%0 Case Reports
%T Herlyn-Werner-Wunderlinch: An unusual presentation in a patient with Prader-Willi syndrome.
%A Costa L
%A Garcia-Grau E
%A Toledo L
%A Burgaya N
%A Cos R
%A Rojas M
%A Giménez-Palop O
%A Caixas A
%J Endocrinol Diabetes Nutr (Engl Ed)
%V 71
%N 4
%D 2024 Apr
%M 38735678
%F 1.833
%R 10.1016/j.endien.2024.01.010
%X Herlyn-Werner-Wunderlich syndrome is an uncommon urogenital anomaly defined by uterus didelphys, obstructed hemi-vagina and unilateral renal anomalies. The most common clinical presentation is dysmenorrhoea following menarche, but it can also present as pain and an abdominal mass. Prader-Willi syndrome is a rare neuroendocrine genetic syndrome. Hypothalamic dysfunction is common and pituitary hormone deficiencies including hypogonadism are prevalent. We report the case of a 33-year-old female with Prader-Willi syndrome who was referred to the Gynaecology clinic due to vaginal bleeding and abdominal pain. Abdominal ultrasound revealed a haematometra and haematocolpos and computed tomography showed a uterus malformation and a right uterine cavity occupation (hematometra) as well as right kidney agenesis. Vaginoscopy and hysteroscopy were performed under general anaesthesia, finding a right bulging vaginal septum and a normal left cervix and hemiuterus. Septotomy was performed with complete haematometrocolpos drainage. The association of the two syndromes remains unclear.