Part of a five-part series on gynecologic care for patients with anorectal malformations (ARM), developed by the Gynecology Committee of the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC). This article discusses considerations for long-term gynecologic care — including preventive screening, fertility, and sexual function — as patients transition from pediatric to adult care. Patient autonomy and shared patient–provider decision-making are the cornerstone of long-term gynecologic care in this population.

Key Points

• Lifelong, multidisciplinary gynecologic care is essential for patients with anorectal malformations, particularly during transition from pediatric to adult care, with attention to anatomy, prior surgeries, and patient-centered care.
• Patient factors and preferences should drive individualized reproductive care planning recommendations.
• Pelvic pain and sexual dysfunction are common and multifactorial, requiring coordinated medical, surgical, and mental health management.
• Patients with prior vaginoplasty may need ongoing long-term surveillance and management.
• Patients benefit from preconception counseling and may need multidisciplinary care throughout pregnancy and at the time of delivery to achieve the best obstetrical outcomes.

Introduction

Advances in the care of patients diagnosed with an anorectal malformation (ARM) have significantly improved, and survival, long-term function, and quality-of-life outcomes have gained increasing focus. Because patients with ARMs are more likely to have Müllerian anomalies, gynecologic care is central to their management. However, adult care is less likely to occur in a multidisciplinary clinic setting, and gynecologic evaluation may not be routinely incorporated. Available literature on long-term menstrual, sexual, and reproductive outcomes in this population is limited, underscoring the need for longitudinal gynecologic care and continued clinical research to support evidence-based guidelines. This article presents an overview of available long-term gynecologic data in patients with ARM and provides recommendations for care as they transition from pediatric to adult settings.

Gynecologic Surveillance in Adults

Longitudinal gynecologic care for patients with ARMs requires considerations beyond standard obstetric–gynecologic recommendations for individuals with typical anatomy. Many adult patients with an ARM have undergone numerous operations and medical interventions beginning in infancy or childhood, and may carry significant psychological trauma, along with concerns related to sexuality, intimacy, and body image that may not have previously been discussed with pediatric providers. When establishing care with an adult patient, a patient-centered, trauma-informed approach starts with open-ended conversation: "How can I best help you? What concerns bring you in today? What is your understanding of the anatomy you were born with and any reconstructive procedures you have had? How has your healthcare journey been so far? Have you previously worked with a gynecologist, and what were those experiences like?" These questions build rapport and let patients share personal priorities, establishing trust with a new team.

Standard cervical cancer screening should be performed on all patients with a cervix, in accordance with general population guidelines; if the patient has two cervices, screening should be collected on separate swabs from both. Patient preferences and factors such as vaginal type, length, discomfort with exam, or a history of trauma will determine whether cervical screening can be done via a speculum exam in the office or requires an examination under anesthesia. Vaginal HPV swabs or Pap tests without a speculum may be a reasonable option for patients who cannot tolerate a speculum exam, such as those with intellectual disabilities, very long or circuitous vaginal passageways, or post-surgical vaginal stenosis. HPV vaccination is recommended for all patients, and may be even more critical in this population, given the potential difficulty of obtaining adequate Pap screening or managing abnormal results with colposcopy, cervical biopsies, or excision procedures.

Contraception

Contraceptive counseling in the adult with an ARM begins with a discussion of the patient's sexuality and desires regarding family building, along with the patient's understanding of their anatomy, reproductive potential, and use of contraception. All patients with the potential to achieve pregnancy should be counseled on available contraceptive options to avoid unintended pregnancy, along with the potential non-contraceptive benefits of each option. Systemic hormonal options include pills, patches, and vaginal rings with estrogen and progestin, as well as progestin-only pills, shots, and implants; for some patients, these are used to suppress menstruation or ovulation.

ARM patients with a stenotic vagina or neovagina may be limited in their ability to use a vaginal ring, as are patients with significant prolapse or distortion of pelvic floor musculature; there are no data on the contraceptive efficacy of vaginal rings in patients with a graft neovagina. Intrauterine devices (IUDs) provide effective long-acting contraception ranging from 3 to 10 years, depending on the device; the copper IUD may make bleeding heavier in some users, while progestin IUDs generally diminish bleeding, with variable rates of suppression depending on dosage. In patients with uterine anomalies, the endometrial cavity may be distorted and may not permit IUD placement. Although U.S. Medical Eligibility Criteria classify IUDs as Category 4 (unacceptable health risk) for uterine cavity distortion, given the elevated risk of expulsion and malposition, several case series have highlighted the potential for IUD placement in patients with uterine anomalies; the risk of unintended pregnancy with an IUD in an atypical cavity is unknown, and reported outcomes vary. The decision to proceed with IUD placement in a patient with a uterine anomaly should be made through shared decision-making, weighing risks, benefits, and alternatives; ultrasound and/or placement under anesthesia, with or without hysteroscopy, may be helpful given the potential need for additional manipulation and immediate evaluation of placement.

Patients with ARMs may also have concomitant renal anomalies and decreased renal function; combined hormonal contraception is not recommended (Category 4) for patients with chronic kidney disease with nephrotic syndrome, hemodialysis, or peritoneal dialysis, nor is a drospirenone-containing progesterone-only pill for patients with known hyperkalemia. Patients with ARMs are also more likely to have cardiac anomalies, which may pose an increased risk in pregnancy. Patients with isolated, repaired, or "simple" cardiac anatomy are likely candidates for all forms of contraception, while those with more complex conditions (such as Fontan circulation, single ventricle physiology, or severe pulmonary hypertension) are at increased risk of thromboembolic events and are not candidates for estrogen-containing medications; these patients also have an increased risk of vasovagal events and may need cardiac monitoring during implant or IUD placement. Anticoagulation is not a contraindication to any contraceptive, though clinicians should be aware of the potential for increased bleeding with the copper IUD and during procedures such as injections or implant placement/removal.

As many patients with ARMs have multiple other medical comorbidities that may increase obstetric risk, decisions regarding contraception must also weigh the patient's risk of unintended pregnancy. A cardiac or renal condition is not a contraindication to emergency contraception, including the copper IUD. Patients undergoing major surgery with prolonged immobilization, or with reduced mobility for other reasons (such as spinal anomalies), should also be cautious about the increased thrombotic risk of estrogen-containing contraceptives. Ongoing collaboration with other clinicians involved in managing these medical conditions helps ensure awareness of health changes that might require alternative contraceptive choices.

Dysmenorrhea, Pelvic Pain, and Adnexal Masses

Dysmenorrhea and non-cyclic pelvic pain may exist in patients with ARMs. Pelvic pain is often multifactorial and may stem from gastrointestinal, bladder, and musculoskeletal structures in addition to gynecologic organs, particularly in patients requiring additional bowel and bladder management or with a significant past abdominal surgical history. Non-steroidal anti-inflammatory drugs are first-line treatment for dysmenorrhea, though acetaminophen and other medications may be warranted for patients unable to take NSAIDs; a trial of menstrual suppression may also help minimize discomfort.

Obstructive anomalies, which can cause progressive dysmenorrhea or severe abdominal pain, are more common in patients with an ARM and include oblique and transverse vaginal septa, distal vaginal agenesis, and obstructed Müllerian remnants. The approach must also consider future reproductive goals, balancing the risk of unnecessary sterilization against the risk of complex operations with high rates of complications and re-operation; exploration of patient and caregiver goals by collaborative multidisciplinary teams allows for holistic decision-making regarding the risks and timing of surgery.

Bowel motility decreases in the last half of the menstrual cycle, which can lead to constipation, then increases during menses due to falling progesterone and rising prostaglandins. For patients with an ARM, this may necessitate bowel regimen changes with menstruation, cyclic NSAID use (if appropriate), or menstrual suppression. Bladder management can also be affected, with increased sensations of fullness or irritation during menses potentially affecting catheterization regimens. Patients may experience menstrually related headaches, acne, or mood changes similar to those without an ARM, and may benefit from menstrual suppression.

Endometriosis is the most common cause of secondary dysmenorrhea, occurring in about 10% of women generally; the risk is increased in patients with Müllerian anomalies, particularly obstructive anomalies, though it may occur even with adequate menstrual outflow and may persist after obstruction has been resolved. It is typically managed with hormonal medications, GnRH agonists or antagonists, or surgery to excise or ablate implants, with care frequently multidisciplinary (including pain medicine, pain psychiatry, and physical therapy). In patients with an ARM, surgical evaluation and treatment of endometriosis may be limited by extensive pelvic scarring and inability to visualize the pelvic peritoneum. Prior pelvic surgery also increases the likelihood of adhesive disease, which may contribute to hemato- or hydrosalpinx, peritoneal inclusion cysts, and cyclic or activity-related pelvic pain; peritoneal inclusion cysts can be managed expectantly, with interventional radiology-guided drainage, or sclerotherapy. Most ovarian cysts in children and adolescents are physiologic and represent normal ovarian function; incidentally detected simple cysts less than 3–5 cm can typically be managed conservatively with repeat ultrasonography in 6 weeks. For patients with recurrent cysts, ovulation suppression with systemic hormones may be beneficial. Severe pain with nausea and vomiting may suggest ovarian torsion, though this may be less likely if the patient has significant abdominal and pelvic adhesions.

More complex adnexal masses warrant further evaluation. When operative intervention is considered, a detailed review of the prior operative history is essential — particularly in patients no longer followed by their original pediatric surgical team — to minimize the risk of injury to reconstructed anatomy (such as MACE, Mitrofanoff, or bladder augmentation) and to anticipate the extent of adhesions. In cases with likely benign adnexal masses and a surgical history that portends higher risk of complication, expectant management may be appropriate. Planned surgical collaboration between gynecologists and general surgeons is useful when managing a complex adnexal mass in patients with an ARM and previous pelvic surgery.

Sexual Health and Function

Clinicians should discuss patient experiences regarding relationships, sexuality, and body image, using a trauma-informed practice to create physical and emotional safety during sensitive examinations for patients with difficulty or pain during intercourse. Pelvic examinations may serve as an opportunity to teach patients about their anatomy using a handheld mirror and to directly address specific concerns. For some patients, concerns may relate less to pain and more to body image and disclosure of their medical history within relationships; medical teams can help connect patients with psychologists and therapists for these complex issues. It is important to be cognizant of vaginal and sexual function when determining the success of surgical repairs and revisions; a collaborative evaluation including mental health, pain physicians, and physical and sex therapy specialists may help improve diagnosis, patient experience, and treatment outcomes.

Dyspareunia (pain with intercourse) is reported in up to 40% of women worldwide, with estimates varying by criteria. Pain may be multifactorial and occur with initial or deep penetration, arousal, or orgasm; some patients report an inability to adequately have penetration. In a study of patients with Hirschsprung disease or an ARM, 36.8% reported sexual dysfunction on a validated questionnaire, with 45% experiencing sexual distress. Patients with more complex malformations are more likely to have non-native vaginal tissue, though it is unclear which tissue types lead to increased dyspareunia or decreased sexual function. Other patients report a lack of sensation rather than pain, limiting pleasure. Contributing factors to difficulty with intercourse may include introital or vaginal stenosis, perineal scarring, the angle of penetration and shape of bony anatomy, residual vaginal septa and/or widely separated hemivaginas, pelvic floor dysfunction, and psychosocial factors such as anxiety, trauma, and body-image concerns.

Treatment may include operative correction of anatomic factors, pelvic floor physical therapy, dyspareunia treatment with lubrication and dilators, and ongoing psychosocial support and therapy. Dilators or vibrators may help with desensitization of the introitus, and penile rings can help limit depth of penetration for those with anatomic shortening of the vagina or pain with deep penetration.

Timing of Vaginal Surgery

Many patients with an ARM had vaginoplasties performed as young children, concurrent with bowel or bladder reconstruction. However, vaginoplasty in many patients may be delayed to the late teenage years or after, which allows for more patient autonomy in decision-making, better estrogenization of tissue, and patient participation in post-operative care (such as pelvic exams and dilation). Patients who undergo vaginoplasty in infancy may also require multiple revision surgeries to achieve menstrual egress and a caliber sufficient for desired menstrual hygiene products or intercourse. Decisions for vaginal surgery may be intimately connected with the patient's gender identity, sexual orientation/practices, and even the desire for a vagina at all. Regardless of when surgery is performed, long-term management of the vagina is required, and surgical revisions are common to achieve the patient's goals for intercourse and menstrual management.

When managing a patient with an ARM with a history of vaginoplasty, it should first be clarified whether the patient has native vaginal tissue, graft vaginal tissue, or a combination.

Vaginal Reconstruction Without a Graft

Some patients may require or desire vaginal lengthening or revision without additional graft tissue. There is no standard optimal vaginal length — adequacy is determined by the sufficiency of sexual function. Techniques include: dilated native vagina (a non-surgical technique using serially enlarging dilators, or coital dilation, to stretch existing vaginal tissue); and native vagina pull-through, modified Heineke-Mikulicz strictureplasty, or Z-plasty techniques (mobilizing vaginal tissue without a graft in patients with a persistent cloaca, distal vaginal atresia, or stenosis of a previous vaginoplasty — a stent and post-operative dilation may be necessary, and stricture at the anastomosis site can be relieved with dilation, steroid injections, or further revisional vaginoplasty).

Native vaginal tissue is generally extremely pliable, and stretched vaginal length is the best assessment of length rather than length at rest. Native vaginal tissue may need regular "maintenance dilation" over the long term; while there is no agreed-upon protocol, most experts recommend at least several times per week using vaginal dilators or penile-vaginal coitus to maintain length. If no cervix is present, Pap smears are not recommended, but HPV-related disease can still occur, so periodic visual inspection should be performed with frequency based on shared decision-making, and HPV vaccination is still recommended. Sexually transmitted infection testing guidelines are the same as in the general population. For those without a uterus, patients with native vaginal tissue only (no graft) remain candidates for uterine transplant, though other factors (such as prior pelvic surgery/adhesive disease or anomalous pelvic vasculature) may affect transplant candidacy.

Vaginal Reconstruction With a Graft

In patients for whom native vaginal tissue is insufficient, a graft may be needed for neovagina creation. Depending on the graft tissue type, patients require ongoing surveillance to ensure healthy tissue, and may have different side effects or complications long-term:

• Bowel: Common in patients who had early childhood vaginal surgery; pediatric surgeons have traditionally preferred bowel grafts, particularly when constructed simultaneously with primary ARM reconstruction. An approximately 10 cm bowel segment is mobilized on a mesenteric vascular pedicle (sigmoid colon tissue typically preferred). There are two anastomoses — one at the perineum, and one at the top of the vagina or attaching to native vaginal tissue — and both may become stenotic over time. Intestinal tissue naturally produces lubrication, though some patients experience over-production resulting in bothersome discharge or mucus plugs; bowel neovaginas are also more prone to malodor and bleeding with sexual activity. Neovaginal length in adulthood is variable, which can make cervical surveillance difficult.
• Buccal: Buccal mucosa is harvested and fenestrated to form a neovagina very similar to native vagina in appearance and pliability/lubrication. Because the amount of tissue harvested may be limited, it is also often used for small graft patches at areas of vaginal stenosis, or for revision/augmentation of other vaginoplasty types such as bowel neovagina.
• Skin: Split-thickness skin grafts require a large surface area and are not commonly performed in young children; they lack natural lubrication and can result in intra-vaginal hair growth, making this technique less favored for primary repair but occasionally used for revision.
• Peritoneum: Used only in patients without a uterus; use may be limited by peritoneal scarring from prior pelvic surgery, and lubrication deficiencies are a concern.
• Bio-engineered grafts: A growing number of tissue grafts are available for clinical use (porcine, amnion, fish skin, lab-grown human tissue), removing the surgical risks and recovery involved in harvesting autologous tissue. These are likely to see increased application in ARM patients, though minimal long-term outcomes data currently exist.

There are no data regarding the safety of a vaginal contraceptive ring, tampon, menstrual cup/disc, or pessary in patients with a graft neovagina. Vaginal delivery is contraindicated for any individual with a bowel neovagina, given the inability of the tissue to stretch to accommodate delivery; for patients with other vaginal surgery/graft history, shared decision-making with a comprehensive discussion of the risks of vaginal delivery, need for unplanned cesarean, and planned cesarean delivery is critical. Patients with graft neovaginas who do not have a uterus are currently not candidates for future uterine transplant. Depending on the tissue type used, there may be problems with under- or over-lubrication, and some patients benefit from periodic douches or vaginal irrigations at home.

Graft tissue is susceptible to diseases affecting the specific tissue type used — for example, tissue-specific cancers such as skin or colorectal cancer, or colitis/inflammatory bowel disease within a bowel neovagina. All grafted tissues likely benefit from short-term vaginal dilation; it remains unclear whether different neovagina types benefit from long-term maintenance dilation, though other graft tissues such as peritoneum and buccal mucosa may require longer-term dilation or regular intercourse to maintain patency (based on expert opinion). The goal of dilation is to prevent vaginal stenosis, which is common in graft neovaginas. There are no clear guidelines on post-surgical surveillance of neovaginal tissue; any new bleeding, discharge, or pain should prompt assessment. Limited evidence suggests vaginoscopy or surveillance exams every 3 years, beginning 5 years post-vaginoplasty, to assess bowel neovagina for inflammatory conditions or disease in the graft tissue.

Pelvic Organ Prolapse

Pelvic organ prolapse (POP) is defined as the descent of one or more aspects of the vagina and uterus, resulting in herniation of the organs through the vagina. In the general population, symptomatic POP is reported in 3–6% of females, with up to 50% prevalence on clinical examination in some studies. Although there is scant literature on POP in patients with an ARM, the same factors that lead to POP in the general population may increase the risk of both POP and rectal prolapse in this group. Prolapse is a known complication after neovaginal creation, including after non-surgical methods such as vaginal dilation, and can be cosmetically unappealing and cause pain, bleeding, and irritation. Chronic constipation, alterations in pelvic floor musculature, poor pelvic floor muscle tone, and pelvic floor dysfunction — all of which affect people with ARM at higher rates — are risk factors for POP.

Management approaches for POP in patients with an ARM reflect recommendations for the general population. Strategies to decrease pelvic pressure, such as addressing bowel or bladder management and maintaining a healthy body weight, remain important. Vaginal pessaries may be considered, though there are no data on their use in graft neovaginas, and foreign bodies may increase local inflammatory changes, particularly in a bowel neovagina; if used, the vagina should be examined periodically to ensure tissue health without erosions or granulation tissue. Pelvic physical therapy is considered highly beneficial for POP and may particularly help patients with an ARM address both pelvic floor muscular dysfunction and pelvic pain. Surgical management for patients with native vaginal tissue requires awareness of previous pelvic surgeries and anatomy (which may limit access for procedures such as sacrospinous ligament fixation or sacrocolpopexy). Management of a prolapsed vaginal graft requires shared decision-making and careful weighing of risks and benefits, including potential difficulty with vaginal sexual activity after excision of prolapsed tissue and the risk of recurrence; for example, colpocleisis would prevent surveillance of the grafted tissue, risking missed neovaginal pathology.

Fertility

There are limited data on the fertility of patients with an ARM, given few studies quantifying patient desires for fertility or attempts at conception. Many factors can impact fertility, most related to anatomic anomalies. Depending on the uterine anomaly, there may be associated fertility or gestational risks, such as recurrent miscarriages, preterm labor/delivery, or fetal malpresentation. Anatomical factors involving the vagina (such as vaginal agenesis or septum) can affect fertility via sexual function. The fallopian tubes may be impacted by hydrosalpinx (iatrogenic, from prior surgery, or related to endometriosis or previous menstrual obstruction), or tubo-ovarian abscesses. Other sexual health issues, such as dyspareunia or mental health and body image factors, can also impact fertility rates.

Factors impacting fertility in ARM patients include: Müllerian anomalies; vaginal anatomy factors (complete or partial stenosis, absent vagina); presence of a bowel neovagina; sexual health factors (pain, mental health, body image); endometriosis; fallopian tube factors (hydrosalpinx, tubo-ovarian abscess); and pelvic adhesions.

The ovaries are typically functional even when other pelvic anomalies are present, though they may be ectopically located high in the pelvis or in the lower abdomen. Ectopic ovaries, or other anatomic factors such as pelvic adhesive disease or a pelvic kidney, can pose challenges in accessing the ovaries for patients attempting egg retrieval for in vitro fertilization.

It is important to note that not all patients with an ARM experience fertility problems, even when one or more of the above factors are present. Patients may misinterpret conversations about potential difficulties with conception as meaning they cannot conceive at all, and may not use contraception when needed, resulting in unintended pregnancy. Clear and confidential discussions should be had with all patients regarding their ability to conceive and carry a pregnancy. A 2022 retrospective review of 105 patients with an ARM over the age of 18 with a bowel neovagina reported no pregnancies among them; it remains unclear whether factors specifically related to a bowel neovagina impact fertility.

Obstetric Care

Obstetric guidance should be highly individualized to the patient's pregnancy risks, past surgical history, and present medical needs. There remains limited published information on obstetric outcomes in patients with a history of an ARM. Multidisciplinary care is recommended, with input from Maternal Fetal Medicine, Pediatric and Adolescent Gynecology, Urology, pediatric and adult Colorectal Surgery, Anesthesia, and others as needed.

Preconception counseling on individual medical and surgical risks is beneficial and may involve a high-risk obstetrics team, with additional specialists if medical co-morbidities are present, such as tracheo-esophageal fistula, spinal differences, or cardiac disease. Records of the patient's childhood surgical history should be obtained whenever possible to help understand current anatomy. Patients with risk factors for tubal disease, such as prior pelvic infection or surgery, may have an increased risk of ectopic pregnancy. Müllerian anomalies also increase the risk of early pregnancy loss and preterm labor, so patients may require surveillance with serial obstetric ultrasound and cervical length measurement. No additional prenatal genetic testing recommendations apply based solely on a personal or family history of a Müllerian anomaly or ARM. Patients with bladder augmentation should be counseled that up to 75% may have false-positive urine pregnancy tests, and pregnancy should be confirmed with serum beta-hCG.

Mode-of-delivery planning should use shared decision-making, considering the patient's anatomy and surgical history, current medical needs, and personal preferences. The overwhelming majority of patients with a known ARM undergo cesarean delivery, though this decision may be less evidence-based and more related to a paucity of data guiding mode-of-delivery decisions. When possible, a hospital with access to anesthesia, neonatal care, and collaborative general surgeons and urologists is preferable, with a multidisciplinary delivery plan discussed in advance of labor. Obstetric lacerations are a major concern, as a 3rd or 4th degree perineal laceration could damage bowel continence — this may be less relevant for patients using an ostomy for stooling. A rectovaginal exam can help determine tissue bulk and pliability between the rectum and vagina, and whether this area has been thinned or scarred from prior surgery, which may increase laceration risk; the perineum should also be assessed for length and scarring, as a short perineal length may increase the risk of severe laceration. More recent perineal-sparing rectal pull-through techniques may reduce the risks of vaginal delivery in the future, though long-term outcome data are needed. The risks of vaginal delivery should be weighed against the surgical risks of planned and emergent cesarean delivery, particularly for patients with high risk of pelvic adhesive disease or bowel/urinary diversions; a scheduled cesarean with a multidisciplinary surgical team available may minimize the risk of injury to existing conduits compared with an emergent unplanned cesarean during labor.

Regional anesthesia (spinal or epidural) is commonly used in obstetrics. Approximately 15–30% of patients with ARMs have a tethered spinal cord (likely an underestimate, as mild cases may be asymptomatic and universal screening is not implemented everywhere), which may impact the ability to safely place spinal or epidural anesthesia. Patients without prior screening should ideally have a spinal MRI during pregnancy, with an anesthesia consult if abnormalities are present.

Bowel management needs will likely change during pregnancy. Constipation, common for all pregnant people, may necessitate changes to baseline bowel medication regimens; those using rectal enemas may find it more difficult to reach the anal opening, and those relying on anterograde enemas may need to change this regimen or revert to rectal enemas during pregnancy. Urinary tract management may also change: in a 2018 review of 11 women with a neurogenic bladder across 15 pregnancies, 8 of 15 involved difficulty with self-catheterization, 13 of 15 had worsening hydronephrosis (6 requiring a nephrostomy tube), and the UTI rate was 67%, with all deliveries via cesarean section with a urologist present. Other studies have shown increased risks of hydronephrosis, urinary tract infections/pyelonephritis, and gestational hypertension/pre-eclampsia in patients with prior urinary tract reconstruction. Those with extensive urinary tract reconstruction, particularly after prior bladder neck surgery, are recommended to undergo cesarean delivery with a urologist present, with the location and path of any diverting channels confirmed with the patient while awake, prior to surgery.

Obstetric Care Timeline

TimepointRecommendationsPreconceptionOngoing gynecologic and pre-conception care (current cervical cancer screening, immunizations, prenatal vitamins); comprehensive preconception evaluation; if ongoing renal disease, evaluate renal function, urinary bacterial profile and infection history, optimize antihypertensive/antibiotic regimens as applicable; understand renal, pelvic, and spinal anatomy (access operative records, review reconstructed anatomy and catheterization channels, consider exams/imaging or spinal MRI if history is unclear); understand current bowel and urinary management regimensFirst trimesterEarly ultrasound to confirm location, viability, and dating; establish multidisciplinary prenatal care team (Maternal-Fetal Medicine, Cardiology, Urology, Gastroenterology as needed); if ongoing renal disease, obtain baseline urine culture, serum creatinine, and glomerular filtration rateSecond/third trimesterAnticipate elevated risk for hypertensive disorders (if renal disease), urinary tract obstruction or catheterization changes (if prior urologic diversions), preterm contractions/delivery, fetal growth restriction, abnormal placentation (uterine anomaly), and constipation/bowel regimen changes; obtain spinal MRI if not already done; consider pre-labor anesthesia consultation; counsel on mode of delivery and document the surgical approach plan; consider urologic and general surgery consultation prior to deliveryDeliveryIf vaginal delivery is planned, delivery in an operating room may optimize transition to cesarean if needed; anesthesia should consider any spinal anomalies or tethered cord; if cesarean, consider alternative abdominal incisions to navigate around urinary or bowel diversions; if history of bladder reconstruction, anticipate significant pelvic adhesions; collaboration between Obstetrics, Urology, and Colorectal Surgery is crucialPostpartumAnticipate elevated risk for hypertensive disorders and UTIs (if renal disease or prior urologic reconstruction)

Transition to Adult Gynecology

The transition of care for patients with complex medical problems presents social and health-systems challenges. Patients may age out of pediatric health systems at variable times, so proactive transition planning is recommended. Many transition tools exist — for example, GotTransition.org provides a six-step tool. In general, these tools recommend identifying potential transition barriers, making a transition plan that includes care goals and a summary of past and current care, identifying a point of medical stability at which transition may be made, and creating an information handoff summary for adult providers.

Early transition planning includes a comprehensive gynecologic/urogenital anatomy assessment (with pelvic exam, and possibly imaging or endoscopy depending on history) and education of the patient and family regarding anatomic findings. Periodic follow-up every 1–2 years allows clinicians to continue educating the patient and family, with increasing emphasis on patient-led (rather than family-led) conversation. This continues into young adulthood, providing opportunities for ongoing evaluation of sexual function and goals, introital and vaginal stenosis, dyspareunia, incontinence, and reproductive/contraceptive needs. Young adults should be counseled on long-term gynecologic screening recommendations, sexual health, and fertility considerations individualized to their anatomy and health needs, with referrals to Reproductive Endocrinology and Infertility physicians as desired.

Written summaries in patient-centered language — given to the patient and included in the medical record — may aid comprehension and provide a record for future providers. Adult obstetrician–gynecologists should be informed of the patient's history of an ARM, prior operative interventions, and potential long-term complications, to provide comprehensive, inclusive, patient-centered care. Clinicians may help support patients in identifying adult reproductive health care providers with expertise in complex pelvic anatomy, and direct communication between pediatric and adult clinicians can help facilitate a coordinated transition with the goal of uninterrupted, lifelong access to gynecologic health care.

Transition to Adult Care: Timepoints

TimepointRecommendationAdolescenceBegin gynecologic preventive care (HPV vaccination and STI screening as indicated, contraception counseling, fertility counseling if desired), with confidential visits as appropriate; periodic follow-up every 1–2 years to review gynecologic symptoms and needs (more frequent if symptomatic); comprehensive gynecologic/urogenital anatomy assessment and education of anatomy for the patient/family; provide a written summary of gynecologic history and anatomy in patient-centered language, given to the patient and included in the electronic medical recordYoung adulthoodContinue gynecologic preventive care (STI screening as indicated, contraceptive counseling, cervical cancer screening, and fertility counseling if desired); for preconception, refer to a multidisciplinary team (obstetrician plus urologic/colorectal surgeon), review surgical history/anatomy, and counsel on likely need for cesarean delivery and possible complications (preterm birth, adhesions, urinary/renal issues); ensure lifelong access to reproductive and sexual health services, with adult OB/GYN providers aware of ARM history, prior surgical operative reports, and potential complications