Diagnosis, management, and outcome of obstructed hemivagina and ipsilateral renal agenesis (OHVIRA syndrome): Is there a correlation between MRI findings and outcome?

• Published in: Clinical imaging 2020-02, Vol.59 (2), p.172-178
• Main Authors: Gungor Ugurlucan, Funda, Dural, Ozlem, Yasa, Cenk, Kirpinar, Gamze, Akhan, Suleyman Engin
• Format: Article
• Language: English
• Subjects: Abdomen; Abnormalities, Multiple - diagnostic imaging; Abnormalities, Multiple - surgery; Adolescent; Adult; Age; Anomalies; Child; Complications; Congenital Abnormalities - diagnostic imaging; Congenital Abnormalities - surgery; Congenital diseases; Correlation; Diagnosis; Female; Hematocolpos; Hematometra; Herlyn-Werner-Wunderlich syndrome; Humans; Hysterectomy - methods; Infections; Infertility; Inflammatory diseases; Kidney - abnormalities; Kidney - diagnostic imaging; Kidney - surgery; Kidney Diseases - congenital; Kidney Diseases - diagnostic imaging; Kidney Diseases - surgery; Kidneys; Laparoscopy; Magnetic resonance imaging; Magnetic Resonance Imaging - methods; Menarche; Menstruation; Mullerian anomaly; NMR; Nuclear magnetic resonance; OHVIRA syndrome; Pain; Patients; Perineum; Sepsis; Septum; Statistical analysis; Surgery; Syndrome; Treatment Outcome; Ultrasonic imaging; Vagina; Vagina - abnormalities; Vagina - diagnostic imaging; Vagina - surgery; Vaginoplasty; Young Adult
• ISSN: 0899-7071; 1873-4499
• DOI: 10.1016/j.clinimag.2019.11.013

To review the experience of a single tertiary center with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA syndrome) and evaluate if MRI findings correlate with outcome. Patients (n = 32) diagnosed with OHVIRA syndrome between 2001 and 2019 were analyzed. Presenting symptoms, age of menarche, age at operation, MRI findings, management, and outcome were reviewed. In sagittal MRI planes, distance from hematocolpos to perineum was measured. Measurements were compared among patients who underwent single-stage vaginoplasty and hemihysterectomy. Mean age at diagnosis and mean age of menarche was 16.8 ± 6.4 (10−33) and 12.8 ± 1.0 (10–15). Main presenting complaints were abdominal pain/dysmenorrhea. Eighteen anomalies (56.3%) were on right side. Twenty-eight had ipsilateral renal agenesis, 3 patients had normal renal anatomy and one had unilateral multicystic dysplastic kidney. In MRI, 21 patients had hematocolpos, 11 patients had both hematocolpos and hematometra. 28 patients underwent single-stage vaginoplasty and vaginal septum resection. One had hemihysterectomy due to sepsis at presentation. Three patients had hemihysterectomy due to proximal vaginal septum and impossibility of vaginoplasty. During follow-up, 7 cases (21.9%) had married and 5 of these (71.4%) were pregnant or had delivered. Five patients had reoperation during follow-up. MRI images of 19 patients indicated mean distances from hematocolpos to perineum whom underwent vaginoplasty or hemihysterectomy were 33.9 ± 18.1 mm (10–79 mm) and 87.3 ± 11.0 mm (80–100), respectively (p = .009). Gold standard treatment of OHVIRA syndrome is single-stage vaginoplasty. Distance from hematocolpos to perineum in MRI may correlate with surgical outcome. Hemihysterectomy may be an alternative for extreme proximal vaginal septum or infectious complications. •Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare Mullerian anomaly which may be missed and diagnosed even in reproductive years and in infertility clinics.•This case series is the one with most number of cases treated with current diagnostic and surgical procedures.•Main methods of diagnosis are pelvic examination, pelvic ultrasound and magnetic resonance imaging.•Main method of treatment is surgery.•Some cases need hemihysterectomy, but the cases needing hemihysterectomy have not been defined clearly in the literature.•In this study magnetic resonance imaging findings correlate with surgical outcome. Therefore; this