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Management of Corneal Perforation Vishal Jhanji, MD,1,2,3 Alvin L

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Management of Corneal Perforation Vishal Jhanji, MD,1,2,3 Alvin L SURVEY OF OPHTHALMOLOGY VOLUME 56 NUMBER 6 NOVEMBER–DECEMBER 2011 MAJOR REVIEW Management of Corneal Perforation Vishal Jhanji, MD,1,2,3 Alvin L. Young, MMedSc (Hons), FRCSI,3 Jod S. Mehta, MD,4 Namrata Sharma, MD,5 Tushar Agarwal, MD,5 and Rasik B. Vajpayee, MS, FRCS (Edin), FRANZCO1,5,6 1Centre for Eye Research Australia, University of Melbourne, Australia; 2Department of Ophthalmology and Visual Sciences, The Chinese University of Hong Kong, Hong Kong; 3Department of Ophthalmology and Visual Sciences, The Chinese University of Hong Kong, Prince of Wales Hospital, Hong Kong; 4Singapore National Eye Centre, Singapore; 5Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India; and 6Royal Victorian Eye and Ear Hospital, Melbourne, Australia Abstract. Corneal perforation may be associated with prolapse of ocular tissue and requires prompt diagnosis and treatment. Although infectious keratitis is an important cause, corneal xerosis and collagen vascular diseases should be considered in the differential diagnosis, especially in cases that do not respond to conventional medical therapy. Although medical therapy is a useful adjunct, a surgical approach is required for most corneal perforations. Depending on the size and location of the corneal perforation, treatment options include gluing, amniotic membrane transplantation, and corneal transplantation. (Surv Ophthalmol 56:522--538, 2011. Ó 2011 Elsevier Inc. All rights reserved.) Key words. corneal perforation diagnosis keratoplasty management patch graft therapeutic keratoplasty I. Introduction The selection of an appropriate treatment option is Corneal perforation is a cause of ocular morbidity mostly guided by size and location of the perfora- and profound visual loss.13,119 It is the end result of tion and the status of the underlying disease. various infectious and noninfectious disorders that include microbial keratitis, trauma, and immune disorders. Although of low prevalence in the de- II. Disorders Leading veloped world, it accounts for a large number of to Corneal Perforation cases requiring an urgent surgical intervention in Corneal melting and subsequent perforation is developing countries.111,131 Eyes with corneal per- a classic feature of corneal ulcers that do not respond foration need immediate treatment in order to to medical therapy. One of the most important events preserve the anatomic integrity of the cornea and to leading to corneal thinning and perforation is a breach prevent complications such as secondary glaucoma in the corneal epithelium; however, a few organisms or endophthalmitis. Management of corneal perfo- such as Corynebacterium diphtheriae, Haemophilus aegyp- ration may range from temporary measures, such as tius, Neisseria gonorrhoeae,andN. meningitidis,and application of bandage contact lens and gluing, to Shigella and Listeria species can penetrate an intact definitive treatment such as corneal transplantation. epithelium.95 Occasionally, keratitis can be established 522 Ó 2011 by Elsevier Inc. 0039-6257/$ - see front matter All rights reserved. doi:10.1016/j.survophthal.2011.06.003 MANAGEMENT OF CORNEAL PERFORATION 523 via the corneoscleral limbus by hematogenous spread. associated with an increased risk of corneal perfora- Further alterations in the basement membrane of the tion in microbial keratitis. In that study Staphylococcus epithelial cells may cause persistent epithelial defects. epidermidis was the most common microbe isolated Stromal melting by proteolytic enzymes elaborated by from perforated corneal ulcers.158 altered epithelial cells and polymorphonuclear leuco- cytes has been demonstrated in experimental animals 2. Herpes Keratitis and in vitro in human corneas.51 Descemet’s mem- In herpetic disease corneal perforations are caused brane is an effective barrier to microorganisms. When by necrosis of corneal stroma. Although active viral most of the stroma melts away, the Descemet’s replication may be present in some cases, the host membrane bulges forward, forming a descemetocele. immune response is believed to be the principal cause. In conditions like rheumatoid arthritis, there may be Destruction of the corneal stroma is largely mediated altered stromal collagen that contributes to further by matrix metalloproteinases and collagenases from corneal melting. the polymorphonuclear cells and macrophages.20,42 The major causes of corneal ulceration leading to Recurrent infection with progressive corneal thinning corneal perforation can be broadly grouped as in- further contributes to corneal perforation.42 In fectious, noninfectious (ocular surface-related and necrotizing stromal keratitis, the epithelium breaks autoimmune), and traumatic. down over a dense stromal infiltrate, forming a super- ficial ulcer that may slowly or rapidly deepen, pro- A. INFECTIOUS CORNEAL PERFORATION ducing a descemetocele and subsequent corneal Severe and recalcitrant infectious keratitis is perforation. Close supervision is crucial because these a common cause of corneal perforation. Whereas ulcers may perforate unpredictably with too much bacterial and fungal corneal infections are frequent topical corticosteroid or antiviral therapy. in the developing world, recurrent herpetic keratitis causing stromal necrosis is the major cause of 3. Fungal Keratitis corneal perforation in developed countries.99 Fungal keratitis is more prevalent in the de- veloping world. The rate of progression of fungal 1. Bacterial Keratitis keratitis is slow, but available antifungal therapy is Bacterial keratitis often produces corneal ulcera- not optimal, mainly due to low ocular penetration. 4,61 tion leading to corneal perforation. Most bacte- Overall, one-third of all fungal infections require ria require a break in the corneal epithelium to gain surgical intervention because of treatment failures access to the corneal tissue. Once bacteria gain or corneal perforations.41 Fungi associated with access, cytokines such as interleukin 1 and tumor corneal perforation include Fusarium solani,171,172 necrosis factor (TNF) are released attracting poly- Aspergillus fumigatus, Penicillium citrinum, Candida morphonuclear cells. TNF induces the release of albicans, Cephalosporium, and Curvularia. The rate of pro-inflammatory cytokines from macrophages, corneal perforation in fungal keratitis ranges from polymorphonuclear cells, and T-cells from the 4% to 33%. Lalitha et al reported a perforation rate corneal epithelium and stroma. In the case of of 61% in cases with treatment failures (overall 19% virulent organisms such as Pseudomonas, release of perforation rate).85 enzymes like collagenase accelerates the process of 65,72,165 corneal perforation. The stromal necrosis B. NONINFECTIOUS CORNEAL PERFORATION progresses and the infection extends deeper into the cornea, ultimately causing perforation. The 1. Ocular Surface--Related native imbalance between the cytokines contributes Noninfectious corneal perforation usually occurs to corneal melting even after the bacterial amplifi- in diseases that adversely effect the precorneal tear cation stops. film and other components of the ocular surface. Infection with Pseudomonas aeruginosa generally has Dry eye syndrome is a major contributor to chronic a poor outcome, and corneal perforation ensues epithelial defects. Corneal xerosis in conditions like rapidly.9,90 Various other organisms that have been keratoconjunctivitis sicca results from the depletion isolated include Staphylococcus spp, Proteus spp, of goblet cells. Loss of goblet cells and accessory Streptococcus pneumoniae, Moraxella spp, and Salmonella lacrimal glands leads to alteration of tear composi- spp.22,73,110 A study from north India found that tion and severe dry eye. Chronic epithelial defects outdoor occupation, trauma with vegetative matter, combined with poor healing may lead to sight- central location of corneal ulcer, lack of corneal threatening infectious corneal ulceration, sterile neovascularization, monotherapy with fluoroquino- thinning, and/or perforation. Corneal perforation lone, and failure to start timely management were has been reported to occur in Sjo¨gren syndrome.27 524 Surv Ophthalmol 56 (6) November--December 2011 JHANJI ET AL Corneas in Sjo¨gren syndrome are predisposed to syndrome.46,66,68,69,83,89,96,110,123,160 Corneal melting stromal degradation, ulceration, and consequent may also occur with chemical injuries of the eye.8,97 perforation as a result of diminished tear secretion, Chemical burns cause extensive limbal and conjunc- corneal epithelial breakdown, and enzymatic degra- tival cell destruction.5 Persistent inflammation pre- dation of collagen by inflammatory cells.44 Other vents epithelialization and accelerates ulceration and systemic conditions associated with xerosis include melting with globe perforation. Increase in the vitamin A deficiency, erythema multiforme, and activity of the enzyme collagenase along with ische- benign mucous membrane pemphigoid.11,23,67,133,137 mia leads to corneal melting and is often associated with a poor prognosis. 2. Autoimmune Causes Collagen vascular diseases such as rheumatoid III. Approach to Management arthritis, systemic lupus erythematosus, temporal of Corneal Perforation arteritis, Wegener granulomatosis, sarcoidosis, and inflammatory bowel disease may be associated with A. HISTORY AND CORNEAL WORK-UP 56,121,143 corneal melting. Peripheral ulcerative kera- Corneal perforation requires prompt manage- titis (PUK) is a rare inflammatory disease of
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