Laparoscopic findings and pelvic anatomy in Mayer-Rokitansky-Küster-Hauser syndrome

OBJECTIVE: To describe analytically the anatomic variety and laparoscopic findings observed in patients with Rokitansky syndrome throughout an 11-year span. METHODS: We analyzed the laparoscopic and chart records of 106 consecutive patients who underwent surgery for the creation of a neovagina, according to the modified laparoscopic Vecchietti procedure. RESULTS: A hypoplastic vagina was observed in 61 women. Müllerian remnants, laterally displaced in the pelvis, were identified in 92 cases; 42 (25.9%, 95% confidence interval [CI] 19.2-33.3) of 162 müllerian remnants were cavitated and contained endometrial mucosa. Ovaries were extrapelvic in 17 (16.0%, 95% CI 9.6-24.4) cases. Anomalies of the urinary tract were identified in 32 (30.2%, 95% CI 21.7-39.9) patients; unilateral renal agenesis was the most frequent finding (18 cases; 18%, 95%CI 10.4-25.5). CONCLUSION: Rokitansky syndrome has a wide variability of anatomic presentations, and, as the do other congenital anomalies of the female genital tract, it represents a continuum of embryonic malformations, which occur at different stages of development.     

Voluminous ectopic polycystic ovaries in Mayer-Rokitansky-Küster-Hauser syndrome

We report the case of a 20-year-old woman with primary amenorrhea, normally developed sexual characteristics, infantile external genitals and absence of vaginal orifice. Blood chemistry studies showed elevated serum levels of gonadotropin, estrogens, testosterone, 17OH-progesterone, prolactin and TSH and low concentrations of FT(3) and FT(4). Cytogenetic karyotyping and in situ fluorescence hybridization revealed a 46,XX karyotype and the presence of Barr chromatin body. Laparoscopy disclosed absence of a vagina, apparently normal, enlarged tubes, multifolicular ectopic gonads and symmetric bilateral uterine buds. Diagnostic work-up included skeletal radiography, renal ultrasonography, intravenous pyelography, pelvic echography, pelvic phlebography and magnetic resonance imaging studies to demonstrate possible associations between the genitourinary and skeletal anomalies. Based on the clinical and laboratory findings, a diagnosis of Mayer-Rokitansky-Küster-Hauser syndrome was established, associated with the presence of enlarged ectopic polycystic ovaries in the abdominal cavity.     

The effect of a group programme on women with the Mayer-Rokitansky-Küster-Hauser syndrome

Objective To evaluate the effect of a group programme on psychological distress in women with the Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome.
Setting Gynaecologic outpatient clinic.
Sample Seventeen women with MRKH syndrome participated in this study.
Method A semi-structured programme of seven sessions was offered dealing with themes of the MRKH syndrome. Psychological distress was measured at the first visit 3–6 month before the group programme was started (pre-test-0), at the first (pre-test) and then at the last group session (post-test).
Main outcome measure The Symptom Check List-90 was used to assess feelings of psychological distress.
Results The post-test subscale scores for anxiety, depression, interpersonal sensitivity and the total score for psychological distress were significantly lower than the pre-test-0 and pre-test scores (   P < 0.05  ). No significant differences were found between the pre-test-0 and pre-test subscale scores and total score.
Conclusion A semi-structured group programme seems valuable in helping women with the MRKH syndrome to deal with their psychological stress.     

Large endometrioma in an adolescent girl with Mayer-Rokitansky-Küster-Hauser syndrome

A 14-year-old 46 XX female presented with primary amenorrhea. A normal vagina ending in a blind pouch was found at gynecological examination. Diagnostic laparoscopy revealed the absence of a uterus with rudimentary fallopian tubes, round ligaments and uterosacral ligaments. The left ovary contained a 7-cm chocolate cyst, which was shown to be an endometrioma by pathological examination. This rare occurrence of ovarian endometrioma coexisting with Mayer-Rokitansky-Küster-Hauser syndrome in an adolescent patient might be secondary to Müllerian-directed metaplasia in the ovaries.     

Typical form of Mayer-Rokitansky-Küster-Hauser syndrome and ectopic kidney. A rare association

The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a spectrum of Müllerian anomalies characterized by congenital vaginal aplasia and absent uterus or a rudimentary one in female subjects with normal endocrine status. The ovaries and fallopian tubes are present. The prime feature is a primary amenorrhea in women presenting normally developed secondary sexual characteristics and normal external genitalia. The etiology remains unknown. This syndrome is subdivided in two types. It may be isolated (type I) or associated with other malformations (type II). Type I is less frequent than type II. We report a case of MRKH syndrome in a 21-year-old woman who presented a primary amenorrhea studied with transabdominal and pelvic ultrasonography and pelvic magnetic resonance, which demonstrated a complete agenesis of uterus, a functional cyst in the right ovary, and a left pelvic ectopic kidney. We studied the MRKH syndrome with review of the literature.     

Mayer-Rokitansky-Küster-Hauser syndrome associated with unilateral gonadal agenesis. A case report

BACKGROUND: Mayer-Rokitansky-Küster-Hauser syndrome is the second most frequent cause of primary amenorrhea, with a reported incidence of 0.002%. Patients have a normal karyo-type and usually normal ovaries. Associated ovarian abnormalities are rarely reported. CASE: A 17-year-old woman with primary amenorrhea was evaluated by diagnostic laparoscopy, which showed complete müllerian agenesis. On the left side, the uterine tube and round ligament were hypoplastic, and the ovary was absent. The karyotype was 46, XX. Intravenous urography revealed a right kidney below the normal site with malrotation abnormality. CONCLUSION: Müllerian duct agenesis coexisting with unilateral ovarian agenesis and a contralateral renal abnormality has not been widely described before. Unilaterality might play a role in the etiologic factor of Mayer-Rokitansky-Küster-Hauser syndrome.     

Use of acellular human dermal allograft for vaginoplasty in Mayer-Rokitansky-Küster-Hauser syndrome: a case report

BACKGROUND: Both autologous and synthetic tissue have been used to create a neovagina in women with Mayer-Rokitansky-Küster-Hauser syndrome. Despite reports on many different techniques, the ideal method of vaginoplasty has not been firmly established. CASE: A 33-year-old woman with Mayer-Rokitansky-Küster-Hauser syndrome had been unsuccessful in using vaginal dilators for the creation of a functional vagina with the Frank technique due to pain and vulvar lichen sclerosis. She thus elected to undergo vaginoplasty by a modified McIndoe procedure using acellular human dermal allograft material. Both the surgery and immediate postoperative course were uncomplicated. Vaginal apex stenosis resulted from inadequate dilatation in the weeks following surgery since the patient experienced pain with dilator use. CONCLUSION: The use of dermal allograft material instead of a split-thickness autologous skin graft is a simple and less invasive method of constructing a neovagina. Due to the many potential advantages of this technique, further investigation is warranted to optimize long-term outcomes of vaginoplasty using this method.     

Creation of a neovagina according to Wharton-Sheares-George in patients with Mayer-Rokitansky-Küster-Hauser syndrome

OBJECTIVE: To introduce a simple and quick surgical alternative for creating a neovagina in patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome that offers good anatomic and functional results. DESIGN: Historical report. SETTING: Tertiary center for gynecologic endocrinology. PATIENT(S): Three patients with MRKH syndrome. INTERVENTION(S): The creation of a neovagina according to Wharton-Sheares-George in patients with MRKH syndrome. MAIN OUTCOME MEASURE(S): Axis, length, and width of neovagina. RESULT(S): The George modification of the Wharton-Sheares neovaginoplasty was successfully performed in three patients. The results were excellent (normal axis and adequate length and width of neovagina), and there were no major complications. CONCLUSION(S): The George modification of the Sheares technique represents a simple, safe, and effective surgical option for creating a neovagina. The procedure is not highly complex and is therefore easy to learn and perform; no special surgical equipment is needed. Anatomic and functional results are very satisfying. Short-term hospitalization, accelerated recovery, and a rapid return to everyday life are important benefits for these young patients. These benefits also result in lower surgery-related expenses and therefore reduce the strain on the hospital's budget compared with other therapeutic options. The creation of a neovagina according to Wharton-Sheares-George might provide a satisfactory alternative for the surgical management of vaginal aplasia in patients with MRKH syndrome.