- Email: [email protected]
Use of glaucoma drainage devices in the management of glaucoma associated with aniridia
Use of Glaucoma Drainage Devices in the Management of Glaucoma Associated With Aniridia CLAUDIA P. ARROYAVE, MD, INGRID U. SCOTT, MD, MPH, STEVEN J. GEDDE, MD, RICHARD K. PARRISH, II, MD, AND WILLIAM J. FEUER, MS
● PURPOSE:
To describe intraocular pressure (IOP) and visual acuity outcomes after glaucoma drainage device placement in eyes with glaucoma associated with aniridia. ● DESIGN: Retrospective, noncomparative, consecutive, interventional case series. ● METHODS: The medical records of all patients who underwent glaucoma drainage device placement for the management of glaucoma associated with aniridia at the Bascom Palmer Eye Institute between January 1, 1989, and December 31, 1999, were reviewed. ● RESULTS: The study included eight eyes of five patients with a median age at the time of glaucoma drainage device placement of 92 months (range, 10 – 495 months) and a median follow-up of 19 months (range, 11– 33 months). Preoperatively, the mean IOP was 35 mm Hg using a mean of one antiglaucoma medication. Postoperatively, the mean IOP was reduced to 14.9 mm Hg and no patient was using antiglaucoma medication. Final visual acuity was improved compared with preoperatively in five of eight eyes (63%) and remained unchanged in two eyes (25%). Loss of light perception occurred in one eye owing to retinal detachment. Success rates using Kaplan– Meier survival analysis were 100% at 6 months and 88% at 1 year. ● CONCLUSIONS: Glaucoma drainage device placement for glaucoma associated with aniridia achieves IOP control and vision preservation in most patients. (Am J Ophthalmol 2003;135:155–159. © 2003 by Elsevier Science Inc. All rights reserved.)
Accepted for publication Aug 29, 2002. From the Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami School of Medicine, Miami, Florida This study was supported in part by Research to Prevent Blindness, Inc., New York, New York. Inquiries to Ingrid U. Scott, MD, MPH, Bascom Palmer Eye Institute, PO Box 016880, Miami, FL 33101; phone: (305) 326-6447; fax: (305) 326-6417; e-mail: [email protected] 0002-9394/03/$30.00 PII S0002-9394(02)01934-7
©
2003 BY
A
NIRIDIA IS A RARE PANOCULAR HUMAN EYE MAL-
formation caused by a heterozygous null mutation within the PAX6 gene (a paired-box transcription factor) or cytogenetic deletions of chromosome 11p13 carrying the PAX6 gene.1 The disorder is characterized by iris hypoplasia and an incidence of glaucoma ranging from 6% to 75%.2 Other associated ocular abnormalities include corneal opacification, cataracts, nystagmus, and foveal and optic nerve hypoplasia.3 Glaucoma associated with aniridia typically develops during the first two decades of life and is attributed to progressive anterior rotation of the rudimentary iris leading to angle closure.4,5 Other mechanisms reported to cause glaucoma in patients with aniridia include an absence of the Schlemm canal and secondary angle closure after miotic therapy.2 Medical therapy is frequently ineffective in controlling glaucoma associated with aniridia.5 Several surgical techniques have been employed when medical therapy fails. Poor results have been obtained with laser trabeculoplasty,2 endoscopic diode laser cyclophotocoagulation,6,7 cyclocryotherapy,8 goniotomy,7,9,10 trabeculotomy,11,12 and trabeculectomy with mitomycin C.4,7,13–15 More favorable results have been reported with glaucoma drainage devices7,16 –18 and internal sclerectomy with an automated trephine.19 Prophylactic modified goniosurgery has been advocated to prevent the development of glaucoma.20 The purpose of the current study is to describe intraocular pressure (IOP) and visual acuity outcomes, as well as complications, of glaucoma drainage device placement in the management of glaucoma associated with aniridia.
DESIGN THE STUDY DESIGN WAS A RETROSPECTIVE, NONCOMPARA-
tive, consecutive, interventional case series including all patients who underwent glaucoma drainage device placement for the management of glaucoma associated with
ELSEVIER SCIENCE INC. ALL
RIGHTS RESERVED.
155
156
AMERICAN JOURNAL
OF
Gonio, Trab, Trab Trab, MMC Trab 39 92 92 11 10 495 336 336 Y Y F M 4 5
W B
M 3
W
N
OD OD OS OD OS OS OD OS Y N B B M M 1 2
OPHTHALMOLOGY
BGI ⫽ Baerveldt glaucoma implant; B ⫽ Black; DPM ⫽ double plate Molteno implant; F ⫽ female; F&F ⫽ fix and follow; FHG ⫽ family history of glaucoma; G ⫽ gender; GDD ⫽ glaucoma drainage device; Gonio ⫽ goniotomy; IOP ⫽ intraocular pressure; M ⫽ male; MMC Trab ⫽ trabeculectomy with mitomycin C; N ⫽ no; NLP ⫽ no light perception; OD ⫽ right eye; OS ⫽ left eye; P ⫽ patient; POS ⫽ previous ocular surgery; R ⫽ race; RRD ⫽ rhegmatogenous retinal detachment; Trab ⫽ trabeculectomy; VA ⫽ visual acuity; W ⫽ white; Y ⫽ yes. *Age at time of glaucoma drainage device placement.
0 0 0 0 0 0 0 0 14 15 3 15 21 21 16 14 20/200 20/200 NLP F&F F&F 20/80 20/200 20/200 1 0 1 0 0 2 2 2 49 50 31 32 28 38 26 26 F&F LP 20/200 F&F F&F 20/200 20/400 20/300 BGI BGI BGI BGI BGI BGI BGI
Meds IOP (mm Hg)
Postoperative
VA GDD Type Preoperative
tients underwent glaucoma drainage device placement as treatment for glaucoma associated with aniridia. Demographic characteristics and surgical outcomes of the study population are shown in Table 1. A majority of patients were male (80%) and of African American descent (60%).
G
DURING THE STUDY INTERVAL, EIGHT EYES OF FIVE PA-
P
RESULTS
R
FHA
Eye
Age* (Months)
POS
TABLE 1. Patient Demographics and Surgical Outcomes
VA
IOP (mm Hg)
Meds
tional review board of the University of Miami School of Medicine. The medical records of all patients who underwent glaucoma drainage device placement for the management of glaucoma associated with aniridia at the Bascom Palmer Eye Institute between January 1, 1989, and December 31, 1999, were reviewed. Data collected included demographic information, presence of sporadic or familial aniridia, associated ocular pathology, age at diagnosis of aniridia, age at time of glaucoma drainage device surgery, previous ocular surgeries, preoperative and postoperative visual acuity and IOP, medical treatment, length of followup, and complications. A similar operative technique was used in all patients for glaucoma drainage device placement. A fornix-based flap of conjunctiva and Tenon’s capsule was raised. When a 250-mm2 or 350-mm2 Baerveldt glaucoma implant (Pharmacia, Kalamazoo, Michigan, USA) was used, the superior and lateral recti muscles were isolated and the implant was positioned beneath both muscle bellies. One eye underwent placement of a double-plate Molteno implant (IOP, Inc, Costa Mesa, California,USA), and the plates were positioned in the superonasal and superotemporal quadrants approximately 10 mm posterior to the limbus. The implant plate was sutured to sclera using two interrupted 9-0 nylon or Prolene sutures. The tube was ligated in a watertight fashion with a 7-0 polyglactin suture. The tube was trimmed to an appropriate length and inserted into the anterior chamber through a 23-gauge needle track. Glycerin-preserved sclera was used to cover the limbal portion of the tube. The conjunctiva was reapproximated to the limbus using 7-0 polyglactin suture. Subconjunctival injections of cefazolin (50 mg) and dexamethasone (2 mg) were administered at the conclusion of the case. Success was defined as postoperative IOP of 21 mm Hg or less with or without glaucoma medications, no need for reoperation for glaucoma, and no complications resulting in visual loss. Failure was defined as postoperative IOP greater than 21 mm Hg, reoperation for glaucoma, or complications resulting in visual loss.
DPM 350-mm2 350-mm2 350-mm2 350-mm2 250-mm2 350-mm2 350-mm2
THE STUDY PROTOCOL WAS APPROVED BY THE INSTITU-
RRD
Complications
METHODS
15 31 11 31 33 14 19 19
Follow-up (Months)
aniridia at the Bascom Palmer Eye Institute between January 1, 1989, and December 31, 1999.
FEBRUARY 2003
A family history of aniridia was present in three of five patients (60%). The median age at the time of glaucoma drainage device placement surgery was 92 months, with a range of 10 to 495 months. Device implantation was performed as the initial surgical procedure in six of eight eyes (75%). Multiple glaucoma procedures were performed before glaucoma drainage device placement in two eyes, including a goniotomy in one eye and two trabeculectomies in each eye. A 250-mm2 Baerveldt glaucoma implant was placed in one of eight eyes (12%), a 350-mm2 Baerveldt was used in six eyes (75%), and a double-plate Molteno was placed in one eye (12%). The median length of follow-up was 19 months, with a range of 11 to 33 months. Preoperatively, the mean IOP was 35 mm Hg (median, 32 mm Hg; range, 26 – 50 mm Hg) using a mean of one antiglaucoma medication (median, 1; range, 0 – 2). Postoperatively, the mean IOP was reduced to 14.9 mm Hg (median, 15 mm Hg; range, 3–21 mm Hg) and no patient was taking antiglaucoma medication. At last follow-up, visual acuity was improved compared with preoperatively in five of eight eyes (63%), and remained unchanged in two eyes (25%). A final visual acuity of 20/200 or better was observed in five eyes (63%). The ability to fix and follow was maintained in two patients; more precise quantification of the visual acuity in these patients was not possible given their young age. Surgical success was achieved in seven eyes (88%) at last follow-up. Kaplan-Meier survival analysis revealed success rates of 100% at 6 months and 88% at 1 year. One eye in an 8-year-old boy developed retinal detachment associated with severe proliferative vitreoretinopathy and giant retinal tear 7 months after glaucoma drainage device placement; owing to severe lung disease, the patient did not undergo retinal detachment repair. No other complications associated with device implantation (such as cataract, lens– cornea touch, lens–tube touch, or tube migration) were observed in the current series.
DISCUSSION THE
INCIDENCE
OF
GLAUCOMA
ASSOCIATED
WITH
aniridia has been reported to range from 6% to 75%, but most studies suggest that approximately 50% of patients with aniridia develop secondary glaucoma.2,20 Glaucoma typically develops because of progressive angle changes that occur during the first two decades of life.2,4,5 In the current series, three of five patients (60%) underwent glaucoma surgery in the first 7 years of life. Glaucoma associated with aniridia is frequently refractory to medical therapy.5,21 Surgical management is generally necessary to control IOP, but there is no consensus as to the best surgical approach. Goniotomy has been used to control IOP in aniridia since 1953, when Barkan13 reported favorable results in one patient followed up for 9 VOL. 135, NO. 2
months. However, since that time, there have been numerous reports of unsuccessful IOP control after goniotomy for glaucoma associated with aniridia.3,5,10,12 Grant and Walton5 reported IOP higher than 21 mm Hg postoperatively in 18 eyes with glaucoma secondary to aniridia in which goniotomy was performed one to three times. Later, Walton10 performed therapeutic goniotomy in 14 eyes and only two (14%) demonstrated successful IOP control, defined as pressure lower than 21 mm Hg postoperatively and no need for further surgery. In 1992, Wiggins and Tomey3 reported that goniotomy and trabeculotomy are usually unsuccessful once glaucoma is present. Walton4 has advocated goniotomy as a prophylactic procedure to separate the iris from the trabecular meshwork before glaucoma develops. In 1978, he reported 38 eyes with aniridia in which goniotomy was performed and only 2 (5%) developed elevated IOP postoperatively. In 1998, Chen and Walton20 reported prophylactic goniosurgery of an average of 200 degrees in 33 patients with aniridia without glaucoma. At a mean follow-up of 9.5 years, 49 eyes (89%) had IOP less than 22 mm Hg without medicine and the remaining 6 eyes had IOP less than 22 mm Hg with one or two medications. Adachi and associates12 reported successful IOP control after goniotomy in only one of five patients with glaucoma associated with aniridia followed up for 11 years. The authors reported 10 of 12 eyes with controlled IOP after one or two trabeculotomies associated with aniridia with a mean follow-up of 9.5 years. They suggested that trabeculotomy is the preferred initial operation for uncontrolled glaucoma. However, Wiggins and Tomey3 reported that the two trabeculotomies performed in their study of patients with glaucoma secondary to aniridia were unsuccessful in controlling IOP. Okada and associates15 reported successful IOP control in young aniridic patients after filtering surgery. In their study, 17 trabeculectomies and 3 trabeculectomies with mitomycin C were performed on 10 eyes of six patients younger than 40 years, followed up for a mean of 14.6 months (range, 2– 54 months). The mean duration of successful IOP control (defined as pressure lower than 20 mm Hg with or without glaucoma medications) after filtering surgery was 14.6 months. Several other studies, however, have reported poor IOP control after trabeculectomy in eyes with glaucoma associated with aniridia. Nelson and Spaeth2 noted that in five of 14 patients with glaucoma associated with aniridia, IOP remained uncontrolled after trabeculectomy. Grant and Walton5 reported poor IOP control (defined as pressure over 22 mm Hg or the need for further surgery) in nine eyes of seven patients treated with filtering surgery for glaucoma associated with aniridia. Adachi and associates12 reported five eyes treated with trabeculectomy as the initial procedure in glaucoma associated with aniridia; only one eye achieved IOP control for 1 year. In the remaining four eyes, failure occurred soon after surgery. All five eyes required further
GLAUCOMA DRAINAGE DEVICES
IN
ANIRIDIA
157
TABLE 2. Previously Published Data on Glaucoma Drainage Device (GDD) Placement in Eyes With Glaucoma Associated With Aniridia
Author
Number of Patients
Age*
GDD Type
Follow-up
Wiggins3 Adachi12 Molteno16 Bilson17 Cunliffe18
6 3 3 2 3
7 yrs 2 wks, 28 yrs, 14 yrs Not indicated 13 yrs 14 yrs, 20 yrs, 26 yrs
Molteno† Molteno† Molteno† Molteno‡ Molteno§
Not indicated 9 yrs Not indicated 3 yrs 8 yrs
IOP Criteria for Success
⬍21 ⬍21 ⬍21 ⬍21 ⬍22
mm mm mm mm mm
Hg Hg Hg Hg Hg without meds
Success Rate
83% 66% 100% 100% 83%
IOP ⫽ intraocular pressure; meds ⫽ antiglaucoma medications; wks ⫽ weeks; yrs ⫽ years. *Age at time of GDD placement. † Type of Molteno implant was not specified in report. ‡ Double-plate Molteno implant was placed in two stages. § Double-plate Molteno implant was placed in two eyes, and a single-plate Molteno was placed in one eye.
surgery for IOP control. In one patient in our series, trabeculectomy was performed twice in each eye, but IOP remained uncontrolled during follow-up of 1 to 7 months. In those two eyes, pressure was controlled after glaucoma drainage device implantation. Previously published data on glaucoma drainage device placement in eyes with glaucoma associated with aniridia are shown in Table 2. In a report of outcomes of juvenile glaucoma (including three eyes with aniridia) after glaucoma drainage device implantation, Molteno and coworkers16 reported that successful reduction and IOP control (defined as pressure consistently less than 20 mm Hg without medical treatment) was achieved in all patients postoperatively; the duration of follow-up in these patients is not provided in the report. In 1989, Billson and associates17 described two patients with glaucoma secondary to aniridia treated successfully with a Molteno implant. Adachi and colleagues12 reported IOP lower than 21 mm Hg and no need for further surgery in two of six patients with glaucoma associated with aniridia treated with glaucoma drainage device. In 1992, Wiggins and Tomey3 reported the outcomes of glaucoma surgery in 17 eyes of 10 patients with aniridia. Forty-five procedures were performed, and 11 eyes achieved IOP control with a mean of 2.8 surgeries. They observed that trabeculectomy and cyclocryotherapy controlled the IOP in 9% (one of 11 eyes) and 25% (five of 20 eyes), respectively, while Molteno implant was successful in 83% (five of six eyes). Reported complications of glaucoma drainage device placement in eyes with aniridia include flattening of the anterior chamber in one of three eyes (33%)12 and tube migration in one of six eyes (17%).3 In the current series, glaucoma drainage device implantation for glaucoma associated with aniridia was performed as the initial procedure in six eyes (75%) and after multiple surgeries in two eyes (25%). One of the eyes that underwent glaucoma drainage device placement as initial surgical treatment developed retinal detachment associated 158
AMERICAN JOURNAL
with severe proliferative vitreoretinopathy and giant retinal tear. The remaining seven eyes (88%) had controlled IOP during a follow-up ranging from 11 to 39 months. The vision was improved in five eyes and preserved in two. In the current series; glaucoma drainage device placement for glaucoma associated with aniridia achieved successful IOP control and vision preservation in most patients. The results of the current study suggest that glaucoma drainage device implantation may be considered as the initial surgical treatment in eyes with glaucoma associated with aniridia.
REFERENCES 1. Lauderdale JD, Wilensky JS, Oliver ER, Walton DS, Glaser T. 3' deletions cause aniridia by preventing PAX6 gene expression. Proc Natl Acad Sci U S A 2000;97:1375–1379. 2. Nelson LB, Spaeth GL, Nowinski, et al. Aniridia. A review. Surv Ophthalmol 1984;28:621– 642. 3. Wiggins RE, Tomey KF. The results of glaucoma surgery in aniridia. Arch Ophthalmol 1992;110:503–505. 4. Walton DS. Aniridia with glaucoma: In: Chandler PA, Grant WM, editors: Glaucoma. Philadelphia, Lea & Febiger, 1979, 351–354. 5. Grant WM, Walton DS. Progressive changes in the angle in congenital aniridia, with development of glaucoma. Am J Ophthalmol 1974;78:842–847. 6. Plager DA, Neely DE. Intermediate-term results of endoscopic diode laser cyclophotocoagulation for pediatric glaucoma. J AAPOS 1999;3:131–137. 7. Wallace DK, Plager DA, Zinder SK, et al. Surgical results of secondary glaucomas in childhood. Ophthalmology 1998; 105:101–111. 8. Wagle NS, Freedman SF, Buckley EG, Davis JS, Biglan AW. Long-term outcome of cyclocryotherapy for refractory pediatric glaucoma. Ophthalmology 1998;105:1921–1927. 9. Callahan A. Aniridia with ectopia lentis and secondary glaucoma. Am J Ophthalmol 1949;32:28 –40. 10. Walton DS. Aniridic glaucoma: the results of gonio-surgery to prevent and treat this problem. Trans Am Ophthalmol Soc 1986;84:59 –70. OF
OPHTHALMOLOGY
FEBRUARY 2003
11. Mintz-Hittner HA. Aniridia. Glaucoma associated with developmental disorders: In: Ritch R, Shields MB, Kruping T, editors: The glaucomas. St Louis, Mosby, 1996, 859 –874. 12. Adachi M, Dickens CJ, Hetherington J, et al. Clinical experience of trabeculotomy for the surgical treatment of aniridic glaucoma. Ophthalmology 1997;104:2121–2125. 13. Barkan O. Goniotomy for glaucoma associated with aniridia. Arch Ophthalmol 1953;49:1–5. 14. Okada K, Mishima HK, Masumoto M, et al. Results of filtering surgery in young patients with aniridia. Hiroshima J Med Sci 2000;49:135–138. 15. Mandal AK, Prasad K, Naduvilath TJ. Surgical results and complications of mitomycin-C–augmented trabeculectomy in refractory developmental glaucoma. Ophthalmic Surg Laser 1999;30:473–480. 16. Molteno ACB, Ancker E, Van Biljon G. Surgical technique for advanced juvenile glaucoma. Arch Ophthalmol 1984; 102:51–57.
VOL. 135, NO. 2
17. Billson F, Thomas R, Aylward W. The use of two stage Molteno implants in developmental glaucoma. J Pediatr Ophthalmol Strabismus 1989;26:3–8. 18. Cunliffe IA, Molteno AC. Long-term follow-up of Molteno drains used in the treatment of glaucoma presenting in childhood. Eye 1998;12:379 –385. 19. Brown RH, Lynch MG, et al. Internal sclerectomy with an automated trephine for advanced glaucoma. Ophthalmology 1988;95:728 –734. 20. Chen CT, Walton DS. Goniosurgery for prevention of aniridic glaucoma. Arch Ophthalmol 1999;117:1144 – 1148. 21. Berlin HS, Ritch R. The treatment of glaucoma secondary to aniridia. Mt Sinai J Med 1981;48:111–115. 22. Arroyave CP, Scott IU, Fantes FE, et al. Corneal graft survival and intraocular pressure control after penetrating keratoplasty and glaucoma drainage device implantation. Ophthalmology 2001;108:1978 –1985.
GLAUCOMA DRAINAGE DEVICES
IN
ANIRIDIA
159
● PURPOSE:
To describe intraocular pressure (IOP) and visual acuity outcomes after glaucoma drainage device placement in eyes with glaucoma associated with aniridia. ● DESIGN: Retrospective, noncomparative, consecutive, interventional case series. ● METHODS: The medical records of all patients who underwent glaucoma drainage device placement for the management of glaucoma associated with aniridia at the Bascom Palmer Eye Institute between January 1, 1989, and December 31, 1999, were reviewed. ● RESULTS: The study included eight eyes of five patients with a median age at the time of glaucoma drainage device placement of 92 months (range, 10 – 495 months) and a median follow-up of 19 months (range, 11– 33 months). Preoperatively, the mean IOP was 35 mm Hg using a mean of one antiglaucoma medication. Postoperatively, the mean IOP was reduced to 14.9 mm Hg and no patient was using antiglaucoma medication. Final visual acuity was improved compared with preoperatively in five of eight eyes (63%) and remained unchanged in two eyes (25%). Loss of light perception occurred in one eye owing to retinal detachment. Success rates using Kaplan– Meier survival analysis were 100% at 6 months and 88% at 1 year. ● CONCLUSIONS: Glaucoma drainage device placement for glaucoma associated with aniridia achieves IOP control and vision preservation in most patients. (Am J Ophthalmol 2003;135:155–159. © 2003 by Elsevier Science Inc. All rights reserved.)
Accepted for publication Aug 29, 2002. From the Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami School of Medicine, Miami, Florida This study was supported in part by Research to Prevent Blindness, Inc., New York, New York. Inquiries to Ingrid U. Scott, MD, MPH, Bascom Palmer Eye Institute, PO Box 016880, Miami, FL 33101; phone: (305) 326-6447; fax: (305) 326-6417; e-mail: [email protected] 0002-9394/03/$30.00 PII S0002-9394(02)01934-7
©
2003 BY
A
NIRIDIA IS A RARE PANOCULAR HUMAN EYE MAL-
formation caused by a heterozygous null mutation within the PAX6 gene (a paired-box transcription factor) or cytogenetic deletions of chromosome 11p13 carrying the PAX6 gene.1 The disorder is characterized by iris hypoplasia and an incidence of glaucoma ranging from 6% to 75%.2 Other associated ocular abnormalities include corneal opacification, cataracts, nystagmus, and foveal and optic nerve hypoplasia.3 Glaucoma associated with aniridia typically develops during the first two decades of life and is attributed to progressive anterior rotation of the rudimentary iris leading to angle closure.4,5 Other mechanisms reported to cause glaucoma in patients with aniridia include an absence of the Schlemm canal and secondary angle closure after miotic therapy.2 Medical therapy is frequently ineffective in controlling glaucoma associated with aniridia.5 Several surgical techniques have been employed when medical therapy fails. Poor results have been obtained with laser trabeculoplasty,2 endoscopic diode laser cyclophotocoagulation,6,7 cyclocryotherapy,8 goniotomy,7,9,10 trabeculotomy,11,12 and trabeculectomy with mitomycin C.4,7,13–15 More favorable results have been reported with glaucoma drainage devices7,16 –18 and internal sclerectomy with an automated trephine.19 Prophylactic modified goniosurgery has been advocated to prevent the development of glaucoma.20 The purpose of the current study is to describe intraocular pressure (IOP) and visual acuity outcomes, as well as complications, of glaucoma drainage device placement in the management of glaucoma associated with aniridia.
DESIGN THE STUDY DESIGN WAS A RETROSPECTIVE, NONCOMPARA-
tive, consecutive, interventional case series including all patients who underwent glaucoma drainage device placement for the management of glaucoma associated with
ELSEVIER SCIENCE INC. ALL
RIGHTS RESERVED.
155
156
AMERICAN JOURNAL
OF
Gonio, Trab, Trab Trab, MMC Trab 39 92 92 11 10 495 336 336 Y Y F M 4 5
W B
M 3
W
N
OD OD OS OD OS OS OD OS Y N B B M M 1 2
OPHTHALMOLOGY
BGI ⫽ Baerveldt glaucoma implant; B ⫽ Black; DPM ⫽ double plate Molteno implant; F ⫽ female; F&F ⫽ fix and follow; FHG ⫽ family history of glaucoma; G ⫽ gender; GDD ⫽ glaucoma drainage device; Gonio ⫽ goniotomy; IOP ⫽ intraocular pressure; M ⫽ male; MMC Trab ⫽ trabeculectomy with mitomycin C; N ⫽ no; NLP ⫽ no light perception; OD ⫽ right eye; OS ⫽ left eye; P ⫽ patient; POS ⫽ previous ocular surgery; R ⫽ race; RRD ⫽ rhegmatogenous retinal detachment; Trab ⫽ trabeculectomy; VA ⫽ visual acuity; W ⫽ white; Y ⫽ yes. *Age at time of glaucoma drainage device placement.
0 0 0 0 0 0 0 0 14 15 3 15 21 21 16 14 20/200 20/200 NLP F&F F&F 20/80 20/200 20/200 1 0 1 0 0 2 2 2 49 50 31 32 28 38 26 26 F&F LP 20/200 F&F F&F 20/200 20/400 20/300 BGI BGI BGI BGI BGI BGI BGI
Meds IOP (mm Hg)
Postoperative
VA GDD Type Preoperative
tients underwent glaucoma drainage device placement as treatment for glaucoma associated with aniridia. Demographic characteristics and surgical outcomes of the study population are shown in Table 1. A majority of patients were male (80%) and of African American descent (60%).
G
DURING THE STUDY INTERVAL, EIGHT EYES OF FIVE PA-
P
RESULTS
R
FHA
Eye
Age* (Months)
POS
TABLE 1. Patient Demographics and Surgical Outcomes
VA
IOP (mm Hg)
Meds
tional review board of the University of Miami School of Medicine. The medical records of all patients who underwent glaucoma drainage device placement for the management of glaucoma associated with aniridia at the Bascom Palmer Eye Institute between January 1, 1989, and December 31, 1999, were reviewed. Data collected included demographic information, presence of sporadic or familial aniridia, associated ocular pathology, age at diagnosis of aniridia, age at time of glaucoma drainage device surgery, previous ocular surgeries, preoperative and postoperative visual acuity and IOP, medical treatment, length of followup, and complications. A similar operative technique was used in all patients for glaucoma drainage device placement. A fornix-based flap of conjunctiva and Tenon’s capsule was raised. When a 250-mm2 or 350-mm2 Baerveldt glaucoma implant (Pharmacia, Kalamazoo, Michigan, USA) was used, the superior and lateral recti muscles were isolated and the implant was positioned beneath both muscle bellies. One eye underwent placement of a double-plate Molteno implant (IOP, Inc, Costa Mesa, California,USA), and the plates were positioned in the superonasal and superotemporal quadrants approximately 10 mm posterior to the limbus. The implant plate was sutured to sclera using two interrupted 9-0 nylon or Prolene sutures. The tube was ligated in a watertight fashion with a 7-0 polyglactin suture. The tube was trimmed to an appropriate length and inserted into the anterior chamber through a 23-gauge needle track. Glycerin-preserved sclera was used to cover the limbal portion of the tube. The conjunctiva was reapproximated to the limbus using 7-0 polyglactin suture. Subconjunctival injections of cefazolin (50 mg) and dexamethasone (2 mg) were administered at the conclusion of the case. Success was defined as postoperative IOP of 21 mm Hg or less with or without glaucoma medications, no need for reoperation for glaucoma, and no complications resulting in visual loss. Failure was defined as postoperative IOP greater than 21 mm Hg, reoperation for glaucoma, or complications resulting in visual loss.
DPM 350-mm2 350-mm2 350-mm2 350-mm2 250-mm2 350-mm2 350-mm2
THE STUDY PROTOCOL WAS APPROVED BY THE INSTITU-
RRD
Complications
METHODS
15 31 11 31 33 14 19 19
Follow-up (Months)
aniridia at the Bascom Palmer Eye Institute between January 1, 1989, and December 31, 1999.
FEBRUARY 2003
A family history of aniridia was present in three of five patients (60%). The median age at the time of glaucoma drainage device placement surgery was 92 months, with a range of 10 to 495 months. Device implantation was performed as the initial surgical procedure in six of eight eyes (75%). Multiple glaucoma procedures were performed before glaucoma drainage device placement in two eyes, including a goniotomy in one eye and two trabeculectomies in each eye. A 250-mm2 Baerveldt glaucoma implant was placed in one of eight eyes (12%), a 350-mm2 Baerveldt was used in six eyes (75%), and a double-plate Molteno was placed in one eye (12%). The median length of follow-up was 19 months, with a range of 11 to 33 months. Preoperatively, the mean IOP was 35 mm Hg (median, 32 mm Hg; range, 26 – 50 mm Hg) using a mean of one antiglaucoma medication (median, 1; range, 0 – 2). Postoperatively, the mean IOP was reduced to 14.9 mm Hg (median, 15 mm Hg; range, 3–21 mm Hg) and no patient was taking antiglaucoma medication. At last follow-up, visual acuity was improved compared with preoperatively in five of eight eyes (63%), and remained unchanged in two eyes (25%). A final visual acuity of 20/200 or better was observed in five eyes (63%). The ability to fix and follow was maintained in two patients; more precise quantification of the visual acuity in these patients was not possible given their young age. Surgical success was achieved in seven eyes (88%) at last follow-up. Kaplan-Meier survival analysis revealed success rates of 100% at 6 months and 88% at 1 year. One eye in an 8-year-old boy developed retinal detachment associated with severe proliferative vitreoretinopathy and giant retinal tear 7 months after glaucoma drainage device placement; owing to severe lung disease, the patient did not undergo retinal detachment repair. No other complications associated with device implantation (such as cataract, lens– cornea touch, lens–tube touch, or tube migration) were observed in the current series.
DISCUSSION THE
INCIDENCE
OF
GLAUCOMA
ASSOCIATED
WITH
aniridia has been reported to range from 6% to 75%, but most studies suggest that approximately 50% of patients with aniridia develop secondary glaucoma.2,20 Glaucoma typically develops because of progressive angle changes that occur during the first two decades of life.2,4,5 In the current series, three of five patients (60%) underwent glaucoma surgery in the first 7 years of life. Glaucoma associated with aniridia is frequently refractory to medical therapy.5,21 Surgical management is generally necessary to control IOP, but there is no consensus as to the best surgical approach. Goniotomy has been used to control IOP in aniridia since 1953, when Barkan13 reported favorable results in one patient followed up for 9 VOL. 135, NO. 2
months. However, since that time, there have been numerous reports of unsuccessful IOP control after goniotomy for glaucoma associated with aniridia.3,5,10,12 Grant and Walton5 reported IOP higher than 21 mm Hg postoperatively in 18 eyes with glaucoma secondary to aniridia in which goniotomy was performed one to three times. Later, Walton10 performed therapeutic goniotomy in 14 eyes and only two (14%) demonstrated successful IOP control, defined as pressure lower than 21 mm Hg postoperatively and no need for further surgery. In 1992, Wiggins and Tomey3 reported that goniotomy and trabeculotomy are usually unsuccessful once glaucoma is present. Walton4 has advocated goniotomy as a prophylactic procedure to separate the iris from the trabecular meshwork before glaucoma develops. In 1978, he reported 38 eyes with aniridia in which goniotomy was performed and only 2 (5%) developed elevated IOP postoperatively. In 1998, Chen and Walton20 reported prophylactic goniosurgery of an average of 200 degrees in 33 patients with aniridia without glaucoma. At a mean follow-up of 9.5 years, 49 eyes (89%) had IOP less than 22 mm Hg without medicine and the remaining 6 eyes had IOP less than 22 mm Hg with one or two medications. Adachi and associates12 reported successful IOP control after goniotomy in only one of five patients with glaucoma associated with aniridia followed up for 11 years. The authors reported 10 of 12 eyes with controlled IOP after one or two trabeculotomies associated with aniridia with a mean follow-up of 9.5 years. They suggested that trabeculotomy is the preferred initial operation for uncontrolled glaucoma. However, Wiggins and Tomey3 reported that the two trabeculotomies performed in their study of patients with glaucoma secondary to aniridia were unsuccessful in controlling IOP. Okada and associates15 reported successful IOP control in young aniridic patients after filtering surgery. In their study, 17 trabeculectomies and 3 trabeculectomies with mitomycin C were performed on 10 eyes of six patients younger than 40 years, followed up for a mean of 14.6 months (range, 2– 54 months). The mean duration of successful IOP control (defined as pressure lower than 20 mm Hg with or without glaucoma medications) after filtering surgery was 14.6 months. Several other studies, however, have reported poor IOP control after trabeculectomy in eyes with glaucoma associated with aniridia. Nelson and Spaeth2 noted that in five of 14 patients with glaucoma associated with aniridia, IOP remained uncontrolled after trabeculectomy. Grant and Walton5 reported poor IOP control (defined as pressure over 22 mm Hg or the need for further surgery) in nine eyes of seven patients treated with filtering surgery for glaucoma associated with aniridia. Adachi and associates12 reported five eyes treated with trabeculectomy as the initial procedure in glaucoma associated with aniridia; only one eye achieved IOP control for 1 year. In the remaining four eyes, failure occurred soon after surgery. All five eyes required further
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TABLE 2. Previously Published Data on Glaucoma Drainage Device (GDD) Placement in Eyes With Glaucoma Associated With Aniridia
Author
Number of Patients
Age*
GDD Type
Follow-up
Wiggins3 Adachi12 Molteno16 Bilson17 Cunliffe18
6 3 3 2 3
7 yrs 2 wks, 28 yrs, 14 yrs Not indicated 13 yrs 14 yrs, 20 yrs, 26 yrs
Molteno† Molteno† Molteno† Molteno‡ Molteno§
Not indicated 9 yrs Not indicated 3 yrs 8 yrs
IOP Criteria for Success
⬍21 ⬍21 ⬍21 ⬍21 ⬍22
mm mm mm mm mm
Hg Hg Hg Hg Hg without meds
Success Rate
83% 66% 100% 100% 83%
IOP ⫽ intraocular pressure; meds ⫽ antiglaucoma medications; wks ⫽ weeks; yrs ⫽ years. *Age at time of GDD placement. † Type of Molteno implant was not specified in report. ‡ Double-plate Molteno implant was placed in two stages. § Double-plate Molteno implant was placed in two eyes, and a single-plate Molteno was placed in one eye.
surgery for IOP control. In one patient in our series, trabeculectomy was performed twice in each eye, but IOP remained uncontrolled during follow-up of 1 to 7 months. In those two eyes, pressure was controlled after glaucoma drainage device implantation. Previously published data on glaucoma drainage device placement in eyes with glaucoma associated with aniridia are shown in Table 2. In a report of outcomes of juvenile glaucoma (including three eyes with aniridia) after glaucoma drainage device implantation, Molteno and coworkers16 reported that successful reduction and IOP control (defined as pressure consistently less than 20 mm Hg without medical treatment) was achieved in all patients postoperatively; the duration of follow-up in these patients is not provided in the report. In 1989, Billson and associates17 described two patients with glaucoma secondary to aniridia treated successfully with a Molteno implant. Adachi and colleagues12 reported IOP lower than 21 mm Hg and no need for further surgery in two of six patients with glaucoma associated with aniridia treated with glaucoma drainage device. In 1992, Wiggins and Tomey3 reported the outcomes of glaucoma surgery in 17 eyes of 10 patients with aniridia. Forty-five procedures were performed, and 11 eyes achieved IOP control with a mean of 2.8 surgeries. They observed that trabeculectomy and cyclocryotherapy controlled the IOP in 9% (one of 11 eyes) and 25% (five of 20 eyes), respectively, while Molteno implant was successful in 83% (five of six eyes). Reported complications of glaucoma drainage device placement in eyes with aniridia include flattening of the anterior chamber in one of three eyes (33%)12 and tube migration in one of six eyes (17%).3 In the current series, glaucoma drainage device implantation for glaucoma associated with aniridia was performed as the initial procedure in six eyes (75%) and after multiple surgeries in two eyes (25%). One of the eyes that underwent glaucoma drainage device placement as initial surgical treatment developed retinal detachment associated 158
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with severe proliferative vitreoretinopathy and giant retinal tear. The remaining seven eyes (88%) had controlled IOP during a follow-up ranging from 11 to 39 months. The vision was improved in five eyes and preserved in two. In the current series; glaucoma drainage device placement for glaucoma associated with aniridia achieved successful IOP control and vision preservation in most patients. The results of the current study suggest that glaucoma drainage device implantation may be considered as the initial surgical treatment in eyes with glaucoma associated with aniridia.
REFERENCES 1. Lauderdale JD, Wilensky JS, Oliver ER, Walton DS, Glaser T. 3' deletions cause aniridia by preventing PAX6 gene expression. Proc Natl Acad Sci U S A 2000;97:1375–1379. 2. Nelson LB, Spaeth GL, Nowinski, et al. Aniridia. A review. Surv Ophthalmol 1984;28:621– 642. 3. Wiggins RE, Tomey KF. The results of glaucoma surgery in aniridia. Arch Ophthalmol 1992;110:503–505. 4. Walton DS. Aniridia with glaucoma: In: Chandler PA, Grant WM, editors: Glaucoma. Philadelphia, Lea & Febiger, 1979, 351–354. 5. Grant WM, Walton DS. Progressive changes in the angle in congenital aniridia, with development of glaucoma. Am J Ophthalmol 1974;78:842–847. 6. Plager DA, Neely DE. Intermediate-term results of endoscopic diode laser cyclophotocoagulation for pediatric glaucoma. J AAPOS 1999;3:131–137. 7. Wallace DK, Plager DA, Zinder SK, et al. Surgical results of secondary glaucomas in childhood. Ophthalmology 1998; 105:101–111. 8. Wagle NS, Freedman SF, Buckley EG, Davis JS, Biglan AW. Long-term outcome of cyclocryotherapy for refractory pediatric glaucoma. Ophthalmology 1998;105:1921–1927. 9. Callahan A. Aniridia with ectopia lentis and secondary glaucoma. Am J Ophthalmol 1949;32:28 –40. 10. Walton DS. Aniridic glaucoma: the results of gonio-surgery to prevent and treat this problem. Trans Am Ophthalmol Soc 1986;84:59 –70. OF
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11. Mintz-Hittner HA. Aniridia. Glaucoma associated with developmental disorders: In: Ritch R, Shields MB, Kruping T, editors: The glaucomas. St Louis, Mosby, 1996, 859 –874. 12. Adachi M, Dickens CJ, Hetherington J, et al. Clinical experience of trabeculotomy for the surgical treatment of aniridic glaucoma. Ophthalmology 1997;104:2121–2125. 13. Barkan O. Goniotomy for glaucoma associated with aniridia. Arch Ophthalmol 1953;49:1–5. 14. Okada K, Mishima HK, Masumoto M, et al. Results of filtering surgery in young patients with aniridia. Hiroshima J Med Sci 2000;49:135–138. 15. Mandal AK, Prasad K, Naduvilath TJ. Surgical results and complications of mitomycin-C–augmented trabeculectomy in refractory developmental glaucoma. Ophthalmic Surg Laser 1999;30:473–480. 16. Molteno ACB, Ancker E, Van Biljon G. Surgical technique for advanced juvenile glaucoma. Arch Ophthalmol 1984; 102:51–57.
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17. Billson F, Thomas R, Aylward W. The use of two stage Molteno implants in developmental glaucoma. J Pediatr Ophthalmol Strabismus 1989;26:3–8. 18. Cunliffe IA, Molteno AC. Long-term follow-up of Molteno drains used in the treatment of glaucoma presenting in childhood. Eye 1998;12:379 –385. 19. Brown RH, Lynch MG, et al. Internal sclerectomy with an automated trephine for advanced glaucoma. Ophthalmology 1988;95:728 –734. 20. Chen CT, Walton DS. Goniosurgery for prevention of aniridic glaucoma. Arch Ophthalmol 1999;117:1144 – 1148. 21. Berlin HS, Ritch R. The treatment of glaucoma secondary to aniridia. Mt Sinai J Med 1981;48:111–115. 22. Arroyave CP, Scott IU, Fantes FE, et al. Corneal graft survival and intraocular pressure control after penetrating keratoplasty and glaucoma drainage device implantation. Ophthalmology 2001;108:1978 –1985.
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