Rare Diseases Symptoms Automatic Extraction

Black diaphragm intraocular lens implantation and penetrating keratoplasty in aphakic eyes with traumatic aniridia.

[aniridia]

To evaluate the long-term outcome of implantation of black diaphragm intraocular (BDI) lens combined with penetrating keratoplasty (PKP) for managing aphakic eyes with traumatic aniridia and corneal damage.Six aphakic eyes of six patients with traumatic aniridia and corneal damage had BDI lens implantation at Qingdao Eye Hospital, Shandong Eye Institute from June 2008 to November 2011. Medical records of the patients were reviewed. Three patients received PKP and after 12-18months were implanted with BDI lens. The other three patients completed PKP and BDI lens implantation at the same time. The corrected visual acuity, intraocular pressure and number of corneal endothelial cells were monitored.The patients were followed up for an average of 24.3±12.1months (range 14-48 months). All BDI lenses were located well. The best corrected visual acuity got improved in 5 patients (0.1-1.0) and decreased in 1 patient from 0.4 to 0.2. Three patients had normal intraocular pressure (IOP) after implantation. Two patients required antiglaucoma medications to control IOP within the normal range and 1 patient implanted Ahmed glaucoma valve to control IOP. The corneal grafts kept transparent in all eyes and the corneal endothelial counting >1 000/mm(2), although two patients experienced acute graft rejection and loss more than 30% corneal endothelial cells.Implantation of BDI lens combined with PKP is an effective option for managing aphakic eyes with traumatic aniridia and corneal damage. Although the results in our study are encouraging, additional studies of the long-term safety and efficacy are required. A larger study population and longer follow-up may be beneficial.

Diseases presenting "long-term outcome" symptom

  • aniridia
  • cholangiocarcinoma
  • classical phenylketonuria
  • cystinuria
  • erythropoietic protoporphyria
  • esophageal squamous cell carcinoma
  • fabry disease
  • hirschsprung disease
  • homocystinuria without methylmalonic aciduria
  • hydrocephalus with stenosis of the aqueduct of sylvius
  • inclusion body myositis
  • kabuki syndrome
  • krabbe disease
  • oligodontia
  • papillon-lefèvre syndrome
  • primary hyperoxaluria type 1
  • trochlear dysplasia
  • von hippel-lindau disease
  • waldenström macroglobulinemia

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