Cervicovaginal Agenesis Research Articles

Functional endometrial preservation in cervicovaginal or vaginal agenesis: A medical versus cultural perspective in a developing country

Functional endometrial preservation in cervicovaginal or vaginal agenesis: A medical versus cultural perspective in a developing country

The presentation and management of complex female genital malformations.

Common uterine anomalies are important owing to their impact on fertility, and complex mesonephric anomalies and certain Müllerian 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. 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 'Müllerian 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. 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 Müllerian 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. 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.

Cryptomenorrhea with cervicovaginal aplasia: endoscopic transfundal development of the lower genital tract

The current case series was done to evaluate the feasibility of transfundal hysteroscopy (TFH) in helping the development of new cervical canal in cases with cervical aplasia. Five cases with obstructive cervicovaginal agenesis with hematometra were included in this report. Laparoscopic- guided TFH was done in conjunction with endoscopic cana- lization to all cases. Additional retropubic balloon vaginoplasty (BV) was needed in three cases with associated vaginal aplasia. The hysteroscope was passed through the uterine fundus. After complete washing, the endometrial lin- ing was inspected, and a properly located intrauterine catheter coming out from the vagina or vaginal dimple was used to drain the uterine cavity and maintain the cervical tract. The procedure was done successfully in all cases with adequate drainage of hematometria. Additional time needed for TFH was between 4 to 15 min. Second-look hysteroscopy revealed adequate canalization in all cases. The five cases had regular menstrual cycles up to the sixth postoperative month. In conclusion, TFH is a safe and feasible procedure as a harmo- nizing technique during endoscopic canalization of cervical atresia with or without BV in cases of obstructive Mullerian anomalies. The procedure accelerates the drainage of uterine contents and localizes the correct site of draining catheter.

Cervicovaginal anastomosis by Gore-Tex in Mullerian agenesis.

To determine usage of polytetrafluoroethylene, Gore-Tex, as a stent in patients with congenital cervicovaginal agenesis. Follow-up study. Tertiary center referral hospital. Eight patients with Mullerian agenesis who had congenital cervical agenesis, associated with partial or complete vaginal aplasia. Laparoscopic uterovaginal anastomosis with placement of a polytetrafluoroethylene stent for reconstruction of cervical agenesis. In cases associated with vaginal agenesis, modified McIndoe vaginoplasty was done concomitantly. Eight patients with cervical agenesis who had partial and complete vaginal aplasia underwent vaginoplasty. Mean (SD) follow-up after surgery (3 years old). All patients except three of them, (62.5 %) experienced relief of abdominal pain and regular menstruation. Due to ascending infection three of them (37.5 %) needed reoperation. So, they underwent laparotomy. Two of these three patients had total abdominal hysterectomy and one case tolerated resection of right obstructed side of didelphys uterus, then she became pregnant spontaneously. Four out of these eight cases were married and the others were single. One out of our married patients conceived three times. Her first pregnancy was aborted. She delivered two alive babies after inserting transabdominal Shirodkar suture in her other pregnancies. Uterovaginal anastomosis assisted laparoscopy with polytetrafluoroethylene (Gore-Tex) is effective and hopeful to relieve patients' symptoms, menstruation regularity, and saving fertility. If ascending infection threatening health happens, hysterectomy will be recommended.

P29.17: Transmyometrial embryo transfer in a patient with cervicovaginal agenesis

<scp>P</scp>29.17: Transmyometrial embryo transfer in a patient with cervicovaginal agenesis

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. DOI: http://dx.doi.org/10.3329/bjms.v13i1.17500 Bangladesh Journal of Medical Science Vol. 13 No. 01 January2014: 101-104

Letter to the Editor Report of surgical correction of a cervicovaginal agenesis case: cervicovaginal reconstruction with pudendal thigh flaps

Letter to the Editor Report of surgical correction of a cervicovaginal agenesis case: cervicovaginal reconstruction with pudendal thigh flaps

Massive Hematometra due to Congenital Cervicovaginal Agenesis in an Adolescent Girl Treated by Hysterectomy: A Case Report

A case of massive hematometra with a bicornuate uterus in a 14-year-old mentally handicapped girl complicated by vaginal agenesis and absent cervix is presented. She was managed by abdominal hysterectomy and right salpingo-oophorectomy that included the ovarian cystadenoma. The left ovary was conserved. This treatment was considered appropriate for this patient.

Uteroneovaginal cannulation using Pezzer catheter in patients with vaginal agenesis and functional uterus

The aim of this study was to introduce a method for uterus preservation in patients with vaginal agenesia and functional uterus. Six patients with vaginal agenesia and one patient with cervicovaginal agenesis with functional uterus were enrolled in the study. Laparoscopy, vaginal reconstruction, laparatomy, hematocolpos evacuation and cannulation were carried out between atretic cervix and neovagina using a Pezzer catheter. No sutures or grafts were used. Patients were trained to use a vaginal mold regularly. Pezzer catheter remained in place for 6 months to maintain menstrual drainage and avoid orifice obstruction. The surgical procedure was successful in all cases. Menses returned and abdominal pain was relieved in all patients. Three patients faced stenosis and two cases suffered from infection. One patient became pregnant and delivered at term. Self-image and quality of life were improved in all cases. Uteroneovaginal cannulation using a Pezzer catheter relieves pain, restores regular menses and fertility and reduces symptoms related to retrograde menstruation in patients with vaginal agenesis and functional uterus.

Vaginoplasty with oxidized cellulose: anatomical, functional and histological evaluation

To present and evaluate the histological, anatomical and functional results of the McIndoe procedure, as modified by the application of oxidized cellulose (Surgicel™) in women with vaginal agenesis. Eleven patients with vaginal agenesis underwent vaginoplasty using a mould that had been wrapped with oxidized cellulose. The surgeries were performed between January 2009 and January 2010. Eight of the patients had been diagnosed with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome, and the remaining three had been diagnosed with cervicovaginal agenesis (CVA). The mean follow-up time was 14 months (range, 6-24 months), and it included clinical examinations and evaluation of the Female Sexual Function Index (FSFI). Neovaginal biopsies were taken at the time of surgery and 1-12 months after surgery. The histology of the samples was evaluated to determine squamous epithelialization of the neovaginal tissue over time, and the total collagen content of the neovaginas were compared with normal control subjects. For statistical analysis we employed the ANOVA test and the t-test. At 6 months, anatomical success was achieved in 100% of the MRKH syndrome patients (neovaginal length ≥ 6 cm), and functional success was achieved in 100% of the patients who started their sexual life (FSFI score ≥ 30). Biopsy results showed complete epithelialization of the neovagina after 5 months in all samples, and the collagen content was comparable to that of a normal vagina. One major postoperative complication occurred in a patient with CVA, which culminated in death. The uterovaginal canalization procedure was unsuccessful at creating an outflow tract for regular menses in all cases. The procedure described here offers patients a functional vagina by means of a simple and low-cost procedure that elicits squamous epithelialization of the neovaginal vault, with total collagen content similar to that of normal vaginal tissue. It is a potential alternative therapeutic approach for MRKH syndrome but not applicable to cases of CVA.

Hysterectomy with Extensive Endometriosis with Cervico Vaginal Agenesis

Hysterectomy with Extensive Endometriosis with Cervico Vaginal Agenesis

Colon replacement of vagina to restore menstrual function in 11 adolescent girls with vaginal or cervicovaginal agenesis

Cervicovaginal or vaginal agenesis with functioning endometrial tissue is rare. We report the construction of a colon conduit which is anastomosed to posterior uterine wall or upper vaginal pouch to allow menstruation. We report seven girls with cervicovaginal agenesis and four with lower vaginal agenesis (aged 12-20 years) who presented with painful cryptomenorrheoa. All the girls wanted to conserve their uterus and menstruate normally. A colon conduit was constructed for the egress of menstrual blood. The colon conduit was anastomosed to the posterior uterine wall in the seven girls with cervicovaginal agenesis and to the distended upper vaginal pouch in the four girls with vaginal agenesis. Utero-colonic neovaginal anastomosis was performed only after excising a circular portion of the posterior myometrium to prevent stenosis. The colon conduit functioned effectively, providing an egress for regular painless menstruation. One patient had stenosis of the perineal neovaginal orifice for which dilations were done. One girl has married and reports satisfactory intercourse. The mean follow up is 2.2 years. This group of patients forms a separate subgroup needing a conduit not only for sexual function but also for menstruation. However, if treated by the method described herein, they should be cautioned against pregnancy if they have cervicovaginal agenesis and against vaginal delivery if they have vaginal agenesis.

Laparoscopic Hemihysterectomy in a Case of Uterus Didelphys with Nonfused Rudimentary Uterine Horns with Functioning Endometria and Complete Cervical-Vaginal Agenesis

Abnormalities of the Mullerian system exist in a variety of forms. Conventional anomalies are easy to manage, but a rare combination of anomalies requires good clinical judgment to diagnose and manage. In this paper, we report on a 17-year-old girl with two rudimentary horns, with functional endometrium in one horn, and cervicovaginal agenesis. She was diagnosed to have vaginal agenesis 2 years prior and underwent vaginoplasty. The presence of an obstructive uterine anomaly was overlooked. She presented to us with cyclic abdominal pain 2 years later. On evaluation, a functional rudimentary horn was diagnosed and removed laparoscopically. Abnormalities in vertical fusion may rarely coexist with defects in lateral fusion and must be looked for in any adolescent with cyclical abdominal pain.

Double uteri with cervicovaginal agenesis

To report results of managing complex malformation of the genital tract and to validate a newer classification (vaginal, cervix, uterus, adnexa-associated malformation [VCUAM]) for describing such complex anomalies. Case report. University hospital. A patient with complex genital malformation. Laparotomy, double hysterectomy, and vaginal reconstruction with use of bilateral neurovascular pudendal thigh flaps. Use of VCUAM classification for description of the anatomy of malformation. Creation of a neovagina and accurate and complete description of the anomaly. Relief of pelvic pain and creation of patent and sensate vagina retaining the innervations of the erogenous zones. The VCUAM classification could accurately depict complex anatomic variations. Vaginal reconstruction with use of bilateral neurovascular pudendal thigh flaps yields good postoperative results. The VCUAM classification reflects anatomic variations in external and internal organs precisely.

Rectovestibular fistula as neovagina in congenital cervico‐vaginal agenesis associated with imperforate anus

An 18-year-old female presented with primary amenorrhea and progressive cyclic abdominal pain, which prompted emergency exploratory laparotomy. Intraoperative findings revealed absent cervix and vagina, partial bicornuate uterus, hematometra, left tubal endometriosis, ruptured left endometrioma and left renal agenesis. Left salpingectomy, left oophorocystectomy, hysterotomy, evacuation of menstrual blood and transverse loop colostomy were performed. Depo-medroxyprogesterone acetate was administered to suppress menses. On re-exploration, utero-rectovestibular fistula anastomosis was described with the distal rectovestibular fistula functioning as a neovagina. Cyclic menses occurred thereafter. Endorectal pull-through with anoplasty was performed after the fistulous tract healed. To the best of our knowledge, this is the first reported case of cervico-vaginal agenesis associated with imperforate anus and rectovestibular fistula. Early diagnosis and surgery are necessary to avoid complications such as endometriosis. The aim was to preserve fertility with conservative management.

Hysterectomy for hematometra in a 15-year-old mentally handicapped girl with congenital cervicovaginal agenesis and concomitant ovarian adenoma

We report a case of hematometra and bilateral hematosalphinx in a 15-year-old mentally handicapped adolescent girl, complicated by vaginal agenesis over cervical atresia in the presence of an ovarian adenoma. The case was managed by abdominal hysterectomy and bilateral salphingectomy of the hematosalphinx, which had formed a tubo-ovarian mass. One ovary was preserved. This case also considers the management of the mentally handicapped patient unable to comprehend pain arising from cryptomenorrhea.

Cervicovaginal agenesis: spontaneous gestation at term after previous reimplantation of the uterine corpus in a neovagina: Case Report

We present an exceptional case of a patient with complete cervical atresia and total vaginal aplasia. After McIndoe vaginoplasty, cervicoitsmic resection with implantation and reimplantation of the uterine corpus in the neovagina, and conservative surgery for endometriosis, the patient had normal menstruations and became pregnant spontaneously. A Caesarean section was performed at week 36. This is the third published case report of a successful spontaneous pregnancy and Caesarean section at term in a patient with complete cervicovaginal aplasia, and the first published case study of a patient becoming pregnant after McIndoe vaginoplasty, cervicoistmic resection and utero-neovaginal anastomosis. Gestation developed successfully without cerclage. We recommend conservative surgery in patients with congenital cervical atresia. McIndoe vaginoplasty should be performed as soon as possible in adolescence if there is associated vaginal aplasia. Nevertheless, fibrotic stenosis can occur, even after several years and, therefore, additional operations and uterine reimplantation may be required.

Bicornuate uterine horns with complete cervical–vaginal agenesis and congenital vesicouterine fistula

We present a case of bicornuate uterine horns with complete cervico-vaginal agenesis and associated congenital uterovaginal fistula. The patient presented with cyclical hematuria, amenorrhea, and abdominal lump. The vagina was blind-ending. The cystoscopic examination during cyclical hematuria revealed bloody efflux through a small fistula on the posterior wall of the urinary bladder. The magnetic resonance imaging (MRI) showed bicornuate uterus and cervical agenesis. Hysterectomy and repair of the uterovesical fistula was done. The vagina was reconstructed using an amniotic mould. The report underlies the importance of MRI in diagnosing complexity of such rare anomalies. It also stresses for the need of hysterectomy if cervicovaginal agenesis is present.