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CA SE PRE SENTATIONA 32-year-old white female presented to her commu-

nity ER with an 8-week complaint of pain in her left eye,headache, and subsequent nausea. Her IOP measured50 mm Hg OS, and the visual acuity in that eye washand motions.

The patient was referred to an outside ophthalmolo-gist, who diagnosed acute angle-closure glaucoma (ACG)and anterior uveitis. Her condition was managed with atopical steroid, a systemic steroid, a topical beta-blocker, sys-temic Diamox (acetazolamide; Duramed Pharmaceuticals,Inc., Pomona, NY), and Valtrex (GlaxoSmithKline, ResearchTriangle, NC), as it was initially thought that there was aviral etiology to the uveitis. Attempts to taper the med-ications caused a rebound IOP spike. The patient’s pastmedical history was significant for non-insulin-dependent

diabetes mellitus of 1 year’s duration, which was con-trolled by diet. She also had hypercholesterolemia and ill-defined psychosocial issues. Her systemic medicationincluded alprazolam. The patient was referred to me forfurther evaluation and management.

She presented to me 2 days after her diagnosis withcomplaints of nausea, vomiting, diarrhea, and photopho-bia. Subjectively, she felt that her headache and visionhad improved. Her BCVA was 20/30 OD and 20/150 OS.Her IOP measured 18 mm Hg OD and 9 mm Hg OS withGoldmann applanation tonometry. The patient’s rightpupil was round and reactive to light, whereas the leftpupil was middilated at 7 mm and minimally reactive. Aslit-lamp examination of her left eye revealed ciliary flushwith 1+ Descemet’s folds. The anterior chamber was dif-fusely shallow with temporal iris atrophy and central

Plateau Iris SyndromeExacerbated by

Lens/Angle Crowding ina Hyperopic Patient

BY GLORIA P. FLEMING, MD

Figure 1. Disc photographs of the patient’s right (A) and left eyes (B) revealed shallow physiologic cupping.

A B

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glaukomflecken of the lens. Gonioscopy revealed anappositionally closed angle of 270º in her right eye andno visible angle structures in her left eye despite gonio-compression. Funduscopy revealed physiological cuppingof 0.25 bilaterally (Figure 1).

My working diagnosis was acute ACG. I discontinuedthe Valtrex, and the patient was scheduled for a prophy-lactic laser peripheral iridotomy (LPI) in her right eye anda trabeculectomy with mitomycin C in her left eye. Bothprocedures were uncomplicated. The angle in the righteye deepened to the level of midtrabecular meshworkfollowing the LPI. After the trabeculectomy, her visualacuity improved to 20/40 OS, the IOP by applanationtonometry was 8 mm Hg, and the posterior bleb was dif-fuse. Once stable, she continued her follow-up care withher original ophthalmologist.

The patient was referred back to me 14 months laterfor progressive narrowing of the angle in her right eye,without an increase in IOP. Her BCVA (+4.75 -1.00 X 180)was 20/30 OD and (+4.25 -0.75 X 180) 20/50 OS. IOP byGoldmann applanation tonometry measured 18 mm HgOD and 9 mm Hg OS. The iridotomy was patent by slit-lamp examination, but gonioscopy revealed appositionalclosure inferonasally, with 1 clock hour of peripheral anteri-or synechiae (PAS) nasally to anterior midtrabecular mesh-work. Corneal horizontal diameters measured 10 mm OU.

The lens was clear and not intumescent in the patient’sright eye. Corneal pachymetry measured 569 µm OD and567 µm OS. A SITA-standard 24-2 Humphrey visual fieldtest (Carl Zeiss Meditec, Inc., Dublin, CA) revealed a fullvisual field in her right eye and superonasal depression inher left eye (Figure 2).

HOW WOULD YOU PROCEED IN THEPATIENT’S RIGHT EYE?

1. Would you begin miotic therapy?2. Would you perform a gonioplasty?3. Would you extract the lens and implant an IOL?

SURGICAL COUR SEI started the patient on low-dose pilocarpine 0.5% b.i.d.

while arrangements were made for an iridoplasty. Thebrief course of pilocarpine therapy significantly relievedthe iridotrabecular apposition as seen by gonioscopy, andsubsequent ultrasound biomicroscopy (UBM) revealedan open angle with evidence of plateau iris configuration(Figure 3A). The thickness of the posterior sclera couldnot be assessed. Given the crowded anterior segment, Iperformed optical biometry using the IOLMaster (CarlZeiss Meditec, Inc.), which measured an axial length of19.79 mm OD and 19.61 mm OS and an anterior cham-ber depth of 2.05 mm OD and 2.16 mm OS (Table 1).

Figure 2. Humphrey visual field testing of the patient’s right (A) and left eyes (B). First-time visual field testing shows super-

onasal depression in the left eye.

A B

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I performed an iridoplasty 360º and lysis of the smallarea of PAS. Postoperatively, she was started on pred-nisolone acetate 1% q.i.d.

OUTCOMEThree weeks postoperatively, the anterior chamber

remained shallow, although the angle in the patient’sright eye remained open without pilocarpine. RepeatUBM imaging demonstrated a continued narrow butopen angle (Figure 3B). Her IOP measured 12 mm HgOD by applanation, and the anterior segment wasquiet on biomicroscopy.

Because she was able to maintain her angle depth offof pilocarpine, I deferred lens extraction in favor ofclose observation.

DISCUSSI ONAs people age, the depth and volume of the anterior

chamber decreases as the crystalline lens thickens andassumes a more anterior position.1 The incidence of pri-mary angle closure (PAC) therefore increases with age,reaching a peak between 55 and 70 years.2 PAC rarelyoccurs in adults as young as this patient.

The major mechanism of PAC in older adults is pupil-lary block3 versus plateau iris in younger adults.2 Otherconditions such as iridociliary cysts, trauma, uveal effu-sion, and nanophthalmos should also be considered inyounger patients.

In this case, gonioscopy and UBM ruled out relativepupillary block, trauma, iridociliary cysts, and uveal effu-sions. Nanopthalmos is characterized by a small eye, hyper-opia, small corneal diameters, shallow anterior chambers,4

high lens/eye volume, and thickened sclera.5 The conditionis quite rare and usually bilateral.4 Although the patient inthis case possessed several of the aforementioned features

of nanophthalmos, they are also typical risk factors seenin patients with PAC.3 Although the characteristic double-hump sign—as seen on gonioscopy—was absent,UBM confirmed anteriorly rotated iris processes. I madethe diagnosis of plateau iris syndrome incomplete2 basedon continued appositional closure, despite the iridotomy’sconfirmed patency without elevated IOP. A crowdedanterior segment only exacerbates the shallow anteriorchamber, which I suspect contributed to the initial pres-entation of acute angle closure in the patient’s left eye.

The options for surgical management include LPI,argon laser peripheral iridoplasty, trabeculectomy, andlens extraction. Psychosocial issues may have delayedthis patient’s initial presentation to the ER. By the timeof my evaluation, the angle of her left eye revealedsynechial closure, without evidence of pupillary block. Ifelt a primary trabeculectomy with antimetaboliteswould afford her the best long-term IOP control.Although others have advocated argon laser peripheraliridoplasty in chronic angle closure, it has been reportedthat more than 36% of study patients required trabeculec-tomy by 3 months after the procedure.6 An alternativeapproach could have been initial argon laser peripheral iri-doplasty followed by cataract extraction and

Figure 3. UBM of the right eye following pilocarpine use demonstrates deepening of the iridotrabecular angle, which was main-

tained after argon laser peripheral iridoplasty (A). Note the anteriorly rotated iris processes and loss of iridociliary sulcus (B).

A B

OD OS

Central corneal thickness, µm 569 567

Axial length, mm 19.79 19.61

Anterior chamber depth, mm 2.05 2.16

Corneal diameter, horizontal, mm 10.00 10.00

TABLE 1. SUMMARY OF OCULARMEASUREMENTS

ANGLE-CLOSURE GLAUCOMA IN YOUNG PATIENTS

By Steven D. Vold, MD

Angle-closure glaucoma (ACG) in young patients pres-ents unique diagnostic and managerial challenges.Controversy continues to exist in regard to how best toapproach these patients. Dr. Fleming presents an instruc-tive case summary illustrating potential diagnostic difficul-ties and the rather wide range of managerial options avail-able to us.

N E W D I AG N O S T I C CO N CE P T S In recent years, our understanding of ACG has been

challenged. Iris thickness, choroidal expansion, and vitre-ous conductivity have been implicated as potential etio-logic factors of ACG.1 Classically, the term plateau iris syndromeinferred a specific iris configuration, increased postiridotomyIOP after dilation, and myopic refractions. Recent imagingadvances have made the identification of anterior rotationof the ciliary processes much more commonplace and ulti-mately have affected our management of ACG patients.

O P T I ON S F O R M ANAG E MENTMedication

In patients with ACG, topical, oral, or intravenous glau-coma medications and topical steroids may be useful inimmediately lowering IOP and quieting inflamed eyes.These methods are often considered temporizing meas-ures prior to more definitive surgical treatment. In eyeswith spherophakia-induced angle closure, cycloplegicagents may be helpful.

Laser Iridotomy and IridoplastyLaser peripheral iridotomy (LPI) remains the most com-

mon first-line treatment for ACG and plateau iris configu-ration. If LPI does not successfully open the anteriorchamber angle or the IOP rises significantly after post-LPIdilation, argon laser peripheral iridoplasty may be considered.

Lens-Based SurgeryCataract surgery is an extremely effective method of

managing many cases of ACG. In patients with pupillaryblock, this surgery may be completely curative. In patientswith a mild plateau iris configuration as well, lens removalalone may be enough to eliminate angle closure. Inpatients with anterior synechial formation of short dura-tion, combining cataract surgery with goniosynechialysismay also be beneficial. In patients with prominent anteriorciliary processes, endoscopic cyclophotocoagulation may

be effective in retracting the ciliary processes posteriorly andopening the angle. In younger patients, loss of accommodationand placement of a presbyopia-correcting IOL should be con-sidered. In nanophthalmic eyes, a limited pars plana vitrectomymay be necessary to successfully complete lens removal andplacement of a posterior chamber IOL.

Filtration SurgeryTrabeculectomy with an antifibrotic agent is certainly

reasonable in patients with substantial synechial angle clo-sure. Surgeons must be wary of the increased risk of ciliaryblock postoperatively. To prevent this potentially seriouscomplication, tight scleral flap sutures are generally advo-cated. Combining trabeculectomy with cataract surgery isoften beneficial to these patients as well. Combined surgerymay reduce the incidence of ciliary block or at the veryleast make treatment of this condition with a YAG laserpossible. In nanophthalmic eyes, some surgeons advocatethe placement of scleral windows to prevent postopera-tive choroidal effusions. Although devices such as tubeshunts may be considered, caution is encouraged, as they maypotentially be associated with corneal decompensation in eyeswith more shallow anterior chambers.

OT H E R CO N S I D E R AT I O N SIn young patients with angle closure, uncontrolled dia-

betes mellitus must be considered as an etiology of lensswelling-induced pupillary block. Oral medications such asTopamax (Ortho-McNeil-Janssen Pharmaceuticals, Inc.,Titusville, NJ) and other sulfa-based drugs may induceuveal effusions that may lead to ACG. In these cases, dis-continuation of the drug and short-term hypotensiveglaucoma medication may be all that is required for suc-cessful treatment. Iridociliary cysts, retinopathy of prema-turity, uveitis, Weill-Marchesani syndrome, Marfan’s syn-drome, lens subluxation, persistent fetal vasculature, andnanophthalmos with uveal effusion syndrome must alsobe considered.

Steven D. Vold, MD, is the chief executive officer and aglaucoma and cataract surgery consultant at Boozman-Hof Regional Eye Clinic, PA, in Rogers, Arkansas. Heacknowledged no financial interest in the product or com-pany mentioned herein. Dr. Vold may be reached at (479)246-1700; svold@cox.net.

1. Quigley HA. Angle-closure glaucoma-simpler answers to complex mechanisms:

LXVI Edward Jackson Memorial Lecture. Am J Ophthalmol. 2009;148(5):657-669.

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