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Anterior Lentiglobus Resulting From
Abnormalities in the Posterior Capsule
Hayat Ahmad Khan MBBS, DOMS, MS; Asim Ali MD, FRCSC; Kamiar Mireskandari MBChB, FRCSEd, FRCOphth, PhD
Hospital for Sick Children, Toronto, Ontario M5G 1X8
REFERENCES
 Ritch R, Chang BM, Liebmann JM. Angle closure in younger patients.
Ophthalmology. 2003 Oct;110(10):1880-9.
 Macken PL, Pavlin CJ, Tuli R, Trope GE. Ultrasound biomicroscopic features of
spherophakia. Aust NZ J Ophthalmol 1995; 23:217–220
 Olga Cero´n,Peter L. Lou, Arnold J. Kroll,David S. Walton. The Vitreo-Retinal
Manifestations of Persistent Hyperplasic Primary Vitreous (PHPV) and Their
Management. International Ophthalmology Clinics, Volume 48, Number 2, 53–62
 Anders Behndig, Phacoemulsification in spherophakia with corneal touch. MD,
PhDJ Cataract Refract Surg 2002; 28:189–191
 Shastry BS. Persistent hyperplastic primary vitreous: congenital malformation of
the eye. Clin. Experiment Ophthalmol. 2009 Dec;37(9):884-90.
 Mira Silbert, Andrew S. Gunvood. Persistent hyperplastic primary vitreous.
Clinical Eye and Vision Care. Volume 12, Issues 3-4, December 2000, Pages
131-137
CASE REPORT
Patient 1: Full term 6 week old girl presented with right exotropia
and leukocoria. On examination, she was found to have
associated latent nystagmus in the right eye with very shallow
anterior chamber (AC) and a spherical anterior lens surface
projecting into the AC with associated central cataract. The
posterior lens capsule was flattened with a central dense fibrous
plaque adherent to it. The UBM scan confirmed that this plaque
was continuous with the ciliary body and flattening out the ciliary
processes and the posterior lens capsule resulting in the anterior
doming of the lens. Fundus examination was normal except for
macular hypoplasia. The left eye was normal. She was diagnosed
with persistent fetal vasculature in right eye. The patient
underwent a successful lensectomy and vitrectomy in right eye.
Patient 2: Full term 1 month old boy presented with right
microphthalmia, leukocoria, horizontal nystagmus and esotropia
with unremarkable birth and family history. On examination,
bilateral shallow ACs with anterior bulging of the lens in both
eyes. The UBM scan confirmed peripheral capsular fibrosis and
flattening causing the anterior lentiglobus. There was a right
complete retinal detachment. There was a lenticular high myopia
and pseudoexotropia due to the macular dragging in left eye.
Familial exudative vitreoretinopathy is suspected and awaiting
molecular confirmation.
CONCLUSIONS
The spectrum of disease in the three affected eyes shows a
consistent picture of posterior capsular fibrosis and flattening
causing anterior lentiglobus. This was demonstrated using
Retcam® and UBM imaging. In patients with this clinical picture
are at risk of angle closure glaucoma and corneal endothelial
touch by the lentiglobus and need to be observed for these
complications.
P-00027
Abstract ID: 800213
None of the author have any affiliation (financial or otherwise) with a commercial organization that may have a direct or indirect connection to the content of our presentation.
Subject # 1 (OD) Subject # 2 (OS)Subject # 2 (OD)
AnteriorSegmentRetcam®PhotoGonioscopicRetcam®Photo50HzUBMScanRetcamFundusPhoto
Normal Morphology Anterior Dome Morphology
OBJECTIVE
To demonstrate anterior doming of the lens resulting from
posterior capsular abnormality using Retcam® and high
frequency Ultrasound Biomicroscopy (UBM).
METHODS AND RESULTS
Three eyes of two patients were examined under anaesthesia
including the use of Retcam® (Clarity Medical Systems)
imaging and high frequency UBM (Sonomed Vumax II).
BScan
Subject # 1
(OD)
Subject # 1
(OS)
Subject # 2
(OD)
Subject # 2
(OS)
Age 6 weeks 4weeks
Diagnosis Persistent Fetal Vasculature
Familial Exudative
Vitreoretinopathy
Presentation Leukocoria (OD) Leukocoria, Microphthalmos (OD)
Vision LP
4.7 CPD
(Tellers cards)
NLP
1.6 CPD
(Tellers cards)
Retinoscopy
Lenticular
myopia
+1.50 D Not Possible
Lenticular
myopia
-16.50D
Tonometry 9 mmHg 13 mmHg 14 mmHg 15 mmHg
Pachymetry 523 µm 565µm 688µm 574µm
Corneal
Diameter
10.0 mm 11.5 mm 9.0 mm 10.0 mm
AC Depth 0.75 mm 2.96 mm 0.56 mm 0.56 mm
Lens
Thickness
3.83 mm 3.38 mm 4.22 mm 4.10 mm
Lens
Description
Severe Anterior
Doming
Normal
Anterior
Doming
Anterior
Doming
Retcam ®
features
Central white
plaque cataract
with dense
fibrous
membrane in
anterior
vitreous
Normal Eye
Total closed
funnel
tractional
retinal
detachment
Macular drag
Normal Lens
Anterior
Lentiglobus

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  • 1. Anterior Lentiglobus Resulting From Abnormalities in the Posterior Capsule Hayat Ahmad Khan MBBS, DOMS, MS; Asim Ali MD, FRCSC; Kamiar Mireskandari MBChB, FRCSEd, FRCOphth, PhD Hospital for Sick Children, Toronto, Ontario M5G 1X8 REFERENCES  Ritch R, Chang BM, Liebmann JM. Angle closure in younger patients. Ophthalmology. 2003 Oct;110(10):1880-9.  Macken PL, Pavlin CJ, Tuli R, Trope GE. Ultrasound biomicroscopic features of spherophakia. Aust NZ J Ophthalmol 1995; 23:217–220  Olga Cero´n,Peter L. Lou, Arnold J. Kroll,David S. Walton. The Vitreo-Retinal Manifestations of Persistent Hyperplasic Primary Vitreous (PHPV) and Their Management. International Ophthalmology Clinics, Volume 48, Number 2, 53–62  Anders Behndig, Phacoemulsification in spherophakia with corneal touch. MD, PhDJ Cataract Refract Surg 2002; 28:189–191  Shastry BS. Persistent hyperplastic primary vitreous: congenital malformation of the eye. Clin. Experiment Ophthalmol. 2009 Dec;37(9):884-90.  Mira Silbert, Andrew S. Gunvood. Persistent hyperplastic primary vitreous. Clinical Eye and Vision Care. Volume 12, Issues 3-4, December 2000, Pages 131-137 CASE REPORT Patient 1: Full term 6 week old girl presented with right exotropia and leukocoria. On examination, she was found to have associated latent nystagmus in the right eye with very shallow anterior chamber (AC) and a spherical anterior lens surface projecting into the AC with associated central cataract. The posterior lens capsule was flattened with a central dense fibrous plaque adherent to it. The UBM scan confirmed that this plaque was continuous with the ciliary body and flattening out the ciliary processes and the posterior lens capsule resulting in the anterior doming of the lens. Fundus examination was normal except for macular hypoplasia. The left eye was normal. She was diagnosed with persistent fetal vasculature in right eye. The patient underwent a successful lensectomy and vitrectomy in right eye. Patient 2: Full term 1 month old boy presented with right microphthalmia, leukocoria, horizontal nystagmus and esotropia with unremarkable birth and family history. On examination, bilateral shallow ACs with anterior bulging of the lens in both eyes. The UBM scan confirmed peripheral capsular fibrosis and flattening causing the anterior lentiglobus. There was a right complete retinal detachment. There was a lenticular high myopia and pseudoexotropia due to the macular dragging in left eye. Familial exudative vitreoretinopathy is suspected and awaiting molecular confirmation. CONCLUSIONS The spectrum of disease in the three affected eyes shows a consistent picture of posterior capsular fibrosis and flattening causing anterior lentiglobus. This was demonstrated using Retcam® and UBM imaging. In patients with this clinical picture are at risk of angle closure glaucoma and corneal endothelial touch by the lentiglobus and need to be observed for these complications. P-00027 Abstract ID: 800213 None of the author have any affiliation (financial or otherwise) with a commercial organization that may have a direct or indirect connection to the content of our presentation. Subject # 1 (OD) Subject # 2 (OS)Subject # 2 (OD) AnteriorSegmentRetcam®PhotoGonioscopicRetcam®Photo50HzUBMScanRetcamFundusPhoto Normal Morphology Anterior Dome Morphology OBJECTIVE To demonstrate anterior doming of the lens resulting from posterior capsular abnormality using Retcam® and high frequency Ultrasound Biomicroscopy (UBM). METHODS AND RESULTS Three eyes of two patients were examined under anaesthesia including the use of Retcam® (Clarity Medical Systems) imaging and high frequency UBM (Sonomed Vumax II). BScan Subject # 1 (OD) Subject # 1 (OS) Subject # 2 (OD) Subject # 2 (OS) Age 6 weeks 4weeks Diagnosis Persistent Fetal Vasculature Familial Exudative Vitreoretinopathy Presentation Leukocoria (OD) Leukocoria, Microphthalmos (OD) Vision LP 4.7 CPD (Tellers cards) NLP 1.6 CPD (Tellers cards) Retinoscopy Lenticular myopia +1.50 D Not Possible Lenticular myopia -16.50D Tonometry 9 mmHg 13 mmHg 14 mmHg 15 mmHg Pachymetry 523 µm 565µm 688µm 574µm Corneal Diameter 10.0 mm 11.5 mm 9.0 mm 10.0 mm AC Depth 0.75 mm 2.96 mm 0.56 mm 0.56 mm Lens Thickness 3.83 mm 3.38 mm 4.22 mm 4.10 mm Lens Description Severe Anterior Doming Normal Anterior Doming Anterior Doming Retcam ® features Central white plaque cataract with dense fibrous membrane in anterior vitreous Normal Eye Total closed funnel tractional retinal detachment Macular drag Normal Lens Anterior Lentiglobus