Osornio-Sánchez V, Santana-Ríos Z, Fulda-Graue SD, Pérez-Becerra R, Urdiales-Ortiz A, Martínez Á, Fernández-Noyola G, Ahumada-Tamayo S, Camacho-Castro A, Muñoz-Ibarra E, García-Salcido F, Garza-Sainz G, Mayorga É, Martínez-Arroyo C, Cantellano-Orozco M, Morales-Montor G, Pacheco-Gahbler C

Language: Spanish
References: 6
Page: 31-34
PDF size: 1148.50 Kb.

ABSTRACT

Introduction: Uterus didelphys with blind hemivagina and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome) is a rare congenital anomaly. There is severe dysmenorrhea and palpable mass due to unilateral hematocolpos. A case of Herlyn-Werner-Wunderlich syndrome with left ureteric bud and bladder diverticulum is presented here.
Clinical case: Patient is an eighteen-year-old female who experienced menarche at twelve years of age. Menstrual cycles lasted twenty-eight days with menstrual bleeding for four days and dysmenorrhea. Left salpingo-oophorectomy was carried out three years prior due to hematosalpinx and ovarian cysts. Disease onset presented with dysuria, cloudy and fetid urine, and recurrent urinary infection symptoms that improved with antimicrobial treatment. Physical examination revealed Tanner IV, normal external genitals, and septate vagina. Imaging studies showed left renal agenesis, left ureterocele, and bladder diverticulum. Cystoscopy revealed left ureterocele and was deroofed by endoscopy. Retrograde pyelography revealed left ureteric bud. Open procedures of left ureterectomy and bladder diverticulectomy were later carried out.
Discussion: The Wolffian ducts give rise to the kidneys and induce Müllerian duct fusion; abnormality in Wolffian duct caudal development is the cause of unilateral renal agenesis. The Müllerian duct on the same side as the absent Wolffian duct is laterally displaced without fusing with the contralateral duct. This results in bicornuate uterus and there is no contact with the central urogenital sinus. The contralateral Müllerian duct gives rise to a vagina and the displaced component forms a blind sac.
Conclusions: It is important to detect these types of rare abnormalities that cause precocious symptomatology, pelvic endometriosis, and collection infections.

REFERENCES

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