Rare metabolic disorder characterized by early onset
of diabetes mellitus in the infancy period, multiple epiphyseal dysplasia,
multiple fractures, microcephaly, short stature, hypertonia, barrel-shaped
chest, hepatomegaly, tooth discoloration, gray-blue sclerae, high-arched
palate, and skin abnormalities. Epiphyseal dysplasia and growth retardation
have onset within the first 2 years of life.
MED-IDDM Syndrome; IDDM-MED Syndrome; Wolcott-Rallison
Acquired condition only in diabetics.
Mauriac syndrome is primarily of historic
interest. It occurred in poorly controlled diabetics when only short-acting
insulin was used. Insufficient tissue glucose results in gluconeogenesis and
fat metabolism and therefore a catabolic state. In addition, somatomedin
production is reduced, resulting in relatively short stature. Although the
pituitary-adrenal axis remains intact, high cortisol levels are typical.
This syndrome should be suspected in poorly controlled
diabetics with cushingoid appearance.
Mauriac syndrome occurs only in children and
adolescents with poorly controlled insulin-dependent diabetes mellitus. They
present with dwarfism, hepatomegaly, and a cushingoid appearance, including
truncal obesity and “buffalo hump.” Hepatomegaly is caused by fatty
deposition in the liver. Hepatic function is usually normal. These findings
are almost completely reversed by improved diabetic control. However,
diabetic end-organ damage, including retinopathy and nephropathy, may be
Preoperative evaluation of diabetic
control is essential. Careful evaluation of end-organ damage. Kidney
function must be assessed because these patients are susceptible to renal
Perioperative control of blood sugar
must be accomplished in cooperation with endocrinologists.
Doses of anesthetic agent may require
adjustment in the presence of renal dysfunction. Medication with renal
excretion only should better be avoided.
Najjar S, Ayash MA: The Mauriac syndrome. Clin Pediatr
Traisman HS, Traisman ES: Mauriac's syndrome revisited. Eur J Pediatr