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.

Ovarian maldescent: a case report and discussion

Undescended ovary or ovarian maldescent is a rare condition usually associated with mullerian malformation and typically found in case of infertility during their workup. Embryologically, the ovarian development from the genital ridge is totally different from the paramesonephric duct which forms the uterus and fallopian tubes. Still the ovarian maldescent has strong association with mullerian abnormalities, especially with unicornuate uterus. Therefore, supporting the hypothesis that abnormal migration could affect their normal fusion. In strong correlation of ovarian maldescent with mullerian abnormality here we report case of a patient presented to infertility clinic with unicornuate uterus with unilateral renal agenesis diagnosed to have unilateral undescended ovary with conclusion that the accurate diagnosis is important and radiological effort should be made to locate the undescended ovary if the ovary could not be found in normal location especially when associated with mullerian abnormality.

Rudimentary horn pregnancy: a rare entity

Unicornuate uterus is the type 2 variety of mullerian duct anomaly resulting in unilateral agenesis or hypoplasia. The hypoplastic (Rudimentary) horn can be functioning or non-functioning and communicating or noncommunicating with the main uterine cavity. Pregnancy in the rudimentary horn is an extremely rare entity. It is often missed at ultrasound in the early trimester, presenting later with massive hemoperitoneum and shock from rupture of the horn.  A “high index of suspicion” is essential to derive at the correct diagnosis. Hence reporting this rare case for emphasizing the importance in early diagnosis and prompt management.

Ruptured rudimentary horn pregnancy: a case report

Uterine anomalies are congenital malformations arising due to embryological mal-development of mullerian ducts. The European society of Human Reproduction and Embryology (ESHRE) classifies these anomalies into 6 classes. Hemi-uterus is one such class of formation defect of mullerian duct with unilateral uterine development; the contralateral part could be either incompletely formed or absent. In a rare form of ectopic pregnancy, implantation can occur in the cavity of a rudimentary horn of the hemi-uterus. Authors report a case of 22-year G2P1L1 with 12 weeks gestation who presented with acute abdomen. Ultrasound showed hemoperitoneum with suspicion of ectopic pregnancy. Laparotomy confirmed the diagnosis of ruptured right rudimentary horn with fetus and placenta in the peritoneal cavity. Immediate laparotomy and excision of the horn with transfusion of blood and blood products saved the patient in the nick of time.

Unicornuate uterus with functional non communicating rudimentary horn: a refractory cause of dysmenorrhea

Mullerian duct anomalies are rare. Unicornuate uterus with a non-communicating rudimentary horn is a rare type of mullerian duct anomaly which occurs due to defective fusion of malformed duct with contralateral duct. The incidence is approximately 1:100000. Patient usually remain asymptomatic due to the absence of functional endometrium in most of the cases. If the rudimentary uterine horn has an endometrium lined uterine cavity and doesn’t communicate externally then the signs and symptoms of obstructed menstruation appears, as soon as menarche begins. It will be associated with severe dysmennorhoea and hematometra. Other complications may be abdominal lump, chronic pelvic pain, infertility, endometriosis, adenomyosis and ectopic pregnancy in rudimentary horn. Authors are presenting a case of refractory dysmenorrhea with lump abdomen in a patient with unicornuate uterus with functional non communicating horn. In a patient with refractory dysmenorrhea mullerian duct anomaly should be kept as differential diagnosis.

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.

A rare case of pregnancy in the rudimentary horn of unicornuate uterus (on table diagnosis): a case report

A rudimentary horn with a unicornuate uterus results due to failure of the complete development of one of the Mullerian ducts and incomplete fusion with the contralateral side. Pregnancy in non-communicating rudimentary horn of unicornuate uterus is a rare form of ectopic gestation and it carries grave consequences for mother and the fetus. The most dreaded complication is rupture during pregnancy which can be life threatening to the mother. The continuation of pregnancy is rare till 3rd trimester and usually ruptures in 2nd trimester. The primary strategy of the management is surgical excision of the rudimentary horn. Here is a case of 24year old, gravida1 with unruptured rudimentary horn pregnancy at gestational age 28 weeks diagnosed on table. IOL (induction of labour) was done with misoprostol for fetal demise and later cesarean section done in view of failed induction. Laparotomy was done and dead fetus with placenta delivered followed by excision of the rudimentary horn. The post-operative period was uneventful. This highlights the need   of an increased awareness of this condition especially in developing countries where most pregnancy are unbooked and uninvestigated as in present case.

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.

Rupture of Term Noncommunicating Rudimentary Horn of Uterus.

Pregnancy in noncommunicating rudimentary horn is rare. It occurs following transperitoneal migration of sperms or zygote. Uterine rupture usually occurs in 70-80% cases in the second trimester by 20 weeks. Neonatal survival in rudimentary horn pregnancy is poor occurring in only about 11% of cases.

Reconstrucción anatómica laparoscópica en útero unicorne no comunicante / Laparoscopic approach for unicornuate uterus

Las alteraciones congénitas de los conductos de Müller son un amplio grupo de patología, siendo el útero unicorne una de sus manifestaciones.Se relaciona con un aumento de complicaciones obstétricas y a su vez la presencia de un cuerno rudimentario no comunicante, es causa de dolor pélvico crónico. Presentamos una paciente de 14 años aquejada de intensa dismenorrea. Mediante técnicas de imagen se evidencia la presencia de útero unicorne con cuerno rudimentario derecho no comunicante. Se realizó abordaje laparoscópico en la que se procedió a exéresis del cuerno rudimentario y posterior reconstrucción anatómica. La paciente cursó un postoperatorio sin complicaciones, obteniendo una mejoría clínica satisfactoria en sus controles posteriores. El abordaje laparoscópico a las pacientes afectas de útero unicorne no comunicante permite restablecer de forma óptima la anatomía y mejorar la sintomatología asociada. Mediante una correcta técnica laparoscópica se consigue disminuir el riesgo de adherencias posteriores, asegurando una baja tasa de complicaciones y una recuperación funcional postoperatoria precoz.

Congenital anomalies of the Müllerian ducts are a large group of pathology, and unicornuate uterus is one of its manifestations. It is associated with increased obstetric complications and the presence of cavitated non communicating rudimentary uterine which cause chronic pelvic pain. We report a 14 years old patient suffering severe dysmenorrhea. Using imaging techniques showed the presence of unicornuate uterus with non communicating rudimentary right horn. Laparoscopy was performed in which we proceeded to resection of rudimentary horn and subsequent reconstruction of the anatomy. The patient had an uneventful postoperative course, obtaining a satisfactory clinical improvement in subsequent tests. The laparoscopic approach in patients with advanced non-communicating unicornuate uterus can optimally restore the anatomy and improving associated symptoms. A proper laparoscopic approach can lower the risk of subsequent adhesions, and ensure few complications and early postoperative functional recovery.

Laparoscopic excision of uterine horn in case of unicornuate uterus firmly attached with non-communicating rudimentary horn / 대한산부인과내시경학회지

Unicornuate uterus is a very rare uterine anomaly caused by developmetal failure of Mullerian duct. This anomay is associated with various degrees of rudimentary horn, which is connected with fibrous band or firmly attached to unicornuate uterus. Different method of operation may be applied according to type of anatomical connection. The unicornuate uterus is commonly associated with severe dysmenorrhea, chronic pelvic pain, and pelvic mass. A case of noncommunicating rudimentary horn firmly attached to unicornuate uterus with severe dysmenorrhea was treated with laparoscopic excison of uterine horn and is presented with brief review of literature.

Conduta frente às malformações genitais uterinas: revisão baseada em evidências / Medical care for uterine genital malformations: evidence-based review

Este trabalho de revisão foi idealizado para analisar as malformações dos ductos de Müller, que, devido à sua frequência de 3 a 7,3% na população em geral, justificam uma análise mais profunda do tema. O objetivo foi avaliar, de acordo com a literatura por meio de metodologia adequada? Os aspectos mais relevantes dessas anomalias, com destaque para a etiopatogenia, classificação, diagnóstico e tratamento. Os resultados obtidos nesta revisão apontaram as melhores evidências, até o momento, de como conduzir as mulheres portadoras dessas malformações.

This review paper was organized in order to analyse Müllerian anomalies, because their frequency from 3 to 7,3% in people in general justify a better evaluation about these malformations. The objective of the study was to evaluate, according to literature - by a proper methodology - the main aspects of these malformations, with special attention to etiology, classification, diagnostic and treatment. The results of this review showed the best evidences up till now of how to manage women with these genital malformations.

Tubal Pregnancy in a Unicornuate Uterus with Rudimentary Horn / 대한산부인과학회잡지

Several cases of ruptured pregnant uterine horn have been documented. However, there have been few reported cases of ectopic tubal pregnancy in a unicornuate with rudimentary horn. So we report this case with a brief review literature.

A Case of Rudimentary Uterine Horn associated with Agenesis of right Kidney and Pelvic Endometriosis / 대한산부인과학회잡지

Congenital M llerian anomalies of uterus are so infrequently encountered and endometriosis, primary infertility, hematometra, and urinary tract anomalies are claimed to be common complaints in women with unicornuate uterus. We report one case of rudimentary horn associated with agenesis of right kidney and pelvic endometriosis which is presented with brief review of literature about anomaly of the female genital tract.