Case #14 (05-10-2004)

[Andrew_Doan]

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CC: 63 y.o. woman with PAS, ?iris mass?, corectopia, and increased IOP OS.

HPI: Previously healthy woman with progressive corectopia and PAS formation OS over the last year. IOP in mid-20s OS. Patient consulted for evaluation of ?iris mass?.

PMH/FH/POH: non-contributory.

EXAM

* Best corrected visual acuities: 20/20 OD & OS.
* mRx: +1.75+2.00 x 179 OD, +0.75+1.50 x 174 OS
* Pupils: normal OD, see below for OS, no RAPD
* Humphrey's 24-2 VF: full OU
* EOM: normal
* IOP: 15 mmHg OD, 17 mmHg OS
* SLE: Normal OD, see below for OS.
* DFE: Normal OU.
* ON: 0.4 C/D ratio OU (normal).

Pupil OS.

Gonioscopy OS.

Cornea OS.

Close-up of Cornea OS.

Feel free to discuss the following:

What tests should you order (I'll post labs when asked for them)?

What's the differential diagnosis?

What's the diagnosis?

What is the treatment of choice, surgically and/or medically?

 

[Redhawk]

DDx:
1) ICE syndrome
2) Axenfeld-Rieger syndrome
3) Posterior polymorphous dystrophy

The first two can both cause correctopia. Perhaps the "mass" on the iris is actually atrophy, as may be seen with ICE, rather than a true mass.

I'm not sure about the gonioscopy as I haven't actually seen much of it, but this appears similar to pictures I have seen of peripheral anterior synechiae (is that what PAS stands for?).

The appearance of the cornea on SLE appears to show a significant "beaten metal" appearance similar to that of Fuch's (but my monitor is old and dying so it's hard to see🙂) which is typical of ICE.

Axenfeld-Rieger syndrome can display similar clinical findings but is differentiated by its congenital nature, bilaterality and associated systemic features (dental and facial abnormalities).

Posterior polymorphous dystrophy is a rare, bilateral, hereditary endothelial dystrophy
which may have changes of the angle and iris that resemble ICE syndrome. Its differentiating features are that it is bilateral and hereditary and that it manifests with different posterior corneal abnormalities.

Testing:
Specular microscopy?


Question:
If this indeed is ICE, is the 2mmHg difference in IOP prognostic in any way in terms of later development of glaucoma? I realize that she has normal C/D and VF and the pressures are normal, I just don't remember what level of asymmetry is considered normal.

 

[Andrew_Doan]

Testing:
Specular microscopy?

Right Eye

Left Eye

 

[Andrew_Doan]

Discussion is posted: http://webeye.ophth.uiowa.edu/eyeforum/case14.htm

 

[shredhog65]

so this is my first reply to a case....

i'm going to agree with redhawk that this is an ICE syndrome.

chandler's syndrome is a combination of mild pupilary changes (i'm not sure if this would be considered mild or not), irregular corneal endothelium (which this patient has), and normal to mildly elevated IOP (which this patient also has).

cogan-reese syndrome pateints display pupilary abnormalities including corectopia, iris holes, and pigmented nodules on the iris (which this patient has). i am not sure if these patients have a beaten metal cornea appearance.

differential also would include an iris tumor with endothelial decompensation, old trauma (both less likely), and mesodermal dysgenesis syndromes

i would go with chandler's syndrome.

 

[Andrew_Doan]

You are both correct. This is ICE syndrome, and it's early essential iris atrophy.

Chandler's syndrome has only the hammered silver appearance of the cornea.

Cogan-Reese syndrome has the corneal findings and iris nodules.

Essential iris atrophy has the corneal findings and corectopia. Late in the disease, patients with essential iris atrophy will present with PAS, melt holes, and stretch holes (see below):

Photography by H. D. Riley, O.D. (Indiana University of Optometry)
borrowed from: http://www.opt.indiana.edu/

As physicians, we are either clumpers or splitters. There are groups that clump diseases together, and there are others who like to split each disease into their own categories. The ICE syndromes, although we aim to "split" them into distinct categories, are really a spectrum of diseases.

Without the early melt hole and PAS, this patient could have easily been diagnosed with Chandler's syndrome. In the end, it really doesn't matter because all of these patients need to be followed for glaucoma.

In regards to Redhawk's question, a small difference in IOP doesn't provide a prognosis for the risk of developing glaucoma. The appearance of the optic nerves, IOP diurnal curve, gonioscopy findings, and visual fields are better indicators of the development of glaucoma.

Perhaps GlaucomaMD from Bascom Palmer can shed some light on which ICE syndromes are at higher risk of developing glaucoma?