Management of Descemetocele and Corneal Perforation

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Corneal ulceration leading to descemetocele formation and corneal perforation can result from infectious, autoimmune, or traumatic and require prompt treatment to prevent significant visual morbidity.

Disease Entity

Corneal perforations and descemetoceles can result from traumatic, infectious or inflammatory conditions.

Disease

Corneal ulceration starts with defect in the corneal epithelial layers that can progress to involve the stroma and ultimately to full thickness corneal perforation. Descemetocele involves herniation or anterior bulging of an intact descemet membrane through a defect of the overlying corneal stromal and epithelial layers.

Etiology

Infectious

Bacterial

  • Pseudomonas aeurginosa
  • Streptococcus species
  • Staphylococcal species
  • Moraxella species
  • Salmonella species


Viral

  • Herpes simplex
  • Herpes zoster


Fungal

  • Fusarium solani
  • Aspergillus fumigatus
  • Penicillium citrinum
  • Candida albicans
  • Cephalosporium
  • Curvularia
Inflammatory

Collagen Vascular Disease

  • Rheumatoid arthritis
  • Systemic lupus erythematosus
  • Granulomatosis with polyangiitis (formerly Wegener's)
  • Polyarteritis nodosa
  • Sarcoidosis
  • Inflammatory bowel disease


Mooren’s ulcer

Trauma
  • Penetrating
  • Chemical injury
  • Surgical
Other

Exposure keratitis Degeneration and Ectasia

  • Keratoconus
  • Terrien’s marginal degeneration
  • Pellucid marginal degeneration


Dry Eye and Xerosis

  • Keratoconjuncitivis sicca
  • Vitamin A deficiency
  • Ocular cicatricial pemphigoid
  • Stevens Johnson Syndrome
  • Sjogren’s syndrome
  • Graft-versus-host disease


Signs

Descemetocele:

  • Descemet folds at base of ulceration
  • Central clear zone within areas of thinning


Perforation:

  • Uveal prolapse
  • Positive Seidel test
  • Flat or shallow anterior chamber

Symptoms

  • Sudden drop in visual acuity
  • Ocular pain
  • Excess "tear" production

Clinical diagnosis

Care should be taken to minimize pressure on the globe while examining the patient with suspected descemetocele or corneal perforation. Uveal prolapse or a positive Seidel test are definitive for confirming the diagnosis of corneal perforation. The Seidel test can be performed with a sterile fluorescein strip that is saturated with a small amount of sterile saline and painted over the suspected area of perforation. A positive test results in dilution of the fluorescein dye when viewed under cobalt blue light. Of note, uveal prolapse may result in plugging of the defect resulting in a Seidel negative test and a formed anterior chamber.

Failure to diagnose this condition can result in further corneal damage, loss of anterior chamber integrity, endophthalmitis, secondary glaucoma and cataract development, profound vision loss and ultimately loss of the eye.

Management

Available treatments of descemetoceles and corneal perforations range from temporary or short-term solutions to more definitive repair. The choice of treatment will depend on the underlying disease in addition to the size, extent of stromal involvement, location of the perforation and visual potential. Multiple treatments are often employed at the same time or in a staged process.

Medical therapy

  1. Frequent lubrication, punctal occlusion and/or tarsorrhaphy can promote re-epithelialization in cases of aqueous deficient dry eye related corneal ulceration and impending corneal perforation.
  2. Topical and/or systemic antibiotics are often used for prophylaxis or therapeutically in cases of infections.
  3. Aqueous suppressant topical medications can reduce intraocular pressure, decrease outflow from the site of defect and encourage healing in cases where the anterior chamber is formed.
  4. Bandage contact lens [BCL] can promote corneal healing and re-epithelialization by protecting new epithelial cells from repeated disturbances from eyelid blinking and eye movements. This treatment is useful in non-infectious impending or small corneal perforations or lacerations that have good apposition of edges and alignment, and no prolapse of uveal tissue. This can also be useful in peripheral defects or dry eye cases related to aqueous deficiency. For corneal penetrating wounds, soft, hydrophilic contact lenses are kept in place for 4 to 7 days, closing off the leak initially by epithelialization and later by scar formation. Contact lenses can be kept in place for longer periods of time if needed and used in conjunction with topical (preferably) and/or systemic antibiotics and topical aqueous suppressant medications.
  5. Anti-collagenase medications, such as systemic tetracyclines, independent from their antimicrobial properties, can be considered as an adjunctive treatment in cases of corneal ulceration. Systemic tetracyclines inhibit matrix metalloproteinases [MMP] produced by neutrophils, epithelial gelatinases, α1-antitrypsin degradation and scavenge reactive oxygen species that can potentiate further collagen breakdown and retard corneal healing.
  6. Anti-inflammatory medications including topical steroid medications, such as prednisolone acetate, can be a useful adjunct for corneal ulcers with concomitant inflammation. Steroids may help dampen inflammation by blocking the entry of polymorphonuclear lymphocytes and prevent the accumulation of collagenases and ultimately promoting re-epithelialization and possibly improving final best-corrected visual acuity by reducing scaring. Of note, in cases of corneal ulceration related to bacterial keratitis, there is debate among whether or not topical steroids started 48 hours after antibiotic treatment has begun is beneficial. A recent large clinical trial, The Steroids for Corneal Ulcer Trial [SCUT], did not find any significant difference or benefit in starting topical prednisolone sodium phosphate 1.0% for 3 weeks 48 hours after topical moxifloxacin was started in terms of best-corrected visual acuity at 3 months, size of scar or infiltrate at 3 months, or difference in epithelial healing as compared to placebo. Steroids are contraindicated in fungal and HSV keratitis.
  7. Vitamin C, topical and systemic, stimulate collagen production and can been used as an adjunct for the treatment of corneal ulceration. In particular, aqueous ascorbic levels are reduced in cases of alkali injuries and can be prolonged in more severe injuries.
  8. Immunosuppressive agents, such as oral or topical cyclosporine, systemic methotrexate, cyclophosphamide, rituximab or infliximab, may be helpful to control the underlying disease process in cases of progressive non-infectious corneal ulceration secondary to corneal inflammatory diseases. Immunosuppressive agents are often used in conjunction with systemic steroids and/or non-steroidal anti-inflammatory medications [NSAIDs].

Surgery

  1. Direct suturing, using 10-0 monofilament nylon sutures, for small defects or lacerations in cases of trauma related corneal perforations.
  2. Corneal gluing with cyanoacrylate glue or fibrin glue can be considered for impending corneal perforations or frank corneal perforations that are small (< 3 mm), concave, and located away from the limbus. Gluing is also a useful technique as a temporary measure for infectious perforations to allow for antimicrobial treatment prior to more definitive management with penetrating keratoplasty. Cyanoacrylate glue has the advantage of being bacteriostatic and longer lasting than fibrin glue. It is also believed to inhibit polymorphonuclear lymphocytes and the production of collagenases, which may halt the corneal melting process. The surgical technique using cyanoacrylate corneal patch application has been well described by Vote et al. In addition, a surgical technique using a double drape tectonic patch with cyanoacrylate glue has been described by Gandhewar et al. for management of corneal perforations complicated by uveal prolapse.
  3. Amniotic membrane transplant [AMT] in combination with fibrin glue or 10-0 polyglactin sutures can be used for impending or small frank corneal perforations (< 3 mm). AMT can be useful in cases of more peripherally located defects and is currently reserved for cases of refractory corneal ulceration or perforations that have failed medical treatment. Human placental amnion is composed of a single layer of epithelial cells, a basement membrane and a layer of avascular stroma. It has been found to have multiple growth factors including fibroblastic growth factor, hepatocyte growth factor and transforming growth factor β that may help promote tissue repair, remodeling, and epithelial healing and reduce inflammation, vascularization and scarring. Multiple techniques have been described: 1) Overlay AMT described by Letko et al. uses a single layer graft, 12 to 14 mm in diameter, and fashioned in place with 10-0 polyglactin sutures to cover the entire corneal, limbal and prelimbal surfaces; 2) Inlay AMT described by Lee et al. uses a single layer graft that is cut to size to fit the area of the defect and fashioned in place with 10-0 polyglactin sutures; 3) AMT fashioned in place with human fibrin glue described by Duchesne et al.; 4) Multilayer AMT technique described by Kruse et al. fills the defect by stacking several small cut pieces of AMT prior to coverage with a larger piece of AMT and fashioned in place with 10-0 nylon sutures; 5) “Swiss roll” technique described by Chan et al. fills the defect by rolling a piece of AMT and fashioning it in place with 10-0 nylon sutures.
  4. Corneal transplantation includes full thickness (penetrating) keratoplasty, corneal patch grafts and partial thickness (lamellar) keratoplasty. Penetrating keratoplasty are reserved for large corneal perforations (> 3 mm) not amenable to other treatments, flat anterior chamber with iris prolapse, or failure of medical treatment or corneal gluing. If cornea ulceration is due to infection, it is beneficial to delay penetrating keratoplasty until aggressive topical fortified antibiotics has been started 24 to 48 hours prior, however, this must be balanced with impending perforation with its risk of endophthalmitis or lateral extension with the risk of infectious scleritis. Temporizing measures including corneal glue or BCL should be considered until infection or inflammation can be controlled prior to performing a penetrating keratoplasty. Corneal patch grafts, either lamellar or full-thickness, can be used temporarily for central corneal perforations or permanently for peripheral corneal perforations or descemetoceles. Ideally, all necrotic tissues and epithelium are removed from the bed of the ulcer or margins of the corneal perforation until a viable tissue is reached before a lamellar disc of donor cornea or patch graft is sutured in place with interrupted 10-0 nylon sutures.
  5. Partial or total conjunctival flaps are reserved for chronic, indolent ulcers that do not heal despite medical or surgical interventions. Total conjunctival flaps such as the Gundersen flap are usually considered for eyes that have poor visual potential. Conjunctival flaps helps to promote healing, control pain and decrease the need for frequent medication. A thin flap of conjunctiva is freed up from the underlying tissues, avoiding Tenon’s capsule, pulled over the affected cornea and secured into place using sutures.
  6. Conjunctival resection can be considered for treating marginal corneal ulcerations secondary to autoimmune diseases such as those related to collagen vascular diseases or Mooren’s ulcer. Approximately 3 mm of conjunctiva is excised posterior to the corneoscleral limbus and parallel to the ulcer.

Prognosis

Tissue adhesives alone may be successful in treatment of corneal perforations and can eliminate the need for further surgical therapy. Success rates range from 44% in a study reported by Weiss et al. to as high as 79% in patients treated with fibrin glue and 86% in patients treated with cyanoacrylate glue for corneal perforations < 3mm at the three month follow-up visit reported by Sharma et al. Re-application of glue in the study performed by Sharmaet al. was required in about 30% of patients. Complications of cyanoacrylate glue may include corneal neovascularization, giant papillary conjunctivitis, elevated intraocular pressures, glaucoma and worsening of infectious keratitis. A trend towards lower enucleation rates (6% vs. 19%) was noted by Hirst et al. with the introduction of tissue adhesives.

Amniotic membrane transplant in corneal ulcers have a 70 to 90% success rate in several small studies with a mean epithelial healing time of 3 to 4 weeks. A high risk for failure was reported in patients with corneal ulcers related to neurotrophic keratitis, ocular cicatricial pemphigoid, Stevens Johnson syndrome and rheumatoid arthritis.

Penetrating keratoplasty for perforated or predescemet corneal ulcers are effective at restoring integrity to the anterior chamber, saving the globe and providing useful vision. In a study by Jonas et al. improvement in post-operative best-corrected visual acuity was attained in 90% of eyes (mean follow-up 14.5 months) with approximately 17% requiring a second transplant for recurrent corneal ulcer. Corneal transplant rejection rates for penetrating keratoplasty are estimated at 20% with higher rates for peripherally placed grafts due to their proximity to limbal blood vessels and lymphatics. Lamellar keratoplasty are found to have lower rejection rates and can be a good option for peripherally located perforations. A study by Huang et al. found rejections rates for lamellar keratoplasty to be approximately 10% for peripheral corneal perforations.

Additional Resources

References

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