Management of glaucoma with Boston type 1 keratoprosthesis.

PURPOSE: To describe the frequency, clinical characteristics, complications, and management of glaucoma in eyes that underwent keratoprosthesis implantation. METHODS: Patients who underwent keratoprosthesis surgery between June 2010 and January 2020 were retrospectively evaluated for glaucoma association and prognoses. RESULTS: Among 17 patients who underwent keratoprosthesis surgery, 9 (52.9%) were associated with underlying or keratoprosthesis-induced glaucoma. Five eyes (29.4%) had underlying glaucoma and underwent a glaucoma drainage device implantation at least 6 months before keratoprosthesis surgery. One eye (5.9%) with normal intraocular pressure had glaucoma drainage device implantation at the same session with keratoprosthesis surgery due to high-risk characteristics of anterior segment structures. Four eyes with preexisting glaucoma showed progression after keratoprosthesis surgery. Additional antiglaucomatous treatment was commenced in two eyes whereas implantation of 2nd glaucoma drainage device was performed in two eyes. Postoperative complications in three eyes (100%) with glaucoma drainage device implanted 6 months before or at the same session with aphakic type keratoprosthesis surgery with partial vitrectomy included rhegmatogenous retinal detachment in two eyes and bacterial endophthalmitis in one eye. Migration of silicone oil through the tube to the subconjunctival area was seen after pars plana vitrectomy in one eye. None of the three eyes (0%) that underwent glaucoma drainage device implantation years before keratoprosthesis surgery experienced a posterior segment complication other than glaucomatous progression. Out of 11 eyes with no previous history of glaucoma, 3 (27.3%) showed high intraocular pressure and glaucomatous disc changes after keratoprosthesis surgery, which could be pharmacologically controlled. CONCLUSIONS: In this cohort, eyes with preexisting glaucoma were more difficult to manage compared to eyes with de novo glaucoma after keratoprosthesis surgery. Retinal complications appeared more often when glaucoma drainage device implantation was performed no more than 6 months before aphakic type keratoprosthesis surgery with partial vitrectomy.

Optic neuritis in CD59 deficiency: an extremely rare presentation.

BACKGROUND: CD59 is the principal cell inhibitor of complement membrane attack on cells. Stroke, peripheral neuropathy, and recurrent central nervous system attacks have been reported in patients with inherited CD59 deficiency. In this paper, we report a patient with CD59 deficiency associated with two attacks of demyelinating peripheral neuropathy and the third attack as an isolated optic neuritis. CASE: An 8-month-old girl whose sibling died at 12th month of age with recurrent weakness episodes responsive to intravenous immune globulin treatment, presented with weakness in legs and poor sucking. Weakness episodes with neurogenic electromyography suggested CD59 deficiency. Immunophenotypic analysis with flow cytometry showed CD59 deficiency. Sanger sequencing of CD59 gene revealed a homozygous c146delA (p.Asp49Valfs*32) mutation. First two attacks were treated with intravenous immunoglobulin therapy without any sequalae. Third attack was an isolated optic neuritis which could not be explained by any other entity. The patient had no response to intravenous immunoglobulin but benefited from pulse steroid therapy. Eculizumab was started every two weeks in order to prevent possible advanced attacks and to reduce their severity. CONCLUSION: Although it is a rarely reported disease, better recognition of CD59 deficiency by pediatric neurologists is necessary because it is curable. In addition to different presentations reported, optic neuritis may also be a manifestation of CD59 deficiency.