The objective of this video is to illustrate our surgical technique to create a cervical and vaginal canal to relieve menstrual obstruction in an adolescent with a rare variant of Meyer-Rokotansky-Kuster-Hauser syndrome that has a functional uterus with hypoplastic distal cervical development and vaginal atresia. MRKH syndrome includes patients with congenital vaginal agenesis with varied uterine development. Most patients have a rudimentary non-functional uterus or a strip of muscle without a recognizable uterus. This patient was the unusual presentation of a functional endometrium with vaginal atresia. The patients have a normal female karyotype and the incidence is 1 in 5,000 female births. MRKH usually presents in adolescents with primary amenorrhea. The anatomic phenotype of MRKH patient varies widely. 2 to 10 percent have a functional endometrium and menstruate. 78 percent have normal ovaries bilaterally, 16 percent have ovaries in locations outside of the pelvis, and 6 percent have a hypoplastic unilateral ovary. Approximately 25 to 50 percent of these women have anomalies of the urologic tract, including unilateral renal agenesis, pelvic or horseshoe kidneys, or irregularities with the collection system. Finally, 10 to 15 percent have skeletal anomalies of the ribs, spine, or extremities. Our patient is a 14-year-old Jordanian nulligravid who presented to our department with primary amenorrhea, cyclic pelvic pain, and vaginal agenesis. She has a history of a diagnostic laparoscopy that revealed endometriosis as well as visualizing a normal appearing uterus, bilateral fallopian tubes, and ovaries. She had her appendix removed during the surgery. Of note, there are significant cultural considerations for this patient and her family involving her female reproductive tract. In her culture, marriage is arranged and the patient is to retain her virginal anatomy until the wedding. General physical examination revealed normal tanner stage 5 development and normal external genitalia. There was a congenital absence of the vagina and tissue near the clitoris that had a granulation-like appearance. Preoperative imaging confirmed a normal urologic tract and uterus with a hypoplastic cervix and vaginal atresia. Preoperatively, the patient had her menstrual cycle suppressed with non-cyclic oral contraceptives. The goals for this surgery were specific to alleviating her cyclic pelvic pain by correcting her obstruction and creating a channel for menstruation. The patient and family were offered a full vaginal reconstruction but declined, choosing to complete it closer to her future wedding. Our patient had normal external genitalia with a slight vaginal dimple. She had a fistula from the granulation-like tissue near the clitoris that connected to the distal urethra. A cystourethroscopy showed that the fistula was in the anterior distal urethra and far from the continence mechanism, suggestive of a congenital sinus tract. This was left undisturbed. A 5-centimeter mini-laparotomy was made and her entire pelvis appeared normal. Two stay sutures were placed in the uterine fundus laterally. A hystereotomy was started with electrocautery, then completed bluntly with a uterine sound. The uterine sound was utilized in combination with intermittent digital rectal examinations to confirm placement into the hypoplastic cervix to create a channel. Attention was in turned to the vaginal portion of the procedure and a Foley catheter was placed into the bladder. A 1-centimeter transverse incision was then made at the location of the vaginal dimple. The neovaginal space was bluntly dissected and should usually accommodate one to two finger breasts. However, the patient desired a virginal vagina, so the caliber was kept quite narrow. Meticulous hemostasis was obtained. When the cervix was felt, a uterine sound was advanced through the cervical canal and out the neovagina. Cystoscopy was then performed to confirm an intact bladder and bilateral ureteral efflux. Two dissolvable sutures were then placed in the paracervical-type tissue to anchor the 30 French red rubber chest tube catheter. A 12 French pediatric Foley catheter was sutured to the uterine sound and then pulled cephalad and advanced through the red rubber catheter, the endocervical canal, and into the uterine cavity. The red rubber catheter was sutured to the paracervical-type tissue and the introitus to line the neovaginal canal from the cervix to the introitus with the pediatric Foley catheter inside acting like a stent for the endocervical canal. Our thought was to leave the catheters in for six months to allow complete epithelialization around the red rubber catheter. However, the patient reported discomfort at three months, so they were removed after noting well-epithelialized vaginal tissue. A Foley catheter was placed again into the bladder to keep the vaginal area clean post-operatively. The newly created channel was tested by injecting blue-stained normal saline into the uterus from the fundal hystereotomy, confirming proper drainage. The hystereotomy was repaired and the laparotomy was closed. When the patient was one week out of surgery, she had an ultrasound which confirmed that the locations of the vaginal and intrauterine tubes were correct prior to traveling back to Jordan. Once arriving back home, she reestablished with her primary physician for her post-operative care. She saw him for a dislodgement of the pediatric Foley catheter that was in the uterine cavity. This was replaced without difficulty. She was examined every two weeks to ensure adequate vaginal healing and had intermittent hysteroscopy with the gentle dilation of the cervix. She had the red rubber catheter removed at three months and she started using a small dilator to maintain the canal. Her menses were suppressed for six months post-operatively with the gonadotropin-releasing hormone, Agonist. She is now two and a half years out of surgery and 17 years old. Her Jordanian physician reports a completely healed, well epithelialized vaginal canal. Hysteroscopy confirmed a patent endocervical canal and uterine cavity. She is having regular menses with no pain. She is not sexually active. The patient and her family feel the surgery was successful and are pleased with the results. In conclusion, Meyer-Rokitansky-Kusterhauser syndrome and its variants are rare. Two to ten percent have a functional endometrium, thus requiring intervention to address the menstrual disorders related to the vaginal obstruction. In these young women, full vaginal reconstruction may be considered, but creation of a small canal to provide menstrual relief can be a temporary solution in those who do not desire sexual function.

surgical technique

cervical and vaginal canal

menstrual obstruction

Meyer-Rokotansky-Kuster-Hauser syndrome

MRKH syndrome