Sexual and reproductive health education for patients with myelomeningocele.

AIM: To evaluate the sexual and reproductive health education received by patients with myelomeningocele, the most severe form of spina bifida. METHOD: A survey designed to assess the sexual and reproductive health education given by a healthcare provider to patients with myelomeningocele was offered to all English-speaking patients aged 12 years or older with a myelomeningocele clinic visit. RESULTS: In total, 67 surveys were completed. Menstruation and menstrual management were discussed at a rate of 85% in females. Few patients had discussions with a provider about fertility (42%), sexuality (37%), risk of sexually transmitted infections (45%), or had a relationship with a reproductive healthcare provider (54%). Differences by sex were observed for contraceptive education. The rate of discussions increased with age. A total of 67% of female patients and 33% of male patients requested a referral to a reproductive healthcare provider. INTERPRETATION: Sexual and reproductive health education is part of comprehensive care for all patients and those with disabilities should not be excluded. The lack of consistent education received by patients supports the need for increased attention to this topic. We encourage multidisciplinary myelomeningocele clinics to establish a process for delivering complete and patient-specific sexual and reproductive health education.

The impact of hiring a full-time fertility navigator on fertility-related care and fertility preservation at a pediatric institution.

Fertility navigators (FNs) are important in communicating infertility risk and fertility preservation (FP) options to patients receiving gonadotoxic therapies. This retrospective study examined electronic medical records of patients with fertility consults at a large pediatric institution (2017-2019), before and after hiring a full-time FN. Of 738 patient encounters, 173 consults were performed pre-navigator and 565 post-navigator. Fertility consults for long-term follow-up cancer survivors increased most substantially: pre-navigator (n = 7) and post-navigator (n = 387). Across diagnoses, females had a larger increase in consults compared to males (χ2 [3, N = 738] = 8.17, p < .05). Findings highlight FNs' impact on counseling rates, particularly in survivorship.

Examining predictors and outcomes of fertility consults among children, adolescents, and young adults with cancer.

Infertility has a negative impact on quality of life among cancer survivors. Studies show establishing a fertility team results in improved patient satisfaction. A review of electronic medical records was performed to examine predictors of fertility referrals, interventions, and the impact of an opt-out consult mechanism. Findings show many patients, particularly those that are younger, are still not receiving fertility counseling despite the presence of a fertility team. Notably, patients were 3.6 times more likely to receive a consult after the opt-out. Strategies are needed to improve access to fertility related care, particularly in groups where consults are underutilized.

The National Physicians Cooperative: transforming fertility management in the cancer setting and beyond.

Once unimaginable, fertility management is now a nationally established part of cancer care in institutions, from academic centers to community hospitals to private practices. Over the last two decades, advances in medicine and reproductive science have made it possible for men, women and children to be connected with an oncofertility specialist or offered fertility preservation soon after a cancer diagnosis. The Oncofertility Consortium's National Physicians Cooperative is a large-scale effort to engage physicians across disciplines - oncology, urology, obstetrics and gynecology, reproductive endocrinology, and behavioral health - in clinical and research activities to enable significant progress in providing fertility preservation options to children and adults. Here, we review the structure and function of the National Physicians Cooperative and identify next steps.

Fertility preservation in children and adolescents: current options and considerations.

PURPOSE OF REVIEW: The purpose of this review is to outline current pediatric fertility preservation options, and discuss ethical and financial considerations impacting this rapidly expanding field. RECENT FINDINGS: With the improvement in treatment of pediatric malignancies and medical conditions that threaten fertility, survival rates are increasing. Therefore, minimizing long-term morbidities such as gonadal damage and infertility is of utmost importance. Impaired fertility not only has a significant negative impact on patient's quality of life; in women, gonadal damage puts patients at risk for premature menopause, and increased risk of cardiac, skeletal, and cognitive issues. Fortunately, fertility preservation options exist for both female and male prepubertal and pubertal patients, and discussion of such options with patients and their families prior to the initiation of therapy and/or before further deterioration of gonadal function is crucial. A multidisciplinary approach to fertility counseling, with attention to the patient's goals and cultural beliefs, is ideal. SUMMARY: Although several national organizations support integrating a fertility consultation into routine care, fertility preservation is still underutilized. Continued research is needed to understand barriers for patients/families and reduce the number of missed opportunities for fertility preservation.

The Shared Ovary: A Multidisciplinary Discussion With Pediatric and Adolescent Gynecology.

Pediatric and adolescent ovarian lesions are common and are frequently managed by both pediatric surgeons and pediatric and adolescent gynecologists. During the 2023 American Academy of Pediatric Section on Surgery meeting, an educational symposium was delivered focusing on various aspects of management of pediatric and adolescent benign and malignant masses, borderline lesions, and fertility options for children and adolescents undergoing cancer therapies. This article highlights the discussion during this symposium.

Sexual and Reproductive Health Care after Gonadotoxic Treatment in Females at a Tertiary Pediatric Hospital.

STUDY OBJECTIVES: Recommendations from the Children's Oncology Group Long-Term Follow-Up (COG-LTFU) Guidelines for Survivors of Childhood, Adolescent, and Young Adult Cancer emphasize the importance of reproductive health care, yet little is known regarding adherence to these recommendations and non-fertility-related sexual and reproductive health (SRH) outcomes. METHODS: Follow-up of outcomes on the basis of the COG-LTFU guidelines was assessed in female patients who underwent fertility preservation consultation before gonadotoxic therapy between 2016 and 2022 at a single institution and were at least 6 months from treatment completion. RESULTS: We included 140 patients, with a mean time of 2.7 years from treatment completion. Eighty-six patients were 12 years old or older, of whom sexual activity was recorded in 59 (68.7%), and 12 of 31 (38.7%) sexually active patients underwent sexual function assessment. The 57 (66.3%) patients at high risk of premature ovarian insufficiency (POI) at diagnosis were more likely than minimal-risk counterparts (29, 33.7%) to have abnormal uterine bleeding (42.1% vs 17.2%, P = .03), to be diagnosed with POI (29.8% vs 0%, P = .01), and to have sexual activity recorded (77.2% vs 51.7%, P = .03). Of 17 patients with POI, 82.4% were on hormone replacement therapy, and 58.8% had undergone bone mineral density testing. CONCLUSION: This study adds to the limited literature regarding non-fertility-related SRH outcomes after gonadotoxic therapy and illustrates opportunities to improve adherence to the COG-LTFU guidelines. Increased attention to SRH guidelines may increase detection and treatment of SRH conditions, improving the health and quality of life of female cancer survivors.

Mobile Application Measurement of Menstrual Cycle Characteristics and Their Association with Dysmenorrhea and Activity Limitation in Early Adolescents.

OBJECTIVE: Real-time tracking of menstrual bleeding is a barrier to research due to limitations with traditional data collection tools. This prospective cohort study utilized a mobile application (TDot app) in young adolescents aged 10-14 years to assess the relationship between heavy menstrual bleeding (HMB), dysmenorrhea, and activity limitation. METHODS: Menstrual cycles were captured over six months in real-time using the Pictorial Blood loss Assessment Chart (PBAC). A median PBAC score of >100 was used to identify participants with HMB. Participants also completed a modified WaLIDD (Working ability, Location, Intensity, Days of pain, Dysmenorrhea) scale. Impact of menses on daily activities was collected for each cycle. RESULTS: A total of 160 participants enrolled and 100 (63%) participants with ≥3 cycles recorded in the mobile app were analyzed. HMB was noted in 41% of participants. Median modified WaLIDD score was significantly higher in participants with HMB than those without HMB (p=0.01). No significant differences were found in activity limitations between participants with and without HMB (p=0.34). Median modified WaLIDD score for participants with activity limitation was significantly higher than those without activity limitation (p=0.01). CONCLUSION: Utilizing mobile app technology, we were able to gather real-time menstrual outcome data from young adolescents on heaviness of flow, dysmenorrhea and activity limitations. While we did not find that patients with HMB were more likely to have activity limitations, we did find that those with limitations had modestly higher dysmenorrhea scores. Future studies should focus on identifying additional variables that impact activity limitation during menstruation.

Examining Results of Post-Thelarche Screening Pelvic Ultrasound in Females with Anorectal Malformations.

STUDY OBJECTIVE: Mullerian duct anomalies are common in females with anorectal malformations (ARMs), although there are no universally recommended screening protocols for identification. Historically, at our institution, we have recommended a screening pelvic ultrasound (PUS) 6 months after thelarche and menarche. We aimed to evaluate outcomes associated with our post-thelarche screening PUS in females with ARMs. METHODS: An institutional review board-approved retrospective chart review was performed for all female patients 8 years old or older with ARMs and documented thelarche. Data were collected on demographic characteristics and clinical course. The primary outcome was adherence to the recommended PUS. Secondary outcomes included imaging correlation with suspected Mullerian anatomy and need for intervention on the basis of imaging findings. RESULTS: A total of 112 patients met the inclusion criteria. Of them, 87 (77.7%) completed a recommended post-thelarche screening PUS. There were no differences in completion on the basis of age, race, establishment with a primary care provider, insurance status, or type of ARM. Nine patients (10.3%) had findings on their PUS that did not correlate with their suspected Mullerian anatomy; five (5.7%) required intervention, with two requiring menstrual suppression, two requiring surgical intervention, and one requiring further imaging. CONCLUSION: Most patients completed the recommended post-thelarche screening PUS. In a small subset of patients, PUS did not correlate with suspected Mullerian anatomy and generated a need for intervention. Post-thelarche PUS can be a useful adjunct in patients with ARMs to identify gynecologic abnormalities.

Sexual and Reproductive Health Outcomes in Females With Cloacal Malformations and Other Anorectal Malformations.

STUDY OBJECTIVE: To improve our understanding of reproductive health and sexual function in women with cloacal malformations and other anorectal malformations (ARMs) METHODS: An observational cross-sectional survey was administered to individuals assigned female at birth aged 12 to 55 with ARMs and cloacal malformations cared for at our institution. Data included age of thelarche/menarche and questions on body image, gynecologic anatomy, sexual function, and pregnancy. RESULTS: Twenty-one patients responded in the ARM group and 30 in the cloacal malformation group. There were no differences in median age of thelarche/menarche in patients with ARMs (11/12.5 years) compared with patients with cloacal malformation (11/12 years). Patients with ARMs were more likely to have native vaginal tissue than those with cloacal malformations (n = 18, 82% vs n = 12, 40%; P = .03). There were no differences between groups regarding concerns about dyspareunia and functionality of their vagina (P > .05). Forty-two percent of patients with cloacal malformations and 30% of patients with ARMs reported having been sexually active. Two patients with cloacal malformations and 2 with ARMs reported having been pregnant. Patients with cloacal malformations reported a lower quality of life score (80.4) compared with those with ARMs (87.0) (difference > 4.5). CONCLUSIONS: Patients with a cloacal malformation were less likely to have native vaginal tissue and reported a lower quality of life than those with ARMs. Despite this, patients with a cloacal malformation had similar reproductive health and sexual function compared with patients with ARMs. Our results reinforce the need for comprehensive sexual and reproductive health care for all women with ARMs.

Model-based geostatistical design and analysis of prevalence for soil-transmitted helminths in Kenya: Results from ten-years of the Kenya national school-based deworming programme.

Background: Kenya is endemic for soil-transmitted helminths (STH) with over 6 million children in 27 counties currently at-risk. A national school-based deworming programme (NSBDP) was launched in 2012 with a goal to eliminate parasitic worms as a public health problem. This study used model-based geostatistical (MBG) approach to design and analyse the impact of the NSBDP and inform treatment strategy changes. Methods: A cross-sectional study was used to survey 200 schools across 27 counties in Kenya. The study design, school selection and analysis followed the MBG approach which incorporated historical data on treatment, morbidity and environmental covariates to efficiently predict the helminths prevalence in Kenya. Results: Overall, the NSBDP geographic area prevalence for any STH was estimated to sit between 2 % and <10 % with a high predictive probability of >0.999. Species-specific thresholds were between 2 % and <10 % for Ascaris lumbricoides, 0 % to <2 % for hookworm, and 0 % to <2 % for Trichuris trichiura, all with high predictive probability of >0.999. Conclusions: Based on the World Health Organization guidelines, STH treatment requirements can now be confidently refined. Ten counties may consider suspending treatment and implement appropriate surveillance system, while another 10 will require treatment once every two years, and the remaining seven will require treatment once every year.

Impact of Pelvic Floor Physical Therapy on Function in Adolescents and Young Adults with Biopsy-Confirmed Endometriosis at a Tertiary Children's Hospital: A Case Series.

STUDY OBJECTIVE: The purpose of this case series is to describe the change in capability to perform self-selected activities in adolescent and young adult patients with chronic pelvic pain and surgically proven endometriosis following pelvic floor physical therapy as part of multidisciplinary treatment. DESIGN: Retrospective case series SETTING: Tertiary care pediatric hospital PARTICIPANTS: Twenty patients with ages ranging from 14 to 22 years and a median age of 16.5 years with biopsy-confirmed endometriosis INTERVENTION: Pelvic floor physical therapy MAIN OUTCOME MEASURE(S): Patient-Specific Functional Scale (PSFS) outcome measure RESULTS: Patients had a clinically significant functional improvement (median score of 6.0 points on the PSFS outcome measure; interquartile range, 3.8-13.5) over the course of care (median number of 12 visits; range 4-48 visits) (P < .001). CONCLUSIONS: Patients with chronic pelvic pain and surgically proven endometriosis experienced significant functional improvement after physical therapy treatment. The results of this case series suggest that physical therapy is a viable additional intervention for adolescents and young adults with chronic pelvic pain and endometriosis and warrants further research.

Tubo-Ovarian Abscess in Non-Sexually Active Adolescent Girls: A Case Series and Literature Review.

STUDY OBJECTIVE: We investigated risk factors and common causes of tubo-ovarian abscess (TOA) in non-sexually active females in order to aid in earlier diagnosis, treatment, and improved outcomes. DESIGN: This is a retrospective observational case series of all non-sexually active females younger than age 25 years who were diagnosed with TOA. Review of the existing literature was also performed. SETTING: Academic tertiary care children's hospital. PARTICIPANTS: Ten patients meeting study inclusion criteria were identified for the study, and 33 other patients were identified in the literature. RESULTS: Average age at time of diagnosis was 14 years. Average body mass index was 24 kg/m2. Most presented with abdominal pain, often associated with fevers, nausea, vomiting, and diarrhea. Seven of 10 patients were treated surgically with pelvic washout (4 primarily and 3 after failing empiric antibiotic therapy). Most frequently, anaerobic gut flora were isolated on culture. All patients received broad-spectrum intravenous antibiotics, and were then discharged on a course of doxycycline and metronidazole or clindamycin. Three patients required additional admissions and multiple rounds of antibiotics due to persistent symptoms. The average length of stay was 3 days for patients treated with antibiotics only and 6 days for patients requiring surgical intervention. Six patients had complete resolution of symptoms and improvement on ultrasound within 2-4 weeks. The remainder were lost to follow-up. CONCLUSION: These cases, in conjunction with previous case reports, emphasize the importance of considering TOA in patients with concerning imaging or examination findings despite lack of sexual activity. Given the large proportion of cases attributable to anaerobic gut flora, treatment with antibiotics with adequate anaerobic coverage is recommended. Surgical drainage is not always necessary, but is often needed for diagnostic purposes or in patients not clinically improving with conservative measures.

Surgical Management of an Obstructive Müllerian Anomaly in a Patient with Anorectal Malformation.

Müllerian duct anomalies are rare in the general population, occurring in less than 3% of women, but much more prevalent in female patients with anorectal malformation, occurring in up to 30% of these patients. Unicornuate uterus with a rudimentary non-communicating horn is a congenital anomaly of Mullerian development which can be seen in isolation or in conjunction with other anomalies, with several case reports described in patients with VACTERL association. These anomalies may be asymptomatic until the patient develops dysmenorrhea or devastating obstetrical complications. We describe the successful surgical management of an obstructive Müllerian anomaly in a post-pubertal female patient with anorectal malformation.

Current Fertility Preservation Options for Female Patients With Hodgkin Lymphoma.

IMPORTANCE: Many adolescents and young adults diagnosed with Hodgkin lymphoma (HL) experience disease progression requiring high-dose alkylating salvage therapy, which often results in permanent infertility. OBJECTIVE: The aim of this report is to discuss fertility preservation options in female patients with consideration of chemotherapeutic agents in HL. EVIDENCE ACQUISITION: An electronic literature review was performed utilizing a combination of the terms "Hodgkin lymphoma," "fertility preservation," "ovarian tissue cryopreservation," "oocyte cryopreservation," "embryo cryopreservation," and "gonadotropin-releasing hormone agonist." References and data from identified sources were searched and compiled to complete this review. RESULTS: Initial treatment of HL is often nonsterilizing; however, salvage therapy and conditioning for stem cell transplantation confer significant gonadotoxicity. Established fertility preservation options for pubertal females include embryo cryopreservation and oocyte cryopreservation. These options are contraindicated within 6 months of receipt of chemotherapy. Ovarian tissue cryopreservation is an option for patients who require salvage therapy within 6 months of first-line therapy. CONCLUSIONS: Timing and choice of fertility preservation techniques depends on planned first-line chemotherapy and response to treatment. In patients initially treated with low-risk chemotherapy, it is reasonable to defer invasive fertility techniques until treatment failure; however, upfront fertility preservation should be considered in patients planning to undergo primary treatment with high-risk therapy.

Factors predicting the need for vaginal replacement at the time of primary reconstruction of a cloacal malformation.

BACKGROUND: A subset of patients with cloacal malformations requires vaginal replacement during their primary reconstruction, increasing the surgical complexity. Identifying factors which predict the need for vaginal replacement would facilitate operative planning. METHODS: We retrospectively reviewed patients who underwent primary cloacal reconstruction at our Center (2014-2018) and assessed the length of the common channel, urethra, and vagina. The presence of hydrocolpos at birth, Müllerian anomalies, sacral ratio, and tethered cord were also assessed between patients who did and did not require vaginal replacement. RESULTS: 50 patients were identified. 17/50 patients (34%) underwent a total urogenital mobilization (TUM), and none required vaginal replacement. 33/50 (66%) patients underwent a urogenital separation. 19/33 (58%) required vaginal replacement. This group had a shorter vagina (4.2 cm vs 6.6 cm, p < 0.01). There was no difference in urethral or common channel length, number of cervices, sacral ratio, presence of a vaginal septum, hydrocolpos, or tethered cord between those who did and those who did not require vaginal replacement. CONCLUSIONS: Urethral and common channel lengths were used to successfully determine the operative plan (TUM or urogenital separation) to reconstruct cloacal malformations. The need for urogenital separation and a shorter vaginal length were predictive of the need for vaginal replacement. LEVEL OF EVIDENCE: Level IV. TYPE OF STUDY: Case series with no comparison groups.

Acquired Urethrovaginal Fistula and Urethral Atresia in a Patient with a Sacrococcygeal Teratoma.

Acquired urethrovaginal fistulae and urethral atresia are rare findings in pediatric patients, but have been described in adult patients related to trauma or iatrogenic injury. Little exists in the published literature to guide management of such conditions in children, but lessons learned from congenital causes can help. Herein we discuss the preoperative evaluation and management of a child with an acquired urethrovaginal fistula and urethral atresia likely related to in utero compression from an intrapelvic sacrococcygeal teratoma and provide several images detailing the complex anatomy.

Urethral length in female infants and its relevance in the repair of cloaca.

AIM OF THE STUDY: The goals of urinary reconstruction in urogenital sinus and cloacal repair include: (1) positioning of the bladder neck above the urogenital diaphragm to maximize future urinary continence, and (2) creating a visible urethra that can be catheterized if needed. A recent algorithm in cloacal reconstruction proposed a urethral length of 1.5 cm as the key determinant in deciding whether to perform a total urogenital mobilization or a urogenital separation, the hypothesis being that a 1.5 cm length urethra is needed for the patient to remain dry. We wondered if the normal female urethral length correlated with this empiric technical determinant. METHODS: We reviewed voiding cystourethrograms of healthy female patients between ages 6 and 36 months and measured the patient's urethral length. RESULTS: Ninety-one children were included. The mean urethral length for patients age 6-12 months was 2.50 cm, age 12-24 months was 2.31 cm, and age 24-36 months was 2.59 cm. There was no difference between the urethral length in the three groups (p = 0.38). Of 91 patients, 87 (96%) had a urethral length >1.5 cm. CONCLUSION: A urethra of at least 1.5 cm was present in the majority of normal control patients. We believe therefore that for urogenital sinus and cloacal repair, surgeons can extrapolate that patients need a 1.5 cm urethra at the end of the reconstruction. Additional follow-up is needed to determine if this urethral length as an independent factor maintains dryness in the long term after cloacal repair. TYPE OF STUDY: Case Series. LEVEL OF EVIDENCE: III.

A descriptive model for a multidisciplinary unit for colorectal and pelvic malformations.

INTRODUCTION: Patients with anorectal malformations (ARM), Hirschsprung disease (HD), and colonic motility disorders often require care from specialists across a variety of fields, including colorectal surgery, urology, gynecology, and GI motility. We sought to describe the process of creating a collaborative process for the care of these complex patients. METHODS: We developed a model of a devoted center for these conditions that includes physicians, psychologists, social workers, nurses, and advanced practice nurses. Our weekly planning strategy includes a meeting with representatives of all specialties to review all patients prior to evaluation in our multidisciplinary clinic, followed by combined exams under anesthesia or surgical intervention as needed. RESULTS: There are 31 people working directly in the Center at present. From the Center's start in 2014 until 2017, 1258 patients were cared for from all 50 United States and 62 countries. 360 patients had an ARM (110 had a cloacal malformation, 11 had cloacal exstrophy), 223 presented with HD, 71 had a spinal malformation or injury causing neurogenic bowel, 321 had severe functional constipation or colonic dysmotility, and 162 had other diagnoses including familial polyposis, Crohn's disease, or ulcerative colitis. We have had 170 multidisciplinary meetings, 170 multispecialty outpatient, and 52 nurse practitioner clinics. In our bowel management program we have seen a total of 514 patients in 36 sessions. CONCLUSION: This is the first report describing the design of a multidisciplinary team approach for patients with colorectal and complex pelvic malformations. We found that approaching these patients in a collaborative way allows for combined medical and surgical decisions with many providers simultaneously, facilitates therapy, and can potentially improve patient outcomes. We hope that this model will help establish new-devoted centers in other locations to encourage centralized care for these rare malformations. LEVEL OF EVIDENCE: IV.

Adnexal Torsion.

Adnexal torsion is an uncommon gynecologic disorder caused by the partial or complete rotation of the ovary and/or the fallopian tube on its vascular support. Delay in treatment can impact fertility adversely. The objective of this report is to provide clinical recommendations based on the latest evidence. Specifically we discuss epidemiology, clinical presentation, diagnostic approach and management of adnexal torsion in adolescents.