Acute urinary retention in an adolescent female with Herlyn-Werner-Wunderlich syndrome

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.