RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Marciniak, Bruno
A1 Dalens, Bernard J.
SR Print(0)
ID 58062623
T1 Aniridia
T2 Syndromes: Rapid Recognition and Perioperative Implications
YR 2006
FD 2006
PB The McGraw-Hill Companies
PP New York, NY
SN 9780071354554
LK accessanesthesiology.mhmedical.com/content.aspx?aid=58062623
RD 2024/04/23
AB It  is  estimated  that  approximately  one  third  of  patients  with  sporadic  aniridia  will  develop  a  Wilms  tumor,  and  approximately  half  of  patients  with  aniridia,  genitourinary  anomalies  (e.g.,  hypospadias),  and  mental  retardation  will  develop  a  Wilms  tumor.  The  association  with  genitourinary  anomalies  and  mental  retardation  is  known  as  WAGR  Syndrome.  Most  patients  have  prominent  lips  and  macrognathia.  Other  features  may  include  congenital  cataracts,  nystagmus,  ptosis,  and  blindness.  The  presence  of  ptosis  and  generalized  hypotonia  in  a  subgroup  of  patients  suggests  a  susceptibility  to  malignant  hyperthermia.