Is Collaborative Care the Future of Medicine? Lessons Learned from the Care of Children with Colorectal Conditions.

The treatment of patients with colorectal disorders requires care from a wide variety of medical and surgical specialties over the course of their lifetime. This is ideally handled by a collaborative center which facilitates the assessment and development of patient care among multiple specialties which can enhance the quality and implementation of treatment plans, improve communication among different specialties, decrease morbidity, and improve patient satisfaction and outcomes. This collaborative approach can serve as a model for other parts of medicine requiring a similar multi-disciplinary and integrated method of care delivery. We describe the process, as well as the lessons learned in developing such a program.

Fetal and Newborn Management of Cloacal Malformations.

Cloaca is a rare, complex malformation encompassing the genitourinary and anorectal tract of the female in which these tracts fail to separate in utero, resulting in a single perineal orifice. Prenatal sonography detects a few cases with findings such as renal and urinary tract malformations, intraluminal calcifications, dilated bowel, ambiguous genitalia, a cystic pelvic mass, or identification of other associated anomalies prompting further imaging. Multi-disciplinary collaboration between neonatology, pediatric surgery, urology, and gynecology is paramount to achieving safe outcomes. Perinatal evaluation and management may include treatment of cardiopulmonary and renal anomalies, administration of prophylactic antibiotics, ensuring egress of urine and evaluation of hydronephrosis, drainage of a hydrocolpos, and creation of a colostomy for stool diversion. Additional imaging of the spinal cord and sacrum are obtained to plan possible neurosurgical intervention as well as prognostication of future bladder and bowel control. Endoscopic evaluation and cloacagram, followed by primary reconstruction, are performed by a multidisciplinary team outside of the neonatal period. Long-term multidisciplinary follow-up is essential given the increased rates of renal disease, neuropathic bladder, tethered cord syndrome, and stooling issues. Patients and families will also require support through the functional and psychosocial changes in puberty, adolescence, and young adulthood.

Clinical Quiz-Newborn Female with an Anorectal Malformation and a Gynecological Abnormality.

We present a case of a newborn female with imperforate anus who on exam was found to have a rectal fistula in the vestibule, no vaginal opening, and a normal urethra. A diagnostic laparoscopy was performed to elucidate the internal anatomy. The case is presented with a focus on surgical strategies in approaching the female patient with anorectal malformation and a Mullerian anomaly, with questions for the readers posed in a quiz format.

Case report: two cases of mature oocytes found in prepubertal girls during ovarian tissue cryopreservation.

OBJECTIVE: To report two cases of mature oocytes found in prepubertal girls undergoing ovarian tissue cryopreservation (OTC). DESIGN: Case report. SETTING: Large tertiary care children's hospital and a private fertility clinic. PATIENTS: An 8-year-old prepubertal girl with ß-thalassemia and a 2-year-old girl with sickle cell disease who both underwent OTC before bone marrow transplantations. INTERVENTIONS: Laparoscopic right oophorectomy was performed in each patient. The ovarian cortical tissue was processed for slow freezing and long-term storage, and all oocytes were subsequently vitrified. MAIN OUTCOME MEASURES: Oocytes found at the time of OTC processing for fertility preservation. RESULTS: After a complete right oophorectomy, one mature metaphase II oocyte was discovered on tissue processing for OTC in each patient. Neither patient has yet returned for use of tissue or oocytes. CONCLUSIONS: To our knowledge, this is the first report of mature oocytes found during prepubertal OTC processing. These findings may indicate the need for increased research regarding prepubertal oocyte development and suggest that the technique of examining the media for both mature and immature oocytes at the time of OTC should become more widespread and perhaps recommended in prepubertal patients to optimize fertility preservation methods.

DICER1 Mutation Detected in an Infant Guides Accurate Diagnosis of Auto-Amputated Embryonal Rhabdomyosarcoma.

BACKGROUND: The DICER1 mutation is a pathogenic, germline mutation that predisposes patients to uncommon malignancies at a young age. CASE: A 6-month-old female infant presented with vaginal bleeding and a protruding vaginal mass of unclear pathogenesis. Chemotherapy was initially targeted toward a germ cell tumor; after pathologic testing and auto-amputation of the tumor, the patient was diagnosed with a rare DICER1-associated embryonal rhabdomyosarcoma. Subsequently, her treatment course was restructured and family genetic surveillance instituted. SUMMARY AND CONCLUSION: Consideration for DICER1 mutation in tumors with complex pathology and unique presentation is critical to aid in diagnosis and management, and direct future comprehensive surveillance.

Vaginal Yolk Sac Tumor: A Case Series and Review of the Literature.

OBJECTIVE: To report diagnosis, treatment, and outcomes of vaginal yolk sac tumor (YST) cases at a single institution and review literature on vaginal YST to outline advancements in diagnosis, treatment, and survival. DESIGN: Retrospective chart review of female patients less than 21 years of age with pathologic diagnosis of vaginal YST treated at a large children's hospital, and summary of a 100-year review of the literature on vaginal yolk sac tumor. SETTING: Children's Healthcare of Atlanta, a tertiary center in Atlanta, GA. PARTICIPANTS: Female patients less than 21 years of age diagnosed with vaginal YST. RESULTS: Two cases of vaginal YST at our institution are outlined. Both patients presented within the first 2 years of life with vaginal bleeding and were treated successfully with chemotherapy alone. After review of the literature, 137 cases of vaginal YST were found. The mean age at diagnosis was 11 months, and all patients presented with vaginal bleeding. Before 2000, more radical treatments were pursued, and 40% resulted in death. Since the year 2000, treatment has shifted toward chemotherapy and more conservative surgical management, with 51% of vaginal YST cases treated with chemotherapy alone with 92% of patients alive at time of publication. CONCLUSION: Our cases contribute to the limited literature demonstrating the efficacy of conservative management of rare cases of vaginal YST with chemotherapy alone. This case series and review of the literature provide mounting evidence that vaginal YST should be in the differential diagnosis in young girls with vaginal tumors, and conservative management of vaginal YST has excellent outcomes.

Nonvirilized Genitalia in 3 Female Newborns With the Salt-Wasting Congenital Adrenal Hyperplasia Phenotype.

Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency, a form of primary adrenal insufficiency characterized by impaired cortisol secretion and elevated androgen production, is the leading cause of atypical genitalia in the female newborn. Females with classic CAH, either salt-wasting or simple-virilizing form, usually present at birth with atypical genitalia ranging from clitoromegaly to male-appearing genitalia, due to in utero to elevated androgens (androstenedione and testosterone). Females with mild nonclassic CAH usually present with typical genitalia. Proving the importance of always keeping an open mind for exceptions to the rule, we report on 3 female newborns who presented with the nonvirilized genitalia, salt-wasting CAH phenotype and genotype most consistent with simple-virilizing CAH. It is only through a positive newborn screen identifying the females with CAH that they were diagnosed before developing adrenal and/or salt-wasting crisis.

Fertility Options for the Transgender and Gender Nonbinary Patient.

Comprehensive care for transgender and gender nonbinary patients has been a priority established by the World Professional Association for Transgender Health. Because pubertal suppression, gender-affirming hormone therapy, and antiandrogen therapy used alone or in combination during medical transition can affect gonadal function, understanding the effects these treatments have on fertility potential is important for practitioners caring for transgender and gender nonbinary patients. In this review, we outline the impacts of gender-affirming treatments on fertility potential and discuss the counseling and the treatment approach for fertility preservation and/or family building in transgender and gender nonbinary individuals.

Compensatory Ovarian Hypertrophy after Unilateral Oophorectomy: Evaluation of Ovarian Volumes in Pediatric and Adolescent Populations.

STUDY OBJECTIVE: Limited data exist on the morphologic and physiologic effect on the remaining ovary after unilateral oophorectomy, especially in the pediatric population. Our aim is to evaluate ovarian volumes following unilateral oophorectomy to determine whether compensatory ovarian hypertrophy occurs in the remaining contralateral ovary. DESIGN: This was a retrospective chart review of ovarian volume measured on ultrasounds that were completed after unilateral oophorectomy. Postoperative ovarian volumes were compared to established radiologic standards. SETTING: Large tertiary care academic children's hospital in Atlanta, GA. PARTICIPANTS: Female patients less than 21 years old who underwent unilateral oophorectomy. MAIN OUTCOME MEASURES: Ovarian volumes measured on postoperative ultrasounds. RESULTS: A total of 93 patients met inclusion criteria for this study. Serial ultrasounds were performed in slightly more than half of the patients (n = 48, 51.6%), totaling 193 postoperative ovarian volumes. The average age of oophorectomy was 10.8 years. Prior to surgery, the majority of patients presented with abdominal pain (n = 51, 54.8%) or pelvic mass (n = 51, 54.8%), and most (n = 77, 82.8%) had benign final pathology. Ovarian volumes were compared to 4 published radiologic ultrasound standards. More than 62.2% of ovarian volumes from girls who had previously had unilateral oophorectomy were larger than age-matched standard ovarian volumes. CONCLUSION: Ovarian enlargement occurs in the contralateral ovary following unilateral oophorectomy in the pediatric and adolescent population. This supports the concept of compensatory ovarian hypertrophy. This knowledge provides valuable information for interpretation of radiologic images in young female individuals who have undergone oophorectomy, and can assist with counseling on the risk of adnexal complications due to ovarian hypertrophy after unilateral oophorectomy.

Transgender Men and the Gynecologist.

Recently, greater attention has been paid to the care of gender dysphoric and gender incongruent individuals. Gynecologists may be called upon to care for individuals who were assigned female at birth throughout or following social, medical, or surgical gender transition. Thus, gynecologists need to be aware of language regarding sex and gender, treatment typically used for the care of gender dysphoric or incongruent individuals, and aspects of well gynecologic care necessary for these individuals. This review highlights these aspects of care for transgender males to aid the general gynecologist in the care and treatment of these individuals.

Health Care Provider Attitudes Toward Safety of Selected Hormonal Contraceptives in Breastfeeding Women.

OBJECTIVES: Little is known about provider attitudes regarding safety of selected hormonal contraceptives among breastfeeding women. METHODS: Using a nationwide survey, associations were analyzed between provider characteristics and perception of safety of combined oral contraceptives (COCs) in breastfeeding women ≥ 1 month postpartum without other venous thrombosis risk factors and depot medroxyprogesterone acetate (DMPA) in breastfeeding women < 1 month postpartum and ≥ 1 month postpartum. RESULTS: Approximately 68% of public-sector providers considered COCs safe for breastfeeding women ≥ 1 month postpartum without other venous thrombosis risk factors, with lower odds among non-physicians versus physicians (adjusted odds ratios [aOR] range 0.34-0.51) and those with a focus on adolescent health/pediatrics versus reproductive health (aOR 0.68, 95% confidence interval [CI] 0.47-0.99). Most public-sector providers considered DMPA safe for breastfeeding women during any time postpartum, with lower odds among non-physicians versus physicians (aOR range 0.20-0.54) and those with primary clinical focus other than reproductive health (aOR range 0.26-0.65). The majority of office-based physicians considered COCs safe for breastfeeding women ≥ 1 month postpartum without other venous thrombosis risk factors, with lower odds among those who did not use, versus those who used, CDC's contraceptive guidance (aOR 0.40, 95% CI 0.21-0.77). Most office-based physicians also considered DMPA safe for breastfeeding women during any time postpartum. CONCLUSIONS FOR PRACTICE: A high proportion of providers considered use of selected hormonal contraceptives safe for breastfeeding women, consistent with evidence-based guidelines. However, certain provider groups might benefit from education regarding the safety of these methods for breastfeeding women.

Delayed Presentation of Cecal Perforation With Tension-Free Vaginal Tape.

BACKGROUND: Tension-free vaginal tape (TVT) is a popular operative treatment for stress urinary incontinence (SUI). It has a low risk of adverse events, and injuries, particularly to the bowel, are rare. Case reports that have previously discussed these injuries and subsequent removal of TVT have not provided additional insight into management of SUI after these injuries occur. CASE: A postmenopausal woman with persistent SUI presented more than 1 year after TVT placement with bowel perforation incidentally discovered on routine screening colonoscopy. She underwent removal of the TVT and subsequent placement of a fascial sling with postoperative resolution of SUI. CONCLUSION: This case provides additional evidence for bowel injury as a postoperative TVT complication and describes an approach to complicated TVT and persistent SUI.

Preconception Care for the General Ob/Gyn.

Preconception counseling is an important aspect of the care of reproductive-aged women. Asking each woman with each interaction her wishes regarding pregnancy allows the health care provider to investigate her history. The areas to review include environmental toxins, nutrition, genetics, substance abuse, medical conditions, infectious diseases, and psychosocial issues. Then preconception counseling can be individualized. The goal is to decrease or eliminate risks that can cause detriments to the patient or her future pregnancies.

Utility of Direct Pancreatic Function Testing in Children.

OBJECTIVES: Exocrine pancreatic insufficiency (EPI) can have a significant impact on a child's growth and nutrition. Our aim was to evaluate the utility of direct endoscopic pancreatic function testing (ePFT) in pediatrics. METHODS: A single-center retrospective chart review was performed of children who underwent ePFT from December 2007 through February 2015. Endoscopic pancreatic function testings were performed by 1 of 2 methods: (1) intravenous cholecystokinin, followed by the collection of a single duodenal aspirate at 10 minutes, or (2) intravenous cholecystokinin or secretin, followed by the collection of 3 duodenal aspirates at a 5, 10, and 15 minutes. Samples were tested for pH and enzyme activities. RESULTS: A total of 508 ePFTs were performed (481 single-sample tests, 27 multiple-sample tests). Based on the multiple-sample group, enzyme levels for chymotrypsin, amylase, and lipase peaked at 5 minutes, followed by a decrease in activity over time. Exocrine pancreatic sufficiency was identified in 373 (73.4%) and EPI in 93 (18.3%). Exocrine pancreatic sufficiency analysis found all pancreatic enzyme activities significantly increase with age: trypsin, chymotrypsin, amylase, and lipase, (P < 0.05). CONCLUSIONS: Endoscopic pancreatic function testing can be used in the evaluation of EPI in children. Normative data suggest that pancreatic enzyme activities mature with age.

Risk perceptions and subsequent sexual behaviors after HPV vaccination in adolescents.

OBJECTIVES: Concerns have been raised that human papillomavirus (HPV) vaccination could lead to altered risk perceptions and an increase in risky sexual behaviors among adolescents. The aim of this study was to assess whether adolescent risk perceptions after the first vaccine dose predicted subsequent sexual behaviors. METHODS: Young women 13 to 21 years of age (N = 339) completed questionnaires immediately after HPV vaccination, and 2 and 6 months later, assessing demographic characteristics, knowledge/attitudes about HPV vaccination, risk perceptions, and sexual behaviors. Risk perceptions were measured by using 2 5-item scales assessing: (1) perceived risk of sexually transmitted infections (STI) other than HPV, and (2) perceived need for safer sexual behaviors after HPV vaccination. We assessed associations between risk perceptions at baseline and sexual behaviors over the next 6 months by using logistic regression, stratifying participants by sexual experience at baseline and age (13-15 vs. 16-21 years). RESULTS: Among all sexually inexperienced participants (42.5%), baseline risk perceptions were not associated with subsequent sexual initiation; in age-stratified analyses, girls 16 to 21 years of age who reported lower perceived risk for other STI (an inappropriate perception) were less likely to initiate sex (odds ratio [OR] 0.13, 95% confidence interval [CI] 0.03-0.69). Among all sexually experienced participants (57.5%) and in age-stratified analyses, baseline risk perceptions were not associated with subsequent number of sexual partners or condom use. CONCLUSIONS: Risk perceptions after HPV vaccination were not associated with riskier sexual behaviors over the subsequent 6 months in this study sample.