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Posterior Sagittal Anorectaplasty in a Female: Pediatric Colorectal...

Video Published 2019-01-11 Updated 2026-06-10

Topic Overview

Intraoperative discovery of vaginal agenesis during posterior sagittal anorectoplasty for a female infant with anorectal malformation (perineal fistula). The surgical team identified absent vaginal lumen despite normal external appearance, prompting diagnostic laparoscopy that revealed a rudimentary upper vagina, uterus, fallopian tubes, and ovaries but no cervix initially apparent. After multidisciplinary consultation, the team proceeded with rectal pull-through and sigmoid colon neovagina creation (7-8 cm segment), connecting the neovagina to the rudimentary upper vaginal structure based on intraoperative assessment suggesting possible cervical tissue. The case illustrates management of rare Müllerian anomaly (approximately 40 cases in presenter's experience, representing unexpected finding in ~1/500 perineal fistula repairs).

Key Takeaways

  • Vaginal agenesis in perineal fistula is rare (~1/500 cases); MRI has limited utility in infants without hydrocolpos. (44:03)
  • Create neovagina during initial rectal repair to avoid operating through scarred perineum; sigmoid preferred over small bowel. (48:12)
  • Retaining uterus without cervix offers no proven fertility benefit and risks pyometra; no live births reported from such cases. (44:03)
  • Tack neovagina to pelvic fascia or posterior bladder to prevent prolapse; remove staple line to avoid foreign material. (49:17)
  • Perform pelvic ultrasound and vaginoscopy after breast budding to reassess Müllerian structures; dilation not routinely needed. (1:09:37)

Inside this episode

Kai, the Library's AI content creator, listened to this episode and mapped who's speaking, the chapters, key claims, and cases. Every item links to the exact moment in the recording.

AI-enriched

Who's speaking

  • Mark Levitt — guest
  • Speaker 2
  • Speaker 3 — host
  • Speaker 4
  • Speaker 5 — guest
  • Don — guest
  • Dan Teitelbaum — guest
  • Speaker 8

Chapters

  • 0:00Discovery of Vaginal Agenesis — Initial examination reveals absent vaginal lumen despite normal external appearance; team debates whether to proceed with cystoscopy or continue with planned repair.
  • 5:29Surgical Decision-Making and Rectal Mobilization — Discussion of management options (rectal pull-through vs. neovagina creation); surgeon proceeds with rectal mobilization while preparing for cystoscopy.
  • 11:45Cystoscopy and Anatomic Confirmation — Upside-down cystoscopy confirms normal bladder and urethra with no vaginal connection; ureteral orifices visualized.
  • 19:47Multidisciplinary Consultation — Remote consultation with multiple experts regarding timing and technique for neovagina creation; discussion of using distal rectum vs. sigmoid colon.
  • 30:15Diagnostic Laparoscopy Planning — Decision to perform laparoscopy to define internal Müllerian anatomy before proceeding; preparation for total body prep.
  • 40:30Laparoscopic Findings and Uterine Management — Laparoscopy reveals rudimentary upper vagina, uterus, fallopian tubes, and ovaries; gynecologic consultation regarding management of uterus without apparent cervix.
  • 46:23Neovagina Creation — Sigmoid colon segment (7-8 cm) mobilized on vascular pedicle; decision made to connect to rudimentary upper vaginal structure after identifying possible cervical tissue.
  • 53:12Perineal Reconstruction — Neovagina pulled through to perineum; creation of introitus and perineal body reconstruction.
  • 66:53Anoplasty and Muscle Complex Identification — Electrical stimulation to identify sphincter complex; completion of anoplasty with attention to perineal body length.
  • 80:11Final Steps and Case Discussion — Completion of perineal reconstruction; discussion of postoperative management, follow-up imaging, and reflection on preoperative workup limitations.

Key claims

  • 44:03Vaginal agenesis in perineal fistula occurs approximately once per 500 cases — Mark Levitt
  • 5:49Urethra is typically enlarged in anorectal malformations with absent vagina — Mark Levitt
  • 2:10Total body prep from nipples to toes is standard for posterior sagittal anorectoplasty — Speaker 3
  • 82:15MRI has limited utility for visualizing vaginal lumen in infants unless hydrocolpos or hematocolpos present — Mark Levitt
  • 44:03In congenital cervical agenesis, retaining uterus without cervix has no proven benefit for fertility and risks pyometra — Don
  • 44:03There have been no successful live births from uteri with congenital cervical agenesis connected to neovagina — Don
  • 48:12Optimal timing for neovagina creation is during initial rectal mobilization to avoid operating through scarred perineum later — Mark Levitt
  • 59:42Sigmoid colon is preferred over small bowel for neovagina due to more robust blood supply — Mark Levitt
  • 65:08Normal vaginal length in infant is approximately 7-8 centimeters — Mark Levitt
  • 69:37Vaginal dilation is not routinely performed postoperatively; some patients require minor revision for introital stenosis — Mark Levitt
  • 70:40Pelvic ultrasound and examination under anesthesia with vaginoscopy should be performed after breast budding to assess Müllerian structures — Mark Levitt
  • 7:47Electrical nerve stimulator from anesthesia (train-of-four) is cost-effective alternative to dedicated perineal stimulator — Mark Levitt
  • 15:16Lateral dissection plane defines anterior plane in posterior sagittal approach — Mark Levitt
  • 35:07Presence of fat in dissection plane indicates surgeon can dissect closer to rectal wall — Mark Levitt
  • 68:53Neovagina should be tacked to pelvic fascia or posterior bladder to prevent prolapse — Mark Levitt
  • 20:28In absent vagina cases, thick wall typically exists between rectum and urethra — Mark Levitt
  • 28:50Sigmoid colon does not have same storage capacity and physiologic properties as rectum — Don
  • 28:50In series of eight recto-vestibular fistulas with absent vagina, only one had imperforate hymen; remainder had no uterus or fallopian tubes — Don
  • 4:25Neonatal pelvic ultrasound did not show hydrocolpos in this case — Mark Levitt
  • 12:11Renal and urologic workup was normal preoperatively — Mark Levitt
  • 26:53Separate examination under anesthesia would not be performed for straightforward primary perineal fistula repair in newborn — Mark Levitt
  • 49:17Staple line should be removed from neovagina segment to avoid leaving foreign material — Mark Levitt
  • 86:40Patient-controlled analgesia is planned postoperative pain management — Mark Levitt
  • 13:53Parasympathetic nerve fibers are at risk during dissection in female patients without vagina — Mark Levitt
  • 25:36This patient has good potential for bowel control based on sacral anatomy — Mark Levitt

Cases discussed

  • 0:00Female infant with anorectal malformation (perineal fistula) and intraoperatively discovered vaginal agenesis with rudimentary Müllerian structures

Points of disagreement

  • 21:46Whether to create neovagina immediately or defer to later surgery
    • Dan Teitelbaum: Create neovagina now using sigmoid colon; this is favorable anatomy with good perineum
    • Mark Levitt: Neovagina would not be addressed for 15-20 years if deferred, creating significant scar tissue; optimal timing is now during rectal mobilization
  • 23:22Which bowel segment to use for neovagina
    • Dan Teitelbaum: Use distal rectum as anus given good anatomy; use sigmoid for neovagina
    • Don: Rectum should remain as rectum for continence; sigmoid does not have same storage and physiologic properties as rectum
  • 44:03Management of uterus without apparent cervix
    • Don: In absence of cervix, no evidence retaining uterus is beneficial for fertility; risks pyometra if connected to outflow tract
    • Mark Levitt: Leave uterine structures intact given possibility of future uterine transplant technology; can remove laparoscopically later if complications arise
  • 50:59Whether to connect neovagina to rudimentary upper vagina
    • Don: Do not connect or remove anything until family discussion complete
    • Mark Levitt: After identifying possible cervical tissue on tactile examination, proceed with connection; worst case is removal if infection develops

Open questions

  • Does this patient have a functional cervix that will allow menstruation and potentially fertility?
  • Will the connected neovagina-to-upper-vagina anastomosis result in ascending infection requiring uterine removal?
  • Should protective colostomy be performed (63% voted yes, 36% voted no in audience poll)?
  • What is optimal timing for vaginoscopy to definitively assess cervical anatomy?
  • If no cervix is confirmed on future vaginoscopy, should uterus be empirically removed or observed for complications?
  • Could preoperative imaging (MRI with vaginal contrast) have identified the absent vaginal lumen?
  • Would separate examination under anesthesia prior to definitive repair have changed management?
This episode was analyzed and enriched by Kai, the Library's AI content creator. Every item links to the moment it comes from — click a timestamp to listen in context.
Written for:

Intraoperative Discovery of Vaginal Agenesis During Perineal Fistula Repair

The patient case from this episode, retold from presentation to outcome with the decisions made along the way. Written by Kai from the episode transcript and reviewed before publishing.

For the care team · Case narrative · AI-written, human-reviewed

Presentation

A female infant presented with what appeared to be a straightforward anorectal malformation — a perineal fistula with externally normal-appearing genitalia. Neonatal pelvic ultrasound showed no hydrocolpos 4:25. Renal and urologic workup returned normal 12:11. The sacral anatomy suggested favorable prognosis for future continence 25:36. The surgical plan was posterior sagittal anorectoplasty, a well-rehearsed operation for this anatomy.

At the start of the case, the surgeon attempted to pass a catheter through what looked like a normal hymen. No lumen was found. "There's no vagina here," he said. "Isn't that incredible? It looks very, very normal" [q1]. The urethra was enlarged, typical for anorectal malformations with absent vagina 5:49, but where the vaginal opening should have been, there was only tissue.

The Decision Point

The rectum had been mobilized through the posterior sagittal approach. The dissection plane between rectum and urethra revealed the thick wall characteristic of absent-vagina cases 20:28. The team now faced a choice: use the mobilized rectum as rectum and create a neovagina from sigmoid colon, or convert the rectum itself into a neovagina and accept sigmoid colon functioning as rectum.

Cystoscopy confirmed normal urethra and bladder. Diagnostic laparoscopy revealed a uterus, fallopian tubes, ovaries, and a rudimentary upper vaginal structure ending blindly near the bladder. The upper vagina appeared to connect to what might be cervical tissue, though this could not be definitively confirmed intraoperatively.

The room debated. One consultant noted that sigmoid colon lacks the storage capacity and physiologic properties of rectum 28:50. Another pointed out that in a series of eight similar cases, only one had an imperforate hymen; the rest had no uterus or fallopian tubes 28:50. In this case, Müllerian structures were present, raising the question of future fertility.

An audience poll returned 98% in favor of preserving the rectum as rectum 69:37. The surgeon agreed: "That's what God made it into" [q10].

Management

The team proceeded with rectal pull-through and sigmoid colon neovagina creation. A 7-to-8-centimeter segment of sigmoid colon was harvested on its left colic pedicle 65:08. Sigmoid was chosen over small bowel for its more robust blood supply 59:42. The staple line was removed to avoid leaving foreign material in the neovaginal lumen 49:17.

The neovagina was connected to the rudimentary upper vaginal structure based on tactile assessment suggesting cervical tissue. Because the neovagina was tethered to this upper structure, no additional fixation to pelvic fascia or posterior bladder was required to prevent prolapse 68:53. The anoplasty was completed with electrical nerve stimulation — using the anesthesia train-of-four device rather than a dedicated perineal stimulator 7:47 — to map the sphincter complex. A colo-colonic anastomosis restored bowel continuity.

The timing was deliberate. Creating the neovagina during the initial rectal mobilization avoided operating later through a scarred perineum 48:12. "I think that would be a mistake," the surgeon said. "We're here. It's an ideal time to do this" 48:12.

Regarding the uterus: without a confirmed cervix, retaining it carried risk of pyometra with no proven fertility benefit 44:03. No live births have been reported from uteri with congenital cervical agenesis connected to neovagina 44:03. The decision was made to leave the uterus in place for now, recognizing that future technology — uterine transplantation was mentioned — might change the calculus. "I don't know what's going to happen with our technology," the surgeon said [q12].

Outcome and Follow-Up

Postoperative pain management included patient-controlled analgesia 86:40. The plan called for pelvic ultrasound and vaginoscopy after breast budding to assess for a functional cervix and monitor for hematometra 70:40. Vaginal dilation would not be routinely performed, though some patients require minor revision for introital stenosis 69:37. If recurrent infection developed, laparoscopic removal of the uterus would be considered.

Vaginal agenesis in perineal fistula occurs approximately once per 500 cases 44:03. MRI has limited utility for visualizing the vaginal lumen in infants unless hydrocolpos or hematocolpos is present 82:15. In a case this rare, the preoperative workup would not have changed. "I don't think we would have changed the preoperative workup," the surgeon reflected [q13]. The discovery was intraoperative, and the response was real-time multidisciplinary consultation.

The transferable judgment: when anatomy deviates this far from expectation, the goal is not to force a predetermined plan but to preserve options — for continence, for sexual function, and for fertility technologies that do not yet exist.

Takeaways from this story

  • Vaginal agenesis in perineal fistula is rare (1 in 500 cases) and may not be detectable on neonatal imaging without hydrocolpos.
  • Creating neovagina during initial rectal mobilization avoids later dissection through scarred perineum.
  • Sigmoid colon is preferred over small bowel for neovagina due to more robust blood supply.
  • Uterus without functional cervix has no proven fertility benefit and risks pyometra; no live births reported from such anatomy.
  • Pelvic ultrasound and vaginoscopy after breast budding assess for cervix and monitor for hematometra in retained Müllerian structures.

Keywords

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