G O Naumann1, V Rummelt. 1. Augenklinik mit Poliklinik, Universität Erlangen-Nürnberg.
Abstract
BACKGROUND: The results of penetrating keratoplasty in patients with systemic mucopolysaccharidosis (MPS) type VI-A are controversial. Clouding of the transplant was often observed and was thought to be related to storage of glycosaminoglycans also in the donor button. PATIENTS AND METHODS: We performed penetrating keratoplasties successfully in three children with MPS VI-A (severe type) at the age of 7-11 years. RESULTS: The transplants remained clear during the follow-up of 2.5-5 years and the longterm visual acuity was encouraging. In two patients we could observe a partial, circular clearing of the host's cornea adjacent to the transplant. The related pathomechanism and the clinical, histopathological and ultrastructural findings of the cornea will be discussed. CONCLUSION: As the intellectual development is normal, decrease of vision impairs the patient's life extremely. Thus, the indication of penetrating keratoplasty should be made early to improve the patient's quality of living, who have a reduced life-span.
BACKGROUND: The results of penetrating keratoplasty in patients with systemic mucopolysaccharidosis (MPS) type VI-A are controversial. Clouding of the transplant was often observed and was thought to be related to storage of glycosaminoglycans also in the donor button. PATIENTS AND METHODS: We performed penetrating keratoplasties successfully in three children with MPS VI-A (severe type) at the age of 7-11 years. RESULTS: The transplants remained clear during the follow-up of 2.5-5 years and the longterm visual acuity was encouraging. In two patients we could observe a partial, circular clearing of the host's cornea adjacent to the transplant. The related pathomechanism and the clinical, histopathological and ultrastructural findings of the cornea will be discussed. CONCLUSION: As the intellectual development is normal, decrease of vision impairs the patient's life extremely. Thus, the indication of penetrating keratoplasty should be made early to improve the patient's quality of living, who have a reduced life-span.