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ARM with vaginal atresia

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quote from "Pediatric Colorectal Conundrums: Case Studies: From Fundamental to Advanced (Pediatric Colorectal Surgery)" by Marc Levitt, Thomas Xu, Hussein Wissanji -

"Up to 10% of female infants with ARM have associated gynecologic anomalies, including an absent vagina in less than 1% of cases. Vaginoscopy is helpful to characterize the fistula and check for a vagina. An absent vagina may not be immediately apparent in the setting of a recto-vestibular fistula, especially if the fistula is mistaken for a vagina and a rectovaginal fistula is assumed to be present. In fact, absence of the vagina is only recognized about 50% of the time prior to anorectoplasty. When an absent vagina is suspected preoperatively, complete work-up can be pursued using US and/or MRI to delineate the pelvic organs although these are often unhelpful as the structures hoping to be seen are so small."

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quote from "Pediatric Colorectal Conundrums: Case Studies: From Fundamental to Advanced (Pediatric Colorectal Surgery)" by Marc Levitt, Thomas Xu, Hussein Wissanji -

"Determining the presence of an upper vagina and uterus is important for future planning, and this anatomy can be clarified with diagnostic laparoscopy. One scenario is visualization of an upper vagina. In such a case, it is recommended to note the anatomy, and at the time of puberty, the vagina can be mobilized via laparoscopy or laparotomy to reach the perineum for an anastomosis to the introitus. Most commonly, there are no Müllerian structures, just remnants of Fallopian tubes. In such a case, dilation of the introitus later in life is possible. Vaginal replacements with bowel are avoided as they do not produce good long-term outcomes and there is no immediate indication or urgency to perform this operation at this age. In such a case, a buccal neovagina is an excellent option later in life, and in the future a tissue engineered vagina may be an option."

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