Presentation
Irregular menses since menarche. Menarche at 14 years old.
Patient Data
AVF uterus showing small size measuring 3.2 x 1.9 x 3.5 cm along its max TS, AP & CC dimensions, uterine cross-sectional area (UXA) : 7.1cm2 ; maintained zonal anatomy.
Small size of both ovaries. The right ovary measures 10 x 8x 10 mm with a mean volume 0.3 mL. The left ovary measures 13 x 9 x 12mm with a mean volume 0.7 mL.
Small-sized uterus with normal uterine body/cervical ratio.
Small-sized ovaries which are hardly seen.
Hormonal profile
FSH level: 0.74 mIU/mL
Reference range: Follicular: 2.5-10.2; Mid-cycle: 3.4-33.4; Luteal: 1.5- 9.1; Post-menopausal: 23.0- 116.3
LH level: 0.52 mIU/mL
Reference range: Follicular: 1.9-12.5; Mid-cycle: 8.7-76.3; Luteal: 0.5- 16.9; Post-menopausal: 15.9- 54; Pregnant: up to 1.5; Contraception: 0.7-5.6
Free testosterone: <0.5
Reference range: <4
Prolactin: 5.6
Reference range: 2.8- 29.2
Antimullerian hormone: 0.996
Reference range: 0.69- 7.3
TSH: 1.03
Reference range 0.5- 4.78
Case Discussion
The combined reduced size of the uterus and ovaries raises the possibility of hypogonadotropic hypogonadism and reduced FDH and LH levels, rather than the possibility of an isolated small hypoplastic uterus. The hormonal profile revealed reduced FSH and LH levels with normal prolactin, TSH, and anti-Mullerian hormone levels.
Tasoula et al in a study reported that uterine and ovarian size is reduced in patients with an isolated growth hormone deficiency, hypogonadotrophic hypogonadism, or panhypopitutarism.
At puberty, uterine growth is mainly dependent on circulating estradiol. The onset of puberty is characterized by a change in the shape of the uterus from a tubular to a pear-shaped appearance, increase in the dimensions of the uterus and endometrial thickness as well as increased ovarian volume.
In 46,XX and 45,X females with ovarian insufficiency (gonadal dysgenesis) and estrogen shortage, Müllerian agenesis is occasionally incorrectly reported. Exogenous estrogen exposure has been found to stimulate uterine development in these patients, indicating that pubertal uterine development was lacking rather than agenesis. Prepubertal uterine imaging should generally be viewed cautiously due to the possibility of drawing erroneous conclusions about agenesis or aplasia.
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