Herlyn-Werner-Wunderlich Syndrome with Ureteric Remnant Abscess Managed Laparoscopically: A Case Report

The incidence of anomalies related to the Müllerian ducts is 2−3% in the general population. Around 0.16−10% of these anomalies are responsible for the HWW syndrome, characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis.2−3 The case is normally diagnosed following an episode of postmenarche pelvic pain secondary to hematocolpos.2 Early diagnosis is imperative to prevent further complications related to urogenital anomalies that may lead to infertility. For detection of the condition, CT, ultrasonography, and MRI are the imaging tests used. However, laparoscopy may be useful when imaging tests are inconclusive.4,5 Of these tests, CT and ultrasonography are the most widely used diagnostic tools, although MRI is considered as the most sensitive imaging method for the evaluation of soft tissue anatomy and characterization of findings associated with anomalies. MRI is recommended before any surgical intervention.6 This syndrome may be accompanied by secondary complications such as endometriosis, infections, and pelvic adhesions. Furthermore, multiple alterations in the urinary tract may occur such as renal agenesis (more common and predominant on the right side), horseshoe kidney, pelvic kidney, cystic renal dysplasia, duplicated collecting system, and ectopic ureter. Thus, due to the intimate relationship between genital and urinary anomalies in females, urinary tract investigation is fundamental when there are genital anomalies and vice-versa.6 In this case report, the patient had already undergone surgery for correction of genital alterations related to the syndrome. Contrast-enhanced abdominal CT performed months ago have failed to identify any significant changes indicating the cause of the pain. Retrospectively, CT images were re-evaluated. An image of the right hydroureter was found, which was erroneously interpreted as hydrosalpinx in the first evaluation. Since clinical conditions characterized by acute or chronic pelvic pain associated with the syndrome were generally determined by complications related to the genital tract, the main diagnostic hypothesis was endometriosis at a later date, due to persistent pelvic pain. MRI was ordered for further evaluation. Alterations identified in the illustrated case alerted to the possibility of changes in the urinary system that may be pain triggers in this clinical scenario. Therefore, imaging studies of the urinary tract are fundamental in paramesonephric duct anomalies, particularly in the HWW syndrome.

Up to the present, we failed to find any article in the literature reporting a case of HWW syndrome complicated by abscess in a ureteric remnant, which produced chronic pelvic pain. In this case, the ureter was removed and showed no signs of malignancy. The proximal third of the ureter was blind ending and the distal portion was atretic, preventing its drainage to the bladder and recognition by cystoscopy. Thus, appropriate diagnosis was critical for therapeutic planning and chronic pain resolution. Disclosure The authors declared no conflicts of interest.

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