Sexual and Reproductive Health Concerns in Adults With Cloacal Anomalies: A Qualitative Study.

STUDY OBJECTIVE: Long-term gynecologic data are lacking to inform the care of patients with cloacal malformations. We seek to examine perceived sexual and reproductive health challenges of patients born with cloacal anomalies and characterize the experiences of patients as adults. DESIGN AND SETTING: Virtual semi-structured focus groups and single-participant interviews were conducted using an online video platform. Retrospective chart review was performed to abstract available demographics and surgical history. PARTICIPANTS: Adult patients were contacted from a database of 143 patients born with cloaca who had been seen at or referred to a tertiary care pediatric colorectal center. Participants were recruited until data collection reached thematic saturation. Twenty patients aged 18-53 years participated in 5 focus groups and 3 single-participant interviews. The hospital IRB determined the research activities to be exempt from IRB review and oversight. INTERVENTIONS AND MAIN OUTCOME MEASURES: Interviews and focus groups were transcribed and analyzed using the constant comparative method to identify themes regarding sexual and reproductive health and compared with medical and surgical history abstracted from chart review. RESULTS: Participants reported many perceived barriers to intercourse and intimacy including bowel and bladder continence management. All participants reported discussion of pregnancy and fertility with healthcare providers as important. Other common themes included concerns about independence and transitioning from pediatric to adult providers. CONCLUSION: Anorectal malformations are associated with sexual and reproductive health concerns. Patients seek guidance on family building, intimacy, and transition to adult care. Future quantitative study of these topics is needed to develop best practices for counseling and clinical management of these patients. LEVEL OF EVIDENCE: Level VI. TYPE OF STUDY: Prognosis Study.

Recurrence Rates for Pediatric Benign Ovarian Neoplasms.

STUDY OBJECTIVE: To examine the recurrence rates of pediatric benign ovarian neoplasms METHODS: A retrospective review of females up to 21 years of age who underwent surgery for a benign ovarian neoplasm at 8 pediatric hospitals from January 2010 through December 2016 was conducted. Data include primary operation details, follow-up imaging, and reoperation details. RESULTS: Four hundred and twenty-six females were included in our cohort, with a median age of 15 years at the time of the primary operation. Of the patients, 69% had a mature teratoma, 18% had a serous cystadenoma, and 8% had a mucinous cystadenoma. Two-thirds of patients underwent ovarian-sparing surgery. There were 11 pathologically confirmed recurrences (2.6%) at a median follow-up of 12.8 months. The pathologically confirmed recurrence was 10.5 per 100 person-months at 12 months (SE = 5.7) for mucinous cystadenomas and 0.4 months (SE = 0.4) for mature teratomas (P = .001). For half of the patients, the pathologically confirmed recurrences occurred by 12.8 months, and for 75%, they occurred by 23.3 months. There were no differences in reoperation or recurrence on the basis of initial procedure (ovary-sparing surgery vs oophorectomy). CONCLUSION: We measured the pathologically confirmed recurrence rate for pediatric benign ovarian neoplasms in a large cohort. Oophorectomy was not protective against recurrence. Mucinous cystadenomas were at a greater risk of pathologically confirmed recurrence.

Does presence of a VACTERL anomaly predict an associated gynecologic anomaly in females with anorectal malformations?: A Pediatric Colorectal and Pelvic Learning Consortium Study.

BACKGROUND: VACTERL association is defined by the presence of 3 or more anomalies in any of the following systems: vertebral, anorectal, cardiac, trachea-esophageal, renal, or limb. This study hypothesized that the presence of VACTERL association would correlate with an increased risk of gynecologic anomalies in patients with anorectal malformation (ARM). METHODS: This study is a cross-sectional, retrospective analysis from the prospectively collected, multicenter registry of the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC). The 834 female patients with ARM who were enrolled in the registry by January 1, 2020 were included in this study. The relationship of VACTERL association with presence of a gynecologic anomaly was evaluated with Fisher's exact test. The relationship of each VACTERL system with presence of a gynecologic anomaly was assessed in patients with cloaca, rectovestibular fistulas and rectoperineal fistulas. P-values reported were based on a 2-sided alternative and considered significant when less than 0.05. RESULTS: 834 patients with ARM underwent VACTERL screening and gynecologic evaluation with the three most common subtypes being cloaca (n = 215, 25.8%), rectovestibular fistula (n = 191, 22.9%) and rectoperineal fistula (n = 194, 23.3%). A total of 223 (26.7%) patients with ARM had gynecologic anomalies. VACTERL association was seen in 380 (45.6%) of patients with ARM. Gynecologic anomalies were present in 149 (39.1%) vs. 74 (16.3%) of subjects with vs. without VACTERL association (p < 0.001). VACTERL association did not significantly increase the risk of gynecologic anomaly in patients with cloaca and VACTERL (n = 88, 61.5%) vs. cloaca without VACTERL (n = 39, 54.2% p = 0.308). VACTERL association increased the risk of gynecologic anomalies in patients with rectoperineal fistulas (n = 7, 14.9% vs n = 9, 6.1% p = 0.014) and rectovestibular fistulas (n = 19, 31.1% vs. n = 13, 10.0% p<0.001). In patients with ARM who had a VACTERL association, when one of the associated anomalies was renal, there was an even higher risk of having an associated gynecologic anomaly (n = 138, 44.2% vs. n = 85, 16.3% p<0.001). CONCLUSIONS: VACTERL association in patients with rectoperineal and rectovestibular fistulas correlates with an increased risk of gynecologic anomalies. The presence of VACTERL associated findings, especially renal, should prompt a thorough evaluation of the gynecologic system. LEVEL OF EVIDENCE: III. Retrospective comparative study.

Failure of Operative Therapy after Ovarian-Sparing Surgery for Pediatric Benign Ovarian Neoplasms: A Retrospective Review.

STUDY OBJECTIVE: To evaluate failure of initial operative therapy (incomplete tumor removal) of ovarian-sparing surgery for pediatric benign ovarian neoplasms. METHODS: A retrospective review of patients up to 21 years of age who underwent ovarian-sparing surgery for a benign ovarian neoplasm from 2010 to 2016 at 8 pediatric hospitals was conducted. Failure of initial operative therapy is defined as a radiologically suspected or pathologically confirmed ipsilateral lesion with the same pathology as the primary neoplasm within 12 weeks of the initial operation. RESULTS: Forty patients received imaging within 12 weeks of their primary operation. Sixteen (40%) patients had a radiologically identified ovarian abnormality ipsilateral to the primary lesion, and 5 patients were suspected to have the same lesion as their primary neoplasm. Three of the 5 patients (7.5%) underwent reoperation with pathologic confirmation of the same lesion, resulting in a pathologically confirmed failure of therapy rate of 7.5%. The other 2 patients had serial imaging that subsequently demonstrated no recurrence with lesion resolution. Age, race/ethnicity, laparoscopy vs laparotomy, presence of torsion, pathology, size of lesion, and surgeon specialty were not associated with failure of therapy. CONCLUSION: In most patients who received imaging within 12 weeks of the primary operation for resection of a benign ovarian neoplasm, ovarian-sparing surgery was successful in complete tumor removal, with a low failure of therapy rate. Selected patients with suspected failure of therapy on initial imaging could be serially monitored to determine the need for repeat surgical intervention.

Recommendations for Postoperative Surveillance of Pediatric Benign Ovarian Neoplasms.

STUDY OBJECTIVE: To assess postoperative management of pediatric patients with benign ovarian neoplasms, to develop recommendations for postoperative care. DESIGN: A retrospective cohort study. SETTING: Eight pediatric hospitals in the midwestern United States. PARTICIPANTS: Patients up to 21 years of age who underwent surgery for a benign ovarian neoplasm between January 2010 and December 2016 were included. INTERVENTIONS: No prospective interventions were evaluated. MAIN OUTCOME MEASURES: Main outcome measures included postoperative imaging findings, recurrence rates, reoperation rates, and the timing of the aforementioned results. RESULTS: A total of 427 patients met inclusion criteria. After the index surgery, 155 patients (36%) underwent a routine imaging study. Among those with routine imaging, abnormalities were noted in 48 patients (31%); 7 went on to have reoperation (5%), and no malignant pathologies or torsion were identified. Excluding the 7 patients who went on to have a reoperation as a result of routine imaging, 113 patients developed symptoms postoperatively and underwent imaging as a result (27%, 113/420). Abnormalities were noted in 44 (10%); 15 of these patients underwent reoperation (4%), among them 2 with malignancies and 3 with torsion. Of these 44 patients, 23 had initially undergone routine imaging and subsequently went on to have symptomatic imaging, with 17% (4/23) undergoing reoperation. CONCLUSIONS: Routine imaging did not identify malignancy; most lesions identified on routine imaging were incidental findings. Although the study was not powered to appreciate a statistically significant difference, patients with malignancy or torsion were identified in the symptomatic group. This suggests no benefit from routine imaging, and supports symptomatic imaging postoperatively to minimize costs and patient/family burden.

Levonorgestrel Intrauterine Device Use for Medical Indications in Nulliparous Adolescents and Young Adults.

PURPOSE: Intrauterine devices (IUDs) are highly effective at preventing pregnancy. Levonorgestrel (LNG) IUDs also have beneficial effects on menstrual bleeding and abdominal and pelvic pain. Although there are increasing data on use of IUDs for contraception in adolescents and for medical indications in adults, there are extremely limited data on LNG IUD use for medical indications in adolescents. Our objective is to describe the characteristics and experiences of LNG IUD use in nulliparous adolescents and young women using IUDs for medical indications. METHODS: We conducted a retrospective chart review of all nulliparous patients aged 22 years and younger who underwent LNG IUD insertion at a tertiary care children's hospital between July 1, 2004 and June 30, 2014 primarily for noncontraceptive indications. Descriptive statistical analysis was performed. RESULTS: We identified 231 LNG IUDs placed in 219 nulliparous women for medical indications during this time period. Mean patient age was 16.8 years (±2.2). Only 41% reported ever being sexually active. IUD continuation rate at 1 year was 86%. The amenorrhea rate at 1 year was 51%. Approximately 80% of women reported improvements in menstrual bleeding and abdominal and pelvic pain. Side effects and complications were low. CONCLUSIONS: This study provides evidence that LNG IUDs are effective, well-tolerated, and safe menstrual management options in young nulliparous women, including younger adolescents and those who have never been sexually active. This method is an excellent first-line therapy option for adolescents and young women for both contraceptive and noncontraceptive indications, regardless of age, parity, or sexual activity.

Intrauterine Device Use in Adolescents With Disabilities.

BACKGROUND AND OBJECTIVES: Intrauterine devices (IUDs) are increasingly being used in adolescents and nulliparous women for contraception. Levonorgestrel IUDs also have beneficial effects on bleeding and pain. Although they are recommended for menstrual suppression in adolescents with disabilities, there are limited data on their use in this population. Our objective is to describe the characteristics and experiences of levonorgestrel IUD use in nulliparous children, adolescents, and young adults with physical, intellectual, and developmental disabilities. METHODS: A retrospective chart review was conducted for all nulliparous patients ages ≤22 with physical, intellectual, or developmental disabilities who had levonorgestrel IUDs placed between July 1, 2004, and June 30, 2014, at a tertiary-care children's hospital. Descriptive statistical analysis and survival analysis were performed. RESULTS: In total, 185 levonorgestrel IUDs were placed in 159 patients with disabilities. The mean age was 16.3 (3.3; range of 9-22) years. Only 4% had ever been sexually active; 96% of IUDs were inserted in the operating room. IUD continuation rate at 1 year was 95% (95% confidence interval: 93%-100%) and at 5 years was 73% (95% confidence interval: 66%-83%). The amenorrhea rate was ∼60% throughout the duration of IUD use among those with available follow-up data. Side effects and complications were ≤3%. CONCLUSIONS: In this study, we provide evidence for the therapeutic benefit and safety of levonorgestrel IUD use in adolescents and young adults with physical, intellectual, and developmental disabilities. It should be considered as a menstrual management and contraceptive option for this population.

Normative values for ultrasound measurements of the female pelvic organs throughout childhood and adolescence.

BACKGROUND: Normative data from pelvic ultrasonography (US) of the pediatric female reproductive organs are outdated and limited by sample size. OBJECTIVE: The purpose of this study was to report normal uterine and ovarian volumes and endometrial stripe thickness in children and young adults and throughout the menstrual cycle in post-menarchal adolescents. MATERIALS AND METHODS: Females ages 0 through 20 years who underwent pelvic US between January 2010 and May 2014 were identified. After excluding patients with pelvic and/or endocrine pathology, nomograms of uterine and ovarian volumes and endometrial thickness by age year were created. Data for patients ages 12 years and older with a recorded day of last menstrual period were used to create additional nomograms of volumes/thickness throughout the menstrual cycle. Student's t-tests and linear regression were performed to assess differences in measurements between groups and association of volumes/thickness with age. RESULTS: During our study period, 5,647 patients underwent 6,953 pelvic US examinations. After further review, 907 examinations from 889 patients were included (mean age: 11.3±6.0 years). Mean pelvic US volumes (cm3) per organ were 25.5±27.0 (uterus), 4.5±4.7 (right ovary) and 4.0±4.1 (left ovary). Mean endometrial thickness was 4.5±3.7 mm. Right ovarian volume was significantly larger than the left (P=0.0126). Uterine volume, ovarian volume and endometrial thickness were significantly associated with age (P-values<0.0001). Plots of mean organ measurements with respect to week of menses are provided. CONCLUSION: We report normal volumes of the uterus and ovaries and endometrial stripe thickness measured by pelvic US throughout childhood and adolescence with reference to the menstrual cycle. These values are significantly associated with age and vary visually by menstrual cycle week.

Menstrual, Sexual, and Obstetrical Outcomes after Vaginal Replacement for Vaginal Atresia Associated with Anorectal Malformation.

Background The authors of this article became aware of significant differences in the management of two females with a rectovestibular fistula and associated vaginal agenesis. In one patient, a sigmoid colovaginoplasty was performed at the time of the posterior sagittal anorectoplasty (PSARP), and the other underwent repair of the rectovestibular fistula with a primary PSARP, but the surgeons elected to delay the timing of vaginal reconstruction. We decided to review the literature, to establish if recommendations could be made to optimize the management of these children based on current evidence. Methods A literature review was conducted to determine the management and long-term outcomes in patients with an anorectal malformation and associated vaginal atresia. Specific gynecological outcomes assessed were menstrual egress and adequacy of the vaginal replacement for penile-vaginal intercourse. Results Eighty-eight cases were included in the review. Age at diagnosis had a bimodal distribution: 0 to 5 years, 56%; >10 years, 37%. Vaginal atresia was recognized before the operation in 45 patients. Types of vaginal atresia encountered were: (a) distal vaginal atresia (n = 17), (b) vaginal agenesis with absent Mullerian development (n = 47), (c) vaginal agenesis with variable Mullerian development (n = 17), and (d) cervico-vaginal agenesis (n = 7). Types of vaginal replacement used were sigmoid colovaginoplasty (n = 26), distal rectal fistula as neovagina (n = 30), terminal ileum (n = 5), vaginal pull-through (n = 9), and others/unknown (n = 8). Two patients followed a perineal dilatation program and nine patients await reconstruction. Median follow-up was 18 months (6 weeks to 31 years). Long-term menstrual outcomes were reported in 18 (21%) patients. Sexual function was reported in 10 (11%) patients. Three pregnancies were reported but none resulted in live births. Conclusion Vaginal atresia is often missed in association with ARMs. Management should be in collaboration with pediatric gynecologists. Due to a lack of long-term outcome data, no definite conclusion can be drawn for the best technique, tissue, or timing of vaginal replacement. The opportunity to perform vaginal replacement in conjunction with the rectal repair may be worth considering because of a shallower pelvis, nonscarred tissue planes, and the excellent surgical exposure.

Ethical quandaries in gamete-embryo cryopreservation related to oncofertility.

While cancer rates continue to increase, therapy has dramatically decreased the mortality rates. The increased efficacy of current therapies may unfortunately have profound toxic effects on gamete function in both adolescent and reproductive age groups, with infertility as an expected consequence of cancer therapy. Significant progress in the advancement of fertility preservation therapies provides realistic options for future fertility in cancer survivors. However, a number of challenging issues need to be considered when presenting fertility preservation options. This overview highlights some of these considerations including religious-cultural-ethical values, access to care and cost of services, developmental capacity and consent, and posthumous reproduction.

Safety, efficacy, actions, and patient acceptability of drospirenone/ethinyl estradiol contraceptive pills in the treatment of premenstrual dysphoric disorder.

Premenstrual dysphoric disorder (PMDD) is estimated to affect 3%-8% of reproductive age women. Multiple therapeutic modalities have been evaluated with varying efficacy for the associated somatic and mood symptoms. The majority of older studies had shown that oral contraceptive pills (OCs) were most effective for the physical symptoms. However, newer OCs containing a novel progestin, drospirenone, have shown promise in alleviating both the somatic and affective/behavioral symptoms. This progestin, which is a derivative of spironolactone, has both antimineralocorticoid and antiandrogenic activity. A 24/4 formulation containing 20 µg of ethinyl estradiol has been found effective in randomized double-blind placebo-controlled trials utilizing established scales documenting symptoms associated with PMDD. Multiple studies have shown that drospirenone-containing OCs are safe without evidence of clinically adverse effects on carbohydrate metabolism, lipids, blood pressure, weight, serum potassium or increased thrombotic events compared to other low dose OCs. In addition, significant improvements have been demonstrated in acne, hirsutism, and fluid retention symptoms. Several open label studies demonstrated good patient compliance and reported satisfaction with the method. Because of the significant placebo effect demonstrated in the blinded placebo-controlled trials, additional large randomized placebo-controlled trials are needed to confirm the efficacy of the drospirenone OCs in the treatment of PMDD. However, this OC formulation appears to be a promising therapeutic modality.

Update in Müllerian anomalies: diagnosis, management, and outcomes.

PURPOSE OF REVIEW: Müllerian anomalies include a fascinating constellation of congenital malformations. There is significant diversity in anatomic variants and their respective long-term sexual and reproductive outcomes. We review the current controversies in classification and management of vaginal, uterine, and fallopian tube anomalies. RECENT FINDINGS: Comparative trials of preoperative magnetic resonance imaging (MRI) and laparoscopic intraoperative evaluation have demonstrated a moderately well correlated prediction of anatomic description. Three-dimensional ultrasound technology appears to be equivalent to MRI in detecting uterine anomalies; however MRI is a consistently superior method of evaluating the vaginal and cervical anatomy. Despite advances in both modalities, care at an experienced center is most highly associated with an accurate preoperative diagnosis and a decrease in the number of inappropriate surgical procedures.Large case series continue to be the main vehicle by which treatment and surgical management of these unique anomalies are described and recommended. Case reports continue to provide information on novel approaches to improve operative techniques. In the absence of prospective studies, these series provide the only emerging information on the long-term sexual and reproductive function of women with vaginal and uterine anomalies. SUMMARY: Recent developments in three-dimensional ultrasonography and MRI improve our ability to accurately describe and diagnose female reproductive tract anomalies. With the description of new complex malformations, which do not fall into the recognized American Society of Reproductive Medicine, formerly American Fertility Society (AFS) classification system, questions arise regarding embryologic development upon which this classification system is based and support attempts to devise a new, comprehensive classification. Advances in surgical correction have expanded the options for the reconstructive surgeon when approaching a patient with an anomaly of the reproductive tract.

Müllerian anomalies.

The development of the Müllerian system and the female reproductive tract is a complex process involving an integrated series of events with significant potential for abnormal development and anomalies. Structural anomalies of the female reproductive tract may be isolated or occur in association with other congenital anomalies, including renal or bladder anomalies and anorectal malformations. Although rare in occurrence, it is important to be familiar with these conditions for appropriate diagnosis, management, and possible referral. The diagnosis, management, and surgical treatments of female reproductive tract anomalies in girls and young women have advanced with improvements in diagnostic imaging techniques, surgical and nonsurgical techniques and innovative instrumentation and developments.

Surgical correction of vaginal anomalies.

Summary of accurate diagnosis and surgical management of vaginal anomalies. Imperforate hymen, transverse septa, and distal vaginal agenesis present similarly with pain and hematocolpos. Likewise, imperforate hymen, transverse septa, distal vaginal agenesis, and Müllerian aplasia may appear similarly on examination. Imaging should be used to better differentiate the anomaly. Although surgical correction is the mainstay of hymenal anomalies, septa, obstructed hemivaginas, and distal vaginal agenesis, first-line therapy for Müllerian aplasia is progressive dilation. Many surgical techniques are also described but no consensus exists as to the best one. Clinicians should be aware of the appropriate evaluation, differential diagnosis, and management options available for vaginal anomalies.

US findings of adnexal torsion in children and adolescents: size really does matter.

BACKGROUND: Adnexal torsion is rare, and symptoms are nonspecific. Clinicians often rely on US examinations to evaluate girls with abdominal or pelvic pain. OBJECTIVE: To determine which sonographic findings can predict adnexal torsion by comparing pediatric and adolescent patients with surgically confirmed torsion (cases) to those without torsion (controls). MATERIALS AND METHODS: Cases and controls were identified retrospectively by searching 7 years of medical record and radiology databases. An adnexal ratio was calculated as the volume of the affected adnexa divided by the volume of the unaffected adnexa. RESULTS: We identified 61 menarchal subjects: 33 cases and 28 controls. Adnexal volume was larger in cases than in controls (185 vs. 37.8 ml, P < 0.001). A volume of >75 ml was more common in cases than in controls (64 vs. 15%, P < 0.001). No cases had an adnexal volume of <20 ml (P < 0.001). The adnexal ratio was larger in cases than in controls (16.1 vs. 6.7, P < 0.001). An adnexal ratio of >15 was seen in 40% of cases and in no controls (P = 0.08). Doppler US results were not predictive of torsion. CONCLUSION: An adnexal volume of <20 ml is strong evidence against adnexal torsion in menarchal females. In this age group, the diagnosis of torsion cannot be established by US examination alone.

Menstruation in girls and adolescents: using the menstrual cycle as a vital sign.

Young patients and their parents often are unsure about what represents normal menstrual patterns, and clinicians also may be unsure about normal ranges for menstrual cycle length and amount and duration of flow through adolescence. It is important to be able to educate young patients and their parents regarding what to expect of a first period and about the range for normal cycle length of subsequent menses. It is equally important for clinicians to have an understanding of bleeding patterns in girls and adolescents, the ability to differentiate between normal and abnormal menstruation, and the skill to know how to evaluate young patients' conditions appropriately. Using the menstrual cycle as an additional vital sign adds a powerful tool to the assessment of normal development and the exclusion of pathological conditions.

Adnexal torsion in pediatric and adolescent girls.

PURPOSE OF REVIEW: This review provides timely information to assist in solving the diagnostic dilemma of adnexal or ovarian torsion. Knowledge and awareness of current literature is essential to help clinicians improve diagnostic accuracy and avoid potentially catastrophic consequences, including loss of ovarian tissue and function. RECENT FINDINGS: This article reviews recent evidence regarding radiologic tools used for efficient, timely diagnosis and management strategies for adnexal torsion, including detorsion. The role of oophoropexy in the pediatric and adolescent population is also reviewed. SUMMARY: The discussion reinforces the role of detorsion in lieu of oophorectomy or adnextomy in an effort to preserve reproductive capacity in a young population. Long-term follow-up, including reproductive outcomes, is needed to determine the role of oophoropexy.