A Case of Preterm Ovarian Hyperstimulation Syndrome Presenting With Vaginal Bleeding

Background: Preterm ovarian hyperstimulation syndrome (POHS) is a rare syndrome described in preterm female infants. This is associated with high serum estradiol and gonadotropin levels, multiple follicular ovarian cysts, and edema of the hypogastric, labial and upper leg regions. This usually occurs at 30 to 39 weeks post-conceptional age (PCA). Vaginal bleeding may or may not be present. We present an unusual case of POHS that had a delayed presentation at 5 months of age (45 weeks PCA) with vaginal bleeding. Case presentation: An extremely premature female infant presented with vaginal bleeding at 5 months of age. Workup revealed pubertal gonadotropins indicating ovarian hyperstimulation. She had mild swelling in the hypogastric region. No breast buds were palpable and there was no clitoromegaly. At the time LH (22.2 mu/ml), FSH (8.3 mu/ml), Estradiol (502.2 pg/mL), and Testosterone (111 ng/dL) were all highly elevated. Multiple ovarian follicles were visualized on her pelvic ultrasound, with generous ovarian dimensions: right ovary (3.6 x 1.6 x 1.9 cm, volume 5.7 cc) and left ovary (2.5 x 2.4 x 1.5 cm, volume 4.7 cc). The largest follicle measured 8.8 mm. The uterus was 3.3 x 1.3 x 1.7 cm, with endometrial stripe thickness of 3 mm. There was no endometrial or adnexal mass. Cosyntropin test showed adequate peak cortisol (32.6 mcg/dL). Her newborn screen had been normal. Serial monitoring of her lab work showed a decrease in her gonadotropin and estradiol levels. She had a recurrence of vaginal bleeding about 3 weeks after initial presentation. By 50 weeks PCA the bleeding had resolved and the LH (1.2 mu/ml), FSH (1.5 mu/ml), estradiol (36 pg/ml) had reduced considerably. Complete normalization of LH (1.2 mu/ml), FSH (1.5 mu/ml), E (36 pg/ml), and testosterone (<7 ng/dl) was demonstrated by 55 weeks PCA. Conclusion: POHS has been attributed to the immaturity of the hypothalamic-pituitary-gonadal axis in preterm infants. Vaginal bleeding has been described in one case [1] several weeks after breast enlargement and swelling of clitoral hood, labia majora, hypogastrium and upper legs. In our case the baby presented with vaginal bleeding at 45 weeks that recurred about 3 weeks later and resolved by 50 weeks, with only minimal swelling. This case underlines the need to be aware of this etiology and its varied presentation. [1] Preterm ovarian hyperstimulation syndrome presented with vaginal bleeding: a case report. Altuntas et al. J Pediatr Endocr Met; 273(3-4):355-358.