Cheng Jocelyn,  Leesmidt Kantheera,  Liu Amanda,  Young Victoria,  Davda Sunit,  Berger-chen Sloane,  Courtier Jesse

Final Pr. ID: Poster #: EDU-039

Three-dimensional (3D) imaging has emerged as a valuable tool in diagnosing pediatric Müllerian duct anomalies (MDAs), offering superior anatomical visualization and assessment compared to traditional imaging techniques with two-dimensional ultrasound. MDAs are frequently associated with other congenital anomalies of the cervix, vagina, or urinary tract, and are a common cause of infertility and pregnancy loss after adolescence.

In pediatric patients, MDAs are often discovered incidentally during imaging for other conditions. Magnetic resonance imaging (MRI) is the preferred modality for evaluating MDAs in children, providing multiplanar capabilities and detailed soft tissue characterization while avoiding ionizing radiation. MRI protocols for MDAs typically include T2-weighted sequences for visualizing uterine morphology and T1-weighted sequences to detect blood products in cases of associated endometriosis or hematometra .

The use of 3D MRI techniques allows for enhanced depiction of uterine and vaginal anatomy, and is particularly advantageous in differentiating complex anomalies such as septate and bicornuate uteri. Additionally, 3D reconstructions can provide a virtual hysterosalpingogram (MR-HSG), offering a comprehensive assessment of the reproductive tract without the need for invasive procedures. 3D imaging's accuracy extends to the detection of associated renal anomalies, given the concurrent development of the urinary and reproductive systems. Identifying these anomalies early is crucial for planning surgical interventions that may involve both gynecologic and urologic components .

Overall, 3D imaging significantly enhances the diagnostic accuracy and management of pediatric Müllerian duct anomalies. By providing detailed anatomical insights and allowing for non-invasive classification, it plays a critical role in guiding treatment strategies, reducing the need for repeat surgeries, and improving long-term reproductive outcomes for affected patients.

The purpose of this educational exhibit is to:
1. Review the embryology, classification, and imaging features of Müllerian duct anomalies using 3-dimensional imaging
2. Review the indications and techniques for surgical management of Müllerian duct anomalies in pediatric patients
3. Provide sample cases and clinical courses of patients with specific Müllerian duct anomalies with tips for implementation of 3-D imaging
4. Allow learners to test their knowledge with a quiz. Read More

Authors:  Cheng Jocelyn , Leesmidt Kantheera , Liu Amanda , Young Victoria , Davda Sunit , Berger-chen Sloane , Courtier Jesse

Keywords:  MRI, Mullerian Duct Anomalies, 3D Imaging

Frank Matt,  List Jeb,  Harris Katherine

Final Pr. ID: Poster #: CR-07 (R)

12 year old female presented with history of ureteral re-implantation and severe pelvic pain for a month. Pelvic ultrasound showed a bi-lobed hypoechoic mass in the pelvis markedly distended with internal intermediate and low level echoes and septations suggestive of hematometrocolpos thought to be secondary to imperforate hymen. Subsequent MRI evaluation of the pelvis demonstrated a complete septate uterus with longitudinal vaginal septum, atresia of the lower 1/3 of the vagina, and hematocolpos. The patient was placed on Lupron and referred for reconstructive surgery. Read More

Authors:  Frank Matt , List Jeb , Harris Katherine

Keywords:  Septate uterus, longitudinal vaginal septum, Mullerian duct anomaly, Septate uterus MRI, longitudinal vaginal septum MRI

Denham Chloe,  Lupo Andrew,  Sokkary Nancy,  Linam Leann

Final Pr. ID: Poster #: EDU-043

Primary amenorrhea, defined as failure to reach the first menstrual cycle by the age of 15 or 3 years after breast development, presents a complex diagnostic challenge. From congenital anomalies to endocrine disorders, there is a wide range of potential etiologies; therefore, a multidisciplinary and comprehensive approach is crucial for accurate and timely diagnosis and management. Radiologic imaging often plays an important role in this process, particularly in the identification of structural abnormalities and assessing the presence of internal reproductive structures that can guide potential management decisions. In this educational exhibit, we will review etiologies of amenorrhea and their associated radiologic imaging, utilizing examples from our institution. Additionally, we aim to provide key findings and tips that can help the pediatric radiologist have a clearer understanding of the clinical questions that gynecologists seek to answer when ordering imaging for the evaluation of primary amenorrhea. Read More

Authors:  Denham Chloe , Lupo Andrew , Sokkary Nancy , Linam Leann

Keywords:  Radiology Education, Mullerian Duct Anomalies, Pelvis

Merriam Caroline,  Ehrlich Lauren

Final Pr. ID: Poster #: CR-031

Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome, also known as Herlyn-Werner-Wunderlich Syndrome, is a rare congenital anomaly of the female urogenital tract. OHVIRA syndrome results from abnormal development of the Mullerian duct during weeks 10-12 of gestation. OHVIRA syndrome is characterized by a classic triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal anomaly. Clinical presentation of OHVIRA syndrome depends on the degree of hemivaginal obstruction, with Type 1 defined as complete obstruction and Type 2 characterized by incomplete obstruction. In patients with complete hemivagina obstruction, OHVIRA syndrome may manifest in adolescence with pelvic distention and cyclic pain secondary to hematometrocolpos. Several renal anomalies have been described in OHVIRA syndrome including renal agenesis, multicystic dysplastic kidney, and duplicated collecting system. In this report, we present two cases of OHVIRA syndrome with different manifestations of unilateral renal anomaly.

The first case features a 12-year-old female who presented with heavy menstrual bleeding. Transpelvic ultrasound demonstrated possible uterine septation and a dilated cervix containing heterogeneous avascular material. Subsequent pelvic MRI revealed duplicated uterine horns with two separate cervices, consist with uterus didelphys. On MRI, the left cervix was distended with non-enhancing debris, suggestive of obstructed left hemivagina. No left kidney was identified in the left renal fossa or elsewhere in the abdomen or pelvis on renal ultrasound. Multiple non-communicating cysts were seen in the left abdomen, raising suspicion for multidysplastic left kidney with arrested ascent.

The second case involves a 10-year-old prepubertal female who presented from an outside hospital for evaluation of a uterine anomaly, which was incidentally seen on MRI pelvis performed for characterization of sacrococcygeal teratoma. Transpelvic ultrasound and pelvic MRI at our institution showed duplicated uterine horns with limited visualization of the cervices due to small size. Differential considerations included uterus didelphys and bicornuate uterus, and follow-up imaging was recommended after puberty for definitive diagnosis. MRI pelvis obtained 4 years later demonstrated complete duplication of the uterus, cervix, and vagina with obstruction of the right hemivagina. MRI pelvis also demonstrated right renal agenesis, consistent with OHVIRA syndrome. Read More

Authors:  Merriam Caroline , Ehrlich Lauren

Keywords:  Mullerian Duct Anomalies, Congenital

Myers Ross,  Li Tianyang,  Wu Jennifer,  Sadowsky David,  Paul Mary,  Gerard Perry,  Brudnicki Adele,  Lecompte Lesli

Final Pr. ID: Poster #: EDU-034

Müllerian Duct Anomalies (MDA) are a broad category of congenital deformities of the urogenital structures due to abnormal development, fusion, or resorption of the fetal Müllerian Ducts. By definition, the Müllerian Ducts are the paired fetal structures that are located medially to the Wolffian Ducts and develop into the Fallopian Tubes, uterus, cervix, and upper two-thirds of the vagina. The multitude of symptoms associated with MDAs vary broadly in severity, ranging from patients with no symptoms and incidentally discovered MDAs to patients with primary infertility, recurrent pregnancy loss and other reproductive problems.

There are well documented associations of MDAs with other genitourinary malformations. The most commonly reported is renal agenesis, which accounts for up to 30% of all associated renal anomalies in the setting of MDA, however abnormalities including ureteral remnants with ectopic insertion or collecting system duplication have been reported as well. The overall incidence of MDAs varies, but have been estimated to be approximately 1-5% within the general population and up to 13-25% in patients with recurrent pregnancy loss.

The most commonly accepted system of classification of the various types of MDAs is that of the American Fertility Society, which divides MDAs into seven categories. However, there is often confusion as to how to categorize MDAs demonstrating characteristics of more than one class. Medical imaging is of essential importance for both this reason and for appropriate planning of therapy. The imaging of suspected MDAs involves the implementation of a combination of 3D Ultrasound, Magnetic Resonance Imaging, Sonohysterography, Hysterosalpingraphy and/or laparoscopy. Sonography and MRI have the benefits of being the least invasive methods of diagnosis.

The goal of our educational exhibit will provide an overview of the embryology and pathophysiology related to a broad variety Müllerian Duct Anomalies. We will discuss the common imaging techniques employed in the workup of patients with Müllerian Anomalies. In addition, we provide a plethora of interesting cases from our home institution including a case of Mayer-Rokitansky-Küster-Hauser Syndrome, Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA), cloacal abnormality, bicornuate uterus and other complex cases utilizing a variety of imaging modalities. Read More

Authors:  Myers Ross , Li Tianyang , Wu Jennifer , Sadowsky David , Paul Mary , Gerard Perry , Brudnicki Adele , Lecompte Lesli

Keywords:  Mullerian Duct Anomalies, OHVIRA