eMedicine Specialties > Ophthalmology > Cornea

Dystrophy, Fuchs Endothelial: Treatment & Medication

Author: Daljit Singh, MBBS, MS, DSc, Professor Emeritis, Department of Ophthalmology, Guru Nanak Dev University, Amritsar, India; Director, Daljit Singh Eye Hospital
Coauthor(s): Ravijit Singh, MD, Consulting Staff, Department of Ophthalmology, Daljit Singh Eye Hospital
Contributor Information and Disclosures

Updated: Feb 2, 2007

Treatment

Medical Care

• Patients with clear corneas need no treatment. It is only when the corneal decompensation starts that medical treatment becomes necessary. This treatment is necessary until it is not possible to preserve good vision; at that point, keratoplasty is necessary.
• Dehydrating agents
  • Sodium chloride 5% eye drops are instilled 4-6 times during the day, especially in the early hours of the day and less frequently in the evening. Sodium chloride ointment is used at bedtime.
  • Glycerine can be used for diagnostic purposes. It causes rapid dehydration of the cornea and clears the vision. Certain patients are able to use it for therapeutic purposes, but it is rather uncomfortable. It is instilled many times a day, as needed.
• Use of warm dry air (evaporation): A hair dryer, kept at arm's distance, can be used to blow warm air over the cornea for 5-10 minutes upon awakening. Drying of the cornea may improve the vision of the patient for some time.
• Lowering the IOP is useful when it is even mildly raised. It occasionally helps even when the pressure is normal, especially in borderline cases of corneal decompensation.
• Topical nonsteroidal anti-inflammatory drug (NSAID): Diclofenac 0.1% and ketorolac 0.5% drops may help to alleviate symptoms (eg, itching, burning, gritty sensation) but may increase the risk of poor epithelial healing and subsequent corneal melting.
• Supportive treatment for ruptured bullae
  • Soft contact lenses can be useful in cases of bullae formation.
  • Cycloplegics, local antibiotics, and pad and bandage treat the eye for a couple of days.
  • Retrobulbar injection of absolute alcohol is useful to patients with painful, totally blind eyes.

Surgical Care

Failing vision in the presence of epithelial edema and stromal haze, which cannot be treated by the instillation of 5% sodium chloride drops and ointment, necessitates recourse to surgery. A selection has to be made between the following 2 options: (1) keratoplasty alone, when no cataract formation is present, or (2) if cataract is present and adds significantly to visual disability and specular endothelial microscopy results suggest the need for a corneal graft, then a combined corneal transplant, cataract extraction, and lens implant procedure should be performed.

If precautions are taken to protect the endothelium during surgery, most cases of confluent guttata, without corneal symptoms, do well with cataract and lens implant surgery.

In eyes with pain, but little or no visual potential, anterior stromal micropuncture, excimer laser phototherapeutic keratectomy, amniotic membrane graft, or a conjunctival flap can be considered.

• Preoperative management for a corneal transplant
  • Phakic patients: Contract the pupil with 3 instillations of 1-2% pilocarpine drops every 5 minutes.
  • Triple procedure: 3 drops each of 1% cyclopentolate, 2.5% oxymetazoline, and flurbiprofen sodium, preoperatively.
  • For high-risk vascularized cornea, oral prednisolone 1 mg/kg/d, is started 4 days preoperatively and tapered over 2 weeks postoperatively.
• Anesthesia
  • Local anesthesia with 50/50 mixture of 0.75% bupivacaine and 2% lidocaine and 150 U of hyaluronidase.
  • Anesthesia may be retrobulbar or peribulbar. Good hypotony should be obtained with a mechanical pressure device (eg, Honan balloon, Super Pinky ball, mercury bag).
  • After thoroughly ascertaining cardiovascular status, general anesthesia may be used in selected cases.
• Preparation of the recipient
  • Position the head to obtain good red reflex and optical centration. Ocular compression and lateral canthotomy may minimize external pressure on the eyeball. Jaffe wire lid speculum is used to open the eye.
  • Flieringa ring of appropriate size is fixed 2 mm from the limbus with 4-6 interrupted 6-0 black silk sutures. Four 4-0 silk sutures are used to fix the ring to the drape. Determine donor size. A size between 7.75 and 8.25 mm typically is selected. The donor button should be 0.25-0.50 mm larger.
• Preparation of the donor eye
  • Allow the donor eye to come to room temperature, about 30 minutes prior to use.
  • Use a posterior punch trephine to cut the button from the endothelial side.
  • Transfer the button, endothelial side up, to a Petri dish containing a few drops of balanced salt solution or corneal preservation medium. Cover the disc.
• Preparing the recipient bed
  • The cornea is dried with the cellulose sponge.
  • The center of the cornea is marked with an angled Sinskey hook.
  • Use the cross-wires to center the trephine. The centration is evaluated by lightly touching the cornea with the trephine.
  • A partial thickness incision is made by turning the trephine 8 quarter turns in a clockwise direction. This procedure creates a 0.5 mm deep incision.
  • For later use, make a stab incision at the limbus to form the anterior chamber.
  • To mark the entry of sutures on the recipient cornea, use 8 or 12 blade radial incision marker.
  • Enter the anterior chamber along the wound margin. Use a delicate scissors to cut the rest of the cornea, cutting perpendicular to the iris plane.
  • Ensure that the Descemet membrane is removed.
  • Remove any tags along the edge.
  • Place viscoelastic material over the iris-lens diaphragm.
  • Transfer the donor button to the prepared site, using a 0.12-mm forceps.
• Graft suturing
  • During the procedure, use the paracentesis incision to deepen the anterior chamber with saline. Place the first suture at the 12-o'clock position to stabilize the corneal button. Place sutures (in this order) at the 6-, 3-, and 9-o'clock positions. Alignment should be completed most carefully, especially at the 6-o'clock position.
  • Take bites at 1.5 mm, on both sides of the incision line. The depth should be 50-90% of the corneal thickness. Bury the knots.
  • Use a 12-bite running suture of 10-0 nylon in an antitorque manner. Adjust tension to equalize apposition throughout the wound. Sixteen interrupted sutures also may be used.
  • Intraoperative keratometry may be performed. Deepen the anterior chamber with saline. Remove viscoelastic. Test the wound for tightness. Then, bury the knot.
  • Remove the Flieringa ring.
  • Give a subconjunctival injection of vancomycin (1 mg), dexamethasone (1 mg), and gentamicin (20 mg).
  • Patch the eye after applying dexamethasone, polymyxin B, and bacitracin ointment. Apply a plastic shield.
• Postoperative management
  • On the first postoperative day, check IOP and examine the epithelium integrity and wound anatomy. If the pressure is higher than 30 mm Hg, start oral or topical carbonic anhydrase inhibitors and local beta-blockers. Prescribe local broad-spectrum antibiotic eye ointment once a day for 1 week; longer for complicated cases. Topical prednisolone acetate 1%, 6 times per day for 10 days, 5 times per day for the next 10 days, and 4 times per day for the next 2 months. Then, the dose is tapered by 1 drop per day every 2 months. Thereafter, the dose is 1 drop/day for about 1 year.
  • Examine the patient every 15 days to look for a sign of graft rejection. Interrupted sutures are removed, starting 2-3 months postoperatively. Remove running sutures after 12 months. Selectively remove interrupted sutures until the astigmatism is less than 3 diopters. Suture removal occurs later in eyes with only interrupted sutures.
• Technical alternatives
  • Posterior lamellar keratoplasty: Most of the early cornea-decompensated Fuchs endothelial dystrophy cases are candidates for this kind of surgery. In this technique, a stromal pocket is created through a 9-mm scleral incision. The pocket is made at about 80% of the stromal depth. A 7.5-mm posterior lamellar disc is excised. This disc removes the disease endothelium, Descemet membrane, and posterior stroma. A same size donor disc replaces this disc. No suture is applied to position this disc. However, the scleral incision is sutured. This technique has many advantages, to include the following: less surgical time, reduced intraoperative complications, less risk of high astigmatism, faster visual recovery, fewer visits for suture management, elimination of suture-induced vascularization and graft reaction, and fewer side effects of steroid therapy.
  • Cataract surgery: The presence of cornea guttata in a clear cornea, with normal thickness, is not a reason to avoid standard cataract surgery. However, extra care is needed to protect the corneal endothelium during surgery. Viscoelastic coating of the endothelium should be maintained throughout the procedure. If the cataract is brunescent, it may be prudent to perform a quick extracapsular surgery through a large incision. Phacoemulsification may be risky if the endothelial reserve is reduced. Rather than Nd:YAG laser capsulotomy, manual capsulotomy by pars plana route is safer for the corneal endothelium.

Diet

No dietary restrictions exist.

Activity

Advise the patient to avoid any kind of trauma to the eye. The eye may be cleaned with boiled and cooled wet cotton swabs. After a period of 2 weeks, the patient can take a brisk walk, watch television, and resume any visual task that the eye is capable of with or without refractive correction.

Medication

Patients undergoing keratoplasty may require carbonic anhydrase inhibitors before and after the surgery. They are highly effective in reducing the IOP to desired levels.

Carbonic anhydrase inhibitors

Carbonic anhydrase (CA) is an enzyme found in many tissues of the body, including the eye. Catalyzes a reversible reaction where carbon dioxide becomes hydrated and carbonic acid dehydrated. By slowing the formation of bicarbonate ions with subsequent reduction in sodium and fluid transport, it may inhibit CA in the ciliary processes of the eye. This effect decreases aqueous humor secretion, reducing IOP.

Acetazolamide (Diamox)

Because of highly predictable effect on IOP. Reduces rate of aqueous humor formation by inhibiting enzyme carbonic anhydrase, which results in decreased IOP. Available in oral and parenteral forms.

Corticosteroids

Are most effective in treating inflammations and preventing and treating graft reactions, following keratoplasty operation.

Prednisolone (AK-Pred, Econopred, Prelone)

Acting on the nucleus of the cell, the steroids produce certain proteins that are immunosuppressive and prevent the production of inflammatory mediators. By their action on the cell wall, they decrease the release of PG. The net result is suppression of inflammation and immune reaction. These effects are obtained through such diverse mechanisms of action as anti-inflammatory, antiallergenic, antiexudative, antiangiogenic, and antiproliferative. Although they have systemic effects, their main action is exerted at the site of inflammation. Therefore, topical application is useful to prevent immune reaction and inflammation.
Has a short biological life of 12-36 h, therefore, interferes less with physiological processes.

Ocular lubricants

Hypertonicity of sodium chloride is used to treat corneal edema.

Sodium chloride 5% (Muro 128, Muroptic-5)

Sterile ophthalmic solution and ointment used to draw water out of cornea of the eye.

Nonsteroidal anti-inflammatory agents

The inhibition of prostaglandin synthesis, results in vasoconstriction, a decrease in vascular permeability, leukocytosis, and a decrease on IOP. However, these agents have no significant effect on IOP.

Diclofenac sodium (Voltaren)

Diclofenac sodium is designated chemically as 2-[(2,6-dichlorophenyl) amino] benzeneacetic acid, monosodium salt, with an empirical formula of C₁₄ H₁₀ C_l2 NO₂ NA. One of a series of phenylacetic acids that has demonstrated anti-inflammatory and analgesic properties in pharmacological studies. Believed to inhibit the enzyme cyclooxygenase, which is essential in the biosynthesis of prostaglandins.

Ketorolac tromethamine 0.5% (Acular)

Member of the pyrrolo-pyrrole group of NSAIDs. When administered systemically, has demonstrated analgesic, anti-inflammatory, and antipyretic activity. Mechanism of action is believed to be due, in part, to its ability to inhibit prostaglandin biosynthesis.

Cycloplegics/mydriatics

Instillation of a long-acting cycloplegic agent can relax any ciliary muscle spasm that can cause a deep aching pain and photophobia.

Cyclopentolate hydrochloride 1% (AK-Pentolate, Cyclogyl)

An anticholinergic prepared as a sterile, borate buffered solution for topical ocular use. Prevents muscle of ciliary body, and sphincter muscle of iris, from responding to cholinergic stimulation. Induces mydriasis in 30-60 min and cycloplegia in 25-75 min.
Use has been associated with psychotic reactions and behavior disturbances in pediatric patients.

• Search for CME/CE on This Topic »

• Dystrophy, Map-dot-fingerprint (Ophthalmology)
• Dystrophy, Crystalline (Ophthalmology)
• Muscular Dystrophy (Orthopedic Surgery)
• Reflex Sympathetic Dystrophy (Rheumatology)

• Antisense Molecule Shows Promise in Duchenne Muscular Dystrophy
• Cyclophilin Inhibitor Shows Promise in Mouse Model of Muscular Dystrophy
• Recipient-Related Risk Factors for Corneal Transplant Failure Identified

• Prevalence of Duchenne/Becker Muscular Dystrophy among Males Aged 5–24 Years â€â€� Four States, 2007
• Abnormally Tall R Waves in V1
• Hair and Nail Disorders of Childhood