Abstract

P 216

Keratoplasty in Mucopolysaccharidosis I (M. Scheie)

Hahner M., Wahl J., Pfeiffer N., Pitz S. 
Department of ophthalmology of the Johannes-Gutenberg-University Mainz

Objective: MPS I patients under enzyme replacement therapy (ERT) exhibit less internal organ involvement and an improved range of motion. Thus, there is a significant increase of the life expectancy. Therefore, an improved visual function seems to be an important element of a better quality of life.
Methods: We report on two female MPS I (Scheie) patients undergoing penetrating keratoplasty. Patient 1 is under ERT since four years, in patient 2 a bone marrow transplantation had been performed in the early childhood. According to the higher risk of general anaesthesia with tracheal intubation (instable chondral system with an eburnated mukosa) an interdisciplinary managment (anaesthesia, paediatrics and ophthalmology) has been accomplished.
Results: Both penetrating keratoplasties has been effective and without complications. In Patient 1, visual acuity has improved from perception of handmovments in 1 meter to 0,2, in Patient 2 visual acuity has improved from 0,05 to 0,16 in the immediate postinterventional course.
Conclusions: Penetrating keratoplasty seems to be a safe and adequate intervention in MPS I patients with a loss of visual acuity due to corneal clouding. An interdisciplinary setting minimizes perioperative risks.

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