Ovarian Hyperstimulation Syndrome in Newborns – A Case Presentation and Literature Review

Abstract

Preterm ovarian hyperstimulation syndrome (POHS) is a rare condition in which immaturity of the gonadal axis is accepted as the cause. Based on our case and 8 cases from the literature, we made an attempt to specify the mechanisms underlying the syndrome and its pathognomonic signs. All POHS newborns were born between 24 and 31 weeks postconception age (WPCA) and developed vulvar, hypogastric and upper leg swelling, and ovarian follicular cyst/cysts (10–40 mm in diameter) with mildly or considerably elevated E₂ concentrations (80–5,300 pmol/l) between 35 and 39 WPCA. The GnRH test, performed in 5 cases, confirmed gonadal axis activation. In our case the observed normalization of elevated gonadotropin values by 43 WPCA, accompanied by a simultaneously increasing E₂ value (approximately 800 pmol/l), could correspond with the maturation of the gonadal steroid-dependent negative-feedback mechanism. The continuously increasing E₂ levels after this period (maximum 1,300 pmol/l) suggest its autonomous secretion. In all the cases, including 3 neonates treated with medroxyprogesterone and surgery, the swelling resolved by 6 months. Conclusions: A pathognomonic sign of POHS is swelling which develops around 37 ± 3 WPCA, and the syndrome is only infrequently diagnosed when the swelling is profound. The direct etiologic factor here is not E₂. POHS does not require therapy as long as there is no danger of cyst torsion.

Preterm ovarian hyperstimulation syndrome presented with vaginal bleeding: a case report

“…It has been rarely reported in medical literature until this time (1, 2, 4). The characteristic features include prematurity (24-31 WPCA), swelling of vulva, hypogastric region and upper leg, elevation of E 2 and gonodotrophin, and multiple cysts in both ovaries (2,5). The only pathognomonic sign of POHS is the swelling of the vulva occurring as a result of high levels of vascular endothelial growth factors secreted by theca and granulose cells (2).…”

“…Preterm ovarian hyper stimulation syndrome (POHS) is a very rare entity characterized by vulvar, hypogastric and upper leg swelling, elevated serum estradiol (E 2 ) and gonadotropin levels, and ovarian follicular cysts (1)(2)(3). The main etiopathology is the immaturity of the gonadal axis and the lack of the negative feedback of the placental steroids.…”

Mini puberty and its interpretation

“…The pathophysiology of this picture which is observed in preterm babies is not known fully. It is thought that lack of maturation of the hypothalamo-pituitary-gonadal axis and lack of complete development of negative feedback mechanisms with discontinuation of placental steroids are involved (42,46). It has been proposed that edema observed in the subjects is related with vascular endothelial growth factor released from the theca and granulosa cells (43,47).…”

“…Hence, reduction in the cysts are observed in the first month in 50% of the cases and at the end of the second month in 25% of the cases. The cysts may persist for longer than three months in 10% of the cases (42). Drainage by aspiration may be necessary because of the risk of tortion in solitary or multiple cysts with a diameter larger than 4-5 cm which do not get smaller (41).…”

“…Drainage by aspiration may be necessary because of the risk of tortion in solitary or multiple cysts with a diameter larger than 4-5 cm which do not get smaller (41). In severe cases where clinical regression is not observed, intracranial medroxyprogesteron acetate may be administered at a dose of 150 mg/m 2 with the aim of decreasing the synthesis of ovarian estradiol (42). …”