In 1991, a 10-year-old white female with a history of unilateral juvenile rheumatoid arthritis (JRA)-related uveitis began 6 years of treatment with intermittent topical steroids, pulsed systemic steroids, and methotrexate. During this time, her vision remained relatively stable in the involved left eye with a BCVA of 20/20 OU. In the seventh year of treatment, the visual acuity of her left eye diminished to 20/80 secondary to progressive posterior cataract changes and a gradual elevation in IOP, despite her advancement to maximum tolerated medical therapy. Increasing the use of topical and systemic steroids resulted in relative quiescence of the uveitis for 3 months in her involved left eye, but it caused a further loss of IOP control and changes in the optic nerve with corresponding visual field loss (nasal step) (Figure 1).
Figure 1. The visual field test showed a nasal step and early arcuate changes.
As a result of these changes, in 1997, when the patient was 17 years old, we performed an uncomplicated combined phacotrabeculectomy with adjunctive mitomycin C (MMC; 0.3 mg/mL X 3 minutes) and placed a PMMA posterior chamber IOL in her left eye. The procedure restored a BCVA of 20/25 with a fluctuating IOP in the midteens, despite episodes of recurrent uveitis over the subsequent 2 years. Persistent inflammation resulted in fibrosis of the episcleral-scleral interface that compromised the filtration function. Despite a reinstitution of maximum tolerated medical therapy, the patient's IOP rose, so we performed repeat trabeculectomy on her left eye with MMC (0.3 mg/mL X 3 minutes) in 1999. The postoperative course was uncomplicated, the patient's IOP dropped to 14 mm Hg, and her BCVA was 20/30. Unfortunately, inflammation again compromised the filter, and the IOP rose to 26 mm Hg.
HOW WOULD YOU PROCEED?
1. Would you simply observe this patient?
2. Perform another trabeculectomy or trabeculodialysis?
3. Implant a glaucoma drainage device? If so, would you choose a valved or nonvalved implant?
SURGICAL COURSE
In March 2000 (14 months after performing the repeat filter), we uneventfully placed a Baerveldt 350-mm glaucoma drainage device (Pfizer Inc., New York, NY) with a ligature suture in the superotemporal quadrant of the patient's involved left eye. Three months postoperatively, her BCVA was 20/40, and the IOP was 10 mm Hg in this eye. The mild uveitis persisted and required low-dose pulsed topical steroids. Fibrosis of the posterior chamber and anterior hyaloid membrane caused a gradual worsening of the inflammation. Nd:YAG laser therapy improved the patient's vision from 20/80 to 20/40, but she subsequently experienced further visual decline (BCVA <20/200) due to hypotony (IOP < 6 mm Hg).
We administered intravitreal steroids on two separate occasions, as well as systemic steroids, but the patient's IOP remained less than 4 mm Hg. We diagnosed hypotony maculopathy in November 2002. Two separate, anterior chamber instillations of Healon GV (Pfizer Inc.) increased the patient's IOP to the midteens (range of 13 to 16 mm Hg) only temporarily, and an improvement in her vision was transient.
Further surgical intervention in December 2002 involved ligating the Baerveldt tube with 9–0 nylon and placing a pediatric Ahmed Glaucoma Valve (surface area 96 mm2, Model S3; New World Medical, Inc., Rancho Cucamonga, CA) in the inferotemporal quadrant. The patient's IOP rose into the midteens for 1 month, and her vision improved slightly to 20/200. Marked inflammation recurred, and her IOP dropped to less than 6 mm Hg. A B-scan (Figure 2) showed atrophy of the ciliary processes and confirmed our suspicion that the patient's eye was possibly prephthisical.
Figure 2. The patient's left eye experienced hypotony and atrophy of the ciliary processes (A), whereas the ciliary processes of her right eye were normal (B).
In June 2002, we removed the Baerveldt tube, closed the fistula-needle tract, and ligated the Ahmed Glaucoma Valve with 7–0 VICRYL sutures (Ethicon Inc., Somerville, NJ). We left Healon GV within the anterior chamber (Figure 3).
Figure 3. The patient's left eye has an inferotemporal glaucoma drainage device.
OUTCOME
The postoperative course involved 48 hours of persistent hypotony, followed by a dramatic elevation of the IOP into the mid-30s. The patient's vision in her left eye improved to 20/60 with marked improvement in the choroidal effusion and macular reflex. At the time of her last follow-up (July 18, 2003), her IOP was 17 mm Hg on topical steroids, and she had a BCVA of 20/40.
DISCUSSION
Pathophysiology determines the management of uveitic glaucoma. A patient may suffer secondary open-angle glaucoma, secondary angle-closure glaucoma, or a combined mechanism. Compromised outflow may be secondary to obstruction of the trabecular meshwork caused by cellular debris or inflammation. Less commonly, a patient experiences elevated IOP secondary to prostaglandin release and hypersecretion. Secondary angle-closure glaucoma may result from peripheral anterior synechia due to chronic inflammation, previous angle closure, and a postoperatively flat anterior chamber. It may also occur in association with ciliary body swelling, pupillary block, and neovascularization. Moreover, the IOP may rise due to a combination of several mechanisms, not the least uncommon of which is steroid use in association to quiet the inflammatory process.
This case of JRA-related uveitis and secondary glaucoma of combined mechanism is not rare. Twenty to 30% of patients with JRA have uveitis,1,2 and secondary glaucoma occurs in 15% to 30% of them.3-6 Unfortunately, approximately one-third of the eyes with JRA-related uveitis and secondary glaucoma become blind.3
The long-term control of this form of secondary glaucoma remains challenging. Medical therapy controls the glaucoma in approximately 30% of the uveitic patients,1,3,7 and surgical procedures are successful in less than 70%.1,3,4,7,8 Although trabeculodialysis may have a successful outcome in 65% of cases,7,9 we chose to perform a combined phacotrabeculectomy with adjunctive MMC in this case because of the patient's significant cataract.
An increasing number of surgeons are implanting glaucoma drainage devices to treat refractory glaucomas. Surgical success rates range from 50% to 100% in cases of uveitis-related glaucoma, and the optimal device for this type of secondary glaucoma remains undetermined. The Baerveldt 350-mm glaucoma drainage device we first chose for this patient initially provided excellent IOP control, but, in retrospect, a valved drainage device might have been better tolerated. The variability of the ocular inflammation and associated fluctuations in aqueous production over a period of time, however, resulted in hypotony maculopathy. The ligation of the Baerveldt tube proved insufficient as the hypotony persisted.
Figure 4. High-resolution ultrasound demonstrates the normal ciliary processes of the patient's right eye (A) and the blunted ciliary processes of her left eye (B).
We were concerned when the initial B-scan showed ciliary process atrophy and possible phthisis (Figure 4A). A later B-scan of the patient's left eye when it had a more normalized IOP demonstrated shrinkage (blunting) of the processes (Figure 4B), but some aqueous production remained. Remarkably, despite prolonged hypotony, the patient's vision has dramatically improved, but it remains premature to assume long-term stability.
Steven V. L. Brown, MD, is Associate Professor of Ophthalmology at Rush Medical College, Rush-Presbyterian-St. Luke's Medical Center, Chicago, and is Senior Attending, Clinical Instructor at Northwestern Medical School, Evanston Northwestern Hospital, Evanston, Illinois. He holds no financial interest in any product or company described herein. Dr. Brown may be reached at (847) 492-3250; drsvlb@aol.com.
Japhlet Arenas, MD, is a fellow with Chicago Glaucoma Consultants in Chicago. He holds no financial interest in any product or company described herein.Dr. Arenas may be reached at (847) 492-3250; drsvlb@aol.com.
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