Rare Diseases Symptoms Automatic Extraction

Outcomes following Boston type 1 keratoprosthesis implantation in aniridia patients at the University of Montreal.

[aniridia]

To describe outcomes after Boston Type 1 Keratoprosthesis (KPro) surgery in aniridic eyes.Retrospective, interventional case series.University-based tertiary care institution.Twenty-six aniridic eyes of 19 patients who underwent KPro implantation by a single experienced surgeon (M.H.-D.) between October 27, 2008 and January 16, 2012.Preoperative and postoperative best-corrected visual acuity (BCVA), intraoperative and postoperative complications, and keratoprosthesis retention.Mean age was 56 years (range, 28-72); 10 patients were male. Seven patients had bilateral procedures. No intraoperative complications were encountered. Preoperatively, BCVA was 20/300 or worse in all eyes. After a mean follow-up time of 28.7±13.5 months (range 4-50), the most recent BCVA was 20/200 or better in 14 eyes. Visual potential was limited by preexisting terminal glaucoma (n=2), phthisis after retinal detachment (n=4), and suprachoroidal hemorrhage (n=2). Other postoperative complications included retroprosthetic membrane formation (n=15), infectious keratitis (n=1), extrusion (n=2), and corneal melt (n=4). Uncomplicated vitritis was reported in 6 eyes. No endophthalmitis occurred in this series. Most eyes have glaucoma and are on medical treatment. The overall retention rate of the initial prosthesis was 77%.The prognosis in aniridic patients after KPro is variable. Meticulous follow-up and a subspecialty team approach are of utmost importance.

Diseases presenting "glaucoma" symptom

  • 22q11.2 deletion syndrome
  • aniridia
  • coats disease
  • cohen syndrome
  • krabbe disease
  • oculocutaneous albinism
  • oligodontia
  • proteus syndrome
  • wiskott-aldrich syndrome
  • wolf-hirschhorn syndrome

This symptom has already been validated