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1.
Am J Case Rep ; 25: e943305, 2024 Jun 13.
Artigo em Inglês | MEDLINE | ID: mdl-38867420

RESUMO

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.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Colo Sigmoide , Laparoscopia , Ductos Paramesonéfricos , Períneo , Vagina , Humanos , Feminino , Adulto , Laparoscopia/métodos , Colo Sigmoide/cirurgia , Vagina/cirurgia , Vagina/anormalidades , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Ductos Paramesonéfricos/anormalidades , Ductos Paramesonéfricos/cirurgia , Períneo/cirurgia , Anormalidades Congênitas/cirurgia , Procedimentos de Cirurgia Plástica/métodos
2.
Niger J Clin Pract ; 27(5): 643-646, 2024 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-38842714

RESUMO

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.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Ductos Paramesonéfricos , Centros de Atenção Terciária , Vagina , Humanos , Feminino , Vagina/cirurgia , Vagina/anormalidades , Ductos Paramesonéfricos/anormalidades , Ductos Paramesonéfricos/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Adulto , Adolescente , Anormalidades Congênitas/cirurgia , Adulto Jovem , Procedimentos de Cirurgia Plástica/métodos , Resultado do Tratamento , Procedimentos Cirúrgicos em Ginecologia/métodos , Estudos de Coortes , Estudos Prospectivos
3.
Int Urogynecol J ; 35(7): 1503-1510, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38869514

RESUMO

INTRODUCTION AND HYPOTHESIS: We investigate the feasibility, safety, and clinical therapeutic effect of laparoscopic sigmoid vaginoplasty in women with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. METHODS: We performed a retrospective case review cohort study of 56 patients with MRKHs undergoing laparoscopic sigmoid vaginoplasty in Wuhan Union Hospital between 2000 and 2020, and all patients were followed up. RESULTS: The median operating time was 165 min (120-420 min). The median hospital stay was 10 days (rang 7-15 days). A functional neovagina was created 11-15 cm in length and two fingers in breadth in all patients. No introitus stenosis was observed. No intra- or post-operative complications occurred. Two patients were lost to follow-up after 3 months of outpatient visits. Six patients had no intercourse and were required to wear a vaginal mold occasionally. None of the patients had complained of local irritation or dyspareunia. Patients who had post-surgery sexual intercourse were satisfied with their sexual life and the mean total Female Sexual Function Index (FSFI) score was 25.17 ± 0.63. The cosmetic results were excellent. CONCLUSIONS: The laparoscopic sigmoid vaginoplasty can achieve the goal of making a functional neovagina. The main advantage of this surgical technique is that it is minimally invasive and that there are fewer complications post-operation. It is an acceptable procedure for patients with MRKH syndrome.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Laparoscopia , Ductos Paramesonéfricos , Vagina , Humanos , Feminino , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Vagina/cirurgia , Vagina/anormalidades , Laparoscopia/métodos , Estudos Retrospectivos , Ductos Paramesonéfricos/anormalidades , Ductos Paramesonéfricos/cirurgia , Anormalidades Congênitas/cirurgia , Adulto , Adulto Jovem , Adolescente , Resultado do Tratamento , Colo Sigmoide/cirurgia , Procedimentos Cirúrgicos em Ginecologia/métodos , Procedimentos de Cirurgia Plástica/métodos , Estruturas Criadas Cirurgicamente , Estudos de Viabilidade , Duração da Cirurgia
4.
Zhonghua Fu Chan Ke Za Zhi ; 59(5): 360-367, 2024 May 25.
Artigo em Chinês | MEDLINE | ID: mdl-38797565

RESUMO

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.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Endometriose , Ductos Paramesonéfricos , Útero , Vagina , Humanos , Feminino , Adolescente , Estudos Retrospectivos , Vagina/anormalidades , Vagina/cirurgia , Ductos Paramesonéfricos/anormalidades , Endometriose/cirurgia , Endometriose/diagnóstico , Endometriose/patologia , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Anormalidades Congênitas/epidemiologia , Útero/anormalidades , Útero/cirurgia , Útero/patologia , Adulto Jovem , Anormalidades Urogenitais/cirurgia , Anormalidades Múltiplas/epidemiologia , Criança , Prognóstico , Genitália Feminina/anormalidades , Genitália Feminina/cirurgia , Genitália Feminina/patologia
5.
Eur J Obstet Gynecol Reprod Biol ; 298: 85-90, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38733778

RESUMO

BACKGROUND: The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare condition with significant psychological implications. However, our understanding of its impact on postoperative sexual function and mental health is still limited. AIM: Evaluate the mental health status and sexual functioning of women with MRKH syndrome after vaginoplasty surgery. METHODS: We enrolled 53 cases with MRKH syndrome who underwent artificial vaginoplasty. The participants were asked to participate in a two-round survey conducted between February 2021 during the covid-19 period and March 2023. The survey included questionnaires to measure depression, anxiety, self-esteem, and sexual functioning. Differences between scores over time were analysed using a paired sample t-test, and we assessed the correlation between mental health and sexual functioning. RESULTS: In the first round, patients' mean ± SD age at surgery was 23.6 ± 4.5 years old, and the mean ± SD time that had elapsed since surgery at the time of the survey was 34.2 ± 20.8 months. None of the patients reported low self-esteem, 45.3 % reported mild-to-moderate depression, and 34.0 % reported mild anxiety. Thirty patients have had vaginal intercourse during the last six months. The mean ± SD Female Sexual Functioning Index score was 24.6 ± 4.4, and 60.0 % had a score of 23.5 or higher, indicating high sexual functioning. The sexual functioning scores were positively correlated with self-esteem scores and negatively correlated with depression or anxiety scores (p < 0.05). There was no significant improvement in patient's mental health status and sexual function between the second round survey (71.3 ± 17.8 months after surgery) and the first round survey (p > 0.05). In contrast, the sexual arousal of FSFI were significantly higher in the second survey round (p < 0.05). CONCLUSION: Most patients undergoing vaginoplasty reported persisting mental health challenges. However, the majority reported good sexual functioning.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Ansiedade , Anormalidades Congênitas , Depressão , Ductos Paramesonéfricos , Autoimagem , Vagina , Humanos , Feminino , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/psicologia , Vagina/cirurgia , Vagina/anormalidades , Ductos Paramesonéfricos/anormalidades , Ductos Paramesonéfricos/cirurgia , Anormalidades Congênitas/cirurgia , Anormalidades Congênitas/psicologia , Adulto , Depressão/psicologia , Depressão/etiologia , Ansiedade/psicologia , Ansiedade/etiologia , Adulto Jovem , Comportamento Sexual/psicologia , Adolescente , COVID-19/psicologia
7.
Fertil Steril ; 122(2): 382-384, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38554765

RESUMO

OBJECTIVE: To present a new surgical technique on the basis of an internal thigh fasciocutaneous flap for generating a compliant and sensitive neovagina with preservation of the external genitalia. DESIGN: Video demonstration of the surgical steps. PATIENT(S): An 18-year-old woman with Müllerian agenesis confirmed at ultrasound and magnetic resonance imaging. The residual vagina was 3 cm long and 1.5 cm wide. After counseling by a gynecologist and plastic surgeon, in which all available techniques with pros and cons were exposed, the patient opted for the new technique. The long time required by conservative approaches and the desire to preserve the external genitalia with the chance to have a sensitive vagina guided the choice. INTERVENTION(S): The cul-de-sac of the vaginal stump was incised transversally. A 4-cm-wide and 9-cm-long canal bounded anteriorly by the bladder, posteriorly by the rectum, and superiorly by the peritoneum of Douglas was developed by blunt dissection. Fasciocutaneous flaps of 12 per 5 cm on the anteromedial aspect of the thighs were developed, identifying the vascular-from the pudendal artery-and nervous pedicles. A tunnel between the flap pedicles and neovagina introitus was created between fascia and subcutaneous tissue, detaching the vulvar structures from the ischiopubic ramus. Flaps were tunneled up to the neovagina introitus and sutured together by interrupted suture to form a tube with outside skin. The flaps were transposed into the canal everting the tube to obtain the skin lining the internal neovagina. The inferior margins of the flaps were sutured to the vaginal stump mucosa. No internal stitches were placed. Antibiotic prophylaxis was used during surgery. The entire procedure lasted 6 hours. During the postoperative period, no special positioning or ambulation restrictions were used. MAIN OUTCOME MEASURE(S): Compliance and sensitivity of the neovagina, esthetic result, and perioperative and long-term complications. RESULT(S): The postoperative course was uneventful, with early mobilization. The length of hospital stay was 16 days to allow proper vaginal dilator use; initial daily followed by intermittent use was planned. At a 2-year follow-up, the neovagina was sensitive and patent, allowing sexual intercourse. No complications were reported, and the patient was satisfied with the functional and esthetic result. CONCLUSION(S): The new surgical technique was feasible and effective, preserving the external genitalia and avoiding graft healing and bowel secretion drawbacks without an intra-abdominal surgical step and related risks. However, more cases-2 cases performed to date with similar results-and long-term follow-up are needed to confirm the efficacy. In this regard, the regular use of vaginal dilators and forecast adherence between flaps and the connective tissue of the bladder and rectum are expected to prevent neovagina prolapse without any anchoring to the pelvic structures.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Ductos Paramesonéfricos , Retalhos Cirúrgicos , Vagina , Humanos , Feminino , Vagina/cirurgia , Vagina/anormalidades , Adolescente , Ductos Paramesonéfricos/anormalidades , Ductos Paramesonéfricos/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Procedimentos de Cirurgia Plástica/métodos , Anormalidades Congênitas/cirurgia , Estruturas Criadas Cirurgicamente , Procedimentos Cirúrgicos em Ginecologia/métodos , Resultado do Tratamento
8.
J Pediatr Adolesc Gynecol ; 37(3): 353-359, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38246338

RESUMO

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.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Ductos Paramesonéfricos , Vagina , Humanos , Feminino , Vagina/cirurgia , Vagina/anormalidades , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/patologia , Ductos Paramesonéfricos/anormalidades , Ductos Paramesonéfricos/cirurgia , Anormalidades Congênitas/cirurgia , Estudos Retrospectivos , Adolescente , Útero/anormalidades , Útero/cirurgia , Estruturas Criadas Cirurgicamente , Adulto , Epitélio/patologia , Adulto Jovem , Procedimentos de Cirurgia Plástica/métodos , Biópsia , Somitos/anormalidades , Microscopia Eletrônica de Varredura
9.
Arch Gynecol Obstet ; 309(3): 801-812, 2024 03.
Artigo em Inglês | MEDLINE | ID: mdl-37466686

RESUMO

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.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Adenocarcinoma , Carcinoma de Células Escamosas , Anormalidades Congênitas , Procedimentos de Cirurgia Plástica , Neoplasias Vaginais , Humanos , Feminino , Vagina/patologia , Neoplasias Vaginais/cirurgia , Neoplasias Vaginais/patologia , Carcinoma de Células Escamosas/patologia , Adenocarcinoma/patologia , Ductos Paramesonéfricos/cirurgia , Ductos Paramesonéfricos/anormalidades , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Anormalidades Congênitas/cirurgia , Anormalidades Congênitas/patologia , Procedimentos Cirúrgicos em Ginecologia/métodos , Resultado do Tratamento
10.
Int Urogynecol J ; 35(2): 467-470, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37823899

RESUMO

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.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Ductos Paramesonéfricos , Ductos Paramesonéfricos/anormalidades , Feminino , Humanos , Ductos Paramesonéfricos/cirurgia , Útero/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Vagina/cirurgia
11.
J Minim Invasive Gynecol ; 31(1): 37-42, 2024 01.
Artigo em Inglês | MEDLINE | ID: mdl-37820829

RESUMO

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.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Derme Acelular , Anormalidades Congênitas , Feminino , Humanos , Idoso , Estudos Retrospectivos , Imagem Corporal , Vagina/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Ductos Paramesonéfricos/cirurgia , Anormalidades Congênitas/cirurgia
12.
J Pediatr Surg ; 59(1): 124-128, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37802758

RESUMO

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.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Procedimentos de Cirurgia Plástica , Adulto , Humanos , Animais , Suínos , Feminino , Criança , Adolescente , Adulto Jovem , Vagina/cirurgia , Vagina/anormalidades , Estudos Retrospectivos , Constrição Patológica/cirurgia , Satisfação do Paciente , Ductos Paramesonéfricos/cirurgia , Ductos Paramesonéfricos/anormalidades , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Anormalidades Congênitas/cirurgia , Resultado do Tratamento
13.
Fertil Steril ; 121(4): 679-692, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38104886

RESUMO

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.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Laparoscopia , Ductos Paramesonéfricos , Procedimentos de Cirurgia Plástica , Vagina , Humanos , Feminino , Ductos Paramesonéfricos/anormalidades , Ductos Paramesonéfricos/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Vagina/cirurgia , Vagina/anormalidades , Laparoscopia/métodos , Laparoscopia/efeitos adversos , Anormalidades Congênitas/cirurgia , Procedimentos de Cirurgia Plástica/métodos , Procedimentos de Cirurgia Plástica/efeitos adversos , Resultado do Tratamento , Procedimentos Cirúrgicos em Ginecologia/métodos , Procedimentos Cirúrgicos em Ginecologia/efeitos adversos , Estruturas Criadas Cirurgicamente/efeitos adversos , Adulto
14.
BMC Womens Health ; 23(1): 634, 2023 11 27.
Artigo em Inglês | MEDLINE | ID: mdl-38012663

RESUMO

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.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Laparoscopia , Feminino , Humanos , Adolescente , Útero/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/complicações , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/diagnóstico , Vagina/cirurgia , Ductos Paramesonéfricos/cirurgia , Laparoscopia/métodos , Anormalidades Congênitas/cirurgia
15.
Int Urogynecol J ; 34(11): 2647-2655, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37490063

RESUMO

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.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Laparoscopia , Humanos , Feminino , Gravidez , Idoso , Colo Sigmoide/cirurgia , Prolapso , Vagina/cirurgia , Laparoscopia/métodos , Colpotomia , Ductos Paramesonéfricos/cirurgia , Anormalidades Congênitas/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia
16.
J Pediatr Adolesc Gynecol ; 36(6): 556-559, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37354985

RESUMO

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.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Laparoscopia , Procedimentos de Cirurgia Plástica , Feminino , Animais , Humanos , Adolescente , Vagina/cirurgia , Vagina/anormalidades , Laparoscopia/métodos , Útero/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Ductos Paramesonéfricos/cirurgia , Ductos Paramesonéfricos/anormalidades , Anormalidades Congênitas/cirurgia , Resultado do Tratamento
17.
J Minim Invasive Gynecol ; 30(9): 705-715, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-37271411

RESUMO

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.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Múltiplas , Anormalidades Congênitas , Feminino , Humanos , Resultado do Tratamento , Vagina/anormalidades , Transtornos 46, XX do Desenvolvimento Sexual/complicações , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Ductos Paramesonéfricos/cirurgia , Ductos Paramesonéfricos/anormalidades
18.
J Low Genit Tract Dis ; 27(3): 275-279, 2023 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-37192410

RESUMO

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.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Ciclídeos , Anormalidades Congênitas , Animais , Feminino , Humanos , Resultado do Tratamento , Estudos Transversais , Vagina/cirurgia , Vagina/patologia , Transtornos 46, XX do Desenvolvimento Sexual/patologia , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Ductos Paramesonéfricos/cirurgia , Anormalidades Congênitas/cirurgia , Anormalidades Congênitas/patologia
19.
BMJ Case Rep ; 16(5)2023 May 08.
Artigo em Inglês | MEDLINE | ID: mdl-37156565

RESUMO

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.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Adenomioma , Anormalidades Congênitas , Laparoscopia , Leiomioma , Neoplasias Pélvicas , Feminino , Humanos , Adenomioma/complicações , Adenomioma/diagnóstico , Adenomioma/cirurgia , Neoplasias Pélvicas/cirurgia , Útero/cirurgia , Útero/anormalidades , Transtornos 46, XX do Desenvolvimento Sexual/complicações , Transtornos 46, XX do Desenvolvimento Sexual/diagnóstico , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Vagina/cirurgia , Vagina/anormalidades , Ductos Paramesonéfricos/cirurgia , Ductos Paramesonéfricos/anormalidades , Laparoscopia/métodos , Leiomioma/cirurgia , Anormalidades Congênitas/diagnóstico , Anormalidades Congênitas/cirurgia
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