Rosewater Syndrome

At a glance

A rare form of gynecomastia with hypogonadism. Similar to the Reifenstein Syndrome, but without hypospadias.

Synonym

Familial Gynecomastia.

History

First described by Stanley Rosewater, an American endocrinologist, in 1965.

Genetic inheritance

X-linked recessive or autosomal dominant.

Pathophysiology

It has been suggested that this condition is the mildest expression of incomplete male pseudohermaphroditism, type I. Masculinization by monthly administration of testosterone has been demonstrated.

Diagnosis

Clinical features (gynecomastia with hypogonadism); biochemical (increased levels of testosterone and estrogen, but with low levels of luteinizing hormones); histology (decreased Leydig cells on testicular biopsy).

Clinical aspects

Gynecomastia, hypogonadism, sterility.

Anesthetic considerations

Endocrine dysfunction appears to be limited to the reproductive system. Psychological attention must be given to patients affected with this condition because of the embarrassment caused by the gynecomastia.

Other condition to be considered

• ☞Reifenstein Syndrome: Characterized by male pseudohermaphroditism, which means that the individual has testes but presents clinically with the secondary sexual characters of both male and female. The severity of androgen insensitivity determines the clinical presentation.

References