Development of a multidisciplinary colorectal and pelvic health program: Program implementation and clinical impact.

INTRODUCTION: Pediatric patients with complex colorectal and genitourinary conditions often require coordinated multidisciplinary care; however, this coordinated care can be hard to structure and deliver. The purpose of this paper is to review the development and implementation of a multidisciplinary colorectal and pelvic health program, one year after the program's initiation. METHODS: This is an observational retrospective 1-year study (10/1/2017 to 9/30/2018). In fiscal year (FY) 2018, a multidisciplinary colorectal and pelvic health program was initiated. The program development incorporated bimonthly team meetings, educational conferences, and initiation of three clinics: a complex colorectal and genitourinary reconstruction clinic, a bowel management clinic, and a colonic motility clinic. Conditions treated included complex anorectal and cloacal malformations, Hirschsprung disease, and idiopathic constipation. The fiscal year was selected to provide comparative administrative data after program implementation. RESULTS: During the study period, 121 patients underwent comprehensive collaborative evaluation of which 58 (47%) were new to the institution compared to 12 (19%) new patients in the previous year (p < 0.001). In FY 2018, there were 130 procedures performed and 512 collaborative visits with an average of 47 visits per month. This was a 3.4-fold increase in visits compared to FY2017 (171 visits). Of the new patients, 60% (35/58), traveled a median of 181 miles, representing 33 statewide counties, and 4 states compared to a median of 93 miles in the previous fiscal year (p = 0.004). CONCLUSION: The development of a colorectal and pelvic health program is feasible and requires a collaborative approach, necessitating multiple service lines within an institution. Program creation and implementation can result in rapid institutional clinical growth by filling a local and regional need through coordinated multidisciplinary care. LEVEL OF EVIDENCE: IV.

Intrauterine Devices: Effective Contraception with Noncontraceptive Benefits for Adolescents.

Although adolescent pregnancy and birth rates have been declining since the early 1990s, the rate of intrauterine device (IUD) use in adolescents remain low. IUDs are a highly effective contraceptive method with a failure rate of less than 1%. There are currently 5 IUDs available and marketed in the United States: the nonhormonal copper-containing IUD (Paragard Copper T380A; Ortho-McNeil) and 4 hormonal levonorgestrel-releasing intrauterine systems (LNG-IUDs). IUDs can be used in adolescents, and the LNG-IUD has many noncontraceptive benefits including the treatment of heavy menstrual bleeding, dysmenorrhea, pelvic pain/endometriosis, and endometrial hyperplasia/endometrial cancer. In addition, the LNG-IUD is an effective tool for suppression of menses.

Tubo-Ovarian Abscesses in Nonsexually Active Adolescent Females: A Large Case Series.

PURPOSE: The aim of the study was to review the incidence, presentation, and management of tubo-ovarian abscesses (TOA) in nonsexually active (NSA) adolescents. METHODS: A retrospective chart review was performed at a single children's hospital. We evaluated self-reported NSA females aged < 21 years diagnosed with TOA. Demographics, presenting symptoms, medical history, laboratory studies, imaging, and treatment were reviewed. RESULTS: Sixteen NSA females met inclusion criteria, with one patient presenting with two separate TOAs. Mean age at diagnosis was 14.6 ± 1.8 years; all were menarchal. Presenting symptoms included abdominal pain (88%), fever (76%), and vomiting (53%). Eleven patients (65%) received transabdominal pelvic ultrasound, 2 (12%) had magnetic resonance imaging of pelvis, and 11 (65%) had computed tomography of abdomen/pelvis. All patients had negative gonorrhea and chlamydia testing. Thirteen (76%) had comorbid disease including obstructed hemivagina ipsilateral renal agenesis, active or recent appendicitis, or genitourinary tract anomalies. Sixteen cases underwent drainage via interventional radiology or surgery. Twelve cases (71%) had TOA fluid cultured; five (29%) grew Escherichia coli species. All cases received intravenous antibiotics, and 88% of cases continued outpatient oral antibiotics. CONCLUSIONS: Although uncommon in NSA females, patients with underlying comorbidities may be at increased risk for TOA formation. Providers should consider the diagnosis of TOA even in NSA females.

Paratubal Cyst Size Correlates With Obesity and Dysregulation of the Wnt Signaling Pathway.

STUDY OBJECTIVE: Paratubal cysts (PTCs) occur in 7%-10% of women, regardless of age. Although common, PTCs often are found incidentally because of the potential for these cysts to be asymptomatic. The specific aims of the study were to determine if PTC number and size correlated with signs of hyperandrogenism and obesity, as well as to investigate the molecular profiles of these PTCs in samples derived from female adolescents. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: A prospective cohort study was performed in a single children's hospital. Girls 18 years of age or younger who underwent surgery for PTC suspected on the basis of the presence of a persistent adnexal cyst on imaging or a concern for adnexal torsion involving a cyst were consented to participate in the study. RESULTS: Nineteen patients met enrollment criteria with a mean age at menarche of 11.2 ± 1.3 years. Most of the patients (84%; n = 16/19) had adnexal torsion at the time of diagnosis of PTC. Irregular menses and hirsutism was found in 52.6% (n = 10/19) of the patients, among whom 36.8% (n = 7/19) were obese. The mean PTC size was 10.4 ± 4.3 cm with 57.9% (n = 11/19) of the cohort having more than 1 PTC. When patients were compared on the basis of their body mass index, the size of PTCs was significantly larger in the overweight/obese group. The wingless-type (WNT) signaling members catenin beta 1 (CTNBB1) and wingless-type MMTV integration site family, member 7A (WNT7A) were upregulated in 86% (n = 12/14) and 79% (n = 11/14) of the patients, respectively. WNT7A was significantly upregulated in girls with 1 cyst and low body mass index. CONCLUSION: A correlation exists between obesity, cyst size, and hyperandrogenism. Activation of the WNT/CTNBB1 pathway via WNT7A might play a role in PTC development.

Intraoperative Rupture of Ovarian Dermoid Cysts in the Pediatric and Adolescent Population: Should This Change Your Surgical Management?

STUDY OBJECTIVE: (1) To determine the incidence of chemical peritonitis after cystectomy for ovarian dermoid cysts with intraoperative cyst rupture in the pediatric and adolescent population; and (2) to examine the intraoperative and postoperative outcomes of cystectomies performed for ovarian dermoid cysts using laparoscopy and laparotomy, especially those with intraoperative cyst rupture. DESIGN: A retrospective cohort study of female patients who underwent ovarian cystectomy with proven ovarian dermoid pathology between July 2007 and July 2015. SETTING: Texas Children's Hospital, Houston, Texas. PARTICIPANTS: One hundred forty-four patients between the ages of 1 and 21 years who underwent an ovarian cystectomy with proven benign ovarian dermoid histology on the basis of pathology reports. INTERVENTIONS AND MAIN OUTCOME MEASURES: Occurrence of spill of cyst contents, chemical peritonitis, postoperative complications, and hospital readmissions. RESULTS: One hundred forty-four female patients underwent cystectomy (38 laparotomy and 106 laparoscopy) resulting in benign ovarian dermoid histology. Their mean age was 12.4 ± 4.1 years (range, 1-21), mean cyst size was 9.2 ± 6.4 cm (range, 1-30 cm), no patients had elevated tumor markers, 42 (29.1%) presented with torsion, 73 (50.7%) had cyst fluid spill, and there were no cases of chemical peritonitis. Few significant differences were found between cases performed via laparoscopy and laparotomy and those with and without intraoperative cyst rupture resulting in spill of contents. Laparotomy cases were found to have larger mean cyst size (P < .001), estimated blood loss (P = .003), and presence of bilateral cysts (P = .017) compared with laparoscopic cases. Cyst fluid spill occurred in more laparoscopic cases ([67/106] 63.2% vs [6/38] 15.8%; P < .001), and risk increased with cyst size greater than 5 cm (P < .001). In the laparoscopy group, cyst size greater than 5 cm was more likely to present with torsion (P < .001). There was no significant difference in the repeat cystectomy rate between the laparoscopy and laparotomy groups even with cyst rupture (P = .394). Only 5 cases presented to the emergency room postoperatively and 2 were admitted postoperatively for umbilical port site dehiscence and pyelonephritis. CONCLUSION: Laparoscopic cystectomy of ovarian dermoid cysts is associated with greater intraoperative cyst rupture. However, cyst rupture is rarely associated with complications, therefore minimally invasive surgical management of ovarian dermoid cysts is a reasonable surgical approach.

Evidence-Based Management of Patients with 45,X/46,XY Gonadal Dysgenesis and Male Sex Assignment: from Infancy to Adulthood.

45,X/46,XY gonadal dysgenesis is a disorder of sexual differentiation with a wide clinical presentation, ranging from Turner-like females to individuals with genital ambiguity to azoospermic but otherwise normal-appearing males. Hence, patients can be assigned female or male sex. Female patients are managed according to the Turner Syndrome Guidelines, whereas males are managed on a case-by-case basis. Male patients present with multiple medical challenges: undervirilization, hypogonadism, gonadoblastoma risk, and short stature. Many require surgeries and hormonal treatments that are time-sensitive and irreversible. Nonetheless, these therapeutic decisions are made without evidence-based guidelines. This review describes the medical concerns and possible interventions in male patients with 45,X/46,XY dysgenesis for each stage of development. Interventions should be addressed within a patient-centered framework by a multidisciplinary team and after thorough discussion with the family. We use the GRADE system to appraise the existing evidence and provide recommendations based on the available evidence.

Regression of Adenomyosis on Magnetic Resonance Imaging after a Course of Hormonal Suppression in Adolescents: A Case Series.

STUDY OBJECTIVE: To demonstrate that adenomyosis is a rare cause of dysmenorrhea or chronic pelvic pain (CPP) in the adolescent population that can be identified with magnetic resonance imaging (MRI) and to report resolution of adenomyosis by MRI after a course of hormonal suppression in 4 adolescents. DESIGN, SETTING, AND PARTICIPANTS: Retrospective case series of 4 adolescents with adenomyosis on pelvic MRI at Texas Children's Hospital. INTERVENTIONS: Continuous oral contraceptive (COC) therapy or leuprolide acetate. MAIN OUTCOME MEASURES: Lesions on pelvic MRI after treatment. METHODS: We reviewed medical records of 4 adolescents with CPP and adenomyosis on T2-weighted pelvic MRI. All patients had initial diagnostic pelvic MRI and then definitive hormonal intervention. Repeat imaging was obtained after a symptom-free interval. RESULTS: Patient ages ranged from 12 to 16 years. One patient had resolution of symptoms with COC therapy. MRI performed 3 years later showed no adenomyosis. Three patients failed COC therapy. All were symptomatically improved after therapy with a gonadotropin-releasing hormone agonist. Follow-up MRI performed at intervals between 6 months and 3 years showed resolution of adenomyosis. CONCLUSION: MRI can raise suspicion for the diagnosis of adenomyosis in adolescents with refractory CPP. Subsequent MRI can show regression of lesions after symptom resolution with hormonal therapy.

Clinical recommendation: pediatric lichen sclerosus.

Lichen sclerosus is a chronic inflammatory condition affecting the anogenital region that may present in the prepubertal or adolescent patient. Clinical presentations include significant pruritus, labial adhesions, and loss of pigmentation. Treatment includes topical anti-inflammatory agents and long-term follow-up as there is a high risk of recurrence and an increased risk of vulvar cancer in adult women with history of lichen sclerosus. These recommendations are intended for pediatricians, gynecologists, nurse practitioners and others who care for pediatric/adolescent girls in order to facilitate diagnosis and treatment.

Diagnosis and management of precocious puberty in atypical presentations of McCune-Albright syndrome: a case series review.

BACKGROUND: McCune-Albright syndrome is a rare syndrome, classically defined as the triad of precocious puberty, fibrous dysplasia of bone, and café au lait lesions. Partial or atypical presentations of McCune-Albright syndrome, with only one or two of the classic symptoms, have been described in the literature and remain particularly challenging due to lack of diagnostic phenotype. In these patients, the utility of analysis of mutations in the gene of the α subunit of the stimulatory G-protein is limited and so the diagnosis is often based on clinical judgment. Three atypical cases of suspected McCune-Albright syndrome with gonadotropin-independent precocious puberty are presented. CASES: Case #1: A 5-year-old female presented with painlesss vaginal bleeding and was found to have café au lait lesions. She was diagnosed with gonadotropin independent precocious puberty with eventual progression to gonadotropin dependent precocious puberty which was successfully treated with both letrozole and gonadotropin-releasing hormone agonist therapy. Case #2: A 3-year-old female presented with painless vaginal bleeding and was found to have café au lait lesions. She was diagnosed with gonadotropin independent precocious puberty and was successfully treated with letrozole. Case #3: A 5-year-old female presented with fibrous dysplasia and was found to have evidence of uterine and ovarian enlargement on ultrasound. She was diagnosed with gonadotropin-independent precocious puberty and was successfully treated with letrozole. SUMMARY AND CONCLUSION: Although different in presentation, all three atypical cases of suspected McCune-Albright syndrome with gonadotropin-independent precocious puberty were successfully treated with aromatase inhibitors. This small case series shows the utility and efficacy of aromatase inhibitors in the setting of atypical cases of suspected MAS with gonadotropin-independent precocious puberty.