Purpose To propose an optimized microsurgical and medical approach to reduce the risk of complications after penetrating keratoplasty (PKP) in patients with aniridia-associated keratopathy (AAK). Methods Retrospective observational case series of 25 PKP performed in 16 patients with AAK. Preoperative indications were endothelial decompensation and vascularized scars (68%) or graft failure (32%) due to limbal stem cell deficiency. The optimized approach included a combination of a small corneal graft size (around 7.0 mm), interrupted 10–0nylon sutures, simultaneous AMT as a patch, large bandage contact lens, temporary lateral tarsorrhaphy, postoperative autologous serum eye drops, and systemic immunosuppression. Main outcome measures included: visual acuity, transplant survival, and complications encountered during follow-up of 107 weeks on average. Results A complete modified keratoplasty scheme was used in 10 of 25 PKP (group 1), while at least one of the modifications was missing in the other 15 PKP (group 2). After 8 weeks of follow-up, the epithelium was closed in 23 eyes. Visual acuity improved in 19 eyes at 6 months of follow-up, and remained stable in six eyes. None of the eyes showed a decrease in visual acuity. At the last post-operative follow-up, this visual improvement persisted in 14 eyes and graft survival rate after 156 weeks (3 years) was 69% in group 1 versus 44% in group 2 (p = 0.39, log-rank test). Secondary corneal neovascularization (8%), scarring (4%), ulcer (4%), or graft rejection (8%) happened mostly in the second group which was missing at least one of the suggested modifications. Conclusions PKP in congenital aniridia must be considered as a high-risk keratoplasty. An optimized therapeutic approach seems to be promising in order to reduce the postoperative complication rate in these most difficult eyes.

Purpose To propose an optimized microsurgical and medical approach to reduce the risk of complications after penetrating keratoplasty (PKP) in patients with aniridia-associated keratopathy (AAK). Methods Retrospective observational case series of 25 PKP performed in 16 patients with AAK. Preoperative indications were endothelial decompensation and vascularised scars (68%) or graft failure (32%) due to limbal stem cell deficiency. The optimized approach included a combination of a small corneal graft size (around 7.0mm), interrupted 10-0-Nylon sutures, simultaneous AMT as a patch, large bandage contact lens, temporary lateral tarsorrhaphy, postoperative autologous serum eye drops, and systemic immunosuppression. Main outcome measures included: Visual acuity, transplant survival and complications encountered during follow-up of 107 weeks on average. Results A complete modified keratoplasty scheme was used in 10 of 25 PKP (group 1), while at least one of the modifications was missing in the other 15 PKP (group 2). After 8 weeks follow-up, the epithelium was closed in 23 eyes. Visual acuity improved in 19 eyes at 6 months follow-up, and remained stable in 6 eyes. None of the eyes showed a decrease in visual acuity. At the last post-operative follow-up, this visual improvement persisted in 14 eyes and graft survival rate after 156 weeks (3 years) was 69% in group 1 vs. 44% in group 2 (p = 0.39, logrank test). Secondary corneal neovascularisation (8%), scarring (4%), ulcer (4%) or graft rejection (8%) happened mostly in the second group which was missing at least one of the suggested modifications. Conclusions PKP in congenital aniridia must be considered as a high-risk keratoplasty. An optimized therapeutic approach seems to be promising in order to reduce the postoperative complication rate in these most difficult eyes.

ZusammenfassungKontaktlinsenassoziierte Keratitiden werden immer häufiger. Die mykotische Keratitis ist ein relativ seltenes, aber sehr ernst zu nehmendes Krankheitsbild. Meist wird im Frühstadium eine falsche Diagnose gestellt und dadurch die adäquate Therapie verzögert. Bei der therapierefraktären kontaktlinsenassoziierten mykotischen Keratitis können nicht selten auch Koinfektionen oder Superinfektionen bestehen. Wir stellen 2 Patienten mit initial unklarer Keratitis vor, bei denen eine Mischinfektion der mykotischen Keratitis mit Pseudomonas aeruginosa bzw. Akanthamöben nachgewiesen werden konnte. In beiden Fällen war die zeitnahe perforierende Excimerlaser-Keratoplastik mit Einzelknüpfnähten und adäquater Lokaltherapie über 8 Wochen therapeutisch erfolgreich.