Presenting symptoms differ with complete and partial obstruction. Median age at presentation is 14 years with a range of 10–29 years, although presentation after age 18 is rare with complete obstruction. With complete obstruction at the level of the vagina presenting symptoms are severe dysmenorrhea, pelvic pain (initially unilateral), chronic pelvic pain, acute urinary retention, and paravaginal mass. For individuals with a partial obstruction, presenting symptoms include prolonged menstrual bleeding or bleeding between menses, unilateral pain, leukorrhea (infected vaginal discharge), foul-odored vaginal discharge, and sepsis. There is often a signifcant delay in diagnosis of months to years in these young women, especially in those with partial obstruction. This delay in diagnosis can lead to complications from retrograde menstruation, which have been observed at laparoscopy and include endometriosis (37%), hematometra (37%), hematosalpinx (22%), and pelvic adhesions (10%) [8]. The sequalae of the delay in diagnosis includes subsequent removal of ovary and fallopian tubes, infertility, and pelvic pain, as well as emotional impact.

The confguration of the uterus seen with obstruction of hemivagina is most commonly uterus didelphys, occurring in approximately 72% of cases. However, it is not the only uterine confguration. The other anomalies seen and their incidence in one study include complete septate uterus (14%), bicornuate bicollis (13%), and uterus didelphys with unilateral cervical atresia in lieu of vaginal obstruction (6%) [2].

Treatment

Goals of treatment are to defnitively correct the Mullerian anomaly in a one-stage procedure and optimize fertility. The size of the obstructed hemivagina is an important consideration when deciding on surgical management. If the distended hemivagina is large and in close proximity to the patent hemivagina, then the most straightforward approach is to create a communication between the two hemivaginas by resecting the vaginal septum. If the obstructed hemivagina is small or far away from the patent vagina, then resection of the vaginal septum carries a high risk of restenosis and consideration should be made to remove the hemi-uterus, cervix, and hemivagina on the obstructed side (Fig. 6.3). Surgical drainage of the hematocolpos is a temporizing measure and should only be employed to treat acute pain when surgical expertise to correct the anomaly is not available. Laparoscopic drainage may be safer and carry less risk than transvaginal drainage. Concern of transvaginal drainage is introduction of bacteria into the obstructed hemivagina with the development of pyometra and risk of sepsis. If drainage of hematocolpos is performed, then referral for defnitive surgery is indicated. The patient may be placed on continuous combined oral contraceptives to suppress menses.

Transvaginal resection of the vaginal septum to treat double uterus with obstructed hemivagina is a relatively straightforward procedure. There is no need for simultaneous laparoscopy unless there is evidence of signifcant upper tract disease on imaging. It is important to have accurate imaging to defne the size and

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Hemi-hysterectomy with removal of the cervix and ipsilateral hemi-vagina is another approach that is typically reserved for those cases where the obstructed hemivagina is small or located far from the normal vagina, making marsupialization diffcult and restenosis likely [10]. In these cases, the procedure may be performed by laparoscopy, robot-assisted laparoscopy, or laparotomy depending on the anatomy and surgeon skill set. It is important to remove the vaginal tissue at the time of hemi-hysterectomy. With arousal the vaginal tissue will secrete uid, which may lead to accumulation of uid in the hemi-vagina and cause pain and dyspareunia. In addition, the ipsilateral fallopian tube should be removed to decrease the risk of ectopic pregnancy.

Discussion

Double uterus with obstructed hemivagina is a well-recognized Mullerian anomaly associated with unilateral out ow obstruction and simultaneous regular menstrual cycles from the non-obstructed side. This anomaly is referred to as double uterus with obstructed hemivagina, or obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), or Herlyn-Werner-Wunderlich syndrome (HWW). This anomaly typically presents shortly after puberty with severe unilateral dysmenorrhea. As many as 95% of individuals have associated ipsilateral renal agenesis, which may be detected during prenatal fetal ultrasonography and should be recognized as a risk factor for the anomaly. Delay in diagnosis is common and may be weeks to years in duration. This delay may be attributed in part to lack of recognition of obstructed Mullerian anomalies in the differential diagnosis. Treatment is surgical and should be planned with future fertility in mind. A vaginal approach with resection of vaginal septum and marsupialization to maintain patency between the hemivaginas is the preferred approach. In those rare cases where the obstructed hemivagina is small or not in proximity to the contralateral patent hemivagina, a hemihysterectomy with removal of cervix, hemivagina, and ipsilateral fallopian tube may be necessary. If the diagnosis is made before there is signifcant damage to the upper reproductive tract, fertility is normal, with 36.5% of pregnancies occurring in the previously obstructed hemiuterus.

References

1.\ 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.

2.\ 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(6):1580–3.

3.\ Smith NA, Laufer MR. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertil Steril. 2007;87(4):918–22.

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Chapter 7

Dysmenorrhea

Hey-Joo Kang

Case

A 32-year-old nulligravida presents with increasing dysmenorrhea for the past 2 years. She has gone to the ER on multiple occasions for pain management especially during the rst 2 days of menstruation, oftentimes missing work. She works as a nurse, and her husband is an ER physician. They have been married for 5 years and have been trying to conceive for 1 year without success. Menarche was at age 13 and initially irregular but became monthly at age 16. Menses were not painful in the beginning but became progressively painful at age 26. Her periods last 5–7 days and are not particularly heavy in fow.

Her past medical history is signi cant for migraines with aura, limiting her hormonal management with oral contraceptive pills. She has tried progesterone-only pills with a small degree of improvement. She has a history of anxiety/depression and has been on a low-dose SSRI for the past 2 years with some improvement. She has never had surgery. Her family history is notable for her mother having had a TAH/BSO in her 40s for chronic pelvic pain and heart disease in both her grandparents. She has a sister who was diagnosed with endometriosis and broid uterus but was able to conceive naturally after 6 months of attempting pregnancy. She is a nonsmoker with minimal alcohol consumption.

Physical exam showed normal vital signs and a BMI of 23 kg/m2. Abdominal and rectal exams were normal, and stool guaiac test was negative. Pelvic exam showed normal female external genitalia and healthy-appearing vaginal mucosa and cervix with small black lesions dotted along the transformation zone. On bimanual exam, uterus was felt to be normal sized, but bilateral adnexa were slightly enlarged, although exam was limited due to patient discomfort. Transvaginal ultrasound

H.-J. Kang (*)

Reproductive Medicine and Ob/Gyn, The Ronald Perelman and Claudia Cohen Center for Reproductive Medicine, Weill Medical College of Cornell University, New York, NY, USA e-mail: hek9004@med.cornell.edu

P. H. Chung, Z. Rosenwaks (eds.), Problem-Focused Reproductive Endocrinology and Infertility, Contemporary Endocrinology, https://doi.org/10.1007/978-3-031-19443-6_7

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