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Eye (2018) 32, 7–8 © 2018 Macmillan Publishers Limited, part of Springer Nature. All rights reserved 0950-222X/18 www.nature.com/eye

The role of COMMENT keratoprostheses

Eye (2018) 32, 7–8; doi:10.1038/eye.2017.287; requirement for a suitable tooth but the retention published online 1 December 2017 and visual results are no match. In most cases, OOKP surgery is performed only in one eye, with the other eye reserved as Corneal blindness is a major cause of global spare, in case the procedure fails in the first eye. blindness, second only to . For many Contraindications include age less than 17 years, patients, could offer a smokers, , eyes with no light second chance of sight. However, in some cases perception, and . Patients (eg, in patients with multiple graft failure, severe must have at least one good canine or premolar chemical burns and autoimmune diseases such tooth, minimal gum disease, and, preferably, as Stevens-Johnson syndrome and ocular reasonably good dental hygiene. This would mucous membrane pemphigoid) the ocular enable a suitable tooth, root and surrounding environment is too hostile for a corneal graft to jaw bone to be harvested to create and adequate survive. Keratoprostheses offer these patients lamina. Patients are made aware of the severity the hope and prospect of visual rehabilitation.1 of their condition and must fully understand the In recent decades multiple keratoprostheses major surgery required and the potential for have been pioneered and developed.2 Many severe complications. Moreover, they must be have come and gone though and only two are prepared for the altered cosmetic appearance. principally used in clinical practice: the Boston Lifelong follow-up is required and patients must be 8 Keratoprosthesis ‘KPro’ type I (Massachusetts highly motivated to comply with postoperative care. 9 Eye & Ear Infirmary, Boston, MA, USA) and the A systematic review done by Tan et al reported that the anatomical survival in all the osteo-odonto keratoprosthesis also known as the OOKP studies was excellent, with a survival rate ‘OOKP’ (originally described by Strampelli, and of 480% even at the 20-year time point. In all later modified by Falcinelli).2 The Boston KPro the studies, more than half of the patients type I is used for eyes with an adequate tear film achieved vision of better than 6/18. In three and an intact blink mechanism. As such, the series visual acuity was better than 6/18 in Boston KPro type I is an alternative to high-risk 460% of eyes. The Boston KPro type I is keratoplasty. The OOKP, on the other hand, is indicated in cases with good tear function and indicated if there is bilateral blindness from ocular surface state; therefore, its results cannot severe, end-stage ocular surface diseases in eyes be compared to those of OOKP studies because with intact retinal and function. The of differences in disease severity and ocular conjunctival and lid abnormalities make the eye surface and tear film stati.6 A recent study by unsuitable for conventional corneal Yaghouti et al10 using the Boston KPro type I and 1 transplantation or ocular surface reconstruction. type II devices to treat SJS and severe chemical Other less common indications for Boston burns suggested poor anatomical and visual KPro type I reported in some case series outcomes at 5 years (success rate zero). The most included primary congenital with recent study by Lee et al7 using Boston KPro type concomitant corneal oedema, , irido- II device reported one third of their patients corneal endothelial syndrome and gelatinous maintaining 20/100 vision or better at the last follow- drop-like but the reported up visit (mean follow-up duration was 70 months) – numbers are small.3 6 A second design, the and they reported 50% device retention rate. Boston KPro type II, in which the optic is The most common long-term blinding implanted through the closed , was complication in all keratoprosthesis types is designed for severe end-stage ocular surface glaucoma with all the difficulties to monitor and disease.7 This was an attempt to replace OOKP treat in those cases. Intraocular pressure is so surgery can be done in a single stage with no difficult to measure following all types of KPro. The role of keratoprostheses A Shalaby Bardan et al 8

Topical and systemic anti-glaucoma medications, 3 Haugsdal JM, Goins KM, Greiner MA, Kwon YH, aqueous shunt surgery, and cyclo-destructive laser are all Alward WL, Wagoner MD. Boston type 1 keratoprosthesis 100 potential management options. The reported retinal for primary congenital glaucoma. Br J Ophthalmol 2016; : 328–331. detachment rate after OOKP varied between 0 and 26%. 4 Akpek EK, Harissi-Dagher M, Petrarca R, Butrus SI, The incidence of other sight-threatening complications Pineda R 2nd, Aquavella JV et al. Outcomes of Boston such as , is relatively low after OOKP (0– keratoprosthesis in aniridia: a retrospective multicenter 8%).9 While for Boston type I and II, the reported rate of study. Am J Ophthalmol 2007; 144(2): 227–231. endophthalmitis incidence was 11% and 14%, respectively 5 Phillips DL, Goins KM, Greiner MA, Alward WL, Kwon YH, 11 Wagoner MD. Boston type 1 keratoprosthesis for and retinal detachment risk after Boston type II as iridocorneal endothelial syndromes. 2015; 34: 7 reported by Lee et al was 18.8%. 1383–1386. There are some other complications reported after 6 Lekhanont K, Jongkhajornpong P, Chuephanich P, OOKP such as mucosal ulceration, lamina exposure, Inatomi T, Kinoshita S. Boston type 1 keratoprosthesis for gelatinous drop-like corneal dystrophy. Optom Vis Sci 2016; extrusion and resorption which can lead to loss of the 93 – 9 : 640 646. eye. Complications common to Boston keratoprostheses 7 Lee R, Khoueir Z, Tsikata E, Chodosh J, Dohlman CH, include but are not limited to, retroprosthetic membrane Chen TC. Long-term Visual Outcomes and Complications of formation (most common complication; reported range Boston Keratoprosthesis Type II Implantation. 124 – 13–60% of the cases), sterile keratolysis, infectious 2017; :27 35. 8 Hille K, Grabner G, Liu C, Colliardo P, Falcinelli G, Taloni M , and sterile vitritis. Device extrusion can occur fi 12 et al. Standards for modi ed osteo-odontokeratoprosthesis when sterile keratolysis is not immediately addressed. (OOKP) surgery according to Strampelli and Falcinelli: the Rome-Vienna Protocol. Cornea 2005; 24: 895–908. Conclusion 9 Tan A, Tan DT, Tan XW, Mehta JS. Osteo-odonto keratoprosthesis: systematic review of surgical outcomes 10 – Keratoprosthesis surgery is complex and requires and complication rates. Ocul Surf 2012; (1): 15 25. 10 Yaghouti F, Nouri M, Abad JC, Power WJ, Doane MG, meticulous care at each step to ensure the overall success Dohlman CH. Keratoprosthesis: preoperative prognostic rate and a careful lifelong follow-up commitment. A categories. Cornea 2001; 20:19–23. multidisciplinary team is needed to provide the best care 11 Nouri M, Terada H, Alfonso EC, Foster CS, Durand ML, for the patients. The literature lacks randomized Dohlman CH. Endophthalmitis after keratoprosthesis: incidence, bacterial causes, and risk factors. Arch Ophthalmol controlled studies comparing different types of 2001; 119: 484–489. keratoprostheses for the different indications, However 12 Lee WB, Shtein RM, Kaufman SC, Deng SX, Rosenblatt MI. taking the current available evidence into account; we can Boston keratoprosthesis: outcomes & and complications: a conclude that Boston type I should be reserved for wet report by the American academy of ophthalmology. 122 – blinking eyes with no keratinisation neither bulbar nor Ophthalmology 2015; : 1504 1511. tarsal, as an alternative to high-risk keratoplasty. We A Shalaby Bardan1,2,3, N Al Raqqad4, would not normally consider offering a Boston if the other M Zarei-Ghanavati1,2,5 and C Liu1,2,6 eye is seeing, as we are converting what is usually a stable situation to one which can have complications causing 1Sussex Eye Hospital, Brighton & Sussex University loss of visual potential. OOKP is for dry keratinised Hospitals NHS Trust, Brighton, UK surface with defective or absent lid or blink mechanism. 2Brighton and Sussex Medical School, Brighton, UK Boston type II does not match the retention and visual 3Faculty of Medicine, University of Alexandria, results of OOKP. Alexandria, Egypt 4King Hussein Medical Centre/Royal Medical Services, Conflict of interest Amman, Jordan 5Eye Research Center, Farabi Eye Hospital, Tehran The authors declare no conflict of interest. University of Medical Sciences, Tehran, Iran 6Tongdean Eye Clinic, Hove, UK References Correspondence: C Liu, Sussex Eye Hospital, Brighton 1 Lam FC, Liu C. The future of keratoprosthesis (artificial and Sussex University Hospitals NHS Trust, corneae). Br J Ophthalmol 2011; 95: 304–305. Eastern Road, Brighton BN2 5BF, UK 2 Liu C, Paul B, Tandon R, Lee E, Fong K, Mavrikakis I et al. Tel: 012 736 696 955; The osteo-odonto-keratoprosthesis (OOKP). Semin Fax: 012 736 696 955. Ophthalmol 2005; 20(2): 113–128. E-mail: [email protected]

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