Volume 154, Issue 1 , Pages 115-116, January 2011
Laparoscopic evaluation of pelvic pain with Surgicel vaginoplasty in a woman with Mayer Rokitansky Kuster Hauser syndrome
Article Outline
Dear Editor,
Mullerian agenesis, or Mayer Rokitansky Kuster Hauser syndrome (MRKH), affects 1 in 4000–10,000 women and is the second most common cause of primary amenorrhoea [1]. We report a case of abdominopelvic pain and its evaluation by laparoscopy in a young woman with MRKH.
A 16-year-old virgin female presented to our gynaecological out-patient department with primary amenorrhoea and colicky non-cyclical abdominopelvic pain for the past 3 years. The pain was not associated with urinary or bowel symptoms and was partially relieved by analgesics. Secondary sexual characteristics were well developed. On local examination, the external genitalia appeared normal with a blind vagina. The uterus was not felt on rectal examination. Transabdominal ultrasound revealed absence of the cervix and uterus with a hypoechoic solid area on the right side of pelvis measuring 3
cm×2.6cm, probably of ovarian origin; the left adnexa were normal. Intravenous pyelography and X-ray of lumbosacral spine were normal. Psychological counselling of the patient and her family was done. On diagnostic laparoscopy, there were completely separated bilateral rudimentary uterine horns connected by a thick fibrous band approximately 8cm in length, and a myoma of 3cm×2.5cm was seen arising from the right uterine horn (Fig. 1), with its surface obscured by omental adhesions. Both tubes and ovaries were normal. Laparotomic excision of the right rudimentary horn along with the myoma was performed. Cut section of the uterine horn revealed no cavity and that of the myoma showed a whorled appearance. Neovaginoplasty was done by creating a space between the bladder and rectum using a foam mould covered with Surgicel. Labial stitches were applied for approximation with nylon 1-0. The procedure was uneventful. The Foley's catheter was removed along with the foam mould under light sedation after 5 days. Perineal hygiene was taken care of with betadine every time the patient passed urine or stools. A glass mould was designed for the patient and she was advised to use it regularly daily, remove it at night and only stop using it once she became sexually active.
Fig. 1.
Right rudimentary uterine horn with leiomyoma and omental adhesions.
Histopathological examination of the rudimentary horn showed myometrium only, and the solid mass was confirmed to be a leiomyoma. The patient had a satisfactory clinical outcome and was discharged on day 5 in a stable condition. She was asymptomatic on follow up at 1 and 3 months, with a well formed vagina.
Vaginal agenesis is a congenital mullerian abnormality with a normal female karyotype (46 XX), functioning ovaries, external genitalia and secondary sexual characters. Its etiology is unclear; a multifactorial mode of inheritance has been proposed. Usually these women have absent uteri or occasionally rudimentary uteri are present connected by fibromuscular remnants as seen in our patient. They present in adolescence with primary amenorrhoea. Leiomyomas and adenomyosis have been reported in women with mullerian agenesis [1], [2] and uterus didelphys [3], but mullerian duct remnant leiomyomas are very uncommon as compared to in the normal uterus. They can be asymptomatic or present with acute or chronic pelvic pain as in our patient. The cause of pain in our patient was probably the adhesion to the rudimentary horn. Ultrasound diagnosis is not very sensitive and needs to be confirmed on MRI or laparoscopy. The use of MRI in the assessment of the syndrome has been described by Pompili et al. [4] but was not possible in our patient due to the cost factor and a long period of waiting. Laparoscopic evaluation not only confirms the diagnosis but also allows the appropriate management depending on the underlying pathology. Treatment is surgical removal of the leiomyoma along with the adjacent uterine remnant by either laparoscopy or laparotomy [5]. Removal of the opposite uterine remnant can also be undertaken during the same procedure with prior informed consent. In our patient, the right horn along with the myoma was removed. Laparoscopic excision was described as early as 1998 by Giatras et al. [6]. Various techniques of vaginoplasty have been described with good results. We used Surgicel (oxidized regenerated cellulose) as has been previously reported by us [7] in our series of MRKH patients with good success.
Women with mullerian agenesis who have normal fallopian tubes and ovaries with or without uterine remnants of varying sizes can develop gynaecological problems like a fibroid from the fibromuscular tissue, or an ovarian cyst. This should be considered in the differential diagnosis of abdominopelvic pain in such patients.
References
- . A menopausal woman with mullerian agenesis, a leiomyoma, an inguinal hernia, and cystadenofibromas. Obstet Gynecol. 2004;103(5 Pt 2):1123–1125
- Development of leiomyomas on the uterine remnants of two women with Mayer-Rokitansky-Kuster-Hauser syndrome. Fertil Steril. 2004;81(5):1385–1387
- . Uterus didelphys with fibroid uterus and ovarian cyst—rare mullerian malformation. J Indian Med Assoc. 2006;104(6):336–337
- Magnetic resonance imaging in the preoperative assessment of Mayer-Rokitansky-Kuster-Hauser syndrome. Radiol Med. 2009;114(5):811–826
- . Leiomyoma in a mullerian remnant. A case report. J Reprod Med. 1988;33:246–248
- . Laparoscopy for pelvic pain in the Mayer-Rokitansky-Kuster-Hauser syndrome. A case report. J Reprod Med. 1998;43(3):203–205
- . Creation of neovagina using oxidized cellulose (surgicel) as a surgical treatment of vaginal agenesis. Arch Gynecol Obstet. 2007;275(April (4)):231–235
PII: S0301-2115(10)00376-3
doi:10.1016/j.ejogrb.2010.07.042
© 2010 Elsevier Ireland Ltd. All rights reserved.
Volume 154, Issue 1 , Pages 115-116, January 2011
