Herlyn-Werner-Wunderlich syndrome: a rare cause of acute urinary retention in an adolescent girl

Abstract

DESCRIPTIONA 14-year-old girl presented to emergency triage with acute retention of urine. There was history of cyclic pelvic pain for the past 6 months, for which she had been prescribed non-steroidal antiinflammatory drugs by a private practitioner. There was no history of ureteric colic or burning during micturition. Gynaecological history indicated menarche at 12 years followed by regular menses. There was no history of abnormal vaginal bleeding. Ultrasound of the abdomen revealed findings suggestive of bicornuate uterus with haematocolpos. Subsequently, MRI was requisitioned, revealing two widely divergent, symmetrical uterine corpii, partially fused at the cervix without any communication between their endometrial cavities ( figure 1A). There was a thick hypointense left hemivaginal septum with a large ipsilateral haematocolpos appearing hyperintense on T1-weighted and hypointense on T2-weighted images, findings consistent with intracellular methaemoglobin stage of bleed. A similar intensity dilated tortuous

Síndrome de Herlyn-Werner-Wunderlich: reporte de caso y revisión de la literatura

“…En las 74 publicaciones incluidas se encontraron 6 estudios observacionales retrospectivos (4, 13-17), 10 series de casos (10)(11)(18)(19)(20)(21)(22)(23)(24)(25) y 58 reportes de caso (12,, que incluyeron un total de 663 pacientes. La mayoría de las publicaciones fueron en India con 11 estudios (17,29,39,(44)(45)(46)(47)52,60,62,63), seguido de USA con 9 estudios (30,32,53,56,69,70,72,75,78), China (10,11,13,15,24,25,33,67) e It alia (4,20,23,34,37,51,66,71) con 8 estudios cada uno. La serie de casos con mayor población fue publicada en China…”

An Unusual Cause of Recurrent Urinary Retention in an Adolescent Female

“…Uterus didelphys represents a failure of fusion of the paired Mullerian ducts. The obstructed hemivagina results from a transverse vaginal septum, which represents vertical fusion defect consequent to non-resorption of tissue between the vaginal plate that originates from the urogenital sinus and the caudal aspect of the paired Mullerian ducts [11]. The renal agenesis on the ipsilateral side of the obstructed hemivagina reflects the close association of the Wolffian duct relative to the Mullerian ducts in embryological development [12].…”

“…With the increased size and external compression, the uterus can cause urethra angulation which leads to an obstruction [14]. We also hypothesized that haematocolpos reduces the extrusion capacity of the urethral sphincter by irritating the sacral plexus [11]. Though ultrasound can be used for visualization of the genitourinary structures, MRI is the modality of choice for the diagnosis of HWWS and other such anomalies due to the better anatomic delineation of pelvic structures and higher sensitivity for blood products [15, 16].…”

A rare case of pelvic pain caused by Herlyn-Werner-Wunderlich Syndrome in an adult: A case report

“…At that size, it causes urethra angulation which leads to an obstruction. Another hypothesis has stated that haematocolpos reduces the extrusion capacity of the urethral sphincter by irritating the sacral plexus [ 12 ].…”

“…The accuracy of MRI is mentioned up to 100% with the benefit of free radiation, non-invasive, able to assess soft tissue such as mass of endometriosis, pelvic infection and adhesion [ 14 ]. It has good accuracy to distinguish the intra and extra uterine anatomy and secondary complex anomalies such as uterine fibroid, endometriosis and hydrosalpinx which offers a better data for appropriate management [ 12 ]. In order to reduce the cost, CT scan or HSG can be chosen for diagnostic.…”