We report a case of a large leiomyoma raised from the rudimentary uterus in a woman with MRKH syndrome. The special feature of this patient is that she had previously undergone bowel vaginoplasty, but retained the rudimentary uterus where the leiomyoma formed. The remaining rudimentary uterus led to some complications several years later and finally resulted in a second operation of this patient. We find few reports about leiomyomas in women with MRKH syndrome who had received vaginoplasty. A review of the literature shows that the incidence of leiomyomas in women with MRKH syndrome was higher than expected(7–10), while there are few reports of hysteromyoma growing so fast in such a short time after surgery. We have to suspect that we failed to detect the growing hysteromyoma in our initial examination.
Currently, the best treatment for Müllerian agenesis remains controversial, while it has been reported that non-surgical methods, mainly vaginal dilatation techniques, should be considered a first-line option before any surgical intervention(4). Once the dilation fails or is inappropriate due to previous scarring or an absent vaginal dimple, surgical vaginoplasty is required. The surgical reconstruction methods include surgical creation of a neovaginal space between the bladder and rectum, bowel vaginoplasty, vulvaginoplasty and surgical traction(11, 12). However, there are no guidelines on whether the rudimentary uteri should be saved during surgery. Table 1 highlights the reports about the course of patients with MRKH syndrome who saved rudimentary uteri when receiving vaginoplasty.
Table 1
Course of the published cases of patients with MRKH syndrome who saved rudimentary uteri when receiving vaginoplasty
Authors
|
Age
|
vaginoplasty
|
hormone
|
cyclic endometrial changes
|
Dimensions of hysteromyoma
|
Symptoms
|
urinary system
|
Surgery
|
Pascale
et al(13)
|
42
|
Abbe-McIndoe operation
|
Normal
|
+
|
9.8×7.6×8.0cm
(MR)
|
NA
|
UN
|
laparoscopy
|
Sungwook et al(14)
|
55
|
Abbe-McIndoe operation
|
Low
|
-
|
5.4×4.8×4.7cm
(MR)
|
NA
|
UN
|
laparotomy
|
|
42
|
Williams’ vaginoplasty
|
UN
|
UN
|
5.9×5.5
cm, (USG)
|
Lower abdominal pain
|
unilateral
right kidney
|
laparoscopy
|
Efthimios et al(15)
|
38
|
Williams’ vaginoplasty
|
Normal
|
UN
|
4.8×3.6 cm
(USG)
|
NA
|
Normal
|
laparoscopy
|
Nikolaos et al(16)
|
44
|
Williams’ vaginoplasty
|
Normal
|
UN
|
9.2×7.9 cm
(MR)
|
pelvic
pain
|
Normal
|
laparotomy
|
Kuhali
et al(17)
|
40
|
Vecchietti’s vaginoplasty
|
Normal
|
+
|
9.1×6.7×8.6cm
(CT)
|
Acute abdominal pain
(torsion)
|
UN
|
laparotomy
|
Varpu
et al(18)
|
47
|
Davidov’s vaginoplasty
|
UN
|
UN
|
6.0cm
(MR)
|
NA
|
Normal
|
NA
|
Present case
|
31
|
bowel vaginoplasty
|
Normal
|
+
|
10.8×9.6 cm
(USG)
|
abdominal distension
|
Normal
|
laparotomy
|
In our literature review, Abbe-McIndoe operation is the most commonly used method of vaginal reconstruction and it was performed in two cases(13, 14). Williams' vaginoplasty was performed in 3 cases (15, 16), Vecchietti’s vaginoplasty was in one case(17)and Davidov’s vaginoplasty was in the other one(18). Estrogen levels were normal in almost all cases(13, 15–17)except one peri-menopausal woman, and cyclic endometrial changes were found in some ones’ rudimentary uteri by ultrasound(13, 17). Some inconspicuous symptoms like mild abdominal pain and abdominal distension were reported in most cases, however it can also cause emergency abdominal surgery due to acute torsion of uterine remnant leiomyoma(17). Laparotomy is the most commonly used in our literature review, especially in the cases of Giant uterine leiomyoma and complicated nature of its feeding arteries(14, 16), as well as emergency abdominal surgery(17). All patients underwent hysterectomies.
Some patients with MRKH syndrome have small rudimentary Müllerian bulbs that result from abnormal development of the Müllerian duct during embryogenesis. Oppelt has reported that 84% (239/290) of patients have uterine remnants, including bilateral rudimentary remnants and a plastic uterine horn(19). Most of the uterine remnants or small rudimentary Müllerian bulbs lack endometrial activity, and the rudimentary uteri are usually composed of smooth muscle cells, which may lead to leiomyomas. 2%-7% of patients with MRKH syndrome have a functional endometrium in the rudimentary uteri(5). The presence of endometrium in rudimentary uteri may lead to pelvic pain. Marsh reported that 48% (23/48) of females with MRKH syndrome had uterine remnants and that 46% (22/48) had pelvic pain. He also found that the presence of endometrium was associated with pelvic pain (RR = 2.3; 95% CI = 1.2–4.7) in females with MRKH syndrome (20). Moreover, in some patients with a functional rudimentary uterus, as has been reported in some cases, pregnancy can be achieved by uterine and cervical reconstruction, creation of a neovagina and placement of a uterovaginal conduit or zygote intrafallopian transfer(21, 22). However, our review of the literature failed to find a case report of successful delivery from women with rudimentary uteri and there are certain risks of uterine rupture during pregnancy among these women that can lead to catastrophic results, with a death rate of 47.6% reported at the beginning of the 20th century(23). Considering the risk of pregnancy and complications of surgical therapy, preserving the rudimentary uteri in patients with MRKH syndrome during vaginoplasty makes little sense.