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Lens subluxation grading system: Predictive

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 Original Article

Lens subluxation grading system:

predictive value for ectopia lentis surgical outcomes
Sistema de classificação de subluxação do cristalino:
valor preditivo de resultados cirúrgicos em ectopia lentis
Mauro Waiswol1, Niro Kasahara2

ABSTRACT cirúrgicos. Métodos: Foram operados 51 olhos de 28 pacientes

Objective: To present a classification system to grade ectopia
lentis and to assess its usefulness as a predictor for surgical
outcomes. Methods: Fifty-one eyes of 28 patients with either
simple (19 patients) or Marfan syndrome-associated ectopia
lentis (nine patients) with variable degrees of subluxation were
operated on. Lens subluxation intensity was graded according to
the lens subluxation grading system (LSGS) from grade 1 (lens
on the whole pupillary area) up to grade 4 (lens absent from the
pupillary area). Thirty eyes underwent cataract extraction (“dry”
aspiration) with endocapsular ring and in-the-bag intraocular
lens (IOL) implantation. Twenty-one eyes underwent cataract
extraction (“dry” aspiration) with scleral fixation of the IOL. The
predictive value of the LSGS was assessed by analyzing the post-
operative outcomes, including visual acuity (VA), endothelial cell
loss, and complications for each grade on the grading system.
Results: Patients were classified into grade 1 (19.6%), grade 2
(51%) and grade 3 (29.4%). Post-operative VA was lower for eyes
with larger degrees of subluxation. The higher the subluxation
grade, the higher the endothelial cell loss, as well as, the frequency
of vitreous loss and surgical time. Higher subluxation grades
prevented optimal surgical outcomes with endocapsular ring and
in-the-bag IOL implantation. Conclusions: The LSGS provides
an estimate of the surgical success of ectopia lentis. Adequate
standardization of lens subluxation is crucial for understanding
studies dealing with the surgical correction of this disorder. 
Keywords: Ectopia lentis/surgery; Marfan syndrome; Lens
subluxation; Lens implantation, intraocular 
portadores de ectopia lentis simples (19 pacientes) ou associados
à síndrome de Marfan (nove pacientes) com diferentes graus de
subluxação. A intensidade da subluxação do cristalino foi graduada
de acordo com o Sistema de Classificação de Subluxação do
Cristalino (SCSC) de grau 1 (cristalino ocupando toda a área
pupilar) a grau 4 (cristalino ausente da área pupilar). Trinta olhos
foram submetidos à facectomia (aspiração “seca”) por meio de
anel endocapsular e implante de lente intraocular (LIO) no saco
capsular. Vinte e um olhos foram submetidos à facectomia
(aspiração “seca”) com fixação escleral da LIO. O valor preditivo
do SCSC foi avaliado por meio da análise dos resultados pós-
operatórios, que incluiu acuidade visual (AV), perda de células
endoteliais e complicações relacionadas a cada grau do sistema
de classificação. Resultados: Os pacientes foram classificados em
grau 1 (19,6%), grau 2 (51%) e grau 3 (29,4%). A AV pós-operatória
foi menor nos olhos com graus maiores de subluxação. Quanto
maior o grau de subluxação, maior a perda de células endoteliais,
bem como a frequência de perda vítrea e o tempo de cirurgia. Graus
mais avançados de subluxação impediram resultados cirúrgicos
ideais com anel endocapsular e implante de LIO no saco capsular.
Conclusões: O SCSC fornece uma estimativa do sucesso cirúrgico
em ectopia lentis. É fundamental uma padronização adequada da
subluxação do cristalino para que seja possível compreender os
estudos que tratam da correção cirúrgica desta condição.
Descritores: Ectopia do cristalino/cirurgia; Síndrome de Marfan;
Subluxação do cristalino; Implante de lente intra-ocular

INTRODUCTION
RESUMO Ectopia lentis is a lenticular dystopia, either congenital or
Objetivo: Apresentar um sistema de classificação para graduar acquired in origin, secondary to trauma or spontaneous.
a ectopia lentis e avaliar seu valor preditivo para os resultados According to the amount of zonular support, the dystopia

Study carried out at the Departamento de Oftalmologia of Santa Casa de Misericórdia de São Paulo and Faculdade de Medicina of Universidade de São Paulo – USP, São Paulo (SP), Brazil.
1
MD; post-doctorate degree from Faculdade de Medicina da Universidade de São Paulo – USP, São Paulo (SP), Brazil; head of the Congenital Cataract Service of the Departamento de Oftalmologia of
Santa Casa de Misericórdia de São Paulo – São Paulo (SP), Brazil.
2
MD; head of the Eye Emergency Service of the Departamento de Oftalmologia at Santa Casa de Misericórdia de São Paulo – São Paulo (SP), Brazil.
Corresponding author: Mauro Waiswol – Avenida Angélica, 916 – suíte 903 – Santa Cecília – CEP 01228-000 – São Paulo (SP), Brasil – Tel.: 11 3825-2382 – e-mail: waiswol@terra.com.br
Received on Dec 17, 2008 – Accepted on Jan 28, 2009

einstein. 2009; 7(1 Pt 1):81-7

82 Waiswol M, Kasahara N
can be classified into subluxation, when the lens partially
remains in the pupillary area at the posterior chamber, or
luxation, when it can migrate to the anterior chamber or
vitreous cavity as a result of complete rupture of zonule
fibers(1).
Spontaneous lens subluxation can occur as result of
an anomalous zonular development, making it short in
length and spherical in shape. It is frequently a family
condition and the zonular fibers can be very elongated
and remain adhered to the lens(2-3). The lens can become
opaque and cause increased intraocular pressure and its
extraction is recommended(2,4).
The lens equator can be seen in the pupillary area,
even without mydriasis. In this situation monocular
dyplopia or binocular quadruplication can occur(5).
The zonular fibers can vary in shape and grouping
on biomicroscopy. Zonular weakness causes the lens
to become spherical in shape, thus leading to severe
myopia. The lens are usually shorter; they can remain
transparent and, occasionally, they are colobomatous(5).
The displacement of the lens usually results in optical
complications. In addition, glaucoma, uveitis, retinal
detachment and cataract can occur(2).
The treatment of ectopia lentis poses a challenge to
clinicians. Not infrequently, a multidisciplinary team
is involved for caring for systemic abnormalities and
genetic counseling(1). The therapeutic approach should
be individualized and the goal should be to provide
good visual acuity (VA)(1).
Optical correction with glasses or contact lenses is
the first choice for many patients. The use of mydriatic
or miotic agents can help to improve vision(6). The
surgical treatment of ectopia lentis is controversial.
While some authors delay lens extraction for fear
of intraoperative and post-operative complications,
others believe that subluxation itself is an indication
for surgery(7-8).
Careful assessment of specific indications for surgery
is suggested(9,4). Continuous development of ocular
instruments and improvement of surgical techniques have
stimulated many people to choose the surgical management
to improve VA in this patients(10-18). Many authors have
proved the efficacy and safety of different techniques
for the surgical correction of ectopia lentis(12,15,19-25).
Although the majority of surgeons are unanimous
on the indication for early surgery on severe subluxation
cases, none has clearly established a lens subluxation
severity graduation.
OBJECTIVE
The purpose of this study is to propose a classification
system for grading lens subluxation and to evaluate its
predictive value for surgical outcomes.
einstein. 2009; 7(1 Pt 1):81-7
METHODS
Sample and selection criteria
This prospective study was approved by the Ethics
Committee from the two Institutions involved
(Department of Ophthalmology of Santa Casa de São
Paulo and Faculdade de Medicina da Universidade de
São Paulo) and the procedures were in agreement with
the tenets of the Declaration of Helsinki.
Patients were enrolled in the study from March,
2000 to June, 2002 based on the presence of lens
subluxation on examination. Exclusion criteria included
lens luxation to the anterior or posterior chamber,
history of ocular trauma, corneal opacity precluding
anterior segment examination, glaucoma, uveitis,
retinal abnormalities compromising visual recovery
or posing risk for retinal detachment, previous ocular
surgery, any medical condition preventing patients
from undergoing general anesthesia and patient
unwillingness. All subjects underwent a complete
eye examination including refraction, biomicroscopy,
aplanation tonometry, indirect ophthalmoscopy,
A-scan, and endothelial cells count. Fifty-one eyes
from 28 patients were enrolled in the study. Nineteen
patients had simple ectopia lentis (SEL) and nine
had Marfan syndrome-associated ectopia lentis (MS).
Sixteen patients were male and 12 were female. Mean
age was 15.9 ± 8.5 years (range, 5.4 to 37.6 years).
Lens subluxation grading system
In order to assess subluxation severity, it was proposed
a classification system based on the lens displacement
in relation to the undilated pupil. The patient’s eye was
assessed at the slit-lamp with 10x magnification, broad
slit for diffuse illumination in order to achieve a wide
panoramic view. A narrow slit with 45° illumination for
locating the lens was used when necessary. Assessment
was completed with the red field illumination and the
result expressed into one of four grades (Figure 1):
grade 1, lens seen on the pupillary area; grade 2; lens
seen on 2/3 of the pupillary area; grade 3, lens seen on
1/2 of the pupillary area or grade 4, lens absent from
the pupillary area.
Surgical technique
Surgery was indicated when the patients VA prevented
them from doing their daily activities, i.e., job, study,
domestic tasks, driving, leisure, etc. The surgeries were
performed by only one surgeon (MW). Twenty-three
patients (82.1%) had bilateral, sequential surgery;
24 eyes were right eyes (47.1%) and 27 were left eyes
(52.9%).

Grade 1: lens seen on the pupillary area Grade 2: lens seen on 2/3 of the pupillary area
Grade 3: lens seen on ½ of the pupillary area Grade 4: lens absent from the pupillary area
Figure 1. Lens subluxation graduation
The procedure was initiated with the creation of
a scleral frown incision (5 mm) using a crescent knife
(Alcon Surgical, Fort-Worth, Texas) and a temporal or
nasal corneal incision (1 mm). The anterior chamber
was filled with a viscoelastic agent and capsulorrhexis
was done with an Utrata forceps. At this point, an
endocapsular ring (CTR -10, Mediphacos, Belo
Horizonte – Minas Gerais, Brazil) was inserted. In the
eyes in which the capsulorrhexis was suboptimal, the
endocapsular ring was not implanted.
Following, it would be the hydrodissection, the
lens content was removed with “dry” aspiration by
means of a canula and syringe and viscoelastics were
used to deepen the anterior chamber as needed. A
polymethylmethacrylate (PMMA) intraocular lens
(IOL) was placed in the bag and the scleral ring was
sutured at the sclera. In eyes in which an endocapsular
ring was not inserted, scleral fixation of the PMMA
IOL was done with 10-0 polypropylene sutures.
The scleral wound was sutured with one 10-0 nylon
suture and a sub-conjuntival injection of gentamycin
1 cc and dexamethasone 1 cc was given at the inferior
fornix.
Post-operative care
Post-operatively, every patient took prednisolone
acetate and tobramycin drops every three hours, tapered
off over six weeks at the surgeon’s discretion. Patients
were seen at the post-operative day 2 and 9 and then
at one, two, three and six months. At every visit, eye
examination included measure of VA, biomicroscopy,
tonometry, and ophthalmoscopy. The IOL was evaluated
Lens subluxation grading system: predictive value for ectopia lentis surgical outcomes 83
for any displacement and recorded using the LSGS. On
post-operative month 6, endothelial cells count and
refraction were done.
Statistical analysis
It was used the χ2 test to compare subluxation grades
between eyes with SEL and MS. The improvement of
VA at each visit was analyzed with ANOVA. Unpaired
t-Student test and Mann-Whitney U test were used to
compare axial length between SEL and MS eyes; pre
and post-operative endothelial cell loss between the two
surgical techniques; post-operative VA (at month 6) and
duration of surgery between eyes with intraoperative
vitreous loss and eyes with no vitreous loss. Pre and
post-operative endothelial cell loss for the 51 eyes were
compared with paired t-Student test. ANOVA and
Kruskal-Wallis test were used to compare axial length
and endothelial cell loss among each LSGS group. A p
value of less than 0.05 was considered to be of statistical
significance.
RESULTS
Preoperative lens subluxation was classified as grade 1
in 10 eyes (19.6%), grade 2 in 26 eyes (51%), and grade
3 in 15 eyes (29.4%) according to LSGS. No patient had
grade 4. These figures were similar for SEL and MS
patients (χ2 = 1.63; p = 0.443).
Mean preoperative uncorrected VA was not
different for each LSGS group (Table 1), although lower
LSGS grades had better VA. As to the post-operative
uncorrected VA, eyes with grade 3 presented with
lower VA at each time point throughout the follow-up
as compared to grades 1 and 2 (Figure 2). However, at
the last follow-up, eyes with LSGS grades 1 and 2 had
better VA as compared to grade 3 (Table 2).
The mean axial length for the 51 eyes was
24.6 ± 2.0 mm and eyes with MS presented longer
measurements (25.5 mm) as compared to those with
SEL (24.2 mm) (t = 2.09; p = 0.04; U = 202.5; p =
0.08). Analysis of axial length stratified per LSGS
grades showed no difference (F = 1.73; p = 0.188;
H = 4.42; p = 0.109).
Preoperative endothelial cell count was 3,497.2 cells/
mm2 and post-operative count was 3,154.2 cells/mm2.
This difference (343 cells/mm2; 9.8%) was statistically
significant (t = 8.41; p < 0.0000001). Difference between
SEL (10.3%) and MS (8.7%) was not significant. The
endothelial cell loss increased proportional to higher
LSGS grades (grade 1, 6.7% loss; grade 2, 9.5% loss; and
grade 3, 12.3% loss); these differences, however, failed
to reach statistical significance (F = 1.47; p  =  0.240;
H = 2.59; p = 0.247).
einstein. 2009; 7(1 Pt 1):81-7
84 Waiswol M, Kasahara N

Table 1. Means of visual acuity (VA) without correction according to the different lens subluxation grades before surgery and in the post-operative follow-up
Lens subluxation grades General ANOVA Kruskal-Wallis
Assessment group
1 2 3 F p-value H p-value
Preoperative means 0.0991 0.0566 0.0493 0.0628 2.45 0.097426 1.70 0.4278
2 post-operative day
nd
0.2050 0.0985 0.0853 0.1155 6.08 0.004441* 9.74 0.0077*
9th post-operative day 0.3050 0.1654 0.1325 0.1831 5.60 0.006538* 11.62 0.0030*
30 post-operative day
th
0.4200 0.2481 0.1708 0.2591 8.55 0.000668* 14.64 0.0007*
60 post-operative day
th
0.5100 0.3269 0.1933 0.3235 14.18 0.000015* 18.59 0.0001*
90th post-operative day 0.5500 0.3962 0.2305 0.3776 10.87 0.000127* 15.40 0.0005*
180 post-operative day
th
0.5400 0.4346 0.2624 0.4046 7.51 0.001457* 11.59 0.0031*
*Significant

Table 2. Means of visual acuity (VA) with correction according to the different lens subluxation grades before and 180 days after surgery
Lens subluxation grades General ANOVA Kruskal-Wallis
Assessment group
1 2 3 F p-value H p-value
Before surgery 0.2350 0.1822 0.1377 0.1795 2.16 0.126805 4.01 0.1344
180 days after surgery 0.7500 0.6788 0.4508 0.6257 5.77 0.005667* 5.57 0.0618
*Significant

V 0.6
Gr ad e 1
i
s 0 .5
u
a 0 .4
l
A 0 .3
c
u 0 .2
i
t 0 .1
y
0
30 60
P o s t-o p era tiv e f o l lo w- u p d a y s
Figure 2. Means of visual acuity (VA) without correction according to the different
lens subluxation grades before surgery and in the post-operative follow-up
When compared as to the surgical technique,
endothelial cell loss was 12.8% in eyes that underwent
cataract extraction with scleral fixation of the IOL
and 7.6% in eyes that had cataract extraction with
endocapsular ring and in-the-bag IOL implantation
(t = 2.29; p = 0.02; U = 224; p = 0.08).
Intraoperative vitreous loss was recorded in five
eyes (9.8%). Vitreous loss occurred in one eye with
LSGS and grade 2 and 4 eyes with LSGS grade 3.
Uncorrected VA at the last follow-up was 0.21 for eyes
with vitreous loss as compared to 0.42 for the remaining
46 eyes (t = 2.24; p = 0.02; U = 55; p = 0.05). Best
corrected VA was 0.37 for eyes with vitreous loss and
0.65 for the remaining 46 eyes (t = 2.36; p = 0.02; U =
53; p = 0.04). Mean surgical time was 93 minutes for the
five eyes with vitreous loss, 34.1% longer than the 69.3
minutes for the 46 eyes with no vitreous loss (t = 3.47;
p = 0.001; U = 48; p = 0.03).
Grade 2 G ra de 3 To ta l
Complications were recorded in three eyes (5.9%)
and included secondary glaucoma in two eyes (3.9%) well
controlled with anti-glaucomatous topical medication.
Post-operative displacement of the IOL occurred in one
eye (2.0%), which was successfully replaced later on.
90 180 DISCUSSION
Fast recovery and adequate optical correction of
refractive errors in patients with ectopia lentis are crucial
for preventing the development of amblyopia in infants.
When the subluxation is small and stable, the refractive
error can be small and easily corrected with eyeglasses
or contact lenses. When no complication is evident,
patients should not be operated on(1,4,6,9,19,26-27).
On the other hand, lens dislocation can cause
optical defects which cannot be easily corrected by
means of glasses and contact lenses. High astigmatism
can be induced by the lens equator placed in the
median pupillary portion or due to irregular shape
and rotation over its axis as a result of partial zonular
rupture. Severe lenticular myopia can result from
partial absence of zonules(28).
The most common cause of surgical indication
to correct ectopia lentis is the pupil bisection through
the lens equator, in the visual axis. Even though visual
correction for both the phakic or aphakic pupil is
possible, the resulting vision might not be adequate(6).
Additionally, the lens can be so unstable that refractive
errors change continuously and, occasionally, complete
luxation may occur.

einstein. 2009; 7(1 Pt 1):81-7


When an acceptable VA for engaging in daily
activities is not achieved, after careful refraction or
when imminent signs of complications develop, surgery
must be considered.
In this study, surgery was indicated when the best
corrected VA was equal to or less than 20/50 or when
the patient’s quality of vision was not appropriate for
them to perform daily activities(10-18,29-36).  lentis (simple or Marfan-associated) was related to post-
In this paper it was decided to include both eyes
of the same patient whenever the need for surgical
intervention was mandatory for both. This was done
considering that lens subluxation is a relatively rare
condition and, by using only one eye per patient, the
sample size would be reduced. A sub-analysis per
group (between SEL and MS) was not carried out,
since the small number of patients in each group
would fail to reach a high statistical power in order to
draw definite conclusions. Additionally, regardless of
the cause for lens subluxation, the clinical picture and
prognosis, as well as the intervention, are the same for
either cause.
The choice for the surgical technique was based
on the safety of the procedure, short learning curve,
adequacy for the complexity of the condition and
dispensability of expensive pieces of equipment and
technology. In this regard, the surgical technique used
in these groups of patients seemed to be a good choice.
In some eyes, the capsulorrhexis was not optimal and
the removal of the lens content with “dry” aspiration
and scleral fixation of the IOL in the posterior chamber
was an alternative approach(37).
Previous studies have shown the efficacy of IOL
implantation using the endocapsular ring for scleral
fixation in the management of zonular dialysis in
cataract surgery(11-13,38-39).  capsulorrhesis could not be achieved. Aspiration of lens
It was observed that endocapsular ring
implantation was feasible in eyes with less extensive
lens subluxation (LSGS grade 1 and 2) in which
capsulorrhexis was optimally performed. These
patients were, indeed, those with better preoperative
VA and less amblyopia.
The patient’s distribution was homogeneous in
terms of age, gender, ethnicity, eye to be operated
on, diagnosis and LSGS grades. Thus, the surgical
outcomes of the study are reliable and free from
possible selection bias.
VA improved steadily after surgery, especially after
post-operative day 9 up to day 90 (three months). In the
first post-operative days, VA is compromised by a slight
corneal edema and, occasionally, by cystoid macular
edema, which can resolve spontaneously(28).
The improvement in VA was related to the LSGS
grade. The present results have shown that the final VA
was better in eyes with grade 1 than in those with grades
Lens subluxation grading system: predictive value for ectopia lentis surgical outcomes 85
2 and 3. Eyes with higher subluxation grades demand
more complex optical correction and have greater
chances of developing amblyopia. The uncorrected VA
was related to the surgical technique. Eyes undergoing
cataract extraction and endocapsular ring had better
final VA than those undergoing cataract extraction and
scleral fixation. On the other hand, the cause of ectopia
operative best corrected VA.
Ectopia lentis can worsen in time, so that, subluxation
grades can increase(1,12,19). Our study has shown the
predictive value of the LSGS in the surgical outcomes.
If worsening of the lens subluxation occurs, we believe
that early surgical intervention can bring better visual
results, thus decreasing the chances of amblyopia in
young children(28).
Mean uncorrected and best corrected VA were 50%
lower in eyes with vitreous loss. These eyes (9.8%) had
LSGS grades 2 (3.9%) and 3 (26.7%), an indication
that vitreous loss is more frequent in eyes with more
advanced lens subluxation. This situation was found in
these eyes. Eyes with LSGS grades 2 and 3 are more
likely to have intraoperative vitreous loss; eyes with
vitreous loss have little improvement in VA; finally, eyes
with little improvement in VA are those with higher
lens subluxation grades from the start. In addition, eyes
with more intense lens subluxation are prone to have
more intraoperative complications and vitreous loss,
demanding extra time for surgery (as reported in the
cases of the present article).
Endothelial cell count decreased by 9.8% on average
and eyes that underwent cataract extraction and scleral
fixation of the IOL had higher loss. This technique was
employed in eyes with higher LSGS grades, since optimal
content without the endocapsular ring had to be done
more repeatedly and the suturing of the IOL caused the
procedure to be more traumatic, leading to additional
loss of endothelial cells.
When comparing post-operative VA with axial
length, the present results showed that eyes with
longer axial length presented worse VA, possibly
due to preoperative amblyopia. In addition, eyes
with longer axial length had higher grades of lens
subluxation. We believe this is a casual relationship,
i.e., subluxation increase as a proportion of the
increase in the axial length. Surgery in eyes with
longer axial length is more difficult as a result of
higher subluxation grades.
It was recorded very few complications, namely,
glaucoma in two eyes (one eye with SLGS grade 2
and one with grade 3) and subluxation of the IOL in
another. Possible causes for glaucoma in these eyes
would include trabecular inflammation, obstruction of
einstein. 2009; 7(1 Pt 1):81-7
86 Waiswol M, Kasahara N
conventional outflow by red blood cells, viscoelastics or
cortical debris, and distortion of the anterior chamber
angle(1). The two eyes with glaucoma were satisfactorily
controlled on medical therapy.
The proper implantation of an IOL is mandatory,
in order to achieve satisfactory VA. Optimal capsular
support for adequate placement of the IOL is an
exception in eyes with lens subluxation. In our
series, we used the endocapsular ring prior to IOL
implantation whenever possible, or the IOL would be
sutured to the sclera in order to have a finely placed
implant. Nevertheless, four eyes presented with minor
displacement of the IOL optic, which compromised
either VA or the patient’s satisfaction. One eye that
had scleral fixation of the IOL underwent a second
procedure for IOL resuturing. This patient denied
any trauma and the reason for the IOL displacement
remained unknown.
Post-operative IOL eccentricity was proportional to
preoperative lens subluxation. No eye with LSGS grade
1 presented with post-operative IOL displacement,
whereas two eyes with LSGS grade 2 and two eyes with
LSGS grade 3 presented with slight IOL dislocation. The
majority of eyes with LSGS grade 1 had endocapsular
ring implantation and it is believed that this technique
enhances the chances of having a properly placed IOL
in the post-operative period.
CONCLUSIONS
Adequate classification on lens subluxation,  as the
one proposed here (SLGS), is helpful in predicting
post-operative outcomes. The lower the amount of
subluxation (SLGS grade 1 and 2), the better the
visual results and the patient’s satisfaction, not to
mention that the surgical procedure is carried out
more safely.
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