(Acronym for the association: tumor of Wilms [Nephroblastoma], Aniridia, Genitourinary anomalies, mental Retardation)(Aniridia-Wilms Tumor syndrome)

Usually de novo mutation: microdeletion of chromosome 11p13 which carries two contiguous genes, WT1 (nephroblastoma) and PAX6 (aniridia). Associates a predisposition to nephroblastoma (variable age of onset) to total or partial aniridia with sometimes cataracts, glaucoma. Variable mental retardation. Genitourinary abnormalities (urinary abnormalities, undescended testes, sexual ambiguity).

Anesthetic implications:

according to the pathologies present

References : 

-         Abadie C, Gauthier-Villars M, Sirvent N, Coupier I. 
Oncogénétique en oncopédiatrie. 
Arch Pédiatr 2012 ; 19 : 863-75

Updated: September 2018