Preferences in Clinical Care of Individuals With Differences of Sex Development.

OBJECTIVES: To identify the most important attributes related to the process of achieving, and outcomes associated with, successful care for differences of sex development (DSD). METHODS: We developed a best-worst scaling survey administered to 520 DSD stakeholders, including individuals or family members of those with DSD, health care specialists, and patient support and advocacy representatives. Fourteen process-related attributes and 16 outcome-related attributes were identified through qualitative research. We estimated relative importance scores and coefficients from regression analysis to understand the relative importance of attributes and conducted latent class analysis to explore heterogeneity in preferences. RESULTS: The 3 most important process attributes were (1) good communication between care team and patient/family, (2) care team educated patient/family about condition, and (3) care team incorporates the values of patient/family. The 3 most important outcome attributes were (1) patient satisfaction, (2) patient mental health, and (3) treatment maintains physical health. Latent class analyses showed that respondents had heterogeneous preferences. For process-related attributes, we identified 3 respondent groups: "Patient autonomy and support" (46% of respondents), "Education and care transitions" (18%), and "Shared decision-making" (36%). For outcome-related attributes, we identified 2 respondent groups: "Preserving function and appearance" (59% of respondents) and "Patient health and satisfaction" (41%). CONCLUSIONS: Outcomes such as patient satisfaction and health were the most important outcome attributes, and good communication and education from the care team were the most important process attributes. Respondents expressed heterogeneous preferences for selected DSD care attributes that providers should consider to improve satisfaction with and quality of DSD care.

Discrepancies Between Imaging Reports and Operative Findings in Patients With Cloaca: A Call for Expansion of the Mullerian Anomalies Classification.

BACKGROUND: There is wide variation in the language used to describe Mullerian structures. To standardize terminology, the American Society of Reproductive Medicine (ASRM) created the Mullerian Anomalies Classification (MAC) in 2021. The objective of this study was to evaluate the applicability of the MAC nomenclature to pediatric patients with cloaca. METHODS: A retrospective review of all patients with cloaca was performed at a single institution. Descriptions of Mullerian structures were evaluated and compared to the ASRM MAC categories. Descriptive statistics were used to report findings. RESULTS: 36 patients with cloaca were identified, 13 (36%) of whom had congenital Mullerian structures that could not be adequately described by the MAC terminology. All 13 patients had two hemiuteri that were not connected in the midline and were not accurately described as uterus didelphys. Additionally, 5 of these 13 patients had reproductive anatomy that was connected by a fistula or ectopic connection to other pelvic structures. CONCLUSION: Despite the ASRM expansion of the Mullerian anomalies nomenclature, more than a third of our patients with cloaca could not have their Mullerian structures accurately described. Describing anatomy with accurate and consistent language can improve communication between healthcare providers and may allow patients and families to better anticipate fertility options. STUDY TYPE: Retrospective. LEVEL OF EVIDENCE: IV.

Surgical Decision-Making for Individuals with Differences of Sex Development: Stakeholders' views.

Introduction: Advocacy and human rights organizations have called for a moratorium on elective surgical procedures until the patient is able to fully participate in the decision-making process. Due to the controversial nature surrounding surgery in differences of sex development (DSD) care, we aimed to assess the factors that teens and adults with DSD, parents, healthcare providers and other allied professionals consider pertinent to complex surgical decisions in DSD. Methods: Stakeholders (n=110) in DSD care participated in semi-structured interviews exploring features and potential determinants of successful healthcare outcomes. Audio-recordings were transcribed, coded, and analyzed using qualitative data software. Codes for "Process of Decision-Making" and "Successful Outcome-Surgery/Appearance/Function" were further searched using keywords "surgery," "procedure," and "timing." Results: Several themes were identified: 1) The nature or type of the decision being made; 2) Who should be involved in the decision-making process; 3) Timing of conversations about surgery; 4) Barriers to decision-making surrounding surgery; 5) The elements of surgical decision-making; and 6) The optimal approach to surgical decision-making. Many stakeholders believed children and adolescents with DSD should be involved in the process as developmentally appropriate. Conclusion: DSD include a wide range of diagnoses, some of which may require urogenital reconstruction to relieve obstruction, achieve continence, and/or address other anatomical differences whether cosmetic or functional. Adolescents and adults with DSD desired autonomy and to be part of the decision-making process. Parents were divided in their opinion of who should be involved in making elective surgical decisions: the child or parents as proxy medical decision-makers. Providers and other professionals stressed the importance of process and education around surgical decisions. Ongoing research examines how decision-makers evaluate tradeoffs associated with decision options.

Decisional Support Needed when Facing Tough Decisions: Survey of Parents with Children having Differences of Sex Development.

Introduction: Parents of infants and young children newly diagnosed with differences of sex development (DSD) commonly face medical and psychosocial management decisions at a time when they are first learning about the condition and cannot consult their child for input. The aim of this study was to identify areas of greatest need for parental decisional support. Methods: 34 parents of children receiving care for DSD at one of three US children's hospitals participated in a survey to learn what clinical and psychosocial decisions needed to be made on behalf of their child. Parents were then asked to identify and focus on a "tough" decision and respond to questions assessing factors affecting decision-making, decision-making preferences, decisional conflict, and decision regret. Descriptive analyses were conducted. Results: Decisions about surgery and aspects of sharing information about their child's condition with others were the two most frequently reported decisions overall, experienced by 97% and 88% of parents, as well as most frequently nominated as tough decisions. Many parents reported mild to moderate levels of decisional conflict (59%) and decision regret (74%). Almost all parents (94%) reported experiencing at least one factor as interfering with decision-making (e.g., "worried too much about choosing the 'wrong' option"). Parents universally reported a desire to be involved in decision-making - preferably making the final decision primarily on their own (79%), or together with their child's healthcare providers (21%). The majority of parents judged healthcare providers (82%) and patient/family organizations (58%) as trustworthy sources of information. Discussion: Parents of children with DSD encounter medical, surgical, and psychosocial management decisions. Despite difficulties including emotional distress and informational concerns (including gaps and overload), parents express strong desires to play key roles in decision-making on behalf of their children. Healthcare providers can help identify family-specific needs through observation and inquiry in the clinical context. Together with families, providers should focus on specific clinical management decisions and support parental involvement in making decisions on behalf of young children with DSD.

"It became easier once I knew": Stakeholder perspectives for educating children and teenagers about their difference of sex development.

OBJECTIVE: Secrecy about a child's difference of sex development (DSD) can lead to internalized shame and stigma. We explored how teenagers and adults with DSD, parents, healthcare providers, and allied professionals value and perceive patient education. METHODS: Stakeholders (n = 110) completed qualitative semi-structured interviews. Relevant themes for educational content were queried and organized. RESULTS: Education was consistently identified as essential to successful outcomes. There was less consistency in how to educate patients. Disagreement existed regarding who should champion the education process. Participants believed medically relevant information should be shared gradually with attention to developmental capacity. Details were lacking regarding how much or what information to share. Participants noted that vetted resources were helpful. Benefits of sharing condition-specific information with patients included supporting their psychosocial development. Barriers included parental resistance to sharing information due to shame/stigma, and cultural and/or family dynamics. CONCLUSIONS: Stakeholders' different perspectives regarding patient DSD education warrant future research to focus on the design, evaluation, and implementation of education-focused interventions. PRACTICE IMPLICATIONS: Healthcare providers are responsible for supporting the education of children and teenagers with DSD about their condition. When considering barriers, adopting a cultural or family systems framework can reduce parental resistance and promote open dialogue.

A Prospective Management Strategy for Heterotaxy Syndrome with Intestinal Rotation Abnormalities: Imaging Does Not Predict Need for Surgery.

PURPOSE: Heterotaxy syndrome (HS) affects right-left anatomical development in 3% of children with congenital heart disease. Commonly, these patients have intestinal rotation abnormalities (IRA) that differ from typical malrotation. In this prospective study, we examine the development of a management pathway, imaging findings, and clinical course of patients with HS and IRA. METHODS: After literature review, a multispecialty focus group designed a pathway for HS. Participants from a single institution were prospectively enrolled from 2016 to 2019. They underwent an abdominal ultrasound and UGI, however timing was variable based on symptoms. Symptomatic IRA was defined as feeding intolerance, bilious or non-bilious vomiting, bloating, severe reflux, and intermittent pain or abdominal distention. Screening for symptoms occurred every three months for the first two years and then annually. RESULTS: 18 participants were followed for a median of 5.0 years. Three clinical categories were identified: 1) asymptomatic, not requiring intestinal surgery, 72.2%, 2) symptomatic with feeding intolerance or failure to thrive requiring gastrostomy placement and diagnostic laparoscopy with Ladd procedure, 16.7%, and 3) symptomatic requiring urgent exploration for suspicion of volvulus, 11.1%. Need for surgery did not necessarily correlate with US and/or UGI findings. CONCLUSIONS: In children with HS and IRA, postnatal imaging did not inform the need for intestinal surgery. Although rare, volvulus or other forms of proximal obstruction can be anticipated, and symptomatic patients should be offered surgical intervention. A multidisciplinary care pathway for patients with HS and IRA can coordinate care and help counsel families on the likelihood of needing intestinal surgery for IRA. LEVEL OF EVIDENCE: Level II. TYPE OF STUDY: Prospective Cohort Study.

Paediatric surgeons' current knowledge and practices of obtaining assent from adolescents for elective reconstructive procedures.

PURPOSE: Adolescents develop their decision-making ability as they transition from childhood to adulthood. Participation in their medical care should be encouraged through obtaining assent, as recommended by the American Academy of Pediatrics (AAP). In this research, we aim to define the current knowledge of AAP recommendations and surgeon practices regarding assent for elective reconstructive procedures. METHODS: An anonymous electronic survey was distributed to North American paediatric surgeons and fellows through the American Pediatric Surgical Association (n=1353). RESULTS: In total, 220 surgeons and trainees responded (16.3%). Fifty per cent of the surgeons who are familiar with the concept of assent had received formal training; 12% of the respondents had not heard of assent before the survey. Forty-seven per cent were aware of the 2016 AAP policy statement regarding assent in paediatric patients. Eighty-nine per cent always include adolescents as part of the consent discussion. Seventy-seven per cent solicit an expression of willingness to accept the proposed care from the patient. The majority (74%) of the surgeons perceived patient cooperation/understanding as the biggest barrier to obtaining assent. Over half of the respondents would consider proceeding with elective surgery despite the adolescent patient's refusal. Reasons cited for proceeding with elective surgery include surgeons' perception of medical necessity, perceptions of disease urgency, and lack of patient maturity. CONCLUSION: Paediatric surgeons largely acknowledge the importance of assent, but variably practice the principles of obtaining assent from adolescent patients undergoing elective reconstructive procedures. Fewer surgeons are explicitly aware of formal policy statements or received formal training. Additional surgeon education and institutional policies are warranted to maximise inclusion of adolescents in their medical care.

High Infertility Rates and Pregnancy Complications in Female Physicians Indicate a Need for Culture Change.

OBJECTIVE: This survey study aims to determine the prevalence of pregnancy complications and infertility in female physicians in comparison to the general population. Risk factors, workplace environment, and education are also examined. BACKGROUND: Physicians undertake long training and have stressful work environments during optimal childbearing years. While growing literature indicates increased rates of pregnancy complications and infertility in female surgeons, the prevalence in female physicians of all specialties is unknown. METHODS: An anonymous, voluntary survey was distributed to female physicians via private physician social media groups. It queried pregnancy demographics and complications, infertility diagnosis and treatment, workplace environment, and prior education on these topics. Results were compared with general population data, between medical and surgical subspecialties, and between physicians who were and were not educated on the risks of delaying pregnancy. RESULTS: A total of 4533 female physicians completed the survey. Compared with the general population, female physicians were older at first pregnancy, more often underwent infertility evaluation and treatment, and had higher rates of miscarriage and preterm birth. During training, only 8% of those surveyed received education on the risks of delaying pregnancy. Those who were educated were significantly less likely to experience miscarriage or seek infertility evaluation or treatment. Compared with physicians in nonsurgical specialties, surgeons had fewer children, were older at first pregnancy, had more preterm births and fetal growth problems, and were more likely to be discouraged from starting a family during training and practice. CONCLUSIONS: Female physicians, particularly surgeons, have a significantly greater incidence of miscarriage, infertility, and pregnancy complications compared with the general population. The culture of medicine and surgery must continue to evolve to better support women with family planning during their training and careers.

Early postoperative feeding in single-stage repair of anorectal malformation with vestibular or perineal fistula is not associated with increased wound complications.

INTRODUCTION: The objective of this study is to assess the postoperative outcomes of single-stage repair of anorectal malformations with vestibular (VF) or perineal fistula (PF) and early initiation of postoperative feeding. METHODS: A retrospective review of patients undergoing single-stage repair of isolated low anorectal malformations (VF and PF) from 2017 to 2020 was conducted. All patients underwent an anterior anoplasty with complete mobilization of the rectal fistula, or posterior sagittal anorectoplasty (PSARP), without protective colostomy. The variables examined include age, timing of postoperative feeding initiation, length of stay (LOS), and complications. RESULTS: Nineteen patients with VF or PF underwent a single-stage repair. 12/19 (63%) patients were female. All 7 males and 9/12 females had a PF. The range of age at surgery was 2 days to 3 years with median age of 92 days [IQR 1,3: 9,193]. The median postoperative day for initiation of feeds was day 0 [IQR 1,3: 0,1] and median LOS was 1 day [IQR 1,3: 1,4.5]. 18/19 (95%) patients were evaluated in follow-up and there were no wound infections, wound dehiscences, or recurrent fistulas. Within 90 days postoperatively, no patients were seen in the emergency department for postoperative issues. Within 6 months, 2/19 (11%) patients required an unplanned return to the operating room for anal dilation. CONCLUSION: In single-stage repair of isolated low anorectal malformations, VF and PF, early initiation of postoperative feeding is safe, results in a short length of stay, and does not lead to increased wound complications. Early enteral feeding eliminates the need for parenteral nutrition and central venous access, and their associated complications. LEVEL OF EVIDENCE: Level IV.

Information sharing in differences of sex development: The creation of a caregiver-support tool.

INTRODUCTION: Social support can be a protective factor against the negative mental health outcomes experienced by some parents and caregivers of children with differences of sex development (DSD). However, established social support networks can be difficult to access due to caregiver hesitancy to share information with others about their child's diagnosis. Health care providers in the field of DSD, and particularly behavioral health providers, are well positioned to help caregivers share information with the important people in their lives in order to access needed social support. This article summarizes the development of a clinical tool to help clinicians facilitate discussions regarding information sharing with caregivers of children with DSD. METHOD: Members of the psychosocial workgroup for the DSD -Translational Research Network completed a survey about their experiences facilitating information sharing discussions with caregivers of children with DSD and other health populations. The results of this survey were used to develop a clinical tool using ongoing iterative feedback from workgroup members, based on principles of user-centered design and quality improvement. RESULTS: Workgroup members consider information sharing an important aspect of working with caregivers of children with DSD. Additional resources and tools were identified as potentially helpful to these discussions. DISCUSSION: The DSD Sharing Health Information Powerfully-Team Version (SHIP-T) is a resource tool for DSD health care team members to utilize in hospital and ambulatory settings to help caregivers of children with DSD share information with their social support networks. The final SHIP-T is included in this article. (PsycInfo Database Record (c) 2023 APA, all rights reserved).

Protocol for a Prospective Observational Study of Revision Palatoplasty Versus Pharyngoplasty for Treatment of Velopharyngeal Insufficiency Following Cleft Palate Repair.

OBJECTIVE: To present the design and methodology for an actively enrolling comparative effectiveness study of revision palatoplasty versus pharyngoplasty for the treatment of velopharyngeal insufficiency (VPI). DESIGN: Prospective observational multicenter study. SETTING: Twelve hospitals across the United States and Canada. PARTICIPANTS: Individuals who are 3-23 years of age with a history of repaired cleft palate and a diagnosis of VPI, with a total enrollment target of 528 participants. INTERVENTIONS: Revision palatoplasty and pharyngoplasty (either pharyngeal flap or sphincter pharyngoplasty), as selected for each participant by their treatment team. MAIN OUTCOME MEASURE(S): The primary outcome is resolution of hypernasality, defined as the absence of consistent hypernasality as determined by blinded perceptual assessment of a standard speech sample recorded twelve months after surgery. The secondary outcome is incidence of new onset obstructive sleep apnea. Statistical analyses will use propensity score matching to control for demographics, medical history, preoperative severity of hypernasality, and preoperative imaging findings. RESULTS: Study recruitment began February 2021. As of September 2022, 148 participants are enrolled, and 78 have undergone VPI surgery. Enrollment is projected to continue into 2025. Collection of postoperative evaluations should be completed by the end of 2026, with dissemination of results soon thereafter. CONCLUSIONS: Patients with VPI following cleft palate repair are being actively enrolled at sites across the US and Canada into a prospective observational study evaluating surgical outcomes. This study will be the largest and most comprehensive study of VPI surgery outcomes to date.

Establishing a Clinical Protocol for Velopharyngeal MRI and Interpreting Imaging Findings.

Traditional imaging modalities used to assess velopharyngeal insufficiency (VPI) do not allow for direct visualization of underlying velopharyngeal (VP) structures and musculature which could impact surgical planning. This limitation can be overcome via structural magnetic resonance imaging (MRI), the only current imaging tool that provides direct visualization of salient VP structures. MRI has been used extensively in research; however, it has had limited clinical use. Factors that restrict clinical use of VP MRI include limited access to optimized VP MRI protocols and uncertainty regarding how to interpret VP MRI findings. The purpose of this paper is to outline a framework for establishing a novel VP MRI scan protocol and to detail the process of interpreting scans of the velopharynx at rest and during speech tasks. Additionally, this paper includes common scan parameters needed to allow for visualization of velopharynx and techniques for the elicitation of speech during scans.

Creation of an Electronic Resource Repository for Differences of Sex Development (DSD): Collaboration Between Advocates and Clinicians in the DSD-Translational Research Network.

INTRODUCTION: People with differences of sex development (DSD) and their families need education about these conditions while receiving emotional and peer support to participate in shared decision-making, reduce social isolation, and optimize physical and psychosocial outcomes. Barriers to education and support include limited knowledge and awareness by healthcare providers, tension among patient and medical communities, varied quality of educational resources, and the sensitive nature of DSD. We aimed to create an electronic repository of vetted quality online resources about DSD. METHODS: The electronic resource repository (e-RR) was a collaboration between affected individuals and advocates and healthcare providers in the DSD-Translational Research Network (DSD-TRN), an NIH-supported consortium of US teams committed to standardizing and optimizing care in DSD. The e-RR development and ongoing growth involved: (1) identification of resources by the project team (3 advocates and 1 physician), (2) evaluation and feedback by DSD-TRN clinical teams, (3) creation of the e-RR, and (4) review and revision. Twitter-like descriptions accompanied each entry; resources were categorized by target age, audience, and condition. RESULTS: Thirty-seven web-based educational, peer and advocacy support, and clinician-oriented resources were reviewed. Eight of 10 DSD-TRN teams responded to a survey regarding resource inclusion. Awareness of individual resources varied widely. Consensus was achieved when opinions differed; 30 resources were included. The e-RR is available online and as a downloadable booklet at http://www.accordalliance.org/resource-guide/. CONCLUSION: The e-RR increases awareness of and access to vetted educational and support resources for those with DSD and healthcare providers. It represents important collaboration between advocates and providers.

Telehealth multidisciplinary prenatal consultation during the COVID-19 pandemic: enhancing patient care coordination while maintaining high provider satisfaction.

OBJECTIVE: Comprehensive fetal care centers address congenital anomalies by developing pre- and post-natal care plans in a multidisciplinary format. To reduce exposure during the Coronavirus Infectious Disease-2019 (COVID-19) pandemic, the Centers for Medicare & Medicaid Services (CMS) broadened access to telehealth services. We assessed provider satisfaction with the rapid transition from in-person prenatal visits to multidisciplinary consultations via telehealth as an adaptive response to the pandemic. METHODS: Patients referred to an urban academic fetal care center during the first 6 weeks of the COVID-19 pandemic underwent advanced imaging including fetal MRI, focused ultrasound, and fetal echocardiography. Subsequently, multidisciplinary telehealth consultations occurred with all providers attending virtually. Patients were given the option of attending the multidisciplinary telehealth consultation in a conference room in the hospital or from home. During these meetings, relevant images were reviewed with all participants via screen sharing through a secure video platform. Provider satisfaction with the telehealth paradigm was assessed using an electronic survey. RESULTS: Twenty-two surveys were administered with a response rate of 82%. 89% of providers were highly satisfied with the telehealth format. 72% of providers would prefer the multidisciplinary telehealth format to an in-person visit for future visits after COVID-19 restrictions are lifted. 22% of providers would leave the choice to the patient's family. One provider preferred in-person visits. Some providers noted that virtual conferences limited the ability to draw pictures, show educational materials, and provide emotional support. CONCLUSION: Providers were overwhelmingly supportive of continuing multidisciplinary telehealth conferences for complex prenatal consultations, even after restrictions are lifted, which has led to the continuation of this model for the duration of the pandemic. Providers highlighted the convenience and improved care coordination across specialties. Further studies to examine the patient experience with virtual consultations are warranted.

Defining successful outcomes and preferences for clinical management in differences/disorders of sex development: Protocol overview and a qualitative phenomenological study of stakeholders' perspectives.

INTRODUCTION: Utilizing a qualitative phenomenological design, the Defining Successful Outcomes and Trade-offs study examined stakeholder perspectives regarding optimal healthcare delivery and outcomes for individuals with a difference/disorder of sex development (DSD). OBJECTIVE: We describe study methods and provide an overview of themes and subthemes. STUDY DESIGN: Interviews were conducted with individuals with a DSD (n = 24), parents of those with a DSD (n = 19), healthcare providers (n = 37), and others (n = 30). Primary questions regarding clinical management of patients with DSD were: "What is a successful outcome?" and "How do you achieve it?" RESULTS: Themes included: understanding of DSD diagnosis and self-efficacy in management is necessary but complex; patient and family psychological well-being; support from others versus being stigmatized; affected person experiences physical health and accepts the implications of their condition; complexities in DSD decision making, roles and expectations; and knowledgeable providers and multidisciplinary teams are essential, notwithstanding persisting barriers. Participants recognized competing values potentially forcing trade-offs in decision making. DISCUSSION: Recognition of diverse and sometimes conflicting perspectives regarding optimal pathways of care and outcomes - both within and among those with DSD and their providers -promises to enhance shared decision making. CONCLUSION: Diverse perspectives and perceptions of trade-offs associated with DSD healthcare emphasize the need to tailor care for patients and families.

The ethics of offering fertility preservation to pediatric patients: A case-based discussion of barriers for clinicians to consider.

Decisions about fertility preservation can be difficult in general but the recent application of preservation techniques to pediatric patients has ushered in a host of new ethical considerations. Fertility preservation (FP) may be considered for all patients who are at risk for infertility due to their medical diagnosis or treatment, including those undergoing gonadotoxic chemotherapy, those with differences of sex development (DSD) undergoing gonadectomy,1-3 and transgender patients undergoing gender affirming surgery. The focus of this paper is to review the ethical issues involved in offering FP to pediatric oncology patients and, to a lesser extent, the new ethical issues that apply to patients with DSD. Some of the techniques and approach to counseling will also apply to transgender individuals, although that is beyond the scope of this work. We aim to discuss several barriers to offering FP and to advise how to counsel families in the setting of rapid changes in this field. Families should be educated about:Specific guidance for clinicians regarding some of these points was recently published in an American Academy of Pediatrics Clinical Report,1 and we will illustrate the use of these guidelines in four case presentations.

Multisite Pain Is Highly Prevalent in Children with Functional Abdominal Pain Disorders and Is Associated with Increased Morbidity.

OBJECTIVES: To characterize the types of multisite pain experienced by children with functional abdominal pain disorders (FAPDs) and to examine differences in psychosocial distress, functional disability, and health-related quality of life in children with multisite pain vs abdominal pain alone. STUDY DESIGN: Cross-sectional study of children ages 7-17 years (n = 406) with pediatric Rome III FAPDs recruited from both primary and tertiary care between January 2009 and June 2018. Subjects completed 14-day pain and stool diaries, as well as validated questionnaires assessing abdominal and nonabdominal pain symptoms, anxiety, depression, functional disability, and health-related quality of life. RESULTS: In total, 295 (73%) children endorsed at least 1 co-occurring nonabdominal pain, thus, were categorized as having multisite pain with the following symptoms: 172 (42%) headaches, 143 (35%) chest pain, 134 (33%) muscle soreness, 110 (27%) back pain, 94 (23%) joint pain, and 87 (21%) extremity (arms and legs) pain. In addition, 200 children (49%) endorsed 2 or more nonabdominal pain symptoms. Participants with (vs without) multisite pain had significantly higher abdominal pain frequency (P < .001) and severity (P = .03), anxiety (P < .001), and depression (P < .001). Similarly, children with multisite pain (vs without) had significantly worse functional disability (P < .001) and health-related quality of life scores (P < .001). Increasing number of multisite pain sites (P < .001) was associated with increased functional disability when controlling for demographic and other clinical factors. CONCLUSIONS: In children with FAPDs, nonabdominal multisite pain is highly prevalent and is associated with increased psychosocial distress, abdominal pain frequency and severity, functional disability, and lower health-related quality of life.

Probiotic VSL#3 Treatment Reduces Colonic Permeability and Abdominal Pain Symptoms in Patients With Irritable Bowel Syndrome.

Background: Little is known regarding the clinical impact of treatment and treatment duration of probiotic VSL#3 on gut and microbiome function in irritable bowel syndrome (IBS). As part of a safety trial, we assessed the effect of VSL#3 treatment duration on abdominal pain, stooling, gut permeability, microbiome composition and function. Methods: Adults with IBS were randomized into an open label trial to receive the probiotic VSL#3 for 4 or 8 weeks. Adverse events, abdominal pain, and stooling patterns were recorded daily. Gut permeability, fecal bile acid levels, and microbiome composition were profiled at baseline and after treatment. Results: Fifteen subjects completed the trial (4-week: n = 8; 8-week: n = 7). Number of pain episodes decreased in both groups (P = 0.049 and P = 0.034; 4- vs. 8-week, respectively). Probiotic organisms contained in VSL#3 were detected in feces by whole shotgun metagenomic sequencing analysis and relative abundances of Streptococcus thermophilus, Bifidobacterium animalis, Lactobacillus plantarum, and Lactobacillus casei subsp. paraccasei correlated significantly with improved abdominal pain symptoms and colonic permeability at study completion. Although abdominal pain correlated significantly with the detection of probiotic species at study completion, a composite view of gut microbiome structure showed no changes in community diversity or composition after VSL#3 treatment. Conclusions: Probiotic organisms identified in stool correlated significantly with improvement in colonic permeability and clinical symptoms, prompting future studies to investigate the mechanistic role of VSL#3 and colonic permeability in IBS pathophysiology in a larger randomized controlled trial. Clinical Trial Registration: www.clinicaltrials.gov, Identifier: NCT00971711.

Facilitators and Barriers to Implementing Standardized Outcome Measurement for Children With Cleft Lip and Palate.

OBJECTIVE: Identify facilitators and barriers to implementing standardized outcome measurement in cleft care. DESIGN: Cross-sectional, qualitative study. SETTING/PARTICIPANTS: Participants included 24 providers and staff from a large, multidisciplinary cleft team in the southwest United States, 5 caregivers of children with cleft palate (with or without cleft lip) treated by this team, and 3 experts involved in implementing a cleft-specific standardized outcome measurement in the United Kingdom. INTERVENTIONS: Semistructured, qualitative interviews were conducted exploring perceived facilitators and barriers to implementing standardized outcome measurement in cleft care. Interviews were audio-recorded, transcribed, and analyzed for content. The Consolidated Framework for Implementation Research was used to guide the interviews and analysis. The analysis focused on the characteristics of standardized outcome measurement that directly influence its adoption. RESULTS: Participants identified both facilitators and barriers to implementing standardized outcome measurement. Facilitators included the strength and quality of evidence supporting improvements in cleft care delivery following implementation of standardized outcome measurement and the relative advantage of standardized outcome measurement over continuing the status quo. Barriers included the difficulty adapting standardized outcome measurement to meet local context and patient-specific needs and the complexity of implementing standardized outcome measurement. CONCLUSIONS: Providers, staff, and caregivers involved in cleft care perceive multiple benefits from standardized outcome measurement, while also recognizing substantial barriers to its implementation. Results from this study can be used to guide development of an implementation strategy for standardized outcome measurement that builds upon perceived strengths of the intervention and reduces perceived barriers.

Associations of Abdominal Pain and Psychosocial Distress Measures With Health-Related Quality-of-Life in Pediatric Healthy Controls and Irritable Bowel Syndrome.

BACKGROUND: Children with irritable bowel syndrome (IBS) have lower health-related quality-of-life (HRQOL) than healthy controls (HC). Abdominal pain and psychosocial distress are negatively associated with HRQOL, although their relative effect is unclear. AIM: The aim of this study was to compare the relative associations of abdominal pain and psychosocial distress with HRQOL in HC and IBS. STUDY: Baseline abdominal pain, psychosocial distress, and HRQOL measures were obtained from HC and IBS pediatric clinical trial participants. Regression assessed which measures were most strongly associated with Physical and Psychosocial HRQOL separately by group. Interaction analyses examined group differences in the associations of abdominal pain and psychosocial distress with HRQOL. RESULTS: Eight-five HC and 213 children with IBS participated. Somatization was most strongly associated with Physical HRQOL in HC, and functional disability was most strongly related in IBS. With respect to Psychosocial HRQOL, somatization was most strongly associated for both HC and IBS; depression was also significantly associated in HC. The strength of association between somatization and Physical HRQOL differed between groups; the negative association was less pronounced for IBS than HC. The association between functional disability and both Physical and Psychosocial HRQOL differed significantly between groups; the negative associations were more pronounced for IBS than HC. CONCLUSIONS: Multiple psychosocial distress measures, including somatization, were associated with HRQOL in children with IBS; HRQOL in HC was driven consistently by somatization, to the exclusion of other psychosocial concerns. The associations of somatization and functional disability with HRQOL are distinctly different between HC and IBS. This knowledge supports utilization of psychosocial interventions to improve overall well-being for children with IBS.