|Year : 2016 | Volume
| Issue : 1 | Page : 28-30
Uterus didelphys with obstructed hemivagina and ipsilateral renal anomaly syndrome: A rare cause of cyclic abdominal pain in a young female
Kapish Mittal, Sharad Gupta, Puneet Mittal, Amit Mittal, Ranjana Gupta, Deepika Rao
Department of Radiodiagnosis, Maharishi Markandeshwar Institute of Medical Sciences and Research, Mullana, Ambala, Haryana, India
|Date of Web Publication||24-Jun-2016|
Dr. Puneet Mittal
448, Opp. Singla Memorial Hospital, Prem Basti, Sangrur - 148 001, Punjab
Source of Support: None, Conflict of Interest: None
Obstructed hemivagina and ipsilateral renal anomaly syndrome is a rare group of urogenital anomalies in which unilateral genital anomaly is associated with ipsilateral renal anomaly. We describe imaging findings in a 14-year-old female patient who presented with cyclical lower abdominal pain.
Keywords: Didelphys, magnetic resonance imaging, obstructed hemivagina and ipsilateral renal anomaly
|How to cite this article:|
Mittal K, Gupta S, Mittal P, Mittal A, Gupta R, Rao D. Uterus didelphys with obstructed hemivagina and ipsilateral renal anomaly syndrome: A rare cause of cyclic abdominal pain in a young female. Int J Adv Med Health Res 2016;3:28-30
|How to cite this URL:|
Mittal K, Gupta S, Mittal P, Mittal A, Gupta R, Rao D. Uterus didelphys with obstructed hemivagina and ipsilateral renal anomaly syndrome: A rare cause of cyclic abdominal pain in a young female. Int J Adv Med Health Res [serial online] 2016 [cited 2021 Mar 8];3:28-30. Available from: https://www.ijamhrjournal.org/text.asp?2016/3/1/28/184679
| Introduction|| |
Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, also known as Herlyn–Werner–Wunderlich syndrome, is a congenital anomaly in which unilateral mullerian duct anomaly is associated with ipsilateral renal anomaly, usually renal agenesis. It is an important cause of painful menstruation in young females. Diagnosis is often missed in early stages due to lack of awareness of this condition and due to concurrent normal menstrual flow in these patients. Awareness of this condition can help in suggesting this condition on sonography. Magnetic resonance imaging (MRI) can provide exquisite detail of the anomaly and its extent.
| Case Report|| |
A 14-year-old female patient who had recent onset of menarche presented with cyclical abdominal pain during menstruation. Menstruation was otherwise normal, occurring every 28 days and lasting for 3–4 days. On transabdominal examination, there was tenderness in the right lower abdomen. Laboratory investigations were within normal limits. Transabdominal ultrasound examination was requested which revealed two uterine horns with collection with echoes in the region of vagina [Figure 1]a and [Figure 2]a. Based on clinical and sonography findings, possibility of uterus didelphys with obstructed hemivagina was kept. Furthermore, there was nonvisualization of the right kidney. MRI confirmed the diagnosis [Figure 1]b, [Figure 2]b, and [Figure 3]. There was no evidence of hematosalpinx or any adnexal mass. The right ureter was not visualized. The patient underwent the resection of obstructive longitudinal vaginal septum with drainage of dark brown-colored chronic hematoma with complete resolution of vaginal collection [Figure 4] and relief of symptoms.
|Figure 1: Axial ultrasound (a) and magnetic resonance imaging T2--weighted (b) images showing uterus didelphys with two uterine horns|
Click here to view
|Figure 2: Axial ultrasound (a) and magnetic resonance imaging T2-weighted images (b) more inferiorly showing collection with echoes in dilated right hemivagina (long dotted white arrow in a). The collection is hyperintense on T2-weighted images with dependent hypointensity and fluid-fluid level indicating hemorrhagic contents. The left hemivagina is collapsed and compressed posterolaterally (short-dotted white arrow in a)|
Click here to view
|Figure 3: Coronal T2-weighted magnetic resonance image showing collection in the right hemivagina and right renal agenesis and normal left kidney|
Click here to view
|Figure 4: Postoperative oblique (a) and axial (b) ultrasound images showing complete resolution of the vaginal collection. White arrows in b indicate both hemivagina|
Click here to view
| Discussion|| |
OHVIRA syndrome was first described as early as 1922 and is also known as Herlyn–Werner–Wunderlich syndrome, named after the authors who described this entity in 1970s.
This condition results due to anomalous development of mullerian and mesonephric ducts during embryonic development. During early life, paired mullerian and mesonephric ducts develop from common dorsal ridge. Failure of fusion of mullerian ducts results in varying degree of anomalies ranging from uterus didelphys to septate uterus. Because of close proximity, anomalies of mullerian duct are frequently associated with ipsilateral mesonephric duct anomalies and vice versa. Mesonephric duct is responsible for the development of renal collecting system, renal pelvis, and ureter and also induces the development of renal parenchymal tissue. Its abnormal development can cause a variety of anomalies; renal agenesis being the most commonly associated renal anomaly with OHVIRA syndrome.,,,
This condition is usually diagnosed around the age of menarche when patient presents with cyclic pain during menstruation along with normal menstrual flow. There are also reports of seven cases of early diagnosis of this condition, before the onset of menarche, under the age of 5 years. This suggests that all the cases of unilateral renal agenesis should be screened for mullerian anomalies. Awareness of this condition and high index of suspicion is required for early diagnosis as failure to recognize this condition can result in inadvertent resection of the pelvic cystic lesion with uncertain eventual reproductive outcome.
Sonography is often the first imaging modality in cases of pelvic pain. Correlation with clinical history of normal menses and hematocolpos should suggest this diagnosis, especially if there is associated renal agenesis. MRI can provide exact detail of the associated anomalies and associated endometriosis, if present.
The preferred treatment of OHVIRA syndrome is directed at resection of vaginal septum and vaginoplasty which can be done as a single- or two-stage procedure. Hemihysterectomy which was performed earlier is no longer preferred because the reproductive outcome is similar with the conservative endovaginal procedure.
| Conclusion|| |
OHVIRA syndrome is a rare cause of cyclical abdominal pain in young females. Awareness of this condition and appropriate imaging is necessary for correct and timely diagnosis. Delayed diagnosis can lead to more accumulation of blood products and eventually hematosalpinx and endometriosis with worsening of reproductive outcome. Similarly, all patients with renal agenesis should be screened for mullerian anomalies and any pelvic “cystic mass” in these patients should not be resected without further imaging.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Orazi C, Lucchetti MC, Schingo PM, Marchetti P, Ferro F. Herlyn-Werner-Wunderlich syndrome: Uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases. Pediatr Radiol 2007;37:657-65.
Mandava A, Prabhakar RR, Smitha S. OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly) with uterus didelphys, an unusual presentation. J Pediatr Adolesc Gynecol 2012;25:e23-5.
Bhoil R, Ahluwalia A, Chauhan N. Herlyn Werner Wunderlich syndrome with hematocolpos: An unusual case report of full diagnostic approach and treatment. Int J Fertil Steril 2016;10:136-40.
Ozturk H, Dagistan E, Ozlu T. Role of OHVIRA syndrome in renal agenesis: A case report. Pediatr Urol Case Rep 2014;1:5-11.
Angotti R, Molinaro F, Bulotta AL, Bindi E, Cerchia E, Sica M, et al.
Herlyn-Werner-Wunderlich syndrome: An “early” onset case report and review of literature. Int J Surg Case Rep 2015;11:59-63.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]