Cervical Agenesis Research Articles

A rare case: Neovagina creation using McIndoe technique and total surgical approach in a patient with Mayer-Rokitansky-Küster-Hauser syndrome

This case report presents an 18-year-old female patient diagnosed with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, a rare congenital anomaly causing primary amenorrhea. MRI revealed vaginal agenesis and a left hemiuterus associated with hematosalpinx and cervical agenesis, while both ovaries were normal. The patient underwent successful neovagina creation using the McIndoe technique, involving a split-thickness skin graft from below the umbilicus. Postoperative follow-up showed excellent graft adherence and significant improvement in sexual function and quality of life. This case highlights the efficacy of the McIndoe technique for neovagina creation in MRKH syndrome and underscores the importance of a multidisciplinary approach in diagnosis and treatment, including psychological support.

Concomitant extragenital malformations of female reproductive tract anomalies: analysis of 444 cases in Peking Union Medical College Hospital

Objective: To analyze the incidence and clinical phenotype of the concomitant extragenital malformations in the patients with female reproductive tract anomalies. Methods: A retrospective study was conducted using clinical data of hospitalized patients diagnosed with uterine, cervical, or vaginal malformations from January 2003 to December 2022 in Peking Union Medical College Hospital. The malformations were classified according to American Society for Reproductive Medicine müllerian anomalies classification 2021, and in each type, the incidence and specific manifestations of concomitant extragnital malformations were analyzed. Results: A total of 444 patients were included. The overall incidence of concomitant extragenital malformations was 43.5% (193/444), including urinary system, skeletal system, and other system malformations. Renal malformations on the obstructed side were present in all patients with oblique vaginal septum syndrome (100.0%, 78/78). The total incidence of concomitant extragnital malformations was as high as 8/11 in uterus didelphys, 43.5% (10/23) in unicornuate uterus, 33.6% (79/235) in Mayer-Rokitansky-Küster-Hauser syndrome, 18.8% (6/32) in septate uterus and 18.5% (12/65) in cervical agenesis. Urinary system malformations (30.6%, 136/444) and skeletal system malformations (13.5%, 60/444) were the most common concomitant malformations in all types, in which, unilateral renal agenesis and scoliosis were the most common. Conclusions: Urinary and skeletal system malformations are important features of female reproductive tract anomalies. Urologic ultrasonography and spinal roentgenogram are recommended for all patients with female reproductive tract anomalies.

Complex cervical dysgenesis and concurrent uterine anomalies: a dynamic case presentation and management

Complex cervical dysgenesis and concurrent uterine anomalies: a dynamic case presentation and management

Congenital Absence of Cervix with Atrial Septal Defect In An Adolescent Girl

Background-Cervical agenesis is a rare congenital Mullerian anomaly. Its association with vaginal agenesis is further rare, reported in 39% of cases of cervical agenesis. Case Presentation-Case report of a 15-year-old adolescent girl congenital absence of cervix with atrial septal defect is reported here. Case presented with a history of Primary amenorrhea with cyclical pain in the abdomen for 3-4 months. Investigations were done. The diagnosis was made & was advised for surgical correction. KEYWORDS: agenesis, Mullerian anomaly, Absence of cervix.

Presentation and Management of Uterine Didelphys with Unilateral Cervicovaginal Agenesis/Dysgenesis (CVAD): A Multicenter Case Series

IntroductionThere are several well-described presentations of uterine didelphys (UD): UD without vaginal septum, UD with non-obstructed longitudinal vaginal septum, or UD with duplicated vaginas and an obstructed hemivagina on one side with ipsilateral renal anomaly. Study ObjectiveTo describe another variant of UD and compare the presentation and management across different institutions MethodsThis was a retrospective case series approved by the NASPAG Fellows Research Consortium. Participating institutions obtained IRB approval. Inclusion criteria included a diagnosis of UD and unilateral cervicovaginal agenesis/dysgenesis (CVAD). Descriptive statistics were used. ResultsFive patients met the inclusion criteria, with ages ranging from 13 to 27 years. Presenting symptoms included dysmenorrhea (80%), irregular bleeding (40%), acute onset left lower quadrant pain (20%), and abdominal mass (20%). Three patients had additional known abnormalities, including solitary kidney and solitary adrenal gland. All patients underwent pelvic magnetic resonance imaging. Two cases were only suspicious for unilateral CVAD on imaging and required pathology review postoperatively to confirm diagnosis. Two cases required a 2-staged approach with an initial diagnostic surgery followed by a second definitive procedure. Three patients were noted to have endometriosis intraoperatively. Postoperative follow-up ranged from 2 months to 2 years, with 1 patient reporting chronic pelvic pain. ConclusionDiagnosis on the basis of pelvic imaging can be difficult, as this unique variant may mimic classic obstructed hemivagina with ipsilateral renal anomaly. In patients with UD with unilateral CVAD, standard management is removal of the obstructed uterine horn. This multicenter series stresses awareness about the clinical presentation, distinguishes cases of cervical agenesis from dysgenesis, and reviews approaches to management.

Congenital Cervical Agenesis- A Rare Case Report

Congenital cervical agenesis is a very uncommon condition. It usually coexists with congenital vaginal agenesis. Years back, many centers considered hysterectomy as the only treatment option. With the advent of newer techniques in reconstruction, most of these cases have achieved normal menstruation and few have had normal pregnancy as well. Herewith, we describe our experience in the management of primary amenorrhea in a 16 years old girl along with highlighting the unconventional uterovaginal anastomosis with V-M technique and thereby, we were able to achieve a successful outcome. Keywords: Cervical agenesis/dysgenesis, congenital malformations, MRKH, obstructive Mullerian anomalies, vaginal agenesis.

3. Robotic Hysterectomy for Cervical Agenesis

3. Robotic Hysterectomy for Cervical Agenesis

MRI Imaging in Congenital Mullerian Duct Abnormalities Using New Classification from the American Society of Reproductive Medicine (ASRM MAC2021)

Infertility is a very important issue. One of the causes of fertility disorders is due to congenital abnormalities in the Mullerian ducts. Congenital abnormalities of the Mullerian ducts are deviations from normal anatomy which include the uterus, fallopian tubes and or the upper two-thirds of the vagina originating from the paramesonephric. Mullerian duct anomalies originate from disturbances that occur in the three phases of uterine development starting from organogenesis, fusion, or septal resorption. MRI is an imaging modality with a fairly high level of accuracy, making it an ideal modality in the diagnosis of congenital Mullerian duct anomaly. With the ASRM MAC2021 classification system, it is easier to assess and analyze anatomical structures to diagnose congenital abnormalities of the Mullerian duct. This research is a retrospective descriptive study conducted at RSUD Dr. Soetomo Surabaya and has ethical license. There were 52 subjects in this study who were obtained from 2018-2021. In this study, the frequency according to the ASRM MAC2021 classification with the mullerian agenesis type was 36 patients (69%), cervical agenesis 4 patients (7.6%), unicornuate uterus 3 patients (5.7%), uterus didelphys 5 patients (9.6%), bicornuate uterus 1 patient (1.9%), septate uterus 1 patient (1.9%), longitudinal vaginal septum 1 patient (1.9%), transverse vaginal septum 1 patient(1.9%) %) and complex anomalies 0 patients (0%) from a total of 52 samples. To sharpen and facilitate the evaluation of congenital abnormalities, segmental descriptions are used in the results of the MRI examination.

Levonorgestrel-Releasing Intrauterine System: A Promising Choice after Uterovaginal Anastomosis in Patients with Cervical Dysgenesis

BackgroundCervical dysgenesis is categorized into cervical fragmentation, cervical fibrous cord, and cervical obstruction. The definitive management for cervical dysgenesis is either uterovaginal anastomosis (UVA) or hysterectomy. ObjectiveTo compare the prevalence of dysmenorrhea, hematometra, and need for dilatation after UVA with and without postprocedural placement of a levonorgestrel intrauterine system (LNG-IUS). MethodsThis was a retrospective cohort study in which 14 patients with cervical dysgenesis were included. Patients had undergone UVA between May 2015 and January 2022 at the Department of Obstetrics and Gynecology of the Cairo University Teaching Hospital. Six patients who had an LNG-IUS inserted after UVA were included in group A, and 8 patients who had undergone UVA without LNG-IUS insertion were included in group B. Transabdominal and/or transvaginal ultrasound was performed monthly for the first 3 months after LNG-IUS insertion in group A and after UVA in group B. Thereafter, the patients were followed up every 6 months. The primary outcomes were dysmenorrhea, hematometra, and need for dilatation of the anastomosis site. ResultsThe number of patients who developed hematometra was significantly lower in group A than in group B (0 [0%] vs 6 [75%], P = .01). The number of patients who required dilatation was significantly lower in group A than in group B (0 [0%] vs 6 [75%], P = .01). There was no significant difference in the incidence of dysmenorrhea between the 2 groups. ConclusionWe recommend offering LNG-IUS after UVA for adolescents who present with cervical dysgenesis. LNG-IUS decreases the recurrence of hematometra and subsequent surgical interventions.

Utero-vaginal anastomosis in cases of cervical malformations: long-term follow-up and fertility challenges

PurposeTo study the long-term results of utero-vaginal anastomosis in cases of cervical malformations.MethodsThis is a retrospective cohort study. Nine patients presented with cryptomenorrhea due to cervical malformations (5 patients with cervical agenesis and vaginal aplasia, 2 patients with cervical agenesis and upper vaginal aplasia, and two patients with cervical dysgenesis in form of cervical obstruction). Five patients had utero-vaginal anastomosis (UVA) with McIndoe vaginoplasty. Four patients had UVA without vaginoplasty. Follow-up was done by transabdominal and/or transvaginal ultrasound monthly for the first 3 months then every 6 months thereafter for a duration that ranged from 15 to 82 months. The main outcome measures are achieving menstruation, dysmenorrhea, pelvic inflammatory disease (PID), needed interventions after primary surgery, infertility, and pregnancy rate.ResultsNine (100%) patients achieved menstruation, one (12%) experienced severe dysmenorrhea, two (22%) had PID, seven (78%) needed dilatation of the anastomosis site, three (33%) needed reoperation, nine (100%) had primary infertility, two (28.5%) achieved clinical pregnancy, and only one (14%) ended by live birth.ConclusionConservative surgery for cervical malformation is a promising choice for relieving the obstructive symptoms. Regular dilatation is recommended. Pregnancy is a remote hope that is hindered by many challenges.

A case of Uterine didelphis with cervical atresia: A Case Report

Congenital uterine malformations are deviations from normal anatomy resulting due to defective fusion of Mullerian ducts or the paramesonephric ducts in the developing embryo. The prevalence of female genital tract anomalies is 4%-7% in general population and up to 8%-10% in women who have recurrent pregnancy loss.(1) The type and degree of anatomical distortion has associated health implications that may include, cyclical abdominal pain, reproductive failure, obstructed menses and inability to engage in sexual intercourse.(2) Uterus didelphys (double uterus) is a developmental abnormality that results from the failure of fusion of the Mullerian ducts leading to separate uterine cavities and 2 cervices 1. It is a rare anomaly accounting for 5% of all uterine anomalies. (3) Cervical agenesis is a rare Mullerian anomaly with an incidence of 1 in 80,000 females. (4) It represents 3% of all uterine anomalies. It is rarely associated with a functioning uterus (4.8%). Cervical agenesis is often associated with vaginal atresia (less than 50%). It is important to classify these anomalies for easy diagnosis and plan appropriate preoperative treatment.

Laparoscopic Uterovaginal Anastomosis for Cervical Agenesia and Upper Vagina

<h3>Study Objective</h3> Cervical agenesis and the upper third of the vagina is a Müllerian malformation, which presents alterations in the patient's quality of life, with primary amenorrhea, dysmenorrhea, for which treatment was initiated for laparoscopic uterovaginal anastomosis. <h3>Design</h3> Report of a case, followed up 6 months after the surgical event. <h3>Setting</h3> This is a female patient undergoing operative laparoscopy, after performing a neovagina. In the laparoscopy operating room, assisted vaginally. <h3>Patients or Participants</h3> A 21-year-old female patient with primary amenorrhea. <h3>Interventions</h3> Uterovaginal anastomosis was performed. <h3>Measurements and Main Results</h3> Post-surgical surveillance was maintained to assess clinical improvement of pelvic pain, she presented menstruation the first three months in follow-up with oral contraceptives based on ethinylestradiol/levonogestrel, and after three months the treatment was suspended, the silastic uterine catheter, and the patient continued to present cyclical menses. <h3>Conclusion</h3> Vaginal-assisted laparoscopic uterovaginal anastomosis is a technique with satisfactory results for patients with C4 and V4 Müllerian malformations, to improve quality of life and reproductive future.

7918 Laparoscopic Repair of Cervico-Vaginal Agenesis in a Previously Failed Attempt of Mcindoe's Vaginoplasty: A Case Report

<h3>Study Objective</h3> A unique Laparoscopic approach for surgical repair of Cervico-vaginal agenesis. <h3>Design</h3> A case report with 6 months follow-up. <h3>Setting</h3> laparoscopy surgery with patient in lithotomy position under anaesthesia. <h3>Patients or Participants</h3> A 23-yrs old female came with primary amenorrhea with lower abdominal pain since 2 years. Her MRI was suggestive of: 1) upper 2/3rd vaginal aplasia with hematoma in the cervical canal 2) b/l hematosalpinx 3) b/l ovarian hemorrhagic cysts. McIndoe's Vaginoplasty was attempted 6months back which had failed. <h3>Interventions</h3> Laparoscopic cervico- vaginal agenesis repair was planned <h3>Measurements and Main Results</h3> The occurrence of cervical agenesis is quite uncommon (1:80 000–1:100 000) and only 39% of these cases simultaneously have vaginal agenesis. Initially, these cases were managed by hysterectomy or abdominal cervicovaginoplasty. Later, a minimally invasive approach came to light with the advent of laparoscopy and surgical expertise. Principles of surgery: 1. Laparoscopic management of Hematosalpinx and hemorrhagic cysts. 2. Dissection of UV fold and pushing the bladder down to expose 3-4 cm length of the vagina. 3. Dissection between cervix and vagina in the right plane. 4. Creating neo-vagina by dissection of the vaginal agenesis. 5. Vertical incision on the cervix and horizontal incision on the vagina Suturing techniques between cervix and vagina and placing a silicon catheter in cervical canal and neo-vagina. <h3>Conclusion</h3> Laparoscopic approach for cervico vaginal agenesis is better than vaginal approach with cutting cervix vertically for patency of cervical canal.

Conservative surgical repair in cervical atresia associated with partial or complete absence of the vagina

To describe the surgical technique of laparoscopically assisted uterovaginal/vestibular anastomosis in patients with cervical atresia associated with partial or complete absence of the vagina. Surgical video article. Local institutional review board approval and written permission from the patients were obtained. Tertiary referral center. The surgical video presents surgical correction in 3 different patients with cervical agenesis. The first patient, aged 14 years, had a normoconformed uterus and total absence of the vagina. The second patient, aged 12 years, demonstrated a left unicornuate uterus and partial absence of the vagina. The third patient, aged 13 years, displayed a right unicornuate uterus and total absence of the vagina. Laparoscopic time and perineal time. During laparoscopy, the entire abdominopelvic cavity was assessed to evaluate the uterine morphology and size to exclude anomalies such as hematometra. The adnexa and adhesions were evaluated and any endometrial flare-ups were treated appropriately. A laparoscopic ultrasound probe was used to evaluate the size and location of the endometrial cavity. In cases with total absence of vaginas, an H-shaped incision in the hymenal dimple allowed a larger area of available tissue for the anastomosis. A tunnel was then created by blunt finger dissection between the bladder and rectum. Simultaneously, the uterus was pushed caudally by an assistant while the operator grasped it from below using an internal probe. A circular myometrial incision at the uterine caudal body allowed to reach the endometrial cavity and open it. The edges of the uterine cavity were then anastomized with the edges of the hymenal incision. In cases with partial absence of vaginas, the creation of the tunnel between the vagina and rectum was not necessary and the open uterus was anastomosed with the margins of the vaginal dome, engraved on the guide of a metal dilator. All patients received broad-spectrum antibiotics (i.e., cephalosporins of the last available generation) on the day before surgery and on the day of surgery. Intraoperative anatomic and ultrasound data, neovaginal length, and recovery of menstrual function 180 days after surgery. The surgical procedure was successful in all cases. No major complications were recorded, and in particular, no bladder or rectal injuries occurred. No stenosis of the neocervix was recorded. The main hospital stay of the patients was 3.5 ± 1.5 days. In each case, the neovagina developed gradually over time after surgery because of the upward traction action exerted by the uterus through its natural ligament apparatus (cardinal ligaments and ovarian vessels). This fact eliminated the requirement for the use of a mold after surgery. At the 15-week follow-up, vaginoscopy was performed, with mucus observed at the site of uterovaginal anastomosis in all cases. None of the patients developed infection after surgery because of the avoidance of molds or pessaries and the natural mucus production. Six months after surgery, the length of the neovagina was >4 cm in all 3 cases. Laparoscopic-assisted uterovaginal/vestibular anastomosis may be considered the treatment of choice for patients with cervical atresia associated with partial or complete absence of the vagina.

Mixed Müllerian and gonadal dysgenesis: A case report

Background: Globally, the incidence of cervical agenesis is estimated at 0.01% and constitutes 3% of all Müllerian anomalies.&#x0D; Case presentation: A 17-year-old nulliparous presented to the gynecology clinic with a transverse vaginal septum and cyclic lower abdominal pain after two failed vaginal surgical procedures to restore genital tract patency. The examination under anesthesia revealed a 3cm short vagina, and a decision was made for vaginoplasty. Magnetic resonance imaging of the pelvis revealed a bulky uterus. Intraoperatively, locating the cervix through the neo-vagina was difficult, and laparotomy was opted for. The left adnexa and cervix were absent, with an atrophic right ovary and a hydrosalpinx on the fallopian tube. Creating a uterovaginal anastomosis was difficult as the uterus was high in the pelvis. Postoperatively, the patient was counseled, and hysterectomy and right oophorectomy were done four months later.&#x0D; Conclusion: Vaginoplasty with total hysterectomy is an alternative management method in failed uterovaginal anastomosis.

Discussion of MRI Findings with Surgical Correlation in Vaginal and Cervical Agenesis: A Short Review of Mullerian Duct Anomalies

Magnetic Resonance Imaging (MRI) is the modality of choice and is considered a highly accurate tool for the evaluation of Müllerian duct anomalies to characterize the uterine and vaginal anomalies [1,2]. In clinical practice, the MRI examination is used by the clinician to reach the diagnosis, for assessing the abnormalities and planning for surgery or future intervention. The information provided by other examinations like pelvic examination, sonography, hysterosalpingography can be confirmed and complemented by MRI. The case described here illustrates the standard MRI findings of vaginal and cervical agenesis with clinical and surgical correlation and revisits the uses of MRI.

Surgical therapy for herlyn-werner-wunderlich (HWW) syndrome cases

Introduction: One of the rare forms of Mullerian congenital ducts is Herlyn-Werner-Wunderlich (HWW) syndrome. HWW syndrome is usually found at puberty with nonspecific symptoms. Pelvic pain can be found both acute and chronic, dysmenorrhoea, as well as hematometra and hematocolpos. Early detection and treatment results in a good prognosis with fertility preservation. Case presentation: Two cases of Herlyn-Werner-Wunderlich (HWW) syndrome surgically performed were reported at Dr. Sardjito Yogyakarta Indonesia Central General Hospital in 2017-2018. Both cases are still children and at the age of puberty. The chief complaint in both cases it was reported to have the same main complaint, abdominal pain, with the condition having never experienced menstruation yet. On further examination it is known that pain is caused due to hematometra and hematosalping that occur in both cases. The first case with hematometra in the right hemiuterus and right hematosalpinx, while second case with hematometra in the uterus didelphys and bilateral hematosalpinx. One case with a normal vagina, and the other case of vaginal agenesis. There was previous history of surgery in both of cases. There are abnormalities in the urinary tract that occur together with these genital abnormalities. Surgical therapy is carried out with the main goal being to drain menstrual blood from hematometra and hematosalpinx. In first case which has a vagina within the normal range (9 cm long) and the inferior area of ​​the right hemiuterus which is bulging toward the vagina, an area can be identified for the drainage incision to drain the entire hematometra and hematosalpinx. In the second case with vaginal agenesis, neovagina which is made by sigmoid colon vaginoplasty surgery can be a way of menstrual blood flow for a lifetime. Conclusion: Clinical manifestations of Herlyn-Werner-Wunderlich (HWW) syndrome cases can be classified into 2 groups, complete and incomplete. Surgical treatment is based on the condition of the uterus and vagina. In the condition of cervical agenesis but with a normal vagina, surgery can be done by making an incision in the drainage of the uterine cervical tissue. If vaginal agenesis is obtained, vaginoplasty surgery can be performed, and one of the options is sigmoid colon vaginoplasty.

Laparoscopic Neocervix Creation in a Woman with Secondary Infertility Following a Radical Trachelectomy for Adenocarcinoma of the Cervix

<h3>Study Objective</h3> Cervical cancer continues to affect young woman of childbearing age even though its incidence is on the decline in Australia. [1] Laparoscopic-assisted vaginal radical trachelectomy and pelvic lymphadenectomy for patients with FIGO stage IA1 with lymphovascular space invasion, stage IA2, and stage IB1 cervical cancer is considered to be an appropriate and fertility-sparring alternative to a radical hysterectomy [2]. Unfortunately, cervical stenosis and agenesis/shortening is a known complication of this procedure causing reduced fertility in these women [3]. We present a case of a 32-year-old woman with secondary infertility due to a radical trachealectomy for stage 1 cervical adenocarcinoma. Her cervical agenesis was treated via a laparoscopic-assisted vaginal neocervix creation. <h3>Design</h3> Case Study. <h3>Setting</h3> Theater. <h3>Patients or Participants</h3> A 32-year-old female presented to our infertility clinic with a background of laparoscopic-assisted vaginal trachelectomy, pelvic lymphadenectomy and concomitant cerclage 6 years prior for stage 1 cervical adenocarcinoma. She remained amenorrhoeic post-surgery, but she was ovulatory based on serology and with follicles noted on ultrasound. A HyCoSy failed due to inability to locate and cannulate the cervix. We offered a hysteroscopic dilatation of the cervical canal with laparoscopic assistance and stitch removal as a means to re-canalize the cervix. The surgery is outlined as be our video submission. <h3>Interventions</h3> Laparoscopically-assited vaginal neocervix creation. <h3>Measurements and Main Results</h3> Patent cervix 5 days post-operatively. <h3>Conclusion</h3> We present a novel and minimally invasive technique for a woman with secondary infertility due to cervical agenesis. This may have the potential improve the fertility of women who face cervical stenosis post radical trachelectomy. Advances in oncological surgery and fertility will potentially help solve the question of fertility preservation in women treated for cervical cancer.

Vaginal Atresia with Cervical Agenesis, a Rare Congenital Malformation

Congenital vaginal atresia and cervical agenesis is a rare congenital abnormality of the female genital tract. Here we report a case of 15-year old girl with primary amenorrhea with hematometra, presented with lower abdominal mass. She was symptomatic since 5 months and visited local hospital after 4 months of onset of her symptoms when it became severe, where diagnostic laparotomy was performed for suspected Adnexal mass. Intraoperatively adnexal mass was adhered with and extended up to the uterus with 16 weeks size of uterus. Abdomen was closed without any further intervention and was referred to higher center for needful. When she presented to Kathmandu Model Hospital, she was asymptomatic on her 5th post-operative day of laparotomy. We planned for surgical intervention after examination and investigation. Drainage with vaginoplasty with amnion graft with placement of mould was done.

SURGICAL RECONSTRUCTION (UTEROVAGINAL ANASTOMOSIS) USING AUTOLOGOUS PERITONEUM (DAVYDOV’S PROCEDURE) IN A CASE OF CERVICAL AGENESIS

Cervical agenesis is a rare Mullerian anomaly requiring surgical reconstruction to restore the uterovaginal canal and thus menstruation in such patients. In the past, treatment in such patients was hysterectomy which has now developed into more conservative approach. We describe a successful canalization of the uterovaginal canal using autologous peritoneum in a case of cervical agenesis