Obstet Gynecol Sci.  2014 Jul;57(4):310-313. 10.5468/ogs.2014.57.4.310.

Complete septate uterus, obstructed hemivagina, and ipsilateral adnexal and renal agenesis in pregnancy

• Kim MS
• Nam SY
• Lee G

• KMID: 1841543
• DOI: http://doi.org/10.5468/ogs.2014.57.4.310

Abstract

Most cases of double uterus with obstructed hemivagina and ipsilateral renal agenesis were diagnosed at adolescents after menarche. This is the first reported case of complete septate uterus with obstructed hemivagina and ipsilateral renal agenesis in addition to ipsilateral agenesis of fallopian tube and ovary in which the diagnosis was delayed until pregnancy. The pregnancy was uneventful in spite of intermittent vaginal spotting. During the cesarean section, the septum of the uterus was resected and about a 3-cmx3-cm window was made on the vaginal septum to allow an opening for the obstructed vaginal discharge. We followed the patient up for one and half years, and she has not had symptoms such as dysmenorrhea or abnormal vaginal bleeding.

Keyword

Hemivagina; Ovarian agenesis; Pregnancy; Renal agenesis; Septate uterus

MeSH Terms

Adolescent
Cesarean Section
Diagnosis
Dysmenorrhea
Fallopian Tubes
Female
Humans
Menarche
Metrorrhagia
Ovary
Pregnancy*
Uterine Hemorrhage
Uterus*
Vaginal Discharge

Figure

• 

  Fig. 1 Two-dimentional ultrasonographic image of septate uterus with hemiobstructed vagina. The gestational sac (GS) in the right (Rt) uterus and fluid collection in the obstructed left (Lt) vagina (white arrows) are shown.

• 

  Fig. 2 Three-dimentional ultrasonographic image of septate uterus with hemiobstructed vagina. The gestaional sac (GS) is seen in the right (Rt) uterus and the left (Lt) obstructed vagina (black arrows) is bulging into the Rt vagina.

Reference

1. Burgis J. Obstructive Müllerian anomalies: case report, diagnosis, and management. Am J Obstet Gynecol. 2001; 185:338–344.

2. Heinonen PK. Clinical implications of the didelphic uterus: long-term follow-up of 49 cases. Eur J Obstet Gynecol Reprod Biol. 2000; 91:183–190.

3. Cox D, Ching BH. Herlyn-Werner-Wunderlich syndrome: a rare presentation with pyocolpos. J Radiol Case Rep. 2012; 6:9–15.

4. Fedele L, Motta F, Frontino G, Restelli E, Bianchi S. Double uterus with obstructed hemivagina and ipsilateral renal agenesis: pelvic anatomic variants in 87 cases. Hum Reprod. 2013; 28:1580–1583.

5. Del Vescovo R, Battisti S, Di Paola V, Piccolo CL, Cazzato RL, Sansoni I, et al. Herlyn-Werner-Wunderlich syndrome: MRI findings, radiological guide (two cases and literature review), and differential diagnosis. BMC Med Imaging. 2012; 12:4.

6. Zurawin RK, Dietrich JE, Heard MJ, Edwards CL. Didelphic uterus and obstructed hemivagina with renal agenesis: case report and review of the literature. J Pediatr Adolesc Gynecol. 2004; 17:137–141.

7. Angthong W, Visetsripong M, Amornvithayachan O, Varavithya V. Herlyn-Werner-Wunderlich syndrome emphasizes ultrasonographic and MR findings: a case report and review of the literatures. J Med Assoc Thai. 2012; 95:Suppl 12. S129–S133.

8. Orazi C, Lucchetti MC, Schingo PM, Marchetti P, Ferro F. Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases. Pediatr Radiol. 2007; 37:657–665.

9. Cicinelli E, Romano F, Didonna T, Schonauer LM, Galantino P, Di Naro E. Resectoscopic treatment of uterus didelphys with unilateral imperforate vagina complicated by hematocolpos and hematometra: case report. Fertil Steril. 1999; 72:553–555.

10. 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; 63:98–101.

11. Haddad B, Barranger E, Paniel BJ. Blind hemivagina: long-term follow-up and reproductive performance in 42 cases. Hum Reprod. 1999; 14:1962–1964.

12. Suh BY, Kalan MJ. Septate uterus with left fallopian tube hypoplasia and ipsilateral ovarian agenesis. J Assist Reprod Genet. 2008; 25:567–569.

13. Lin PC, Bhatnagar KP, Nettleton GS, Nakajima ST. Female genital anomalies affecting reproduction. Fertil Steril. 2002; 78:899–915.

14. Rackow BW, Arici A. Reproductive performance of women with müllerian anomalies. Curr Opin Obstet Gynecol. 2007; 19:229–237.

15. Heinonen PK. Reproductive performance of women with uterine anomalies after abdominal or hysteroscopic metroplasty or no surgical treatment. J Am Assoc Gynecol Laparosc. 1996; 3:4, Supplement. S17.