Unicornuate uterus with rudimentary horn is a rare structural uterine anomaly resulting from incomplete Mullerian duct development and/or fusion. Pregnancy in rudimentary horn is an uncommon presentation of a Mullerian anomaly and may lead to substantial morbidity and mortality due to high risk of uterine rupture with intraabdominal hemorrhage. Medical and/or surgical management may be undertaken; however, currently, no treatment guidelines exist. We describe the management of a 12-week rudimentary horn pregnancy in a 25-year-old multiparous patient with a prior spontaneous preterm breech vaginal delivery and one spontaneous early term cephalic vaginal delivery in whom this congenital uterine condition was previously unknown. The rudimentary horn, nonviable pregnancy, and contiguous ipsilateral fallopian tube were excised laparoscopically without complication. Given the infrequency of rudimentary horn pregnancies and the high risk for obstetric complications, a high index of suspicion should be maintained. We emphasize that a history of preterm birth or malpresentation should raise suspicion for maternal Mullerian anomaly, and that a minimally invasive approach can be feasible for treatment of a rudimentary horn pregnancy.

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare anomaly of the female urogenital tract characterized by the combination of uterine didelphys, obstructed hemivagina, and ipsilateral renal anomalies. The exact incidence of the syndrome remains unknown, but it has been reported to be 1 per 2000 to 1 per 28,000 women. It is believed that the triad is a mesonephric duct-induced paramesonephric duct anomaly. In majority of the cases with complete hemivaginal obstruction, the pathology is diagnosed after menarche due to retained menstrual flow. While the common clinical presentations are dysmenorrhea, pelvic pain, intermenstrual bleeding, and pelvic mass, it can also manifest itself with unusual gastrointestinal and urinary tract symptoms. We present a case of HWW syndrome with gastrointestinal symptoms like worsening constipation and abdominal fullness. The unusual clinical presentation of this syndrome makes diagnosis more challenging. To solve such medical puzzles and prevent complications, detailed history-taking and radiological guidance are critical.

Unicornuate uteri are a type of Mullerian duct anomaly and the majority present with rudimentary horns. Rudimentary horn pregnancies are extremely rare and have a high risk of rupture. A high index of suspicion is needed to diagnose them early and unfortunately, the majority of cases are undetected until the patient presents with a ruptured uterus. Early diagnosis and management will reduce morbidities and mortality for patients. We present a case of a 29 year old who had a routine ultrasound scan in the first trimester that raised an index of suspicion for a rudimentary horn pregnancy. An MRI scan was performed and supplemented the ultrasound findings. The patient underwent laparoscopic management, and the non-communicating rudimentary horn, the foetus, and the attached tube were excised. The patient had a smooth recovery and had no complications.  Due to the rarity of rudimentary horn pregnancies, a high index of suspicion is needed for a diagnosis. Timely detection and intervention are crucial to prevent complications. Ultrasound scans and MRIs can aid in the diagnosis. Traditional management involved laparotomy, but with surgical advancements, laparoscopic surgery can be utilized as a less invasive alternative.

BACKGROUND: Obstructed hemi-vagina and ipsilateral renal agenesis (OHVIRA), or Herlyn-Werner-Wunderlich syndrome, is a rare Mullerian duct anomaly. Several associations of OHVIRA with other anomalies are being reported.
METHODS: A 13-year-old girl reported with acute onset abdominal pain. Patient was operated on in the past for type IV congenital pouch colon (all stages complete). She was diagnosed with a hematometrocolpos and underwent tube vaginostomy. Further workup was suggestive of OHVIRA, which was subsequently managed with surgical repair.
CONCLUSIONS: Delineation of reproductive anomaly in female patients of anorectal malformations is of paramount importance. OHVIRA syndrome should be considered at a high index of suspicion in female patients with solitary functioning kidney. Nonspecific symptoms in an adolescent female can lead to erroneous judgment, leading to unnecessary investigations; and if not well managed in time, can be detrimental to fertility.

UNASSIGNED: Mullerian duct anomaly such as uterine didelphys is a rare congenital anomaly of female genitourinary tract presenting with dysmenorrhea, hematocolpus, and subfertility in adolescent females. It is commonly associated with wolffian duct anomaly like renal agenesis. Here we present a rare association of psoas abscess with uterine didelphys and renal agenesis in a 21-year-old unmarried female.
UNASSIGNED: We report a case of 21-year-old female presenting with right hip pain, recurrent genital infections and dysmenorrhea. Her labs suggested infectious etiology whereas radiological investigation revealed right psoas abscess. In addition, she was found to have uterine didelphys with pyometra, right adnexal mass, and right renal agenesis.
UNASSIGNED: Uterine didelphys commonly present with dysmenorrhea and hematocolpos along with various non-specific symptoms. Patients can develop psoas abscess secondary to uterine didelphys, but uterine didelphys presenting with psoas abscess is fairly rare. Psoas abscess on itself is a difficult condition to diagnose, more so when associated with rare uterine anomalies.
UNASSIGNED: This case highlights the possibility of psoas abscess as a primary presentation of Mullerian duct anomaly. Further, a differential of uterine didelphys should be considered in every reproductive age female presenting with recurrent pelvic infection.

Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is a rare Mullerian duct anomaly characterized by an obstructed hemivagina, ipsilateral renal agenesis, and uterine didelphys. There are only a few published case reports of OHVIRA syndrome, and cases of OHVIRA syndrome associated with cancer have rarely been reported. In fact, there is only one published report of a case with clear cell carcinoma (CCC) of the cervix. Here, we report a case of CCC of the cervix with OHVIRA syndrome that underwent abdominal radical hysterectomy; we also provide a short literature review of this topic. A 52-year-old woman presented with abnormal vaginal bleeding for 1 month 2 years after menopause. A pelvic examination and preoperative imaging showed uterine didelphys, an obstructed hemivagina with a mass measuring approximately 2 cm located in her left cervix, and an absence of her left kidney. A colposcopy biopsy reported CCC of the cervix. Clinical staging classified her with stage IB1 disease. Abdominal radical hysterectomy was performed. Her left ectopic ureter led to the left cervix and opened in the endometrium, resulting in a so-called ectopic ureter. Macroscopic examination of the excised specimens showed two cervixes, two corpora of the uterus, and a tumor measuring 1.0 × 2.0 cm on the left cervix. In addition to typical OHVIRA symptoms including uterine didelphys, obstructed hemivagina, and renal agenesis, several anatomical variants were present. The current case included those variants as well as an atrophic kidney with an ectopic ureter to the obstructed hemivagina. Based on the results of our case, clinicians should be aware of the risks of cancer and anatomical variants associated with OHVIRA syndrome.

Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital anomaly characterized by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. Usually, such patients present with dysmenorrhea shortly after menarche, increasing pelvic pain and a palpable mass due to the obstructed hemivagina. Interestingly in the present case, the patient had her menarche seven years ago, but dysmenorrhea started only 1-year back. She never sought medical help previously as she was mostly asymptomatic all through the years. It was only after she conceived and got investigated for antenatal concerns that she was found to have HWWS. A tortuous history and an unusual clinical presentation made this case an interesting one.

OBJECTIVE: Mullerian duct anomalies (MDA) are common female genital tract malformations. Genetic and environmental factors are important causes of MDA in women. Although many genes and mutations have been found to be associated with the pathogenesis of MDA, in most cases, the genetic pathogenic factors of MDA are still unknown.
METHODS: We first analyzed the three sisters using low coverage whole-genome sequencing. Then whole-exome sequencing was carried out in each patient. The identified sequence variant was confirmed by Sanger sequencing. In silico pathogenicity analysis and conservative analysis of the mutation site were also performed. Protein structural modeling was used to analyze the effect of the mutated amino acid.
RESULTS: We first analyzed the three sisters with septate uterus using low coverage whole-genome sequencing, but no possible pathogenic copy number variation was found. Then whole-exome sequencing was performed on the three sisters, and a rare homozygous variant, CDC42BPB:c.2012G>A:p.R671Q, was identified. All three patients were found with this variant. Sanger sequencing validated that this variant was segregated within the family. In silico pathogenicity analysis and conservative analysis of the mutation site suggested that the variant might be damaging. Protein structural analysis suggested that R671Q might weaken the electrostatic potential of this region, which may be a significant regulation target or protein interaction surface of CDC42BPB.
CONCLUSIONS: We demonstrated that CDC42BPB genetic variant might be potentially associated with the pathogenesis of MDA.

Background This study aimed to evaluate the imaging findings of patients who underwent an abdominal and pelvic magnetic resonance imaging (MRI) due to primary amenorrhea. Methods The pelvic and abdominal images of 34 female patients (mean age 15.61 years, range 14-19 years) were retrospectively analyzed by a single radiologist blinded to the clinical and laboratory data of the patients (other than primary amenorrhea) to evaluate the etiology of primary amenorrhea. The anatomy and anomalies of the internal genital organs and other accompanying abdominopelvic anomalies were investigated. Results Gonadal dysgenesis was present in 14 patients (41.17%) and Müllerian duct anomalies (MDAs) were present in 20 (58.83%) (Mayer-Rokitansky-Kuster-Hauser [MRKH] syndrome in 13 [65%], distal vaginal obstruction [DVO] findings in five [25%], and obstructed hemivagina and ipsilateral renal anomaly [OHVIRA] syndrome in two [10%]). Seven patients with MRKH (53.84%) were of type 1 and six (46.15%) were of type 2. A total of eight additional anomalies (vertebral and renal) were detected, six in MRKH and two in OHVIRA syndrome cases. Endometrioma and hematosalpinx were observed in one of the five patients with DVO (5%). Conclusions Primary amenorrhea is a common symptom that affects both the physical and psychosocial status of individuals. Determination of the underlying etiology is the first step in planning treatment. The evaluation of internal genital organ anomalies involved in the etiology is important for sexual function and fertility. MRI is a non-invasive imaging modality that should be preferred in these cases as it provides detailed data about the anatomy and anomalies of internal genital organs due to its high soft tissue contrast resolution.

OBJECTIVE: There are some reports of cervical cancer with uterus didelphys but a case of clear cell carcinoma (CCCC) with Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) syndrome is extremely rare. The aim of this paper was to report a case of CCCC with OHVIRA syndrome and the difficulty in making a preoperative diagnosis.
METHODS: A 65 years old woman presented with postmenopausal bleeding and pelvic examination showed right paracervical mass. Preoperative confirmation of cervical carcinoma was difficult due to the location of the mass, which was inaccessible by cervical punch biopsy. Pelvic examination revealed a large mass in pelvic cavity without parametrial invasion and ultrasound showed approximately 70 mm cervical tumor. Laparoscopic surgery revealed clear cell carcinoma of the para-endocervix with OHVIRA syndrome.
CONCLUSIONS: In the case of cervical carcinoma with OHVIRA syndrome, laparoscopic surgery is preferable for the diagnosis and management.