Exploring Factors Associated with Decisions about Feminizing Genitoplasty in Differences of Sex Development.

STUDY OBJECTIVE: Infants with genital development considered atypical for assigned female sex may undergo feminizing genitoplasty (clitoroplasty and/or vaginoplasty) in early life. We sought to identify factors associated with parent/caregiver decisions regarding genitoplasty for their children with genital virilization. DESIGN: Longitudinal, observational study SETTING: Twelve pediatric centers in the United States with multidisciplinary differences/disorders of sex development clinics, 2015-2020 PARTICIPANTS: Children under 2 years old with genital appearance atypical for female sex of rearing and their parents/caregivers INTERVENTIONS/OUTCOME MEASURES: Data on the child's diagnosis and anatomic characteristics before surgery were extracted from the medical record. Parents/caregivers completed questionnaires on psychosocial distress, experience of uncertainty, cosmetic appearance of their child's genitalia, and demographic characteristics. Urologists rated cosmetic appearance. For 58 patients from the study cohort with genital virilization being raised as girls or gender-neutral, we compared these data across 3 groups based on the child's subsequent surgical intervention: (i) no surgery (n = 5), (ii) vaginoplasty without clitoroplasty (V-only) (n = 15), and (iii) vaginoplasty and clitoroplasty (V+C) (n = 38). RESULTS: Fathers' and urologists' ratings of genital appearance were more favorable in the no-surgery group than in the V-only and V+C groups. Clitorophallic length was greater in the V+C group compared with the V-only group, with substantial overlap between groups. Mothers' depressive and anxious symptoms were lower in the no-surgery group compared with the V-only and V+C groups. CONCLUSIONS: Surgical decisions were associated with fathers' and urologists' ratings of genital appearance, the child's anatomic characteristics, and mothers' depressive and anxious symptoms. Further research on surgical decision-making is needed to inform counseling practices.

Stigma, Intrusiveness, and Distress in Parents of Children with a Disorder/Difference of Sex Development.

OBJECTIVE: The impact of parent-reported stigma due to their child's disorder/difference of sex development (DSD) on parent psychosocial adjustment is poorly understood. In other pediatric populations, perceived interference of medical conditions into daily activities (i.e., illness intrusiveness ) mediates the relationship of stigma to adjustment. This study assessed relationships between parent-focused and child-focused stigma → illness intrusiveness → depressive and anxious symptoms . Exploratory analyses sought to identify patient characteristics associated with stigma. METHOD: Caregivers (59 women and 43 men) of 63 children diagnosed with a DSD up to age 4 years completed measures of demographics, parent-focused and child-focused stigma, illness intrusiveness, and depressive and anxious symptoms. RESULTS: Increased parent-focused and child-focused stigma were associated with increased illness intrusiveness, which, in turn, was associated with increased depressive and anxious symptoms for parents nested within dyads. Among children with DSD family histories, parents reported greater child-focused stigma. CONCLUSION: Parents who experience DSD-related stigma report greater interference of their child's DSD into their daily activities, which is associated with poorer psychosocial adjustment. Findings support developing clinical interventions related to parents' perceptions of stigma and illness intrusiveness to improve parent adjustment.

Decisional regret about surgical and non-surgical issues after genitoplasty among caregivers of female infants with CAH.

INTRODUCTION: Caregivers of female infants with congenital adrenal hyperplasia (CAH) often confront complex medical decision-making (e.g., early feminizing genitoplasty). OBJECTIVE: This study aimed to evaluate the relevant medical decisions and subsequent decisional regret of caregivers following their child's genitoplasty. STUDY DESIGN: Caregivers (N = 55) were recruited from multidisciplinary treatment programs for participation in a longitudinal study. Qualitative data was collected at 6-12 months following feminizing genitoplasty to evaluate caregiver-reported decision points across their child's treatment. Quantitative exploratory analysis evaluated pre-operative predictors of subsequent decisional regret. DISCUSSION: When prompted about their decision-making and potential regret, most caregivers (n = 32, 80%) reported that their daughter's genital surgery was their primary medical decision. Specific themes regarding genital surgery included the timing and type of surgery. Most caregivers reported no decisional regret (62%), with 38% reporting some level of regret. Greater pre-operative illness uncertainty predicted heightened decisional regret at follow-up, p = .001. CONCLUSION: Two-thirds of caregivers of female infants with CAH reported not regretting their decision-making. Nevertheless, over one-third of caregivers reported some level of regret, suggesting the need for improvements in shared decision-making processes. Many, but not all, families reported that this regret was related to surgical decision-making. Reducing caregiver illness uncertainty (e.g., providing clear information to families) may increase their satisfaction with decision-making. Further research is needed to determine how the evolving care practices surrounding early genitoplasty will impact families.

Distress Trajectories for Parents of Children With DSD: A Growth Mixture Model.

OBJECTIVE: This study identifies trajectories of parent depressive symptoms after having a child born with genital atypia due to a disorder/difference of sex development (DSD) or congenital adrenal hyperplasia (CAH) and across the first year postgenitoplasty (for parents who opted for surgery) or postbaseline (for parents who elected against surgery for their child). Hypotheses for four trajectory classes were guided by parent distress patterns previously identified among other medical conditions. METHODS: Participants included 70 mothers and 50 fathers of 71 children diagnosed with a DSD or CAH with reported moderate to high genital atypia. Parents were recruited from 11 US DSD specialty clinics within 2 years of the child's birth and prior to genitoplasty. A growth mixture model (GMM) was conducted to identify classes of parent depressive symptoms over time. RESULTS: The best fitting model was a five-class linear GMM with freely estimated intercept variance. The classes identified were termed "Resilient," "Recovery," "Chronic," "Escalating," and "Elevated Partial Recovery." Four classes have previously been identified for other pediatric illnesses; however, a fifth class was also identified. The majority of parents were classified in the "Resilient" class (67.6%). CONCLUSIONS: This study provides new knowledge about the trajectories of depressive symptoms for parents of children with DSD. Future studies are needed to identify developmental, medical, or familial predictors of these trajectories.

Illness Uncertainty Longitudinally Predicts Distress Among Caregivers of Children Born With DSD.

OBJECTIVE: A subset of parents of children with disorders/differences of sex development (DSD) including ambiguous genitalia experience clinically elevated levels of anxious and depressive symptoms. Research indicates that uncertainty about their child's DSD is associated with parent psychosocial distress; however, previous studies have been cross-sectional or correlational in nature. The current study is the first to examine the longitudinal trajectory of the relationship between caregiver-perceived uncertainty about their child's DSD and caregiver anxious and depressive symptoms across the first 12 months following genital surgery in young children, or if surgery was not performed, the first 12 months following study entry. METHODS: One hundred and thirteen caregivers (Mage = 32.12; 57.5% mothers; 72.6% Caucasian) of children (N = 70; Mage = 9.81 months; 65.7% female) with DSD were recruited from 12 DSD specialty clinics in the United States. Caregivers completed psychosocial measures at baseline, 6 and 12 months following genitoplasty, or study entry if parents elected not to have surgery for their child. RESULTS: Caregiver illness uncertainty and both anxious and depressive symptoms were highest at baseline and decreased over time (ps < .05). Caregiver illness uncertainty predicted symptoms of anxious and depressive symptoms across all time points (ps < .05). CONCLUSIONS: Caregivers' perceptions of uncertainty about their child's DSD are highest soon after diagnosis, and uncertainty continues to predict both anxious and depressive symptoms across time. Thus, the initial diagnostic period is a critical time for psychological assessment and intervention, with parent illness uncertainty being an important clinical target.

Post-operative complications following feminizing genitoplasty in moderate to severe genital atypia: Results from a multicenter, observational prospective cohort study.

Disorders/differences of sex development (DSD) are congenital conditions in which there is atypical chromosomal, gonadal and/or phenotypic sex. While there remains controversy around the traditionally binary concept of sex, most patients with DSD are reared either male or female depending on their genetic sex, gonadal sex, genital phenotype and status of their internal genital tract. This study uses prospective data from 12 institutions across the United States that specialize in DSD care. We focused on patients raised female. Eligible patients had moderate to severe genital atypia (defined as Prader score >2), were ≤2 years of age at entry, and had no prior genitoplasty. The aim of this study is to describe early post operative complications for young patients undergoing modern approaches to feminizing genitoplasty. Of the 91 participants in the cohort, 57 (62%) were reared female. The majority had congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (n = 52), 1 had ovo-testicular syndrome, 2 had mixed gonadal dysgenesis and 2 had partial androgen insensitivity syndrome (PAIS). Of the 50 participants who received early genitoplasty, 43 (86%) had follow-up at 6-12 months post-surgery. Thirty-two participants (64%) received a clitoroplasty, 31 (62%) partial urogenital mobilization and 4 (8%) total urogenital sinus mobilization. Eighteen percent (9/50) experienced post-surgical complications with 7 (14%) being rated as Clavien-Dindo grade III. Both parents and surgeons reported improved satisfaction with genital appearance of participants following surgery compared to baseline. This information on post-operative complications associated with contemporary approaches to feminizing genitoplasty performed in young children will help guide families when making decisions about whether or not to proceed with surgery for female patients with moderate to severe genital atypia.

Comorbid monosymptomatic nocturnal enuresis and snoring exhibit an additive effect on impairments in health-related quality of life.

INTRODUCTION/BACKGROUND: Health-related quality of life (HRQOL) is a relatively new concept in pediatrics, but it has shown to be extremely important in terms of clinical care, treatment planning, and compliance with medical and behavioral recommendations. Two groups of children who are at risk for impairments in HRQOL are those who have bed-wetting and those who snore or have sleep apnea. OBJECTIVE: The present study sought to determine whether the combination of monosymptomatic nocturnal enuresis (MNE) and sleep-disordered breathing (SDB) results in diminished HRQOL in a pediatric sample. STUDY DESIGN: A retrospective chart review was conducted in an outpatient pediatric urology clinic and in an outpatient pulmonology clinic. Parents of the patients completed the Pediatric Sleep Questionnaire (PSQ) to assess the presence of SDB and snoring, and HRQOL was assessed using the Obstructive Sleep Apnea Syndrome-18-item (OSAS-18) scale, both validated measures, as part of the child's clinical workup. RESULTS: One hundred fifty-two (85 males and 67 females ) patients were included and were categorized into any of the following four groups: (1) MNE only, (2) SDB only, (3) MNE + SDB, or (4) healthy control. Patients in the SDB-only group had significantly more severe SDB than those in the other groups. As such, severity of SDB was controlled for in analyses. Results revealed that the four groups significantly differed on all subscales of the OSAS-18, which are Sleep Disturbance, Physical Symptoms, Emotional Symptoms, Daytime Functioning, and Caregiver Concerns. Post hoc analyses revealed the MNE + SDB group had more impairments on each subscale. Analysis of the total impairments revealed that children with only one condition (MNE or SDB) reported similar levels of impairments in HRQOL and patients with MNE + SBD reported significantly higher levels of impairments, as it appears that these comorbid conditions have an additive effect on HRQOL. DISCUSSION: Children with comorbid MNE and SDB are at significantly higher risk for reporting impairments in their HRQOL than children who have only one of these conditions. While it is already known that children with MNE are at risk for emotional, behavioral, and social difficulties and children with SDB are at risk for neurobehavioral difficulties, it is possible that children with both conditions are at risk for a multitude of negative consequences. CONCLUSION: These data emphasize the importance of urologist screening for SDB and pulmonologist screening for MNE as part of their routine clinical practice.

Predictors of Psychosocial Distress in Parents of Young Children with Disorders of Sex Development.

PURPOSE: We evaluated demographic, financial and support predictors of distress for parents of young children with disorders of sex development including atypical genital development, and characterized early parental experiences. This work extends our previous findings to identify those parents at risk for distress. MATERIALS AND METHODS: Participants included mothers (76) and fathers (63) of a child (78) diagnosed with disorders of sex development characterized by moderate to severe genital atypia. Parents completed a demographic questionnaire, measures of anxious and depressive symptoms, quality of life, illness uncertainty and posttraumatic stress symptoms, and rated their satisfaction with the appearance of their child's genitalia. RESULTS: Depressive and posttraumatic stress symptoms of caregivers were comparable to standardized norms while levels of anxious symptoms were below norms. A subset of parents reported clinically elevated symptoms. Overall 26% of parents reported anxious symptoms, 24% reported depressive symptoms and 17% reported posttraumatic stress symptoms. Levels of illness uncertainty were lower than those of parents of children with other chronic illnesses. Differences by parent sex emerged, with mothers reporting greater distress. Lower income, increased medical care and travel expenses, and having no other children were related to increased psychosocial distress. CONCLUSIONS: Early psychosocial screening is recommended for parents of children with disorders of sex development. Clinicians should be aware that financial burden and lack of previous parenting experience are risk factors for distress.

Uncertainty and Posttraumatic Stress: Differences Between Mothers and Fathers of Infants with Disorders of Sex Development.

Parents of children with disorders of sex development (DSD) report significant psychological distress, including posttraumatic stress symptoms (PTSS), with mothers consistently reporting higher rates of psychological distress than fathers. However, psychological factors contributing to PTSS in both parents are not well understood. The present study sought to fill this gap in knowledge by examining PTSS and illness uncertainty, a known predictor of psychological distress, in parents of children recently diagnosed with DSD. Participants were 52 mothers (Mage = 32.55 years, SD = 5.08) and 41 fathers (Mage = 35.53 years, SD = 6.78) of 53 infants (Mage = 9.09 months, SD = 6.19) with DSD and associated atypical genital development. Participants were recruited as part of a larger, multisite study assessing parents' psychosocial response to their child's diagnosis of DSD. Parents completed measures of illness uncertainty and PTSS. Mothers reported significantly greater levels of PTSS, but not illness uncertainty, than fathers, and were more likely than fathers to report clinical levels of PTSS (21.2% compared to 7.3%). Hierarchical regression revealed that parent sex, undiagnosed or unclassified DSD status, and illness uncertainty were each associated with PTSS. The overall model accounted for 23.5% of the variance associated with PTSS. Interventions targeting illness uncertainty may be beneficial for parents of children with newly diagnosed DSD.

Baseline Characteristics of Infants With Atypical Genital Development: Phenotypes, Diagnoses, and Sex of Rearing.

PURPOSE: Little is known about the phenotypes, diagnoses, and sex of rearing of infants with atypical genital development in the United States. As part of a multicenter study of these infants, we have provided a baseline report from US difference/disorder of sex development clinics describing the diagnoses, anatomic features, and sex of rearing. We also determined whether consensus guidelines are followed for sex designation in the United States. METHODS: Eligible participants had moderate-to-severe genital atypia, were aged <3 years, and had not undergone previous genitoplasty. Karyotype, genetic diagnosis, difference/disorder of sex development etiology, family history, and sex of rearing were collected. Standardized examinations were performed. RESULTS: Of 92 subjects, the karyotypes were 46,XX for 57%, 46,XY for 34%, and sex chromosome abnormality for 9%. The median age at the baseline evaluation was 8.8 months. Most 46,XX subjects (91%) had congenital adrenal hyperplasia (CAH) and most 46,XY subjects (65%) did not have a known diagnosis. Two individuals with CAH underwent a change in sex of rearing from male to female within 2 weeks of birth. The presence of a uterus and shorter phallic length were associated with female sex of rearing. The most common karyotype and diagnosis was 46,XX with CAH, followed by 46,XY with an unknown diagnosis. Phenotypically, atypical genitalia have been most commonly characterized by abnormal labioscrotal tissue, phallic length, and urethral meatus location. CONCLUSIONS: An increased phallic length was positively associated with rearing male. Among the US centers studied, sex designation followed the Consensus Statement recommendations. Further study is needed to determine whether this results in patient satisfaction.

Parent-Rated Severity of Illness and Anxiety among Caregivers of Children Born with a Disorder of Sex Development Including Ambiguous Genitalia.

BACKGROUND/AIMS: Parents of children born with disorders of sex development (DSD) often experience anxiety, but risk factors, including parental perception of the severity of their child's DSD, have not been examined. We hypothesized that severity of illness (SOI) ratings would relate to parental anxiety, and would be higher for parents of children with a potentially life-threatening DSD (e.g., 21-hydroxylase deficiency). METHODS: Eighty-nine parents (Mage = 33.0, 56.2% mothers) of 51 children (Mage in months = 8.7) with a DSD including ambiguous genitalia were recruited from 12 specialized DSD clinics. Parents completed questionnaires prior to genitoplasty, 6 months post-genitoplasty, and 12 months post-genitoplasty (if completed). Data were analyzed with linear mixed modeling. RESULTS: Parental anxiety decreased over time, χ2(1) = 10.14, p < 0.01. A positive relationship between SOI and anxiety was found, with SOI being a strong predictor of anxiety (b = 0.53, p < 0.01; χ2[1] = 5.33, p < 0.05). An SOI by time interaction indicated SOI had an increasing effect on anxiety over time, b = 0.06, p < 0.05; χ2(1) = 6.30, p < 0.05. There was no diagnosis by SOI interaction. CONCLUSION: Parental anxiety decreased over time, but those with higher SOI ratings reported greater initial anxiety followed by slower resolution over time. Underlying etiology of DSD had no effect on the relationship between SOI and anxiety.

Differences in early career operative experiences among pediatric urologists.

INTRODUCTION: Previous research suggests that pediatric urologists feel well trained by their fellowship for cases encountered early in their career. We questioned the complexity and diversity of cases new pediatric urologists were actually performing. OBJECTIVE: The aim was to identify the frequency with which newly trained pediatric urologists are performing various procedures, investigate which factors are associated with case complexity and diversity, and evaluate for differences between male and female surgeons. STUDY DESIGN: Case logs of urologists from July 30, 2007, to June 30, 2013, initially applying for the certifying examination who self-identified as pediatric urologists were reviewed. Data points included cases/dates, and surgeon demographics. An in-depth analysis was performed on 51 index cases from the 71 included pediatric urologists, for which a level of complexity was assigned. RESULTS: Compared with the bottom volume quartile, surgeons in the top quartile performed more cases of minimal (115.9 ± 8.7 vs. 51.7 ± 8.7, p < 0.001), moderate (31.1 ± 2.7 vs. 10.1 ± 1.0, p < 0.001) and significant (10.8 ± 1.9 vs. 2.0 ± 0.4, p < 0.001) complexity. More than 90% logged circumcisions, orchiopexies, and inguinal hernia repairs, while less than 1.5% logged open nephroureterectomies or complete male epispadias repair. Surgeons submitted at least one of 17.2 ± 0.5 (range 5-28) unique codes. The figure presents the percentage of current procedural terminology (CPT) codes performed by each urologist. Surgeons with the least case diversity performed a higher percentage of low-complexity cases, and lower percentages of moderate and complex cases (p < 0.001). Males, comprising 60.6% of urologists, performed more cases than females (342.9 ± 30.9 vs. 229.1 ± 18.1, p = 0.007), averaging more cases of minimal (95.0 ± 6.6 vs. 73.3 ± 4.6, p = 0.018) and significant (6.7 ± 1.0 vs. 2.8 ± 0.5, p = 0.005) complexity. There was no difference in cases of moderate complexity (22.0 ± 1.9 vs. 18.1 ± 2.1, p = 0.201). DISCUSSION: In general, pediatric urologists should expect to perform many minor cases when they enter practice. Women are entering urology in increasing numbers. In our study, female urologists performed fewer cases. This could have implications for the workforce, which in urology in general is expected to decrease. CONCLUSIONS: Case diversity and degree of complexity vary among newly trained pediatric urologists. The urologist with the greatest case diversity never performed 45% of the 51 analyzed CPT codes, while the one with the least case diversity never performed 90% of the codes. Male surgeons performed more operations, particularly those of minimal and significant complexity. The variability in operative experience reinforces the importance of continuing education and mentorship after completion of fellowship.

Children with nocturnal enuresis and attention deficit hyperactivity disorder: A separate entity?

INTRODUCTION: There is ongoing debate about whether nocturnal enuresis (NE) is affected by the presence of attention deficit hyperactivity disorder (ADHD) in children. Clarifying this relationship could categorize them as a separate entity, potentially requiring a different management from enuretic children without ADHD. AIMS: To study whether (1) children with ADHD exhibited greater severity of enuresis and associated voiding symptoms compared with children without ADHD, (2) whether there was a difference in enuretic response to treatment between children with/without ADHD, (3) whether previously prescribed medication for ADHD affected severity of enuresis or voiding symptoms, and response to treatment. METHODS: Data on all consecutive children seen with primary NE and ADHD and age- and gender-matched children with NE without ADHD over the course of 2 years were extracted from our database. Controls were identified using a random number generator to choose patients within each age/gender group. Changes in Dysfunctional Voiding Scoring System (DVSS) and Bristol scores were assessed before and after treatment for NE using repeated measures ANOVAs. Number of weekly wet nights was compared between the groups before and after the treatment of enuresis. Children with more than a 50% decrease in number of wet nights per week were categorized as responding to treatment. RESULTS: Compared with controls, ADHD children (N = 95) had significantly more severe voiding symptoms, NE and constipation (Table). No significant differences were found between children on ADHD medication (63.2%) and those who were not on ADHD medication (36.8%) on any of the demographic and pre-treatment clinical parameters. Similar enuretic response rates to behavioral modification alone were seen in both study (13.5%) and control (12.8%) groups (p = 0.73). Response to pharmacological treatment was seen in nine patients with ADHD (42.9%) and in six (20.7%) controls, with pharmacological intervention being significantly more effective than behavioral modification for patients with ADHD (p = 0.012). Additionally, no difference was found between ADHD patients on and off ADHD medication in terms of response to enuresis treatment. CONCLUSION: In children with primary NE, the presence of ADHD was associated with more severe NE, voiding symptoms and constipation. The severity of voiding symptoms and/or NE was unrelated to the use of stimulant medication. The response to behavioral modification was comparable in both groups. However, patients with ADHD were significantly more responsive to medication for NE compared with behavioral modification, indicating a possible benefit for earlier pharmacological treatment for enuresis in this population subgroup.

Recommendations for the Establishment of Disorders/Differences of Sex Development Interdisciplinary Care Clinics for Youth.

PURPOSE: Provide recommendations for the development of an interdisciplinary care (IDC) clinic for the treatment of youth with disorders/differences of sex development (DSD). DSD consist of a group of complex congenital medical disorders in which the development of chromosomal, gonadal, or anatomical sex is atypical. Youth with DSD require care from multiple specialized healthcare disciplines, including several medical specialties, surgery, nursing, and mental health. METHOD: Recommendations are based on an interdisciplinary care clinic model that allows for a team of relevant professionals who share knowledge, ideas, and responsibility of care. The framework established in this article is based largely on experiences at an established DSD clinic, as well as observations of multiple clinics across the United States. RESULTS: Preliminary outcome data on clinic adherence to treatment protocol under an IDC model are provided. CONCLUSIONS: To meet the diverse healthcare needs of youth with DSD, comprehensive care clinics are recommended; however, few such clinics exist in the United States. Establishing new comprehensive DSD clinics can be challenging due to the highly unique treatment of DSD, but the current paper expands the literature available to guide clinic development in the United States.

Psychological Adjustment of Parents of Children Born with Atypical Genitalia 1 Year after Genitoplasty.

PURPOSE: We examined the psychological adjustment of parents of children born with moderate to severe genital atypia 12 months after their child underwent genitoplasty. MATERIALS AND METHODS: Parents were recruited longitudinally from a multicenter collaboration of 10 pediatric hospitals with specialty care for children with disorders/differences of sex development and/or congenital adrenal hyperplasia. Parents completed measures of depressive and anxious symptoms, illness uncertainty, quality of life, posttraumatic stress and decisional regret. RESULTS: Compared to levels of distress at baseline (before genitoplasty) and 6 months after genitoplasty, data from 25 mothers and 20 fathers indicated significant improvements in all psychological distress variables. However, a subset of parents continued endorsing clinically relevant distress. Some level of decisional regret was endorsed by 28% of parents, although the specific decision that caused regret was not specified. CONCLUSIONS: Overall the majority of parents were coping well 1 year after their child underwent genitoplasty. Level of decisional regret was related to having a bachelor's level of education, increased levels of illness uncertainty preoperatively and persistent illness uncertainty at 12 months after genitoplasty but was unrelated to postoperative complications.

Gender identity outcomes in children with disorders/differences of sex development: Predictive factors.

Disorders/differences of sex development (DSD) comprise multiple congenital conditions in which chromosomal, gonadal, and/or anatomical sex are discordant. The prediction of future gender identity (i.e., self-identifying as male, female, or other) in children with DSD can be imprecise, and current knowledge about the development of gender identity in people with, and without DSD, is limited. However, sex of rearing is the strongest predictor of gender identity for the majority of individuals with various DSD conditions. When making decisions regarding sex of rearing biological factors (e.g., possession of a Y chromosome, degree and duration of pre- and postnatal androgen exposure, phenotypic presentation of the external genitalia, and fertility potential), social and cultural factors, as well as quality of life should be considered. Information on gender identity outcomes across a range of DSD diagnoses is presented to aid in sex of rearing assignment.

Changes in levels of parental distress after their child with atypical genitalia undergoes genitoplasty.

BACKGROUND: The birth of a child with a disorder of sex development (DSD) and atypical genitalia can be traumatizing and isolating for families. Parents of children with DSD are at risk for increased levels of psychological distress, including depression, anxiety, illness uncertainty (IU), post-traumatic stress symptoms (PTSS), and impairments in quality of life (QOL). Our previous report indicated that although the majority of parents of children with atypical genitalia were coping well prior to the child's genitoplasty, approximately 25% of them reported experiencing some type of psychological distress. OBJECTIVE: The current study sought to examine the trajectory of parental psychological distress prior to, and 6 months after their child underwent genitoplasty. METHODS: Parents were recruited as part of an ongoing, prospective, multi-site study involving 10 pediatric hospitals with specialized care for children with atypical genitalia. Results from 49 parents (27 mothers, 22 fathers) of 28 children (17 female sex of rearing, 11 male sex of rearing) born with atypical genitalia (Prader rating of 3-5 in 46,XX DSD or by a Quigley rating of 3-6 in 46,XY DSD or 45,XO/46,XY) were included in the study. RESULTS: There were no significant changes in level of depressive or anxious symptoms or quality of life between baseline and 6-month post-operative follow-up, although mothers continued to report significantly higher levels of depressive symptoms than fathers, and as a group, these parents reported lower QOL than published norms. The level of PTSS significantly decreased for all parents, suggesting that parents may have come through the acute stress phase associated with their child's diagnosis. Finally, while there were no significant changes in IU over the time period, the level of IU for parents of boys actually increased, while parents of girls reported no change (Figure). CONCLUSION: Six months after their child has undergone genitoplasty, the majority of parents report minimal levels of psychological distress. However, a subset of these parents continue to experience significant distress related to their child's diagnosis. Specifically, parents of boys appear to be at increased risk for difficulties, which may be related to either the lack of clinical diagnosis for almost half of these children or the necessity of two-stage surgeries for the majority of them. We will continue collecting data on these families to better understand the trajectory of these adjustment variables.

Postoperative urinary retention in men is common after carotid endarterectomy and is associated with advanced age and prior urinary tract infection.

OBJECTIVE: This study was undertaken to analyze the occurrence of postoperative urinary retention (POUR) after carotid endarterectomy (CEA) and determine whether there are any associated modifiable risk factors. CEA was chosen to minimize the confounding effects of known risk factors for POUR, including immobilization, regional and severe pain, and neuroaxial anesthesia. METHODS: This was a retrospective record review of 186 male patients undergoing CEA between 2007 and 2011. Demographic, comorbidities, and operative characteristics were compared. Continuous variables are reported as median and interquartile range (IQR) and categoric variables as frequencies and proportions. Pearson χ(2) or Mann-Whitney U tests compared categoric and continuous variables, respectively. Logistic regression was used to examine univariate and multivariate odds of POUR. Multivariate analysis controlled for known predictors of urinary retention. Association with other complications was examined with the Pearson correlation coefficient. RESULTS: POUR occurred in 34 patients (18.3%). Median age and history of urinary tract infection (UTI) were significantly associated with POUR: median age was 73.0 years (IQR, 67-80 years) for those with POUR vs 69.5 years (IQR, 63-76 years) for those without (P = .047); 17.6% of patients with a history of UTI developed POUR vs 5.9% without (P = .023). These findings persisted on multivariate analysis controlling for known predictors of POUR (body mass index, history of diabetes, benign prostate hyperplasia, and prior prostate surgery): median age (odds ratio, 1.05; 95% confidence interval, 1-1.1) and history of UTI (odds ratio, 4.16; 95% confidence interval, 1.23-14.05; P = .022). The occurrence of POUR was significantly correlated with postoperative UTI: 18.8% with POUR vs 0.7% without (Pearson r = 0.369; P < .001). CONCLUSIONS: POUR requiring bladder catheterization after CEA predisposes patients to postoperative UTI and is more common in older patients and those with a history of UTI. CEA patients lack inherent risk factors for POUR and would be a useful population for prospective studies involving POUR.

Evidence of Need to Use Self-Report Measures of Psychosocial Functioning in Older Children and Adolescents with Voiding Dysfunction.

PURPOSE: Previous reports have revealed significantly higher rates of psychosocial difficulties in children and adolescents with voiding dysfunction compared to their healthy peers. However, these findings are based solely on parental reporting and do not include self-reporting of psychosocial problems in older pediatric patients. MATERIALS AND METHODS: We collected data from 200 consecutive patients 11 to 16 years old during outpatient clinic visits. Patients completed the Pediatric Symptom Checklist-Youth Report, parents completed the parental report of the same measure, and patients and parents collaboratively completed the Dysfunctional Voiding Scoring System. RESULTS: Of the patients 25.5% met the cutoff score for clinically significant levels of psychosocial difficulties. However, only a fourth of those patients met the cutoff on the parent and self-report measures. Additionally patient self-reports of internalizing and externalizing problems were significantly related to severity of voiding dysfunction. CONCLUSIONS: Screening for psychosocial problems in older children and adolescents with voiding dysfunction should include reports from the parent and the child. In our sample 37.3% of patients needing a mental health followup would have been missed if only 1 version of the measure had been administered.

Adenotonsillectomy Normalizes Hormones and Urinary Electrolytes in Children With Nocturnal Enuresis and Sleep-Disordered Breathing.

OBJECTIVE: To assess (1) plasma levels of antidiuretic hormone (ADH) and brain natriuretic peptide (BNP) and urinary levels of electrolytes in children with sleep disordered breathing (SDB), with or without nocturnal enuresis (NE), and (2) the effect of adenotonsillectomy (T&A) on urinary electrolytes and the secretion of ADH and BNP in children with NE and SDB. We previously reported post-T&A improvements in plasma levels of BNP and ADH in children with SDB and NE. However, the differences in plasma concentration of these hormones in SDB children with and without NE, and their relationships with urinary electrolytes, have not yet been addressed. METHODS: This prospective study compared concentrations of urinary electrolytes and plasma ADH and BNP in (1) children with SDB and NE (study group) and an age- and sex-matched control group of children with SDB without NE, and (2) the study group before and 1-month after T&A. RESULTS: Compared with the control group (n = 31), the study group (n = 37) exhibited significantly lower ADH (P = .04) and higher BNP (P = .009) plasma levels. The differences in urinary electrolytes were not significant. Post-T&A, the study group showed significantly decreased BNP (P = .018), urinary sodium-to-creatinine ratio (P = .02), and urinary calcium-to-creatinine ratio (P = .007) compared with the pre-T&A values. Post-T&A changes in urinary calcium were significantly correlated with changes in sodium excretion (P = .002) and in plasma levels of BNP (P <.001). CONCLUSION: The presence of NE is associated with altered ADH and BNP levels in children with SDB. T&A led to normalization of ADH and BNP, probably through a calcium- and sodium-dependent mechanism.