Vaginal atresia with hematometrocolpos, ectopic right ureter and multicystic dysplastic right kidney
Presentation
The patient presented with 5 days of acute pelvic pain. They had experienced other similar episodes of pain over the previous 6 months, but none as severe as this. The pain was predominantly within the right side of the pelvis, with some associated back pain. They were previously fit and well, with no previous imaging of this region. No antenatal or neonatal concerns were noted. They had yet to start menstruating. There was no vomiting, diarrhea, urinary symptoms, or fever associated with the symptoms. Their observations were all within the normal range. They had an elevated CRP (110 mg/L), but their other bloods (FBC, U&E) were within the normal range. They were reviewed by pediatrics and the general surgeons and were referred for an ultrasound to look for appendicitis or ovarian pathology.
Patient Data
The ultrasound appearances are in keeping with hematometrocolpos, with a dilated uterine cavity filled with heterogeneous fluid, and a further dilated fluid-filled structure inferior to and contiguous with the uterus.
The uterus is deviated to the right, but no second uterine cavity is visible on the left. The right ovary is normal, but the left is not visualized.
Within the right flank, there are also several simple cystic structures, with no normal right kidney identified.
Conclusion:
Hematometrocolpos - gynecology opinion recommended.
Possible Mullerian duct abnormality and multicystic dysplastic right kidney - pelvic MRI recommended to further assess.
The patient was seen by the gynecology team and taken to theater for a hymenectomy to relieve the hematometrocolpos.
A hymenectomy was performed, but no vaginal opening was identified.
The patient underwent an urgent pelvic MRI the following day.
The MRI demonstrates complex anatomy. It confirms the presence of a single obstructed uterus, cervix, and upper vagina. No lower vagina is identified (see sagittal views).
There is also a right hematosalpinx. Both ovaries are present and normal in appearance.
No normal right renal tissue is identified. There are multiple cystic structures within the right flank, and a tubular structure which inserts into the right inferolateral aspect of the dilated cervix/upper vagina. This tubular structure passes superiorly towards the right flank cysts. Although a definite connection is not visualized, this most likely represents an ectopic right ureter, and the right flank cysts an involuted multicystic dysplastic kidney.
The ectopic ureter and right flank cysts are bright on the T1 fat sat sequences, suggesting they also contain blood products, most likely refluxed from the hematometrocolpos via the ectopic ureter.
There is a small volume of free blood and fluid within the pelvis.
Normal left kidney and bladder.
Case Discussion
The patient was commenced on the COCP before discharge to prevent further periods. She also had a short course of antibiotics due to an episode of pyrexia and a raised CRP.
She was referred to the tertiary gynecological center for definitive management.
This is a very unusual case of a complex congenital abnormality. Renal anomalies are not unusual with Mullerian duct abnormalities (e.g. OHVIRA syndrome), but less common with lower vaginal atresia (which in itself is rare).
OHVIRA syndrome was initially suspected based on the ultrasound appearances (hematometrocolpos and multicystic dysplastic right kidney), but the renal anomaly is actually due to an ectopic ureter inserting into the obstructed cervix/upper vagina. An ectopic ureter usually leads to issues with continence in girls, as urine drains via the vagina. In this case as the upper vagina was obstructed, the kidney presumably became obstructed and dysplastic as there was no route for drainage. Antenatal imaging was not available, but there were no recorded concerns of renal anomalies, and no history of UTIs as an infant.
Hematometrocolpos is an uncommon but important cause for acute lower abdominal pain in girls of this age. This case demonstrates the importance of the clinical history (the cyclical nature of the pelvic pain was not recorded by the clinical team on the ultrasound request, nor was the menstrual history). Unfortunately, due to patient discomfort, it was difficult to delineate the anatomy on ultrasound, but the MRI demonstrates it clearly.
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