Genital Tract Outflow Obstruction Treatment & Management

Once the diagnosis of a genital tract outflow obstruction has been established, consultation with a pediatric gynecologist or urologist who is familiar with such conditions is recommended. In determining the appropriate management approach, the age of the patient, as well as the psychological implications the condition poses for the patient, must be taken into account. ^[6]  The two available treatments are surgical repair and menstrual suppression.

For some younger patients who are not yet able to grasp complex medical decisions, delaying surgical treatment can be particularly beneficial. This is commonly done in cases of cervical agenesis because definitive treatment requires a hysterectomy that will render the patient infertile. Delaying surgery is also beneficial for patients who need to be compliant with vaginal dilation for a successful repair.  

Menstrual suppression can be achieved with a variety of methods, including the following ^[1] :

• Combined oral contraceptives
• Progesterone only
• Gonadotropin-releasing hormone agonists

Not all suppressive therapies result in complete amenorrhea. Consequently, some of these patients will not be successfully treated and will return with severe abdominal pain, in which case surgery will be the only remaining therapeutic option.  

Surgery is the only way of definitively treating genital outflow obstructions. Puncture and drainage of a hematocolpos without a definitive surgical procedure is not recommended, because of the high risk that an ascending infection will develop from lack of proper drainage. The type of surgical repair necessary is highly dependent on the anomaly found; consequently, accurate assessment of the structural anatomy is imperative before surgical intervention is initiated. Surgical treatments indicated for various obstructions are listed below on the basis of the level at which the obstruction occurs, proceeding from cephalad to caudad.  

Uterus 

Rudimentary uterine horn 

Rudimentary uterine horn is treated by means of surgical resection, which can be done by using minimally invasive techniques. ^[1] Resection is also recommended for a communicating nonobstructed horn to prevent pregnancy in that horn. If a pregnancy occurs in the rudimentary horn, there is a high chance of uterine rupture if it advances into the second trimester. ^[14]   

Mayer-Rokitansky-Küster-Hauser syndrome

Treatment of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome typically consists of surgical removal of the rudimentary structures to eliminate outflow obstruction. Patients who do not have an outflow obstruction, however, can usually avoid surgery. Vaginal elongation using only vaginal dilators has been successful 90-96% of the time. ^[6]  Patients in whom management with dilators fails can undergo surgical creation of a neovagina using either the McIndoe or the Vecchietti technique. ^[20]  

Cervix 

Cervical agenesis 

The recommended surgical procedure for relieving the obstruction caused by cervical agenesis is a total hysterectomy. The decision to undergo this procedure can be a difficult one, especially for young patients, because it renders them infertile. It may therefore be beneficial to delay surgery in order to give the patient more time to mature and become better informed before making her decision. To this end, medication can be given for menstrual suppression (see Medical Care) to delay a significant buildup of blood. ^[8] .

Some cases of uterovaginal anastomosis have been described, but these have been associated with significant morbidity, including pelvic inflammatory disease, endometritis, recurring obstruction and stenosis, and even death. ^[15]   

Vaginal canal 

Transverse vaginal septum

Although the physiology of a transverse vaginal septum may be similar to that of an imperforate hymen, there are a few important management differences. Surgical repair of a vaginal septum requires end-to-end anastomosis of vaginal epithelium, usually with sutures, to prevent stricture formation. ^[8]  Vaginal septa can also be found with other müllerian anomalies, most commonly a rudimentary uterine horn; accordingly, patients should be evaluated for these anomalies as well. ^[7] ​

For some of the higher and thicker septa (see Presentation, Levels of Outflow Obstruction), a combined abdominal and vaginal approach to surgical repair may be required. ^[16]  Alternatively, these septa can be managed with the help of a Foley catheter. Needle aspiration is performed to confirm the correct location of the vaginal canal. The septum is then serially dilated by advancing guide wires through the needle until there is an opening large enough to permit passage of a Foley catheter. The Foley catheter is then placed and the balloon inflated. Downward traction is applied to facilitate resection of the septum. ^[8]   

Partial vaginal agenesis

Surgical repair of partial vaginal agenesis requires not only relief of the obstruction but also elongation of the vagina. The procedure should be performed after there has been time for hematocolpos to build up. This buildup naturally distends the tissue, decreasing the need for grafts and enabling easier identification of the correct space. ^[20]  Preoperatively, the use of daily dilators can also be beneficial for decreasing the distance from the introitus to the vaginal canal. ^[8]

During the surgical procedure, the introitus between the urethra and the anus is incised and dissected caudally until the pouch or hematocolpos is met. ^[1]  This dissection can be technically difficult, being so near important structures (including the bladder and bowel); ultrasonographic (US) guidance is often helpful during this stage. ^[8]  The vagina is then grasped and elongated down to the perineum through a variety of pull-through procedures. ^[1]   

Obstructed hemivagina and ipsilateral renal agenesis syndrome

No intervention for obstructive hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome (also known as Herlyn-Werner-Wunderlich syndrome) is carried out until the patient reaches puberty, after which time vaginoplasty is performed to relieve the obstruction. In the majority of patients, this can be done via a vaginal approach. ^[11]  It may be beneficial to allow for a few menstrual cycles to distend the hemivagina and thin the wall and to facilitate determination of the incision site. Intraoperative US and needle aspiration can also help confirm that the surgeon is in the correct space before the incision is made. ^[11]   

Hymen 

Imperforate hymen

The timing of surgical treatment of imperforate hymen is controversial. Although repair is facilitated when estrogen stimulation is present, it can be performed in infancy, childhood, or adolescence. ^[3]  The majority of surgeons would probably recommend that it be done just after thelarche but before menarche, when the hymen is well estrogenized.

The surgical procedure involves making a cruciate or circular incision in the hymen to open it, thereby allowing for a normal menstrual flow and providing for comfort with future tampon use or intercourse. ^[8]  Because the cervix is often slightly dilated from the buildup of blood, irrigation of the vaginal canal is not recommended. Stenosis is rare in these cases, and therefore, postoperative dilation is not required. ^[8]  In the setting of microperforation, there have been cases where surgery was avoided and the condition was treated with serial dilation instead. ^[21]  

A follow-up examination 4-6 weeks after repair is recommended to ensure that scar tissue has not caused a recurrence of obstruction. In the majority of patients, endometriosis resolves spontaneously after the obstruction is relieved, but continued monitoring for pelvic pain is nevertheless warranted. ^[7]  If no stricture has formed and pelvic pain persists, laparoscopy for endometriosis resection could be considered.