Journal of Obstetrics and Gynaecology, 2014; Early Online: 1–2 © 2014 Informa UK, Ltd. ISSN 0144-3615 print/ISSN 1364-6893 online

CASE REPORT

Finding the obstructed hemivagina in uterus didelphys

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D. M. da Silva Coelho1, R. F. Sousa Santos1,2 & M. L. Graça Fernandes1 1Department of Obstetrics and Gynaecology, Centro Hospitalar do Alto Ave, Guimarães and 2CINTESIS, Faculty of Medicine, University of Porto, Porto, Portugal

DOI: 10.3109/01443615.2014.915292 Correspondence: D. M. da Silva Coelho, Rua dos Cutileiros, Creixomil, 4835-044 Guimarães, Portugal. E-mail: [email protected]

Introduction Müllerian malformations include a broad range of anomalies, resulting from the incomplete formation and/or differentiation of Müllerian ducts. Uterus didelphys with obstructed hemivagina is the result of a lateral non-fusion of the Müllerian ducts with asymmetric obstruction (Golan et al. 1999). Uterus didelphys arises from midline fusion arrest of the Müllerian ducts, in either a complete or incomplete form, and it comprises 11% of uterine malformations. The complete form has two hemiuteri and two endocervical canals, with cervices fused at the lower uterine segment. The vagina may be single or double, in which case a longitudinal septum is present that extends either completely or partially from the cervices to the introitus (Zurawin et al. 2004). Renal agenesis associated with uterus didelphys is more frequent than with any other type of Müllerian anomaly. The reported incidence of renal anomalies in this group of patients is 20% (Tanaka et al. 1998). Patients are usually asymptomatic unless there is an obstruction, in which case haematometrocolpos, haematometra and haematosalpinx may develop. In hemivaginal obstruction, the most common presenting symptoms are onset of dysmenorrhoea within the first years following menarche and progressive pelvic pain. A unilateral pelvic mass may be present (Vercellini et al. 2007). An obstructed unilateral vagina is an indication for vaginal septum resection. Surgery is necessary to preserve reproductive capacity and to prevent uterine and tubal impairment. Unless the obstruction is readily removed after diagnosis, retrograde menstruation continues and haematometra, haematosalpinx, endometriosis and pelvic adhesions may develop.

Case report A 16-year-old girl with a dysmenorrhoea complaint since menarche and progressive pelvic pain was referred to our institution. She had a history of left nephrectomy for a non-functional pelvic kidney at the age of 9. At physical examination, she had an intact annular hymen but no palpable abdominal masses or tenderness. Transabdominal ultrasound and magnetic resonance imaging (MRI) revealed a complete uterus didelphys with a double vagina that was distended on the left side with a complete septum (Figure 1). The patient was medicated with a combined oral contraceptive (COC) pill to trigger withdrawal bleeding and subjected to diagnostic laparoscopy and vaginal septum removal, allowing left hemi-vagina opening. During the procedure, two uterine formations suggestive of didelphys uterus were observed at laparoscopy. Right tube permeability was confirmed laparoscopically after chromopertubation. Peroperative hysterosalpingography showed only a right uterine cavity with respective tubal permeability.

Figure 1. Pelvic magnetic resonance imaging revealing complete didelphys uterus with double vagina, distended on the right side with a complete septum.

After speculum positioning, an atretic right cervix was observed, but no bulging was present, which indicated the presence of the blind hemivagina. Intraoperative transvaginal ultrasound showed two uterine cavities, the right one with normal size/morphology, and a thin endometrium. The left hemiuterus was larger and had a thicker endometrium with clots inside (Figure 1). After instillation of 70 cc of patent blue dye into the left uterine cavity through ultrasound-guided transuterine puncture, distension of the uterine cavity was noted, together with the appearance of a liquid collection on the isthmic region, just left of the womb, and a bulged and bluish left vaginal wall, which presented patent blue outflow when punctured with a large-gauge needle. The left hemivagina was open and the vaginal septum was totally excised, leaving two distinct cervices at inspection. Finally, left cervix chromopertubation confirmed left tubal patency. The postoperative period was uneventful, and recovery was rapid. Clinical reassessment 2 months after the operation confirmed the complete resolution of clinical symptoms.

Discussion The triad of obstructed hemivagina, renal anomaly and uterus didelphys is an uncommon cause of abdominal pain and menstrual discomfort in adolescent girls. Accurate diagnosis and surgical treatment can be delayed for several months or even years. Adolescent girls presenting with these symptoms should have a baseline pelvic ultrasound scan to establish uterine anatomy. In girls with ultrasound suspicion of Müllerian anomalies, the appropriate follow-up includes MRI in addition to an early consultation and referral to a centre with experience in the management of these rare conditions (Kimble et al. 2009). At this time, MRI is the imaging modality of choice because of its high accuracy and detailed elaboration of uterovaginal anatomy (Troiano and McCarthy 2004). The obstructed unilateral vagina is an indication for vaginal septum resection. The preferred surgical approach is full excision and marsupialisation of the vaginal septum, and it is performed as a single procedure. After the septum has been excised, laparoscopy can be performed for treatment of potentially associated endometriosis and/or adhesions (Stassart et al. 1992). In this particular case, it was difficult to localise the obstructed hemivagina. An attempt to create a haematocolpos through a COC

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cycle was unsuccessful; therefore, it was decided to dilate the uterine cavity and obstructed hemivagina through patent blue dye instillation to locate the hidden hemivagina. For this purpose, we used a needle designed for transvaginal oocyte retrieval. Although this patient had an intact hymen, the vaginal valves were used for better exposure. Successful preservation of hymenal integrity has been reported with the vaginoscopic approach (Cetinkaya et al. 2011). Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

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References Cetinkaya SE, Kahraman K, Sonmezer M et al. 2011. Hysteroscopic management of vaginal septum in a virginal patient with uterus didelphys and obstructed hemivagina. Fertility and Sterility 96:16–18.

Golan A, Langer R, Bukovsky I et al. 1999. Congenital anomalies of the Mullerian system. Fertility and Sterility 51:747–755. Kimble RM, Khoo SK, Baartz D. 2009. The obstructed hemivagina, ipsilateral renal anomaly, uterus didelphys triad. Australian and New Zealand Journal of Obstetrics and Gynaecology 49:554–557. Stassart JP, Nagel TC, Prem KA et al. 1992. Uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis: the University of Minnesota experience. Fertility and Sterility 57:756–761. Tanaka YO, Kurosaki Y, Kobayashi T et al. 1998. Uterus didelphys associated with obstructed hemivagina and ipsilateral renal agenesis: MR findings in seven cases. Abdominal Imaging 23:437–441. Troiano RN, McCarthy SM. 2004. Mullerian duct anomalies: imaging and clinical issues. Radiology 233:19–34. Vercellini P, Daguati R, Somigliana E. 2007. Asymmetric lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys: institutional case series and a systematic literature review. Fertility and Sterility 87:719–724. Zurawin RK, Dietrich JE, Heard MJ et al. 2004. Didelphic uterus and obstructed hemivagina with renal agenesis: case report and review of the literature. Journal of Pediatric and Adolescence Gynecology 17:137–141.

Finding the obstructed hemivagina in uterus didelphys.

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