Case Summary: 1yoF with vaginal atresia, ASD, anomalous venous return, polydactyly. No tethered cord, L hydronephrosis and hydrocolpos. Percutaneous drainage of hydrocolpos with 400 ml fluid. Catheter dislodged on multiple occasions finally laparoscopic guided placement. Open heart repair at 6 months of age. Contrast study with significant distance between vagina and perineum. EUA at 3 months post op.
Takeaway: Important to place drain until repair due to fluid re-accumulation. Technically easier to repair when child is young. Due to distance between vagina and perineum, will need to go from above (laparotomy) and below (perineum). Ensure bowel prep prior in case trans-anorectal approach becomes necessary or if bowel is needed to create vagina. Patient may need introitoplasty when older to make the introitus adequate size, at the skin level, but should have normal sexual and reproductive function.
Patient Case Video: