Herlyn-Werner-Wunderlich Syndrome: Uterus Didelphys, Obstructed Hemivagina, and Ipsilateral Renal Agenesis–Role of Sonographically Guided Minimally Invasive Vaginal Surgery

Introduction

The Mullerian ducts are the primordium from which the female reproductive tract is derived. Aberrations in Mullerian duct development such as non fusion, mal-development, defective canalisation of the ducts and failure of uterovaginal septum regression results in a wide spectrum of congenital anomalies of the female reproductive system. The estimated prevalence is 1% in women with normal fertility, with rates rising to as high as 3% in women with repeated pregnancy loss.[1] In this case report, we present a rare variant of a class 3 Mullerian duct anomaly (MDA), known as Herlyn-Werner-Wunderlich syndrome (HWWS) in a 12-year-old girl.

• References

• 1 Troiano RN, McCarthy SM. Mullerian duct anomalies: imaging and clinical issues. Radiology 2004; 233 (1) 19-34
• 2 Kimble RM, Khoo SK, Baartz D. The obstructed hemivagina, ipsilateral renal anomaly, uterus didelphys triad. Aust N Z J Obstet Gynaecol 2009; 49 (5) 554-557
• 3 Gholoum S, Puligandla PS, Hui T , et al. Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome). J Pediatr Surg 2006; 41 (5) 987-992
• 4 Chaim W, Kornmehl P, Kopernik G , et al. Uterus didelphys with unilateral imperforate vagina and ipsilateral renal agenesis: the challenge of noninvasive diagnosis. J Clin Ultrasound 1991; 19 (9) 583-585
• 5 Orazi C, Lucchetti MC, Schingo PM , et al. Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases. Pediatr Radiol 2007; 37 (7) 657-665
• 6 Candiani GB, Fedele L, Candiani M. Double uterus, blind hemivagina, and ipsilateral renal agenesis: 36 cases and long-term follow-up. Obstet Gynecol 1997; 90 (1) 26-32
• 7 Sherer DM, Rib DM, Nowell RM , et al. Sonographic-guided drainage of unilateral hematometrocolpos due to uterus didelphys and obstructed hemivagina associated with ipsilateral renal agenesis. J Clin Ultrasound 1994; 22 (7) 454-456
• 8 Amagai T, Ohkawa H, Kaneko M. Endoscopic septotomy: a new surgical approach to infantile hydrometrocolpos with imperforate hemivagina and ipsilateral renal agenesis. J Pediatr Surg 1999; 34 (4) 628-631
• 9 Prada Arias M, Muguerza Vellibre R, Montero Sanchez M , et al. Uterus didelphys with obstructed hemivagina and multicystic dysplastic kidney. Eur J Pediatr Surg 2005; 15 (6) 441-445
• 10 Haddad B, Barranger E, Paniel BJ. Blind hemivagina: long-term follow-up and reproductive performance in 42 cases. Hum Reprod 1999; 14 (8) 1962-1964
• 11 Green LK, Harris RE. Uterine anomalies. Frequency of diagnosis and associated obstetric complications. Obstet Gynecol 1976; 47 (4) 427-429