Engku-Husna Engku Ismail, Nik-Ahmad-Zuky Nik Lah, Muhammad-Nashriq Kadir
Department of Obstetrics & Gynaecology, School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian, Kelantan, 16150, Malaysia.
J Med Case Rep. 2020 Nov 2;14(1):209. doi: 10.1186/s13256-020-02506-6.
Müllerian duct anomaly is a rare condition. Many cases remain unidentified, especially if asymptomatic. Thus, it is difficult to determine the actual incidence. Müllerian duct anomaly is associated with a wide range of gynecological and obstetric complications, namely infertility, endometriosis, urinary tract anomalies, and preterm delivery. Furthermore, congenital anomalies in pregnant mothers have a high risk of being genetically transmitted to their offspring.
We report a case of a patient with unsuspected müllerian duct anomaly in a term pregnancy. A 33-year-old Malay woman with previously uninvestigated involuntary primary infertility for 4 years presented with acute right pyelonephritis in labor at 38 weeks of gestation. She has had multiple congenital anomalies since birth and had undergone numerous surgeries during childhood. Her range of congenital defects included hydrocephalus, for which she was put on a ventriculoperitoneal shunt; imperforated anus; and tracheoesophageal fistula with a history of multiples surgeries. In addition, she had a shorter right lower limb length with limping gait. Her physical examination revealed a transverse scar at the right hypochondrium and multiple scars at the posterior thoracic region, levels T10-T12. Abdominal palpation revealed a term size uterus that was deviated to the left, with a singleton fetus in a nonengaged cephalic presentation. The cervical os was closed, but stricture bands were present on the vagina from the upper third until the fornices posteriorly. She also had multiple rectal prolapses and strictures over the rectum due to previous anorectoplasty. An emergency cesarean delivery was performed in view of the history of anorectoplasty, vaginal stricture, and infertility. Intraoperative findings showed a left unicornuate uterus with a communicating right rudimentary horn.
Most cases of müllerian duct anomaly remain undiagnosed due to the lack of clinical suspicion and the absence of pathognomonic clinical and radiological characteristics. Because it is associated with a wide range of gynecological and obstetric complications, it is vital for healthcare providers to be aware of its existence and the role of antenatal radiological investigations in its diagnosis. The presence of multiple congenital abnormalities and a history of infertility in a pregnant woman should warrant the exclusion of müllerian duct anomalies from the beginning. Early detection of müllerian duct anomalies can facilitate an appropriate delivery plan and improve the general obstetric outcome.
苗勒管异常是一种罕见病症。许多病例未被识别,尤其是无症状者。因此,难以确定其实际发病率。苗勒管异常与多种妇产科并发症相关,即不孕、子宫内膜异位症、泌尿系统异常和早产。此外,怀孕母亲的先天性异常有很高的遗传给后代的风险。
我们报告一例足月妊娠中未被怀疑有苗勒管异常的患者。一名33岁的马来女性,既往有4年未经检查的原发性不孕,在妊娠38周分娩时出现急性右肾盂肾炎。她自出生以来有多种先天性异常,童年时期接受过多次手术。她的先天性缺陷包括脑积水,为此她接受了脑室腹腔分流术;肛门闭锁;以及有多次手术史的气管食管瘘。此外,她右下肢较短,步态跛行。体格检查发现右季肋部有一条横向瘢痕,胸后区域T10 - T12水平有多处瘢痕。腹部触诊发现子宫大小与孕周相符,但向左偏移,单胎头先露未入盆。宫颈口关闭,但阴道从上部三分之一直至后穹窿有狭窄带。由于既往肛门直肠成形术,她还存在多处直肠脱垂和直肠狭窄。鉴于肛门直肠成形术、阴道狭窄和不孕史,进行了急诊剖宫产。术中发现为左侧单角子宫,右侧有一个与之相通的残角子宫。
由于缺乏临床怀疑以及缺乏特征性的临床和放射学表现,大多数苗勒管异常病例仍未被诊断。由于其与多种妇产科并发症相关,医疗保健人员了解其存在以及产前放射学检查在其诊断中的作用至关重要。孕妇存在多种先天性异常和不孕史应从一开始就排除苗勒管异常。早期发现苗勒管异常可促进制定合适的分娩计划并改善总体产科结局。