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\u27s 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

Complexities of gender assignment in 17β-hydroxysteroid dehydrogenase type 3 deficiency: is there a role for early orchiectomy?

BACKGROUND: 17β-Hydroxysteroid dehydrogenase type-3 (17βHSD-3) deficiency is a rare cause of 46,XY disorders of sex development. The enzyme converts androstenedione to testosterone, necessary for masculinization of male genitalia in utero. 17βHSD-3 deficiency is frequently diagnosed late, at puberty, following virilization, with consequent female-to-male gender reassignment in 39-64%. The decision for sex of rearing is difficult, especially if diagnosed in early childhood. Consensus guidelines are equivocal or support male gender assignment. Long-term outcomes data to guide decisions are also lacking; however, in the few cases of early diagnosis and orchiectomy, female gender retention appears more likely. We report two patients with 17βHSD-3 deficiency, who presented at unusual ages, in whom female gender was chosen. We performed a focused literature review and summary of gender outcomes in 17βHSD-3 deficiency following early orchiectomy. CASES: Patient A was a phenotypic female who presented at one year of age with bilateral inguinal hernias and external female genitalia. Testes were identified at surgery. The karyotype was 46,XY. She was initially diagnosed with complete androgen insensitivity syndrome; however, androgen receptor mutation analysis was negative. Human chorionic gonadotropin stimulation yielded a low testosterone: androstenedione ratio (0.6, normal >0.8). Genetic testing demonstrated compound heterozygosity for two known mutations of the HSD17B3 gene. She underwent bilateral orchiectomy at two years of age. Patient B was born with female genitalia and virilized at 13 years of age. She did not seek evaluation until 22 years of age. Her karyotype was 46,XY. She had bilateral inguinal testes and low testosterone: androstenedione ratio (0.3). HSD17B3 gene sequencing showed her to be a compound heterozygote for two known mutations. She identified herself as female and underwent bilateral orchiectomy and estrogen replacement therapy. CONCLUSIONS: These two patients highlight the complexities of diagnosis and management in 17βHSD-3 deficiency. Although existing data are limited, early orchiectomy is likely to result in retention of female gender identity, avoiding the complications related to virilization in adolescence. As such, it is important to pursue a definitive diagnosis to guide clinical decisions, and to have the support and long term follow up with an inter-disciplinary disorders of sex development team

A View from the Past Into our Collective Future: The Oncofertility Consortium Vision Statement

Today, male and female adult and pediatric cancer patients, individuals transitioning between gender identities, and other individuals facing health extending but fertility limiting treatments can look forward to a fertile future. This is, in part, due to the work of members associated with the Oncofertility Consortium. The Oncofertility Consortium is an international, interdisciplinary initiative originally designed to explore the urgent unmet need associated with the reproductive future of cancer survivors. As the strategies for fertility management were invented, developed or applied, the individuals for who the program offered hope, similarly expanded. As a community of practice, Consortium participants share information in an open and rapid manner to addresses the complex health care and quality-of-life issues of cancer, transgender and other patients. To ensure that the organization remains contemporary to the needs of the community, the field designed a fully inclusive mechanism for strategic planning and here present the findings of this process. This interprofessional network of medical specialists, scientists, and scholars in the law, medical ethics, religious studies and other disciplines associated with human interventions, explore the relationships between health, disease, survivorship, treatment, gender and reproductive longevity. The goals are to continually integrate the best science in the service of the needs of patients and build a community of care that is ready for the challenges of the field in the future

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

Lesley L Breech, Paula K BravermanDivision of Adolescent Medicine, Department of Pediatrics, Cincinnati Children’s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH, USAAbstract: 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.Keywords: drospirenone, premenstrual dysphoric disorder, premenstrual syndrome, oral contraceptive pil

Rectovestibular fistula-rarely recognized associated gynecologic anomalies

Introduction: Vestibular fistulas are the most common anorectal malformations (ARMs) in females. Associated gynecologic defects are rarely mentioned in the literature but may have serious clinical implications if undetected. The definitive repair of the ARM offers an opportunity for diagnosis and treatment of these conditions. Methods: Two hundred seventy-two patients with vestibular fistula were retrospectively reviewed, with emphasis on gynecologic defects. Results: Forty-eight patients (17%) had 83 gynecologic anomalies. Fourteen patients had a vaginal septum, all with 2 uterine cervices. All septa were resected at the main repair. Twenty-six patients had no vaginal opening. Twenty of them had absent vagina. Eighteen of those had an absent uterus. Patients with absent vagina underwent vaginal replacement with distal rectum (12), sigmoid (6), and ileum (2). Six patients had a patent upper vagina; 3 reached the perineum after mobilization and 3 required replacement; 2 with sigmoid and 1 with rectum. Conclusion: Vaginal septa are easily diagnosed and can be resected during the repair of the vestibular fistula. The presence of 2 cervices has important obstetric implications. Absent vagina requires a technically demanding repair, with special preoperative planning. Vaginoscopy or careful inspection should precede surgical reconstruction. © 2009 Elsevier Inc. All rights reserved

Covered cloacal exstrophy - A poorly recognized condition: Hints for a correct diagnosis

Introduction: Covered cloacal exstrophy requires a high index of suspicion for its diagnosis. Low implantation of the umbilical cord, separated pubic bones, and anorectal malformation are the most common signs. Methods: Thirty-one patients with this defect were retrospectively analyzed. Results: Besides the anorectal malformation, the patients had important unique anatomic findings, including a colon shorter than 20 cm (17 patients) and absent bladderneck (27 patients). Twenty-four patients underwent a colonic pullthrough; of those, only 5 of them have voluntary bowel movements. Twelve patients underwent a urinary reconstruction. Eleven of them are dry with catheterization, and one leaks in between catheterization. Two patients are urinary continent. Conclusions: Covered exstrophy is a serious condition. Externally, the patients may look like having a rather simple malformation. However, the intra-abdominal findings are similar to those seen in cloacal exstrophy. An early correct diagnosis is important to plan a reconstructive strategy and to adjust the parent\u27s expectations concerning bowel and urinary function. In addition to the traditional prognostic factors for bowel and urinary control (sacral ratio, tethered cord, and level of the rectum) these patients have other anatomic defects (absent bladderneck and short colon) that negatively affect the functional prognosis. © 2013 Elsevier Inc. All rights reserved

Hydrocolpos in cloacal malformations

Introduction: Hydrocolpos is a condition rarely mentioned in the literature. The purpose of this report is to increase the index of suspicion for hydrocolpos in patients with cloaca and to describe our approach for its treatment with the hope that errors in the management of hydrocolpos can be avoided. Methods: We reviewed 411 medical records of patients diagnosed with cloaca and managed at our Center during the last 26 years. Emphasis was placed on evaluating for the presence of hydrocolpos, type of drainage, and complications related to the persistence of the hydrocolpos. Results: One hundred seventeen cloaca patients had an associated hydrocolpos (28.4%). Forty-two cases (36%) were initially managed at other institutions at which the hydrocolpos was not drained. Complications experienced by this group included: multiple urinary tract infections (8), hydrocolpos infection (7), sepsis (7), failure to thrive (6), ruptured hydrocolpos (4), and development of hydronephrosis in previously normal kidneys (2). Forty-one patients (35%) had other modalities of treatment, aimed to drain the hydrocolpos, including vesicostomy (26), intermittent perineal catheterization (8), single aspiration (6), or plasty of the perineal orifice (1). In all of these cases, the hydrocolpos persisted or reaccumulated. Thirty-four patients (29%) underwent an effective drainage of the hydrocolpos at birth; 29 at other institutions, 15 with a tube vaginostomy, 13 with a tubeless vaginostomy, and 1 with a catheter placed and left in the vagina through cystoscopy. Five cases had a tube vaginostomy done by us. In all these cases, the vagina remained adequately drained as demonstrated radiologically. Proper drainage of the hydrocolpos alone, with no urologic intervention, dramatically improved the hydronephrosis in 13 cases. Conclusions: Hydrocolpos in patients with cloacas must be diagnosed and treated early in life. Our preferred approach is a transabdominal indwelling vaginostomy tube. The drainage of the hydrocolpos alone may dramatically improve the hydronephrosis, and therefore, we suggest that only after the hydrocolpos is drained should a urological intervention be contemplated. Failure to drain the hydrocolpos can result in serious complications. © 2010 Elsevier Inc. All rights reserved

Vaginal switch - A useful technical alternative to vaginal replacement for select cases of cloaca and urogenital sinus

Aim The aim of this study was to describe the indications, technical details, and complications of a surgical maneuver designed to repair the vagina in select cases of cloaca and urogenital sinus. Methods A vaginal switch maneuver (VSM) is applicable when patients have two high large hemivaginas, and the distance between both hemiuteri is longer than the longitudinal length of the vaginas. It consists of resecting one hemiuterus and the vaginal septum, tubularizing both hemivaginas to create a single one, and switching down the vaginal dome of the side of the resected hemiuterus. Sixty patients who underwent this procedure were reviewed. Results Mean common channel length was 5.2 cm. Complications occurred in twenty-six (43%) and included: acquired vaginal atresia that later required a vaginal replacement (n = 11), introital stricture (n = 6), incidental oophorectomy owing to damaged blood supply (n = 4), vaginal-urethral fistula (n = 3), partial vaginal dehiscence (n = 1), and partial vaginal mucosal prolapse (n = 1). In four cases VSM was unsuccessfully attempted owing to ischemia, and a partial vaginal replacement was performed. Forty-five patients (75%) were able to keep their native vaginal tissue as a vagina. Thirty patients are older than 12 years of age, and eight are menstruating. Conclusion The vaginal switch maneuver is a useful alternative to vaginal replacement. In spite of the morbidity, it is valuable because of the inherent advantages of preserving native vaginal tissue. © 2013 Elsevier Inc