Kate McCracken

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 patients 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 patients 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.

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.

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 Centers 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, Crohns 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.

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.

Gynecologic anatomic abnormalities following anorectal malformations repair

BACKGROUND/AIM Patients may present with gynecologic concerns after previous posterior sagittal anorectoplasty (PSARP) for repair of an anorectal malformation (ARM). Common findings include an inadequate or shortened perineal body, as well as introital stenosis, retained vaginal septum, and remnant rectovestibular fistula. An inadequate or shortened perineal body may impact fecal continence, sexual function and recommendations regarding obstetrical mode of delivery. We describe our experience with female patients referred to our center for evaluation of their previously repaired ARM, with a specific focus on perineal body anatomy and concomitant gynecologic abnormalities. We outline our collaborative evaluation process and findings as well as subsequent repair and outcomes. MATERIAL/METHODS A single site retrospective chart review from May 2014 to May 2016 was performed. Female patients with a history of prior ARM repair who required subsequent reoperative surgical repair with perineoplasty were included. The decision for reoperation was made collaboratively after a multidisciplinary evaluation by colorectal surgery, urology, and gynecology which included examination under anesthesia (EUA) with cystoscopy, vaginoscopy, rectal examination, and electrical stimulation of anal sphincters. The type of original malformation, indication for reoperative perineoplasty, findings leading to additional procedures performed at time of perineoplasty, postoperative complications, and the length of follow up were recorded. RESULTS During the study period 28 patients were referred for evaluation after primary ARM repair elsewhere and 15 patients (60%) met inclusion criteria. Thirteen patients (86.6%) originally had a rectovestibular fistula with prior PSARP and 2 patients (13.4%) originally had a cloacal malformation with prior posterior sagittal anorectovaginourethroplasty. The mean age at the time of the subsequent perineoplasty was 4.6years (0.5-12). Patients had an inadequate perineal body requiring reoperative perineoplasty due to: anterior mislocation of the anus (n=11, 73.3%), prior perineal wound dehiscence with perineal body breakdown (n=2, 13.4%), acquired rectovaginal fistula (n=1, 6.6%), and posterior mislocated introitus with invasion of the perineal body (n=1, 6.6%). During the preoperative evaluation, additional gynecologic abnormalities were identified that required concomitant surgical intervention including: introital stenosis (n=4, 26.6%), retained vaginal septum (n=3, 20%) and remnant recto vestibular fistula (n=2, 13.3%). CONCLUSIONS Patients with a previously repaired ARM may present with gynecologic concerns that require subsequent surgical intervention. The most common finding was an inadequate perineal body, but other findings included introital stenosis, retained vaginal septum and remnant recto vestibular fistula. Multidisciplinary evaluation to assess and identify abnormalities and coordinate timing and surgical approach is crucial to assure optimal patient outcomes. TYPE OF STUDY Case series with no comparison group. LEVEL OF EVIDENCE IV.

A standardized approach for the assessment and treatment of internationally adopted children with a previously repaired anorectal malformation (ARM).

INTRODUCTION A significant number of internationally adopted children have congenital birth defects. As a specialist center for colorectal diagnoses, we evaluate such children with an anorectal malformation (ARM) and have found that a significant number need a reoperation. Knowledge of the common complications following ARM surgery has led us to develop treatment algorithms for patients with unknown past medical and surgical history, a situation typically encountered in the adopted population. METHODS The results of investigations, indications, and rate of reoperation were assessed for adopted children with an ARM evaluated between 2014 and 2016. RESULTS 56 patients (28 males) were identified. 76.8% required reoperative surgery. Mislocation of the anus outside the sphincter complex was seen in 50% of males and 39.3% of females. Anal stricture, rectal prolapse, retained vaginal septum, and a strictured vaginal introitus were also common. CONCLUSION The reoperative surgery rate in the internationally adopted child with an ARM is high. Complete, systematic evaluation of these children is required to identify complications following initial repair. Development of mechanisms to improve the primary surgical care these children receive is needed.

Gynecologic Pain in Adolescents

Opinion statementGynecologic pain affects a significant number of adolescents. While the differential diagnosis for such pain is often extensive, the treatment goals are the same—to relieve pain and minimize the effect it has on an adolescent’s daily functioning, and to maximize their future reproductive potential. For management of acute gynecologic pain—such as in cases of adnexal torsion or obstructive anomalies—surgical intervention is often warranted. Pain related to more chronic processes—such as endometriosis—is treated both medically and surgically. Hormonal therapy (either combined estrogen/progestin or progestin-only) is the mainstay of medical therapy. Newer methods, such as the levonorgestrel intrauterine devices (LNG-IUD), show promise as a management option for chronic endometriosis-related pain. The LNG-IUD also provides highly effective long-acting reversible contraception, and is a first-line recommended contraceptive method for adolescents. Other etiologies of gynecologic pain may be infectious and are therefore treated by antibiotics. Providers must perform a thorough history and physical exam—with attention to patient confidentiality. Establishing a good rapport with both patients and their caregivers is crucial. Regular follow-up to assess treatment efficacy is necessary—as certain conditions may require a stepwise progression of treatment modalities for resolution of symptoms. While it is often challenging caring for an adolescent with chronic gynecologic pain, it is also extremely important and rewarding. Optimizing the gynecologic health of adolescent females will allow them to function at their full potential and lead productive, enjoyable lives.