Dr. Sanket Deshmukh, D16977, Dr. Sirisha
Senthil
Synopsis:
Lensectomy was effective in controlling intraocular pressure (IOP) in half of all spherophakic eyes with subluxated or dislocated crystalline lens and glaucoma. Younger age (< 6 years), IOP > 32 mm Hg and higher cup-to-disc ratio predicted need for longterm anti-glaucoma medication (AGM) or glaucoma surgery.
Abstract:
Purpose: To report the outcomes of lensectomy in spherophakic eyes with subluxated or dislocated crystalline lenses and secondary glaucoma.
Methods: Lensectomy was performed in 52 eyes, 36 eyes with lens subluxation and 16 eyes with lens dislocation with secondary glaucoma from1991 to 2016. Glaucoma was diagnosed based on intraocular pressure (IOP) ≥ 22 mm Hg and/or glaucomatous optic disc damage. Complete success was defined as IOP ≥5 and ≤21 mmHg without anti-glaucoma medications (AGM) or surgery, eyes needing oral AGM or surgical intervention for IOP control or those with complication causing loss of light perception were considered failure.
Results: Median (interquartile range) age at lensectomy was 12 (6,18) years and median spherical equivalent was -14.5 (-23.7,-13) diopters. Median follow up was 30.6 (5.4, 103.4) months. Median Log MAR visual acuity improved from 0.95 (0.6, 1.8) to 0.4 (0.2, 1.3) after lensectomy (p=0.01). Median IOP decreased from 22 mmHg (17, 31) to 14 mmHg (11,19) at final follow up (P=0.01). Median number of AGM decreased from 2 (2,3) to 1 (0,2) at final follow up (p<0.0001) and glaucoma surgery was needed in 4 eyes(7.7% eyes). Complete success probability was 69% at 1 year, 51% at 5 years. Younger age (<6 years), higher presenting IOP (>32 mm Hg) and larger cup to disc ratio at presentation were found to be significant risk factors for failure.
Conclusion: Lensectomy was effective in controlling IOP in close to half of all eyes with spherophakia and secondary glaucoma, 40% eyes needed AGM and only7.7% eyes needed glaucoma surgery for IOP control. In this cohort, younger age, higher IOP and larger cup to disc ratio at presentation were risk factors for poor glaucoma control after lensectomy.
Introduction: Spherophakia is a rare, often bilateral condition, in which the crystalline lens assumes a spherical shape due to abnormally lax zonules. Spherophakic lenses have reduced equatorial diameter and increased anteroposterior diameter causing high lenticular myopia.[1, 2] The increased anteroposterior diameter of the lens and its anterior displacement due to weak zonules predisposes these eyes to acute pupillary block or chronic angle closure glaucoma. The triad of angle-closure glaucoma (ACG), shallow anterior chamber, and refractive myopia in a young patient are described as hallmarks of this condition.[3, 4]The most important cause of permanent visual loss in spherophakia is glaucoma,[2]angle closure being the commonest cause.[3-7] Weak zonules in these eyes often lead to lens subluxation or dislocation resulting in complications like elevated intraocular pressure (IOP), corneal decompensation and rarely retinal detachment.[2]Although subluxation or dislocation of the lens causing acute or chronic angle closure is the primary cause of elevated IOP, open angles with developmental angle anomaly also contributes to decrease in aqueous outflow and elevated IOP.[8, 9]Management options for subluxated or dislocated lenses with elevated IOP include, lens removal alone or combining lens extraction with glaucoma surgery.[7-17] Controversy exists on the role of lensectomy alone in lowering the IOP in spherophakic eyes. Some studies have reported no benefit, few case reports have shown IOP control in the early postoperative period, however needing additional medications or surgery in the longterm. Muralidhar and Yasar et al reported no benefit on IOP reduction following lensectomy,[10, 17] on the contrary, lens extraction with or without intraocular lens implantation was found to be effective in decreasing the IOP in few studies with short follow up.[7, 9 14-16] However, Yang J etal, have reported longterm IOP elevation needing additional AGM or glaucoma surgery to control the IOP.[14]There are no studies that have looked at the longterm outcomes and risk factors for predicting longterm failure in eyes with subluxated or dislocated lenses in eyes with spherophakia and secondary glaucoma.
Methods: We retrospectively reviewed records of consecutive patients who underwent lensectomy for subluxated or dislocated lens in eyes with spherophakia and secondary glaucoma over a 25-year period (1991-2016). The study was approved by the Institutional review board of L V Prasad Eye Institute and adhered to the tenets of Declaration of Helsinki. Informed consent was taken from the parents of all subjects prior to the intervention All patients diagnosed with spherophakia and secondary glaucoma that underwent pars plana lensectomy or lensectomy through a limbal route were included. Patients with prior filtering surgery or those that underwent combined glaucoma filtration surgery with lensectomy or scleral fixated IOL (SFIOL) or glued IOL along with lensectomy were excluded. Eyes that underwent lens aspiration or cataract extraction with in-the-bag IOL placement were also excluded.
The variables collected included age, gender, presenting complaint, associated systemic conditions, pre and postoperative best corrected visual acuity (BCVA), pre and postoperative refraction, IOP at presentation (IOP was measured either with Goldmann applanation tonometer or Perkins tonometer), pre and post-operative IOP, number of pre and post-operative antiglaucoma medications used, laser peripheral iridotomy, lens status (subluxation / dislocation) at presentation, gonioscopy, cup-to-disc ratio, indication for lensectomy, technique of lensectomy, complications of the procedure or any other surgical intervention and duration of follow up.
Spherophakia was diagnosed clinically based on the spherical shape of the lens on slit lamp and the visibility of the lens equator on pharmacological mydriasis (or with indirect ophthalmoscopy when posteriorly dislocated), with high refractive myopia (when possible). For the purpose of the study, glaucoma was defined based on one or more of the following criteria: 1. IOP ≥22 mmHg, or IOP ≤22 mmHg on antiglaucoma medication 2.glaucomatous optic disc damage. Elevated IOP ≥22 mm Hg in the absence of disc damage is ocular hypertension, however in eyes with secondary glaucoma, we often refer to this as glaucoma, hence we have combined ocular hypertension and glaucoma and would refer to them as glaucoma in the manuscript.
Surgical Technique: Lensectomy was performed by parsplana approach or limbal route. In the pars plana approach, two/three sclerotomies were performed, 3 mm from limbus (or age appropriate distance) in the infero-temporal, supero-temporal, and supero-nasal quadrants. Infusion cannula was fixed in the infero-temporal quadrant. Complete lensectomy was performed using a vitreous cutter making sure the capsular remnants were removed. Limited anterior vitrectomy was also performed upto anterior one third of vitreous cavity. At the end of the surgery, peripheral retinal examination was performed to rule out any inadvertent retinal breaks. The sclerotomies were closed with 8-0 vicryl suture. In the recent times, the PPV and lensectomy is performed with 23/ 25-gauge surgery after technological advancements. In the limbal approach, two paracentesis were performed at 2’0 or 10’0 clock position for the vitrectomy probe, and at 6’0 clock position for the anterior chamber maintainer. A complete lensectomy was performed with limited anterior vitrectomy. All surgical sites were closed with 10-0 nylon sutures. The sutures were removed after 1-6 weeks in most cases. The limbal route or pars plana route for lensectomy was based on the position of the subluxated or dislocated lens, the status of the cornea and the surgeons preference.
Complete success was defined as IOP ≤21 and ≥5 mmHg without the need for anti-glaucoma medication. Qualified success was defined as IOP ≤21 and ≥5 mm Hg with topical anti-glaucoma medications. The eyes with medically uncontrolled IOP ≥22 mmHg requiring oral anti-glaucoma medication to achieve IOP control or those requiring additional glaucoma procedures to lower IOP <21 mm Hg or a complication resulting in loss of light perception were labeled as failure.
Statistical analysis: Descriptive statistics included mean and standard deviation (SD) for normally distributed variables and median and inter-quartile range (IQR) for non-normally distributed variables. Kaplan-Meier survival curves were used to assess cumulative probability of success. A multivariate Cox-regression model with stepwise elimination using Akaike information criteria (AIC) was used to assess the risk factors for failure. A P ≤ 0.05 was considered statistically significant. Statistical analysis was performed using commercial software (Stata ver. 12.0; StataCorp, College Station, Tx and R software).
Results:
Demographic, preoperative and postoperative parameters: (table 1) There were 21 male (58.3%) and 15 female (41.6%) subjects. Systemic associations were noted in 13 subjects, 11 had Weil Marchesani syndrome, 1 each had Marfans Syndrome and Homocystinemia. The most common presenting complaint was decrease in vision in 23 subjects, 6 subjects presented with painful diminution of vision, 2 subjects had photophobia, 3 came for discomfort in one eye, 1 had family history of glaucoma and came for routine eye examination.
Preoperative refractive error data (n=32) revealed a median spherical equivalent of -14.5 Diopters (D) (IQR: -13, -23.75; range:) -4.5D to -39.25D). The median Log MAR (Logarithm of minimal angle of resolution) best corrected visual acuity at presentation was 0.95 (IQR: 0.6, 1.8). The median IOP at presentation (n=50) was 26 mm of Hg (IQR: 20, 37); range: 10 to 63 mm of Hg), in 2 eyes with severe corneal edema with anterior lens subluxation, digital IOP was recorded high. Pre-operatively, 48 of the 52 eyes were on topical anti-glaucoma medication (AGM), 5 eyes were on 4 AGM, 13 eyes were on 3 AGM, 23 eyes were on 2 AGM, 7 eyes were on 1 AGM. Median number of preoperative AGM was 2 (2,3). Median pre-operative IOP (n=48 eyes) before undergoing lensectomy was 22 mmHg (IQR 17, 31). Gonioscopy showed closed angles in 19 eyes and open angles in 10 eyes and in 23 eyes gonioscopy was not possible or was not available. Eight eyes had undergone prior laser peripheral iridotomy. Crystalline lens was subluxated in 37 eyes, anteriorly dislocated in 12 eyes and posteriorly dislocated in 3 eyes. The median cup to disc ratio (n= 44 eyes) was 0.6 (IQR 0.35, 0.8). Glaucomatous disc damage was present in 23 eyes, twenty-one eyes had normal disc with IOP >21 mm Hg and the remaining 8 eyes had elevated IOP but disc details were not available.
Table 1: Demographic, preoperativeand postoperative clinical details of eyes with spherophakia and glaucoma that underwent lensectomy (n= 52 eyes of 36 subjects)
Parameters | Preoperative Median (IQR) | Postoperative Median (IQR) | P value |
Age (years) | 12 (6, 18) | ||
Gender (M:F) | 21: 15 | ||
SE in Diopters (n=32) | -14.5 (-23.75, -13) | 10.6 (9.4,13) | |
VA by Log MAR (n=42) | 0.95 (0.6, 1.8) | 0.4 (0.2, 1.3) | <0.001 |
IOP (mmHg) (n=50) | 26 (20, 37) | 14 (11, 19) | <0.001 |
Median AGM (n=48) | 2 (2,3) | 1 (0,2) | <0.001 |
Cup-disc ratio (n=44) | 0.6 (0.35, 0.8) | ||
Median follow up(months) | 30.6 (5.5, 103) |
n= number, SE: spherical equivalent, VA: visual acuity, LogMAR= Logarithm of minimal angle of resolution, M=male, F=female, IOP= intraocular pressure, AGM=antiglaucoma medications.
Lensectomy:The median age (interquartile range) at lensectomy was 12 years (IQR: 6-18 years). Bilateral lensectomy was performed in16 subjects. Five eyes underwent lensectomy by limbal route and 47 eyes underwent lensectomy by parsplana route.
Preoperative and postoperative parameters are shown in Table 1. Median follow up after lensectomy was 30.6 months (5.5, 103). Median postoperative LogMAR visual acuity at last follow up (n=49 eyes) improved from 0.95 (0.6, 1.8) at presentation to 0.4 (IQR 0.2- 1.3) (p 0.001). Post-operatively median spherical equivalent (n=48) was 10.6D (IQR 9.4,13 D). The median preoperative IOP was 26 (20,37) mm Hg. This reduced significantly at final follow up IOP to 14 mmHg (IQR 11,19; p=0.001). Median number of AGM at last follow up was 1 (0,2), which was significantly less compared to the preoperative AGM of 2 (2,3), (p<0.001). Following lensectomy 23 eyes (44.2%) had IOP control without any AGM, additional 21 eyes requiring 1 or more AGM (40.4%) for IOP control. It is interesting to note that 14 of the 21 (66.6%) eyes that needed AGM did so within 3 months after lensectomy. Failure was seen in 8 eyes (15.4%; 95% CI: 6.9-28.1); due to uncontrolled IOP in 7 eyes and posterior segment complication with decrease in vision in 1 eye. Of the 8 eyes that failed, 4 eyes underwent glaucoma surgery for IOP control, 3 eyes were controlled with oral anti-glaucoma medications and 1 eye developed RD with no perception of light with nil visual improvement despite intervention.
The cumulative complete success probability of lensectomy in eyes with spherophakia and glaucoma by Kaplan-Meier analysis was 71% at 3 months, (number at risk ‘n’=32) 69% at 1 year (n=28) and 50.9% at 5 years (n=18) (Figure 1). The cumulative qualified success probability was 100% at 1 year (n=34), which was maintained until 3 years (n=28) and was 88% at 5 years (n=25) (Figure 2).
Interesting to note that, among the 15 eyes, which had dislocated lenses either anterior or posterior, 12 had complete success, 2 had qualified success and only one eye had failure.
Post lensectomy complications included choroidal detachment (CD) in 3 eyes and all responded to intensive topical steroids and topical cycloplegics, and retinal detachment (RD) in one eye. The eye with RD underwent belt buckle with pars plana vitrectomy and silicone oil injection. The final visual acuity of this eye was light perception despite attached retina.
Risk factors for predicting need for AGM or glaucoma surgery: A multivariate Cox-regression model with stepwise elimination using Akaike information criteria (AIC) was used to assess the effect of preoperative characteristics on the risk of failure of lensectomy for glaucoma control. The factors considered in the model were age, sex, BCVA at presentation, preoperative IOP, cup to disc ratio at presentation, subluxation or dislocation of lens, preoperative number of AGM, angle status and presence of PI.
The final model retained age, preoperative IOP, cup to disc ratio and number of AGM as the risk factors (Table 2). Age was divided into 3 clusters by quantile distribution. Cluster A ranged from 2-6 years of age, cluster B ranged from 7-18 years of age and cluster C ranged >=19 years age at the time of surgery. Younger age had high risk for postoperative elevated IOP compared to age older than 6 years (older age was protective, 7-18 years 1/0.13=7.6 times and >19 years=1/0.12=8.33 times). By K-means clustering technique, the preoperative IOP was divided into 3 groups. Group A: IOP less than 19 mm Hg, group B: IOP 20-32 mm Hg and Group C: IOP> 32 mm Hg. IOP > 32 mm Hg at presentation was a significant risk factor with a hazard ratio of 6.27 in univariate model and 4.1 in multivariate model. Higher cup to disc ratio at presentation was also a significant risk factor with 22.2 times greater risk for failure on multivariate model. Higher number of preoperative AGM (>2 AGM) was a risk factor for failure in univariate model but not in multivariate model.
Table 2: Risk factors for failure (increase in IOP or need for glaucoma surgery).
Cox’s proportional hazard model on time to event (risk factors for complete failure) | ||||
Univariate | Multivariate | |||
Crude HR (95%CI) | Crude P value | Adjusted HR
(95%CI) |
P-value | |
*Age:
Reference= Cluster A |
||||
Cluster B | 0.49 (0.2,1.17) | 0.109 | 0.13 (0.03,0.5) | 0.003 |
Cluster C | 0.63 (0.21,1.92) | 0.419 | 0.12 (0.03,0.55) | 0.006 |
#Preoperative IOP:
Reference= Group A |
||||
Group B | 1.04 (0.3,3.57) | 0.947 | 0.38 (0.1,1.5) | 0.169 |
Group C | 6.27 (1.92,20.43) | 0.002 | 4.1 (1.08,15.54) | 0.038 |
CDR (preop) | 1.4 (0.24,8.08) | 0.708 | 22.21(1.68,93.) | 0.019 |
No. of AGM (preop) | 1.72 (1.09,2.72) | 0.02 | 1.62 (0.93,2.8) | 0.086 |
HR: hazard ratio, CI: confidence interval, CDR: cup-to-disc ratio; AGM: anti glaucoma medications, IOP: intraocular pressure, preop: preoperative.
*Preoperative age was divided into 3 clusters by quantile distribution. Cluster A ranged from 2-6 years of age, cluster B ranged from 7-18 years of age and cluster C ranged >=19 years age during surgery.
#By K-means clustering technique, the preoperative IOP was divided into 3 groups. Group A: IOP less than 19 mm Hg, group B: IOP 20-32 mm Hg and Group C: IOP> 32 mm Hg.
Angle status and YAG PI were not associated with failure. Of the 19 eyes with closed angles on gonioscopy, in 12 eyes the IOP was controlled after lensectomy, 4 eyes needed AGM for IOP control and 3 eyes needed glaucoma surgery for IOP control. Of the 10 eyes with open angles, 4 eyes had good IOP control after lensectomy, 5 eyes needed AGM and one eye needed trabeculectomy for IOP control.
Discussion
Glaucoma is the most common blinding complication in spherophakia. In the largest retrospective study by Senthil et al,glaucoma was diagnosed in 51% of the eyes at presentation, majority were young and a large proportion of them were blind due to glaucomatous optic neuropathy.[7] This emphasizes the need to detect and treat glaucoma early and appropriately, to prevent visual impairment and blindness associated with this condition.
Lens position abnormality plays a significant role in causing glaucoma in eyes with spherophakia. Hence removing the lens should play an important role in decreasing the IOP, however, there are conflicting reports in the literature. The current study, to our knowledge is the largest series with 52 eyes and 30.6 month median follow up, reporting outcomes of lensectomy in spherophakic eyes with subluxated or dislocated lenses associated with secondary glaucoma. Our long term follow up results and the risk factor analysis we believe would add new knowledge to the existing literature.
In our study, following lensectomy in eyes with spherophakia and secondary glaucoma, there was significant improvement in visual acuity , IOP control was noted in half of all eyes over 5 years without need for AGM. The number of AGM significantly decreased post lensectomy and close to 40% eyes had IOP control with AGM, only 7.7% eyes needed glaucoma surgery for IOP control.
Glaucoma in spherophakia can be multifactorial.[7] Elevated IOP could be due to acute or chronic angle closure due to lens position abnormality or may have open angles with angle anomaly or dysgenesis.[7] When glaucoma is associated with lens position abnormality like subluxated or dislocated lenses, various surgical modalities have been recommended based on isolated case reports, either by lensectomy, extracapsular cataract extraction (ECCE), phacoemulsification, SFIOL, trabeculectomy, shunt surgeries or a combination of these.[9-20]
There are limited and conflicting reports on the outcome of lensectomy alone in the treatment of glaucoma associated with spherophakia. Willoughby and Wishart reported IOP control with only lensectomy (phacoemulsification) in 2 eyes of a patient with chronic pupillary block and patent PI with 4 to 6 months follow up.[9]Kaushik et al reported a case of bilateral acute angle closure glaucoma wherein following lensectomy, IOP was under control without AGM until 6 weeks.[19] Another report by Tailor et al, showed good IOP control over 2 years in two spherophakic eyes after ECCE and intraocular lens implantation.[20]
In a study by Dagi et al, 8 spherophakic eyes with subluxated lenses, elevated IOP and angle closure, lensectomy was useful to control the IOP and prevent progression to complete angle closure in eyes that were operated early. Two of the cases in their series that underwent delayed lensectomy had persistent elevated IOP even after lensectomy. Hence they concluded that early lensectomy was beneficial for IOP control in spherophakic eyes with subluxated lenses by preventing synechial angle closure.[21]
In our earlier study by Senthil et al, out of the 14 eyes with elevated IOP that underwent lensectomy, 50% eyes had IOP control and the rest needed AGM to control the IOP and none needed glaucoma surgery to control the IOP.[7]Another study by the same group showed that, following trabeculectomy in spherophakic eyes with glaucoma, 45% of the eyes required lensectomy in the postoperative period.[18] It was noted that majority of these eyes that needed lensectomy had subtle subluxation of the lens prior to trabeculectomy. Hence they concluded that in the presence of subluxated lens and high IOP, lensectomy would be a better choice rather than a trabeculectomy.
Few studies have reported lensectomy alone to be ineffective in controlling the IOP in eyes with spherophakia and elevated IOP. In a study by Muralidhar et al, out of the 16 eyes in which lensectomy was performed for dislocated lens, 4 eyes with preoperative elevated IOP continued to have high IOP post lensectomy and all four eyes needed surgical intervention for IOP control. Hence, they concluded that lensectomy was not beneficial in controlling IOP in spherophakic eyes.[10] They also reported that glaucoma surgery was also not successful in controlling the IOP in these eyes and many needed cyclo diode as a second intervention. Possible delayed presentation was responsive for refractory nature in their study, although they have not explained the reason for the refractory nature of the eyes in their cohort.
Transient complications were seen in 3 eyes (CD), which improved with conservative management. Hypotony post surgery could have led to the complication. One eye had retinal detachment, a sight threatening complication. Retinal detachment following parsplana lensectomy and vitrectomy is an uncommon but known complication due to the retinal breaks that develop intraoperatively or in the late post-operative period,[22]in predisposed patients with Marfans syndrome.[23] Our patient had no syndromic association,had subluxated lens and had an uneventful surgery. Although the complication was detected and surgically managed, the visual acuity did not improve.
We found younger age at presentation, higher IOP, more number of AGM (in univariate model) use and greater disc damage at presentation to be significantly associated with higher risk of failure, i.e need for AGM or surgical intervention for IOP control after lensectomy. Closed angles on gonioscopy and need for YAG PI could be a parameter that could be associated with failure of IOP control after lensectomy. However, there was no association between laser PI or angle status with development of glaucoma in our study. This observation is similar to what was noted in a study by Muralidhar et al.[10] In their study, following lensectomy, 2/6 eyes that required trabeculectomy had open angles, trabeculectomy failed and both eyes needed.re-trabeculectomy and ultimately needed Trans-scleral cyclophotocoagulation (TSCPC) for IOP control. This further justifies that observation that although angle closure is the most common presentation, eyes with open angles with possible developmental angle anomaly could be associated with refractory glaucoma in eyes with microspherophakia. In our series, although lensectomy helped decrease the IOP in 4/10 eyes with open angles, the rest needed additional medications or surgery for IOP control strengthening the hypothesis of angle dysgenesis in these eyes. It is possible that the subluxated or dislocated lens were also partly contributing to the rise in IOP which could explain IOP control with lensectomy in some of these eyes.
One of the limitation of our study is its retrospective design with its inherent problems and variable follow up. However, for a rare condition like spherophakia, to evaluate the long term outcomes and risk factors in a uniform cohort of eyes with secondary glaucoma and subluxated or dislocated lenses, a retrospective study is valuable. Another limitation is, incomplete data on gonioscopy for close to 40% of the eyes. Among the 56% eyes where gonioscopy was available, majority of the eyes had closed angles and in the risk factor analysis, the angle status did not turn out to be a risk factor, this could possibly be related to developmental angle anomoly in eyes with open angles that contributed to glaucoma and also due to less sample size. The extent of angle closure was not documented which may have helped us to look for association between extent of angle closure and failure. Not including eyes that underwent any other lens procedure like SFIOL/ Glued IOL or combined surgeries could be a limitation. However, for the uniformity of the cohort and to decrease the variations in the outcomes that would be related to different surgical techniques, we excluded them.
If lensectomy alone can control the IOP in spherophakic eyes with glaucoma and lens position abnormality, trabeculectomy which is associated with serious complications like shallow/ flat AC and its sequelae can be avoided. In our series, after lensectomy, half of all the eyes had well controlled IOP, additional 40% eyes had IOP control with AGM. Sight threatening complication (RD) was noted in only one eye. Based on these results, in spherophakic eyes with subluxated or dislocated lenses and elevated IOP, we recommend lensectomy as the procedure of choice. AGM or glaucoma intervention could be planned if and when needed. Younger children with IOP > 32 mm Hg with higher cup to disc ratio and need for higher number of AGM pre-operatively, predicted need for AGM or glaucoma surgery after lensectomy. Subjects with these risk factors should be counselled about need for additional AGM or glaucoma surgery after lensectomy. Close follow up in the postoperative period is necessary to monitor IOP since majority of those that needed AGM required them within 3 months after lensectomy. The role of early lensectomy in prophylaxis against angle closure glaucoma in these eyes would also need to be evaluated.
Competing interest-none
Research funding: Hyderabad Eye Research Foundation
References:
Macken PL, Pavlin CJ, Tuli R, Trope GE. Ultrasound biomicroscopic features of spherophakia. Aust N Z J Ophthalmol 1995;23(3):217-20
Nelson LB, Maumenee IH. Ectopia lentis. Surv Ophthalmol 1982;27(3):143-60
S D-E. Normal and Abnormal development. St Louis: The CV Mosby Co, 1963.
Chan RT, Collin HB. Microspherophakia. Clin Exp Optom 2002;85(5):294-9
Willi M, Kut L, Cotlier E. Pupillary-block glaucoma in the Marchesani syndrome. Arch Ophthalmol 1973;90(6):504-8
Johnson VP, Grayson M, Christian JC. Dominant microspherophakia. Arch Ophthalmol 1971;85(5):534-7 passim
Senthil S, Rao HL, Hoang NT, et al. Glaucoma in microspherophakia: presenting features and treatment outcomes. J Glaucoma 2014;23(4):262-7 doi: 10.1097/IJG.0b013e3182707437[published Online First: Epub Date]|.
Feiler-Ofry V, Stein R, Godel V. Marchesani’s syndrome and chamber angle anomalies. Am J Ophthalmol 1968;65(6):862-6
Willoughby CE, Wishart PK. Lensectomy in the management of glaucoma in spherophakia. J Cataract Refract Surg 2002;28(6):1061-4
Muralidhar R, Ankush K, Vijayalakshmi P, George VP. Visual outcome and incidence of glaucoma in patients with microspherophakia. Eye (Lond) 2015;29(3):350-5 doi: 10.1038/eye.2014.250[published Online First: Epub Date]|.
Teekhasaenee C, Ritch R. Combined phacoemulsification and goniosynechialysis for uncontrolled chronic angle-closure glaucoma after acute angle-closure glaucoma. Ophthalmology 1999;106(4):669-74; discussion 74-5 doi: 10.1016/s0161-6420(99)90149-5[published Online First: Epub Date]|.
Kanamori A, Nakamura M, Matsui N, et al. Goniosynechialysis with lens aspiration and posterior chamber intraocular lens implantation for glaucoma in spherophakia. J Cataract Refract Surg 2004;30(2):513-6 doi: 10.1016/s0886-3350(03)00670-9[published Online First: Epub Date]|.
Harasymowycz P, Wilson R. Surgical treatment of advanced chronic angle closure glaucoma in Weill-Marchesani syndrome. J Pediatr Ophthalmol Strabismus 2004;41(5):295-9
Yang J, Fan Q, Chen J, et al. The efficacy of lens removal plus IOL implantation for the treatment of spherophakia with secondary glaucoma. Br J Ophthalmol 2016;100(8):1087-92 doi: 10.1136/bjophthalmol-2015-307298[published Online First: Epub Date]|.
Chandler PA. CHOICE OF TREATMENT IN DISLOCATION OF THE LENS. Arch Ophthalmol 1964;71:765-86
Khokhar S, Pangtey MS, Sony P, Panda A. Phacoemulsification in a case of microspherophakia. J Cataract Refract Surg 2003;29(4):845-7
Yasar T. Lensectomy in the management of glaucoma in spherophakia: is it enough? J Cataract Refract Surg 2003;29(6):1052-3; author reply 53
Senthil S, Rao HL, Babu JG, Mandal AK, Addepalli UK, Garudadri CS. Outcomes of trabeculectomy in microspherophakia. Indian J Ophthalmol 2014;62(5):601-5 doi: 10.4103/0301-4738.129785[published Online First: Epub Date]|.
Kaushik S, Sachdev N, Pandav SS, Gupta A, Ram J. Bilateral acute angle closure glaucoma as a presentation of isolated microspherophakia in an adult: case report. BMC Ophthalmol 2006;6:29 doi: 10.1186/1471-2415-6-29[published Online First: Epub Date]|.
Taylor JN. Weill-Marchesani syndrome complicated by secondary glaucoma. Case management with surgical lens extraction. Aust N Z J Ophthalmol 1996;24(3):275-8
Dagi LR, Walton DS. Anterior axial lens subluxation, progressive myopia, and angle-closure glaucoma: recognition and treatment of atypical presentation of ectopia lentis. J aapos 2006;10(4):345-50 doi: 10.1016/j.jaapos.2006.01.218[published Online First: Epub Date]|.
Singh MS, Casswell EJ, Boukouvala S, Petrou P, Charteris DG. PARS PLANA VITRECTOMY AND LENSECTOMY FOR ECTOPIA LENTIS WITH AND WITHOUT THE INDUCTION OF A POSTERIOR VITREOUS DETACHMENT. Retina 2017 doi: 10.1097/iae.0000000000001534[published Online First: Epub Date]|.
Hubbard AD, Charteris DG, Cooling RJ. Vitreolensectomy in Marfan’s syndrome. Eye (Lond) 1998;12 ( Pt 3a):412-6 doi: 10.1038/eye.1998.97[published Online First: Epub Date]|.
Figures:
Figure 1: Kaplan-Meier curve showing the complete success probability (solid line with 95% confidence intervals as shown by dotted lines) of lensectomy in eyes with glaucoma in spherophakia.
Figure 2: Kaplan-Meier curve showing the qualified success probability (solid line with 95% confidence intervals as shown by dotted lines) of lensectomy in eyes with glaucoma in spherophakia
Leave a Comment