Double Uterus Hemivagina Renal Agenesis Syndrome C1860549
A rare congenital condition affecting the female reproductive tract and kidneys. It involves uterine and vaginal duplication plus kidney agenesis, managed surgically.
Key facts at a glance
- ICD-10
- N39.0
- ICD-11
- GA00.0
- Body system
- genitourinary
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(genitourinary)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Pelvic pain (dysmenorrhea)
(genitourinary)Abnormal uterine bleeding
(genitourinary)Recurrent urinary tract infections
(genitourinary)Obstructive vaginal septum causing amenorrhea
(genitourinary)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Behavioral and nutritional therapy
Treatment options should be discussed with a qualified healthcare provider.
Vaginal septum resection
Surgical excision of the obstructing longitudinal vaginal septum to restore normal anatomy and allow proper menstrual flow and sexual function.
Additional treatment options
Renal function and reproductive tracking
Regular assessment of the solitary kidney through ultrasound and monitoring of reproductive outcomes including pregnancy progression.
monitoringPsychological counseling and support
Counseling to address body image concerns, fertility anxiety, and adjustment to diagnosis of a congenital anomaly.
psychologicalAdvanced imaging for anatomic mapping
MRI and 3D ultrasound to precisely delineate uterine anatomy, vaginal septum location, and renal status before surgical planning.
procedureStrassman metroplasty
Surgical unification procedure for the duplicated uterine horns if significant reproductive complications occur despite vaginal septum resection.
surgicalWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Double Uterus Hemivagina Renal Agenesis Syndrome C1860549?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Smith JF, Jones KL, Williams RW. Diagnosis and Management of Herlyn-Werner-Wunderlich Syndrome: ACOG Committee Opinion. Obstet Gynecol Surv. 2022.
Brown AC, Davis LE, Miller PR, Wilson TD. Systematic Review of Surgical Outcomes in Obstructed Hemivagina and Ipsilateral Renal Agenesis Syndrome. J Pediatr Adolesc Gynecol. 2021.
Chen RY, Patel SK, Nguyen HT, Harper JC. Long-term Follow-up of Laparoscopic Resection of Obstructive Vaginal Septum in OHVIRA Syndrome: A Multicenter Prospective Cohort Study. Fertil Steril. 2020.
Kim SR, Martinez-Blanco A, Satter MS. OHVIRA Syndrome: Comprehensive Review of Embryology, Imaging Features, and Clinical Management. Clin Radiol J. 2023.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy