Clinical Imaging 38 (2014) 357–359
Contents lists available at ScienceDirect
Clinical Imaging journal homepage: http://www.clinicalimaging.org
OHVIRA syndrome presenting with acute abdomen: a case report and review of the literature☆ Funda Gungor Ugurlucan a, Ercan Bastu a,⁎, Gokce Gulsen b, Meryem Kurek Eken a, Suleyman Engin Akhan a a b
Istanbul University School of Medicine, Department of Obstetrics and Gynecology, Istanbul, Turkey Istanbul Haseki State Hospital, Department of Radiology, Istanbul, Turkey
a r t i c l e
i n f o
Article history: Received 25 June 2013 Received in revised form 3 December 2013 Accepted 16 December 2013 Keywords: OHVIRA syndrome Obstructed hemivagina Mullerian anomaly Vaginoplasty Uterus didelphys Unilateral hysterectomy
a b s t r a c t Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) or Herlyn-WernerWunderlich (HWW) syndrome is a rare congenital urogenital anomaly. A 13-year-old female presented with acute abdominal pain and dysmenorrhea. Ultrasonography and magnetic resonance imaging showed uterus didelphys, hematometrocolpos, obstructed hemivagina, and right renal agenesis. Hemivaginal septal resection and anastomosis between the obstructed hemivagina and the normal vagina was tried, but it was not possible. Unilateral hysterectomy was performed. HWW syndrome may present with acute abdomen and is usually treated with vaginal septum resection and drainage of the hematometrocolpos. © 2014 Elsevier Inc. All rights reserved.
1. Introduction Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) or Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital urogenital anomaly. Usually it is diagnosed after menarche due to recurrent severe dysmenorrhea. Here we report a case of OHVIRA syndrome that presented with acute abdomen and review the literature. This case report is important to emphasize that although rarely encountered, uterus didelphys with obstructed hemivagina should be in the differential diagnosis of adolescent girls presenting with acute abdomen. 2. Case A 13-year-old female presented to our emergency department with complaints of acute severe abdominal pain and dysmenorrhea. Her medical and surgical history was unremarkable. Her menarche was six months ago at the age of 13 and she had regular menstrual cycles, once every 30 days with 3- to 4-day. At the abdominal and gynecologic examination there was lower abdominal tenderness and a palpable pelvic mass on the right side. Abdominal and pelvic ultrasonography revealed a 50×45 mm cystic lesion with welldefined borders and internal echoes in the pelvis and left lower ☆ Disclosure: The authors declare no conflict of interest. ⁎ Corresponding author at: Istanbul University School of Medicine, Department of Obstetrics and Gynecology, Capa 34093, Istanbul, Turkey. Tel.: +90 532 413 4195. E-mail address: [email protected] (E. Bastu). 0899-7071/$ – see front matter © 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.clinimag.2013.12.011
abdomen posterior to the uterus. Both ovaries were normal. Right kidney was not visualized. Magnetic resonance imaging (MRI) revealed uterus didelphys and a single vagina. The left uterus, cervix, and vagina were normal. Between the cervix on the right side and the proximal vagina on the left, there was a 5 cm mass filled with fluid suggestive of hematometrocolpos observed (Fig. 1a–c). Both ovaries were normal. The right kidney was not visualized. Regarding right renal agenesis, uterus didelphys, unilateral obstructed hemivagina with resultant hematometrocolpos, the case was diagnosed as OHVIRA or HWW syndrome. Under general anesthesia and dorsal lithotomy position, examination with virgin-size speculum revealed a single normal vagina and one cervix. Hemivaginal septal resection and anastomosis between the obstructed hemivagina and the normal vagina was planned. But the hematometrocolpos could not be visualized through the normal vagina. In order to determine the right pathway for anastomosis, a 16gauge needle (Cook, Australia) with TVUSG guidance was used to puncture through the vaginal fornix to enter the hematometrocolpos located posterior to the left vagina. But due to the thick fibrotic tissue, which was over 8 cm in length between the hematometrocolpos and the normal vagina, anastomosis was not possible. A mini-laparotomy was performed. Hematometrocolpos and hematosalpinx were observed on the right side, the left uterus and salpinx were normal. There was a fibrotic band between the two uterine corpuses. Both ovaries were normal. Bimanuel palpation revealed that the uterus on the right side ended in the obstructed hemivagina. Unilateral hysterectomy was performed and the obstructed hemivagina was resected. No complications developed during the procedure. The
358
F. Gungor Ugurlucan et al. / Clinical Imaging 38 (2014) 357–359
patient was discharged on the postoperative 3rd day. She has regular menstrual periods with no complications. 3. Discussion Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is named as HWW or OHVIRA syndrome [1]. The association of renal agenesis with ipsilateral blind hemivagina was reported as Herlyn–Werner syndrome in 1971, and the association of renal aplasia, bicornuate uterus with isolated hematocervix, and a normal vagina were reported by Wunderlich in 1976 [2]. The incidence of uterus didelphys, related to HWW, is approximately 1/2,000 to 1/28,000, and it is accompanied by unilateral renal agenesis in 43% of cases [3]. The pathogenesis of OHVIRA syndrome has been related to anomalous development of both paramesonephric (Mullerian) and mesonephric (Wolffian) ducts [4]. The Wolffian ducts induce the normal development of Mullerian ducts in addition to giving origin to kidneys. Abnormal development of the Wolffian ducts leads to unilateral renal agenesis and imperforate hemivagina associated with OHVIRA syndrome [1]. On the side where the Wolffian duct is absent, the Mullerian duct is displaced laterally and cannot fuse with the contralateral duct, resulting in uterus didelphys with fully developed uterine horns and two cervices [4]. However, the displaced Mullerian duct that cannot come into contact with the urogenital sinus leads to an imperforate or obstructed hemivagina. A longitudinal or transverse vaginal septum may accompany [4,5]. The diagnosis is usually made at puberty, shortly after menarche because of the cyclic, increasing lower abdominal pain secondary to ever-increasing hematometrocolpos due to obstructed hemivagina [2]. This syndrome may remain unrecognized if not suspected because of normal menstrual flow through the unobstructed hemivagina and symptoms resembling dysmenorrhea, which is common among this age group [4]. Rarely, cases may be diagnosed in the prenatal and newborn period due to renal agenesis or muco- or hydrocolpos [6–9]. There are only a few reports of cases presenting with acute abdominal pain as in our case or abnormal vaginal discharge, or even acute retention of urine [4,10,11]. Other etiologies causing acute abdomen such as ovarian torsion or acute appendicitis must be ruled out. Early diagnosis and surgery are essential to relieve the symptoms and prevent complications such as endometriosis and infertility. Ultrasonography and MRI are used for diagnosis. Ultrasonography is very helpful in detection of hematocolpos and identification of the type of Mullerian anomaly [3]. MRI is the gold standard for diagnosis with higher sensitivity in detecting the uterine morphology and the continuity of the vagina when compared to ultrasonography [12]. However, three-dimensional ultrasonography may also be used with high sensitivity and specificity. We believe that laparoscopy is not needed for the diagnosis of most of the cases, since it can be done adequately by ultrasonography and MRI findings. The treatment of choice for OHVIRA syndrome is resection of the vaginal septum in order to relieve the obstructed hemivagina. Smith and Laufer reported the management of OHVIRA syndrome in their retrospective study of twenty-seven cases [13]. Twenty-six patients underwent vaginal reconstruction, and eight of those additionally underwent laparoscopy for clarification of diagnosis. Six required a
Fig. 1. (a) T2-weighted turbo-spin-echo MR image in coronal view showing the twouterin corpuses (indicated with blue arrows) and the 5 cm mass filled with fluid suggestive of hematometrocolpos on the right side (indicated with red arrow).(b) T2weighted turbo-spin-echo MR image in sagittal view showing the 5 cm mass filled with fluid suggestive of hematometrocolpos posterior to the bladder (indicated with red arrow) and inferior to the uterine corpus (indicated with blue arrow).(c) T1-weighted turbo-spine echo MR image in axial view showing 5 cm mass filled with fluid suggestive of hematometrocolpos (indicated with red arrow) on the right side of the uterine corpus (indicated with blue arrow). (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)
F. Gungor Ugurlucan et al. / Clinical Imaging 38 (2014) 357–359
two-stage vaginoplasty because of incomplete previous resection, infection or anatomic distortion, or restenosis. They suggested that most patients with OHVIRA syndrome can be treated solely with single-stage vaginoplasty and routine laparoscopy was not essential to management. Hysteroscopic resection of the septum under transabdominal guidance has also been reported in order to preserve the hymenal integrity [14]. Rarely, as in our case, resection of the vaginal septum may not be possible to relieve the hematometrocolpos or recurrent vaginal stenosis may develop and these cases may mandate a unilateral hysterectomy [15,16]. In conclusion, OHVIRA or HWW syndrome is a rare congenital urogenital anomaly that may present with acute abdominal pain. It is usually treated with vaginal septum resection and drainage of the hematometrocolpos. In cases where septum resection cannot be accomplished or recurrent stenosis develops, unilateral hysterectomy is the treatment of choice. References [1] Orazi C, Lucchetti MC, Schingo PM, Marchetti P, Ferro F. Herlyn-WernerWunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases. Pediatr Radiol 2007;37: 657–65. [2] Park NH, Park HJ, Park CS, Park SI. Herlyn-Werner-Wunderlich Syndrome with unilateral hemivaginal obstruction, ipsilateral renal agenesis, and contralateral renal thin GBM disease: a case report with radiological follow up. J Korean Soc Radiol 2010;62:383–8. [3] Del Vescovo R, Battisti S, Di Paola V, Piccolo CL, Cazzato RL, Sansoni I, Grasso RF, Zobel BB. Herlyn-Werner-Wunderlich syndrome: MRI findings, radiological guide (two cases and literature review) and differential diagnosis. BMC Med Imaging 2012;12:4.
359
[4] Mandava A, Prabhakar RR, Smitha S. OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly) with uterus didelphys, an unusual presentation. J Pediatr Adolesc Gynecol 2012;25:e23–5. [5] Troiano RN, McCarthy SM. Mullerian duct anomalies: imaging and clinical issues. State of the art. Radiology 2004;233:19–34. [6] Prada Arias M, Muguerza Vellibre R, Montero Sanchez M, Vázquez Castelo JL, Arias González M, Rodríguez Costa A. Uterus didelphys with obstructed hemivagina and multicystic dysplastic kidney. Eur J Pediatr Surg 2005;15:441. [7] 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;34:628. [8] Burbige KA, Hensle TW. Uterus didelphys and vaginal duplication with unilateral obstruction presenting as a newborn abdominal mass. J Urol 1984;132:1195. [9] Han BH, Park SB, Lee YJ, Lee KS, Lee YK. Uterus didelphys with blind hemivagina and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome) suspected on the presence of hydrocolpos on prenatal sonography. J Clin Ultrasound Jun 7 2012. http://dx.doi.org/10.1002/jcu.21950 (Epub ahead of print). [10] Broseta E, Boronat F, Ruiz JL, Alonso M, Osca JM, Jiménez-Cruz JF. Urological complications associated to uterus didelphys with unilateral hematocolpos. A case report and review of the literature. Eur Urol 1991;20:85–8. [11] Adams Jr GW, Wilson CE, Holloway JH, Williamson HF. Uterus didelphys with unilateral imperforate vagina: a rare cause of acute urinary retention. J Urol 1979;121:131–2. [12] Dhar H, Razek YA, Hamdi I. Uterus didelphys with obstructed hemivagina, ipsilateral renal agenesis and right pyocolpos: a case report. Oman Med J 2011; 26(6):447–50. [13] Smith NA, Laufer MR. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertil Steril 2007;87:918–22. [14] Kim TE, Lee GH, Choi YM, Jee BC, Ku SY, Suh CS, Kim SH, Kim JG, Moon SY. Hysteroscopic resection of the vaginal septum in uterus didelphys with obstructed hemivagina: a case report. J Korean Med Sci 2007;22(4):766–9. [15] Donnez O, Jadoul P, Squifflet J, Donnez J. Didelphic uterus and obstructed hemivagina: recurrent hematometra in spite of appropriate classic surgical treatment. Gynecol Obstet Invest 2007;63:98. [16] Guducu N, Gonenc G, Isci H, Yigiter AB, Dunder I. Herlyn-Werner-Wunderlich syndrome: timely diagnosis is important to preserve fertility. J Pediatr Adolesc Gynecol 2012;25:e111–2.
Contents lists available at ScienceDirect
Clinical Imaging journal homepage: http://www.clinicalimaging.org
OHVIRA syndrome presenting with acute abdomen: a case report and review of the literature☆ Funda Gungor Ugurlucan a, Ercan Bastu a,⁎, Gokce Gulsen b, Meryem Kurek Eken a, Suleyman Engin Akhan a a b
Istanbul University School of Medicine, Department of Obstetrics and Gynecology, Istanbul, Turkey Istanbul Haseki State Hospital, Department of Radiology, Istanbul, Turkey
a r t i c l e
i n f o
Article history: Received 25 June 2013 Received in revised form 3 December 2013 Accepted 16 December 2013 Keywords: OHVIRA syndrome Obstructed hemivagina Mullerian anomaly Vaginoplasty Uterus didelphys Unilateral hysterectomy
a b s t r a c t Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) or Herlyn-WernerWunderlich (HWW) syndrome is a rare congenital urogenital anomaly. A 13-year-old female presented with acute abdominal pain and dysmenorrhea. Ultrasonography and magnetic resonance imaging showed uterus didelphys, hematometrocolpos, obstructed hemivagina, and right renal agenesis. Hemivaginal septal resection and anastomosis between the obstructed hemivagina and the normal vagina was tried, but it was not possible. Unilateral hysterectomy was performed. HWW syndrome may present with acute abdomen and is usually treated with vaginal septum resection and drainage of the hematometrocolpos. © 2014 Elsevier Inc. All rights reserved.
1. Introduction Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) or Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital urogenital anomaly. Usually it is diagnosed after menarche due to recurrent severe dysmenorrhea. Here we report a case of OHVIRA syndrome that presented with acute abdomen and review the literature. This case report is important to emphasize that although rarely encountered, uterus didelphys with obstructed hemivagina should be in the differential diagnosis of adolescent girls presenting with acute abdomen. 2. Case A 13-year-old female presented to our emergency department with complaints of acute severe abdominal pain and dysmenorrhea. Her medical and surgical history was unremarkable. Her menarche was six months ago at the age of 13 and she had regular menstrual cycles, once every 30 days with 3- to 4-day. At the abdominal and gynecologic examination there was lower abdominal tenderness and a palpable pelvic mass on the right side. Abdominal and pelvic ultrasonography revealed a 50×45 mm cystic lesion with welldefined borders and internal echoes in the pelvis and left lower ☆ Disclosure: The authors declare no conflict of interest. ⁎ Corresponding author at: Istanbul University School of Medicine, Department of Obstetrics and Gynecology, Capa 34093, Istanbul, Turkey. Tel.: +90 532 413 4195. E-mail address: [email protected] (E. Bastu). 0899-7071/$ – see front matter © 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.clinimag.2013.12.011
abdomen posterior to the uterus. Both ovaries were normal. Right kidney was not visualized. Magnetic resonance imaging (MRI) revealed uterus didelphys and a single vagina. The left uterus, cervix, and vagina were normal. Between the cervix on the right side and the proximal vagina on the left, there was a 5 cm mass filled with fluid suggestive of hematometrocolpos observed (Fig. 1a–c). Both ovaries were normal. The right kidney was not visualized. Regarding right renal agenesis, uterus didelphys, unilateral obstructed hemivagina with resultant hematometrocolpos, the case was diagnosed as OHVIRA or HWW syndrome. Under general anesthesia and dorsal lithotomy position, examination with virgin-size speculum revealed a single normal vagina and one cervix. Hemivaginal septal resection and anastomosis between the obstructed hemivagina and the normal vagina was planned. But the hematometrocolpos could not be visualized through the normal vagina. In order to determine the right pathway for anastomosis, a 16gauge needle (Cook, Australia) with TVUSG guidance was used to puncture through the vaginal fornix to enter the hematometrocolpos located posterior to the left vagina. But due to the thick fibrotic tissue, which was over 8 cm in length between the hematometrocolpos and the normal vagina, anastomosis was not possible. A mini-laparotomy was performed. Hematometrocolpos and hematosalpinx were observed on the right side, the left uterus and salpinx were normal. There was a fibrotic band between the two uterine corpuses. Both ovaries were normal. Bimanuel palpation revealed that the uterus on the right side ended in the obstructed hemivagina. Unilateral hysterectomy was performed and the obstructed hemivagina was resected. No complications developed during the procedure. The
358
F. Gungor Ugurlucan et al. / Clinical Imaging 38 (2014) 357–359
patient was discharged on the postoperative 3rd day. She has regular menstrual periods with no complications. 3. Discussion Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is named as HWW or OHVIRA syndrome [1]. The association of renal agenesis with ipsilateral blind hemivagina was reported as Herlyn–Werner syndrome in 1971, and the association of renal aplasia, bicornuate uterus with isolated hematocervix, and a normal vagina were reported by Wunderlich in 1976 [2]. The incidence of uterus didelphys, related to HWW, is approximately 1/2,000 to 1/28,000, and it is accompanied by unilateral renal agenesis in 43% of cases [3]. The pathogenesis of OHVIRA syndrome has been related to anomalous development of both paramesonephric (Mullerian) and mesonephric (Wolffian) ducts [4]. The Wolffian ducts induce the normal development of Mullerian ducts in addition to giving origin to kidneys. Abnormal development of the Wolffian ducts leads to unilateral renal agenesis and imperforate hemivagina associated with OHVIRA syndrome [1]. On the side where the Wolffian duct is absent, the Mullerian duct is displaced laterally and cannot fuse with the contralateral duct, resulting in uterus didelphys with fully developed uterine horns and two cervices [4]. However, the displaced Mullerian duct that cannot come into contact with the urogenital sinus leads to an imperforate or obstructed hemivagina. A longitudinal or transverse vaginal septum may accompany [4,5]. The diagnosis is usually made at puberty, shortly after menarche because of the cyclic, increasing lower abdominal pain secondary to ever-increasing hematometrocolpos due to obstructed hemivagina [2]. This syndrome may remain unrecognized if not suspected because of normal menstrual flow through the unobstructed hemivagina and symptoms resembling dysmenorrhea, which is common among this age group [4]. Rarely, cases may be diagnosed in the prenatal and newborn period due to renal agenesis or muco- or hydrocolpos [6–9]. There are only a few reports of cases presenting with acute abdominal pain as in our case or abnormal vaginal discharge, or even acute retention of urine [4,10,11]. Other etiologies causing acute abdomen such as ovarian torsion or acute appendicitis must be ruled out. Early diagnosis and surgery are essential to relieve the symptoms and prevent complications such as endometriosis and infertility. Ultrasonography and MRI are used for diagnosis. Ultrasonography is very helpful in detection of hematocolpos and identification of the type of Mullerian anomaly [3]. MRI is the gold standard for diagnosis with higher sensitivity in detecting the uterine morphology and the continuity of the vagina when compared to ultrasonography [12]. However, three-dimensional ultrasonography may also be used with high sensitivity and specificity. We believe that laparoscopy is not needed for the diagnosis of most of the cases, since it can be done adequately by ultrasonography and MRI findings. The treatment of choice for OHVIRA syndrome is resection of the vaginal septum in order to relieve the obstructed hemivagina. Smith and Laufer reported the management of OHVIRA syndrome in their retrospective study of twenty-seven cases [13]. Twenty-six patients underwent vaginal reconstruction, and eight of those additionally underwent laparoscopy for clarification of diagnosis. Six required a
Fig. 1. (a) T2-weighted turbo-spin-echo MR image in coronal view showing the twouterin corpuses (indicated with blue arrows) and the 5 cm mass filled with fluid suggestive of hematometrocolpos on the right side (indicated with red arrow).(b) T2weighted turbo-spin-echo MR image in sagittal view showing the 5 cm mass filled with fluid suggestive of hematometrocolpos posterior to the bladder (indicated with red arrow) and inferior to the uterine corpus (indicated with blue arrow).(c) T1-weighted turbo-spine echo MR image in axial view showing 5 cm mass filled with fluid suggestive of hematometrocolpos (indicated with red arrow) on the right side of the uterine corpus (indicated with blue arrow). (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)
F. Gungor Ugurlucan et al. / Clinical Imaging 38 (2014) 357–359
two-stage vaginoplasty because of incomplete previous resection, infection or anatomic distortion, or restenosis. They suggested that most patients with OHVIRA syndrome can be treated solely with single-stage vaginoplasty and routine laparoscopy was not essential to management. Hysteroscopic resection of the septum under transabdominal guidance has also been reported in order to preserve the hymenal integrity [14]. Rarely, as in our case, resection of the vaginal septum may not be possible to relieve the hematometrocolpos or recurrent vaginal stenosis may develop and these cases may mandate a unilateral hysterectomy [15,16]. In conclusion, OHVIRA or HWW syndrome is a rare congenital urogenital anomaly that may present with acute abdominal pain. It is usually treated with vaginal septum resection and drainage of the hematometrocolpos. In cases where septum resection cannot be accomplished or recurrent stenosis develops, unilateral hysterectomy is the treatment of choice. References [1] Orazi C, Lucchetti MC, Schingo PM, Marchetti P, Ferro F. Herlyn-WernerWunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases. Pediatr Radiol 2007;37: 657–65. [2] Park NH, Park HJ, Park CS, Park SI. Herlyn-Werner-Wunderlich Syndrome with unilateral hemivaginal obstruction, ipsilateral renal agenesis, and contralateral renal thin GBM disease: a case report with radiological follow up. J Korean Soc Radiol 2010;62:383–8. [3] Del Vescovo R, Battisti S, Di Paola V, Piccolo CL, Cazzato RL, Sansoni I, Grasso RF, Zobel BB. Herlyn-Werner-Wunderlich syndrome: MRI findings, radiological guide (two cases and literature review) and differential diagnosis. BMC Med Imaging 2012;12:4.
359
[4] Mandava A, Prabhakar RR, Smitha S. OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly) with uterus didelphys, an unusual presentation. J Pediatr Adolesc Gynecol 2012;25:e23–5. [5] Troiano RN, McCarthy SM. Mullerian duct anomalies: imaging and clinical issues. State of the art. Radiology 2004;233:19–34. [6] Prada Arias M, Muguerza Vellibre R, Montero Sanchez M, Vázquez Castelo JL, Arias González M, Rodríguez Costa A. Uterus didelphys with obstructed hemivagina and multicystic dysplastic kidney. Eur J Pediatr Surg 2005;15:441. [7] 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;34:628. [8] Burbige KA, Hensle TW. Uterus didelphys and vaginal duplication with unilateral obstruction presenting as a newborn abdominal mass. J Urol 1984;132:1195. [9] Han BH, Park SB, Lee YJ, Lee KS, Lee YK. Uterus didelphys with blind hemivagina and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome) suspected on the presence of hydrocolpos on prenatal sonography. J Clin Ultrasound Jun 7 2012. http://dx.doi.org/10.1002/jcu.21950 (Epub ahead of print). [10] Broseta E, Boronat F, Ruiz JL, Alonso M, Osca JM, Jiménez-Cruz JF. Urological complications associated to uterus didelphys with unilateral hematocolpos. A case report and review of the literature. Eur Urol 1991;20:85–8. [11] Adams Jr GW, Wilson CE, Holloway JH, Williamson HF. Uterus didelphys with unilateral imperforate vagina: a rare cause of acute urinary retention. J Urol 1979;121:131–2. [12] Dhar H, Razek YA, Hamdi I. Uterus didelphys with obstructed hemivagina, ipsilateral renal agenesis and right pyocolpos: a case report. Oman Med J 2011; 26(6):447–50. [13] Smith NA, Laufer MR. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertil Steril 2007;87:918–22. [14] Kim TE, Lee GH, Choi YM, Jee BC, Ku SY, Suh CS, Kim SH, Kim JG, Moon SY. Hysteroscopic resection of the vaginal septum in uterus didelphys with obstructed hemivagina: a case report. J Korean Med Sci 2007;22(4):766–9. [15] Donnez O, Jadoul P, Squifflet J, Donnez J. Didelphic uterus and obstructed hemivagina: recurrent hematometra in spite of appropriate classic surgical treatment. Gynecol Obstet Invest 2007;63:98. [16] Guducu N, Gonenc G, Isci H, Yigiter AB, Dunder I. Herlyn-Werner-Wunderlich syndrome: timely diagnosis is important to preserve fertility. J Pediatr Adolesc Gynecol 2012;25:e111–2.
