Journal of Pediatric Ophthalmology and Strabismus

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Short Subjects 

Use of Anterior Segment OCT in the Evaluation and Management of Congenital Aphakia

Vanissa W. S. Chow, AFCOphthHK; Amy L. Wong, MRCSEd, Mmed; Dorothy S. P. Fan, FRCS, MSc

Abstract

Anterior segment optical coherence tomography (AS-OCT) is a noninvasive technology capable of capturing high-resolution images of the anterior segment. With AS-OCT, a 7-year-old boy with nystagmus was diagnosed as having secondary congenital aphakia. The presence of lens capsules on the AS-OCT was of significant prognostic and management value and the child later benefited from bilateral sulcus-fixated intraocular lens implantation surgery. AS-OCT also required less cooperation from the patient compared to ultrasound biomicroscopy. To the authors’ knowledge, this is the first reported case of congenital aphakia diagnosed by AS-OCT.

Abstract

Anterior segment optical coherence tomography (AS-OCT) is a noninvasive technology capable of capturing high-resolution images of the anterior segment. With AS-OCT, a 7-year-old boy with nystagmus was diagnosed as having secondary congenital aphakia. The presence of lens capsules on the AS-OCT was of significant prognostic and management value and the child later benefited from bilateral sulcus-fixated intraocular lens implantation surgery. AS-OCT also required less cooperation from the patient compared to ultrasound biomicroscopy. To the authors’ knowledge, this is the first reported case of congenital aphakia diagnosed by AS-OCT.

From Hong Kong Eye Hospital and the Department of Ophthalmology & Visual Sciences, the Chinese University of Hong Kong, Hong Kong, People’s Republic of China.

Presented as a poster at the World Ophthalmology Congress 2008, Hong Kong, People’s Republic of China, June 28–July 2, 2008.

The authors have no financial or proprietary interest in the materials presented herein.

The authors acknowledge the contributions made by Professor Dennis S. C. Lam to the clinical and surgical care of the patient and writing of this manuscript.

Address correspondence to Dorothy S. P. Fan, FRCS, MSc, Department of Ophthalmology & Visual Sciences, 3rd Floor, Hong Kong Eye Hospital, 147K Argyle Street, Kowloon, Hong Kong, People’s Republic of China.

Received: October 19, 2008
Accepted: January 23, 2009
Posted Online: May 21, 2010

Introduction

Anterior segment optical coherence tomography (AS-OCT) is increasing in popularity in the field of adult anterior segment and glaucoma conditions for its accuracy and easy-to-use operation. Such advantages may also benefit the pediatric population, as in the current case, where it helped us in the diagnosis and management of congenital aphakia.

Case Report

A 7-year-old boy from rural China who had been born full-term presented with bilateral poor vision since an early age (the exact timing could not be verified). Both eyes were nystagmic, with best-corrected visual acuity of 20/320 bilaterally. Refraction for the right eye was +12.00 D / −1.00 D × 90 and the left eye was +12.00 D. Both eyes were microphthalmic, with corneal diameters of 9 mm. A-scan measured axial lengths of 19.83 mm in the right eye and 19.89 mm in the left eye. Keratometry was 50.50 D at 178° / 51.25 D at 88° in the right eye, and 51.37 D at 6° / 53.87 D at 96° in the left eye. Both crystalline lenses were absent but transparent membranes could be vaguely seen across the pupil (Fig. 1). Other anterior segment structures and posterior segments were normal.

Slit-Lamp Photographs of the Anterior Segment Showing Aphakia with a Membrane (arrow) Across the Pupil. (A) Right Eye. (B) Left Eye.

Figure 1. Slit-Lamp Photographs of the Anterior Segment Showing Aphakia with a Membrane (arrow) Across the Pupil. (A) Right Eye. (B) Left Eye.

AS-OCT (1,310 nm, Visante OCT Model 1000; Carl Zeiss Meditec, Dublin, CA) was used to further delineate the lens status. In each eye, membranes that were presumably the anterior and posterior lens capsules were seen in the posterior chamber in place of the lens (Fig. 2). Otherwise, corneas, irises, and angles appeared normal.

Anterior Segment Optical Coherence Tomography Showing Two Intact Membranes (arrows) in Place of the Lens in an Otherwise Normal Anterior Chamber. (A) Right Eye. (B) Left Eye.

Figure 2. Anterior Segment Optical Coherence Tomography Showing Two Intact Membranes (arrows) in Place of the Lens in an Otherwise Normal Anterior Chamber. (A) Right Eye. (B) Left Eye.

Because of the nystagmus, aphakic glasses and contact lenses were not useful and intraocular lens (IOL) implantation was suggested. On the basis of the AS-OCT findings, in-the-bag IOL implantation was planned. Ultrasound biomicroscopy during general anesthesia and intraoperative findings verified the presence of two membranes, with histology confirming them as lens capsules. However, the posterior capsular support was weak and both posterior capsules ruptured intraoperatively, requiring sulcus-fixated IOLs. The latest 6-month postoperative visual acuity was 20/100 unaided in both eyes (right eye: plano / −2.50 D × 95; left eye: plano / −1.75 D × 10).

Discussion

AS-OCT is quick, noninvasive, and produces images of high resolution and reproducibility despite distortions from motion errors,1–4 making it a useful tool in evaluating pediatric and even nystagmic eyes. Although the conventional ultrasound biomicroscopy usually penetrates the iris better than OCT, this case shows that AS-OCT can also help detect lens capsule, which has significant prognostic and management implications in congenital aphakia. The lens capsules suggest the secondary nature of the condition, where lens resorption has taken place after normal embryonic development,5 implying a good visual potential after restoration of the refractive power of the absent lens. Also, although posterior chamber IOL implantation is now widely accepted for pediatric cataracts,6 it can now be done for congenital aphakia where lens capsules are present but a weak posterior capsule may better support a sulcus-fixated than an in-the-bag IOL. Preoperative use of AS-OCT may thus enhance the management of congenital aphakia and possibly supplement ultrasound biomicroscopy in other pediatric anterior segment conditions.

References

  1. Konstantopoulous A, Hossain P, Anderson DF. Recent advances in ophthalmic anterior segment imaging: a new era for ophthalmic diagnosis?Br J Ophthalmol. 2007;91:551–557. doi:10.1136/bjo.2006.103408 [CrossRef]
  2. Baikoff G, Lutun E, Ferraz C, Wei J. Static and dynamic analysis of the anterior segment with optical coherence tomography. J Cataract Refract Surg. 2004;30:1843–1850. doi:10.1016/j.jcrs.2004.05.024 [CrossRef]
  3. Baikoff G, Jodai HJ, Bourgeon G. Measurement of the internal diameter and depth of the anterior chamber: IOLMaster versus anterior chamber optical coherence tomographer. J Cataract Refract Surg. 2005;31:1722–1728. doi:10.1016/j.jcrs.2005.02.030 [CrossRef]
  4. Memarzadeh F, Li Y, Francis BA, Smith RE, Gutmark J, Huang D. Optical coherence tomography of the anterior segment in secondary glaucoma with corneal opacity after penetrating keratoplasty. Br J Ophthalmol. 2007;91:189–192. doi:10.1136/bjo.2006.100099 [CrossRef]
  5. Valleix S, Niel F, Nedelec B, et al. Homozygous nonsense mutation in the FOXE3 gene as a cause of congenital primary aphakia in humans. Am J Hum Genet. 2006;79:358–364. doi:10.1086/505654 [CrossRef]
  6. Fan DS, Yip WW, Yu CB, Rao SK, Lam DS. Updates on the surgical management of paediatric cataract with primary intraocular lens implantation. Ann Acad Med Singapore. 2006;35:564–570.
Authors

From Hong Kong Eye Hospital and the Department of Ophthalmology & Visual Sciences, the Chinese University of Hong Kong, Hong Kong, People’s Republic of China.

Presented as a poster at the World Ophthalmology Congress 2008, Hong Kong, People’s Republic of China, June 28–July 2, 2008.

The authors have no financial or proprietary interest in the materials presented herein.

Address correspondence to Dorothy S. P. Fan, FRCS, MSc, Department of Ophthalmology & Visual Sciences, 3rd Floor, Hong Kong Eye Hospital, 147K Argyle Street, Kowloon, Hong Kong, People’s Republic of China.

10.3928/01913913-20090818-02

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