Prevalence of ovarian adrenal rest tumours and polycystic ovaries in females with congenital adrenal hyperplasia: results of ultrasonography and MR imaging

Abstract

The aim of the investigation was to assess the prevalence of ovarian adrenal rest tumours and polycystic ovaries in female patients with congenital adrenal hyperplasia (CAH). Thirteen female CAH patients (median age 19.8 years, range 14.8-23.5 years) underwent transvaginal (n=6) or transabdominal (n=7) ultrasonography by a gynaecologist and MR imaging (n=13) of the ovaries (pre and post contrast-enhanced T1- and T2-weighted images). Ovarian adrenal rest tumours were defined as small hypoechoic and multifocal nodules on ultrasound and isointense lesions on T1- and hypointense on T2-weighted MR images (derived from characteristics of testicular adrenal rest tumours). Polycystic ovaries were defined as the presence of > or = 10 follicles arranged peripherally around or scattered throughout increased stroma. No ovarian adrenal rest tumours were found either on ultrasound, or by MR imaging. Polycystic ovaries were found in 2 of the 13 patients (15.4%), both with ultrasound and MR. No ovarian adrenal rest tumours were detected in these female CAH patients, which suggests that ovarian adrenal rest tumours in CAH females are rare. The prevalence of polycystic ovaries corresponded to that in the general population. From these results, we would suggest that routine ovarian imaging in CAH females is not indicated. However, when ovarian dysfunction is present, ovarian imaging is advised, first by ultrasonography, to detect ovarian adrenal rest tumours or polycystic ovaries.

Fertility in Patients with Congenital Adrenal Hyperplasia

“…Stikkelbroeck et al investigated the prevalence of PCOS in 13 female patients with CAH. Polycystic ovaries were found in two patients (15.4%) reflecting a prevalence corresponding to the general population 56 . Therefore it is unlikely that PCOS is a frequent cause of infertility in women with CAH.…”

A Woman with Salt-wasting Congenital Adrenal Hyperplasia Presenting with a Mucinous Ovarian Cystadenoma during Pregnancy

“…They are characterized by the presence of mucins in some or all of the tumor cells, which resemble endocervical, gastric, or goblet cells. The exact incidence of ovarian tumors including the mucinous cystadenoma in women with CAH is yet undefined, although a number of reports indicate the development of polycystic ovaries in CAH women (33,34). The frequency of adrenal rest tumor in women with CAH is less than in men (33).…”

“…The exact incidence of ovarian tumors including the mucinous cystadenoma in women with CAH is yet undefined, although a number of reports indicate the development of polycystic ovaries in CAH women (33,34). The frequency of adrenal rest tumor in women with CAH is less than in men (33). Polycystic ovaries and adrenal rest tumor are generally thought to result from intraovarian androgen excess and may be ACTH-dependent in women with CAH.…”

Long-Term Gynecological Outcomes in Women with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

“…As the adrenals develop in the immediate vicinity of the gonads, some adrenal tissue may adhere to the gonads, and thereby descent with the testis or the ovary [24]. Adrenal rest tumours in female CAH patient have been inconsistently reported [25, 26], but are a well-known phenomenon in male patients, in whom they may be associated with a risk of subfertility [27, 28]. Unfortunately, our ultrasound equipment was not sensitive enough to allow detailed description of ovarian morphology.…”