Nicole W. Karjane
History of present illness
A 14-year-old girl presents to the emergency department with a 5-day history of severe pelvic pain. This episode began on the first day of her menstrual period and has been progressively worsening. She reports crampy pain with menses since menar- che approximately six months ago. The pain is located slightly to the right of midline and occasionally radiates to her back.
Initially, her cramping was mild and improved with ibuprofen;
however, with each menstrual period, her pain has gotten worse and more resistant to treatment with nonsteroidal anti- inflammatory drugs (NSAIDs). Her pediatrician started her on oral contraceptive pills (OCPs) after her third menstrual period, but her pain has continued to intensify with each cycle, such that she has missed multiple days of school. Her menses have been regular, lasting about 4–5 days, with an interval of 28–29 days. Although her pain was originally just with the duration of her menses, for the past three months it has been lingering for several days after her bleeding stops. She reports that she has no difficulty using tampons, but she must always use a pad in addition to the tampon to avoid soiling her underwear. She denies fevers, chills, nausea, vomiting, diarrhea, or constipation. She has never been sexually active. Her medical history is significant for right renal agenesis that was inciden- tally diagnosed at age five during the workup of abdominal pain, and she has had no prior medical illnesses or surgeries.
Physical examination
General appearance:Nontoxic appearing girl who is apprehensive and in moderate distress
Vital signs:
Temperature: 37.0°C Pulse: 92 beats/min Blood pressure: 115/62
Respiratory rate: 18 breaths/min Oxygen saturation: 100% on room air HEENT:Normal
Cardiovascular:Regular rate and rhythm without murmurs, rubs, or gallops
Lungs:Clear to auscultation bilaterally Breasts:Tanner stage IV breast development
Abdomen:Soft, tender throughout lower abdomen, right greater than left, nondistended, no guarding or rebound tenderness, palpable mass to right of midline just above pubic symphysis at area of maximal tenderness
Genitalia:Normal external genitalia, normal, well- estrogenized hymen. Bimanual examination reveals a tense bulge in the right vagina that begins 2 cm cephalad to the introitus and extends to the cervix. No cervical motion tenderness, but unable to delineate uterine size due to patient discomfort
Laboratory studies:Urine pregnancy test: Negative Imaging:A transabdominal pelvic ultrasound showed a large cystic massfilling the pelvis, normal appearing uterus above the mass, unable to identify ovaries. The patient was admitted, given intravenous narcotics, and MRI of the pelvis was ordered (Figs 71.1,71.2, and71.3)
How would you manage this patient?
The patient’s diagnosis is dysmenorrhea due to menstrual outflow obstruction in a patient with uterine didelphys, obstructed hemivagina and ipsilateral renal agenesis. This diagnosis was made based on history and examination and confirmed on MRI. The patient was taken to the operating room where her physicians performed an examination under anesthesia and excision of the obstructive vaginal septum. The
Fig. 71.1 Right-sided renal agenesis (A) as well as the obstructed hemivagina (B).
Acute Care and Emergency Gynecology, ed. David Chelmow, Christine R. Isaacs and Ashley Carroll. Published by Cambridge University Press.
© Cambridge University Press 2015.
patient’s pain resolved following surgery, and her dysmenor- rhea gradually improved over the next several months.
Obstructive hemivagina
Dysmenorrhea, or painful menses, is the most common pre- senting menstrual disorder in adolescent females. Most of these young patients are ultimately diagnosed with primary dysmenorrhea, or cyclic menstrual pain without an identifiable cause; however, there are many potential causes of dysmenor- rhea that must be considered. These include endometriosis, pelvic inflammatory disease, leiomyomas, and menstrual obstruction.
The initial assessment of an adolescent with dysmenorrhea includes a thorough history, with particular attention to men- strual history and sexual history. Physical examination should include an adequate abdominal examination and visualization of the external genitalia. In virginal patients with regular menses and normal external genitalia, pelvic examination can generally be avoided during the preliminary evaluation, par- ticularly if they are able to use tampons. Patients with a history of sexual activity should undergo speculum examination and bimanual pelvic examination to evaluate for sexually transmit- ted infections, and those with infections should be treated appropriately. In addition, a urine pregnancy test should be obtained to rule out pregnancy associated bleeding and pain.
The release of prostaglandins that occurs during sloughing of the endometrium is thought to be the cause of primary dysmenorrhea; therefore, NSAIDs are a reasonable first-line treatment in young women with dysmenorrhea once the initial evaluation is complete. OCPs are also effective in the treatment of dysmenorrhea as they decrease the production of uterine prostaglandins. Oral contraceptive use has been shown to improve dysmenorrhea in over 70% of women [1] and may
be used asfirst- or second-line treatment depending on patient preference and the need for contraception. Patients who fail to respond to combination therapy with both NSAIDS and OCPs require further workup to determine the etiology of their menstrual pain. This workup should be guided by the history and examination. In patients with a normal, adequate pelvic examination and no evidence of infection, endometriosis must be considered as an etiology. If the initial history and examination are concerning for an obstructive anomaly, as in the patient described in this case, imaging is indicated to confirm the diagnosis and clarify the anatomy.
It is well-established that women with Mullerian anomalies are at higher risk for concomitant renal anomalies, and vice versa. Therefore, when patients with known renal anomalies present with dysmenorrhea, as did this patient, the practitioner should have a high index of suspicion for a Mullerian anomaly.
Initial imaging of the pelvis with ultrasound is a reasonable first step, however, MRI is often required to make a definitive diagnosis [2,3]. Once an anomaly is identified, prompt surgical intervention should be undertaken to relieve any obstruction and to prevent the possible long-term consequences of men- strual obstructions such as endometriosis and adhesive disease.
Uterine didelphys with obstructed hemivagina and ipsilat- eral renal agenesis, also referred to as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome or Herlyn–
Werner–Wunderlich syndrome, has been well-described in the medical literature dating back to as early as 1922. Embryo- logically, it appears to be caused by an abnormal development of both the Wolffian duct and the ipsilateral Mullerian duct, resulting in renal agenesis (or other anomaly) as well as incomplete fusion of the Mullerian duct with its contralateral side and with the more distal urogenital sinus [4,5]. As with many unilateral birth defects, this anomaly is significantly more likely to occur on the right side of the body than the left
Fig. 71.2 Coronal view of the obstructed hemivagina (B), the normal left uterus in the midline (C), and the obstructed right uterus that is deviated to the right (D).
Fig. 71.3 Midline, sagittal view showing the nonobstructed uterus (C) overlying the obstructed hemivagina (B).
side, for reasons that remain unclear [4]. Although the most common findings in this syndrome are uterine dildelphys, obstructed hemivagina, and ipsilateral renal agenesis, numer- ous anatomic variants have been reported. These variants may occur in up to 27% of cases and include a septate uterus with an obstructed hemivagina, a bicornuate bicollis uterus with an obstructed hemivagina, a bicornuate uterus with a septate cervix and obstructed hemivagina, and a uterine didelphys with unilateral cervical atresia [5]. The possibility of alternate anatomic variants makes MRI in these patients essential to define the anomaly and treat the circumstances appropriately.
Definitive treatment of uterine didelphys with obstructed hemivagina involves excision of the obstructive vaginal septum. This is generally performed in the operating room by incising the bulging longitudinal vaginal septum, excising it up to the level of the cervix, and marsupializing the vaginal epithelium with absorbable suture. Attempts to perform needle drainage of the hematocolpos without excising the septum should be avoided, as this may seed the obstructed hematocolpos with bacteria and lead to an ascending pelvic infection with subsequent abscess formation, adhesive disease, and/or sepsis.
Concomitant laparoscopy was previously advocated in patients with OHVIRA to aide in the diagnosis. More recently, however, it appears that the correct diagnosis can generally be made on the basis of history, examination and imaging, and therefore laparoscopy may be avoided in most cases [6].
Because menstrual obstruction and the resultant retrograde menstruation may cause endometriosis, considering concomi- tant laparoscopy to evaluate and treat endometriosis implants
may be reasonable. The natural history of endometriosis in patients with obstructive anomalies, however, is unclear, and spontaneous resolution often occurs following correction of the causative obstruction. It seems prudent, therefore, to reserve laparoscopy to patients with persistent symptoms following correction of their menstrual obstruction, or to patients in whom the diagnosis remains unclear after the initial workup.
Key teaching points
Dysmenorrhea that is refractory to conservative
management with nonsteroidal anti-inflammatory drugs and oral contraceptive pills should be further evaluated for potential anatomic causes.
Although most patients with menstrual obstructions present with cyclic pain without menses, patients with a partial obstruction will present with worsening
dysmenorrhea refractory to medical management.
Young women with known renal anomalies are at higher risk for concomitant Mullerian anomalies. Therefore, practitioners should have a high index of suspicion when these patients have persistent menstrual complaints in order to avoid delays in diagnosis.
Patient with suspected menstrual obstructions should undergo MRI to accurately define the anomaly and aid in surgical planning.
Uterine didelphys with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is an uncommon yet well-defined entity that is treated with excision of the obstructive vaginal septum.
References
1. American College of Obstetricians and Gynecologists. Noncontraceptive uses of hormonal contraceptives. Practice Bulletin No. 110.Obstet Gynecol 2010;115:206–18.
2. Church DG, Vancil JM, Vasanawala SS.
Magnetic resonance imaging for uterine and vaginal anomalies.Curr Opin Obstet Gynecol2009;21(5):379–89.
3. Behr SC, Courtier JL, Qayyum A.
Imaging of Müllerian duct anomalies.
Radiographics2012;32(6):E233–50.
DOI: 10.1148/rg.326125515.
4. Vercellini P, Daguati R, Somigliana E, et al. Asymmetric lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys: Institutional case series and a systematic literature review.Fertil Steril 2007;87(4):719–24.
5. Fedele L, Motta F, Frontino G, Restelli E, Bianchi S. Double uterus with obstruced hemivagina and ipsilateral renal agenesis: pelvic anatomic variants in 87 cases.Hum Reprod2013;28(6):1580–3.
6. Smith NA, Laufer MR. Obstructed hemivagina and inpsilateral renal anomaly (OHVIRA) syndrome:
Management and follow-up.Fertil Steril2007;87(4):918–22.
Case 71: Worsening dysmenorrhea in a 14-year-old girl