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1.
Niger J Clin Pract ; 27(5): 643-646, 2024 May 01.
Article in English | MEDLINE | ID: mdl-38842714

ABSTRACT

BACKGROUND: Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome, the second important cause of primary amenorrhea, is characterized by complete mullerian agenesis in the presence of normal karyotype and normal functioning ovaries. Incidence is one in 4500 females. Treatment options include surgical and non-surgical methods. Surgical treatment by creating a neovagina between bladder and rectum is preferred as it gives immediate results. AIM: To evaluate the anatomical and functional outcomes of modified vaginoplasty procedures conducted in our institution. METHODS: An ambispective cohort study was conducted in the Department of Obstetrics and Gynaecology, at a tertiary care hospital and included 10 cases of MRKH syndrome, who underwent surgical treatment in our department. Postoperative outcome was noted. Sexual function was evaluated using the Female Sexual Function Index (FSFI) score. RESULTS: The mean duration of surgery was 40 minutes. The average blood loss during surgery in patients undergoing vaginoplasty was 60 ml. The mean length of the neovagina as measured 1 month after surgery was 7.9 cms. FSFI score was >30 in eight patients. Two patients were lost to follow-up. CONCLUSION: Modified McIndoe vaginoplasty is a simple, safe, and cost-effective procedure in the hands of experts. Only mature patients willing to follow the instructions and planning to get married soon should undergo this surgery.


Subject(s)
46, XX Disorders of Sex Development , Congenital Abnormalities , Mullerian Ducts , Tertiary Care Centers , Vagina , Humans , Female , Vagina/surgery , Vagina/abnormalities , Mullerian Ducts/abnormalities , Mullerian Ducts/surgery , 46, XX Disorders of Sex Development/surgery , Adult , Adolescent , Congenital Abnormalities/surgery , Young Adult , Plastic Surgery Procedures/methods , Treatment Outcome , Gynecologic Surgical Procedures/methods , Cohort Studies , Prospective Studies
2.
Am J Case Rep ; 25: e943305, 2024 Jun 13.
Article in English | MEDLINE | ID: mdl-38867420

ABSTRACT

BACKGROUND Laparoscopic-perineal neovagina construction by sigmoid colpoplasty is a popular therapeutic approach for patients with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. The conventional approach requires an auxiliary abdominal incision to exteriorize the descending colon to fix the anvil for end-to-end colorectal anastomosis. We modified the natural orifice specimen extraction surgery (NOSES) approach by exteriorizing the descending colon through the artificial neovaginal tunnel to replace the anvil extracorporeally, without requiring an auxiliary abdominal incision. It was a more minimally invasive technique. CASE REPORT We performed this modified laparoscopic-perineal sigmoid colpoplasty in a 26-year-old woman with MRKH syndrome. We cut off a segment of the sigmoid colon with a vascular pedicle to make a new vagina out of it, the same as in the traditional laparoscopic-perineal sigmoid colpoplasty. What is new about this technique is that it has no need for abdominal incision and is more minimally invasive. The operating time was 315 min. No postoperative complications occurred. The postoperative hospital stay was 4 days. The modified laparoscopic-perineal approach, free from an auxiliary abdominal incision, demonstrated advantages, including a shorter hospital stay, expedited recovery, and comparable anatomical outcomes, when compared with the traditional approach. This innovation improves the surgical experience for patients with MRKH syndrome, addressing the physical and psychological aspects of their condition. CONCLUSIONS This refined laparoscopic-perineal neovagina construction by sigmoid colpoplasty represents a feasible and minimally invasive technique. It is an attractive option for MRKH syndrome patients in need of vaginal reconstruction, offering a streamlined procedure with reduced postoperative recovery time and enhanced patient outcomes.


Subject(s)
46, XX Disorders of Sex Development , Colon, Sigmoid , Laparoscopy , Mullerian Ducts , Perineum , Vagina , Humans , Female , Adult , Laparoscopy/methods , Colon, Sigmoid/surgery , Vagina/surgery , Vagina/abnormalities , 46, XX Disorders of Sex Development/surgery , Mullerian Ducts/abnormalities , Mullerian Ducts/surgery , Perineum/surgery , Congenital Abnormalities/surgery , Plastic Surgery Procedures/methods
4.
Zhonghua Fu Chan Ke Za Zhi ; 59(5): 360-367, 2024 May 25.
Article in Chinese | MEDLINE | ID: mdl-38797565

ABSTRACT

Objective: To explore the age of onset and consultation, the main clinical manifestations, common types of combined malformations, the relationship of endometriosis, surgical prognosis and different types of proportion of adolescent female reproductive system dysplasia. Methods: The medical records of 356 patients (aged 10-19) with female reproductive system dysplasia in Women's Hospital, School of Medicine, Zhejiang University from January 2003 to August 2018 were collected and retrospectively analyzed. Results: (1) Among the 356 adolescent dysplasia patients, uterine dysplasia (23.6%, 84/356), oblique vaginal septum syndrome (OVSS; 22.5%, 80/356) and vaginal dysplasia (21.6%, 77/356) were the most frequent ones, followed by multi-sectional dysplasia (16.0%, 57/356), other types of developmental abnormalities like external genitaliaand urogenital fistula (13.5%, 48/356) and Mayer-Rokitansky-Küster-Hauser syndrome (MRKH syndrome; 2.8%, 10/356). (2) There were significant differences between the median age of onset and the age of consultation of patients with OVSS and other types of abnormalities except hymen atresia (both P<0.05). In contrast, there were no significant differences between the age of onset and the age of consultation of the patients of uterine dysplasia, vaginal dysplasia, hymen atresia, MRKH syndrome and multi-sectional dysplasia (all P>0.05). (3) The clinical manifestations were lack of specificity, and mainly abnormal finding was lower abdominal pain. (4) After admission, the majority of patients underwent comprehensive cardiopulmonary examination (71.3%, 254/356) and urinary system examination (63.5%, 226/356). Only 18.3% (65/356) of patients had completed abdominal organ examination, and 5.9% (21/356) skeletal system examination. About other systemic malformations, urological malformations were the most common (27.5%, 98/356), followed by anorectal malformation (0.6%, 2/356), heart malformations (0.3%, 1/356), and spinal malformations (0.3%, 1/356). 46.4% (84/181) of the surgical patients were diagnosed with combined endometriosis. Patients with obstructive genital tract malformations were more likely to combine with endometriosis than non-obstructive ones [50.3% (74/147) vs 29.4% (10/34); P<0.05]. However, there was no significant difference between the severity of endometriosis of those two kinds (P>0.05). (5) Totally 308 patients were followed up successfully with a median of 25.0 years old, and 20 cases were treated again; 12.0% (37/308) of them were suffering from menstrual disorder and 33.1% (102/308) of them with dysmenorrhea. Totally 130 patients had sexually active reported no sexual problems. Conclusions: Uterine dysplasia, OVSS and vaginal dysplasia are the most common syndromes in adolescent female reproductive system dysplasia along with frequent cases of coexisting urinary malformations and increasing risks of endometriosis. Meanwhile, the lack of specificity of clinical manifestations might delay the timely diagnosis and treatment after the onset of symptoms. Nonetheless, most patients could achieve good surgical outcomes.


Subject(s)
46, XX Disorders of Sex Development , Congenital Abnormalities , Endometriosis , Mullerian Ducts , Uterus , Vagina , Humans , Female , Adolescent , Retrospective Studies , Vagina/abnormalities , Vagina/surgery , Mullerian Ducts/abnormalities , Endometriosis/surgery , Endometriosis/diagnosis , Endometriosis/pathology , 46, XX Disorders of Sex Development/surgery , Congenital Abnormalities/epidemiology , Uterus/abnormalities , Uterus/surgery , Uterus/pathology , Young Adult , Urogenital Abnormalities/surgery , Abnormalities, Multiple/epidemiology , Child , Prognosis , Genitalia, Female/abnormalities , Genitalia, Female/surgery , Genitalia, Female/pathology
5.
Tunis Med ; 102(5): 310-314, 2024 May 05.
Article in French | MEDLINE | ID: mdl-38801290

ABSTRACT

INTRODUCTION: Rokitansky syndrome or Mayer Rokitansky Kuster Hauser (SRKMH) is a rare congenital malformation defined by uterovaginal aplasia. The aim of the treatment is to create a neovagina and restore sexual life. However, postoperative results in terms of sexual and overall quality of life of patients remain controversial. AIM: To evaluate the quality of life and sexuality of patients operated on for Rokitansky syndrome. METHODS: This was a retrospective and comparative study between two groups of patients. The first group consisted of patients who had undergone vaginoplasty as part of SRKMH. The second group was that of the controls. Control in terms of sexual function by the Ar FSFI (Arab FemaleSexualFunction Index) and quality of life by the SF36 (Short Forms Health Survey) were used in both groups. RESULTS: The average age of patients operated on for SRKMH was 22.53 years. Eighteen of these patients (60%) were sexually active at the time of the study. Patients operated on for SRKMH had a significantly lower Ar FSFI score compared to the control group. The two areas most affected were lubrication and pain. Likewise, patients who underwent vaginoplasty had a significantly lower SF 36 score compared to the control group. The psychological component was the most affected of the different components of quality of life. CONCLUSION: Sexual function and quality of life after vaginoplasty in the context of Rokitansky syndrome remains unsatisfactory despite the anatomical result.


Subject(s)
46, XX Disorders of Sex Development , Congenital Abnormalities , Mullerian Ducts , Quality of Life , Sexuality , Uterus , Vagina , Humans , Female , Retrospective Studies , Congenital Abnormalities/surgery , Congenital Abnormalities/psychology , 46, XX Disorders of Sex Development/surgery , 46, XX Disorders of Sex Development/psychology , 46, XX Disorders of Sex Development/complications , Mullerian Ducts/abnormalities , Mullerian Ducts/surgery , Young Adult , Adult , Vagina/abnormalities , Vagina/surgery , Sexuality/psychology , Adolescent , Uterus/abnormalities , Uterus/surgery , Sexual Behavior/psychology
6.
J Pediatr Adolesc Gynecol ; 37(3): 353-359, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38246338

ABSTRACT

OBJECTIVE: To analyze the features of the epithelia coating neovaginas after vaginoplasty in women affected by Mayer-Rokitansky-Küster-Hauser syndrome STUDY DESIGN: We conducted a retrospective analysis of prospectively collected data. Women affected by Rokitansky syndrome who underwent neovaginal biopsy after vaginoplasty (McIndoe surgery, intestinal vaginoplasty, Vecchietti surgery, and Davydov surgery) were included. Macroscopic mucosal features were assessed through clinical examination and the Schilling test. Each biopsy specimen was prepared for examination by light microscopy and in some cases by scanning electron microscopy (SEM). RESULTS: Thirty-six patients (4 McIndoe, 2 intestinal vaginoplasty, 14 Vecchietti, and 16 Davydov) were included. All biopsies were performed without complications. In McIndoe's neovaginas, the mucosal microscopic features were similar to normal skin, with large areas of preserved epithelium, heterogeneous presence of dermal papillae, and superficial keratinization. The characteristics of the intestinal neovagina's surface were similar to those of a sigmoid colon, with well-shaped glands, cylindrical cells, and a secreting mucosa. In Vecchietti neovaginas, the surface the epithelium was flat and multilayered, highly similar to that of a normal vagina, with the presence of glycogen and superficial desquamation. On medium SEM magnification evaluation, the epithelium presented flattened polygonal cells. Finally, in Davydov neovaginas, none of the specimens had persistent mesothelial elements. The squamous neo-epithelium had regular aspects of differentiation with the presence of glycogen. At greater SEM magnification, microridges were evident, with a regular distribution. CONCLUSION: Each different technique of vaginoplasty leads to unique histological and structural features of the neovagina's mucosa. Knowledge of these elements must be the basis for the choice of the most appropriate intervention.


Subject(s)
46, XX Disorders of Sex Development , Congenital Abnormalities , Mullerian Ducts , Vagina , Humans , Female , Vagina/surgery , Vagina/abnormalities , 46, XX Disorders of Sex Development/surgery , 46, XX Disorders of Sex Development/pathology , Mullerian Ducts/abnormalities , Mullerian Ducts/surgery , Congenital Abnormalities/surgery , Retrospective Studies , Adolescent , Uterus/abnormalities , Uterus/surgery , Surgically-Created Structures , Adult , Epithelium/pathology , Young Adult , Plastic Surgery Procedures/methods , Biopsy , Somites/abnormalities , Microscopy, Electron, Scanning
7.
Arch Gynecol Obstet ; 309(3): 801-812, 2024 03.
Article in English | MEDLINE | ID: mdl-37466686

ABSTRACT

PURPOSE: This systematic review aims to provide a data synthesis about the risk of neovaginal cancer in women with Müllerian anomalies and to investigate the association between the adopted reconstructive technique and the cancer histotype. METHODS: PubMed, MEDLINE, Embase, Scopus, ClinicalTrials.gov and Web of Science databases were searched from inception to March 1st, 2023. Studies were included if: (1) only women affected by Müllerian malformations were included, (2) the congenital defect and the vaginoplasty technique were clearly reported, (3) the type of malignancy was specified. RESULTS: Literature search yielded 18 cases of squamous cell carcinoma and two cases of vaginal intraepithelial neoplasia 3 (VAIN 3). Of these, 3 had been operated on according to the Wharton technique, 8 according to the McIndoe technique, 3 with a split-skin graft vaginoplasty, 2 according to the Davydov technique, 2 with a simple cleavage technique, 1 according to the Vecchietti technique and 1 with a bladder flap vaginoplasty. A total of 17 cases of adenocarcinoma and 1 case of high-grade polypoid dysplasia were also described. Of these, 15 had undergone intestinal vaginoplasty, 1 had been operated on according to the McIndoe technique and 1 had undergone non-surgical vaginoplasty. Finally, 1 case of verrucous carcinoma in a woman who had undergone a split-skin graft vaginoplasty, was reported. CONCLUSION: Although rare, neovaginal carcinoma is a definite risk after vaginal reconstruction, regardless of the adopted technique. Gynaecologic visits including the speculum examination, the HPV DNA and/or the Pap smear tests should be scheduled on an annual basis.


Subject(s)
46, XX Disorders of Sex Development , Adenocarcinoma , Carcinoma, Squamous Cell , Congenital Abnormalities , Plastic Surgery Procedures , Vaginal Neoplasms , Humans , Female , Vagina/pathology , Vaginal Neoplasms/surgery , Vaginal Neoplasms/pathology , Carcinoma, Squamous Cell/pathology , Adenocarcinoma/pathology , Mullerian Ducts/surgery , Mullerian Ducts/abnormalities , 46, XX Disorders of Sex Development/surgery , Congenital Abnormalities/surgery , Congenital Abnormalities/pathology , Gynecologic Surgical Procedures/methods , Treatment Outcome
8.
Int Urogynecol J ; 35(2): 467-470, 2024 Feb.
Article in English | MEDLINE | ID: mdl-37823899

ABSTRACT

INTRODUCTION AND HYPOTHESIS: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a condition with an underdeveloped or absent vagina and uterus due to embryological growth failure of the Müllerian ducts. Many techniques have been described to construct a neovagina with an acceptable depth that allows penetrative intercourse. This is a step-by-step video tutorial on the Wharton-Sheares-George surgical technique for vaginoplasty in patients with MRKH syndrome. METHOD: With Wharton-Sheares-George vaginoplasty, the rudimentary Müllerian ducts are incrementally dilated by pushing Hegar dilators in the direction of the pelvic axis, and the resulting median raphe is then intersected using diathermy. As a result, a neovagina is created and an estriol-coated vaginal mold is inserted for 3 days. The patient receives comprehensive discharge instructions, a self-dilation program three times a day, and a monthly follow-up. RESULTS: A 3-month follow-up showed a high subjective degree of satisfaction with surgery and sexual satisfaction in both patients. CONCLUSION: Wharton-Sheares-George vaginoplasty is a safe and efficient technique for creating a neovagina for patients with MRKH syndrome.


Subject(s)
46, XX Disorders of Sex Development , Congenital Abnormalities , Mullerian Ducts , Mullerian Ducts/abnormalities , Female , Humans , Mullerian Ducts/surgery , Uterus/surgery , 46, XX Disorders of Sex Development/surgery , Vagina/surgery
9.
J Pediatr Surg ; 59(1): 124-128, 2024 Jan.
Article in English | MEDLINE | ID: mdl-37802758

ABSTRACT

PURPOSE: Various techniques for neovaginal construction have been employed in the pediatric and adult populations, including the use of intestinal segments, buccal mucosal grafts, and skin grafts. Small intestinal submucosa (SIS) extracellular matrix grafts have been described as a viable alternative, though prior experience is limited. Our purpose was to assess operative characteristics and patient outcomes with neovaginal construction using SIS grafts. METHODS: Thirteen patients underwent vaginoplasty with acellular porcine SIS grafts at our institution between 2018 and 2022. Operative and clinical data, postoperative mold management, vaginal dilating length, and complications were reviewed. RESULTS: Age at time of repair ranged from 13 to 30 years (median 19 years). Patient diagnosis included cloacal anomalies (n = 4), Mayer-Rokitansky-Küster-Hauser syndrome (n = 4), isolated vaginal atresia with or without a transverse vaginal septum (n = 4), and vaginal rhabdomyosarcoma requiring partial vaginectomy (n = 1). Following dissection of the neovaginal space, a silicon mold wrapped with SIS graft was placed with retention sutures and removed on postoperative day 7. Median (IQR) operative time was 171 (118-192) minutes, estimated blood loss was 10 (5-20) mL, and length of stay was 2 (1-3) days. The follow-up period ranged from 3 to 47 months (median 9 months). Two patients developed postoperative vaginal stenosis that resolved with dilation under anesthesia. Mean vaginal length on latest follow-up was 8.97 cm. All thirteen patients had successful engraftment and progressed to performing self-dilations or initiating intercourse to maintain patency. There were no cases of graft reaction or graft extrusion. CONCLUSIONS: We conclude that acellular small intestinal submucosa grafts are effective and safe alternatives for mold coverage in neovaginal construction. Our experience demonstrates minimal perioperative morbidity, early mold removal, and progression to successful dilation with maintenance of a functional vaginal length. Future study on sexual outcomes, patient satisfaction, and comparison against alternative techniques has been initiated. LEVEL OF EVIDENCE: IV. TYPE OF STUDY: Retrospective Study.


Subject(s)
46, XX Disorders of Sex Development , Congenital Abnormalities , Plastic Surgery Procedures , Adult , Humans , Animals , Swine , Female , Child , Adolescent , Young Adult , Vagina/surgery , Vagina/abnormalities , Retrospective Studies , Constriction, Pathologic/surgery , Patient Satisfaction , Mullerian Ducts/surgery , Mullerian Ducts/abnormalities , 46, XX Disorders of Sex Development/surgery , Congenital Abnormalities/surgery , Treatment Outcome
10.
J Minim Invasive Gynecol ; 31(1): 37-42, 2024 01.
Article in English | MEDLINE | ID: mdl-37820829

ABSTRACT

STUDY OBJECTIVE: To describe the long-term anatomic and sexual functional results of vaginoplasty with acellular dermal matrix (ADM) in patients with Mayer-Rokitansky-Küster-Hauser (MRKH) and to evaluate the changes in body image pre- and postoperatively in these patients. DESIGN: A retrospective study from March 2015 to September 2021. SETTING: A tertiary teaching hospital. PATIENTS: Forty-two patients with MRKH syndrome who underwent vaginoplasty with ADM (the MRKH group) and 30 sexually active, nulliparous, aged-matched women (the control group). INTERVENTION: The relevant data were retrospectively collected via our electronic medical record system and were analyzed statistically. MEASUREMENTS AND MAIN RESULTS: Vaginal length was assessed using a 3-cm-diameter mold. The Chinese version of the Female Sexual Function Index questionnaire was used to evaluate sexual function. The Chinese version of the modified body image scale was applied to evaluate body image. The median follow-up time was 57 months (range, 13-91 months). Granulomatous polyps in the neovagina were the most common postoperative complication (7 of 42, 16.7%). Patients with MRKH syndrome can achieve long-term satisfactory outcomes both anatomically and functionally after vaginoplasty with ADM, comparable with those of healthy control women. The vaginal length in the MRKH group was comparable to that in the control group ( 8.04 ± 0.51 cm vs. 8.15 ± 0.46 cm, respectively). The FSFI scores were similar between the MRKH (26.54 ± 3.44) and control (26.80 ± 2.23) groups. The modified body image scale score was significantly decreased after vaginoplasty with ADM. CONCLUSION: Vaginoplasty with ADM is a minimally invasive and effective procedure for patients with MRKH syndrome.


Subject(s)
46, XX Disorders of Sex Development , Acellular Dermis , Congenital Abnormalities , Female , Humans , Aged , Retrospective Studies , Body Image , Vagina/surgery , 46, XX Disorders of Sex Development/surgery , Mullerian Ducts/surgery , Congenital Abnormalities/surgery
11.
Fertil Steril ; 121(4): 679-692, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38104886

ABSTRACT

OBJECTIVE: To summarize the available evidence on the laparoscopic Davydov and Vecchietti methods to create a neovagina and to compare these techniques with a focus on neovaginal length, sexual function, operative time, and complications. DESIGN: A systematic electronic search up to August 2022 using PubMed and Embase is performed. SETTING: Not applicable. PATIENTS: Women with Mayer-Rokistansky-Küster-Hauser syndrome. INTERVENTIONS: All published clinical studies concerning the laparoscopic Davydov and laparoscopic Vecchietti procedures as a surgical technique to create a neovagina in women with Mayer-Rokistansky-Küster-Hauser syndrome were obtained. The guidelines for the preferred reporting items for systematic reviews and meta-analysis were followed. The following data were extracted: operative time, hospital stay, major early complications (within 3 months postsurgery), dilation therapy, neovaginal length, vaginal discharge, vaginal stenosis, time to sexual activity, sexual satisfaction, penetrative sexual activity, dyspareunia, score on the Female Sexual Function Index (FSFI), and duration of follow-up. The Newcastle Ottawa Scale was used to assess the quality of articles. MAIN OUTCOME MEASURES: Neovaginal length, FSFI scores, operative time, and complications. RESULTS: A total of 1,163 articles were identified, of which 33 studies were included in this systematic review. Of these, 12 studies (380 patients) are related to the Davydov method, 19 studies (1,126 patients) to the Vecchietti method, and 2 articles concern both. There is clinical heterogeneity and variety in the quality of the studies. Eighteen studies were included in the meta-analyses. The mean neovaginal length 12 months after the Davydov method is 8.3 cm (95% confidence interval [CI] 8.1-8.6), vs. 8.7 cm (95% CI 7.2-10.3) after the Vecchietti method. The mean FSFI score after the Davydov method is 28.9 (95% CI 26.8-31.1), compared with 27.5 (95% CI 25.0-30.1) after the Vecchietti method. The operative time of the Davydov method is 126 minutes (95% CI 109-143), compared with 40 minutes (95% CI 35-45) of the Vecchietti method. CONCLUSIONS: The operations yield comparable neovaginal length, sexual function, and complication rates. The mean FSFI scores indicate no sexual dysfunction in either group. The operative time of the Davydov method is significantly longer. There is no superiority shown for one of the surgical techniques in functional terms.


Subject(s)
46, XX Disorders of Sex Development , Congenital Abnormalities , Laparoscopy , Mullerian Ducts/abnormalities , Humans , Female , Vagina/surgery , Constriction, Pathologic/surgery , Laparoscopy/adverse effects , Laparoscopy/methods , 46, XX Disorders of Sex Development/diagnosis , 46, XX Disorders of Sex Development/surgery , Mullerian Ducts/surgery , Congenital Abnormalities/diagnosis , Congenital Abnormalities/surgery , Treatment Outcome
12.
BMC Womens Health ; 23(1): 634, 2023 11 27.
Article in English | MEDLINE | ID: mdl-38012663

ABSTRACT

BACKGROUND: In the past, the primary treatment for MRKH syndrome (Mayer-Rokitansky-Küster-Hauser syndrome) with a functional primordial uterus was surgical removal of the functional primordial uterus. In rare instances, the endometrium of the functional primordial uterus is well developed, and surgical preservation of the functional primordial uterus provides the possibility of preserving reproductive function for these patients. CASE PRESENTATION: A 14-year-old female was diagnosed with type I MRKH syndrome with a functional primordial uterus through physical examination and imaging investigations. We freed the functional primordial uterus through laparoscopic surgery and excised a portion of the lower myometrium to create an outlet at a lower uterine segment, which we then intermittently anastomosed to the tip of the artificial vagina. The patient recovered well after the surgery, and a re-examination showed no significant abnormalities. CONCLUSION: We were successful in preserving the functional primordial uterus using laparoscopic surgery in a patient with MRKH syndrome and connecting it to an artificial vagina through reconstructive surgery to ensure unobstructed menstrual drainage and preserve the reproductive potential of the patient.


Subject(s)
46, XX Disorders of Sex Development , Congenital Abnormalities , Laparoscopy , Female , Humans , Adolescent , Uterus/surgery , 46, XX Disorders of Sex Development/complications , 46, XX Disorders of Sex Development/surgery , 46, XX Disorders of Sex Development/diagnosis , Vagina/surgery , Mullerian Ducts/surgery , Laparoscopy/methods , Congenital Abnormalities/surgery
13.
J Gynecol Obstet Hum Reprod ; 52(10): 102674, 2023 Dec.
Article in English | MEDLINE | ID: mdl-37805077

ABSTRACT

OBJECTIVE: To better understand patients' conditions and expectations before starting a uterus transplantation (UTx) program for women suffering from Mayer-Rokitansky-Küster-Hauser syndrome (MRKH syndrome). METHOD: A web-based survey was conducted among MRKH patients via the French national association network from March to August 2020. The questionnaire comprised twenty-eight questions about their desire for parenthood, their condition's characteristics and previous reconstructive procedures, opinions and knowledge about UTx. RESULTS: Among the 148 participants, 88 % reported a desire for parenthood, and 61 % opted for UTx as their first choice to reach this aim. The possibility of bearing a child and having the same genetic heritage were the main motivations. Once informed about the usual course of an UTx protocol, only 13 % of the participants changed their mind and 3 out of 4 of them opted for UT. CONCLUSION: Uterus transplantation seems to be the first option to reach motherhood in patients suffering from MRKH syndrome. The development of UTx programs could meet the demands of this already well-informed population.


Subject(s)
46, XX Disorders of Sex Development , Motivation , Child , Humans , Female , Uterus , 46, XX Disorders of Sex Development/surgery , Mullerian Ducts/surgery
14.
Int Urogynecol J ; 34(11): 2647-2655, 2023 Nov.
Article in English | MEDLINE | ID: mdl-37490063

ABSTRACT

BACKGROUND: Bowel vaginoplasty is a surgical method for neovagina construction that, despite its advantages over other techniques, is still burdened by complications such as prolapse. The incidence of sigmoid neovagina prolapse (SNP) is difficult to determine, and there are no evidence-based recommendations for treatment. We present a case of SNP and a systematic review of previous cases. CASE: A 73-year-old woman presented with stage III prolapse of her sigmoid neovagina constructed 51 years prior. Dynamic pelvic MRI revealed that the majority of the prolapse was due to the mucosa's loss of support. Due to the presence of numerous pelvic adhesions, an alternative to the laparoscopic approach was evaluated by a multidisciplinary team which led to the patient being treated using a modification of Altemeier's procedure. SYSTEMATIC REVIEW: After PROSPERO Registration (CRD42023400677), a systematic search of Medline and Scopus was performed using specific search terms. Study metadata including patient demographics, prolapse measurements, reconstruction techniques, recurrence rates, and timing were extracted. Fourteen studies comprising 17 cases of SNP were included. Vaginal resection of the redundant sigmoid, comprising Altemeier's procedure, was the most definitive surgery, but it was also associated with recurrences in three cases. Laparoscopic sacropexy was the second most definitive surgery with no recurrence reported. CONCLUSION: Our review shows that the recurrence after correction of sigmoid neovagina prolapses is higher than previously reported. Laparoscopy colposacropexy appeared to be the best approach, but it's not always feasible. In these scenarios, a mucosal resection using the Altemeier's procedure is the most effective surgery.


Subject(s)
46, XX Disorders of Sex Development , Congenital Abnormalities , Laparoscopy , Humans , Female , Pregnancy , Aged , Colon, Sigmoid/surgery , Prolapse , Vagina/surgery , Laparoscopy/methods , Colpotomy , Mullerian Ducts/surgery , Congenital Abnormalities/surgery , 46, XX Disorders of Sex Development/surgery
15.
J Pediatr Adolesc Gynecol ; 36(6): 556-559, 2023 Dec.
Article in English | MEDLINE | ID: mdl-37354985

ABSTRACT

OBJECTIVE: To describe surgical correction of vaginal agenesis via a modified laparoscopic Vecchietti procedure with the goal of disseminating knowledge and improving surgical technique CASE: An 18-year-old female presented with primary amenorrhea, age-appropriate secondary sex characteristics, a shallow vagina, and 46,XX karyotype. Imaging showed rudimentary uterine horns and normal ovaries, kidneys, and spine. Diagnosis of Mayer-Rokitansky-Küster-Hauser syndrome type I was made. After an unsuccessful attempt at vaginal dilation and extensive counseling, the patient chose to have a laparoscopic Vecchietti procedure. Vecchietti vaginoplasty eliminates the need for grafts and creates a neovagina with accelerated vaginal dilation by stretching the introital mucosa with a spring mechanism. RESULTS: A modified laparoscopic Vecchietti procedure was performed. Postoperatively, daily suture adjustments were made. When the device was removed after 7 days, the examination revealed a 9-cm vaginal canal, which was maintained with self-dilation.


Subject(s)
46, XX Disorders of Sex Development , Congenital Abnormalities , Laparoscopy , Plastic Surgery Procedures , Female , Animals , Humans , Adolescent , Vagina/surgery , Vagina/abnormalities , Laparoscopy/methods , Uterus/surgery , 46, XX Disorders of Sex Development/surgery , Mullerian Ducts/surgery , Mullerian Ducts/abnormalities , Congenital Abnormalities/surgery , Treatment Outcome
16.
J Minim Invasive Gynecol ; 30(9): 705-715, 2023 09.
Article in English | MEDLINE | ID: mdl-37271411

ABSTRACT

OBJECTIVE: In patients with Rokitansky syndrome, vaginal agenesis can be treated using various surgical techniques, and various factors can affect each surgery outcome in the long term. This meta-analysis aimed to evaluate sexual function outcomes in patients with Rokitansky syndrome after various surgery techniques. DATA SOURCES: Searches were conducted in Google Scholar, PubMed, Cochrane database, ScienceDirect, Web of Science, and ClinicalTrials.gov. Systematic searches were conducted on studies published until November 2022 (CRD42022370735). METHODS OF STUDY SELECTION: During the first stage of database scanning and reference check, 1820 results were identified, and an evaluation of the total 10 studies was finally conducted. The inclusion criteria involved selecting randomized controlled trials that focused on assessing sexual function after surgical treatment in patients of all ages with Rokitansky syndrome. TABULATION, INTEGRATION, AND RESULTS: EndNote version 20 software was used to organize and identify duplicate articles through screening. The Joanna Briggs Institute's critical appraisal tool was used to evaluate each study's quality for bias potential. The results showed that the total scores of female sexual functions in patients with Rokitansky syndrome after vaginoplasty were significantly lower than in healthy women (standardized mean difference, -0.233; p <.05; range, -0.376 to -0.090). The 6-domain analysis of the Female Sexual Function Index questionnaire revealed that lubrication (p <.05) and satisfaction (p <.05) were significantly lower in patients undergoing vaginoplasty. CONCLUSION: All surgical techniques to create a neovagina for patients with Rokitansky syndrome have successfully affected the sexual function outcomes. Considering all other factors affecting sexual function outcomes in the long term, more quantitative and qualitative studies are needed to assess sexual satisfaction in patients treated with surgical techniques.


Subject(s)
46, XX Disorders of Sex Development , Abnormalities, Multiple , Congenital Abnormalities , Female , Humans , Treatment Outcome , Vagina/abnormalities , 46, XX Disorders of Sex Development/complications , 46, XX Disorders of Sex Development/surgery , Mullerian Ducts/surgery , Mullerian Ducts/abnormalities
17.
Aust N Z J Obstet Gynaecol ; 63(4): 599-602, 2023 08.
Article in English | MEDLINE | ID: mdl-37200477

ABSTRACT

Uterine transplantation (UT) is an emerging medical treatment for women affected by absolute uterine factor infertility (AUFI). To date there have been over 90 documented cases of UT performed worldwide, with over 50 live births. UT allows women affected by AUFI the opportunity to carry and deliver a childd. The Royal Prince Alfred Hospital (RPAH) introduced a UT study in 2019; however, due to the impacts of the COVID pandemic the study was placed on hold for two years. In February 2023, RPAH performed the centre's first UT from a living unrelated donor to a 25-year-old woman with Mayer-Rokitansky-Küster-Hauser syndrome. The donor and recipient surgeries were uncomplicated and both are recovering well in the early post-operative period.


Subject(s)
46, XX Disorders of Sex Development , COVID-19 , Congenital Abnormalities , Infertility, Female , Female , Humans , Adult , Uterus/surgery , Infertility, Female/etiology , Infertility, Female/surgery , Hospitals , 46, XX Disorders of Sex Development/complications , 46, XX Disorders of Sex Development/surgery
18.
J Low Genit Tract Dis ; 27(3): 275-279, 2023 Jul 01.
Article in English | MEDLINE | ID: mdl-37192410

ABSTRACT

OBJECTIVES: To study the clinical, cytological, and vaginal microbiota findings in patients with Mayer-Rokitansky-Küster-Hauser syndrome who underwent neovaginoplasty using Nile tilapia fish skin. METHODS: This is a cross-sectional study with 7 cisgender women with Mayer-Rokitansky-Küster-Hauser syndrome who had previously undergone neovagina reconstruction using Nile tilapia fish skin at a university hospital. Local institutional review board approval and written permission from the patient were obtained. Between August 2019 and November 2021, within 12 to 24 months after surgery, vaginal specimens were obtained for conventional oncotic and hormonal cytology, and for Gram staining. The Nugent scores were calculated. Colposcopy was also performed. RESULTS: Squamous cells without atypia were found in all patients. Five patients had intermediate vaginal microbiota (Nugent score of 4), which was determined by the presence of few lactobacilli on Gram staining. In hormonal cytology, 4 patients presented with findings compatible with menacme. No colposcopic change was observed. When postsurgical dilation was performed correctly, a mean vaginal length of 8.3 cm was maintained after 1 year of follow-up. CONCLUSIONS: Squamous cells without atypia were present in neovaginas with Nile tilapia fish skin. Most vaginal contents revealed intermediate microbiota and hormonal results compatible with menacme. Studies with a greater number of patients are necessary for a more comprehensive understanding of the microbiome in neovaginas with this new technique, thereby providing support for the treatment and prevention of associated pathologies.


Subject(s)
46, XX Disorders of Sex Development , Cichlids , Congenital Abnormalities , Animals , Female , Humans , Treatment Outcome , Cross-Sectional Studies , Vagina/surgery , Vagina/pathology , 46, XX Disorders of Sex Development/pathology , 46, XX Disorders of Sex Development/surgery , Mullerian Ducts/surgery , Congenital Abnormalities/surgery , Congenital Abnormalities/pathology
19.
BMJ Case Rep ; 16(5)2023 May 08.
Article in English | MEDLINE | ID: mdl-37156565

ABSTRACT

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, a condition caused by Müllerian anomalies, is characterised by congenital vaginal aplasia and a rudimentary uterus. Case reports concerning uterine fibroids associated with MRKH syndrome are limited, and differentiating between uterine fibroids and ovarian solid tumours prior to surgical intervention is often challenging. Here, we present the case of a patient with MRKH syndrome and asymptomatic bilateral pelvic solid tumours located close to both ovaries. Based on intraoperative and histopathological findings, the tumours were diagnosed as adenomyomas of the rudimentary uterus. This is the first reported case of a uterine adenomyoma associated with MRKH syndrome. Moreover, our report highlights the fact that diagnostic laparoscopy is a valuable method to evaluate pelvic tumours in MRKH syndrome.


Subject(s)
46, XX Disorders of Sex Development , Adenomyoma , Congenital Abnormalities , Laparoscopy , Leiomyoma , Pelvic Neoplasms , Female , Humans , Adenomyoma/complications , Adenomyoma/diagnosis , Adenomyoma/surgery , Pelvic Neoplasms/surgery , Uterus/surgery , Uterus/abnormalities , 46, XX Disorders of Sex Development/complications , 46, XX Disorders of Sex Development/diagnosis , 46, XX Disorders of Sex Development/surgery , Vagina/surgery , Vagina/abnormalities , Mullerian Ducts/surgery , Mullerian Ducts/abnormalities , Laparoscopy/methods , Leiomyoma/surgery , Congenital Abnormalities/diagnosis , Congenital Abnormalities/surgery
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