Final Pr. ID: Poster #: CR-012
Imaging findings are diagnostic of obstructed hemi-vagina with ipsilateral renal agenesis (OHVIRA). This is a rare condition consisting of a triad of uterine didelphys (a type III Müllerian duct anomaly), hematocolpos, and ipsilateral renal agenesis. If any of these three features are absent, the diagnosis of OHVIRA is excluded. Clinical presentation is typically 2-12 months post menarche. Though rare pre-pubertal onset has been documented secondary to maternal hormonal influence, the mean age of presentation is 17 years old. Symptoms are typically non-specific including dysmenorrhea and a palpable pelvic mass.
Acute complications of OHVIRA include primarily infection of retained blood products resulting in pyohematocolpos or pyosalpinx. Rarely pelviperitonitis can occur if hematocolpos progresses to a hematosalpinx which ruptures. Long term complications include endometriosis, thought secondary to retrograde flow of blood products, which can lead to development of pelvic adhesions. Infertility issues are thought largely to be secondary to endometriosis and/or infection more so than uterine didelphys. Additional obstetric complications are greater in patients without surgical correction and most patients who carry a pregnancy to term have no increased difficulty.
Surgical correction with vaginal septectomy or division is the first line treatment for OHVIRA. If surgical correction is achieved early, the risk of complications including infertility are greatly diminished. Read More
Final Pr. ID: Poster #: CR-018
Show the imaging findings in a series of 4 OHVIRA syndrome, cases reported, in our hospital in a period of 10 years (May 2009 and May2019). Read More
Final Pr. ID: Poster #: CR-005
To demonstrate an unusual association of VECTERL and uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndromes in fetal MRI with post mortem MRI correlation. Read More
Authors: Supakul Nucharin
Final Pr. ID: Poster #: EDU-126
1. To describe imaging appearance of OHVIRA ( Obstructed hemivagina and ipsilateral renal agenesis) on different imaging modalities including Ultrasound, CT and MRI.
2. To understand the close interrelationship between urinary and reproductive system development during embryogenesis. Stress the importance of association of urinary and reproductive abnormalities so that if multicystic dysplastic kidney (MCDK), renal dysplasia or agenesis is seen in a female neonate, a careful search for associated genital malformation such as uterus didelphys with obstructed hemivagina (UDWOH) /OHVIRA should be performed.
3. To demonstrate the association of MCDK with OHVIRA and to further support the fact that renal agenesis seen in cases of OHVIRA may actually represent involuted MCDK rather than true renal agenesis.
4. To stress the importance of early diagnosis of OHVIRA (obstructed hemivagina with ipsilateral renal agenesis), so that correct management at the onset of puberty can prevent abdominal pain and dysmenorrhea and other complications.
Embryology of urinary and reproductive system will be discussed briefly. Imaging appearance of OHVIRA on different imaging modalities including US, CT and MRI with cases showing both prenatal and postnatal imaging will be presented. Read More
Authors: Sandhu Preet
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
Keywords: Mullerian Duct Anomalies, OHVIRA