Mauriac Syndrome

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 Syndrome.

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 present.

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 failure.

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.