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Diagnosis, management, and outcome of obstructed hemivagina and ipsilateral renal agenesis (OHVIRA syndrome): Is there a correlation between MRI findings and outcome?
- Source :
- Clinical Imaging. 59:172-178
- Publication Year :
- 2020
- Publisher :
- Elsevier BV, 2020.
-
Abstract
- Aim 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. Methods 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. Results 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). Conclusions 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.
- Subjects :
- Adult
Abdominal pain
medicine.medical_specialty
Adolescent
Hysterectomy
Kidney
Congenital Abnormalities
030218 nuclear medicine & medical imaging
Young Adult
03 medical and health sciences
0302 clinical medicine
medicine
Hematocolpos
Humans
Vaginal septum
Abnormalities, Multiple
Radiology, Nuclear Medicine and imaging
Child
Hematometra
business.industry
Gold standard
Syndrome
medicine.disease
Magnetic Resonance Imaging
Perineum
Treatment Outcome
medicine.anatomical_structure
030220 oncology & carcinogenesis
Vagina
Vaginoplasty
Female
Kidney Diseases
Radiology
medicine.symptom
Presentation (obstetrics)
business
Subjects
Details
- ISSN :
- 08997071
- Volume :
- 59
- Database :
- OpenAIRE
- Journal :
- Clinical Imaging
- Accession number :
- edsair.doi.dedup.....d9cefaa6e55f6bf36373f1bfc50e1480