[Clearing of the para-transplant host cornea after perforating keratoplasty in Maroteaux-Lamy syndrome (type VI-A mucopolysaccharidosis)].
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.


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.


CONCLUSIONS
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.
Naumann
G O
GO
Augenklinik mit Poliklinik, Universität Erlangen-Nürnberg.
Rummelt
V
V
D000293:Adolescent; D002648:Child; D002869:Chromosome Aberrations / Q000235:genetics; D025063:Chromosome Disorders; D003315:Cornea / Q000473:pathology*; D003318:Corneal Opacity / Q000235:genetics / Q000473:pathology / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D005808:Genes, Recessive / Q000235:genetics; D006801:Humans; D015948:Keratoplasty, Penetrating / Q000473:pathology*; D008854:Microscopy, Electron; D009087:Mucopolysaccharidosis VI / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D014617:Vacuoles / Q000473:pathology
Aufklaren der transplantatnahen Wirtshornhaut nach perforierender Keratoplastik beim Maroteaux-Lamy-Syndrom (Mukopolysaccharidose Typ VI-A).