Shrestha Pradeep, Shrestha Bibek, Mishra Amit K, Katuwal Neeta
Maharajgunj Medical Campus, Tribhuvan University, Institute of Medicine, Maharajgunj, Nepal.
Department of Obstetrics and Gynaecology, Tribhuvan University Teaching Hospital, Maharajgunj, Nepal.
Ann Med Surg (Lond). 2025 Jan 9;87(1):331-334. doi: 10.1097/MS9.0000000000002752. eCollection 2025 Jan.
Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital anomaly characterized by the triad of uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal agenesis. However, atypical presentations such as acute urinary retention are uncommon and pose diagnostic challenges. Early identification and treatment are crucial to prevent long-term complications like endometriosis and infertility.
A 12-year-old female presented with the unusual symptom of acute urinary retention, which was likely caused by compression of the bladder neck due to hematocolpos. Imaging via ultrasound and MRI confirmed the presence of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis, consistent with HWWS. Surgical excision of the vaginal septum was performed to relieve the hematocolpos, with the patient experiencing a full recovery.
Congenital abnormalities of Mullerian Duct are rare, and atypical presentations such as acute urinary retention, vaginal discharge, and even pyocolpos or hematosalpinx have been reported. MRI played a crucial role in confirming the diagnosis and guiding treatment. The importance of early detection and long-term monitoring is highlighted, though limitations exist due to the inability to assess future reproductive outcomes.
This case emphasizes the importance of recognizing atypical presentations of HWWS, such as acute urinary retention. Timely diagnosis and appropriate surgical intervention are critical for preventing complications and restoring normal function. Early recognition of this rare syndrome, especially in patients with unusual symptoms such as urine retention, is essential for ensuring optimal clinical outcomes.
赫林-韦纳-温德利希综合征（HWWS）是一种罕见的先天性异常，其特征为双子宫、单侧梗阻性半阴道和同侧肾缺如三联征。然而，诸如急性尿潴留等非典型表现并不常见，且带来诊断挑战。早期识别和治疗对于预防诸如子宫内膜异位症和不孕症等长期并发症至关重要。
一名12岁女性出现急性尿潴留这一异常症状，可能是由于阴道积血压迫膀胱颈所致。超声和磁共振成像（MRI）检查证实存在双子宫、梗阻性半阴道和同侧肾缺如，符合HWWS。进行了阴道纵隔手术切除以缓解阴道积血，患者完全康复。
苗勒管先天性异常罕见，已报道过诸如急性尿潴留、阴道分泌物增多，甚至积脓或输卵管积血等非典型表现。MRI在确诊和指导治疗方面发挥了关键作用。强调了早期检测和长期监测的重要性，不过由于无法评估未来生殖结局存在局限性。
本病例强调了识别HWWS非典型表现（如急性尿潴留）的重要性。及时诊断和适当的手术干预对于预防并发症和恢复正常功能至关重要。早期识别这种罕见综合征，尤其是对于有诸如尿潴留等异常症状的患者，对于确保最佳临床结局至关重要。
