Menstruation and contraception patterns of female adolescent transplant recipients.

BACKGROUND: There is a lack of knowledge regarding menstrual cycles and contraception usage for adolescent transplant patients and families. Solid organ transplant patients start teratogenic medications which have pregnancy implications. We explore adolescent female solid organ transplant recipient and guardian's contraceptive knowledge and attitudes as well as menstrual patterns. METHODS: A cross-sectional descriptive research design was utilized to collect information from adolescent female transplant patients who are on mycophenolate mofetil after solid organ transplant and their guardians within our freestanding children's hospital via a survey. The sample consisted of all female adolescent patients who have completed a solid organ transplant and are on mycophenolate mofetil from April 2016 through May 2017. RESULTS: Twenty-one patients were approached, of which nineteen patients and seventeen guardians completed the survey. The average age of the patient was 16.2 years. The average age at time of transplant was 12.2 years. The type of transplants includes renal (57.1%), heart (23.8%), and liver (4.8%). There were six patients (33.3%) who had a history of sexual activity; among these patients, the mean number of partners in the last year was 1.2. Menstrual concerns included dysmenorrhea, irregular bleeding, and heavy bleeding pre- and post-transplant, respectively. Participants reported contraceptive counseling prior to and after transplant approximately half of the time. CONCLUSIONS: Adolescent solid organ transplant patients have multiple reproductive needs including contraception, dysmenorrhea, and irregular bleeding. Integration of contraceptive knowledge into clinical care received by adolescents with solid organ transplants is supported by knowledge gained through this study.

Endometriosis in adolescents.

PURPOSE OF REVIEW: The current article addresses recent literature regarding the diagnosis and management of endometriosis in adolescents. RECENT FINDINGS: An increasing body of literature suggests that advanced-stage endometriosis (revised scoring system of the American Society for Reproductive Medicine Stage III or IV) and deeply invasive endometriosis are relatively common in adolescents. There remains limited data on the efficacy of postoperative hormonal management of endometriosis in the adolescent population. SUMMARY: Strong consideration should be made for surgical diagnosis of endometriosis in adolescents with pelvic pain, including noncyclic pain, with a concurrent family history of endometriosis and personal history of atopic disease. More research is needed regarding the benefits of the routine use of hypoestrogenic and other hormonal agents in the prevention of disease progression and long-term sequela in adolescents with endometriosis.

Physician attitudes toward over the counter availability for oral contraceptives.

To assess physician attitude towards making oral contraceptives available over the counter in the United States (US). We assessed physician attitudes towards a transition from prescription only to over-the-counter availability (rx-OTC) for oral contraceptive pills by disseminating an electronic survey directed primarily to residents training in Obstetrics and Gynecology (OBGYN) and Family Practice in the US. An overwhelming majority of 638 respondents (71 %) were against an rx-OTC switch for combined oral contraceptives and among this subset of respondents the primary concern was safety (92.3 %). Overall, respondents were evenly divided on the issue of an rx-OTC switch for progestin-only-pills but of those who opposed, 73.2 % cited safety as their primary concern. For progestin-only-pills female respondents were more likely to support OTC availability. Most OBGYN and Family Practice residents opposed to OTC availability for oral contraceptives cite safety as their primary concern. Considering the abundant evidence as to the overall safety of oral contraceptives, especially progestin-only-pills, there appears to be a knowledge deficit among OBGYN and Family Practice residents regarding the safety of oral contraceptives.

What are the factors predictive of hysterosalpingogram compliance after female sterilization by the Essure procedure in a publicly insured population?

To determine what factors are predictive of post-Essure hysterosalpingogram (HSG) compliance. We conducted a retrospective chart review of all patients who underwent the Essure procedure at the two campuses of the Truman Medical Center, Kansas City, Missouri, from January 1, 2005 through December 31, 2010. Our study population consisted primarily of women who were publicly insured (89.0 %) and unmarried (76.7 %). Of 132 patients referred for HSG, 70 (53.0 %) complied. In adjusted analyses women 35 years and older had an almost fourfold higher odds of HSG compliance (OR = 3.72, 95 % CI 1.35-10.23) and women with 3 or more living children had a 64 % lower odds of HSG compliance (OR = 0.36, 95 % CI 0.16-0.82). Women younger than 35 who had 3 or more children had the lowest compliance rate (36.4 %) suggesting an interaction between age and parity. Women undergoing the Essure procedure at the campus with a dedicated protocol to ensure compliance had an almost fourfold higher odds of HSG compliance (OR = 3.67, 95 % CI 1.01-13.40). In a population consisting largely of publicly insured, unmarried women, several factors are predictive of post-Essure HSG compliance. These include age, parity and the presence or absence of an institutional protocol to keep track of patients after their Essure procedure.

Labial Anatomy Concerns in the Adolescent: A Review.

Labial size concerns are an increasingly common chief complaint by both adolescents and adults despite studies showing a wide variation in sizes of the labia minora in the prepubertal, adolescent, and adult population. A thorough history will elucidate what or whom is driving the concerns, which can then direct management. Educating the patient, caregiver, and referring physician is often all that is needed. Surgery should never be used for cosmetic reasons in a minor.

Unique Evaluation and Management Considerations for Adolescents with Late Gynecologic and Colorectal Issues in the Setting of Anorectal Malformations.

STUDY OBJECTIVE: There is little guidance for managing pubertally identified Mullerian anomalies in patients with anorectal malformations (ARMs). We sought to assess these unique issues. DESIGN: Retrospective review SETTING: Single-institution study PARTICIPANTS: Natal female patients aged 10-25, with an ARM, cloaca, or exstrophy, who presented from 2009 to 2019 with a gynecologic concern were included. INTERVENTION: Data collection was performed and included the presenting problem, psychological evaluation, fertility and sexuality concerns, and management strategies for these problems. MAIN OUTCOME MEASURES: The main outcome was unique needs that had to be addressed in the young adult population and the type of colorectal and gynecological procedures needed on representation. RESULTS: Twelve patients were identified; all had gynecologic concerns. Ten had ARMs, including cloaca (n = 3) and cloacal exstrophy (n = 5). Median age at representation was 14.6 years (IQR = 12.7, 15.3). Colorectal revisions included posterior sagittal anorectoplasty (n = 1), resection of bowel attached to urogenital sinus (n = 1), and appendicostomy revision (n = 1). Gynecologic issues included dysmenorrhea (n = 8), obstructed Mullerian anomaly (n = 6), and introital stenosis (n = 5). Behavioral health concerns (n = 9) and fertility/sexuality concerns (n = 4) were identified. Median time from first visit to reconstruction was 1.5 years (IQR = 0.5, 1.5), providing multiple visits to achieve consensus among patients and providers before intervention, including vaginal or introital repair (n = 5) and hysterectomy of obstructed uterine horns (n = 3). CONCLUSIONS: Goal-directed follow-up is required before surgical management to identify psychological and reproductive issues in patients with ARMs who have gynecologic concerns. Patient input and psychologic consultation are helpful for patients requiring staged reconstruction.

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.

Use of intraoperative fluoroscopy during laparotomy to identify fragments of retained Essure microinserts: case report.

In previous case-reports of Essure microinsert perforation, the microinsert was successfully removed at laparoscopy. Herein is discussed the scenario of persistent pelvic pain over several years after an apparently successful laparoscopic retrieval of a perforating right-sided microinsert. In the interim, the patient underwent 2 unsuccessful exploratory laparotomy procedures in an attempt to retrieve additional microinsert fragments that had perforated the uterus. Successful management of Essure microinsert perforation in this patient ultimately required use of intraoperative fluoroscopy. Surgeons performing laparoscopy or laparotomy to retrieve Essure microinserts that have perforated should be aware that these are not always visible to the naked eye, and there should be a low threshold to use intraoperative fluoroscopy to ensure that all perforating fragments have been removed.

Can Boys Have Turner Syndrome? More than a Question of Semantics.

Individuals with 45,X mosaicism with Y chromosome material raised as boys are not diagnosed with Turner syndrome, a label restricted to phenotypic females. We sought to determine if boys with 45,X mosaicism had features consistent with Turner syndrome. Twenty-two patients (14 girls, 8 boys) seen in our Differences of Sex Development (DSD) clinic were identified for review. Standardized height (z-scores) by sex of rearing and results of cardiology, renal, audiology, thyroid, and celiac screenings were recorded. All subjects had heights below the mean for sex. Z-scores were not significantly different between boys and girls (p = 0.185). There were no significant differences in the incidence of cardiac anomalies between boys and girls (p = 0.08). Girls were more likely to have additional screenings (p = 0.042), but there were no significant differences in the number of positive screenings between boys and girls (p = 0.332). Patients with 45,X mosaicism raised as boys appear to have features similar to patients with the same karyotype raised as girls. Routine screening of boys following the Turner Syndrome Clinical Practice Guidelines may allow early recognition of comorbidities. Additionally, obtaining karyotypes on boys with short stature or other features of Turner syndrome may identify unrecognized cases of 45,X mosaicism.

Optimization of Application-Driven Development of In Vitro Neuromuscular Junction Models.

Neuromuscular junctions (NMJs) are specialized synapses responsible for signal transduction between motor neurons (MNs) and skeletal muscle tissue. Malfunction at this site can result from developmental disorders, toxic environmental exposures, and neurodegenerative diseases leading to severe neurological dysfunction. Exploring these conditions in human or animal subjects is restricted by ethical concerns and confounding environmental factors. Therefore, in vitro NMJ models provide exciting opportunities for advancements in tissue engineering. In the last two decades, multiple NMJ prototypes and platforms have been reported, and each model system design is strongly tied to a specific application: exploring developmental physiology, disease modeling, or high-throughput screening. Directing the differentiation of stem cells into mature MNs and/or skeletal muscle for NMJ modeling has provided critical cues to recapitulate early-stage development. Patient-derived inducible pluripotent stem cells provide a personalized approach to investigating NMJ disease, especially when disease etiology cannot be resolved down to a specific gene mutation. Having reproducible NMJ culture replicates is useful for high-throughput screening to evaluate drug toxicity and determine the impact of environmental threat exposures. Cutting-edge bioengineering techniques have propelled this field forward with innovative microfabrication and design approaches allowing both two-dimensional and three-dimensional NMJ culture models. Many of these NMJ systems require further validation for broader application by regulatory agencies, pharmaceutical companies, and the general research community. In this summary, we present a comprehensive review on the current state-of-art research in NMJ models and discuss their ability to provide valuable insight into cell and tissue interactions. Impact statement In vitro neuromuscular junction (NMJ) models reveal the specialized mechanisms of communication between neurons and muscle tissue. This site can be disrupted by developmental disorders, toxic environmental exposures, or neurodegenerative diseases, which often lead to fatal outcomes and is therefore of critical importance to the medical community. Many bioengineering approaches for in vitro NMJ modeling have been designed to mimic development and disease; other approaches include in vitro NMJ models for high-throughput toxicology screening, providing a platform to limit or replace animal testing. This review describes various NMJ applications and the bioengineering advancements allowing for human NMJ characteristics to be more accurately recapitulated. While the extensive range of NMJ device structures has hindered standardization attempts, there is still a need to harmonize these devices for broader application and to continue advancing the field of NMJ modeling.

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.

Vulvovaginal Involvement in Pediatric Stevens-Johnson Syndrome: A Case Series.

BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening spectra of mucocutaneous delayed hypersensitivity reactions. Prodromal viral-like symptoms are followed by a characteristic diffuse rash caused by keratinocyte apoptosis and epidermal detachment. CASES: Three adolescents were admitted with SJS/TEN and vulvovaginal involvement following initiation of lamictal, bactrim, and phenobarbital. The patients received intravenous immunoglobulin and intravenous steroids. One patient received etanercept. Topical emollients and strict perineal hygiene were initiated. No permanent sequelae were noted following vaginoscopy. SUMMARY AND CONCLUSIONS: Vulvovaginal involvement in SJS/TEN can occur and may result in permanent architectural changes. Basic management includes withdrawal of causative medication, intravenous steroids, intravenous immunoglobulin (IVIG), and supportive care. Early initiation of perineal hygiene, vaginal barrier creams, and menstrual suppression should be employed. Vaginoscopy may be used to document full recovery.

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.

High Molecular Diagnosis Rate in Undermasculinized Males with Differences in Sex Development Using a Stepwise Approach.

Differences of sex development (DSDs) are a constellation of conditions that result in genital ambiguity or complete sex reversal. Although determining the underlying genetic variants can affect clinical management, fewer than half of undermasculinized males ever receive molecular diagnoses. Next-generation sequencing (NGS) technology has improved diagnostic capabilities in several other diseases, and a few small studies suggest that it may improve molecular diagnostic capabilities in DSDs. However, the overall diagnostic rate that can be achieved with NGS for larger groups of patients with DSDs remains unknown. In this study, we aimed to implement a tiered approach to genetic testing in undermasculinized males seen in an interdisciplinary DSD clinic to increase the molecular diagnosis rate in this group. We determined the diagnosis rate in patients undergoing all clinically available testing. Patients underwent a stepwise approach to testing beginning with a karyotype and progressing through individual gene testing, microarray, panel testing, and then to whole-exome sequencing (WES) if no molecular cause was found. Deletion/duplication studies were also done if deletions were suspected. Sixty undermasculinized male participants were seen in an interdisciplinary DSD clinic from 2008 to 2016. Overall, 37/60 (62%) of patients with Y chromosomes and 46% of those who were 46XY received molecular diagnoses. Of the 46,XY patients who underwent all available genetic testing, 18/28 (64%) achieved molecular diagnoses. This study suggests that the addition of WES testing can result in a higher rate of molecular diagnoses compared to genetic panel testing.

Management and Prevention of Postoperative Complications in a Neonate with a Symptomatic Imperforate Hymen.

BACKGROUND: Untreated symptomatic imperforate hymens at birth can result in renal complications and ascending infection. Although guidelines recommend incision and drainage, little is discussed regarding postoperative management and surveillance. CASE: A 2-day-old infant with symptomatic imperforate hymen (hydrometrocolpos and hydronephrosis) underwent incision and drainage using sterile technique. On postoperative day 19 she developed hymen reclosure, fluid reaccumulation, and concern for sepsis. After stabilization, redrainage was performed in the operating room with interrupted suture placement around an annular incision. She rapidly improved. Serial postoperative follow-up was performed to ensure ongoing patency of the hymen during healing. SUMMARY AND CONCLUSION: Neonatal hymenotomies can have postoperative complications. We recommend consideration of annular suture placement and close follow-up, because of risk for reclosure and rapid deterioration from infection in this age range.

Dysgerminoma in a 10-Year Old with 45X/46XY Turner Syndrome Mosaicism.

BACKGROUND: Turner syndrome is a genetic disorder resulting from the absence of or structural abnormality of one X chromosome. The presence of Y chromosome material in girls with Turner syndrome confers an increased risk of benign and malignant germ cell tumor and prophylactic bilateral gonadectomy is recommended. CASE: A 10-year-old Turner mosaic syndrome (45X/46XY) patient underwent prophylactic gonadectomy after unremarkable preoperative pelvic imaging. Histopathology showed a streak right gonad, and left gonad with gonadoblastoma with limited degree of infiltrating germinoma. SUMMARYAND CONCLUSION: Gonadoblastoma and dysgerminoma have been reported in girls with Turner mosaic who carry Y chromosome material. Prophylactic gonadectomy should be considered in these girls without delay.

Treatment of Prepubertal Labial Adhesions: A Randomized Controlled Trial.

STUDY OBJECTIVE: Although various treatment options have been proposed for the treatment of labial adhesions, there are currently no clearly outlined limits on the duration of topical therapy, amount of lateral traction to apply, and methods to decrease the recurrence. This clinical trial was undertaken to assess the need for estrogen for treatment of prepubertal labial adhesions. DESIGN: Randomized, double-blinded, controlled trial. SETTING: Pediatric and Adolescent Gynecology Clinic at a children's hospital in a metropolitan area. PARTICIPANTS: Prepubertal girls ages 3 months to 12 years with labial adhesions. INTERVENTIONS: Lateral traction with topical estrogen or topical emollient. MAIN OUTCOME MEASURES: The primary outcome was resolution of labial adhesions. The secondary outcome was the change in severity of labial adhesions over time between the 2 groups. RESULTS: Forty-three girls were enrolled and 38 (88%) completed the study. The difference in complete resolution between the topical emollient group (19%) and the topical estrogen group (36%) was not statistically significant (P = .21). There was a statistically significant decrease in severity of labial adhesions over time, with the magnitude of improvement favoring the topical estrogen group. CONCLUSION: Although labial adhesion severity decreased when treated with lateral traction and topical emollient or topical estrogen, the magnitude of the effect was significantly greater for topical estrogen.

Patterns of Treatment of Accidental Genital Trauma in Girls.

STUDY OBJECTIVE: To evaluate the characteristics of girls with accidental genital trauma (AGT) who can be managed in the emergency department (ED) vs the operating suite (OS). DESIGN: Retrospective cohort. SETTING: ED at a children's hospital in a metropolitan area. PARTICIPANTS: Girls aged 0-18 years with AGT. INTERVENTIONS AND MAIN OUTCOME MEASURES: Factors associated with need for evaluation and repair of AGT in the OS. RESULTS: A total of 359 girls were included in the analysis. The mean age was 6 ± 3 years. Most girls presented with pain and bleeding, 321/359 (89%). Straddle injury was the most common mechanism, 258/355 (73%). The most commonly injured site was the labia, 225/358 (63%) and the most common type of injury was laceration, 308/357 (86%). Factors significantly associated with treatment in the OS included older age, transfer from another institution, penetrating injuries, injuries involving the hymen/vagina/urethra/anus, and injuries larger than 3 cm in size. The odds of requiring general anesthesia in the OS were 5.5 times higher for injuries larger than 3 cm (95% confidence interval, 2.8-10.9; P < .0001) and 4.1 times greater if the patient was transferred from another facility (95% confidence interval, 1.3-13.3; P < .02). CONCLUSION: Most AGT can be managed expectantly. Penetrating injuries, injuries to the hymen/vagina/urethra/anus, and injuries with a maximal size of 3 cm should be considered as indications for management in the OS. With adequate procedural sedation, most girls with minor injuries as a result of AGT can undergo a thorough examination and repair of AGT in the ED.

Decidual cast in a girl receiving depot medroxyprogesterone acetate--a case report.

BACKGROUND: The passage of tissue per vaginam in a young girl raises several diagnostic considerations that include aborted pregnancy, benign polyp, rhabdomyosarcoma, and rarely decidual cast. CASE: A 9-yr-old girl who was receiving depot medroxy-progesterone acetate to suppress menstruation passed tissue per vaginam. Pathological examination of the tissue showed a decidual cast with no chorionic villi, while a serum hCG test was also negative. CONCLUSIONS: Decidual cast formation is a rare side effect of progestogen use and should be considered in the differential diagnosis of passage of tissue per vaginam.

Ethical Controversy About Hysterectomy for a Minor.

One of the most complicated ethical issues that arises in children's hospitals today is the issue of whether it is ever permissible to perform a procedure for a minor that will result in permanent sterilization. In most cases, the answer is no. The availability of good, safe, long-acting contraception allows surgical options to be postponed when the primary goal of such surgical options is to prevent pregnancy. But what if a minor has congenital urogenital anomalies or other medical conditions for which the best treatment is a hysterectomy? In those cases, the primary goal of therapy is not to prevent pregnancy. Instead, sterility is an unfortunate side effect of a medically indicated treatment. Should that side effect preclude the provision of a therapy that is otherwise medically appropriate? We present a case that raises these issues, and asked experts in law, bioethics, community advocacy, and gynecology to respond. They discuss whether the best option is to proceed with the surgery or to cautiously delay making a decision to give the teenager more time to carefully consider all of the options.