背景:Herlyn-Werner-Wunderlich综合征,一种罕见的苗勒管先天性疾病,以白喉子宫为特征,失明的半阴道,和同侧肾发育不全。诊断是在年轻时通过超声和磁共振成像,预后良好.通常,并发症演变为子宫内膜异位症和继发性盆腔炎。
方法:一名40岁女性患者,巴西,白色,primigravida,30岁时超声诊断为双形子宫,四年后,患有左卵巢子宫内膜瘤,多发性卵巢囊肿,磁共振成像显示左肾发育不全。随后,由于性交困难和肿胀的感觉,患者接受了经阴道超声检查并进行肠道准备,并发现了血液综合征,怀疑是Herlyn-Werner-Wunderlich综合征;诊断后10年,她计划怀孕。她在怀孕第15周后出现频繁的宫缩,幸运的是没有并发症或早产。在第40周和第6天引产,无进展,并指示剖宫产,无并发症。Herlyn-Werner-Wunderlich综合征经常被忽视,导致治疗不充分。患有Herlyn-Werner-Wunderlich综合征的人通常面临生育问题,如高流产率(21-33%),产科并发症,如自然流产(40%的风险),宫内生长受限,产后出血,增加胎儿死亡率,早产(21-29%),剖宫产率升高。此外,子宫内膜异位症的易感性更高,尤其是阴道半梗阻,和盆腔粘连。
结论:早期诊断可以及时治疗,因此,并发症少。尽管如此,当这些因素不存在时,阴道分娩仍然是可能的。与Herlyn-Werner-Wunderlich综合征相关的并发症的真实患病率和发生率仍然未知。
BACKGROUND: Herlyn-Werner-Wunderlich syndrome , a rare Müllerian ducts congenital disease, is characterized by a diphtheritic uterus, blind hemivagina, and ipsilateral renal agenesis. Diagnosis is at young age by ultrasound and magnetic resonance imaging, and the prognosis is good. Usually, complications evolve endometriosis and secondary pelvic inflammation.
METHODS: A 40-year-old female patient, Brazilian, white, primigravida, diagnosed at 30 years with a didelphic uterus on ultrasound, and 4 years later, with a left ovarian endometrioma, multiple ovarian cysts, and left renal agenesis on magnetic resonance imaging. Subsequently, due to dyspareunia and a feeling of swelling, the patient underwent transvaginal ultrasound with bowel preparation, and a hematocolpos was found and Herlyn-Werner-Wunderlich syndrome was suspected; 10 years after the diagnosis she had a planned pregnancy. She presented frequent contractions following the 15th week of pregnancy and fortunately there were no complications or premature labor. Labor was inducted at 40 weeks and 6 days without progress and a cesarean section was indicated and performed without complications. Herlyn-Werner-Wunderlich syndrome often goes unnoticed, leading to inadequate treatment. Individuals with Herlyn-Werner-Wunderlich syndrome commonly face fertility issues, such as high miscarriage rate (21-33%), and obstetric complications, such as spontaneous abortions (40% risk), intrauterine growth restriction, postpartum hemorrhage, increased fetal mortality, preterm delivery (21-29%), and elevated rates of cesarean sections. In addition, there is higher susceptibility of developing endometriosis, especially with hemivaginal obstruction, and pelvic adhesions.
CONCLUSIONS: Early diagnosis enables timely treatment and, consequently, fewer complications. Still, when these factors are absent, vaginal birth may still be possible. The true prevalence and incidence of complications related to Herlyn-Werner-Wunderlich syndrome are still unknown.