1. Post Keratoplasty Atopic Sclerokeratitis (PKAS) after Deep Anterior Lamellar Keratoplasty (DALK). Sharmina R Khan William H Ayliffe Mayday University Hospital, Croydon, London, UK. April 7-9, 2010 Boston, MA, USA The authors have no financial interest in the subject matter of this presentation.

2. Introduction PKAS is a severe (non-rejection) inflammation that infrequently occurs following keratoplasty. A retrospective Japanese series found 6 eyes developed PKAS, of 29 with atopic dermatitis, of a total 247 eyes with keratoconus that underwent a keratoplasty procedure between May 2000 and December 2005 (1). Clinical features: - Occurs in the early post operative period within the first few weeks. - Diffuse anterior scleritis. - Loosening of sutures, persistent epithelial defects and graft melting. Differential diagnosis: Acute epithelial rejection Microbial keratitis (staphylococcal) 1)Tomita M, Shimmura S, Tsubota K, Shimazaki J. Postkeratoplasty Atopic Sclerokeratitis in Keratoconus Patients. Ophthalmology, 2008, May, 115 (5); 851-856. Sharmina R Khan, William H Ayliffe.

3. Introduction Risk factors for PKAS: i) Atopic dermatitis/ Allergic eye disease (2) /Asthma (1) ii) Active blepharitis (1) iii) Corneal neovascularisation (1) iv) Elevated serum IgE (2) Treatment includes early loose suture removal +/- resuturing, systemic Prednisolone +/- immunosuppressant. We describe our experience using po Cyclosporin and po Tacrolimus. Prognosis can be excellent if treatment is timely. However, there is significant co-morbidity associated with the use of immunosuppressants. Purpose of Presentation: We describe four cases of PKAS following DALK which has been described once before (3), all other reports have been following penetrating keratoplasty (PK). 2)Daniell MD, Dart JKG, Lightman S Use of cyclosporin in the treatment of steroid resistant post-keratoplasty atopic sclerokeratitis. Br J Ophthalmol 2001;85:91–92. 3)Lyons CJ, Dart JKG, Aclimandos WA, et al. Scleritis after keratoplasty in atopy. Ophthalmology 1990;97:729–33. Sharmina R Khan, William H Ayliffe.

4. Case 1: 34 year old woman Keratoconus,severe eczema, atopic keratoconjunctivitis (AKC). Right DALK (donor 8.25mm, host 8.0mm) 10/0 nylon continuous). Preoperatively g. Fluoromethalone and g. Sodium Cromoglycate 1 week later multiple suture related infiltrates developed. po Prednisolone 30mg started and tapered rapidly 3 weeks later loose sutures. Inferiorly necrotic host cornea (arrow). Graft re-sutured with interrupted 10/0 nylon. po Prednisolone 40mg started and tapered over 6 months. Loose sutures without inflammatory episodes were removed as required. BCVA RE 6/9 Sharmina R Khan, William H Ayliffe.

5. Case 2: 14 year old boy Keratoconus, atopic dermatitis. Right DALK (donor 8.25mm donor, host 8.00mm, 10/0 nylon interrupted). Pre-operatively g. Lodoxamide 6 weeks post-op developed a graft epithelial defect followed by rapid stromal necrosis that did not respond to topical steroids and po Acyclovir. 8 weeks later right tectonic DALK (lyophilised donor) interrupted 10/0 nylon with po Acyclovir prophylaxis. 3/7 post-op developed loose sutures and non-necrotising anterior scleritis that persisted for 10/52. po Prednisolone 30mg tapered over 6 months. Repeat DALK due to early suture loosening. Left DALK carried out under po Prednisolone cover and tapered over 6 months with an uneventful post-op course. BCVA RE 6/9 BCVA LE 6/9 Sharmina R Khan, William H Ayliffe.

6. Case 3: 29 year old man Keratoconus, severe atopic dermatitis, AKC. Left DALK (donor 8.0mm, host 8.25mm 10/0 continuous nylon). Pre-operatively g. Lodoxamide TDS and g. Fluoromethalone TDS LE 3 days post-op developed loose sutures, epithelial defect and non-necrotosing anterior scleritis. 3 weeks later epithelial rejection line noted & po Prednisolone 60mg OD started Inflammation continued resulting in host melting. po Cyclosporin 150mg BD started and tapered over 6 months. Right DALK was carried out with po Tacrolimus prophylaxis (commenced by dermatologist) post-op course was uneventful. BCVA RE 6/6 BCVA LE 6/12 (stromal fibrosis 2⁰ recurrent shield ulcers). Sharmina R Khan, William H Ayliffe.

7. Case 4: 18 year old woman Keratoconus, asthma, eczema, hayfever. Right DALK (donor 8.0mm, host 8.25mm, 10/0 nylon continuous). po Prednisolone 40mg OD and po Tacrolimus 2mg OD commenced 1/52 pre-op. po Prednisolone tapered over 6/52 post op. po Tacrolimus stopped 10/52 post op. 1/12 later she had an exacerbation of eczema that required po Prednisolone and oral antibiotics. Concomitantly developed sectoral anterior scleritis, loosening of sutures, deep corneal neovascularisation. po Tacrolimus resumed and continuous suture removed. Sharmina R Khan, William H Ayliffe.

8. Case 4: 18 year old woman 4/12 later developed epithelial rejection which responded to topical treatment. Deep corneal interface vascularisation and fibrosis (arrows) progressed despite being on po Tacrolimus 2mg BD and po Prednisolone 7.5mg OD and g. Predsol 0.5% PF BD RE Compliance with medication was a problem throughout. BCVA RE 6/12 Sharmina R Khan, William H Ayliffe.

9. Discussion Pre-operative management Treat blepharitis and AKC as an atopic ocular surface is at increased risk of microbial (Staphylococcus aureus) keratitis. Plan surgery when atopic disease is at its most quiescent, so that you are operating on a minimally inflamed eye. Sharmina R Khan, William H Ayliffe.

10. Discussion Intra-operative management Use interrupted sutures. Post-operative management In a DALK, PKAS may be less acutely destructive and more difficult to diagnose than in a PK. Sharmina R Khan, William H Ayliffe.

11. Conclusion Early recognition is important as PKAS responds well to prompt suture management and systemic treatment. We propose: i) Plan DALK instead of a PK in such high risk cases due to requirements for repeat grafting. ii) Use of systemic immunosuppressant as a prophylactic measure if PKAS has occurred in the first eye. Sharmina R Khan, William H Ayliffe.