SAT-292 Superimposed Peripheral Precocious Puberty Caused by Autonomous Ovarian Follicular Cyst in Being Treated Central Precocious Puberty Patient: Underlying Condition of Herlyn-Werner-Wunderlich Syndrome

Abstract

Autonomous ovarian follicular cysts are known as the most common cause of peripheral precocious puberty(PPP) in prepubertal girls, and relapses of autonomous ovarian cysts resulting in prolonged estrogen exposure can precipitate early maturation of the hypothalamic-pituitary-gonadal axis, resulting in progression from PPP to central precocious puberty(CPP), which is called “combined precocious puberty”, an evolution which may require GnRH therapy. In present case, PPP caused by autonomous ovarian follicular cyst was developed concomitantly during the treatment course of CPP, with suppressed status of hypothalamic-hypophyseal-axis. In addition, a rare congenital Müllerian anomaly known as Herlyn-Werner-Wunderlich syndrome(HWWS), consisting of uterus didelphys, obstructed hemivagina and unilateral renal agenesis was incidentally diagnosed as an underlying condition. A 7-year-old girl was presented to our clinic with the development of breast buds which was examined as Tanner stage 2-3. Right renal agenesis was diagnosed in infancy. Her bone age was advanced as 10 years of age, and Luteinizing hormone(LH) showed pubertal response in LHRH stimulation test. The pelvic US revealed absence of right kidney and uterine enlargement. Brain MRI showed no specific abnormalities. Based on these results, We diagnosed idiopathic central precocious puberty. Thereafter, we commenced the treatment with Leuprolide acetate 3.75mg every 4 weeks. After the 11th injection of Leuprolide acetate, however, the patient complained of sudden breast engorgement and vagnal bleeding. Being considered as unsuccessful suppression of HPG axis, Leuprolide acetate was boosted. Hormonal analysis results revealed an extremely elevated level of estradiol and suppressed baseline gonadotropin levels. The pelvic US revealed double uterus, which had not been detected to that point. Abdominopelvic MRI demonstrated right renal agenesis and uterus didelphys with clearly separated uterine body, cervix and vagina. About 3cm sized cystic lesion with several internal daughter cysts is also discovered in left adnexa area. Based on all examinations, we concluded that PPP caused by autonomous ovarian cyst was superimposed on treatment course of CPP and HWWS was incidentally found as underlying disease. Maintaining GnRH agonist treatment, the progression of secondary sexual characteristics was halted and left ovarian cyst disappeared. In couclusion, clinician should be aware of possibility of autonomous ovarian follicular cyst when sudden progression of secondary sexual characteristics is shown in being treated CPP patients, and discriminate PPP from unsuccessfully treated CPP. Additionally, careful suspicion for HWWS that is tied to indications of renal agenesis in children may be the key for successful early diagnosis and immediate treatment intervention, which could prevent long-term complications.