have remained stable 14 months after laser treatment. Case 3. A 35-year-old man with a history of prematurity, perinatal oxygen therapy, and poor vision in his left eye since birth presented to the emergency department complaining of pain in his left eye of 3 weeks' duration. His spectacle correction was -4.25 + 0.25 X 125 in the right eye and -3.50 sphere in the left. Visual acuity was 20/25- in the right eye and counting fingers in the left. Examination of the left eye showed ciliary injection, mid-dilated and fixed pupil, and a dense brunescent cataract. Intraocular pressures were 15 mmHg in the right eye and 37 mmHg in the left. Results of gonioscopic examination showed the right angle to be open to the ciliary body band and the left closed for nearly 360 0 • There was no view of the fundus of the left eye.
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Administration of a single dose oftimolol 0.5% in the left eye and oral acetazolamide 500 mg lowered the lOP to 17 mmHg in the left eye. The patient underwent neodymium:YAG peripheral iridectomy and the angle deepened appreciably. One week later his lOP was lowered to 15 mmHg in the left eye on timolol 0.5% twice daily. He has since failed to return for further follow-up evaluations. Case 4. A 39-year-old woman who received perinatal oxygen therapy was evaluated for elevated lOP in the left eye. Visual acuity was 20/50 in the right eye with spectacle correction of -2.50 + 3.25 X 5 and counting fingers in the left with plano correction. Results of examination showed bilateral band keratopathy. The anterior chamber of the left eye was shallow. Gonioscopic examination showed that the right angle was open to the ciliary body band with no synechiae. The left angle was quite narrow, with an apparent iris insertion just behind Schwalbe's line. Indentation gonioscopy demonstrated 180 0 of the angle to be closed with peripheral anterior synechiae and the remainder open to the ciliary body band. Temporal dragging of the retina was present in both eyes. Intraocular tensions were 13 mmHg in the right eye and 28 mmHg in the left. Ultrasonic biometry measured the axial lengths to be 22.8 mm in the right eye and 22.5 mm in the left. Anterior chamber depths were 2.9 mm in the right eye and 2.6 mm in the left. Levobunolol 0.5% twice daily lowered the lOP of the left eye to 17 mmHg. However, because the anterior chamber angle remained occludable, a neodymium:YAG peripheral iridectomy was performed. Nine months after treatment, pressure in the left eye has ranged between I 7 and I 9 mmHg on levobunolol 0.5% once daily in the left eye. The anterior chamber is deeper, with the apparent iris insertion at the scleral spur and no change in the synechiae. Case 5. A 37-year-old woman with a history of ROP was referred for management of her glaucoma. She had undergone pars plana vitrectomy for retinal detachment in the right eye at age 31. Best corrected visual acuity was counting fingers in each eye. She had a spectacle correction of -12.75 + 2.75 X 80 in the right eye and -12.25 + 3.25 X 105 in the left. Results of slit-lamp examination showed a shallow anterior chamber in both eyes. Intraocular pressures were 32 mmHg in the right eye and 18 mmHg in the left. Both angles were narrow, with steeply convex peripheral irides and apparent insertions at the scleral spur. The iridocorneal angle was 15 0 in the right eye with 90 0 of peripheral anterior synechiae. The angle was a slightly deeper 20 0 with no synechiae in the left. Ultrasonic measurements of axial length yielded 24.8 mm in the right eye and 24.1 mm in the left. A neodymium:YAG peripheral iridectomy deepened the angle of the right eye but failed to lower lOP. Three months later she underwent trabeculectomy in the right eye with postoperative administration of 5-fluorouracil. Six months after surgery, lOPs in the right eye ranged from 16 to 19 mmHg on timolol 0.5% once daily. Case 6. A 15-year-old boy with a history ofROP was referred for control of his glaucoma. Visual acuity was hand motions in the right eye and no light perception in the left. Results of slitlamp examination showed extensive anterior segment neovascularization of the right eye. Intraocular tensions were 38 mmHg in the right eye and 32 mmHg in the left. A trabeculectomy was performed on the right eye, which failed to lower lOP. Thus, the right eye underwent cyclocryotherapy, which lowered the lOP to 19 mmHg. After six months, the pressure in the right eye remained 19 mmHg and the visual acuity was light perception. Case 7. A 39-year-old man with ROPpresented with severe pain in his right eye of3 days' duration. Visual acuity was count-
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Table 1. Presenting Features of Ten Eyes with Late-onset Secondary Angle-closure Glaucoma Associated with Retinopathy of Prematurity Case No.
Age (yrs)
Eye
1 2 3 4 5 6 7 8 9 10
12 45 35 39 37 15 39 27 27 40
OS OS OS OS 00 00 00 00 OS OS
Visual Acuity
Refraction (diopters)
lOP (mmHg)
Angle Configuration
PAS
20/400 20/40
-4.00 -16.75 -3.50
42 17 37 28 32 38 48 35 34 59
Narrow Occludable Closed Narrow Open, steep iris Closed neovascularization Shallow chamber Flat chamber Closed Closed neovascularization
-360 0 60 0 -360 0 180 0 90 0 360 0
CF CF CF HM CF HM NLP CF
Plano
-10.75 NA NA
-5.00 -4.00 +5.50
NA NA
-360 0 360 0
Prior Treatment None Medical None Medical Medical None None Medical, PI Medical Medical
lOP = intraocular pressure; PAS = peripheral anterior synechiae; OS = left eye; CF = counting fingers; 00 = right eye; HM = hand motions; NA = not available; PI = surgical peripheral iridectomy; NLP = no light perception.
ing fingers in his right eye and 20/40+ in the left. Results of slitlamp examination of the right eye showed a shallow anterior chamber and dense intumescent cataract. Intraocular pressures were 48 mmHg in the right eye and 16 mmHg in the left. Limballensectomy was performed in the right eye, with subsequent deepening of the anterior chamber. Postoperative lOPs have ranged from 14 to 18 mmHg for 6 years, and visual acuity in the right eye has remained hand motions for 4 years. Case 8. A 27-year-old woman with a history of ROP was referred for management of her glaucoma. Her right eye had undergone surgical iridectomy for glaucoma at age 2. Her glaucomatous blind left eye was enucleated for pain control at age 17. Visual acuity in her right eye was hand motions, and she was wearing a spectacle correction of -5.00 sphere. Results of slit-lamp examination showed marked corneal irregularitywith band keratopathy. The anterior chamber was flat and there was a superior iridectomy, which did not appear patent. A dense cataract precluded fundus evaluation. The lOP was 35 mmHg by pneumotonometry. Multiple medications, including topical iJ-blockers, dipivefrin, apraclonidine, and oral carbonic anhydrase inhibitors failed to lower the lOP below 24 mmHg. The patient refused to undergo intraocular surgery but agreed to neodymium:YAG cyclophotocoagulation with three treatments over 8 months. Her lOP remains 8 mmHg without medication 14 months after her last laser procedure, and her visual acuity remains hand motions. Case 9. A 27-y~r-old woman with a birth weight of 1250 g and ROP was referred for her painful glaucomatous left eye. She was wearing a refractive correction of -4.00 sphere in both eyes. Visual acuity was 20/200 in the right eye and no light perception in the left. Results of slit-lamp examination showed microcornea in both eyes. There was microcystic corneal edema and an extremely shallow anterior chamber in the left eye. Results of gonioscopy showed the right eye to have an open angle to the ciliary body band, while the left had a closed angle for nearly 360 0 • Intraocular pressures while taking timolol 0.5% twice daily, dipivefrin 0.1 % twice daily, prednisolone acetate 1% every 3 hours, and oral acetazolamide 500 mg twice daily were 8 mmHg in the right eye and 34 mmHg in the left. Results of fundus evaluation of the right eye showed temporal dragging of posterior pole structures. No view of the left fundus was possible. A Bscan ultrasound demonstrated an inferior retinal detachment that had been present on prior ultrasound examinations and no intraocular mass. Administration of atropine I %three times daily, prednisolone acetate I %hourly, and timoiol 0.5% twice daily to the left eye
failed to control either the pain or the lOP. Her left eye underwent neodymium:YAG cyclophotocoagulation, neodymium: YAG peripheral iridectomy, and retrobulbar injection of ethanol. After the peripheral iridectomy, the anterior chamber was noted to deepen dramatically. One month later, the lOP was 22 mmHg in the left eye and the eye remains comfortable. Case 10. A 40-year-old woman with a history of ROP presented with a I-week history of increasing pain and worsening vision in the left eye. Best-corrected visual acuities were hand motions in the right eye and counting fingers in the left. Her spectacle correction was +4.00 + 3 2. 5 X 86 in the right eye and +3.75 + 3.50 X 89 in the left. Results of slit-lamp examination showed microcornea in both eyes. The left eye had microcystic epithelial and stromal corneal edema, the anterior chamber was shallow, and iris neovascularization was present. Intraocular pressures were 19 mmHg in the right eye and 59 mmHg in the left. Results of gonioscopic examination showed the right eye to have an angle open to the ciliary body band, while the left angle was closed for 360 0 • Results of fundus examination showed temporally dragged optic discs, maculae, and retinal vessels in both eyes. The patient underwent implantation of an alloplastic tube shunt and thermal sclerostomy in the left eye. Two months after surgery, visual acuity had fallen to hand motions while lOPs ranged from 6 to 15 mmHg after administration of atropine 1 % and prednisolone acetate 1 %.
RESULTS The ten cases described in the present series are summarized in Tables 1 and 2. All ten eyes of ten patients had secondary angle-closure glaucoma associated with regressed ROP. All patients were born before the reporting of the international classification of ROP,1O and, as a result, information regarding staging of ROP in these patients was not available. Patients ranged in age from 12 to 45 years at the time of presentation (mean age, 32 years). Eight eyes had nonneovascular mechanisms for their angle closure while two had neovascular angle closure. Of the eight eyes with nonneovascular angle closure, six presented with chronic angle closure while two presented with a component of acute angle closure. One of these eyes had been painful for 3 weeks (case 3) and the 1095
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Table 2. Management and Posttreatment Features of Ten Eyes with Late-onset Secondary Angle-closure Glaucoma Associated with Retinopathy of Prematurity Gase No.
Treatment
lOP (mmHg)
1 2 3 4 5 6 7 8 9 10
Medical YAG PI YAG PI YAG PI YAG PI, trabeculectomy Trabeculectomy, GGT Lensectomy GPG GPG, YAG PI, ethanol Tube shunt
17 20 15 18 17 19 16 8 22 6
Visual Acuity (if changed)
GF- HM HM- LP GF- HM GF- HM
Angle (if changed) Deeper Deeper Deeper Deeper Deeper
Follow-up (mos) 12 14 1 wk 9 6 6 72 14
1
2
lOP = intraocular pressure at last follow-up; GGT = cyclocryotherapy; GPG = neodymium:YAG cyclophotocoagulation; YAG PI = neodymium:YAG peripheral iridectomy; GF = counting fingers; HM = hand motions; LP = light perception; ethanol = retrobulbar injection of ethanol.
other for 3 days (case 7). One of the two eyes with neovascular angle closure (case lO) also presented in an acute manner, with I week of increasing pain. Only one eye of each patient presented with angle-closure glaucoma requiring treatment. The right eye was involved in six patients and the left in four. The mean lOP of these eyes at presentation was 37 mmHg (range, 17 to 59 mmHg). Only two eyes (cases 2 and 4) had initial lOPs in the normal range. The most recent refraction, in dioptric spherical equivalent, demonstrated a myopic correction in six eyes, plano correction in one eye, and a hyperopic correction in one eye. Recent refraction was unavailable for two eyes. The visual acuities of affected eyes at the time of presentation ranged from 20/40 to no light perception. Nine of the ten eyes had initial visual acuity of 20/400 or worse. Results of pretreatment gonioscopy showed that two of the eyes that had angles that were completely closed had anterior segment neovascularization. Six eyes had narrow or very narrow angle configurations and peripheral anterior synechiae ranging from 60 0 to nearly 360 0 • Three of these eyes (cases 2, 4, and 5) had less than or equal to 180 0 of synechial angle closure and three eyes (cases I, 3, and 9) had nearly 360 0 of synechiae. Two eyes (cases 7 and 8) had poorly visualized angles. On presentation, six eyes had prior medical management for glaucoma whereas four had undergone no prior treatment. One of the eyes (case 8) with glaucoma that had been managed medically had undergone a surgical peripheral iridectomy when the patient was 2 years old. At the time of presentation, the iridectomy in this eye was not patent. Treatment of eyes with this form of secondary angle closure included medical management alone (I eye), neodymium:YAG peripheral iridectomy (5 eyes), trabeculectomy (2 eyes), limballensectomy (I eye), alloplastic tube shunt implantation (I eye), and cilioablative procedures (3 eyes). Three eyes required more than one of these treatments. Follow-up ranged from I.week to 6 years (mean, 1.1 years). lO96
In all eyes in this series, initial attempts were made to control the glaucoma with medication. Only one eye (case I) was successfully managed with medications alone, and the lOP has been well-controlled for 1 year. This is also the only eye in the present series in which a retrolental mass was demonstrated. Five eyes were treated with neodymium:YAG peripheral iridectomy. Four of these five eyes presented with no anterior segment neovascularization, less than 270 0 of synechial angle closure, and the potential for useful vision (cases 2,3,4, and 5). The fifth eye (case 9) presented with nearly 360 0 of synechial angle closure and no useful vision. In this eye, a component of pupillary block was suspected and a neodymium:YAG peripheral iridectomy was performed in conjunction with cyclophotocoagulation. Three of the four eyes treated with neodymium:Y AG peripheral iridectomies alone have not required further surgical intervention (cases 2, 3, and 4). The mean followup of these four eyes is 7 months (range, I week to 14 months). One eye that underwent neodymium:YAG peripheral iridectomy (case 5) required subsequent trabeculectomy. A limballensectomy was performed in one eye (case 7). This patient presented with symptoms of acute angle closure and a dense intumescent cataract. Two patients presented with neovascular angle-closure glaucoma. In one monocular patient with visual acuity of hand motions, a trabeculectomy was attempted, but failed (case 6). This eye subsequently required cyclocryotherapy, and the visual acuity changed to light perception with good control of lOP. The second patient (case lO) presented with extensive anterior segment neovascularization and an lOP of 59 mmHg in her better seeing eye. An alloplastic tube shunt was implanted in this eye, but despite good lOP control, visual acuity is only hand motions. Two additional eyes with nonneovascular angle closure were successfully treated with cilioablative procedures. One of these was in a monocular patient with visual acuity of hand motions (case 8). The other eye was painful and
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without light perception (case 9). This latter case was additionally managed with retrobulbar injection of ethanol for pain control. The postoperative visual acuity of six eyes did not change from the pretreatment level. Four eyes had a decline in visual acuity. In three eyes (cases 5, 7, and 10) the decline was from counting fingers to hand motions and in one (case 6) from hand motions to light perception. Posttreatment lOPs ranged from 6 to 22 mmHg (mean, 16 mmHg). In five of the ten eyes, a posttreatment change in the anterior chamber angle configuration was noted. The angle was observed to deepen in these five cases.
DISCUSSION Angle-closure glaucoma complicating ROP most commonly occurs in infancy and early childhood. McCormick and Pratt-Johnson 5 examined pathologic specimens from four cases of infants with cicatricial ROP and angle-closure glaucoma and found the anterior chamber angle to be obliterated. Pollard II published his series of 15 infants and children with advanced cicatricial ROP in which he performed lensectomy for secondary angle-closure glaucoma. Included were two patients previously treated with peripheral iridectomy in which the glaucoma progressed, requiring lensectomy. McCormick and Pratt-Johnson5 described a case in which they performed bilateral needle aspiration of the lenses, which deepened the anterior chamber and lowered lOP. Johnson and Swan l2 performed 42 peripheral iridectomies in 26 infants and children with ROP for acute or chronic glaucoma. Residual vision was preserved, at least temporarily, in 16 eyes, However, Walton 2 noted that iridectomy was not uniformly successful in averting chronic angle closure and thus suggested that pupillary block was not the primary mechanism for angle closure in these young patients. In infants and children with stage 5 ROP, a variety of mechanisms for the development of glaucoma have been hypothesized. I - 4,7 However, the mechanism considered most likely to cause angle-closure glaucoma in these infants and children is the anterior displacement of the lensiris diaphragm secondary to contraction of a retrolental fibroglial mass. I ,4 The youngest patient in the preseni study (case 1) is 12 years of age. She is also the only patient in this series in whom a retrolental mass was demonstrated by either clinical examination or ultrasonography. Thus, she may represent a relatively late presentation of the more common angle-closure glaucoma in children with ROP, rather than an example of the late-onset angle-closure glaucoma we distinguish in the present series. Nonetheless, her case is included to document the range of clinical presentations in the setting of angle closure later in the course of ROP. Her glaucoma has been well-controlled on oral acetazolamide and topicallevobunolol for 1 year. She may require additional intervention in the future, and a filtering operation would be recommended in this situation since
there is extensive synechial angle closure and useful vision to preserve. In adult eyes with ROP and no retrolental mass, another mechanism for the development of glaucoma may be operative. Proposed mechanisms include both ciliary block and pupillary block. 7-9 The average age of the eight patients in the present series with nonneovascular angle closure mechanisms is 33 years. In contrast, the average age of presentation for primary angle closure is the seventh to eighth decade of life. Primary angle-closure glaucoma most often occurs in a small hyperopic eye with a shallow anterior chamber and a large lens. Most eyes in the present series are myopic. In the current study, the two eyes with axial length measurements of 22.5 and 24.8 mm had spherical equivalent refractions of plano and -10.75 diopters, respectively. Thus, axial lengths of these two eyes are not as long as might be expected for their degree of myopia. Hibino et al 13 reported that the ratio of the thickness of the lens to the axial length of the eye was increased in cases of ROP. However, the etiology of the large lens in ROP remains unknown. It is possible that the hypoxia of peripheral retina may inhibit normal ciliary body and zonular development, and a lens develops that is large in relation to other anterior segment structures. The presence of an enlarged lens in patients with a history of ROP can account for both the myopia and the proclivity for angle closure. Thus, adults with regressed ROP and an already enlarged lens are at risk for developing angle closure at an early age as their lens begins to enlarge with cortical changes and slight intumescence. Smith and Shivitz8 reported three cases of angle-closure glaucoma in adults with regressed ROP. Peripheral iridectomy prevented further attacks of angle closure in these eyes. They postulated that a pupillary block mechanism was the cause of the angle closure and suggested that peripheral iridectomy was the procedure of choice in these cases. Ueda and Ogin09 reported a similar case that was also successfully treated with iridectomy. Ultrasonographic biometry in their case demonstrated a long axial length, shallow anterior chamber, and large lens. Their measurements are in concert with prior reports l3 and our clinical observations. Kushner7 reported three cases of glaucoma (two children and one 25-year-old adult) complicating ROP. The mechanism for the glaucoma in these eyes was believed to be ciliary block. In our series, no patients were assessed as having a similar mechanism for angle closure. Based on our experience with the present series of ten patients with late-onset angle-closure glaucoma associated with ROP, pupillary block appears to be the operative mechanism of angle closure in nonneovascular cases and in those without a retrolental mass. Eyes without neovascularization and less than 270° of synechial angle closure may be amenable to glaucoma control with peripheral iridectomy. Three of the four such eyes in the present series have had their glaucoma controlled by neodymium:YAG peripheral iridectomy alone. We have used filtering surgery for treatment of eyes with useful vision and no anterior segment neovascularization 1097
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Observe
Peripheral N Iridectomy
...
Observe
Observe
Trabeculectomy
Enucleation
Fig 1. Proposed management scheme for late-onset chronic angle-closure glaucoma complicating retinopathy of prematurity. PAS = peripheral anterior synechiae; rop = intraocular pressure.
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(case 9). In painful eyes with no light perception, retrobulbar injection of ethanol may be an effective adjunctive therapy for pain control. Thus, late-onset angle-closure glaucoma associated with ROP may present via two operative mechanisms: (1) pupillary block that if not treated early may progress to total synechial angle closure; or (2) neovascularization-induced secondary angle closure. Based on our clinical experience, a scheme for the management of late-onset angle-closure glaucoma complicating ROP is summarized in Figure 1 (eyes with chronic angle closure) and Figure 2 (eyes with acute angle closure). At each step of the outlined management scheme, attempts should be made to control the glaucoma with medications before proceeding to the next therapeutic intervention. It has been predicted 1 that late-onset angle-closure glaucoma complicating ROP will become a more common entity in the future. The increased incidence ofROP in patients born in the 1960s and 1970s may directly result in a rise in the incidence oflate-onset angle closure. Thus, a heightened awareness and better understanding of this condition will aid in its prompt recognition and appropriate management.
REFERENCES Peripheral Iridectomy I
Cataract Extraction
Trabeculectomy
Observe
Ciliodestruction (repeat if required) (+/- retrobulbar alcohol)
Fig 2. Proposed management scheme for late-onset acute angle-closure glaucoma complicating retinopathy of prematurity. rop = intraocular pressure.
that have failed laser iridectomy. Lensectomy has been reserved for eyes with a dense intumescent cataract, less than 270° of synechial angle closure, and no neovascularization. Tube shunt procedures should be considered in the treatment of neovascular angle-closure glaucoma in eyes with useful visual potential. The management of neovascular angle-closure glaucoma in these eyes remains challenging and the current prognosis for these eyes is quite guarded. For patients who are not surgical candidates and for those with blind and painful eyes, we recommend the use of ciliodestructive procedures. If pupillary block is suspected as being a component of the angle-closure mechanism, a peripheral iridectomy may also be performed
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1. Hittner HM, Rhodes LM, McPherson AR. Anterior segment abnormalities in cicatricial retinopathy of prematurity. Ophthalmology 1979; 86:803-16. 2. Walton OS. Retrolental fibroplasia with glaucoma. In: Chandler PA, Grant WM, eds. Glaucoma, 2nd ed. Philadelphia: Lea & Febiger, 1979; 361-2. 3. Pollard ZF. Secondary angle-closure glaucoma in cicatricial retrolental fibroplasia. Am J Ophthalmol1980; 89:651-3. 4. Blodi FC. Symposium: retrolental fibroplasia (retinopathy of prema· turity). Management. Trans Am Acad Ophthalmol Otolaryngol1955; 59:35-8. 5. McCormick AQ, Pratt·Johnson JA. Angle closure glaucoma in infancy. Ophthalmic Surg 1971; 2:91-3. 6. Kushner BJ, Sondheimer S. Medical treatment of glaucoma associated with cicatricial retinopathy of prematurity. Am J Ophthalmol1982; 94: 313-7. 7. Kushner BJ. Ciliary block glaucoma in retinopathy of prematurity. Arch Ophthalmol1982; 100:1078-9. 8. Smith J, Shivitz I. Angle-closure glaucoma in adults with cicatricial retinopathy of prematurity. Arch Ophthalmol 1984; 102:371-2. 9. Ueda N, Ogino N. Angle·closure glaucoma with pupillary block mech· anism in cicatricial retinopathy of prematurity. Ophthalmologica 1988; 196:15-8. 10. Committee for the Classification of Retinopathy of Prematurity. An international classification of retinopathy of prematurity. Arch Ophthal· mol 1984; 102:1130-4. 11. Pollard ZF. Lensectorny for secondary angle·closure glaucoma in advanced cicatricial retrolental fibroplasia. Ophthalmology 1984; 91: 395-8. 12. Johnson DR, Swan KC. Retrolental fibroplasia-a continuing problem. Trans Pacific Coast Otolaryngol Ophthalrnol Soc 1966; 47:129-33. 13. Hibino Y, Takahashi M, Majirna A. Studies on ocular functions of cicatricial retinopathy of prematurity: rneasurements of refractive elernents. Jap J Clin Ophthalmol 1978; 32:655-62.
Abstract: Late-onset angle-closure glaucoma secondary to retinopathy of prematurity (ROP) occurred in ten eyes of ten patients. The age at presentation ranged from 12 to 45 years (mean, 32 years). Eight eyes had nonneovascular mechanisms for the angle closure while two had neovascular angle closure. Treatment of eyes with this form of secondary angle closure included medical management alone, peripheral iridectomy, trabeculectomy, lensectomy, alloplastic tube shunt implantation, and cilioablative procedures. Three eyes required more than one of these treatments. The choice of therapy was based on the initial intraocular pressure and vision, degree of lens opacity and intumescence, presence of anterior segment neovascularization, and the gonioscopic appearance of the anterior chamber angle. The clinical features of this condition are described and a proposed mechanism and therapeutic approach are presented. Ophthalmology 1991; 98:1093-1098
Secondary angle-closure glaucoma is a well recognized complication of retinopathy of prematurity (ROP). Most commonly, this glaucoma occurs in infancy or childhood. \-3 Blodi4 estimated this complication to occur in as many as 30% of eyes in infants with more severe forms of ROP. Various treatments for angle-closure glaucoma occurring in children with ROP have been proposed, including peripheral iridectomy and sector iridectomy, 2 lensectom y alone or with vitrectomy,3.5 and medical management with miotics4 or corticosteroids. 6
Originally received: October 28, 1990. Revision accepted: February 18, 1991. From the Glaucoma and Retina Departments, Wills Eye Hospital, Jefferson Medical College, Thomas Jefferson University, Philadelphia. Dr. Pesin is currently affiliated with Washington University, St. Louis. Presented at the American Academy of Ophthalmology Annual Meeting, Atlanta, Oct/Nov 1990. Reprint requests to L. Jay Katz, MD, Wills Eye Hospital, Ninth and Walnut Sts, Philadelphia, PA, 19107.
However, to our knowledge, only five cases of angleclosure glaucoma complicating ROP presenting in adults have previously been reported. 7- 9 This report describes ten additional cases of this entity, reviews the literature regarding angle-closure glaucoma complicating ROP, discusses the possible mechanisms involved, and suggests a rational approach to the management oflate-onset angleclosure glaucoma in ROP.
MATERIALS AND METHODS This retrospective study is based on the case histories often patients seen at Wills Eye Hospital with late-onset angle-closure glaucoma and ROP. The files were obtained from the Glaucoma and Retina Registry at Wills Eye Hospital. Patients in this series presented between 1984 and 1990 and were examined and treated by one or more of the authors. Initial evaluation of each patient included Snellen visual acuity, manifest refraction, intraocular tension measurement by applanation (pneumotonometry was used in one case in which there was marked corneal
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irregularity), slit-lamp biomicroscopy, indentation gonioscopy, and fundus examination. Records of prior ophthalmic care and most recent spectacle correction were obtained. The choice of therapy was based on the initial examination and response to subsequent therapy.
CASE REPORTS Case 1. A 12-year-old girl with a history of prematurity (26 weeks gestation, birth weight 850 g) and poor vision was seen in consultation for her glaucoma. Best corrected visual acuity was light perception in the right eye and 20/400 in the left. Spectacle correction was -3.00 + 1.50 X 23 in the right eye and -4.00 in the left. Results of slit-lamp examination showed a dense cataract in the right eye and posterior subcapsular opacity in the left. A retrolental glial mass was noted in each eye. Results of gonioscopy showed an open angle with the ciliary body band visible and a flat iris configuration in the right eye, whereas the left angle was very narrow with Schwalbe's line visible and nearly 360 0 of peripheral anterior synechiae without neovascularization. Intraocular pressures (lOPs) were 20 mmHg in the right eye and 42 mmHg in the left. The patient was treated with oral acetazolamide 125 mg twice daily and levobunolol 0.5% twice daily in the left eye. The lOP of the left eye has been maintained at 17 mmHg and the patient's visual acuity has remained unchanged for I year. Case 2. A 45-year-old man with a history of prematurity (birth weight 1350 g) and perinatal oxygen administration was referred for management of his glaucoma. His right eye had been enucleated at age 2 for uncontrolled glaucoma and pain. He had a 2-year history of lOPs in his left eye ranging from 17 to 28 mmHg on dipivefrin 0.1 % twice daily and timolol 0.5% twice daily. Best corrected visual acuity was 20/40 with a spectacle correction of -18.50 + 3.50 X 96. Results of slit-lamp examination showed a shallow anterior chamber and mild lenticular nuclear sclerosis. Intraocular pressure by applanation was 17 mmHg. Results of gonioscopy showed a very narrow occludable angle with only the most anterior trabecular meshwork visible. Indentation gonioscopy demonstrated 60 0 of peripheral anterior synechiae and brought the scleral spur into view elsewhere. Results of fundus examination showed findings of regressed ROP and peripheral retinoschisis. A neodymium:YAG laser peripheral iridectomy was performed, after which lOPs ranged from 14 to 20 mmHg on timolol 0.5% twice daily in the left eye. Results of subsequent gonioscopic examination showed a deeper angle configuration, with scleral spur visible without indentation and no worsening of the peripheral anterior synechiae. Visual acuity, automated visual fields, optic disc and fundus appearance have remained stable 14 months after laser treatment. Case 3. A 35-year-old man with a history of prematurity, perinatal oxygen therapy, and poor vision in his left eye since birth presented to the emergency department complaining of pain in his left eye of 3 weeks' duration. His spectacle correction was -4.25 + 0.25 X 125 in the right eye and -3.50 sphere in the left. Visual acuity was 20/25- in the right eye and counting fingers in the left. Examination of the left eye showed ciliary injection, mid-dilated and fixed pupil, and a dense brunescent cataract. Intraocular pressures were 15 mmHg in the right eye and 37 mmHg in the left. Results of gonioscopic examination showed the right angle to be open to the ciliary body band and the left closed for nearly 360 0 • There was no view of the fundus of the left eye.
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Administration of a single dose oftimolol 0.5% in the left eye and oral acetazolamide 500 mg lowered the lOP to 17 mmHg in the left eye. The patient underwent neodymium:YAG peripheral iridectomy and the angle deepened appreciably. One week later his lOP was lowered to 15 mmHg in the left eye on timolol 0.5% twice daily. He has since failed to return for further follow-up evaluations. Case 4. A 39-year-old woman who received perinatal oxygen therapy was evaluated for elevated lOP in the left eye. Visual acuity was 20/50 in the right eye with spectacle correction of -2.50 + 3.25 X 5 and counting fingers in the left with plano correction. Results of examination showed bilateral band keratopathy. The anterior chamber of the left eye was shallow. Gonioscopic examination showed that the right angle was open to the ciliary body band with no synechiae. The left angle was quite narrow, with an apparent iris insertion just behind Schwalbe's line. Indentation gonioscopy demonstrated 180 0 of the angle to be closed with peripheral anterior synechiae and the remainder open to the ciliary body band. Temporal dragging of the retina was present in both eyes. Intraocular tensions were 13 mmHg in the right eye and 28 mmHg in the left. Ultrasonic biometry measured the axial lengths to be 22.8 mm in the right eye and 22.5 mm in the left. Anterior chamber depths were 2.9 mm in the right eye and 2.6 mm in the left. Levobunolol 0.5% twice daily lowered the lOP of the left eye to 17 mmHg. However, because the anterior chamber angle remained occludable, a neodymium:YAG peripheral iridectomy was performed. Nine months after treatment, pressure in the left eye has ranged between I 7 and I 9 mmHg on levobunolol 0.5% once daily in the left eye. The anterior chamber is deeper, with the apparent iris insertion at the scleral spur and no change in the synechiae. Case 5. A 37-year-old woman with a history of ROP was referred for management of her glaucoma. She had undergone pars plana vitrectomy for retinal detachment in the right eye at age 31. Best corrected visual acuity was counting fingers in each eye. She had a spectacle correction of -12.75 + 2.75 X 80 in the right eye and -12.25 + 3.25 X 105 in the left. Results of slit-lamp examination showed a shallow anterior chamber in both eyes. Intraocular pressures were 32 mmHg in the right eye and 18 mmHg in the left. Both angles were narrow, with steeply convex peripheral irides and apparent insertions at the scleral spur. The iridocorneal angle was 15 0 in the right eye with 90 0 of peripheral anterior synechiae. The angle was a slightly deeper 20 0 with no synechiae in the left. Ultrasonic measurements of axial length yielded 24.8 mm in the right eye and 24.1 mm in the left. A neodymium:YAG peripheral iridectomy deepened the angle of the right eye but failed to lower lOP. Three months later she underwent trabeculectomy in the right eye with postoperative administration of 5-fluorouracil. Six months after surgery, lOPs in the right eye ranged from 16 to 19 mmHg on timolol 0.5% once daily. Case 6. A 15-year-old boy with a history ofROP was referred for control of his glaucoma. Visual acuity was hand motions in the right eye and no light perception in the left. Results of slitlamp examination showed extensive anterior segment neovascularization of the right eye. Intraocular tensions were 38 mmHg in the right eye and 32 mmHg in the left. A trabeculectomy was performed on the right eye, which failed to lower lOP. Thus, the right eye underwent cyclocryotherapy, which lowered the lOP to 19 mmHg. After six months, the pressure in the right eye remained 19 mmHg and the visual acuity was light perception. Case 7. A 39-year-old man with ROPpresented with severe pain in his right eye of3 days' duration. Visual acuity was count-
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Table 1. Presenting Features of Ten Eyes with Late-onset Secondary Angle-closure Glaucoma Associated with Retinopathy of Prematurity Case No.
Age (yrs)
Eye
1 2 3 4 5 6 7 8 9 10
12 45 35 39 37 15 39 27 27 40
OS OS OS OS 00 00 00 00 OS OS
Visual Acuity
Refraction (diopters)
lOP (mmHg)
Angle Configuration
PAS
20/400 20/40
-4.00 -16.75 -3.50
42 17 37 28 32 38 48 35 34 59
Narrow Occludable Closed Narrow Open, steep iris Closed neovascularization Shallow chamber Flat chamber Closed Closed neovascularization
-360 0 60 0 -360 0 180 0 90 0 360 0
CF CF CF HM CF HM NLP CF
Plano
-10.75 NA NA
-5.00 -4.00 +5.50
NA NA
-360 0 360 0
Prior Treatment None Medical None Medical Medical None None Medical, PI Medical Medical
lOP = intraocular pressure; PAS = peripheral anterior synechiae; OS = left eye; CF = counting fingers; 00 = right eye; HM = hand motions; NA = not available; PI = surgical peripheral iridectomy; NLP = no light perception.
ing fingers in his right eye and 20/40+ in the left. Results of slitlamp examination of the right eye showed a shallow anterior chamber and dense intumescent cataract. Intraocular pressures were 48 mmHg in the right eye and 16 mmHg in the left. Limballensectomy was performed in the right eye, with subsequent deepening of the anterior chamber. Postoperative lOPs have ranged from 14 to 18 mmHg for 6 years, and visual acuity in the right eye has remained hand motions for 4 years. Case 8. A 27-year-old woman with a history of ROP was referred for management of her glaucoma. Her right eye had undergone surgical iridectomy for glaucoma at age 2. Her glaucomatous blind left eye was enucleated for pain control at age 17. Visual acuity in her right eye was hand motions, and she was wearing a spectacle correction of -5.00 sphere. Results of slit-lamp examination showed marked corneal irregularitywith band keratopathy. The anterior chamber was flat and there was a superior iridectomy, which did not appear patent. A dense cataract precluded fundus evaluation. The lOP was 35 mmHg by pneumotonometry. Multiple medications, including topical iJ-blockers, dipivefrin, apraclonidine, and oral carbonic anhydrase inhibitors failed to lower the lOP below 24 mmHg. The patient refused to undergo intraocular surgery but agreed to neodymium:YAG cyclophotocoagulation with three treatments over 8 months. Her lOP remains 8 mmHg without medication 14 months after her last laser procedure, and her visual acuity remains hand motions. Case 9. A 27-y~r-old woman with a birth weight of 1250 g and ROP was referred for her painful glaucomatous left eye. She was wearing a refractive correction of -4.00 sphere in both eyes. Visual acuity was 20/200 in the right eye and no light perception in the left. Results of slit-lamp examination showed microcornea in both eyes. There was microcystic corneal edema and an extremely shallow anterior chamber in the left eye. Results of gonioscopy showed the right eye to have an open angle to the ciliary body band, while the left had a closed angle for nearly 360 0 • Intraocular pressures while taking timolol 0.5% twice daily, dipivefrin 0.1 % twice daily, prednisolone acetate 1% every 3 hours, and oral acetazolamide 500 mg twice daily were 8 mmHg in the right eye and 34 mmHg in the left. Results of fundus evaluation of the right eye showed temporal dragging of posterior pole structures. No view of the left fundus was possible. A Bscan ultrasound demonstrated an inferior retinal detachment that had been present on prior ultrasound examinations and no intraocular mass. Administration of atropine I %three times daily, prednisolone acetate I %hourly, and timoiol 0.5% twice daily to the left eye
failed to control either the pain or the lOP. Her left eye underwent neodymium:YAG cyclophotocoagulation, neodymium: YAG peripheral iridectomy, and retrobulbar injection of ethanol. After the peripheral iridectomy, the anterior chamber was noted to deepen dramatically. One month later, the lOP was 22 mmHg in the left eye and the eye remains comfortable. Case 10. A 40-year-old woman with a history of ROP presented with a I-week history of increasing pain and worsening vision in the left eye. Best-corrected visual acuities were hand motions in the right eye and counting fingers in the left. Her spectacle correction was +4.00 + 3 2. 5 X 86 in the right eye and +3.75 + 3.50 X 89 in the left. Results of slit-lamp examination showed microcornea in both eyes. The left eye had microcystic epithelial and stromal corneal edema, the anterior chamber was shallow, and iris neovascularization was present. Intraocular pressures were 19 mmHg in the right eye and 59 mmHg in the left. Results of gonioscopic examination showed the right eye to have an angle open to the ciliary body band, while the left angle was closed for 360 0 • Results of fundus examination showed temporally dragged optic discs, maculae, and retinal vessels in both eyes. The patient underwent implantation of an alloplastic tube shunt and thermal sclerostomy in the left eye. Two months after surgery, visual acuity had fallen to hand motions while lOPs ranged from 6 to 15 mmHg after administration of atropine 1 % and prednisolone acetate 1 %.
RESULTS The ten cases described in the present series are summarized in Tables 1 and 2. All ten eyes of ten patients had secondary angle-closure glaucoma associated with regressed ROP. All patients were born before the reporting of the international classification of ROP,1O and, as a result, information regarding staging of ROP in these patients was not available. Patients ranged in age from 12 to 45 years at the time of presentation (mean age, 32 years). Eight eyes had nonneovascular mechanisms for their angle closure while two had neovascular angle closure. Of the eight eyes with nonneovascular angle closure, six presented with chronic angle closure while two presented with a component of acute angle closure. One of these eyes had been painful for 3 weeks (case 3) and the 1095
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Table 2. Management and Posttreatment Features of Ten Eyes with Late-onset Secondary Angle-closure Glaucoma Associated with Retinopathy of Prematurity Gase No.
Treatment
lOP (mmHg)
1 2 3 4 5 6 7 8 9 10
Medical YAG PI YAG PI YAG PI YAG PI, trabeculectomy Trabeculectomy, GGT Lensectomy GPG GPG, YAG PI, ethanol Tube shunt
17 20 15 18 17 19 16 8 22 6
Visual Acuity (if changed)
GF- HM HM- LP GF- HM GF- HM
Angle (if changed) Deeper Deeper Deeper Deeper Deeper
Follow-up (mos) 12 14 1 wk 9 6 6 72 14
1
2
lOP = intraocular pressure at last follow-up; GGT = cyclocryotherapy; GPG = neodymium:YAG cyclophotocoagulation; YAG PI = neodymium:YAG peripheral iridectomy; GF = counting fingers; HM = hand motions; LP = light perception; ethanol = retrobulbar injection of ethanol.
other for 3 days (case 7). One of the two eyes with neovascular angle closure (case lO) also presented in an acute manner, with I week of increasing pain. Only one eye of each patient presented with angle-closure glaucoma requiring treatment. The right eye was involved in six patients and the left in four. The mean lOP of these eyes at presentation was 37 mmHg (range, 17 to 59 mmHg). Only two eyes (cases 2 and 4) had initial lOPs in the normal range. The most recent refraction, in dioptric spherical equivalent, demonstrated a myopic correction in six eyes, plano correction in one eye, and a hyperopic correction in one eye. Recent refraction was unavailable for two eyes. The visual acuities of affected eyes at the time of presentation ranged from 20/40 to no light perception. Nine of the ten eyes had initial visual acuity of 20/400 or worse. Results of pretreatment gonioscopy showed that two of the eyes that had angles that were completely closed had anterior segment neovascularization. Six eyes had narrow or very narrow angle configurations and peripheral anterior synechiae ranging from 60 0 to nearly 360 0 • Three of these eyes (cases 2, 4, and 5) had less than or equal to 180 0 of synechial angle closure and three eyes (cases I, 3, and 9) had nearly 360 0 of synechiae. Two eyes (cases 7 and 8) had poorly visualized angles. On presentation, six eyes had prior medical management for glaucoma whereas four had undergone no prior treatment. One of the eyes (case 8) with glaucoma that had been managed medically had undergone a surgical peripheral iridectomy when the patient was 2 years old. At the time of presentation, the iridectomy in this eye was not patent. Treatment of eyes with this form of secondary angle closure included medical management alone (I eye), neodymium:YAG peripheral iridectomy (5 eyes), trabeculectomy (2 eyes), limballensectomy (I eye), alloplastic tube shunt implantation (I eye), and cilioablative procedures (3 eyes). Three eyes required more than one of these treatments. Follow-up ranged from I.week to 6 years (mean, 1.1 years). lO96
In all eyes in this series, initial attempts were made to control the glaucoma with medication. Only one eye (case I) was successfully managed with medications alone, and the lOP has been well-controlled for 1 year. This is also the only eye in the present series in which a retrolental mass was demonstrated. Five eyes were treated with neodymium:YAG peripheral iridectomy. Four of these five eyes presented with no anterior segment neovascularization, less than 270 0 of synechial angle closure, and the potential for useful vision (cases 2,3,4, and 5). The fifth eye (case 9) presented with nearly 360 0 of synechial angle closure and no useful vision. In this eye, a component of pupillary block was suspected and a neodymium:YAG peripheral iridectomy was performed in conjunction with cyclophotocoagulation. Three of the four eyes treated with neodymium:Y AG peripheral iridectomies alone have not required further surgical intervention (cases 2, 3, and 4). The mean followup of these four eyes is 7 months (range, I week to 14 months). One eye that underwent neodymium:YAG peripheral iridectomy (case 5) required subsequent trabeculectomy. A limballensectomy was performed in one eye (case 7). This patient presented with symptoms of acute angle closure and a dense intumescent cataract. Two patients presented with neovascular angle-closure glaucoma. In one monocular patient with visual acuity of hand motions, a trabeculectomy was attempted, but failed (case 6). This eye subsequently required cyclocryotherapy, and the visual acuity changed to light perception with good control of lOP. The second patient (case lO) presented with extensive anterior segment neovascularization and an lOP of 59 mmHg in her better seeing eye. An alloplastic tube shunt was implanted in this eye, but despite good lOP control, visual acuity is only hand motions. Two additional eyes with nonneovascular angle closure were successfully treated with cilioablative procedures. One of these was in a monocular patient with visual acuity of hand motions (case 8). The other eye was painful and
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without light perception (case 9). This latter case was additionally managed with retrobulbar injection of ethanol for pain control. The postoperative visual acuity of six eyes did not change from the pretreatment level. Four eyes had a decline in visual acuity. In three eyes (cases 5, 7, and 10) the decline was from counting fingers to hand motions and in one (case 6) from hand motions to light perception. Posttreatment lOPs ranged from 6 to 22 mmHg (mean, 16 mmHg). In five of the ten eyes, a posttreatment change in the anterior chamber angle configuration was noted. The angle was observed to deepen in these five cases.
DISCUSSION Angle-closure glaucoma complicating ROP most commonly occurs in infancy and early childhood. McCormick and Pratt-Johnson 5 examined pathologic specimens from four cases of infants with cicatricial ROP and angle-closure glaucoma and found the anterior chamber angle to be obliterated. Pollard II published his series of 15 infants and children with advanced cicatricial ROP in which he performed lensectomy for secondary angle-closure glaucoma. Included were two patients previously treated with peripheral iridectomy in which the glaucoma progressed, requiring lensectomy. McCormick and Pratt-Johnson5 described a case in which they performed bilateral needle aspiration of the lenses, which deepened the anterior chamber and lowered lOP. Johnson and Swan l2 performed 42 peripheral iridectomies in 26 infants and children with ROP for acute or chronic glaucoma. Residual vision was preserved, at least temporarily, in 16 eyes, However, Walton 2 noted that iridectomy was not uniformly successful in averting chronic angle closure and thus suggested that pupillary block was not the primary mechanism for angle closure in these young patients. In infants and children with stage 5 ROP, a variety of mechanisms for the development of glaucoma have been hypothesized. I - 4,7 However, the mechanism considered most likely to cause angle-closure glaucoma in these infants and children is the anterior displacement of the lensiris diaphragm secondary to contraction of a retrolental fibroglial mass. I ,4 The youngest patient in the preseni study (case 1) is 12 years of age. She is also the only patient in this series in whom a retrolental mass was demonstrated by either clinical examination or ultrasonography. Thus, she may represent a relatively late presentation of the more common angle-closure glaucoma in children with ROP, rather than an example of the late-onset angle-closure glaucoma we distinguish in the present series. Nonetheless, her case is included to document the range of clinical presentations in the setting of angle closure later in the course of ROP. Her glaucoma has been well-controlled on oral acetazolamide and topicallevobunolol for 1 year. She may require additional intervention in the future, and a filtering operation would be recommended in this situation since
there is extensive synechial angle closure and useful vision to preserve. In adult eyes with ROP and no retrolental mass, another mechanism for the development of glaucoma may be operative. Proposed mechanisms include both ciliary block and pupillary block. 7-9 The average age of the eight patients in the present series with nonneovascular angle closure mechanisms is 33 years. In contrast, the average age of presentation for primary angle closure is the seventh to eighth decade of life. Primary angle-closure glaucoma most often occurs in a small hyperopic eye with a shallow anterior chamber and a large lens. Most eyes in the present series are myopic. In the current study, the two eyes with axial length measurements of 22.5 and 24.8 mm had spherical equivalent refractions of plano and -10.75 diopters, respectively. Thus, axial lengths of these two eyes are not as long as might be expected for their degree of myopia. Hibino et al 13 reported that the ratio of the thickness of the lens to the axial length of the eye was increased in cases of ROP. However, the etiology of the large lens in ROP remains unknown. It is possible that the hypoxia of peripheral retina may inhibit normal ciliary body and zonular development, and a lens develops that is large in relation to other anterior segment structures. The presence of an enlarged lens in patients with a history of ROP can account for both the myopia and the proclivity for angle closure. Thus, adults with regressed ROP and an already enlarged lens are at risk for developing angle closure at an early age as their lens begins to enlarge with cortical changes and slight intumescence. Smith and Shivitz8 reported three cases of angle-closure glaucoma in adults with regressed ROP. Peripheral iridectomy prevented further attacks of angle closure in these eyes. They postulated that a pupillary block mechanism was the cause of the angle closure and suggested that peripheral iridectomy was the procedure of choice in these cases. Ueda and Ogin09 reported a similar case that was also successfully treated with iridectomy. Ultrasonographic biometry in their case demonstrated a long axial length, shallow anterior chamber, and large lens. Their measurements are in concert with prior reports l3 and our clinical observations. Kushner7 reported three cases of glaucoma (two children and one 25-year-old adult) complicating ROP. The mechanism for the glaucoma in these eyes was believed to be ciliary block. In our series, no patients were assessed as having a similar mechanism for angle closure. Based on our experience with the present series of ten patients with late-onset angle-closure glaucoma associated with ROP, pupillary block appears to be the operative mechanism of angle closure in nonneovascular cases and in those without a retrolental mass. Eyes without neovascularization and less than 270° of synechial angle closure may be amenable to glaucoma control with peripheral iridectomy. Three of the four such eyes in the present series have had their glaucoma controlled by neodymium:YAG peripheral iridectomy alone. We have used filtering surgery for treatment of eyes with useful vision and no anterior segment neovascularization 1097
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Observe
Peripheral N Iridectomy
...
Observe
Observe
Trabeculectomy
Enucleation
Fig 1. Proposed management scheme for late-onset chronic angle-closure glaucoma complicating retinopathy of prematurity. PAS = peripheral anterior synechiae; rop = intraocular pressure.
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(case 9). In painful eyes with no light perception, retrobulbar injection of ethanol may be an effective adjunctive therapy for pain control. Thus, late-onset angle-closure glaucoma associated with ROP may present via two operative mechanisms: (1) pupillary block that if not treated early may progress to total synechial angle closure; or (2) neovascularization-induced secondary angle closure. Based on our clinical experience, a scheme for the management of late-onset angle-closure glaucoma complicating ROP is summarized in Figure 1 (eyes with chronic angle closure) and Figure 2 (eyes with acute angle closure). At each step of the outlined management scheme, attempts should be made to control the glaucoma with medications before proceeding to the next therapeutic intervention. It has been predicted 1 that late-onset angle-closure glaucoma complicating ROP will become a more common entity in the future. The increased incidence ofROP in patients born in the 1960s and 1970s may directly result in a rise in the incidence oflate-onset angle closure. Thus, a heightened awareness and better understanding of this condition will aid in its prompt recognition and appropriate management.
REFERENCES Peripheral Iridectomy I
Cataract Extraction
Trabeculectomy
Observe
Ciliodestruction (repeat if required) (+/- retrobulbar alcohol)
Fig 2. Proposed management scheme for late-onset acute angle-closure glaucoma complicating retinopathy of prematurity. rop = intraocular pressure.
that have failed laser iridectomy. Lensectomy has been reserved for eyes with a dense intumescent cataract, less than 270° of synechial angle closure, and no neovascularization. Tube shunt procedures should be considered in the treatment of neovascular angle-closure glaucoma in eyes with useful visual potential. The management of neovascular angle-closure glaucoma in these eyes remains challenging and the current prognosis for these eyes is quite guarded. For patients who are not surgical candidates and for those with blind and painful eyes, we recommend the use of ciliodestructive procedures. If pupillary block is suspected as being a component of the angle-closure mechanism, a peripheral iridectomy may also be performed
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1. Hittner HM, Rhodes LM, McPherson AR. Anterior segment abnormalities in cicatricial retinopathy of prematurity. Ophthalmology 1979; 86:803-16. 2. Walton OS. Retrolental fibroplasia with glaucoma. In: Chandler PA, Grant WM, eds. Glaucoma, 2nd ed. Philadelphia: Lea & Febiger, 1979; 361-2. 3. Pollard ZF. Secondary angle-closure glaucoma in cicatricial retrolental fibroplasia. Am J Ophthalmol1980; 89:651-3. 4. Blodi FC. Symposium: retrolental fibroplasia (retinopathy of prema· turity). Management. Trans Am Acad Ophthalmol Otolaryngol1955; 59:35-8. 5. McCormick AQ, Pratt·Johnson JA. Angle closure glaucoma in infancy. Ophthalmic Surg 1971; 2:91-3. 6. Kushner BJ, Sondheimer S. Medical treatment of glaucoma associated with cicatricial retinopathy of prematurity. Am J Ophthalmol1982; 94: 313-7. 7. Kushner BJ. Ciliary block glaucoma in retinopathy of prematurity. Arch Ophthalmol1982; 100:1078-9. 8. Smith J, Shivitz I. Angle-closure glaucoma in adults with cicatricial retinopathy of prematurity. Arch Ophthalmol 1984; 102:371-2. 9. Ueda N, Ogino N. Angle·closure glaucoma with pupillary block mech· anism in cicatricial retinopathy of prematurity. Ophthalmologica 1988; 196:15-8. 10. Committee for the Classification of Retinopathy of Prematurity. An international classification of retinopathy of prematurity. Arch Ophthal· mol 1984; 102:1130-4. 11. Pollard ZF. Lensectorny for secondary angle·closure glaucoma in advanced cicatricial retrolental fibroplasia. Ophthalmology 1984; 91: 395-8. 12. Johnson DR, Swan KC. Retrolental fibroplasia-a continuing problem. Trans Pacific Coast Otolaryngol Ophthalrnol Soc 1966; 47:129-33. 13. Hibino Y, Takahashi M, Majirna A. Studies on ocular functions of cicatricial retinopathy of prematurity: rneasurements of refractive elernents. Jap J Clin Ophthalmol 1978; 32:655-62.
