泌尿生殖系统发育不全的磁共振波谱：Zinner和Mayer-Lokitansky-Küster-Hauser综合征病例系列
Spectrum of Genitourinary Dysgenesis on Magnetic Resonance Imaging: A Case Series of Zinner and Mayer-Rokitansky-Küster-Hauser Syndromes.
摘要(中文译文)
泌尿生殖系统先天性异常虽不常见，但因影响泌尿及生殖功能而具有重要临床意义。Zinner综合征与Mayer-Rokitansky-Küster-Hauser（MRKH）综合征是分别累及中肾管和苗勒管的罕见发育障碍，常表现为非特异性临床特征，因此影像学检查对准确诊断至关重要。本文报道三例采用磁共振成像（MRI）评估的病例。其中一例为年轻男性，表现为排尿困难和睾丸不适，影像显示单侧肾缺如、同侧精囊囊肿及相关的输尿管闭锁，符合Zinner综合征。另两例为年轻女性，均表现为原发性闭经。MRI显示两例子宫及上段阴道缺如而卵巢正常，符合MRKH综合征，其中一例伴有双侧异位肾。MRI对盆腔解剖结构提供了全面评估，清晰显示了先天性异常及相关表现。识别特征性影像学表现对于准确诊断和恰当的临床处理至关重要。
Abstract
Congenital anomalies of the genitourinary system are uncommon but clinically significant due to their impact on urinary and reproductive function. Zinner syndrome and Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome are rare developmental disorders involving the mesonephric and Müllerian ducts, respectively, and often present with nonspecific clinical features, making imaging essential for accurate diagnosis. We present a series of three cases evaluated using magnetic resonance imaging (MRI). One case involved a young male presenting with dysuria and testicular discomfort, demonstrating unilateral renal agenesis, an ipsilateral seminal vesicle cyst, and an associated atretic ureter, consistent with Zinner syndrome. The other two cases involved young females presenting with primary amenorrhea. MRI revealed the absence of the uterus and upper vagina with normal ovaries in both cases, consistent with MRKH syndrome, with one case showing associated bilateral ectopic kidneys. MRI provided a comprehensive evaluation of pelvic anatomy, clearly delineating the congenital anomalies and associated findings. Recognition of characteristic imaging features is essential for accurate diagnosis and appropriate clinical management.
如何引用
AMA
Varun S Trivedi, Narendra G Tembhekar, Varsha Rathi, Nitin R Bhutada, Amit G Disawal, Ramesh Parate. Spectrum of Genitourinary Dysgenesis on Magnetic Resonance Imaging: A Case Series of Zinner and Mayer-Rokitansky-Küster-Hauser Syndromes.. Cureus. 2026; doi:10.7759/cureus.107993.
APA
Varun S Trivedi, Narendra G Tembhekar, Varsha Rathi, Nitin R Bhutada, Amit G Disawal, Ramesh Parate (2026). Spectrum of Genitourinary Dysgenesis on Magnetic Resonance Imaging: A Case Series of Zinner and Mayer-Rokitansky-Küster-Hauser Syndromes.. Cureus. https://doi.org/10.7759/cureus.107993
GB/T 7714
Varun S Trivedi, Narendra G Tembhekar, Varsha Rathi, Nitin R Bhutada, Amit G Disawal, Ramesh Parate. Spectrum of Genitourinary Dysgenesis on Magnetic Resonance Imaging: A Case Series of Zinner and Mayer-Rokitansky-Küster-Hauser Syndromes.[J]. Cureus, 2026 doi:10.7759/cureus.107993.
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