Angle Closure in Highly Myopic Eyes
8
Angle Closure in Highly Myopic Eyes Yaniv Barkana, MD, 1 Wisam Shihadeh, MD, 1 Cristiano Oliveira, MD, 1 Celso Tello, MD, 1 Jeffrey M. Liebmann, MD, 2,3 Robert Ritch, MD 1,4 Purpose: Patients with angle-closure glaucoma and high myopia are uncommon. We evaluated the clinical characteristics of all patients with angle closure and high myopia in our database and propose possible mechanisms for angle closure in these atypical patients. Design: Retrospective noncomparative case series. Participants: Our database of 17 938 patients was searched for patients with myopia of spherical equivalent of more than 6.0 diopters and angle closure. Data recorded included age at time of initial consultation, gender, slit-lamp examination results, gonioscopy results, biometric parameters, ultrasound biomicroscopy results (from 1993 onward), clinical diagnosis, and therapy. Results: Twenty patients (11 females, 9 males) were identified. Mean age at the time of consultation was 52.919.3 years. Angle-closure diagnoses included primary pupillary block (9 patients), pupillary block in an eye with keratoconus (1 patient), pupillary block secondary to a pupillary membrane associated with retinopathy of prematurity (1 patient), plateau iris configuration and syndrome (3 patients), phacomorphic glaucoma in Weill- Marchesani syndrome (2 patients), malignant glaucoma secondary to a scleral buckle (2 patients), miotic-induced angle closure (1 patient), and Marfan syndrome (1 patient). Conclusions: Angle closure can occur in eyes with high myopia. Causes of angle closure other than relative pupillary block are more common than in the general angle-closure glaucoma population. Careful gonioscopy accompanied by biometry and ultrasound biomicroscopy can lead to the correct diagnosis and individualized management in these eyes. Ophthalmology 2006;113:247–254 © 2006 by the American Academy of Ophthalmology. Narrowing of the anterior chamber angle and angle-closure glaucoma (ACG) typically are associated with hyperopia. High myopia rarely is observed in patients with these con- ditions. 1–6 To our knowledge, no case series of such eyes has been reported. We found detailed clinical descriptions of only 3 patients with high myopia and angle closure in the literature. 3,4,6 The purpose of this study was to describe the clinical characteristics, treatment, and course in a series of patients with high myopia and angle closure. Patients and Methods The charts of all patients with myopia 6.0 diopters (D) and angle closure were reviewed. Angle closure was diagnosed based on findings characteristic of acute angle closure, subacute (intermit- tent reversible) angle closure, chronic angle closure with periph- eral anterior synechiae (PAS), or appositionally closed angles on the basis of gonioscopy or ultrasound biomicroscopic (UBM) dark room provocative testing. Eyes with angle closure secondary to anterior chamber dysgenesis, proliferating membranes, or inflam- mation were excluded. Patient demographic and ocular and sys- temic findings were extracted from the medical records. The width of the iridocorneal angle, as visualized on gonios- copy, was graded according to Schaffer’s system. Gonioscopy was performed in a dark room using a 4-mirror lens with a small slit beam that did not cross the pupillary border so as to avoid a miotic response. Corneal indentation was performed with the goniolens, and if the angle widened to expose more posterior landmarks, appositional angle closure was diagnosed. If indentation did not bring about widening of a closed angle, synechial angle closure was diagnosed. Results The records of 17 938 patients were available in the database. Twenty patients with high myopia and angle closure were identi- fied. Their demographics, refraction, and biometric data are listed in Table 1. Case Reports: Primary Pupillary Block Patient 1. A 57-year-old white man had moderately deep anterior chambers and angles that were slit grade superiorly and grade I Originally received: April 27, 2005. Accepted: July 29, 2005. Manuscript no. 2005-382. 1 Department of Ophthalmology, The New York Eye and Ear Infirmary, New York, New York. 2 Department of Ophthalmology, Manhattan Eye, Ear & Throat Hospital, New York, New York. 3 Department of Ophthalmology, New York University Medical Center, New York, New York. 4 Department of Ophthalmology, New York Medical College, Valhalla, New York. Supported in part by the Shirl and Ira Oppenheimer Research Fund of the New York Glaucoma Research Institute, New York, New York, and the American Physicians Fellowship, Boston, Massachusetts. The authors have no conflicts of interest with regard to the article. Correspondence to Robert Ritch, MD, Glaucoma Service, Department of Ophthalmology, The New York Eye and Ear Infirmary, 310 East 14th Street, New York, NY 10003. E-mail: [email protected]. 247 © 2006 by the American Academy of Ophthalmology ISSN 0161-6420/06/$–see front matter Published by Elsevier Inc. doi:10.1016/j.ophtha.2005.10.006
Transcript of Angle Closure in Highly Myopic Eyes
Angle Closure in Highly Myopic Eyes
Yaniv Barkana, MD,1 Wisam Shihadeh, MD,1 Cristiano Oliveira, MD,1 Celso Tello, MD,1
Jeffrey M. Liebmann, MD,2,3 Robert Ritch, MD1,4
Purpose: Patients with angle-closure glaucoma and high myopia are uncommon. We evaluated the clinicalcharacteristics of all patients with angle closure and high myopia in our database and propose possiblemechanisms for angle closure in these atypical patients.
Design: Retrospective noncomparative case series.Participants: Our database of 17 938 patients was searched for patients with myopia of spherical equivalent
of more than �6.0 diopters and angle closure. Data recorded included age at time of initial consultation, gender,slit-lamp examination results, gonioscopy results, biometric parameters, ultrasound biomicroscopy results (from1993 onward), clinical diagnosis, and therapy.
Results: Twenty patients (11 females, 9 males) were identified. Mean age at the time of consultation was52.9�19.3 years. Angle-closure diagnoses included primary pupillary block (9 patients), pupillary block in an eyewith keratoconus (1 patient), pupillary block secondary to a pupillary membrane associated with retinopathy ofprematurity (1 patient), plateau iris configuration and syndrome (3 patients), phacomorphic glaucoma in Weill-Marchesani syndrome (2 patients), malignant glaucoma secondary to a scleral buckle (2 patients), miotic-inducedangle closure (1 patient), and Marfan syndrome (1 patient).
Conclusions: Angle closure can occur in eyes with high myopia. Causes of angle closure other than relativepupillary block are more common than in the general angle-closure glaucoma population. Careful gonioscopyaccompanied by biometry and ultrasound biomicroscopy can lead to the correct diagnosis and individualized
management in these eyes. Ophthalmology 2006;113:247–254 © 2006 by the American Academy of Ophthalmology.Narrowing of the anterior chamber angle and angle-closureglaucoma (ACG) typically are associated with hyperopia.High myopia rarely is observed in patients with these con-ditions.1–6 To our knowledge, no case series of such eyeshas been reported. We found detailed clinical descriptionsof only 3 patients with high myopia and angle closure in theliterature.3,4,6
The purpose of this study was to describe the clinicalcharacteristics, treatment, and course in a series of patientswith high myopia and angle closure.
Originally received: April 27, 2005.Accepted: July 29, 2005. Manuscript no. 2005-382.1 Department of Ophthalmology, The New York Eye and Ear Infirmary,New York, New York.2 Department of Ophthalmology, Manhattan Eye, Ear & Throat Hospital,New York, New York.3 Department of Ophthalmology, New York University Medical Center,New York, New York.4 Department of Ophthalmology, New York Medical College, Valhalla,New York.
Supported in part by the Shirl and Ira Oppenheimer Research Fund of theNew York Glaucoma Research Institute, New York, New York, and theAmerican Physicians Fellowship, Boston, Massachusetts.
The authors have no conflicts of interest with regard to the article.
Correspondence to Robert Ritch, MD, Glaucoma Service, Department ofOphthalmology, The New York Eye and Ear Infirmary, 310 East 14th
Street, New York, NY 10003. E-mail: [email protected].© 2006 by the American Academy of OphthalmologyPublished by Elsevier Inc.
Patients and Methods
The charts of all patients with myopia �6.0 diopters (D) and angleclosure were reviewed. Angle closure was diagnosed based onfindings characteristic of acute angle closure, subacute (intermit-tent reversible) angle closure, chronic angle closure with periph-eral anterior synechiae (PAS), or appositionally closed angles onthe basis of gonioscopy or ultrasound biomicroscopic (UBM) darkroom provocative testing. Eyes with angle closure secondary toanterior chamber dysgenesis, proliferating membranes, or inflam-mation were excluded. Patient demographic and ocular and sys-temic findings were extracted from the medical records.
The width of the iridocorneal angle, as visualized on gonios-copy, was graded according to Schaffer’s system. Gonioscopy wasperformed in a dark room using a 4-mirror lens with a small slitbeam that did not cross the pupillary border so as to avoid a mioticresponse. Corneal indentation was performed with the goniolens,and if the angle widened to expose more posterior landmarks,appositional angle closure was diagnosed. If indentation did notbring about widening of a closed angle, synechial angle closurewas diagnosed.
Results
The records of 17 938 patients were available in the database.Twenty patients with high myopia and angle closure were identi-fied. Their demographics, refraction, and biometric data are listedin Table 1.
Case Reports: Primary Pupillary Block
Patient 1. A 57-year-old white man had moderately deep anterior
chambers and angles that were slit grade superiorly and grade I247ISSN 0161-6420/06/$–see front matterdoi:10.1016/j.ophtha.2005.10.006
ved t
Ophthalmology Volume 113, Number 2, February 2006
elsewhere, with a convex iris approach. On complete room dark-ening and using the smallest possible slit beam, there was appo-sitional angle closure for 180° in both eyes. There was earlynuclear sclerosis. Observation was recommended.
Patient 2. A 60-year-old white man had a long history ofmedical treatment for glaucoma. The right angle was wide openwith a flat iris contour after cataract extraction surgery. The leftangle was appositionally closed to the pigmented trabecular mesh-
Table 1. Summary of Demographics, Refraction, Biometric DatHigh
PatientNo.
Date ofInitial
ConsultationAge(yrs) Gender Eye
SphericalEquivalentRefractiveError (D)
Best-correcte
VisuaAcuity
1 Feb. 1989 57 M Right �12.00 0.5Left �10.25 0.5
2 Nov. 1989 60 M Right �5.75* 0.8Left �13.25 0.4
3 Feb. 1990 57 M Right �7.00 1.0Left �6.25 1.0
4 June 1990 57 F Right �8.00 NLPLeft �10.00 0.1
5 April 1992 74 F Right �7.25 0.05Left �0.25 0.8
6 Dec. 1993 57 F Right �11.00 0.05†
Left �10.00 0.87 Feb. 2002 67 M Right �8.00 0.5
Left �9.75 0.58 July 2002 71 M Right �6.25 0.8
Left �9.25 0.679 Oct. 2003 52 F Right �8.25 1.0
Left �9.00 1.010 Oct. 2003 71 F Right �14.75 0.5
Left �16.25 0.6711 Dec. 1990 7 F Right �20.00 CF
Left �20.00 0.25
12 June 1999 58 M Right �8.75 1.0Left �8.50 0.5
13 Feb. 2001 53 F Right �10.25 0.8 � 1Left �11.50 0.8 � 1
14 May 2004 60 F Right �6.25 1.0Left �1.75 1.0
15 April 1991 31 M Right �7.75 0.8Left �8.25 0.8
16 Oct. 1991 28 F Right �9.00 0.67Left �8.25 0.4
17 Dec. 1987 75 F Right �9.00 0.05Left �8.25 0.29
18 April 2004 72 M Right �8.75 0.8Left �12.00 0.1
19 June 1992 28 M Right �7.75 1.0Left �18.00 0.67
20 June 2000 23 F Right �17.00 1.0Left �13.75 0.8
CF � counting fingers; D � diopters; F � female; M � male; NA � noBiometric data are presented when available.*Patient already had undergone cataract extraction surgery in the right e†Patient had BCVA of 20/400 in the right eye at presentation but impro
work (TM) for 360° with a markedly convex iris contour. There
248
was 3� nuclear sclerosis on the left. Laser iridotomy (LI) resultedin a grade II open angle.
Patient 3. A 57-year-old white man had a history ofhigh myopia and medical treatment for elevated intraocularpressure (IOP) in both eyes. Gonioscopy revealed a grade IIangle with a convex iris approach with segmented areas ofincreased pigmentation in both eyes, consistent with intermit-tent apposition to the TM. On room darkening and minimizing
d Clinical Presentation of 20 Patients with Angle Closure andopia
Axialength
(mm)
AnteriorChamber
Depth(mm)
LensThickness
(mm) Keratometry (D) Diagnosis
NA NA NA NA Primary pupillaryblockNA NA NA NA
NA NA NA NA Primary pupillaryblockNA NA NA NA
NA NA NA NA Primary pupillaryblockNA NA NA NA
NA NA NA NA Primary pupillaryblockNA NA NA NA
NA NA NA NA Primary pupillaryblockNA NA NA NA
24.6 2.39 5.56 42.25/44.00�107 Primary pupillaryblock24.5 2.36 5.02 42.37/44.00�107
NA NA NA NA Primary pupillaryblock24.76 4.13 3.43 NA
NA NA NA NA Primary pupillaryblockNA NA NA NA
26.04 2.96 4.77 NA Primary pupillaryblockNA NA NA NA
24.84 2.50 4.88 49.00/57.75�124 Pupillary block,keratoconus24.99 2.97 4.96 51.50/55.75�75
NA NA NA NA Pupillarymembrane,retinopathy ofprematurity
NA NA NA NA
25.43 NA NA 44.25/44.50�69 Plateau irisconfigurationand iris cyst
26.17 3.25 4.39 44.62/45.00�68
27.22 NA NA 44.75/43.50�115 Plateau irisconfiguration26.78 2.63 5.24 44.00/43.00�73
22.45 2.97 5.14 46.25 Plateau irissyndrome22.49 2.39 5.19 46.62
20.25 2.2 4.54 NA Weill-Marchesanisyndrome20.35 2.2 4.4 NA
19.76 NA 4.6 45.75/52.25�80 Weill-Marchesanisyndrome19.99 NA 4.35 45.00/52.00�100
NA NA NA NA Malignantglaucomasecondary toscleral buckle
NA NA NA NA
27.07 NA NA 42.25/41.25�99 Malignantglaucomasecondary toscleral buckle
27.76 NA NA 39.50/42.25�63
25.81 3.25 4.23 NA Miotic-inducedangle closure26.49 2.44 5.32 NA
24.57 2.81 4.51 40.6/41.75�41 Marfan syndrome23.86 2.71 4.55 40.5/41.62�110
lable; NLP � no light perception.
o 20/40 after resolution of the acute attack and corneal edema.
a, anMy
dl L
t avai
ye.
the slit beam, apposition of the iris to the upper TM was
Barkana et al � Angle Closure in Highly Myopic Eyes
visualized. Laser iridotomy resulted in open angles withoutapposition.
Patient 4. A 57-year-old Chinese woman had absolute glau-coma in the right eye and advanced optic neuropathy in the left atpresentation. The irides were convex, with 360° apposition toscleral spur on the right and grade II open angle with extensivePAS superiorly on the left. There was a patent LI on the left.Because of high IOP on maximally tolerated medical therapy,trabeculectomy with 5-fluorouracil was performed on the left eye.
Patient 5. A 74-year-old white woman with a history of righteye amblyopia had been treated for glaucoma with medicationsand laser trabeculoplasty. Her right angle was slit with areas ofapposition to TM. The left angle was wide open. Lenses wereclear. After LI on the right, the angle widened to grade III. Tenyears subsequently, there was 3� nuclear sclerosis in both eyes.The right angle was wide but the left had narrowed with iridocor-neal apposition. Laser iridotomy was performed in the left eye withconsequent widening of the angle.
Patient 6. A 57-year-old white woman had had acute ACG inthe left eye treated with LI and acute ACG in the right eye 1 dayafter hysterectomy. The right angle was appositionally closed andthe left angle was open to grade II. Laser iridotomy and argon laserperipheral iridoplasty (ALPI) resulted in lowering of IOP andwidening of the right angle.
Patient 7. A 67-year-old white man had ocular hypertensionand high myopia at presentation. On gonioscopy, both angles wereopen slit superiorly and grade I inferiorly. Ultrasound biomicros-copy showed similar findings, with narrowing superiorly and ap-position inferiorly in dark. There was 2� nuclear sclerosis with noexfoliative material in both eyes. Laser iridotomy resulted inuniformly grade II open angles in both eyes, with no change during18 months of follow-up.
Patient 8. A 71-year-old white man had moderately deepanterior chambers and slit to grade I lightly pigmented angles.Laser iridotomy resulted in grade II open angles in both eyes, withno change during 15 months of follow-up.
Patient 9. A 52-year-old white woman with a history of sys-temic lupus erythematosus and uveitis had narrow angles in bothlight and dark without occlusion demonstrated by UBM at presen-tation. Twelve months later, UBM demonstrated no change in theright eye but extensive iridotrabecular contact on the left, and LIwas performed. Four months later, the angle was uniformly open
Figure 1. Ultrasound biomicroscopy images of patient 12. A, Closed supflat contour of the iris. Arrow, scleral spur; arrowhead, iridocorneal contaccontact anterior to the scleral spur.
to grade III.
Case Reports: Diagnoses Other than PrimaryPupillary Block
Patient 10. A 71-year-old white woman with keratoconus had anarrow left angle, grade I-II superiorly and slit inferiorly, with aconvex iris. The right angle was wide open. Ultrasound biomicros-copy corroborated these findings. There was 2� nuclear sclerosisin both eyes. After LI on the left, the angle was wide open. She wastreated with IOP-lowering drops for glaucomatous neuropathy,which was more advanced in the left eye.
Patient 11. A 7-year-old Indian girl with retinopathy of pre-maturity (ROP) had a pupillary membrane in the right eye withappositional angle closure. Laser iridotomy and ALPI were per-formed, with opening of the angle to grade II and decrease in IOP.On follow-up examination 3 years later, the iridotomy was notpatent and the angle was slit open.
Patient 12. A 58-year-old white man with progressive anglenarrowing in the left eye had a focal iris cyst on UBM with plateauiris configuration elsewhere in the angle (Fig 1). Laser iridotomyresulted in slight widening of the angle. Two years later, the angleremained open but medical therapy was required for elevated IOP.
Patient 13. A 53-year-old white woman with a history ofelevated IOP had slit to grade I angles with a double-hump signcharacteristic of plateau iris in both eyes, without apposition.Ultrasound biomicroscopy of the left eye in room light revealed anarrow angle, which in the dark became slit inferiorly and slit-to-closed superiorly (Fig 2). There has been no sign of progressiveangle closure during further follow-up of 14 months.
Patient 14. A 60-year-old white woman had had acute ACG, LI,selective laser trabeculoplasty, and documented emmetropia 10 yearspreviously in both eyes. At the time of consultation, high myopia waspresent in the right eye and low myopia in the left. Her IOP was 30mmHg in both eyes. There was a deep chamber, patent iridotomy, andappositional closure to the top of the TM 270° with scattered PAS inboth eyes. Ultrasound biomicroscopy confirmed the presence of angleclosure and a plateau iris configuration. There was 3� nuclear scle-rosis in the right and 1� in the left. She was offered cataract extrac-tion in the right eye and ALPI in the left.
Patient 15. A 31-year-old white man with Weill-Marchesanisyndrome had appositional angle closure in both eyes at presen-tation, despite having a patent LI in both eyes and having under-gone ALPI in the right eye. Repeat ALPI resulted in a wide angle
ngle and plateau iris configuration with thick ciliary body (asterisk) andrior to scleral spur. B, Iridociliary cysts (white arrow) causing iridocorneal
erior at ante
in the right eye. Subsequently, acute ACG developed in the left eye
249
in da
Ophthalmology Volume 113, Number 2, February 2006
and was treated successfully with ALPI. Nine years later, ALPIwas repeated because of focal apposition in both eyes.
Patient 16. A 28-year-old white woman, the sister of patient15, had Weill-Marchesani syndrome and appositionally closedangles with patent LI in both eyes at presentation. Argon laserperipheral iridoplasty was performed. Examination 7 years latershowed elevated IOP and alternating areas of synechial and appo-sitional closure with a patent iridotomy in the right eye, and ALPIwas repeated.
Patient 17. A 75-year-old white woman had had a retinaldetachment repair with scleral buckle 9 months previously withconsequent elevated IOP in the right eye. On initial consultation,treated IOP was 48 mmHg. There were extensive PAS 360° in theright eye, while the angle was wide open on the left. Antiglaucomatherapy was unsuccessful and she underwent surgery.
Patient 18. A 72-year-old white man who had undergonescleral buckling for retinal detachment and subsequent LI fornarrow angles had a patent iridotomy and PAS to the posterior TMand a volcano crater-type iris configuration along the lens contourin both eyes at presentation. There was 2� nuclear sclerosis inboth eyes. A diagnosis of chronic angle closure was made, and thepatient was offered combined cataract extraction and trabeculec-tomy surgery in the left eye.
Patient 19. A 28-year-old white man had a history of glau-coma and anisometropic amblyopia in the left eye. Examination
Figure 2. Ultrasound biomicroscopy images of the light and dark provocascleral spur). B, Iridocorneal contact (arrowhead) anterior to scleral spur
Figure 3. Ultrasound biomicroscopy images of patient 20. A, Closed supe
arrow). B, Atypical long and fingerlike ciliary processes (white arrow).250
showed a wide right angle and an appositionally closed left angle.Discontinuation of miotics led to angle widening but also IOPelevation; consequently, continued miotic use and LI wererecommended.
Patient 20. A 23-year-old marfanoid Indian woman with ahistory of glaucoma had deep anterior chambers, iridodonesis andphacodonesis, and scattered PAS with some areas of indentableappositional angle closure in both eyes. Corneal diameters were13.25 mm in the right eye and 13.0 mm in the left eye. Ultrasoundbiomicroscopy of the right eye showed long fingerlike ciliaryprocesses with narrowing of the angle (Fig 3).
Discussion
We have described the diverse clinical features of 20 pa-tients with high myopia and various types of angle closure.Review of the literature has revealed almost no mention ofsuch patients. Lowe2 reported on 127 eyes of patients diag-nosed with primary ACG. Only 2 eyes had myopia of morethan �2 D (�6.25 to �8.0). Their clinical characteristicswere not mentioned. Bonomi et al1 screened 4297 patientsfor angle closure according to the Van Herick method.Among 117 patients (2.8%) with grade 0 or 1, termed
est of patient 13. A, Narrow superior angle in room light (arrow showingrk demonstrating occludable angle.
ngle with iridocorneal contact (arrowhead) anterior to scleral spur (black
tive t
rior a
Ophth
Barkana et al � Angle Closure in Highly Myopic Eyes
high-risk occludable angles, there was only 1 patient withcataract and lenticular high myopia (�7 D). The authorsnoted that he had “nuclear cataract with a major indexmyopia.” In a series reporting refraction in patients withangle-closure glaucoma, none of the 54 patients studied hadhigh myopia.7 Gazzard et al8 prospectively recruited for asurgical trial patients with primary angle closure and glau-comatous optic neuropathy who were candidates for glau-coma filtering surgery. During the enrollment process, only2 eyes had myopic spherical equivalent of more than �5.0D (axial lengths, 26.1 mm and 23.6 mm; Gazzard G, per-sonal communication, 2005). We previously included 4 ofthe presently described patients in our analysis of angleclosure in young patients (patients 6, 7, 8, and 13).5 Haganand Lederer3,4 detailed the clinical course of 1 patient whoinitially was reported to have primary ACG, but subse-quently was observed to have lens subluxation and wasdiagnosed, together with other family members who did nothave high myopia, with genetic spontaneous late subluxa-tion of the lens. Two adults who had ROP and in whom highmyopia and angle closure developed were included in theseries by Michael et al.6
When attempting to explain angle closure in these atyp-ical patients, one should have in mind a system that facili-tates the inclusion and understanding of the various mech-anisms involved in iridocorneal apposition. We previouslydescribed such a classification based on the anatomical level
Table 2. Published Biometric Parameters in Patients with Angl
Source
Axial Length (mm)
ACG Normal
Sixty-one patients (118 eyes) withprimary ACG, 80 (157 eyes)age- and sex-matched normalcontrols*
22.01 � 1.06(18.5–26.0)
23.1 � 0.82(20.7–25.3)
Thirty-two white patients withprimary acute/intermittentACG†
22.31 � 0.83(22.02–22.60)
23.38 � 1.2(23.01–23.75
Twenty-two white patients withprimary chronic ACG†
22.27 � 0.94(21.88–22.66)
23.38 � 1.2(23.01–23.75
Forty-three consecutive south-eastAsian patients with chronicACG‡
22.4 � 1.0
Eighty-four contralateral eyes ofChinese patients with acuteAC§
21.856 23.188
Sixteen patients with ACG, 49normal controls matched forage, sex and refractive error�
22.06 22.58
AC � angle closure; ACG � angle-closure glaucoma.Mean values are given and, when available, standard deviation and rang*Bonomi L, Marchini G, Marraffa M, et al. Epidemiology of angle-closurechamber depth in the Egna-Neumarkt Glaucoma Study. Ophthalmology†Lowe RF. Aetiology of the anatomical basis for primary angle-closure glangle-closure glaucoma. Br J Ophthalmol 1970;54:161–9.‡Hagan JC III, Lederer CM Jr. Primary angle closure glaucoma in a myop§Hagan JC III, Lederer CM Jr. Genetic spontaneous late subluxation ofglaucoma. Arch Ophthalmol 1992;110:1199–200.�Ritch R, Chang BM, Liebmann JM. Angle closure in younger patients.
at which anteriorly directed forces act to change the angle
configuration: the level of the iris, ciliary body, lens, orposterior segment.9–11 Diagnosis of the correct mechanismpermits treatment to be directed at the underlying source ofpathophysiology. It should be remembered that variousmechanisms for angle closure may coexist in the same eye.
Angle closure at the level of the iris is caused by pupil-lary block, which is the most common mechanism in pa-tients with angle closure. The typical eye with primaryrelative pupillary block has a hyperopic refractive error,shorter-than-average axial length, larger-than-average lensthickness, and a smaller-than-average anterior chamberdepth (Table 2).2,8,12–14 Continued growth of the lens duringadulthood results in progressively increased lens thickness,forward movement of the anterior lens surface, and decreasein anterior chamber depth and volume.15 As a result, angleclosure caused by relative pupillary block is a disease ofmiddle-aged and older individuals. Laser iridotomy pro-vides the definitive treatment and results in an open anglewhen pupillary block is the dominant mechanism.
Although primary pupillary block was the most commonpresumed mechanism for angle closure in this series, itaccounted for less than half of patients, much less than inthe general angle-closure population. In patients 2, 3, 5, 6,7, 8, and 9, the angle widened after LI. We did not performLI in patients 1 and 4, but their gonioscopic appearance wastypical of pupillary block. In all of these patients, signs ofexfoliation syndrome were not identified on biomicroscopy.
sure and, When Available, Comparison with Normal Controls
Anterior Chamber Depth(mm) Lens Thickness (mm)
ACG Normal ACG Normal
1.8 � 0.25(1.1–2.4)
2.8 � 0.36(2.1–3.6)
5.09 � 0.63(4.4–6.2)
4.50 � 0.34(3.7–5.4)
2.41 � 0.25(2.32–2.50)
3.33 � 0.31(3.24–3.42)
5.10 � 0.33(4.99–5.21)
4.60 � 0.53(4.44–4.76)
2.77 � 0.31(2.64–2.90)
3.33 � 0.31(3.24–3.42)
4.92 � 0.27(4.81–5.03)
4.60 � 0.53(4.44–4.76)
2.3 � 0.4 5.1 � 0.5
1.687 2.585 5.224 4.763
5.23 4.67
coma: prevalence, clinical types, and association with peripheral anterior;107:998–1003.
a. Biometrical comparisons between normal eyes and eyes with primary
nship. Arch Ophthalmol 1985;103:363–5.ens previously reported as a myopic kinship with primary angle closure
almology 2003;110:1880–9.
e Clo
3)
3)
e.glau
2000aucom
ic kithe l
This is significant because this syndrome may be associated
251
Ophthalmology Volume 113, Number 2, February 2006
with loose zonules that lead to a more mobile lens that canreposition anteriorly and can exacerbate pupillary block. Ithas been noted that patients with exfoliation syndrome havea higher prevalence of primary ACG.16,17
In many of these patients, progressive cataract conceiv-ably led to worsening pupillary block. Progressive cataractalso may cause progressive lenticular myopia, and thuspatients with a relatively short axial length who are predis-posed anatomically to angle closure may have significantmyopia at presentation. This occurrence is probably notrare, but the literature is sparse.
Patient 6 had very thick lenses that probably led topupillary block and angle closure, despite relatively longaxial lengths and relatively deep anterior chambers (com-pare with Table 2). High myopia in the contralateral eye ofa patient with acute angle closure does not exclude angleclosure, and the decision regarding prophylactic iridotomyshould be based on careful gonioscopic evaluation.
Patient 10 had keratoconus in both eyes, with a narrowangle only in 1 eye that widened after LI. The high myopiain this patient is attributable to the abnormally steep cornea.
Secondary pupillary block was the main contributingmechanism in patient 11, who had a pupillary membraneassociated with ROP. Although angle-closure glaucoma isextremely rare in children, it can be found infrequently withROP, which is commonly associated with high myo-pia.6,18–22 Two patients with ROP and high myopia werereported previously with late-onset ACG.6 Both weretreated with LI, with IOP subsequently controlled medicallyin 1 patient and the other patient undergoing trabeculec-tomy. It is extremely important to diagnose and treat angleclosure in patients with ROP, who need frequent pupildilation during routine follow-up.
Angle closure at the level of ciliary body is caused byplateau iris. This denotes an angle appearance in which theiris root angulates forward and then centrally. Primary pla-teau iris is associated with a large or anteriorly positionedciliary body that physically supports the iris root against theTM. Patients with plateau iris tend to be female, younger,and less hyperopic than those with relative pupillary blockand often have a family history of angle-closure glaucoma.However, high axial myopia is rare and has not been re-ported previously in a patient with plateau iris. Secondary,or pseudoplateau, iris is caused by lesions such as iris orciliary body cysts. Because some element of pupillary blockusually is present in these patients, iridotomy may result inan open angle. Periodic gonioscopy is indicated because theangle can narrow further with age with enlargement of thelens. Continued angle closure after iridotomy should betreated with argon laser peripheral iridoplasty.23
Patients 12, 13, and 14 had plateau iris configurationon gonioscopy and UBM. In patient 14, the biometricparameters of both eyes were similar and compatible withpupillary-block angle closure. The angles, however, wereappositionally closed despite patent iridotomies. There-fore, additional mechanisms must have contributed. Irisplateau was demonstrated on UBM, which we believewas the main mechanism for angle closure. Progressivenuclear sclerosis clearly was responsible for the high
myopia in the right eye and may have contributed a252
component of lens-induced angle closure. The case ofthis patient illustrates again that myopic refraction doesnot exclude the presence of angle closure and that thisshould be sought by careful gonioscopy.
Patient 12 had a complicated picture that ultimatelyresulted in chronic ACG. He had plateau iris configurationin the left eye. Laser iridotomy resulted in slight wideningof the angle, indicating some component of pupillary blockmechanism. The iridociliary cyst and possibly progressivecataract played additional roles.
Lens-related angle closure occurs when abnormalities insize or position of the lens lead to the lens pushing the irisforward, mechanically narrowing the angle. It is importantto realize that in cases that involve an enlarged or anteriorlydisplaced lens, a component of pupillary block also may bepresent and that the relative contribution of the 2 mecha-nisms forms a spectrum that needs to be assessed in astepwise fashion. Lens-induced angle closure may worsenwith miotic therapy. Miotics relax the zonules, causing thelens to become more spherical and the lens–iris diaphragmto move anteriorly.24,25
Patients 15 and 16 had Weill-Marchesani syndrome, withangle closure despite patent iridotomies. Ocular involve-ment in this syndrome is associated with anterior subluxa-tion of the microspheric lens resulting from increased zonu-lar laxity. The lenticular abnormality also causes highmyopia. Spontaneous and miotic-induced angle closure inthese patients traditionally has been attributed to pupillaryblock mechanism.26,27 However in some patients withWeill-Marchesani, as in our patients, angle closure maypersist in the presence of a patent iridotomy because of amechanical phacomorphic mechanism. Two siblings withWeill-Marchesani syndrome, high myopia, and angle clo-sure previously were described.28 Acute rise in IOP afterinstillation of pilocarpine also has been reported.29,30
Malignant glaucoma is a multifactorial disorder in whicha pressure differential is created between the posterior andanterior segments. In one form of this disorder, anteriorrotation of the ciliary body is present, with forward rotationof the lens–iris diaphragm and angle closure. Patients 17and 18 had chronic ACG after previously undergoing ascleral-buckling procedure. Angle closure has been notedafter scleral buckling.31–33 It usually is not associated withpupillary block.31 It has been proposed that when pupillaryblock is absent, the underlying mechanism is detachment oredema of the ciliary body, or both, resulting in anteriordisplacement and angle closure.32,33 If this condition isdiagnosed in the immediate postoperative period, PAS andchronic angle closure can be prevented with ALPI.33 In ourpatients, the buckling procedure may have been responsible,at least in part, for both axial myopia and non–pupillary-block angle closure.
Angle closure developed in patient 19 in response to theuse of miotics. Pilocarpine previously was used successfullyand was necessary to reduce IOP. Careful repeated gonios-copy throughout his management revealed its effect on theangle, which led us to recommend a peripheral iridotomywith continued miotic use.
Patient 20 had musculoskeletal features of Marfan syn-
drome. Her flat corneas also are consistent with Marfan
Barkana et al � Angle Closure in Highly Myopic Eyes
syndrome.34,35 The biometric measurements and the lack ofmyopic funduscopic changes suggest that her very highmyopia was mainly lenticular rather than axial. In Marfansyndrome, defective zonules lead to increased curvature andanteroposterior lens thickness,36 especially in younger pa-tients in whom the lens is more pliable, and, together withanterior forward movement of the lens, lenticular myopiaresults. Zonular weakness was evident in this patient byiridophacodonesis and anterior movement of the lens ondilation.
It may be expected that these very same lens changesmay bring about pupillary block and consequent angleclosure. The gonioscopic features in our patient wereconsistent with chronic angle closure. However, angleclosure is very uncommon in patients with Marfan syn-drome, and chronic angle closure has not been describedin the literature even in 1 patient. Maumenee35 reportedon 186 patients with Marfan syndrome. The angle wasusually wide open to grade IV. Glaucoma was present inonly 5 eyes and was associated with open angles in allpatients. Cross and Jensen37 diagnosed glaucoma in 14 of181 patients with Marfan syndrome (7.9%); angle-clo-sure was not described in any of these patients. Izquierdoet al38 reported on 573 patients with a definitive diagnosisof Marfan syndrome over the course of 20 years. Thirteenpatients had primary open-angle glaucoma, and 2 patientshad acute angle closure. The authors commented that arelative pupillary block mechanism, which would be ex-pected because of increased lens thickness and curvatureinduced by defective zonules, did not lead to chronicangle closure in any of their patients.
In our patient, we observed an unusual appearance of theciliary processes. Whether this is a consistent feature inMarfan syndrome remains to be elucidated. Previously,UBM has demonstrated loose zonules in patients withMarfan syndrome, but details of ciliary body anatomy havenot been reported.36
In summary, we have described 20 patients with a spec-trum of ophthalmic conditions leading to high myopia andangle closure. Primary relative pupillary block, which is thecause for angle closure in the large majority of patients inthe general population, was identified in less than half of thepatients. Because angle closure in highly myopic patients isunusual and because gonioscopy in these patients may notbe performed routinely, the clinician must maintain a highindex of suspicion. We advocate performing careful darkroom gonioscopy on all patients undergoing initial exami-nation. Biometric evaluation and UBM may be used todiagnose the underlying mechanism in these patients. Fur-thermore, periodic gonioscopy is needed to detect furtherangle closure requiring iridotomy or iridoplasty to preventprogressive trabecular meshwork dysfunction, PAS forma-tion, and chronic ACG.
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