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Forme fruste anterior segment dysgenesis
  1. M R Banitt1,
  2. A Romano1,2,
  3. S Iragavarapu1,
  4. D L Budenz1,
  5. R K Lee1
  1. 1Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, Florida, USA
  2. 2Vision Institute, Federal University of São Paulo, São Paulo, Brazil
  1. Correspondence to RK Lee, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, 900 NW 17th Street, Miami, FL 33136, USA; rlee{at}

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A 38-year-old woman with a history of poor vision in her left eye was referred for evaluation of closed-angle glaucoma. Best corrected visual acuity was 20/25 in the right eye with a manifest refraction of −2.00+2.50×180 and 20/400 in the left eye with +1.00+0.75×090. The anterior chamber was deep centrally with clear lenses bilaterally. The intraocular pressure was 15 mmHg in both eyes.

Slit lamp examination revealed prominent posterior embryotoxon in both eyes and a central disc-shaped posterior corneal stromal haze more prominent in the left eye than in the right eye (figure 1A–F). Gonioscopy demonstrated posterior embryotoxon bilaterally and anomalous appearing open angles with fine areas of peripheral anterior synechiae (figure 1G,H) and high iris processes in both eyes. The posterior segment examination was unremarkable with a normal-appearing 0.3 vertical cup to disc ratio bilaterally. Central corneal thickness was 626 μm and 601 μm in the right and left eyes, respectively.

Figure 1

Photograph of the right eye demonstrating mild central corneal opacity (white arrow) and posterior embryotoxon (black arrows) of the right (A) and left (B) eyes. Slit lamp photograph demonstrating deep central corneal opacity in the right (C) and left (D) eyes and by retroillumination of the right (E) and left (F) eyes. Gonioscopy photographs of the peripheral iris processes to Schwalbe's line in the right (G) and left (H) eyes.

Both eyes of the patient were assessed using in vivo confocal microscopy (Nidek, Inc., Fremont, California, USA). In vivo scanning laser confocal microscopy is an imaging modality that visualises thin serial sections of the cornea without disrupting the living tissue often referred to as optical sectioning. The objective of the microscope is an immersion lens with 60× magnification, and coupling gel was used on the cornea for contact with the microscope objective.


  1. What is the differential diagnosis of the patient?

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  • Funding NIH grant EY016775, Research to Prevent Blindness, and the NIH Core Grant to the University of Miami. The authors have no proprietary or financial interest in any of the work discussed in this manuscript. RK Lee is supported by NIH grant EY016775. The Bascom Palmer Eye Institute is supported by an unrestricted grant from the Research to Prevent Blindness (New York, New York) and the NIH Center grant P30-EY014801.

  • Competing interests None.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.