A 55-year-old man with myotonic dystrophy underwent phacoemulsification with IOL implantation in the right eye

AL Sorenson

B Reyntjens

DK Newman

H Hayashi

JM Marcantonio

K Hayashi

K Miyake

Luisa Politano

Maddalena De Bernardo

Maria Borrelli

Michele Lanza

Nicola Rosa

O Nishi

S Masket

English

PubMed

Crossref

BioMed CentralCases Journal
ssOpen AcceCase Report
Anterior capsule phimosis and capsular block syndrome in a patient 
with Steinert myotonic dystrophy: a case report
Nicola Rosa*1,2, Michele Lanza1,2, Maddalena De Bernardo2, Maria Borrelli1 
and Luisa Politano3
Address: 1Centro Grandi Apparecchiature, 2nd University of Naples, Naples, Italy, 2Eye Department, 2nd University of Naples, Naples, Italy and 
3Department of Experimental Medicine, Cardiomyology and Medical Genetics 2nd University of Naples, Naples, Italy
Email: Nicola Rosa* - nicola.rosa@unina2.it; Michele Lanza - michele.lanza@unina2.it; Maddalena De Bernardo - nicola.rosa@unina2.it; 
Maria Borrelli - nicola.rosa@unina2.it; Luisa Politano - nicola.rosa@unina2.it
* Corresponding author    
Abstract
A 55-year-old man with myotonic dystrophy underwent phacoemulsification with IOL implantation
in the right eye.
3 months after surgery, the patient showed a decreased visual acuity and an intraocular pressure
(IOP) of 30 mmHg. Slit lamp examination showed a dense fibrosis of the anterior capsule with
capsulorexis' shrinkage. Gonioscopy showed a closed angle. After a YAG laser iridotomy no
decrease in the IOP was detected; following surgical peeling of the anterior capsule, the slit lamp
showed a distended capsular bag. A YAG laser posterior capsulotomy was performed, without
decrease in the IOP. Myotonic patients need to be closely followed up after cataract surgery,
because in case of CBS development a prompt posterior capsulotomy could avoid more severe
complications.
Background
Cataract is one of the most common eye disease in
patients with Steinert myotonic dystrophy.
Anterior capsule phimosis (ACP) is one of the most com-
mon complications after continuous curvilinear capsulo-
rexis (CCC) [1].
The degree of ACP is believed to be related to some types
of disease such as pseudoexfoliation syndrome [2], dia-
betic retinopathy [3], retinitis pigmentosa [4], myotonic
dystrophy [5].
Another rare complication of CCC is the capsular block
syndrome (CBS) that is characterized by distension and
accumulation of a liquefied substance inside the capsular
bag.
We report a case of CBS and ACP in a patient with Steinert
myotonic dystrophy.
Case Report
A 55-year-old man with Steinert myotonic dystrophy was
referred to us for cataract surgery. His best spectacle cor-
rected visual acuity (BSCVA) was hand motion in the right
eye (RE) and 20/200 in the left eye (LE). The intraocular
pressure (IOP) was 12 mmHg in the RE and 10 mmHg in
the LE. Slit lamp examination showed a total cataract in
the RE and a dense corticonuclear opacity in the LE. The
patient underwent phacoemulsification in the RE through
Published: 10 December 2009
Cases Journal 2009, 2:9298 doi:10.1186/1757-1626-2-9298
Received: 7 January 2009
Accepted: 10 December 2009
This article is available from: http://www.casesjournal.com/content/2/1/9298
© 2009 Rosa et al; licensee BioMed Central Ltd. 
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), 
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Page 1 of 4
(page number not for citation purposes)
Cases Journal 2009, 2:9298 http://www.casesjournal.com/content/2/1/9298a 3.00 mm superior corneal incision under peribulbar
anesthesia with capsular staining with methilen blue. A 21
diopters single-piece hydrophilic acrylic IOL (Akreos
Adapt, Bausch & Lomb) was implanted in the capsular
bag after a 5.0 mm CCC was created. One month after sur-
gery his BSCVA was 20/25 with sph +1.0. At this time he
underwent phacoemulsification in his LE with a 23.5
diopter single-piece hydrophobic acrylic foldable lens
(Hoya) implanted with the same modalities of the RE. 15
days later BSCVA was 20/20 with sph -0.50 D.
Two months after the second surgery the clinical picture in
the LE was unchanged, but the RE showed a BSCVA of 20/
50 with sph. -0.50 D with a myopic shift and an IOP of 30
mmHg.
Slit lamp examination showed a dense fibrosis of the ante-
rior capsule with ACP (Fig. 1) that made difficult to visu-
alize the posterior structures of the eye.
Gonioscopy showed a closed angle, and a YAG laser iri-
dotomy was performed, but no decrease in the IOP was
achieved.
The patient was placed on hypotonizing topical therapy,
and a surgical peeling of the anterior capsule was per-
formed. The following days the slit lamp examination
showed the presence of slightly milky fluid behind the
IOL and a distended capsular bag, that was confirmed by
Pentacam (Fig. 2), that were not detected before for the
presence of the opaque anterior capsule. The IOP was still
30 mmHg. A YAG laser posterior capsulotomy was
attempted but no decrease in the IOP was obtained.
Discussion
In the international literature we found several cases of
ACP or CBS, but we were unable to find an association of
these two complications.
ACP depends on contraction of a fibrous membrane that
can be present either beneath the anterior capsule or on
the outer surface of the anterior capsule [6].
Although the pathogenic mechanisms responsible for
excessive capsule fibrosis and contracture are not well
understood, several histopathologic studies have identi-
fied the cell types associated with pseudophakic fibrosis
[2].
Some studies suggest that the IOL optic material may
influence the development of anterior capsule fibrosis.
Intraocular lens shape, IOL biomaterial, and, more specif-
ically, the hydrophilicity of the biometarial have been
associated with a high degree of postoperative inflamma-
tion [7,8].
It has been suggested that surgical invasion and contact
with the IOL stimulate residual lens epithelial cells (LECs)
to produce cytokines. These cytokines may induce colla-
gen production and fibrous proliferation.
Some of the cytokines induce transformation of the LECs
into myofibroblasts. that contain contractile filaments
[9].
In parallel with these processes, aqueous prostaglandin E2
concentration is elevated, leading to blood aqueous bar-
rier breakdown and an increased aqueous protein concen-
tration [10].
Previous studies have demonstrated disruption of both
the blood-retinal and blood-aqueous barriers in eyes of
patients with retinitis pigmentosa [4]. An increase in
blood-derived cytokines in the aqueous humor of these
eyes after cataract surgery, which results in increased acti-
vation of LECs with fibrosis and contracture of the ante-
rior capsule.
The same mechanism could be present in patients with
Steinert Distrophy, but in our patient we believe that the
mechanism of cytokine production was induced by the
unnoticed CBS. This is supported by Sorenson [6] who
reported that in patients with CBS the observation
resulted in acute angle closure glaucoma, chronic anterior
chamber inflammation, and development of posterior
capsular opacification.
In limited forms of capsular shrinkage without invasion
of the optical zone, YAG laser capsulotomy is considered
Slit lamp examination showing a dense fibrosis of the anterior capsule with anteri r capsule phimosisF gur 1
Slit lamp examination showing a dense fibrosis of the 
anterior capsule with anterior capsule phimosis.Page 2 of 4
(page number not for citation purposes)
Cases Journal 2009, 2:9298 http://www.casesjournal.com/content/2/1/9298the first choice. In severe cases with dense fibrous plaques,
laser capsulotomy will result in incompletely resolved
capsular debris, enhancing the risk for inflammation and
recurrence [9]. For this reason, and to avoid the risk of fur-
ther rise in the IOP due to these debris, we decided to per-
form the surgical approach to take off the fibrotic tissue.
In case of CBS the prompt treatment should restore the
IOP. Unfortunately in our patient we did not succeed; the
reason for this could have been the late posterior capsulot-
omy treatment due to the absence of the patient at the fol-
low up visit, that leaded to inflammation and fibrosis of
the capsular bag. In conclusion, our case report could sug-
gest to perform further studies to check if the implanta-
tion of hydrophilic lenses should be avoided in myotonic
patients and to closely follow patients after cataract sur-
gery because in case of CBS development a prompt poste-
rior capsulotomy should be performed to avoid more
severe complications.
Competing interests
The authors declare that they have no competing interests.
Consent
Written informed consent was obtained from the patient
for publication of this case report and accompanying
images. A copy of the written consent is available for
review by the Editor-in-Chief of this journal.
Authors' contributions
NR has made substantial contributions to conception and
design, analysis and interpretation of data; he has given
final approval of the version to be published. ML has been
involved in drafting the manuscript, performed the pho-
Pentacam image showing the presence of slightly milky fluid behind the IOL and a distended capsular bagFigure 2
Pentacam image showing the presence of slightly milky fluid behind the IOL and a distended capsular bag.Page 3 of 4
(page number not for citation purposes)
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tographic description of the case. MDB has been involved
in the research of the bibliographic data. MB has been
involved in drafting the manuscript. LP played an impor-
tant role in the selection of the patient, has given final
approval of the version to be published. All authors read
and approved the final manuscript.
References
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Anterior capsule phimosis and capsular block syndrome in a patient with Steinert myotonic dystrophy: a case report

Springer - Publisher Connector

A 55-year-old man with myotonic dystrophy underwent phacoemulsification with IOL implantation in the right eye.3 months after surgery, the patient showed a decreased visual acuity and an intraocular pressure (IOP) of 30 mmHg. Slit lamp examination showed a dense fibrosis of the anterior capsule with capsulorexis' shrinkage. Gonioscopy showed a closed angle. After a YAG laser iridotomy no decrease in the IOP was detected; following surgical peeling of the anterior capsule, the slit lamp showed a distended capsular bag. A YAG laser posterior capsulotomy was performed, without decrease in the IOP. Myotonic patients need to be closely followed up after cataract surgery, because in case of CBS development a prompt posterior capsulotomy could avoid more severe complications.A 55-year-old man with myotonic dystrophy underwent phacoemulsification with IOL implantation in the right eye. 3 months after surgery, the patient showed a decreased visual acuity and an intraocular pressure (IOP) of 30 mmHg. Slit lamp examination showed a dense fibrosis of the anterior capsule with capsulorexis' shrinkage. Gonioscopy showed a closed angle. After a YAG laser iridotomy no decrease in the IOP was detected; following surgical peeling of the anterior capsule, the slit lamp showed a distended capsular bag. A YAG laser posterior capsulotomy was performed, without decrease in the IOP. Myotonic patients need to be closely followed up after cataract surgery, because in case of CBS development a prompt posterior capsulotomy could avoid more severe complications. © 2009 Rosa et al; licensee BioMed Central Ltd

Rosa N

LANZA, Michele

De Bernardo M

Borrelli M

POLITANO, Luisa

Archivio Istituzionale della Ricerca - Università degli Studi della Campania "Luigi Vanvitelli"

Anterior capsule phimosis and capsular block syndrome in a patient with Steinert myotonic dystrophy: A case report

AN: Ultrasonographic measurement of induced myopia associated with capsular bag distention syndrome. Ophthalmology

Anterior capsule contraction and intraocular lens dislocation after implant surgery in eyes with retinitis pigmentosa. Ophthalmology

Anterior capsule contraction and intraocular lens dislocation in eyes with pseudoexfoliation syndrome.

Area reduction in the anterior capsule opening in eyes of diabetes mellitus patients. J Cataract Refract Surg

G: Lens cell populations studied in human donor capsular bags with implanted intraocular lenses. Invest Ophthalmol Vis Sci;

Intraocular lens encapsulation by shrinkage of the capsulorhexis opening. J Cataract Refract Surg

Maekubo K: Correlation between intraocular lens hydrophilicity and anterior capsule opacification and aqueous flare. J Cataract Refract Surg

Marck E: Capsular peeling in anterior capsule contraction syndrome: surgical approach and histopathological aspects. J Cataract Refract Surg

Postoperative complications of capsulorhexis.

Severe capsulorhexis contracture after cataract surgery in myotonic dystrophy. J Cataract Refract Surg

http://citeseerx.ist.psu.edu/viewdoc/summary?doi=10.1.1.356.6571

Anterior capsule phimosis and capsular block syndrome in a patient with Steinert myotonic dystrophy: a case report

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Archivio Istituzionale della Ricerca - Università degli Studi della Campania "Luigi Vanvitelli"