Objectives

To comprehensively evaluate the pelvic magnetic resonance imaging (MRI) findings of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and summarize the typical and atypical characteristics.

Methods

A retrospective analysis of 201 consecutive MRKH patients was carried out. Pelvic MRI was reviewed by two experienced gynecological radiologists in consensus. Characteristics including the morphology, signal pattern and volumes of the uterine rudiments, location and volume of the ovaries, and the degree of vaginal dysgenesis were evaluated. Other noted abnormalities were also recorded.

Results

Morphologically, the majority (95%) of patients displayed bilateral uterine rudiments combined with a fibrous band. The minority of patients showed no (3.5%) or unilateral (1.5%) uterine rudiments. A total of 385 uterine rudiments were detected which showed four types of signal patterns: one-layer differentiation (325, 84.4%), two-layer differentiation (27, 7%), three-layer differentiation without subsequent alteration (23, 6.0%), and three-layer differentiation with hematometra and/or ipsilateral hematosalpinx (10, 2.6%). The median volumes of these four types of uterine rudiments were 2.6 ml (1.69–3.81 ml), 3.19 ml (2.67–4.51 ml), 6.05 ml (3.37–12.44 ml), and 31.97 ml (19.2–38.7 ml), respectively. The mean ovarian volume was 6.49 ± 3.91 ml. Abnormally located ovaries were detected in 63 (31.3%) patients. The distal vagina was discernable in 25.1% of patients.

Conclusion

MRKH patients typically display bilateral uterine rudiments combined with a fibrous band and normally located ovaries. The uterine rudiments are generally small with only one-layer differentiation, a subset of which might be large and exhibited other atypical presentations, including two- or three-layer differentiation or even hematometra. Abnormally located ovaries are not rare.

Key Points

• Morphologically, MRKH patients typically displayed bilateral uterine rudiments combined with a fibrous band.

• Typically, the uterine rudiments (84.4%) were small and displayed only one-layer differentiation.

• About 15.6% of rudiments showed atypical characteristics including two- or three-layer differentiation, even complicated with hematometra or hematosalpinx.

目标

为了全面评估Mayer-Rokitansky-Küster-Hauser（MRKH）综合征的骨盆磁共振成像（MRI）结果，并总结典型和非典型特征。

方法

回顾性分析201例连续MRKH患者。两名经验丰富的妇科放射科医生对骨盆MRI进行了共识。评估了特征，包括形态，信号模式和子宫the的体积，卵巢的位置和体积以及阴道发育不全的程度。还记录了其他注意到的异常情况。

结果

在形态上，大多数（95％）患者表现出双侧子宫行伴纤维带。少数患者未见（3.5％）或单侧（1.5％）子宫瘤。总共检测到385个子宫di，显示出四种信号类型：一层分化（325，84.4％），两层分化（27，7％），三层分化而没有随后的改变（23，6.0％ ），以及血球和/或同侧血索平三层分化（10，2.6％）。这四种类型的子宫的中位体积分别为2.6 ml（1.69–3.81 ml），3.19 ml（2.67–4.51 ml），6.05 ml（3.37–12.44 ml）和31.97 ml（19.2–38.7 ml）。卵巢平均体积为6.49±3.91 ml。在63名（31.3％）患者中检测到卵巢位置异常。25处可见远端阴道。

结论

MRKH患者通常表现出双侧子宫伴纤维带和正常位置的卵巢。子宫瘤一般很小，只有一层分化，其中一部分可能很大，并表现出其他非典型表现，包括两层或三层分化甚至血流。卵巢位置异常并不罕见。

关键点

•在形态上，MRKH患者通常表现出双侧子宫行伴纤维带。

•通常，子宫瘤（84.4％）很小，仅表现出一层分化。

•约有15.6％的果肉表现出非典型特征，包括两层或三层分化，甚至与血丝或血红蛋白并发。