Herlyn-Werner-Wunderlich syndrome, characterized by uterus didelphys with blind hemivagina and ipsilateral renal agenesis, is a rare Mullerian duct anomaly. This case series shows a wide spectrum of the condition, one presenting in an adolescent, managed conservatively and the other in the perimenopausal age group given a more definitive management. The first case is an 18-year- old nulligravid who manifested with progressive dysmenorrhea and foul smelling vaginal discharge a few years after menarche. She subsequently underwent vaginal septotomy followed by diagnostic hysteroscopy. On the other hand, the second case is a 46- year-old nulligravid whose chief complaint is a foul smelling vaginal discharge and consequently went through a total abdominal hysterectomy with salpingo-oophorectomy. To our knowledge, the second case is the only patient diagnosed in the perimenopausal stage and underwent a total hysterectomy. Ultrasound is the first-line imaging modality used in both cases and its merits are highlighted to prove its importance and diagnostic value in the workup of this condition.
Mullerian aplasia ; Mullerian Ducts ; Urogenital Abnormalities

Turner syndrome and Mayer-Rokitansky-Kuster-Hauser syndrome are the most common causes of primary amenorrhea. However, mullerian agenesis in patients with Turner syndrome is rare, with only 15 reported cases worldwide. Described in this report is an 18-year-old female who presented with primary amenorrhea, absent secondary sexual characteristics, short stature, webbed neck, and shield chest. Work up revealed hypergonadotropic hypogonadism, absence of normal ovaries and uterus, and hypothyroidism. She had a 45,X karyotype and was diagnosed with a very rare case of Turner syndrome with associated Mayer-Rokitansky-Kuster-Hauser syndrome. Early detection of the etiology of amenorrhea is critical for prompt management and initiation of hormonal replacement. She was counseled on the possible systemic problems associated with her condition. The importance of regular follow-up and continuous care were also emphasized. With the coexistence of these two conditions, adoption is the only option for having children in the Philippines, where oocyte donation and uterine transplantation are currently unavailable.
Turner Syndrome ; Mullerian aplasia

A six year-old presented with a 10-month history of vaginal discharge. On initial gynecologic evaluation, a vaginal mass was noted. A thorough investigation included pelvic sonogram, KUB sonogram, pelvic examination under anesthesia. A close team-up with a pediatrician and a urologist was established. Intra-operative findings revealed uterine didelphys and a blind hemivagina. This report discusses the approach to the investigation of vaginal discharge in association with a vaginal mass in a 6-year old.
HEMIVAGINA ; MULLERIAN DEFECT ; UTERINE DIDELPHYS

Background: Anti-Mullerian hormone (AMH) levels have been used as an invaluable tool in reproductive medicine for over a decade, especially in predicting ovarian reserve and follicular response during in vitro fertilization (IVF) cycles. Age-specific reference values of AMH levels have been derived from mostly Western and few Asian population groups but none from the Philippines. In this study, we attempted to determine the first age-specific AMH reference values from infertile Filipino women to be able to provide local infertility centers a guide in treating Filipinos and those with similar racial and lifestyle characteristics. Materials and Methods: This was a retrospective cross-sectional study that was conducted at the Center for Advanced Reproductive Medicine and Infertility at St. Luke's Medical Center Global City. Age, serum AMH levels, body mass index (BMI), and cause of infertility of Filipino women who underwent IVF from August 2015 to March 2020 were taken. AMH was assayed using the automated Access AMH Immunoassay (Beckman Coulter). Results: A total of 1463 women who underwent IVF and with valid AMH results were initially found but only 1233 were included in the study. Mean age was 36.67 + 4.35 years and mean BMI was 24.43 + 4.14 kg/m2. There was minimal effect of BMI on AMH levels and increasing age (R2 = 0.0068), but there were significant differences of mean AMH levels among the general causes of infertility. The mean and median AMH values decreased with advancing age (R2 = 0.1391) although the mean values were consistently higher than the median values. The mean level of the AMH was 2.32 ± 1.90 ng/mL with a 0.16 ng/ml (confidence interval: 0.14–0.19 ng/ml) level decrease per year of increase in age. By age category, the following were the derived AMH values (ng/ml) at the 25th to 75th percentiles: 25–29 = 1.52–4.92; 30–34 = 1.60–4.10; 35–39 = 0.95–3.13; 40–44 = 0.44–1.99; 45 = 0.47–1.08. The mean AMH in this study appears to be similar to several but lower than most other reported AMH nomograms from other population groups. Conclusion The first age-specific AMH reference values for infertile Filipino women are presented and may serve as a useful diagnostic marker in local infertility centers, especially those treating Filipino patients or others with similar characteristics.
Fertilization in Vitro ; Anti-Mullerian Hormone

Extrauterine mullerian adenosarcoma is a very rare tumor and it is characterized by a benign glandular component and a low-grade sarcomatous stromal component. These tumors have been reported to arise from ovarian or extraovarian endometriosis. However, there are scant reports on the MR findings of extrauterine mullerian adenosarcoma arising from deep pelvic endometriosis. We describe here a case of a large infiltrating extrauterine mullerian adenosarcoma arising from recurrent deep pelvic endometriosis and we discuss its MR findings.
Adenosarcoma ; Endometriosis ; Female ; Mixed Tumor, Mullerian ; Uterus

No abstract available.
Anti-Mullerian Hormone* ; Phenotype* ; Polycystic Ovary Syndrome*

No abstract available.
Anti-Mullerian Hormone* ; Phenotype* ; Polycystic Ovary Syndrome*

Transverse vaginal septum is a rare Mullerian anomaly which is related to vertical fusion disorder. It is an uncommon anomaly that is not associated with a well-defined inheritance pattern. The vase majority of these obstructed mullerian duct abnormalities are first seen at puberty. Hematocolpos, hematometra and hematometrocolpos are some of its most common presentations. Some authors have suggested that retrograde flow through the uterus and falopian tubes could have occured earlier. It may even present before puberty. Transverse vaginal septum is infrequently associated with genitourinary tract, gastrointestinal tract, musculoskeletal, and cardiac malformations. However, it is still prudent to work up patients of the possibility of having any concomitant anomaly. The differential diagnosis of transverse vaginal septum includes imperforate hymen, Mayer-Rokitansky-Kuster-Hauser syndrome and vaginal agenesis. The complex anatomy of mullerian duct anomalies requires complete evaluation before surgical repair which would entail the use of magnetic resonance imaging, pelvic sonographic and computed tomography. In a study by Thabet, et al. in 2002, a newer modality, sonocolpography can be used to evaluate transverse vaginal septum and other allied conditions. Treatment is surgical. This presentation aims to refocus attention to the disease by reviewing the case of a 14 year old female who presented with cyclical pain and primary amenorrhea.
Human ; Female ; Adolescent ; MULLERIAN DUCTS ; ABNORMALITIES ; ENDOMETRIOSIS ;

Wunderlich-Herlyn-Werner syndrome is an uncommon but already well-established mullerian anomaly consisting of uterine didelphys, hemivaginal septum and ipsilateral renal agenesis. The discussion here would concentrate on the occurrence of foul-smelling vaginal discharge in some of these cases, its pathogenesis and its impact on generally-accepted assumptions on the embryogenesis of the mullerian system. Reported here are three such cases with discussions on the goals of therapy being relief of the obstructive symptoms, restoration of normal menstrual egress and sexual function, with preservation of reproductive potential.
UTERINE DIDELPHYS ; VAGINAL SEPTUM ; MULLERIAN ANOMALY

OBJECTIVE: To study the etiology of primary amenorrhea in 19-aged woman. MATERIALS ANDMETHODS: Case report. RESULTS: I found that there were not gonads and organ of mullerian ducts origin on diagnostic laparoscopy. She was normal 46XX on chromosomal study and normal external genitalia except short vaginal canal. CONCLUSION: It is very rare that absent gonads and lack of mullerian ducts with 46XX female and primary amenorrhea.
Amenorrhea* ; Female ; Genitalia ; Gonads* ; Humans ; Laparoscopy ; Mullerian Ducts*