Mullerian Anomaly with Large Ovarian Mass — Where Two Worlds Collide

When young women diagnosed with Large Ovarian Masses present with associated mullerian abnormality it shakes the world of Gynaecology and requires the joint help of General Gynaecologists and gynae-oncologists as reconstruction of Mullerian anomaly and fertility preservation is as important as management of Malignant Ovarian Masses. We report a case of 21 year, unmarried woman with complaints of primary amenorrhea and large abdominopelvic mass and short, blind vagina. Magnetic Resonance Imaging showed Uterus Didelphis with normal endometrium and upper vaginal agenesis. Computed Tomography images revealed a large ovarian mass. Intraoperatively there was a 30cm large Ovarian Tumour, Didelphyic and hypoplastic (2cm) uterus and 2 cm blind vagina. Management of this case involved extensive discussion among the Gynecologists, Gynae-oncologist and radiologists. In non-oncology setup where the rate of surgeries are high, option between frozen section and Mullerian reconstruction are required especially when the Ovarian mass looks benign as both surgeries require time and expertise. Hence, involvement of patient and family members in decision making form an integral part of management.Intraoperative findings also influence surgical decisions in Mullerian anomaly

Pregnancies in anomalous uterus: series of three cases

Uterine malformations can result from various alterations in the normal development of Mullerian ducts that make up a diverse category of congenital anomalies. In most cases, the defect remains undiagnosed and exhibits the normal number of chromosomes. Very few malformations require any intervention after thorough investigations. The most frequently used classification by the American Society for Reproductive Medicine is comprehensive, but does not include cervical or vaginal malformations. The European Society of Human Reproduction and Embryology has postulated a consensus that precisely shows independent cervical malformations.

Accessory and cavitated uterine mass: A rare and unclassified mullerian anomaly

@#<p style="text-align: justify;">Accessory and cavitated uterine mass is a rare developmental mullerian anomaly theorized to be related to gubernaculum dysfunction. It presents typically in young women as severe dysmenorrhea and chronic pelvic pain refractory to medical therapy. It is an accessory cavity lined by functional endometrium and surrounded by myometrium-like smooth muscle located in an otherwise normal uterus, typically located at the right anterior wall at the level of the round ligament attachment. Ultrasound, hysterosalpingography and magnetic resonance imaging are helpful tools to diagnose and distinguish this entity from a wide array of differential diagnoses. Surgical excision and histopathologic studies confirm the diagnosis and effectively relieves severe dysmenorrhea and chronic pelvic pain. This is a case of a 39 year old nulligravid who presented with severe dysmenorrhea initially diagnosed as rudimentary horn versus myoma. Excision revealed a cavitated mass containing chocolate-colored fluid within located at the right postero-fundal area. Histopathology revealed a diagnosis of accessory and cavitated uterine mass.</p>

Robert’s uterus: a rare mullerian anomaly mystery unfolded

Anomalies of female genital tract may not be detected until after menarche when they present a cyclical pain due to outlet obstruction. Mullerian anomalies represent a vast array of structural abnormalities resulting from improper development and fusion of embryological mullerian ducts. 19-year-old girl attained menarche at the age of 14, had progressive dysmenorrhoea and diagnosed as right haemotosalphinx and ovarian endometrioma which were removed in 2008. As pain progressed, she underwent laparoscopic adhesiolysis in 2013. Since, pain persisted, diagnosed as right haematometra, and drainage done by laparotomy. Left adnexa were normal. She was given depot provera till she completed schooling. She developed recurrent dysmenorrhoea after stopping depot provera. USG and MRI revealed recurrent haematometra on right side with normal left horn. The possibility of atypical septum was thought about and hystero laparoscopy was done. It showed right side haemetometra with absent right adnexa. Left adnexa normal. Hysteroscopy showed normal left horn with septum with a bulge towards the left side. Hence, proceeded with hysteroscopic septostomy and haemetometra was drained to the left horn. Later patient was free from dysmenorrhea and repeat hysteroscopy was found to be normal. This case highlighting mullerian anomalies have to be considered when young girls present with severe progressive dysmenorrhoea and diagnosis remains a challenge most of the clinicians. This rare entity has to be kept in mind while evaluating such patients. Prompt diagnosis and early surgical correction are essential to avoid future morbidity in the form of repeated unnecessary surgeries.

Ectopic pregnancy in a case of congenital mullerian anomaly: a diagnostic dilemma

Ectopic or extrauterine pregnancy occurring in a case with mullerian defect is very rare and poses diagnostic challenges. Undescended and non-communicating fallopian tubes are extremely rare mullerian anomalies. Here authors present a case of ectopic pregnancy occurring in an undescended non-communicating fallopian tube in a patient with unicornuate uterus with absent horn, which was managed laparoscopically. A 32-year-old lady, diagnosed case of left unicornuate uterus with absent right horn, was referred to us with the suspicion of ruptured ectopic pregnancy. The abdominopelvic ultrasound showed a soft tissue lesion of size 32×24 mm, towards the right lateral pelvic wall near the iliac vessels, with increased vascularity on colour flow doppler.  The patient underwent laparoscopy which showed left sided unicornuate uterus with normal left tube and ovary. The right uterine horn was absent.  An undescended right ovary and tube were found attached to the peritoneum at the level of pelvic brim along the right lateral pelvic wall.  Right sided tubal ectopic pregnancy with rupture was present along with 300-350 cc of hemoperitoneum. The patient was treated with laparoscopic right sided total salpingectomy. In patients with unicornuate uterus and atypical presentation, ectopic pregnancy should be ruled out in an undescended non-communicating fallopian tube. Salpingectomy of incidentally diagnosed non-communicating fallopian tubes is recommended to prevent future ectopic pregnancy.

A case report on ACUM: a rare mullerian anomaly

Accessory and cavitated uterine mass (ACUM) is a rare, newly recognized mullerian anomaly. It is an accessory cavity lined by functional endometrium within an otherwise normal uterine cavity, in contrast to the other mullerian anomalies in which the uterus is malformed. It is often misdiagnosed as myoma, adenomyosis or adenomyoma. The entity needs expertise to diagnose as it is a rare but treatable cause of severe dysmenorrhea and chronic pelvic pain in young females with a wide range of differential diagnosis. A 30 years old female with history of one abortion 2 years back, came to infertility OPD with complaints of lower abdominal pain with severe dysmenorrhea, dyspareunia and inability to conceive since 2 years. Her menstrual cycle was regular with normal flow. USG pelvis showed subserosal fibroid of 4×3 cm in fundus region of uterus. On HSG, bilateral fallopian tubes were patent. MRI-pelvis revealed normal uterus with thick walled cavitatory lesion of size 4.1×3.6 cm with thick T2W hypointense wall similar to myometrium in continuation with right fundal wall of uterus containing blood products suggestive of ACUM. Both ovaries were normal, no obvious adnexal lesion/collection/free fluid noted in peritoneal cavity. Laparoscopy followed by laparotomy with excision of ACUM was performed. The MRI findings of an accessory cavitated uterine mass located below the attachment of round ligament with haemorrhagic contents, normal shaped uterus with normal bilateral tubes and ovaries should suggest the diagnosis of ACUM pre-operatively.

Successful outcome in a perplexing case of Roberts uterus: a rare uterine anomaly, a rare case report with review of literature

Robert uterus is a rare Mullerian development anomaly with very few cases reports available. It presents with triad of morphologic features of - Blind hemi cavity with or without unilateral hematometra, contralateral unicornuate uterine cavity and normal uterine fundus with or without small external indentation. The major difficulty lies in making the diagnosis of Robert’s uterus. All the reported cases of Robert’s uterus have been managed differently according to patient’s complaints. A 25-year-old married female, resident of Agra presented to gynecology OPD of Dr RML Hospital and associated PGIMER in June 2013, with primary infertility and cyclical left sided dysmenorrhoea since menarche. Patient was consulting at her hometown Agra for 2 years and had an USG and MRI pelvis report of Feb. 2013 with her showing unicornuate uterus with rudimentary horn. Infertility investigations were done in our hospital. HSG report was of localized spill on right with left tubal block. Patient was posted for diagnostic hystero-laproscopy which was further followed by laprotomy, after which we could reach to a diagnosis of Robert uterus with non-communicating left cavity and with severe endometriosis. Patient needed a second hysteroscopy for lysis of septum and subsequently conceived with IVF. She delivered a healthy male baby of 2.65 kg by elective LSCS at 37 weeks with associated breech presentation with gestational hypertension with severe IHCP on 20 June 2019. Paediatric surgeons and gynecologists should be aware of this rare atypical obstructive Mullerian malformation and its management to avoid inappropriate management delays in these patients. A timely diagnosis and definite treatment have a great impact on future reproductive and endocrine function.

Ovarian dysgerminoma with Müllerian anomaly: a case report

Mullerian Duct Anomaly- A Spectrum of Varied Clinical Manifestations

Introduction: Multiple integrated steps are required fornormal development of the female genital tract. A wide varietyof malformations can occur when this system is disruptedeither in form of non- development or defective fusion orfailure of resorption. The purpose of this study was to reviewthe spectrum of symptoms with which mullerian anomaliespresent so that timely intervention could be done to savesexual and reproductive life.Material and methods: The study was done in the departmentof Obstetrics and Gynaecology of a tertiary care teachinghospital in Bihar. Only the admitted cases i.e,those whorequired surgical correction were taken into study.Results: Majority of patients presented with severeabdominal pain(71.4%) followed by menstrual problems(46.4%), and urinary symptoms (35.7%) including vvf. 25%patients reported subfertility, 14.2% abdominal lump and14.2% dyspareunia, 10.7%PID and 10.7% rectal pressuresymptoms.Majority had transverse vaginal septum (28.5%),followed byMRKHS and OHVIRA syndrome (14.2% each),Conclusion: The variety of ways with which mullerian ductanomalies present hints that clinical suspicion should be thereif early diagnosis is not to be missed.

Site of pregnancy obstetrician’s dilemma

A 28-yr-old P2L2 lady, with history of two previous caesarean deliveries and tubal ligation, presented at 6weeks of amenorrhea with pain lower abdomen. The operative notes from her previous caesarean section stated that she had a unicornuate uterus without a rudimentary horn (ASRM Class II D Mullerian anomaly)5 and only right fallopian tube was visualised, which was ligated by Parkland’s method.On workup, she had a positive urine pregnancy test and ultrasound showed a left adnexal mass without any evidence of intraperitoneal collection, possibly an unruptured left ovarian ectopic pregnancy secondary to failed tubal ligation. Further investigation by MRI revealed an entirely new finding. The suspicious left adnexal mass was the left horn of bicornuate uterus which had an intrauterine gestational sac. Hence, her revised diagnosis was G3P2L2, post LSCS, bicornuate uterus (ASRM class IV B) with 6 weeks of intrauterine left horn pregnancy following failed tubal ligation.She underwent a medical followed by surgical evacuation of intrauterine pregnancy as patient was unwilling to continue the pregnancy.This case highlights the importance of a comprehensive evaluation, whenever a lady is diagnosed with a Mullerian anomaly, in order to correctly classify the patient and identify associated anomalies of urogenital tract which would avoid such erroneous diagnosis of site of pregnancy as illustrated in our case.

Uterine Isthmic Atresia: Hope for a rare mullerian anomaly

@#The true incidence and prevalence of congenital Mullerian duct anomalies are difficult to determine. Some patients may present as adolescents with apparent primary amenorrhea, cyclical abdominal pain and sexual difficulties. It is important to ascertain a correct diagnosis for timely and appropriate interventions necessary to prevent sequelae that often affect the future fertility of these patients. This is a case of a fifteen year old with severe cyclical pelvic pain and hematuria with amenorrhea. Work up included a transrectal ultrasound and a magnetic resonance imaging of the pelvis revealing presence of a uterine corpus and cervix but absence of uterine isthmus. A conservative surgical approach was planned. The patient underwent end-to-end anastomosis of the cervix and uterine corpus. At present, the patient is regularly menstruating with no pelvic pain.

A case of right rudimentary uterus with ipsilateral renal agenesis misdiagnosed as an ovarian tumor / 대한산부인과학회지

Rudimentary uterus with ipsilateral renal agenesis is a very rare Mullerian duct malformation. The most common clinical presentation is pelvic pain and dysmenorrhea shortly after menarche, in associated with the finding of a vaginal or pelvic mass. An appropriate and prompt diagnosis and treatment will prevent unnecessary procedures and offer relief of symptoms. We report one case of rudimentary uterine horn with ipsilateral renal agenesis with a brief review of concerned literatures.

A Case of Vaginal Prolapse after McIndoe Operation Managed by Transvaginal Sacrospinous Colpopexy / 대한산부인과학회잡지

McIndoe procedure using the colonic segment has been used in treatment of patients with Mullerian agenesis. Prolapse of a colonic neovagina is rare condition and its treatment is not yet standardized. We experienced a case of neovaginal prolapse following McIndoe procedure using colonic segment. A 51-year-old woman developed prolapse of a neovagina 17 years after the McIndoe operation. She had been diagnosed to have Mullerian agenesis and was managed by McIndoe operation using colonic segment. The prolapse was successfully managed with transvaginal sacrospinous colpopexy. She has no sign of recurrent prolapse and is satisfied with sexual intercourse. Patients with neovaginal prolapse following McIndoe procedure can be treated successfully with transvaginal sacrospinous colpopexy.

A Case of Reconstruction of Utero-neovaginal Continuity / 대한산부인과학회잡지

Vaginal and cervical agenesis is an uncommon Mullerian anomaly. Total hysterectomy and vaginoplasty remains the classical treatment of this malformation. We present a case of reconstruction of utero-neovaginal continuity in the patient with normal functioning uterus and previously constructed neovagina by McIndoe method. She experienced two episodes of withdrawal bleeding during 2 months after surgery and fully satisfied with the results.

Three cases of successful pregnancy outcome after intensive care in the patients with complete uterine septum, cervical duplication and longitudinal vaginal septum / 대한산부인과학회잡지

Three cases of successful pregnancy outcome after intensive care in the patients with rare mullerian anomaly of complete uterine septum, hypoplastic cervical duplication and longitudinal vaginal septum in infertility clinic are reported. These presented three cases are the reports of supporting the embryologic hypothesis of bidirectional mullerian development which fusion and resorption begins at the isthmus and proceeds simultaneously in both the cranial and caudal directions rather than the classical views of unidirectional(caudal to cranial) mullerian development. Thus, we present here three cases of rare mullerian anomaly and their pregnancy outcomes in an attempt to alert gynecologist to the possible occurrence of such a malformation. In 2 cases, successful pregnancy outcomes were achieved after abdominal or hysteroscopic uterine septotomy before pregnancy and in the last case, successful pregnancy outcome intensive obstetric care was achieved even under uncorrected uterine anomaly and complete removal of uterine septum resulted in uneventful postoperative courses at the same time of cesarean section.