Three-dimensional Printer Molds for Vaginal Agenesis: An Individualized Approach as Conservative Treatment / Moldes de impressão tridimensionais para agenesia vaginal: Uma abordagem individualizada como tratamento conservador

Abstract Objective The aim of this study was to evaluate the use of vaginal molds, made with three-dimensional (3D) printing, for conservative treatment through vaginal dilation in patients with vaginal agenesis (VA). Methods A total of 16 patients with a diagnosis of VA (Mayer-Rokitansky-Küster-Hauser syndrome, total androgen insensitivity syndrome, and cervicovaginal agenesis) from the Federal University of São Paulo were selected. Device production was performed in a 3D printer, and the polymeric filament of the lactic polyacid (PLA) was used as raw material. A personalized treatment was proposed and developed for each patient. Results There were 14 patients who reached a final vaginal length of 6 cm or more. The initial total vaginal length (TVL) mean (SD) was 1.81(1.05) and the final TVL mean (SD) was 6.37 (0.94); the difference, analyzed as 95% confidence interval (95% CI) was 4.56 (5.27-3.84) and the effect size (95% CI) was 4.58 (2.88-6.28). Conclusion The 3D printing molds for vaginal dilation were successful in 87.5% of the patients. They did not present any major adverse effects and offered an economical, accessible, and reproducible strategy for the treatment of VA.

Resumo Objetivo O objetivo deste estudo foi avaliar o uso de moldes dilatadores vaginais, confeccionados com impressão tridimensional (3D), para tratamento conservador através da dilatação vaginal em pacientes com agenesia vaginal (AV). Métodos Foram selecionadas 16 pacientes com diagnóstico de AV (síndrome de Mayer-Rokitansky-Küster-Hauser, síndrome de insensibilidade androgênica total e agenesia cervicovaginal), da Universidade Federal de São Paulo. A produção dos dispositivos foi realizada em uma impressora 3D e, como matéria-prima, foi utilizado o filamento polimérico do poliácido lático (PLA). Um tratamento personalizado foi proposto e desenvolvido para cada paciente. Resultados Quatorze pacientes atingiram um comprimento vaginal final (CVF) de 6 cm ou mais. A média inicial do CVF (DP) foi de 1,81 (1,05) e a média final do CVF (DP) 6,37 (0,94); a diferença (IC 95%) foi de 4,56 (5,27-3,84) e o tamanho do efeito (IC 95%) foi de 4,58 (2,88-6,28). Conclusão Os moldes de impressão 3D para dilatação vaginal obtiveram sucesso em 87,5% das pacientes. Como impacto secundário, não apresentaram efeitos adversos importantes e ofereceram uma estratégia econômica, acessível e reprodutível para o tratamento da AV.

Mayer-Rokitansky-Kuster-Hauser syndrome – An unusual case of primary amenorrhea

The onset of menstruation is a significant milestone of sexual maturation in a girl child. Although there are numerous causes of primary amenorrhea, Mayer-Rokitansky-Küster-Hauser syndrome, also known as Müllerian aplasia, is one of the extremely rare causes of primary amenorrhea which is usually picked up by suitable imaging techniques like magnetic resonance imaging. Affected females have a normal female hormonal function and a normal karyotype but non-functional vagina and uterus which make it an interesting entity. Although specific treatment is not known for this entity except for vaginoplasty and creation of a neovagina for sexual gratification, the clinical and diagnostic workup of these patients has evolved in recent years and infertility treatment and child-bearing through in vitrofertilization have become a part of the long-term treatment plan for these females.

Neovagina creation using sigmoid colon in vaginal agenesis: a case report and review of literature

Mullerian duct anomalies are rare and can present with abnormalities in upper vagina, cervix, uterus, and fallopian tubes. The exact incidence of cervicovaginal agenesis is unknown and there are very few cases recorded in the literature. Authors report a 12-year-old girl referred from gynaecologist with chronic cyclical abdominal pain with underdeveloped, geniatilia and developed secondary sexual characters. Radiological investigations showed hypoplastic cervix with hematometra and left hemato salpinx. A definitive repair with creation of a neovagina using a sigmoid colon segment was performed in a single stage. Many techniques are described for reconstruction of cervicovaginal canal. Use of colon in creation of a neovagina is described by authors. Here we report a case of complete vaginal agenesis presenting with Hematometra-Hematosalpinx where a neovagina was created using a segment of sigmoid colon. Single staged surgery for neovagina creation using segment of sigmoid colon offers a promising and safe alternative for cervicovaginal agenesis.

Mayer-Rokitansky-Küster-Hauser syndrome

@#Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome is a rare disease found in 1 :4,000-5,000 live female births. It presents with vaginal and uterine agenesis in females. Ultrasound of the pelvis is the initial imaging of choice. Pelvic Magnetic Resonance Imaging (MRI) is the gold standard to confirm the presence of a rudimentary uterus. Surgical and nonsurgical options to create a neovagina may be offered to the patient. Counselling of patients is necessary. This report presents a case of a 1 5-year old phenotypic female with cyclic abdominal pain subsequently noted with absent vaginal canal. Ultrasound and MRI of the pelvis showed the absence of a uterus and upper vagina with intact ovaries. Karyotyping showed 46, XX, confirming that the patient is a female. Analgesics were prescribed for the abdominal pain. Regular counselling was provided by Adolescent Medicine.

How is Vaginal Function in Patients with A History of Sigmoid Vaginoplasty Surgery?

@#Introduction: Sigmoid colon vaginoplasty is a surgical procedure for the management of vaginal agenesis cases. This technique produces a neovagina that can function as a menstrual blood outlet as well as on penetration during sexual activity. Methods: A retrospective case study of vaginal agenesis, where sigmoid colon vaginoplasty surgery was performed at Dr. Sardjito Hospital Yogyakarta Indonesia in 2016-2018. The following post-surgical evaluation were assessed by observing the vaginal function to drain menstrual blood out, as well as function on penetration during sexual activity. Results: Sixteen cases of vaginal agenesis surgically performed were reported. Identification of the reproductive organs was carried out, in which 3 patients were without uterus, and 13 patients had menstrual uterine function. The length of sigmoid colon graft was around 15-20 cm. There were no complications during surgery. Post-surgical evaluation of 5 cases who have been married, all have been able to penetrate in sexual activity without meaningful complaints. All cases that had a uterus underwent regular menstrual cycles, with no dysmenorrhea. It can be said as a whole that this surgical procedure can be accepted by the patients, because it produced good vaginal function. Conclusions: Sigmoid colon vaginoplasty was a surgical procedure for congenital vaginal agenesis with satisfactory results. There were no complaints of dyspareunia or post-surgical dysmenorrhoea, resulting in a good cosmetic appearance of the external genitalia, and relatively mild complications.

Reporte de serie de casos: neovagina en pacientes con Síndrome Mayer-Rokitansky-Kuster-Hauser / Case series report: neovagina in patients with Mayer-Rokitansky-Kuster-Hauser Syndrome

RESUMEN INTRODUCCIÓN: La ausencia congénita de vagina es una condición poco común, algunas causas son el síndrome de Mayer-Rokitansky-Kuster-Hauser y la insensibilidad periférica a los andrógenos. Múltiples técnicas quirúrgicas y no quirúrgicas se han descrito para el manejo de esta condición, siendo el objetivo principal la creación de un canal vaginal de diámetro y longitud adecuada que permitan restaurar la función coital. El objetivo de este estudio es detallar la experiencia del procedimiento de neovagina con la técnica quirúrgica de McIndoe en pacientes con Mayer-Rokitansky-Kuster-Hauser realizados en la Unidad de Uroginecología de la Clínica Universitaria Bolivariana. METODOLOGÍA: Reporte de 5 casos de pacientes con agenesia de vagina secundarios al síndrome de Mayer-Rokitansky-Kuster-Hauser, a las cuales se les realizó neovagina con la técnica de McIndoe con algunas modificaciones en el molde para el implante de piel. RESULTADOS: Se incluyeron cinco pacientes con diagnóstico de Mayer-Rokitansky-Kuster-Hauser, todas tenían desarrollo de características sexuales secundarias, perfil hormonal normal, y un cariotipo XX. Se utilizó la técnica quirúrgica de McIndoe para la realización de la neovagina sin complicaciones intraoperatorias asociadas y con adecuada evolución posoperatoria, con una longitud vaginal entre 7-9 cm y 3 pacientes con vida sexual activa. El tiempo de estancia hospitalaria fue 7 a 9 días. CONCLUSIÓN: La técnica quirúrgica de McIndoe es una opción para restaurar la función sexual en mujeres con agenesia vaginal con resultados favorables. El tiempo para decidir su realización es electivo, sin embargo, se debe contar con madurez física y emocional para ser llevado a cabo. Las pacientes de nuestro reporte tenían una edad promedio de 18 años.

SUMMARY INTRODUCTION: The congenital absence of the vagina is an uncommon condition, some causes are the Mayer-Rokitansky-Küster-Hauser syndrome and peripheral insensitivity to androgens. Multiple surgical and non-surgical techniques have been described for the management of this condition, being the main objective the creation of a vaginal canal of adequate diameter and length to restore coital function. The objective of this study is to detail the experience of the neovagina procedure with the McIndoe surgical technique performed in patients with Mayer-Rokitansky-Küster-Hauser syndrome at the Clinica Universitaria Bolivariana. METHODOLOGY: Report of five cases of patients with vaginal agenesis secondary to the Mayer-Rokitansky-Kuster-Hauser syndrome, who underwent neovagina with the McIndoe technique and some modifications in the mold for the skin implant. RESULTS: Five patients with diagnosis of Mayer-Rokitansky-Kuster-Hauser were included, all had development of secondary sexual characteristics, normal hormonal profile, and a XX karyotype. The McIndoe surgical technique was used to perform the neovagina without associated intraoperative complications and with adequate postoperative evolution, with a vaginal length between 7-9 cm and three patients with active sexual life. The length of hospital stay was 7 to 9 days. CONCLUSION: The McIndoe surgical technique is an option to restore sexual function in women with vaginal agenesis with favorable results. The time to decide its realization is elective, however, they must have the physical and emotional maturity to be carried out. The patients in our report have an average age of 18 years.

A second glance on Cervicovaginal Agenesis

@#Mullerian anomalies arise from the failure in the development of Mullerian ducts and their associated structures during organogenesis which confers adverse impact in fertility and reproductive health. Presented is a rare case of a 15 year old nulligravid, who presented with a chief complaint of severe cyclic hypogastric pain associated with primary amenorrhea. Complete clinical history, physical examination and sonographic findings pointed to a diagnosis of cervical hypoplasia associated with functioning uterine corpus and an absent vagina. Patient underwent total abdominal hysterectomy with left salpingectomy and bilateral oophorocystectomy, for hematometra, bilateral endometriotic cysts, and hematosalpinx. This case report discusses the management of cervicovaginal agenesis through a multidisciplinary approach by a team composed of an obstetrician-gynecologist, reproductive endocrinologist, pediatrician, and pediatric surgeon for proper evaluation, diagnosis, and management of this case.

Mayer-Rokitansky-Küster-Hauser Syndrome with Imperforate Anus: A Rare Association and an Innovative Surgical Management.

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is one of the rare disorder of Mullerian agenesis leading to non-development of uterus and vagina. Its association with anorectal malformation is rare. We report a case of MRKH syndrome with recto-vestibular in a female child. The child had undergone a sigmoid loop colostomy in the neonatal period. On clinical examination of the perineum, a fistula was present in the vestibule just below the urethral opening, but no vaginal canal was found. It was managed with an innovative surgical technique, preserving the fistula and lower rectum to function as vagina.

Cervical and vaginal agenesis report.

Cervical agenesis is a very rare condition often associated with atresia of vagina. Clinical diagnosis is usually difficult before surgery. Transverse vaginal septum or vaginal agenesis is also a rare condition that results from incomplete fusion between vaginal components of the mullerian ducts and urogenital sinus. Here we presented case of 16 years old girl who presented with primary amenorrhea and cyclical lower abdominal pain for 6 months. Abdomino-pelvic scan showed hematometra with bilateral hematosalpinx with mild free fluid in pouch of Douglas with collapsed vagina and cervix. MRI pelvis confirmed USG findings giving differential diagnosis of high vaginal septum or cervical agenesis. She was operated for cervical recanalization and vaginosplasty.

Unilateral Functional Uterine Horn with Non Functioning Rudimentary Horn and Cervico-Vaginal Agenesis: Case Report.

Developmental anomalies involving Mullerian ducts are one of the most fascinating disorders in Gynaecology. The incidence rates vary widely and have been described between 0.1-3.5% in the general population. We report a case of a fifteen year old girl who presented with primary amenorrhea and lower abdomen pain, with history of instrumentation about two months back. She was found to have abdominal lump of sixteen weeks size uterus. On examination vagina was found to be represented as a small blind pouch measuring 2-3cms in length. A rectovaginal fistula (2x2 cms) was also observed. Ultrasonography of abdomen revealed bulky uterus (size 11.2x6 cm) with 150 millilitre of collection. Adiagnosis of hematometra with iatrogenic fistula was made. Vaginal drainage of hematometra was done which was followed by laparotomy. Peroperatively she was found to have a left side unicornuate uterus with right side small rudimentary horn. Left fallopian tube and ovary showed dense adhesions and multiple endometriotic implants. Both cervix and vagina were absent. Total abdominal hysterectomy was done and rectovaginal fistula repaired. The present case is reported due to its rarity as it involved both mullerian agenesis with cervical and vaginal agenesis along with disorder of lateral fusion. This is an asymmetric type of mullerian duct development in which arrest has occurred in different stages of development on two sides.

Reconstruction of Congenital Absence of Vagina using Vulvoperineal Fasciocutaneous Flap: A Case Report

PURPOSE: Congenital absence of the vagina is a rare case. It occurs as a result of Mullerian duct aplasia or complete androgen insensitivity syndrome. The reconstructive modality includes skin graft, use of intestine and various methods of flap. We report a patient who underwent vulvoperineal fasciocutaneous flap to reconstruct congenital absence of the vagina, while the external genitalia and ovaries are normal. METHODS: A 26-year-old woman presented with vaginal agenesis. Under general anesthesia, a U-shaped incision was made between the urethral meatus and the anus. The new vaginal pocket was created up to the level of the peritoneal reflection between the urinary structures and the rectum. Next, the vulvoperineal fasciocutaneous flaps were designed in a rectangular fashion. Flap elevation was begun at the lateral margin which the adductor longus fascia was incised and elevated, and the superficial perineal neurovascular pedicle was invested by the fascial layer. The medial border was then elevated. A subcutaneous tunnel was created beneath the inferior of the labia to rotate the flaps. The left vulvoperineal flap was rotated counterclockwise and the right was rotated clockwise. The neovaginal pouch was formed by approximating the medial and lateral borders. The tubed neovagina was then transposed into the cavity. RESULTS: In 3 weeks, the vaginal canal remained supple After 6 weeks, the physical examination showed normal-appearing labia majora and perineum with an adequate vaginal depth. A year after the operation, the patient had a 7 cm vagina of sufficient width with no evidence of contractures nor fibrous scar formation. The patient was sexually active without difficulty. CONCLUSION: Although many methods were described for reconstruction of vaginal absence, there is not a method yet to be approved as a perfect solution. We used the vulvoperineal fasciocutaneous flap to reconstruct a neovagina. This method had a following merits: a single-stage procedure, excellent flap reliability, the potential for normal function, minimal donor site morbidity and no need for subsequent dilatation, stents, or obturators. We thought that this operation has a good anatomic and functional results for reconstruction of the vagina.

Six cases of laparoscopic assisted neovaginoplasty using pelvic peritoneal flap in MRKH syndrome / 대한산부인과학회지

Vaginal agenesis is rare gynecologic condition, and the most common etiology is Mayer-Rokitansky-Kster-Hauser (MRKH) syndrome, characterized by the absence of uterus and vagina and presence of normal ovaries and tubes. In such patients, the evaluation for associated malformations as well as careful non-surgical and surgical approach are essential. The neovaginoplasty is an important issue for these patients in regard of functional and psychological standpoint. There are many options available for creation of neovagina. We report six cases of laparoscopic assisted neovaginoplasty using pelvic peritoneal flap.

Non-surgical Treatment of Vaginal Agenesis Using a Simplified Version of Ingram's Method

Non-surgical vaginal dilation is a safe and effective method for the creation of neovagina in the patient with vaginal agenesis. Compared to surgical methods, non-surgical vaginal dilation has the advantage of low morbidity, the creation of a more physiologic vaginal milieu, and no surgical scarring. To overcome some technical limitations of original Frank's method, in 1981 Ingram proposed a modification of the technique that used dilators of gradually increasing size mounted on a bicycle seat stool. Although several studies have shown satisfactory outcomes using Ingram's method, there are some practical difficulties in making and handling the bicycle seat stool. This article reports a case of a 24-year-old woman with Mayer-Rokitansky-Kster-Hauser syndrome whose vaginal agenesis is successfully treated with a simplified version of Ingram's method. This method uses dilators of gradually increasing size mounted on an ordinary chair instead of a bicycle seat stool. When necessary, the patient may use a fulcrum under the dilator.

Non-surgical Treatment of Vaginal Agenesis Using a Simplified Version of Ingram's Method

Non-surgical vaginal dilation is a safe and effective method for the creation of neovagina in the patient with vaginal agenesis. Compared to surgical methods, non-surgical vaginal dilation has the advantage of low morbidity, the creation of a more physiologic vaginal milieu, and no surgical scarring. To overcome some technical limitations of original Frank's method, in 1981 Ingram proposed a modification of the technique that used dilators of gradually increasing size mounted on a bicycle seat stool. Although several studies have shown satisfactory outcomes using Ingram's method, there are some practical difficulties in making and handling the bicycle seat stool. This article reports a case of a 24-year-old woman with Mayer-Rokitansky-Kster-Hauser syndrome whose vaginal agenesis is successfully treated with a simplified version of Ingram's method. This method uses dilators of gradually increasing size mounted on an ordinary chair instead of a bicycle seat stool. When necessary, the patient may use a fulcrum under the dilator.

A Modified McIndoe Operation for Treatment of Vaginal Agenesis

The reconstructive modalities for vaginal reconstruction include simple dilatation, skin graft, use of intestinal segments and various methods using flaps. However, skin grafting procedure is the most commonly used technique and the McIndoe procedure is a representative technique among skin grafting procedures. McIndoe procedure is easier, faster and has a lower morbidity compared to other techniques. However the conventional McIndoe procedure has several problems such as incomplete vestibule formation, excessive bleeding during dissection, possibility of recto-vaginal or urethro-vaginal fistula formation, late vaginal contracture and discomfort in wearing hard plastic mold for a long time after operation. To solve these problems, the authors modified the conventional McIndoe procedure in several perspectives. The undeveloped vestibule was incised with X-shaped mucosal incision between the urethral opening and posterior margin of the vestibule and deepened by blunt finger dissection to provide a sufficient diameter & length of the neovagina and to minimize bleeding. A sizable medium thickness split skin graft was harvested and wrapped over a roll gauze-filled condom mold. Applying multiple stab incision on the skin grafted condom mold, it was inserted into the prepared neovaginal canal. Distal margin of the skin graft was secured with tips of the mucosal flaps created by X-shaped vestibular incision to prevent accidental extrusion of the skin grafted mold. During last 15 years, we applied this modification to 20 vaginal agenesis patients and investigated results of the 12 patients who could be followed up serially including hematoma formation and skin graft survival rate, size, depth, presence of late contracture, appearance, comfortness, and hygiene of the neovagina. And they were compared with 8 patients of 20 patients who underwent conventional McIndoe procedures. The modified McIndoe procedure revealed lower complication rate, higher patient satisfaction and better functional results.

Two Cases of Mullerian Agenesis Undergone Vaginal Reconstruction by McIndoe Technique / 대한산부인과학회잡지

Vaginal agenesis is uncommon gynecologic condition, and the most common etiology is Mullerian agenesis also called Mayer-Rokitansky-Kster-Hauser (MRKH) syndrome. In such patients, the evaluation for associated malformations as well as careful non-surgical and surgical approach are essential. The neovaginoplasty is an important issue for these patients from a functional and a psychological standpoint. We describe two patients with Mullerian agenesis, who have undergone vaginal reconstruction by McIndoe technique.

Two Cases of Mullerian Agenesis Undergone Vaginal Reconstruction by McIndoe Technique / 대한산부인과학회잡지

Vaginal agenesis is uncommon gynecologic condition, and the most common etiology is Mullerian agenesis also called Mayer-Rokitansky-Kster-Hauser (MRKH) syndrome. In such patients, the evaluation for associated malformations as well as careful non-surgical and surgical approach are essential. The neovaginoplasty is an important issue for these patients from a functional and a psychological standpoint. We describe two patients with Mullerian agenesis, who have undergone vaginal reconstruction by McIndoe technique.

A Case of a M llerian Duct Remnant Leiomyoma Suspected of Ovarian Tumor in a Woman with M llerian Dysgenesis / 대한산부인과학회잡지

In women, leiomyomas are very common diseases and may arise from any organs containing smooth muscle. However, they are extremely rare in M llerian duct remnant. A medline research disclosed only 13 published cases including 3 domestic cases. We report a case of a M llerian duct remnant leiomyoma in a woman of congenital vaginal agenesis with M llerian dysgenesis whose chief complaint was a palpable pelvic mass suspected of an ovarian tumor.

A Case of Mayer-Rokitansky-K ster-Hauser Syndrome combined with Unilateral Renal Agenesis and Pelvic Cystic Mass in Child / 대한비뇨기과학회지

The Mayer-Rokitansky-K ster-Hauser syndrome represents a spectrum of m llerian anomalies, including vaginal agenesis with or without renal anomalies, in genotypically and phenotypically normal female subjects with normal endocrine status. We experienced a case of this anomaly which combined with unilateral renal agenesis and pelvic cystic mass in child.

Six Cases of Congenital Absence of the Vagina Managed by McIndoe Operation / 대한산부인과학회잡지

Mullerian agenesis or dysgenesis, rare developmental anomaly presenting as primary amenorrhea, is characterized by absence of vagina and absence or rudimentary development of the uterus. Anatomic anomalies of the axial skeleton and kindney often accompany this psychologically devatating condition. There are various methods of surgical treatment for congenital absence of the vagina. The six cases reviewed in this paper were all treated with McIndoe technique. All of 6 women experienced satisfactory sexual activity and were satisfied with vaginal depth following vaginal creation using a split thickness skin graft technique. Despite the existence of several alternative methods, the McIndoe technique is a relatively simple and highly successful procedure and hence is still prefered by many surgeons.