[MIM 608 978]

(Meacham-Winn-Culler syndrome)

Rare. Mutations in the WT1 gene on 11p13 that can also cause the Denys-Drash or Frasier syndrome.

Association of:

-        abnormalities of the diaphragm: diaphragmatic hernia; sometimes rhabdomyoid dysplasia of lung

-        cardiac malformation: cyanotic heart disease, anomalous pulmonary venous return

-        genital malformations: double vagina, absence of uterus, male pseudohermaphrodism

In general, rapidly letal.

Anesthetic implications:

management of a congenital diaphragmatic hernia; cardiopathy

References : 

Updated: January 2019