MRI-based Stratification and Surgical Management of Hydrocolpos in Children and Adolescents.

This study looked at a condition called hydrocolpos, where girls are born with a blockage in their vaginal area that prevents normal fluid drainage. The researchers examined 13 girls (ages 1 month to 14 years) who had this condition and developed a new way to classify how severe the blockage is using MRI scans. They use a bone landmark (the pubic bone) as a reference point to determine if the blockage is "low," "intermediate," or "high" - which helps doctors plan the best surgical approach. What's particularly relevant for families dealing with Bardet-Biedl Syndrome is that nearly half of the girls in this study (6 out of 13) had genetic syndromes, with several specifically having Bardet-Biedl or related conditions. Three of these girls also had chronic kidney disease, which is another common feature of Bardet-Biedl Syndrome. The study shows that children with these genetic syndromes are at higher risk for this type of vaginal blockage. The researchers used different surgical techniques depending on where the blockage was located, with generally good results. However, they found that girls with higher-level blockages were more likely to have complications like scarring or narrowing after surgery. This new classification system using MRI scans could help doctors better plan surgeries and potentially improve outcomes for children with these conditions, including those with Bardet-Biedl Syndrome.

In this report we present a new anatomical stratification for vaginal obstruction (hydrocolpos) based on MRI findings while referring the level of obstruction to a fixed bony landmark (the pubic symphysis). This new approach can overcome the limitations of current classifications, which are prone to approximation errors during measurement and fail to account for variations in body mass across different age groups.Data of cases diagnosed with vaginal obstruction were retrospectively analyzed. Cases of cloaca with vaginal obstruction were excluded. MRI confirmed the diagnosis and enabled classification of the level of vaginal obstruction relative to the pubic symphysis in the mid-sagittal plane as low, intermediate, or high-opposite the lower, mid, or upper end of the pubic symphysis, respectively.A total of 13 girls presented with vaginal obstruction during the period 2010 through 2024. Their age ranged between 1 month and 14 years (mean: 54 months; median: 18 months). Three cases presented in the neonatal period with antenatal diagnosis of hydrocolpos, while six cases were referred later during infancy/childhood from other centers. Another group of adolescent girls (four cases) presented with a clinical picture of cryptomenorrhea. The cause of vaginal obstruction was imperforate hymen in one, vaginal atresia in six, persistent urogenital sinus (five cases), and one case of obstructed hemi-vagina. In this series, six cases (46%) had features related to genetic syndromes (Bardet Biedl/ McKusick-Kaufman spectrum). Chronic parenchymatous renal disease was present in three cases among other syndromic features of Bardet Biedl syndrome, in addition to another case with obstructed left hemi-vagina that had absent left kidney (Herlyn-Werner-Wunderlich syndrome). Surgical techniques included simple excision of distal obstructing membrane (four cases), abdominal assisted vaginoplasty (two cases), vaginal pull-through (four cases), simple introitoplasty (one case), urogenital sinus mobilization (one case), and division of longitudinal vaginal septum for a case of obstructed hemi-vagina. Vaginal stenosis or retraction occurred in three cases with intermediate to high-level obstruction-two following abdominal assisted vaginoplasty and one after vaginal pull-through.MRI-based stratification of vaginal obstruction using the pubic symphysis as a reference provides a practical and reproducible approach for surgical planning.This is a case series (level IV evidence).