Case #5 (02-27-2004)

[Andrew_Doan]

CC: 35 y.o. woman with c/o intermittent blurry vision OU lasting hours.

HPI: Patient was sent to neuro-ophthalmology for evaluation of blurry vision OU. The patient c/o of decreased vision upon awakening that is not associated with pain. She stated that it's difficult for her to read. The patient also mentioned that her vision seems to clear up as the day progresses.

EXAM:
Vision: 20/25 OU at distance and near.
Pupils: 5 mm in dark, 2 mm in light, no RAPD.
Extraocular Motility: Full motility without pain.
IOP: 18 mmHg OU.
Visual Field: Full OU.
DFE: normal macular, vessels, and periphery OU.
SLE: notable for a beaten metal appearance of the corneal endothelium OU

Slit beam through cornea. Notice the imperfections of the corneal endothelium on the left side of the beam.

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?

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[TomOD]

Andrew,

Is this best-corrected acuity of 20/25 in each eye.....is the cornea appearance bilateral? And how long has her symptoms been occuring?

Family ocular history?

I'd be interested in her pachymetry.

 

[Andrew_Doan]

Originally posted by TomOD
Andrew,

Is this best-corrected acuity of 20/25 in each eye.....is the cornea appearance bilateral? And how long has her symptoms been occuring?

Family ocular history?

I'd be interested in her pachymetry.

20/25 is the best corrected visual acuity.

Cornea appearance is bilateral.

Symptoms have been occuring for 6 months.

Mother had a similar problem.

Pachymetry notable for corneal thicknesses of 560 and 565.

 

[Redhawk]

It sounds like Fuch's dystrophy due to endothelial cell loss. They have worse vision in the morning I guess because of the eyes being closed all night causing a buildup of corneal edema (since the endothelium helps keep the cornea dry and clear). During the day, with the eyes open, the cornea becomes a little more dehydrated. You can treat it with some dehydrating ointments or even with a hair dryer in the earlier symptomatic stages prior to PK.

Differential: the wonderful wold of corneal diseases of which I currently know little but hopefully more eventually....

Can you do specular microscopy to check the endothelium? It might show decreased endothelial cells consistent with Fuch's.

 

[Richard_Hom]

Total Cholesterol?
Triglycerides?
BUN?
Richard_Hom

 

[Andrew_Doan]

Originally posted by Redhawk
Can you do specular microscopy to check the endothelium? It might show decreased endothelial cells consistent with Fuch's.

Specular microscopy showed very low endothelial cell count. Less than 1800 cells/mm3. Most people have more than 3000 cells/mm3.

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We didn't check any labs because the diagnosis was made clinically.

 

[Redhawk]

Are the dark areas on the SM guttae (outgrowths of D's membrane) or do they simply indicate dropped endothelial cells (or both)?

 

[Andrew_Doan]

Originally posted by Redhawk
Are the dark areas on the SM guttae (outgrowths of D's membrane) or do they simply indicate dropped endothelial cells (or both)?

Kresge Eye Institute is going to be proud to have you as a resident.

The dark areas are regions where the endothelial cells have died. Because these cells are terminally differentiated cells, they do not divide, so the remaining cells have to stretch to cover more area.

Guttae can be there, but it's hard to tell with a 2-D study. Guttae are seen on histo-pathology with PAS that stain Descemet's membrane (basement membrane of the corneal endothelium). There are outpouchings of Descemet's. The finding of guttae on histo-pathology distinquishes Fuch's from pseudophakic bullous keratopathy resulting from endothelium decompensation after cataract or intraocular surgery.

 

[Andrew_Doan]

Most of the key features of this disorder were discussed above. Here is a link with more information:

http://www.eyemdlink.com/Condition.asp?ConditionID=204