Cloacal Malformation/Urogenital Sinus





KEY FACTS


Terminology





  • Cloaca is single cavity present in early embryonic development when bladder, genital tract, and bowel are all connected



  • Spectrum of abnormal anatomy is related to timing of developmental arrest




    • Range from urogenital sinus (2 perineal openings) to cloacal dysgenesis (no perineal opening)



    • Classic cloaca defined as coalescence of urethra, vagina, and hindgut into common channel draining through single perineal orifice (exclusively in females)




Imaging





  • Most common presentation is cystic pelvic mass in female fetus; “cyst” is grossly dilated vagina (hydrocolpos)



  • Uterine and vaginal duplication is observed in ~ 80% of cases in which hydrocolpos is present




    • Vaginal duplication creates linear septation within conical fluid collection




  • Abnormal perineum; absent anal dimple



  • High percentage have additional abnormalities, most commonly genitourinary, bowel, and lumbosacral



Scanning Tips





  • Vagina is most distended structure and appears as cystic pelvic mass




    • Scan in coronal plane looking for vertical septum



    • Even if no septum is present, may still see fluid-debris level (vaginal secretions ± meconium mixing with urine)




  • Do not confuse with bladder




    • Bladder may be compressed by distended vagina



    • Be very suspicious if female fetus is sent for “urethral atresia”; far more likely to be cloacal malformation




  • When any lower GI or GU abnormality is suspected, perform high-resolution images of perineum looking for abnormal genitalia and absent anal dimple




    • If abnormal, highly suspicious for cloacal malformation even if no cystic mass is present








Sagittal graphic shows a common channel (cloaca) with hydrocolpos compressing the rectum and bladder. The distended vagina is the most obvious finding on US and may be confused with the bladder.








Coronal US through the pelvis of a female fetus shows fluid-fluid levels representing layering debris in obstructed, duplicated vaginas (note the linear septum ). Debris results from the mixing of urine with vaginal secretions ± meconium. This is the most specific finding for a cloacal malformation.

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Nov 10, 2024 | Posted by in ULTRASONOGRAPHY | Comments Off on Cloacal Malformation/Urogenital Sinus

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