Achard-Thiers Syndrome

At a glance

Insulin-resistant diabetes mellitus Type II associated with increased plasma androgen levels affecting primarily postmenopausal women.

Synonyms

Diabetic Bearded Women Syndrome; Adenoma Associated Virilism of Older Women.

History

First described in 1921 by the two French doctors Emile Charles Achard, an internist, and Joseph Thiers, a neurologist.

Incidence

Occurs primarily in postmenopausal women. The incidence remains unknown.

Genetic inheritance

Unknown, but autosomal dominant inheritance is most likely, as it often seems to run in families, but probably can also be considered acquired in some patients. No racial, ethnic, or geographical predominance has been described.

Pathophysiology

Adrenal overproduction of androgens.

Diagnosis

Based on the clinical findings of diabetes mellitus Type II and signs of hyperandrogenism with virilization in a postmenopausal woman.

Clinical aspects

Diabetes mellitus Type II with increased serum levels of insulin (insulin-resistance) and glucose (with abnormal 2-hour oral glucose tolerance test) and signs of hyperandrogenism with virilization, ie, hirsutism, receding scalp hairline or male pattern baldness, acne, deepening of the voice, osteoporosis, increased muscularity, clitoris hypertrophy, and irregular or absent menstruation and infertility (in premenopausal women). Arterial hypertension and obesity increase the risk for cardiovascular disease.

Precautions before anesthesia

Obtain a complete medical history with physical examination focusing on the cardiovascular system. An ECG and echocardiography may be required to exclude left-ventricular hypertrophy and coronary heart disease as a result of long standing arterial hypertension. Consultation with an endocrinologist is recommended. In severe obesity, chronic gastrointestinal reflux disease may be present. Administration of oral sodium citrate prior to induction of general anesthesia has occasionally been recommended to potentially reduce the severity of aspiration-related complications.

Anesthetic considerations

Significant obesity may render vascular access challenging and predisposes the patient to increased risk of pulmonary aspiration during induction of general anesthesia, thus a rapid sequence induction technique is advisable. The functional residual capacity in obesity is often decreased, advocating for proper preoxygenation before induction of anesthesia. Depending on the type of surgery, postoperative ventilatory support may be required. Chronic arterial hypertension may result in unstable blood pressure during anesthesia.

Pharmacological implications

None specific to this disorder.

Other conditions to be considered

• Acquired ☞Adrenogenital Syndrome: In contrast to the congenital form of AGS (congenital adrenal hyperplasia), this disorder occurs due to an adrenal tumor with excess production of androgens. In an adult female, the symptoms may include virilization (hirsutism, male pattern alopecia and receding scalp hairline, acne, deepening of the voice, and abnormally large muscles).

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