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Fuchs Endothelial Dystrophy

INTRODUCTION:
Cornea is dome shaped transparent structure in the eye surrounded by white portion (Sclera). Cornea is made up of 6 layers. The back layer of cornea is known as Endothelium. Endothelium has major role in keeping cornea transparent. Fuchs’ endothelial dystrophy is a non-inflammatory, genetic (autosomal dominant inheritance) dystrophy involving the endothelial layer of the cornea. Patient suffers from glare, halo, and reduced visual acuity due to corneal swelling.

WHAT CAUSES FUCH’S DYSTROPHY?
It is genetic in nature. If you have either mother or father affected, then there is 50% chance that it will be inherited by you. Some spontaneous mutation can cause this without positive family history. Exact cause is unknown, still research is going on to find out cause.

WHAT ARE THE SIGNS AND SYMPTOMS OF FUCH’S ENDOTHELIAL DYSTROPHY?
Signs and symptoms of Fuch’s Endothelial Dystrophy are dependent on the stage of the disease. In early stages patient may be asymptomatic and gets detected on routine eye check-up. Slowly as the eye disease worsens patient may complain of these symptoms-

1.    More common in women

2.    Age: 4th or 5th decade

3.    Blurring of vision (Vision may be worse on humid or rainy days, and better on dry days)

4.    Mild to severe intolerance to light

5.    Pain 

6.    Foreign body sensation

7.    Redness
 

Most common sign of this corneal disease is corneal edema. Initially corneal swelling is more in the mornings but as the disease progresses it can be present throughout the day. 

DIAGNOSIS:
It can be diagnosed on slit lamp examination by an eye specialist. Appearance of Guttata is pathognomonic of fuch’s endothelial dystrophy. Guttae look similar to microscopic mushroom caps on the on endothelial surface of the cornea. Corneal endothelium gets a beaten metal appearance in more advanced stages. Corneal thickness increases in fuch’s dystrophy. Pachymeter reading more than 620 microns suggests endothelial dystrophy. Specular microscopy gives scanned image of endothelial cells with quantitative and qualitative indices which help to diagnose various endothelial layer disorder.

MANAGEMENT:
Progression of fuch’s dystrophy can’t be stopped. Down the line, cornea is destined to get decompensated. Palliative treatment includes hypertonic saline, ointment. Patient can use hair dryer with cold settings at arm’s length to evaporate fluids off cornea. This activity hastens visual recovery in mild cases. In case of pain, Bandage contact lens helps to reduce eye pain. It is better to be under the care of cornea specialist as they can decide at what stage what treatment is needed. 

SURGICAL TREATMENT:

Ultimate treatment is cornea transplantation which can be performed by your eye doctor specialising in cornea. It can be of various types-

DSEK /DMEK (Descemet Stripping Endothelial keratoplasty / Descemet Membrane Endothelial Keratoplasty): This is newest treatment modality of fuch’s dystrophy. In this procedure defective endothelial layer of patient is replaced with healthy donated endothelial layer from donor tissue which can be obtained from eye bank. Prognosis & visual recovery of DSEK is good in fuch’s dystrophy though complications associated with any other organ transplantation like rejection, infection, healing related issues can occur. Patient should be counselled that the potential of graft rejection persistsas long as graft is functioning well and remains clear. Suture & incision induced astigmatism is very low in DSEK & DMEK.


Full Thickness penetrating keratoplasty: This is one of the treatment options. As compared to DSEK, visual recovery is slow in PK. Suture related complications, and astigmatism is more in PK. PK has good prognosis in Fuch’s dystrophy though chances of rejection can’t be ruled out.

Long term medical treatment (e.g. Steroids Drop) post PK or DSEK can cause cataract &/or glaucoma in patient.Considering advances in medical sciences, it is possible to give good vision to patients suffering from advanced Fuch’s dystrophy with above treatment options.