Meneses Aurus Dourado, Filho Walberto Monteiro Neiva Eulálio, Raulino Débora Maria Ribeiro, Martins Eduardo Bruno Lobato, Vieira Sabas Carlos
Department of Urology, São Marcos Hospital, Piauí, Brazil.
Department of General Surgery, Universidade Federal do Piauí, Piauí, Brazil.
Oman Med J. 2017 Mar;32(2):157-160. doi: 10.5001/omj.2017.28.
Herlyn-Werner-Wunderlich (HWW) syndrome is part of a spectrum of Müllerian duct anomalies that occur during embryonic development. The syndrome is characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Only few cases of this disease were reported worldwide. We present a 23-year-old female patient with chronic pelvic pain for years. The patient was diagnosed with HWW syndrome with a history of hematocolpos and vaginoplasty at the age of 12. Five months later, she sought urgent medical care due to intense pain, and a clinical picture suggestive of peritoneal irritation. Clinical condition deteriorated and the patient underwent an exploratory laparotomy, which failed to identify anything to justify the abdominal pain. Uroculture and blood culture were negative. A magnetic resonance imaging showed a tube-shaped formations extending from the right retrovesical region to the mesogastrium corresponding to the persistence of the mesonephric duct, related to ipsilateral renal agenesis. The patient underwent laparoscopic procedure, identifying a right ureteric remnant blind ending, with distal and proximal obliteration, filled with purulent secretion, which was totally resected. This case differs from the other reported cases due to pelvic pain secondary to infection and abscess in ureteric remnant. We did not find any case with similar clinical presentation.
赫林-韦纳-温德利希(HWW)综合征是胚胎发育过程中发生的苗勒管异常谱系的一部分。该综合征的特征是双子宫、阴道半段梗阻和同侧肾缺如。全球仅报道了少数该疾病病例。我们报告一名23岁女性患者,多年来一直患有慢性盆腔疼痛。该患者在12岁时因阴道积血和阴道成形术病史被诊断为HWW综合征。五个月后,她因剧痛寻求紧急医疗护理,临床表现提示有腹膜刺激征。临床状况恶化,患者接受了剖腹探查术,但未发现任何能解释腹痛的原因。尿培养和血培养均为阴性。磁共振成像显示一个管状结构从右膀胱后区域延伸至胃系膜,对应于中肾管的持续存在,与同侧肾缺如有关。患者接受了腹腔镜手术,发现右侧输尿管残端盲端,远端和近端闭塞,充满脓性分泌物,将其全部切除。该病例与其他报道的病例不同,是由于输尿管残端感染和脓肿继发盆腔疼痛。我们未发现有类似临床表现的病例。