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Posterior Segment Mass in a Woman With a Subluxed Crystalline Lens

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 72-year-old woman was referred for evaluation of a choroidal mass and subluxed crystalline lens in the right eye. The patient had a history of congestive heart failure, cleft lip surgery as an infant, and right eye amblyopia. She reported occasional floaters and flashes in the right eye but denied any ocular complaints in the left eye. On examination, her visual acuity measured 3/200 OD and 20/30 OS, stable compared with her baseline. Her intraocular pressure was 18 mm Hg OD and 14 mm Hg OS. Right eye slitlamp biomicroscopy showed an irregular pupil, aphakia, and a pigmented mass behind the temporal iris (Figure 1A). Ophthalmoscopic examination results revealed a subluxed white cataractous lens in the inferior vitreous base (Figure 1B). An evaluation of the left eye yielded normal results aside for a moderate cataract. B-scan ultrasonography results showed the 2 masses described previously; both were moderately reflective, oval, and separate from the wall of the eye. Ultrasonography biomicroscopy (UBM) confirmed that the temporal mass and the inferiorly subluxed lens were separate from the iris and ciliary body and were similar to each other in size, shape, and density.

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Congenital biphakia with double subluxed crystalline lenses

C. Magnetic resonance imaging of the brain and orbits

This is a case of a patient who was referred for evaluation of a likely choroidal mass. However, B-scan ultrasonography and UBM results show that the mass was adjacent to, but separate from, the ciliary body temporally, and the presence of a subluxed lens in the inferior vitreous further complicates the diagnosis.

Because of the uncertainty of the etiology of the mass as to whether it is benign or malignant or even from which tissue it arose, treatment with plaque radiotherapy would not be appropriate. With the patient’s ocular history of amblyopia and poor visual prognosis, there was no visual benefit to removing the subluxed lens. Further imaging of the right eye with magnetic resonance imaging (MRI) was obtained and reaffirmed that the mass was similar in shape and density to the inferior subluxed lens (Figure 2). The MRI, along with autofluorescence, B-scan ultrasonography, and UBM, led us to diagnose congenital biphakia with 2 subluxed lenses1 and we elected observation as opposed to a biopsy.

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Article Information

Corresponding Author: Geoffrey G. Emerson, MD, PhD, Retina Center of Minnesota, 710 E 24th St, Ste 304, Minneapolis, MN 55404 (geoffrey.g.emerson@gmail.com).

Published Online: June 27, 2019. doi:10.1001/jamaophthalmol.2019.1500

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information. We also thank Stephen E. Jens, COA, Retina Center of Minnesota, and Natalia Y. Kramarevsky, MD, New Ulm Medical Center, for their individual contributions. These individuals were not compensated for their contributions.

References
1.
Hemady  RK, Blum  S, Sylvia  BM.  Duplication of the lens, hourglass cornea, and cornea plana.  Arch Ophthalmol. 1993;111(3):303. doi:10.1001/archopht.1993.01090030021013PubMedGoogle ScholarCrossref
2.
Grey  RHB, Rice  NSC.  Congenital duplication of the lens.  Br J Ophthalmol. 1976;60(10):673-675. doi:10.1136/bjo.60.10.673PubMedGoogle ScholarCrossref
3.
Gerkowicz  K, Toczolowski  J.  Biphakia congenita [in German].  Klin Monbl Augenheilkd. 1983;183(2):136-137. doi:10.1055/s-2008-1054890PubMedGoogle ScholarCrossref
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Lyford  JH, Roy  FH.  Arhinencephaly unilateralis, uveal coloboma, and lens reduplication.  Am J Ophthalmol. 1974;77(3):315-318. doi:10.1016/0002-9394(74)90736-3PubMedGoogle ScholarCrossref
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Evans  K, Hickey-Dwyer  MU.  Cleft anterior segment with maternal hypervitaminosis A.  Br J Ophthalmol. 1991;75(11):691-692. doi:10.1136/bjo.75.11.691PubMedGoogle ScholarCrossref
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Stefani  FH, Hausmann  N, Lund  OE.  Unilateral diplophthalmos.  Am J Ophthalmol. 1991;112(5):581-586. doi:10.1016/S0002-9394(14)76862-XPubMedGoogle ScholarCrossref
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