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.



Correspondence:

Hayat A KhanFellow, Department of Ophthalmology

Hospital for Sick Children, Toronto ON, M5G 1H4hayatkhan123@yahoo.com

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-00027Abstract 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)

Illustration of Lens Morphology Changes

An

teri

or S

egm

ent

Ret

cam

® P

hot

oG

onio

scop

ic R

etca

m®

Ph

oto

50H

z U

BM

Sca

nR

etca

m F

un

du

s P

hot

o

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).

B S

can

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 LP4.7 CPD (Tellers cards)

NLP1.6 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