Abstract
BACKGROUND: Obstructed hemi-vagina and ipsilateral renal anomaly syndrome is a rare congenital condition, which is described in a combination of uterus didelphys and obstructed hemi-vagina with ipsilateral renal agenesis. Until now, the diagnosis of this anomaly is challenging as there are diverse symptoms depending on pre-puberty and post-puberty age onset. Early diagnosis of this syndrome allows for improvement of quality of life and prevents fertility-related complications. CASE REPORT: We present the case of a 13-year-old Vietnamese girl who was admitted to the emergency department with colicky lower abdominal pain and a tender cystic mass in the left vaginal wall. Her menarche started 6 months ago, and she had an experience of progressive menstruation-related pain. The ultrasound and magnetic resonance imaging identified presence of uterus didelphys, an absence of left kidney associated with a menstrual-blood-retained mass from the uterus cavity to the left side of vagina (hematocolpos). The patient underwent a vaginal excision to drain blood-containing mass and was discharged from hospital 5 days after the operation. The patient and her parents have been provided with a detailed follow-up plan to prevent the complications of renal failure and infertility. CONCLUSION: The possibility of obstructed hemi-vagina and ipsilateral renal anomaly syndrome should be considered in both pre-puberty and post-puberty girls who present with acute lower abdominal pain. Ultrasound plays a crucial role in making an early diagnosis of this condition, while magnetic resonance imaging allows for classification and prediction of fertility capacity. Vaginal excision is a minimally invasive procedure to relieve the pain and prevent the adverse effects.