关键词: Hemivagina obstruida Herlyn–Werner–Wunderlichs syndrome Hipogonadismo Hypogonadism Obstructed hemivagina Prader–Willi syndrome Síndrome de Herlyn-Werner-Wunderlich Síndrome de Prader-Willi

Mesh : Humans Female Adult Prader-Willi Syndrome / complications Vagina / abnormalities surgery Kidney / abnormalities Uterus / abnormalities diagnostic imaging Abnormalities, Multiple Hematometra / etiology Hematocolpos / etiology Urogenital Abnormalities / complications Congenital Abnormalities Abdominal Pain / etiology Kidney Diseases / congenital

来  源:   DOI:10.1016/j.endien.2024.01.010

Abstract:
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.
摘要:
Herlyn-Werner-Wunderlich综合征是一种罕见的泌尿生殖系统异常,由子宫didelphys定义,阻塞的半阴道和单侧肾脏异常。最常见的临床表现是初潮后的痛经,但它也可以表现为疼痛和腹部肿块。Prader-Willi综合征是一种罕见的神经内分泌遗传综合征。下丘脑功能障碍是常见的,包括性腺机能减退在内的垂体激素缺乏很普遍。我们报告了一名33岁的Prader-Willi综合征女性,由于阴道出血和腹痛而被转诊至妇科诊所。腹部超声检查显示血肿和血肿,计算机断层扫描显示子宫畸形和右子宫腔占据(血肿)以及右肾发育不全。在全身麻醉下进行阴道镜检查和宫腔镜检查,找到右阴道隔膜和正常的左子宫颈和半子宫。进行隔离切开术并进行完全的血吸虫引流。这两种综合征的关联尚不清楚。
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