The Experts below are selected from a list of 138 Experts worldwide ranked by ideXlab platform
Luigi Fedele - One of the best experts on this subject based on the ideXlab platform.
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creation of a neovagina in Rokitansky Syndrome comparison between two laparoscopic techniques
Fertility and Sterility, 2011Co-Authors: Stefano Bianchi, Elisa Restelli, Giada Frontino, Nevio Ciappina, Luigi FedeleAbstract:Objective To compare Vecchietti's and Davydov's laparoscopic techniques for creation of a neovagina in patients with Rokitansky Syndrome. Design Comparative retrospective study. Setting Tertiary referral center for the treatment of Rokitansky Syndrome. Patient(s) Eighty patients with Rokitansky Syndrome. Intervention(s) Patients underwent surgical creation of a neovagina. Fifteen patients who underwent the Vecchietti procedure from October 2003 to December 2004 and 30 patients who underwent the Davydov procedure from June 2005 to August 2008 were also included from two previously published studies. Follow-up lasted at least 12 months. Main Outcome Measure(s) Intraoperative parameters and anatomic results were compared. Functional results were compared through the Female Sexual Function Index. Epithelization of the neovagina was assessed in both groups through vaginoscopy and Schiller's test. Result(s) No major intraoperative complications were encountered in either group. Mean (±SD) duration of surgery was 30 ± 9.6 and 134 ± 24 minutes in Vecchietti's and Davydov's approach, respectively. At 12 postoperative months, length and width of the neovagina in the two groups were 7.5 ± 1.1 and 2.8 ± 0.6 cm, and 8.5 ± 1.6 and 2.8 ± 0.65, respectively. Epithelization of the neovagina at 6-month follow-up was 60% and 80%, respectively, and 100% in both groups at 12 postoperative months. Conclusion(s) Anatomic and functional outcomes of the two approaches tend to be comparable at 12-month follow-up; the only significant difference seems to be in greater length for the neovagina obtained by Davydov's approach.
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creation of a neovagina by davydov s laparoscopic modified technique in patients with Rokitansky Syndrome
American Journal of Obstetrics and Gynecology, 2010Co-Authors: Luigi Fedele, Giada Frontino, Elisa Restelli, Nevio Ciappina, Francesca Motta, Stefano BianchiAbstract:Objective The purpose of this study was to assess anatomic and functional results after the laparoscopic Davydov procedure for the creation of a neovagina in Rokitansky Syndrome. Study Design Thirty patients with Rokitansky Syndrome underwent the laparoscopic Davydov technique from June 2005–August 2008. Mean follow-up time lasted 30 months (range, 6–44 months) and included clinical examinations and evaluation of the quality of sexual intercourse; vaginoscopy, Schiller's test, and neovaginal biopsies were performed after 6 and 12 months. Functional results were assessed with the use of Rosen's Female Sexual Function Index and were compared with age-matched normal control subjects. Results No perioperative complications occurred. At 6 months, anatomic success was achieved in 97% of the patients (n = 29); functional success and optimal results for the Female Sexual Function Index questionnaire were obtained in 96% of patients. Vaginoscopy and biopsy results showed a normal iodine-positive vaginal epithelium. Conclusion The Davydov technique seems to be a safe and effective treatment for vaginal agenesis in patients with Rokitansky Syndrome.
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the laparoscopic vecchietti s modified technique in Rokitansky Syndrome anatomic functional and sexual long term results
American Journal of Obstetrics and Gynecology, 2008Co-Authors: Luigi Fedele, Giada Frontino, Elisa Restelli, Stefano Bianchi, Eleonora Fontana, Vincenzina BruniAbstract:Objective The objective of the study was to assess the anatomical and functional long-term follow-up results of the laparoscopic Vecchietti approach for the creation of a neovagina in the Rokitansky Syndrome. Study Design One hundred ten patients underwent clinical follow-up visits at 1, 3, 6, and 12 months after surgery and every 6 months thereafter. The following were performed: evaluation of the quality of sexual intercourse, vaginal and rectal examinations, vaginoscopy, Schiller’s test, and vaginal cytology with microbiologic testing. Functional results were assessed by using Rosen’s Female Sexual Function Index questionnaire, of which the results were analyzed comparing normal age-matched controls. Results Four patients were lost to follow-up. Anatomic and functional success was achieved in 104 of 106 (98%) and 103 of 106 (97%) patients, respectively. Female Sexual Function Index scores were comparable with those of controls. Conclusion Vecchietti’s technique is simple, safe, and effective and allows normal and satisfying sexual intercourse, comparable with that of normal controls.
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laparoscopic findings and pelvic anatomy in mayer Rokitansky kuster hauser Syndrome
Obstetrics & Gynecology, 2007Co-Authors: Luigi Fedele, Giada Frontino, Nevio Ciappina, Stefano Bianchi, Eleonora Fontana, Franco BorrutoAbstract: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, accordi
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laparoscopic creation of a neovagina and recovery of menstrual function in a patient with Rokitansky Syndrome a case report
Human Reproduction, 2006Co-Authors: Luigi Fedele, Stefano Bianchi, Nicola Berlanda, Alessandro Bulfoni, Eleonora FontanaAbstract:A 16-year-old woman experiencing primary amenorrhoea and cyclic pelvic pain was diagnosed with Rokitansky Syndrome, which was characterized by the absence of the uterus and the upper two-thirds of the vagina, normal salpinges and ovaries and a 4 x 3 cm Miillerian remnant containing functioning endometrium located near the left adnexa. With a combined laparoscopic-vaginal operation, the remnant was anastomized with the apex of the retrohymenal fovea. The operation allowed not only the creation of a neovagina but also the recovery of a regular menstrual activity and the theoretical restoration of the reproductive capacity of this patient. An accurate pre- and intra-operative evaluation of patients with Rokitansky Syndrome is necessary to identify those who might benefit from this procedure.
Stefano Bianchi - One of the best experts on this subject based on the ideXlab platform.
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creation of a neovagina in Rokitansky Syndrome comparison between two laparoscopic techniques
Fertility and Sterility, 2011Co-Authors: Stefano Bianchi, Elisa Restelli, Giada Frontino, Nevio Ciappina, Luigi FedeleAbstract:Objective To compare Vecchietti's and Davydov's laparoscopic techniques for creation of a neovagina in patients with Rokitansky Syndrome. Design Comparative retrospective study. Setting Tertiary referral center for the treatment of Rokitansky Syndrome. Patient(s) Eighty patients with Rokitansky Syndrome. Intervention(s) Patients underwent surgical creation of a neovagina. Fifteen patients who underwent the Vecchietti procedure from October 2003 to December 2004 and 30 patients who underwent the Davydov procedure from June 2005 to August 2008 were also included from two previously published studies. Follow-up lasted at least 12 months. Main Outcome Measure(s) Intraoperative parameters and anatomic results were compared. Functional results were compared through the Female Sexual Function Index. Epithelization of the neovagina was assessed in both groups through vaginoscopy and Schiller's test. Result(s) No major intraoperative complications were encountered in either group. Mean (±SD) duration of surgery was 30 ± 9.6 and 134 ± 24 minutes in Vecchietti's and Davydov's approach, respectively. At 12 postoperative months, length and width of the neovagina in the two groups were 7.5 ± 1.1 and 2.8 ± 0.6 cm, and 8.5 ± 1.6 and 2.8 ± 0.65, respectively. Epithelization of the neovagina at 6-month follow-up was 60% and 80%, respectively, and 100% in both groups at 12 postoperative months. Conclusion(s) Anatomic and functional outcomes of the two approaches tend to be comparable at 12-month follow-up; the only significant difference seems to be in greater length for the neovagina obtained by Davydov's approach.
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creation of a neovagina by davydov s laparoscopic modified technique in patients with Rokitansky Syndrome
American Journal of Obstetrics and Gynecology, 2010Co-Authors: Luigi Fedele, Giada Frontino, Elisa Restelli, Nevio Ciappina, Francesca Motta, Stefano BianchiAbstract:Objective The purpose of this study was to assess anatomic and functional results after the laparoscopic Davydov procedure for the creation of a neovagina in Rokitansky Syndrome. Study Design Thirty patients with Rokitansky Syndrome underwent the laparoscopic Davydov technique from June 2005–August 2008. Mean follow-up time lasted 30 months (range, 6–44 months) and included clinical examinations and evaluation of the quality of sexual intercourse; vaginoscopy, Schiller's test, and neovaginal biopsies were performed after 6 and 12 months. Functional results were assessed with the use of Rosen's Female Sexual Function Index and were compared with age-matched normal control subjects. Results No perioperative complications occurred. At 6 months, anatomic success was achieved in 97% of the patients (n = 29); functional success and optimal results for the Female Sexual Function Index questionnaire were obtained in 96% of patients. Vaginoscopy and biopsy results showed a normal iodine-positive vaginal epithelium. Conclusion The Davydov technique seems to be a safe and effective treatment for vaginal agenesis in patients with Rokitansky Syndrome.
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the laparoscopic vecchietti s modified technique in Rokitansky Syndrome anatomic functional and sexual long term results
American Journal of Obstetrics and Gynecology, 2008Co-Authors: Luigi Fedele, Giada Frontino, Elisa Restelli, Stefano Bianchi, Eleonora Fontana, Vincenzina BruniAbstract:Objective The objective of the study was to assess the anatomical and functional long-term follow-up results of the laparoscopic Vecchietti approach for the creation of a neovagina in the Rokitansky Syndrome. Study Design One hundred ten patients underwent clinical follow-up visits at 1, 3, 6, and 12 months after surgery and every 6 months thereafter. The following were performed: evaluation of the quality of sexual intercourse, vaginal and rectal examinations, vaginoscopy, Schiller’s test, and vaginal cytology with microbiologic testing. Functional results were assessed by using Rosen’s Female Sexual Function Index questionnaire, of which the results were analyzed comparing normal age-matched controls. Results Four patients were lost to follow-up. Anatomic and functional success was achieved in 104 of 106 (98%) and 103 of 106 (97%) patients, respectively. Female Sexual Function Index scores were comparable with those of controls. Conclusion Vecchietti’s technique is simple, safe, and effective and allows normal and satisfying sexual intercourse, comparable with that of normal controls.
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laparoscopic findings and pelvic anatomy in mayer Rokitansky kuster hauser Syndrome
Obstetrics & Gynecology, 2007Co-Authors: Luigi Fedele, Giada Frontino, Nevio Ciappina, Stefano Bianchi, Eleonora Fontana, Franco BorrutoAbstract: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, accordi
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laparoscopic creation of a neovagina and recovery of menstrual function in a patient with Rokitansky Syndrome a case report
Human Reproduction, 2006Co-Authors: Luigi Fedele, Stefano Bianchi, Nicola Berlanda, Alessandro Bulfoni, Eleonora FontanaAbstract:A 16-year-old woman experiencing primary amenorrhoea and cyclic pelvic pain was diagnosed with Rokitansky Syndrome, which was characterized by the absence of the uterus and the upper two-thirds of the vagina, normal salpinges and ovaries and a 4 x 3 cm Miillerian remnant containing functioning endometrium located near the left adnexa. With a combined laparoscopic-vaginal operation, the remnant was anastomized with the apex of the retrohymenal fovea. The operation allowed not only the creation of a neovagina but also the recovery of a regular menstrual activity and the theoretical restoration of the reproductive capacity of this patient. An accurate pre- and intra-operative evaluation of patients with Rokitansky Syndrome is necessary to identify those who might benefit from this procedure.
Pedro Acién - One of the best experts on this subject based on the ideXlab platform.
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Clinical pilot study to evaluate the neovaginal PACIENA prosthesis® for vaginoplasty without skin grafts in women with vaginal agenesis
BMC Women's Health, 2019Co-Authors: Pedro Acién, Francisco J. Nohales-alfonso, Maria-luisa Sánchez-ferrer, Miguel Sánchez-lozano, Victoria Navarro-lillo, Maribel AciénAbstract:Background To evaluate the feasibility and clinical outcomes of vaginoplasties using a neovaginal polylactic acid prosthesis made with 3-dimensional (3D) printing technology as an intraneovaginal mould. Methods This was an interventionist, prospective, and multicentre clinical pilot investigation of a sanitary product (PACIENA prosthesis®) aiming to recruit and operate on 8 patients over 6 months with a follow-up period of 6 months. Only six patients with Rokitansky Syndrome and one patient with Morris Syndrome (7 patients in total) were operated on in two university hospitals: “La Fe”, Valencia (H1) and “Arrixaca”, Murcia (H2). Interventions : Extensive surgical dissection of a defined space between the urethra and bladder in the front and of the rectum in the back as well as insertion of the PACIENA prosthesis® covered with Interceed® were performed. After 12 days, the prosthesis was changed to the silicone-covered version for daily application. Results In the 6 patients with Rokitansky Syndrome (86%), the primary endpoint (satisfactory vaginal outcome in terms of appearance, function, and sensation without relevant additional morbidity) was achieved, although only 2 patients (28%) were sexually active at the end of 6 months of follow-up. The patient with Morris Syndrome withdrew from the study after 1 month. Patients without bacterial colonization showed positive Schiller tests at 1 month, and subsequent biopsies showed adequate keratinization and epidermization. Epithelization and iodopositivity were delayed in the patients who developed inflammatory granulomas. Conclusions Good anatomical and functional results can be achieved with the PACIENA prosthesis® for vaginoplasties without skin grafts. However, adequate patient selection and education, good surgical techniques and haemostasis, postoperative support, and prevention of bacterial colonization are important. Trial registration This clinical study was approved by the Ethical Clinical Investigation Committee of San Juan University Hospital on September 27, 2016, to be conducted in the participating centres; it was authorized by the Spanish Agency of Medicines and Health Products (AEMPS) on April 24, 2017 (exp. no. 585/16/EC), to be carried out in that hospitals.
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On a New Neovaginal Prosthesis of PLA (Polylactic Acid)
American Journal of Pharmacology and Pharmacotherapeutics, 2016Co-Authors: Pedro AciénAbstract:Vaginal dilation is proposed as the first choice treatment for patients with vaginal aplasia, but many patients with MRKH or Rokitansky Syndrome (and cases with CAIS) may require a vaginoplasty. It is also necessary to make vaginoplasty in patients with vaginal or cervico-vaginal atresia with a functional uterus before performing an utero-neovaginal anastomosis, also in trans-sexual men.
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The presentation and management of complex female genital malformations
Human Reproduction Update, 2015Co-Authors: Pedro Acién, Maribel AciénAbstract:BACKGROUND: Common uterine anomalies are important owing to their impact on fertility, and complex mesonephric anomalies and certain Mullerian malformations are particularly important because they cause serious clinical symptoms and affect woman's quality of life, in addition to creating fertility problems. In these cases of complex female genital tract malformations, a correct diagnosis is essential to avoid inappropriate and/or unnecessary surgery. Therefore, acquiring and applying the appropriate embryological knowledge, management and therapy is a challenge for gynaecologists. Here, we considered complex malformations to be obstructive anomalies and/or those associated with cloacal and urogenital sinus anomalies, urinary and/or extragenital anomalies, or other clinical implications or symptoms creating a difficult differential diagnosis. METHODS: A diligent and comprehensive search of PubMed and Scopus was performed for all studies published from 1 January 2011 to 15 April 2015 (then updated up to September 2015) using the following search terms: 'management' in combination with either 'female genital malformations' or 'female genital tract anomalies' or 'Mullerian anomalies'. The MeSH terms 'renal agenesis', 'hydrocolpos', 'obstructed hemivagina' 'cervicovaginal agenesis or atresia', 'vaginal agenesis or atresia', 'Herlyn-Werner-Wunderlich Syndrome', 'uterine duplication' and 'cloacal anomalies' were also used to compile a list of all publications containing these terms since 2011. The basic embryological considerations for understanding female genitourinary malformations were also revealed. Based on our experience and the updated literature review, we studied the definition and classification of the complex malformations, and we analysed the clinical presentation and different therapeutic strategies for each anomaly, including the embryological and clinical classification of female genitourinary malformations. RESULTS: From 755 search retrieved references, 230 articles were analysed and 120 studied in detail. They were added to those included in a previous systematic review. Here, we report the clinical presentation and management of: agenesis or hypoplasia of one urogenital ridge; unilateral renal agenesis and ipsilateral blind or obstructed hemivagina or unilateral cervicovaginal agenesis; cavitated and non-communicating uterine horns and Mullerian atresias or agenesis, including Rokitansky Syndrome; anomalies of the cloaca and urogenital sinus, including congenital vagino-vesical fistulas and cloacal anomalies; malformative combinations and other complex malformations. The clinical symptoms and therapeutic strategies for each complex genitourinary malformation are discussed. In general, surgical techniques to correct genital malformations depend on the type of anomaly, its complexity, the patient's symptoms and the correct embryological interpretation of the anomaly. Most anomalies can typically be resolved vaginally or by hysteroscopy, but laparoscopy or laparotomy is often required as well. We also include additional discussion of the catalogue and classification systems for female genital malformations, the systematic association between renal agenesis and ipsilateral genital malformation, and accessory and cavitated uterine masses. CONCLUSIONS: Knowledge of the correct genitourinary embryology is essential for the understanding, study, diagnosis and subsequent treatment of genital malformations, especially complex ones and those that lead to gynaecological and reproductive problems, particularly in young patients. Some anomalies may require complex surgery involving multiple specialties, and patients should therefore be referred to centres that have experience in treating complex genital malformations.
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the female gubernaculum role in the embryology and development of the genital tract and in the possible genesis of malformations
European Journal of Obstetrics & Gynecology and Reproductive Biology, 2011Co-Authors: Pedro Acién, Francisco Sanchez Del Campo, Mariajose Mayol, Maribel AciénAbstract:Abstract The female gubernaculum is an embryonic structure that gives rise to the uterine round ligament and seems to be important in Mullerian development. In the absence of androgens and anti-Mullerian hormone, the paramesonephric or Mullerian ducts complete their invagination and development, interfering with the connection of the tissue column that begins at the inguinal cone (the gubernaculum) and targets the mesonephric duct and caudal ligament of the gonad. The gubernaculum then grows over the Mullerian ducts, incorporating its muscular fibres. Outside and above this point, the Mullerian ducts give rise to the Fallopian tubes, whereas medially to the point of insertion of the gubernaculum, the Mullerian ducts develop into the normal uterus, the adequate formation of which is also induced by the mesonephric ducts. Diverse human anatomical and physiological characteristics such as the simplex uterus, as well as pathological conditions and certain female genital malformations, could be related to gubernaculum dysfunction. The main conclusions in this article are: (1) The female gubernaculum is the origin of the uterine round ligament but probably not of the uteroovarian ligament. Gubernacula are composed of muscular fibres that probably derive from the abdominal wall and that, when fixed and fused with the Mullerian ducts, allow or induce, together with the mesonephric ducts, the adequate development and formation of the uterus. (2) The female gubernaculum seems to be responsible for many of the specific human characteristics of Mullerian development, including the uterus simplex, the anteflexion and low intra-abdominal position of the uterus, and the disposition of uterine muscular fibres. (3) The female gubernaculum seems to be related to pathologies arising from the round ligaments and inguinal hernia. Likewise, certain uterine malformations (e.g., didelphys uterus, Rokitansky Syndrome) and accessory and cavitated uterine masses might be related to gubernaculum dysfunction.
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Complex malformations of the female genital tract. New types and revision of classification
Human Reproduction, 2004Co-Authors: Pedro Acién, Maribel Acién, Marisa Sánchez-ferrerAbstract:BACKGROUND: Complex malformations of the female genital tract are often incorrectly identified, treated and reported, probably due to not considering the malformation as a cause of the clinical symptoms and neither the embryological origin of the different elements of the genitourinary tract. METHODS: Complex malformations are studied and classified, and new types are presented. The new types of complex malformations presented are: (i) Cases of unilateral vaginal or cervico-vaginal atresia with renal agenesis and uterine duplication, with or without communication between hemiuteri; (ii) the unilateral Rokitansky Syndrome; and (iii) the combination in the same patient of unilateral Rokitansky Syndrome (Mullerian defect) on one side and blind vagina and ipsilateral renal agenesis Syndrome (Wolffian defect) on the other side. RESULTS: A revised version of the clinical and embryological classification of genital malformations is presented and an associated diagram points out the origin of these malformations. CONCLUSIONS: These genital malformative anomalies reaffirm our hypothesis about the embryology of the human vagina as deriving from the Wolffian ducts and the Mullerian tubercle; and they show that gynecologists should be aware of the related symptoms and the embryology of the female genital tract in order to achieve a better comprehension of the malformations for their right correction or therapeutic approach.
Maribel Acién - One of the best experts on this subject based on the ideXlab platform.
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Clinical pilot study to evaluate the neovaginal PACIENA prosthesis® for vaginoplasty without skin grafts in women with vaginal agenesis
BMC Women's Health, 2019Co-Authors: Pedro Acién, Francisco J. Nohales-alfonso, Maria-luisa Sánchez-ferrer, Miguel Sánchez-lozano, Victoria Navarro-lillo, Maribel AciénAbstract:Background To evaluate the feasibility and clinical outcomes of vaginoplasties using a neovaginal polylactic acid prosthesis made with 3-dimensional (3D) printing technology as an intraneovaginal mould. Methods This was an interventionist, prospective, and multicentre clinical pilot investigation of a sanitary product (PACIENA prosthesis®) aiming to recruit and operate on 8 patients over 6 months with a follow-up period of 6 months. Only six patients with Rokitansky Syndrome and one patient with Morris Syndrome (7 patients in total) were operated on in two university hospitals: “La Fe”, Valencia (H1) and “Arrixaca”, Murcia (H2). Interventions : Extensive surgical dissection of a defined space between the urethra and bladder in the front and of the rectum in the back as well as insertion of the PACIENA prosthesis® covered with Interceed® were performed. After 12 days, the prosthesis was changed to the silicone-covered version for daily application. Results In the 6 patients with Rokitansky Syndrome (86%), the primary endpoint (satisfactory vaginal outcome in terms of appearance, function, and sensation without relevant additional morbidity) was achieved, although only 2 patients (28%) were sexually active at the end of 6 months of follow-up. The patient with Morris Syndrome withdrew from the study after 1 month. Patients without bacterial colonization showed positive Schiller tests at 1 month, and subsequent biopsies showed adequate keratinization and epidermization. Epithelization and iodopositivity were delayed in the patients who developed inflammatory granulomas. Conclusions Good anatomical and functional results can be achieved with the PACIENA prosthesis® for vaginoplasties without skin grafts. However, adequate patient selection and education, good surgical techniques and haemostasis, postoperative support, and prevention of bacterial colonization are important. Trial registration This clinical study was approved by the Ethical Clinical Investigation Committee of San Juan University Hospital on September 27, 2016, to be conducted in the participating centres; it was authorized by the Spanish Agency of Medicines and Health Products (AEMPS) on April 24, 2017 (exp. no. 585/16/EC), to be carried out in that hospitals.
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The presentation and management of complex female genital malformations
Human Reproduction Update, 2015Co-Authors: Pedro Acién, Maribel AciénAbstract:BACKGROUND: Common uterine anomalies are important owing to their impact on fertility, and complex mesonephric anomalies and certain Mullerian malformations are particularly important because they cause serious clinical symptoms and affect woman's quality of life, in addition to creating fertility problems. In these cases of complex female genital tract malformations, a correct diagnosis is essential to avoid inappropriate and/or unnecessary surgery. Therefore, acquiring and applying the appropriate embryological knowledge, management and therapy is a challenge for gynaecologists. Here, we considered complex malformations to be obstructive anomalies and/or those associated with cloacal and urogenital sinus anomalies, urinary and/or extragenital anomalies, or other clinical implications or symptoms creating a difficult differential diagnosis. METHODS: A diligent and comprehensive search of PubMed and Scopus was performed for all studies published from 1 January 2011 to 15 April 2015 (then updated up to September 2015) using the following search terms: 'management' in combination with either 'female genital malformations' or 'female genital tract anomalies' or 'Mullerian anomalies'. The MeSH terms 'renal agenesis', 'hydrocolpos', 'obstructed hemivagina' 'cervicovaginal agenesis or atresia', 'vaginal agenesis or atresia', 'Herlyn-Werner-Wunderlich Syndrome', 'uterine duplication' and 'cloacal anomalies' were also used to compile a list of all publications containing these terms since 2011. The basic embryological considerations for understanding female genitourinary malformations were also revealed. Based on our experience and the updated literature review, we studied the definition and classification of the complex malformations, and we analysed the clinical presentation and different therapeutic strategies for each anomaly, including the embryological and clinical classification of female genitourinary malformations. RESULTS: From 755 search retrieved references, 230 articles were analysed and 120 studied in detail. They were added to those included in a previous systematic review. Here, we report the clinical presentation and management of: agenesis or hypoplasia of one urogenital ridge; unilateral renal agenesis and ipsilateral blind or obstructed hemivagina or unilateral cervicovaginal agenesis; cavitated and non-communicating uterine horns and Mullerian atresias or agenesis, including Rokitansky Syndrome; anomalies of the cloaca and urogenital sinus, including congenital vagino-vesical fistulas and cloacal anomalies; malformative combinations and other complex malformations. The clinical symptoms and therapeutic strategies for each complex genitourinary malformation are discussed. In general, surgical techniques to correct genital malformations depend on the type of anomaly, its complexity, the patient's symptoms and the correct embryological interpretation of the anomaly. Most anomalies can typically be resolved vaginally or by hysteroscopy, but laparoscopy or laparotomy is often required as well. We also include additional discussion of the catalogue and classification systems for female genital malformations, the systematic association between renal agenesis and ipsilateral genital malformation, and accessory and cavitated uterine masses. CONCLUSIONS: Knowledge of the correct genitourinary embryology is essential for the understanding, study, diagnosis and subsequent treatment of genital malformations, especially complex ones and those that lead to gynaecological and reproductive problems, particularly in young patients. Some anomalies may require complex surgery involving multiple specialties, and patients should therefore be referred to centres that have experience in treating complex genital malformations.
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the female gubernaculum role in the embryology and development of the genital tract and in the possible genesis of malformations
European Journal of Obstetrics & Gynecology and Reproductive Biology, 2011Co-Authors: Pedro Acién, Francisco Sanchez Del Campo, Mariajose Mayol, Maribel AciénAbstract:Abstract The female gubernaculum is an embryonic structure that gives rise to the uterine round ligament and seems to be important in Mullerian development. In the absence of androgens and anti-Mullerian hormone, the paramesonephric or Mullerian ducts complete their invagination and development, interfering with the connection of the tissue column that begins at the inguinal cone (the gubernaculum) and targets the mesonephric duct and caudal ligament of the gonad. The gubernaculum then grows over the Mullerian ducts, incorporating its muscular fibres. Outside and above this point, the Mullerian ducts give rise to the Fallopian tubes, whereas medially to the point of insertion of the gubernaculum, the Mullerian ducts develop into the normal uterus, the adequate formation of which is also induced by the mesonephric ducts. Diverse human anatomical and physiological characteristics such as the simplex uterus, as well as pathological conditions and certain female genital malformations, could be related to gubernaculum dysfunction. The main conclusions in this article are: (1) The female gubernaculum is the origin of the uterine round ligament but probably not of the uteroovarian ligament. Gubernacula are composed of muscular fibres that probably derive from the abdominal wall and that, when fixed and fused with the Mullerian ducts, allow or induce, together with the mesonephric ducts, the adequate development and formation of the uterus. (2) The female gubernaculum seems to be responsible for many of the specific human characteristics of Mullerian development, including the uterus simplex, the anteflexion and low intra-abdominal position of the uterus, and the disposition of uterine muscular fibres. (3) The female gubernaculum seems to be related to pathologies arising from the round ligaments and inguinal hernia. Likewise, certain uterine malformations (e.g., didelphys uterus, Rokitansky Syndrome) and accessory and cavitated uterine masses might be related to gubernaculum dysfunction.
-
Complex malformations of the female genital tract. New types and revision of classification
Human Reproduction, 2004Co-Authors: Pedro Acién, Maribel Acién, Marisa Sánchez-ferrerAbstract:BACKGROUND: Complex malformations of the female genital tract are often incorrectly identified, treated and reported, probably due to not considering the malformation as a cause of the clinical symptoms and neither the embryological origin of the different elements of the genitourinary tract. METHODS: Complex malformations are studied and classified, and new types are presented. The new types of complex malformations presented are: (i) Cases of unilateral vaginal or cervico-vaginal atresia with renal agenesis and uterine duplication, with or without communication between hemiuteri; (ii) the unilateral Rokitansky Syndrome; and (iii) the combination in the same patient of unilateral Rokitansky Syndrome (Mullerian defect) on one side and blind vagina and ipsilateral renal agenesis Syndrome (Wolffian defect) on the other side. RESULTS: A revised version of the clinical and embryological classification of genital malformations is presented and an associated diagram points out the origin of these malformations. CONCLUSIONS: These genital malformative anomalies reaffirm our hypothesis about the embryology of the human vagina as deriving from the Wolffian ducts and the Mullerian tubercle; and they show that gynecologists should be aware of the related symptoms and the embryology of the female genital tract in order to achieve a better comprehension of the malformations for their right correction or therapeutic approach.
Eleonora Fontana - One of the best experts on this subject based on the ideXlab platform.
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the laparoscopic vecchietti s modified technique in Rokitansky Syndrome anatomic functional and sexual long term results
American Journal of Obstetrics and Gynecology, 2008Co-Authors: Luigi Fedele, Giada Frontino, Elisa Restelli, Stefano Bianchi, Eleonora Fontana, Vincenzina BruniAbstract:Objective The objective of the study was to assess the anatomical and functional long-term follow-up results of the laparoscopic Vecchietti approach for the creation of a neovagina in the Rokitansky Syndrome. Study Design One hundred ten patients underwent clinical follow-up visits at 1, 3, 6, and 12 months after surgery and every 6 months thereafter. The following were performed: evaluation of the quality of sexual intercourse, vaginal and rectal examinations, vaginoscopy, Schiller’s test, and vaginal cytology with microbiologic testing. Functional results were assessed by using Rosen’s Female Sexual Function Index questionnaire, of which the results were analyzed comparing normal age-matched controls. Results Four patients were lost to follow-up. Anatomic and functional success was achieved in 104 of 106 (98%) and 103 of 106 (97%) patients, respectively. Female Sexual Function Index scores were comparable with those of controls. Conclusion Vecchietti’s technique is simple, safe, and effective and allows normal and satisfying sexual intercourse, comparable with that of normal controls.
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laparoscopic findings and pelvic anatomy in mayer Rokitansky kuster hauser Syndrome
Obstetrics & Gynecology, 2007Co-Authors: Luigi Fedele, Giada Frontino, Nevio Ciappina, Stefano Bianchi, Eleonora Fontana, Franco BorrutoAbstract: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, accordi
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laparoscopic creation of a neovagina and recovery of menstrual function in a patient with Rokitansky Syndrome a case report
Human Reproduction, 2006Co-Authors: Luigi Fedele, Stefano Bianchi, Nicola Berlanda, Alessandro Bulfoni, Eleonora FontanaAbstract:A 16-year-old woman experiencing primary amenorrhoea and cyclic pelvic pain was diagnosed with Rokitansky Syndrome, which was characterized by the absence of the uterus and the upper two-thirds of the vagina, normal salpinges and ovaries and a 4 x 3 cm Miillerian remnant containing functioning endometrium located near the left adnexa. With a combined laparoscopic-vaginal operation, the remnant was anastomized with the apex of the retrohymenal fovea. The operation allowed not only the creation of a neovagina but also the recovery of a regular menstrual activity and the theoretical restoration of the reproductive capacity of this patient. An accurate pre- and intra-operative evaluation of patients with Rokitansky Syndrome is necessary to identify those who might benefit from this procedure.
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neovaginal mucosa after vecchietti s laparoscopic operation for Rokitansky Syndrome structural and ultrastructural study
American Journal of Obstetrics and Gynecology, 2006Co-Authors: Luigi Fedele, Ricciarda Raffaelli, Stefano Bianchi, Nicola Berlanda, Alessandro Bulfoni, Eleonora Fontana, Paola BraidottiAbstract:Objective This study was undertaken to evaluate structural and ultrastructural characteristics of the mucosa of neovaginae created by Vecchietti's laparoscopic operation for Rokitansky Syndrome. Study design Vaginoscopy and Schiller test were performed 3, 6, and 12 months after the operation in 106 patients. A biopsy specimen of the neovagina obtained 12 to 18 months after surgery in 19 patients was examined by light, scanning electron, and transmission electron microscopy. Results At vaginoscopy, the neovaginal mucosa appeared smooth, lacking the folds that characterize the normal vagina; 12 months after the operation, an iodium-positive epithelium was present in all neovaginae. Mild ultrastructural modifications, as compared with normal vaginal mucosa, were reduced maturation, inflammatory infiltration, and tendency to superficial desquamation. Conclusion At a 12-month follow-up, the mucosa of neovaginae created by the Vecchietti technique is comparable to the normal vaginal mucosa, with mild structural and ultrastructural modifications that we believe might be due to reduced vascularization.
