A 35 year old presents with a painful eye.
The IOP is raised.
The eye is enucleated.
There are 3 figures.
1. What does fig 1 demonstrate?
2.What is the arrow pointing at in Fig 2.
3. What does Fig 3 show?
4. What is the likliest clinico-pathological diagnosis.
1. Figure 1 shows broad adhesion to the retrocorneal surface, via stromal strands.
2. Prominent, nodular Schwalbe’s line or posterior embryotoxon.
3. Loss of retinal ganglion cells, with preservation of the inner nuclear layer, indicating glaucomatous atrophy.
4. The pathology is that of an anterior chamber dysgenesis. The combination of a posterior embryotoxon, with broad irido-corneal adhesion via iris strands, and the clinical observation of raised IOP, is highly suggestive of Axenfeld-Rieger anomaly (AXRA).
See the review by Idrees et al ‘A review of the anterior chamber dysgeneses’ in Surveys of Ophthalmology, volume 51 (3) 2006.