[MIM 617 503]

Acronym for Myelodysplasia, Infection, Growth restriction, Adrenal hypoplasia,

Genital phenotypes, and Enteropathy.

Very rare. Part of the group of syndromic adrenal dysplasias such as the Allgrove (AAA) and IMAGe syndromes. Autosomal dominant transmission of a mutation of the SAMD9 gene (7q21.2). 

Clinical features:

-         short stature: intrauterine and post-natal growth retardation

-         myelodysplasia: anemia, sometimes low platelets

-         adrenal insufficiency due to adrenal dysplasia: addisonian crisis [hypoNa, hyperK, hypoglycemia, hypotension] during the first month of life, sometimes later; skin hyperpigmentation

-         frequent infections due to immune deficiency

-         enteropathy

-         genital anomalies: micropenis, hypospadias, cryptorchidism

Anesthetic implications: 

check total blood count; adapt cortisone replacement doses; short stature; antibiotic prophylaxis; blood glucose monitoring

References:

-        Gökdemir BN, Çekmen N.
Anesthetic management of a patient with MIRAGE syndrome: a case report.
A&A Practice 2024;18:e01770

Updated: April 2024