Peripheral precocious puberty with unilateral ovarian agenesis in early childhood: a case report and review of literature

Peripheral precocious puberty (PPP) is a rare condition in early childhood, particularly among girls under 3 years of age. One cause of PPP is functional ovarian cysts that secrete estrogen at levels sufficient to trigger the development of premature secondary sexual characteristics. Unilateral ovarian agenesis is an uncommon congenital anomaly. The coexistence of both conditions in a single patient is extremely rare and poses unique diagnostic and management challenges. We report the case of a 2-year and 10-month-old girl presenting with concurrent breast development, pubarche, and recurrent vaginal bleeding. Pelvic imaging revealed a cyst in the left ovary and agenesis of the right ovary. Laparoscopy confirmed absence of the right ovary and ipsilateral fallopian tube. Based on clinical, hormonal, and radiologic findings, a diagnosis of PPP due to a functional ovarian cyst was made. Given the rapid progression of symptoms, increase in cyst size, and concern for torsion, a short observation period of three months was followed by ovarian-preserving laparoscopic cystectomy. Histopathology confirmed a benign follicular cyst. Postoperatively, the patient experienced partial regression of pubertal signs and complete resolution of vaginal bleeding. This case highlights the importance of individualized decision-making in managing PPP,especially in the presence of congenital anomalies.