Volume 37, October 2025, 151543Author links open overlay panel, , , , , , Introduction

Anorectal malformations (ARM) comprise a spectrum of congenital anomalies arising from abnormal embryonic development of the anus and rectum. Similarly, müllerian anomalies encompass a broad range of anatomical differences resulting from atypical development of the uterus, fallopian tubes, cervix and vagina. The vagina has two distinct embryologic origins, with the upper two-thirds developing from the paramesonephric (also called müllerian) ducts, and the lower one-third arising from the urogenital sinus1. Vaginal agenesis can be total, also called cervicovaginal agenesis, or partial, also called distal vaginal agenesis. Surgical management is determined by the extent of vaginal agenesis and the presence or absence of upper müllerian structures, particularly cervical tissue.

ARMs are associated with müllerian anomalies in upwards of 50 % of cases2. Identifying individuals with ARM who have co-occurring müllerian anomalies can ensure simultaneous surgical management when appropriate. Among these patients, there is a subset who do not have enough vaginal tissue for vaginal reconstruction, creating a surgical challenge. In this review, we discuss the surgical considerations in these patients undergoing primary ARM repair and present the surgical options for vaginal management.

Section snippetsImperforate anus

Imperforate anus includes a spectrum of anomalies including rectovaginal, rectovestibular, and rectoperineal fistulas. Rectovestibular fistula—the most common ARM subtype in females—presents with partial or total vaginal agenesis in 1–9 % of cases3,4. Rectoperineal fistulas can also co-exist with vaginal agenesis but at much lower rates.

Imperforate anus and isolated distal vaginal agenesis

This patient population will have upper müllerian structures including a uterus and upper vagina, but with absent lower vaginal tissue (Fig. 1). Optimal

Cloaca

Vaginal replacement is also an important consideration in patients with cloaca. Using native vaginal tissue is always preferred over vaginal replacement, but in a large case series of 490 patients with cloaca, full vaginal replacement was necessary in 18 % of patients10,11. Depending on the length of the common channel and the urethra, cloacal repair will require either urogenital (UG) sinus separation or total urogenital mobilization.

For cases in which a UG sinus separation is deemed necessary

Current options for neovagina creation

When needed, neovaginas can be created using autografts, allografts, or artificial biomaterials. Neovaginas rely on the creation of a space posterior to the urethra and anterior to the rectum where graft material is placed. Depending on the approach, the neovagina may be blind ending without connection to pelvic müllerian structures, or it can be anastomosed to more proximal vaginal tissue, thereby creating a continuous lumen from müllerian structures to introitus.

Bowel grafts

Bowel neovaginas were first described in 1904 but then temporarily abandoned due to their high morbidity prior to widespread use of antibiotics. They regained popularity in the 1990s and remain a popular option13. Bowel neovaginas are constructed through resection of a bowel segment while maintaining its mesenteric vascular supply. The segment is then mobilized, and the distal end is pulled through the peritoneal reflection and affixed to the introitus between the urethra and the posterior

Role of neovaginal bowel grafts in patients with ARM

In patients with ARM, bowel grafts have broad applicability for both partial and complete vaginal replacement. Although alternative grafting options exist, bowel remains the preferred material when neovaginal creation is indicated in pediatric ARM patients. Bowel grafts obviate the need for post-operative dilation, which is often impractical and potentially traumatic in children. Moreover, in ARM reconstructions where tissue planes between the vagina and urethra are attenuated—particularly

Alternatives to bowel graft

Although bowel grafts have long been considered the standard for neovaginoplasty in patients with ARM, alternative techniques exist and warrant consideration, particularly in patients for whom a delayed vaginal repair may be feasible. Methods for vaginal reconstruction that do not rely on bowel mucosa are well established and predate the use of bowel vaginoplasty.

As noted by B.H. Sheares in 196021, the first documented attempt at creating an artificial vagina occurred in 1817, involving the

Timing of vaginal repair or replacement

As discussed previously, timing of vaginal repair is a critical consideration in the ARM population. Performing vaginal reconstruction concurrently with the anorectal repair can be advantageous, particularly in patients without prior perineal or pelvic surgery or in those where isolating the urinary system will allow for adequate bladder emptying—a key component of renal health in this population. This combined approach reduces anesthesia exposures and allows dissection and neovaginal creation

Long-term gynecologic management

In certain populations, up to 50 % of patients undergoing vaginal replacement require subsequent surgery to facilitate menstrual egress or penetrative intercourse48. Dysmenorrhea and dyspareunia are common sequelae. Patients should be counseled regarding all potential complications of vaginal replacement and informed that lifelong follow up with a gynecologist is necessary to maintain gynecologic health and to monitor for graft-specific issues.

Although there are no standardized follow-up

Conclusion

Given the frequent co-occurrence of ARMs and müllerian anomalies, a subset of ARM patients will require vaginal replacement. While numerous graft options are available, bowel, buccal mucosa, and biologic materials remain the most widely utilized in this population. Bowel grafts are particularly advantageous when vaginal repair must be completed in childhood. However, there are many reasons to consider delaying vaginal repair until after puberty, at which point buccal mucosa, skin grafts, and

IRB approval

None.

Funding

This research was supported in part by the Intramural Research Program of the National Institutes of Health (NIH). The contributions of the NIH author(s) are considered Works of the United States Government. The findings and conclusions presented in this paper are those of the author(s) and do not necessarily reflect the views of the NIH or the U.S. Department of Health and Human Services.

Declaration of competing interest

The authors have no conflicts of interest to disclose associated with this work.

Acknowledgements

None.

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