RESUMEN
PURPOSE: Our goal was to understand the general attitudes of pediatric urologists regarding the U.S. News & World Report rankings for Best Children's Hospitals in Urology. MATERIALS AND METHODS: We performed a cross-sectional survey study distributed via email to active and candidate members of the Societies for Pediatric Urology from October 2022 to December 2022. This anonymous survey was iteratively developed to contain multiple choice questions gathering information on demographics, personal interaction with the rankings system, and about attitudes toward the rankings across various domains. RESULTS: Of the 515 members surveyed, 264 pediatric urologists responded to the survey for a response rate of 51%. There was representation from all sections of the AUA and across all age categories and practice models. Overall, of the respondents, 71% disagreed that the rankings had led to improvements in care and 75% disagreed that programs were reporting their data honestly. Additionally, 71% believed the rankings are inaccurate in reflecting patient outcomes. The majority (86%) of respondents stated they would support organized efforts to withdraw from the rankings. This was significantly different by ranking cohort, with 78% from top 10 programs endorsing withdrawal vs 89% from those programs not in the top 10. CONCLUSIONS: Our survey found that most pediatric urologists would support efforts to withdraw from participating in the rankings and believe that programs are dishonest in reporting their data. The majority also do not believe a survey can adequately distinguish between programs. This highlights a clear need for a critical reevaluation of the rankings.
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Actitud del Personal de Salud , Urólogos , Urología , Humanos , Estudios Transversales , Estados Unidos , Masculino , Femenino , Urólogos/estadística & datos numéricos , Niño , Hospitales Pediátricos/normas , Hospitales Pediátricos/estadística & datos numéricos , Encuestas y Cuestionarios , Adulto , Pediatría/normas , Persona de Mediana EdadRESUMEN
PURPOSE: Mixed gonadal dysgenesis is a difference of sex development that is often confused with other conditions. Individuals have a 45,X/46,XY karyotype. Gonads are characterized by a streak gonad and a dysgenetic testis at varying levels of descent. Persistent Müllerian structures are typical (eg, hemi-uterus). There is significant phenotypic heterogeneity of the internal and external genitalia that, together with different interpretations of the definition, have contributed to a poor understanding of the condition among pediatric urologists. Mixed gonadal dysgenesis is one manifestation of the 45,X/46,XY karyotype. 45,X/46,XY mosaicism can also be associated with typical female or male external genitalia. This review aims to clarify the mixed gonadal dysgenesis definition and to provide urologists with diagnostic and management considerations for affected individuals. MATERIALS AND METHODS: We searched 3 medical databases for articles related to mixed gonadal dysgenesis. Two hundred eighty-seven full-text abstracts and manuscripts were reviewed for content pertinent to: (1) clarifying the definition of mixed gonadal dysgenesis, and (2) describing the following related to the care of affected individuals: prenatal and neonatal evaluation and management, genital surgery, gonadal malignancy risk and management, fertility, gender dysphoria/incongruence, puberty and long-term outcomes, systemic comorbidities, and transitional care. RESULTS: Fifty articles were included. Key points and implications for each of the above topics were summarized. CONCLUSIONS: Mixed gonadal dysgenesis exists on a wide phenotypic spectrum and management considerations reflect this heterogeneity. Care for individuals with mixed gonadal dysgenesis is complex, and decisions should be made in a multidisciplinary setting with psychological support.
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Disgenesia Gonadal Mixta , Humanos , Masculino , Disgenesia Gonadal Mixta/genética , Disgenesia Gonadal Mixta/diagnóstico , Femenino , UrólogosRESUMEN
OBJECTIVE: Over half of infant boys born in the United States undergo newborn circumcision. However, available data indicate that boys who are publicly insured, or Black/African American, have less access to desired newborn circumcision, thus concentrating riskier, more costly operative circumcision among these populations. This study ascertains perinatal physician perspectives about barriers and facilitators to providing newborn circumcisions, with a goal of informing future strategies to ensure more equitable access. METHODS: Qualitative interviews about newborn circumcision care were conducted from April-June 2020 at eleven Chicago-Area hospitals. Physicians that provide perinatal care (pediatricians, family medicine physicians, and obstetricians) participated in qualitative interviews about newborn circumcision. Inductive and deductive qualitative coding was performed to identify themes related to barriers and facilitators of newborn circumcision care. RESULTS: The 23 participating physicians (78% female, 74% white, median 16 years since medical school graduation [range 5-38 years], 52% hospital leadership role, 78% currently perform circumcisions) reported multiple barriers including difficulty with procedural logistics and inconsistent clinician availability and training; corresponding suggestions for operational improvements were also provided. Regarding newborn circumcision insurance coverage and reimbursement, physicians reported limited knowledge, but noted that some insurance reimbursement policies financially disincentivize clinicians and hospitals from offering inpatient newborn circumcision. CONCLUSIONS: Physicians identified logistical/operational, and reimbursement-related barriers to providing newborn circumcision for desirous families. Future studies and advocacy work should focus on developing clinical strategies and healthcare policies to ensure equitable access, and incentivize clinicians/hospitals to perform newborn circumcisions.
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Circuncisión Masculina , Médicos Generales , Masculino , Lactante , Recién Nacido , Humanos , Estados Unidos , Femenino , Cobertura del Seguro , Pediatras , ChicagoRESUMEN
PURPOSE: Robot-assisted laparoscopic pyeloplasty (RALP) use in children has increased, though many centers still favor open pyeloplasty (OP) in infants. This study aims to compare safety and efficacy of RALP and OP in infants. MATERIALS AND METHODS: A single-institution, retrospective cohort study of infants <1 year of age who underwent primary RALP or OP between January 2009 and June 2020 was performed. Primary outcomes were intraoperative and 30-day complications, postoperative radiographic improvement at last clinic visit, and operative failure leading to redo pyeloplasty. Multivariable logistic regression was performed for 30-day complications to adjust for demographic variation between groups. Survival analysis was performed to compare time to diagnosis of operative failure leading to redo pyeloplasty. RESULTS: Among 204 patients, 121 underwent OP and 83 underwent RALP (74.5% male). RALP patients were older (median 7.2 vs 2.9 months, p <0.001) and larger (median 8.2 vs 5.9 kg, p <0.001) than OP patients. Radiographic improvement was seen in 91.1% of RALP patients and 88.8% of OP patients at last visit. Median (interquartile range) followup in months was 24.4 (10.8-50.3) for the full cohort. In adjusted analysis, the odds of a 30-day complication (OR 0.40, 95% CI 0.08-2.00) was lower for RALP compared to OP, though not statistically significant. In survival analysis, there was no difference in time to diagnosis of operative failure and redo pyeloplasty between groups (p=0.65). CONCLUSIONS: RALP is a safe and effective alternative to OP for infants, with comparable intraoperative and 30-day complications, radiographic improvement at last followup, and risk of pyeloplasty failure.
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Pelvis Renal/cirugía , Laparoscopía/efectos adversos , Procedimientos de Cirugía Plástica/efectos adversos , Complicaciones Posoperatorias/epidemiología , Procedimientos Quirúrgicos Robotizados/efectos adversos , Obstrucción Ureteral/cirugía , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Pelvis Renal/diagnóstico por imagen , Laparoscopía/estadística & datos numéricos , Tiempo de Internación/estadística & datos numéricos , Masculino , Tempo Operativo , Complicaciones Posoperatorias/etiología , Procedimientos de Cirugía Plástica/métodos , Procedimientos de Cirugía Plástica/estadística & datos numéricos , Estudios Retrospectivos , Procedimientos Quirúrgicos Robotizados/estadística & datos numéricos , Análisis de Supervivencia , Resultado del Tratamiento , Uréter/diagnóstico por imagen , Uréter/cirugía , Obstrucción Ureteral/congénito , Obstrucción Ureteral/mortalidadRESUMEN
OBJECTIVES: Prenatal diagnoses of differences of sex development (DSD) are increasing due to availability of cell-free DNA screening (cell-free DNA screening (cfDNA)). This study explores first-hand experiences of parents whose children had prenatal findings of DSD. METHODS: Eligible parents were identified through chart review at a pediatric center and interviewed about their prenatal evaluation, decision making, informational sources, and support systems. Interviews were coded using a combined inductive and deductive thematic analysis. Parents also completed quantitative measures of decisional regret. RESULTS: Seventeen parents (13 mothers; 4 fathers) of 13 children (with 7 DSD diagnoses) were recruited. Four children had discordance between sex predicted by cfDNA versus prenatal ultrasound, and 2 had non-binary appearing (atypical) genitalia on prenatal ultrasound. Of these 6, 3 were not offered additional prenatal testing or counseling. Most parents described tension between obtaining support through disclosure of their child's diagnosis and preserving their child's autonomy/privacy, highlighting the need for mental health support. CONCLUSION: This is the first study to gather qualitative data from parents whose children had prenatal findings of DSD. We identified multiple targets for intervention to improve care for patients with DSD across the lifespan, including improvements in clinician education, pre- and post-test counseling, and patient education materials.
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Ácidos Nucleicos Libres de Células , Padres , Niño , Consejo , Emociones , Femenino , Humanos , Padres/psicología , Embarazo , Investigación CualitativaRESUMEN
PURPOSE: Among some populations access to neonatal circumcision has become increasingly limited despite evidence of its benefits. This study examines national neonatal circumcision trends before and after the 2012 American Academy of Pediatrics recommendation for neonatal circumcision reimbursement. MATERIALS AND METHODS: A retrospective cohort study of boys aged 28 days or less was conducted using data from the Kids' Inpatient Database (2003 to 2016). Boys who underwent neonatal circumcision prior to discharge were compared to boys who did not. Boys with coagulopathies, penile anomalies or a history of prematurity were excluded. RESULTS: An estimated 8,038,289 boys comprised the final cohort. Boys were primarily White (53.7%), privately insured (49.1%) and cared for at large (60.8%) teaching (49.4%) hospitals in metropolitan areas (84.1%). While 55.0% underwent circumcision prior to discharge, neonatal circumcision rates decreased significantly over time (p <0.0001). Black (68.0%) or White (66.0%) boys, boys in the highest income quartile (60.7%) and Midwestern boys (75.0%) were most likely to be circumcised. Neonatal circumcision was significantly more common among privately (64.9%) than publicly (44.6%) insured boys after controlling for demographics, region, hospital characteristics and year (p <0.0001). The odds of circumcision over time were not significantly different in the years before vs after 2012 (p=0.28). CONCLUSIONS: Among approximately 8 million boys sampled over a 13-year period 55.0% underwent neonatal circumcision. The rate of neonatal circumcision varied widely by region, race and socioeconomic status. The finding that boys with public insurance have lower circumcision rates in all years may be related to lack of circumcision access for boys with public insurance.
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Circuncisión Masculina/tendencias , Accesibilidad a los Servicios de Salud/economía , Disparidades en Atención de Salud/economía , Negro o Afroamericano/estadística & datos numéricos , Circuncisión Masculina/economía , Geografía , Accesibilidad a los Servicios de Salud/estadística & datos numéricos , Accesibilidad a los Servicios de Salud/tendencias , Disparidades en Atención de Salud/estadística & datos numéricos , Disparidades en Atención de Salud/tendencias , Humanos , Recién Nacido , Cobertura del Seguro/economía , Cobertura del Seguro/estadística & datos numéricos , Seguro de Salud/economía , Seguro de Salud/estadística & datos numéricos , Masculino , Estudios Retrospectivos , Clase Social , Estados Unidos , Población Blanca/estadística & datos numéricosRESUMEN
PURPOSE: This study aims to examine contemporary practice patterns and compare short-term outcomes for vesicoureteral reflux procedures (ureteral reimplant/endoscopic injection) using National Surgical Quality Improvement Program-Pediatric data. MATERIALS AND METHODS: Procedure-specific variables for antireflux surgery were developed to capture data not typically collected in National Surgical Quality Improvement Program-Pediatric (eg vesicoureteral reflux grade, urine cultures, 31-60-day followup). Descriptive statistics were performed, and logistic regression assessed associations between patient/procedural factors and outcomes (urinary tract infection, readmissions, unplanned procedures). RESULTS: In total, 2,842 patients (median age 4 years; 76% female; 68% open reimplant, 6% minimally invasive reimplant, 25% endoscopic injection) had procedure-specific variables collected from July 2016 through June 2018. Among 88 hospitals, a median of 24.5 procedures/study period were performed (range 1-148); 95% performed ≥1 open reimplant, 30% ≥1 minimally invasive reimplant, and 70% ≥1 endoscopic injection, with variability by hospital. Two-thirds of patients had urine cultures sent preoperatively, and 76% were discharged on antibiotics. Outcomes at 30 days included emergency department visits (10%), readmissions (4%), urinary tract infections (3%), and unplanned procedures (2%). Over half of patients (55%) had optional 31-60-day followup, with additional outcomes (particularly urinary tract infections) noted. Patients undergoing reimplant were younger, had higher reflux grades, and more postoperative occurrences than patients undergoing endoscopic injections. CONCLUSIONS: Contemporary data indicate that open reimplant is still the most common antireflux procedure, but procedure distribution varies by hospital. Emergency department visits are common, but unplanned procedures are rare, particularly for endoscopic injection. These data provide basis for comparing short-term complications and developing standardized perioperative pathways for antireflux surgery.
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Hospitales Pediátricos , Pautas de la Práctica en Medicina/estadística & datos numéricos , Reflujo Vesicoureteral/cirugía , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Complicaciones Posoperatorias , Mejoramiento de la Calidad , Estados UnidosRESUMEN
BACKGROUND: A significant proportion of boys present to surgical specialists later in infancy/early childhood for elective operative circumcision despite the higher procedural risks. This study aims to assess physician perspectives on access to neonatal circumcision across the United States and identify potential reasons contributing to disparities in access. METHODS: A cross-sectional survey was electronically distributed to physician members of the Societies for Pediatric Urology and the American Academy of Pediatrics Section on Hospital Medicine. Hospital characteristics and circumcision practices were assessed. Associations between NC availability and institutional characteristics were evaluated using chi-squared testing and multivariable logistic regression. Qualitative analyses of free-text comments were performed. RESULTS: A total of 367 physicians responded (129 urologists [41%], 188 pediatric hospitalists [59%]). Neonatal circumcision was available at 86% of hospitals represented. On univariate and multivariate analysis, the 50 hospitals that did not offer neonatal circumcision were more likely to be located in the Western region (odds ratio [OR] = 8.33; 95% confidence interval [CI] 3.1-25 vs. Midwest) and in an urban area (OR = 4.2; 95% CI 1.6-10 vs. suburban/rural) compared with hospitals that offered neonatal circumcision. Most common reasons for lack of availability included not a birth hospital (N = 22, 47%), lack of insurance coverage (N = 8, 17%), and low insurance reimbursement (N = 7, 15%). Institutional, regional, or provider availability (68%), insurance/payment (12.4%), and ethics (12.4%) were common themes in the qualitative comments. CONCLUSIONS: Overall availability of neonatal circumcision varied based on hospital characteristics, including geography. Information from this survey will inform development of interventions designed to offer neonatal circumcision equitably and comprehensively.
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Circuncisión Masculina/estadística & datos numéricos , Accesibilidad a los Servicios de Salud/estadística & datos numéricos , Encuestas Epidemiológicas , Disparidades en Atención de Salud/estadística & datos numéricos , Estudios Transversales , Médicos Hospitalarios , Hospitales , Humanos , Recién Nacido , Cobertura del Seguro , Reembolso de Seguro de Salud , Masculino , Pautas de la Práctica en Medicina , Estados Unidos , UrólogosRESUMEN
PURPOSE: The prevalence of endocrine/genetic anomalies in boys with proximal hypospadias is unknown. We describe an endocrine/genetic evaluation for boys with proximal hypospadias to determine who may have a difference/disorder of sex development and/or benefit from additional testing. MATERIALS AND METHODS: We retrospectively reviewed boys with hypospadias seen at our hospital between January 2013 and October 2018. Those with proximal (penoscrotal, scrotal, perineal) hypospadias who presented before age 6 months and underwent endocrine/genetic testing were included. Demographics, test results, testicular examination at presentation, comorbidities and diagnoses were abstracted. RESULTS: A total of 1,789 boys with hypospadias were identified. Of 131 boys with proximal hypospadias all 60 who underwent endocrine/genetic evaluation were included. Most of these patients had bilateral palpable testes (52, 86%) that were fully descended (41, 68%). Associated anatomical anomalies were found in 53% of patients. All boys underwent endocrine testing, which was completely typical for a male infant in most (41, 68%). Common genetic tests included karyotyping (100%), microarray (38%) and multigene panel (13%). Genetic anomalies were found in 17 boys (28%), including 7 of 41 (17%) with bilateral descended testes and 10 of 19 (53%) with 1 or more undescended testes (p=0.01). Six of 8 boys with at least 1 nonpalpable testis had a genetic anomaly vs 11 of 52 with bilateral palpable testes (p=0.005). Differences/disorders of sex development were found in 9 patients (15%). CONCLUSIONS: Of 60 boys with proximal hypospadias 53% had nongenital anomalies, 28% had genetic anomalies and 15% had a difference/disorder of sex development. Although endocrine testing was clinically useful, genetic testing was most diagnostically revealing. Endocrine/genetic evaluation should be considered for boys with proximal hypospadias.
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Pruebas Genéticas , Hormonas Esteroides Gonadales/análisis , Hipospadias/genética , Trastornos del Desarrollo Sexual/genética , Humanos , Lactante , Recién Nacido , Masculino , Estudios RetrospectivosRESUMEN
PURPOSE: Many individuals with differences of sex development experience subfertility. We describe a novel gonadal tissue cryopreservation protocol for those individuals. MATERIALS AND METHODS: Before 2018 patients with differences of sex development electing gonadal tissue cryopreservation were enrolled in a cancer focused protocol. Thereafter, our hospital developed a protocol specifically for these patients undergoing gonadectomy due to neoplasia risk. Protocol development steps and procedures are reported. A retrospective chart review was conducted for patient characteristics and cryopreservation choices. RESULTS: During the institutional review board approval process there were multidisciplinary meetings with stakeholders. The main concerns discussed included preoperative counseling, pathological evaluation and final tissue disposition. Detailed multidisciplinary preoperative counseling is provided regarding potential gonadal tissue cryopreservation for patients undergoing gonadectomy. For enrolled patients the gonad is bisected after removal, with half being sent to pathology and half being processed for cryopreservation. If neoplasia is noted, cryopreserved tissue is recalled for further pathological analysis. Postoperative counseling is performed after pathology results are available, and the final gonadal tissue cryopreservation decision is made. During the study period 7 patients with 5 diagnoses and a median age of 10.99 years (IQR 1.29 to 14.84) elected to attempt gonadal tissue cryopreservation. Of the patients 4 (57%) had germ cells and elected to store tissue. CONCLUSIONS: Gonadal tissue cryopreservation at the time of gonadectomy is feasible for patients with differences of sex development at risk for gonadal neoplasia. The protocol described represents a template for institutions wishing to offer gonadal tissue cryopreservation to patients electing gonadectomy. More than half of patients thus far have cryopreserved gonadal tissue.
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Protocolos Clínicos/normas , Criopreservación/normas , Trastornos del Desarrollo Sexual/cirugía , Preservación de la Fertilidad/normas , Infertilidad Femenina/terapia , Ovario , Adolescente , Niño , Preescolar , Consejo/normas , Trastornos del Desarrollo Sexual/complicaciones , Estudios de Factibilidad , Femenino , Preservación de la Fertilidad/métodos , Humanos , Lactante , Infertilidad Femenina/etiología , Estudios RetrospectivosRESUMEN
PURPOSE: The medical terminology applied to differences/disorders of sex development has been viewed negatively by some affected individuals. A clinical population of patients with differences/disorders of sex development and their caregivers were surveyed regarding current nomenclature, hypothesizing that those unaffiliated with support groups would have more favorable attitudes. MATERIALS AND METHODS: We recruited English and Spanish speaking patients 13 years old or older with differences/disorders of sex development and their caregivers at 5 national tertiary care clinics from July 2016 to December 2018. No diagnoses were excluded. Participants completed a survey rating terminology commonly applied to differences/disorders of sex development. Responses were compared between subgroups, including members vs nonmembers of a support group. RESULTS: Of 185 potential participants approached 133 completed the survey (72% response rate). Congenital adrenal hyperplasia (33%) was the most common diagnosis. "Variation of sex development" was the most liked term (37%) but was not liked more significantly than "disorders of sex development" (27%, p=0.16). No term was liked by a majority of respondents. "Disorders of sex development" (37%) and "intersex" (53%) were the only terms most frequently viewed unfavorably. Support group members were significantly more likely to dislike the term "intersex" (p=0.02) and to like "variation of sex development" (p=0.02). CONCLUSIONS: A clinical population of patients and their caregivers had generally neutral attitudes toward nomenclature applied to differences/disorders of sex development. Members of a support group had clearer terminology preferences. "Variation of sex development" was the most liked term, and "disorders of sex development" and "intersex" were the most disliked. No term was liked by most respondents, and no clear alternative to the present nomenclature was identified.
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Actitud Frente a la Salud , Cuidadores/psicología , Trastornos del Desarrollo Sexual , Pacientes/psicología , Terminología como Asunto , Adolescente , Estudios Transversales , Femenino , Humanos , MasculinoRESUMEN
OBJECTIVE: Discrepancies between cfDNA and ultrasound predicted fetal sex occur, possibly indicating disorders/differences of sex development (DSDs). Among expectant/recent parents, this study assessed cfDNA knowledge/use, fetal sex determination attitudes/behaviors, general knowledge of DSD, and possible psychological impact of discrepancy between fetal sex on cfDNA and ultrasound. METHOD: Parents were surveyed about fetal sex determination methods, knowledge of cfDNA and DSD, distress related to possible cfDNA inaccuracy. RESULTS: Of 916 respondents, 44% were aware of possible discrepancy between cfDNA and ultrasound, 22% were aware of DSD. 78% and 75% would be upset and worried, respectively, with results showing fetal sex discrepancy. Most (67%) revealed predicted fetal sex before delivery. 38% were offered cfDNA. Of those revealing fetal sex, 24% used cfDNA results, 71% ultrasound, and 7% both. cfDNA users were more frequently aware of possible discrepancy between cfDNA and ultrasound (76% vs 41%, P < .0001), but not of DSD (29% vs 23%, P = .29). CONCLUSION: Fetal sex determination is favored, and cfDNA is frequently used for predicting fetal chromosomal sex. Many parents are unaware of possible discrepancies between cfDNA and ultrasound, and potential for DSD. Most would be distressed by discordant results. Accurate counseling regarding limitations cfDNA for fetal sex determination is needed.
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Trastornos del Desarrollo Sexual/diagnóstico , Conocimientos, Actitudes y Práctica en Salud , Pruebas Prenatales no Invasivas , Análisis para Determinación del Sexo , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Ácidos Nucleicos Libres de Células/análisis , Femenino , Humanos , Masculino , Persona de Mediana Edad , Encuestas y Cuestionarios , Adulto JovenRESUMEN
PURPOSE: Reoperative pyeloplasty is commonly used in children with recurrent obstruction after pyeloplasty. We previously reported on reoperative robot-assisted laparoscopic repair for failed pyeloplasty in 16 children and concluded that short-term and intermediate outcomes were comparable to open reoperative repair. In this updated series we describe longer term outcomes from an extended study. MATERIALS AND METHODS: We retrospectively reviewed outcomes of consecutive children with prior failed primary pyeloplasty who underwent robot-assisted laparoscopic reoperative repair at a single institution from January 2008 to June 2018. RESULTS: Overall, 36 children 0.6 to 15.2 years old (median 3.7) underwent robot-assisted laparoscopic reoperative repair (pyeloplasty in 31, ureterocalicostomy in 5) at a median of 24.3 months (range 3.9 to 136.7) after primary repair. Median reoperative time was 285.0 minutes (range 207 to 556) and median length of stay was 1 day (1 to 8). Crossing vessels were present in 8 of 30 children (26.7%) with prior open repair and in 0 of 6 with prior minimally invasive repair. Clavien-Dindo grade 1 to 2 perioperative complications occurred in 4 children (11.1%) and grade 3 to 5 complications in 2 (5.6%). Median followup was 35.3 months (range 1.4 to 108.3), with 18 children (50.0%) being followed for more than 3 years. Postoperative ultrasound in 34 children revealed improvement in 31 (91.2%), stability in 2 (5.9%) and worsening hydronephrosis in 1 (2.9%). All 11 children undergoing preoperative and postoperative diuretic renography demonstrated stable or improved differential renal function. All children were symptom-free at last followup. CONCLUSIONS: To our knowledge, this is the largest series of robot-assisted laparoscopic reoperative repair for failed pyeloplasty in children. Our results indicate the feasibility, efficacy, safety and durability of this procedure.
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Hidronefrosis/cirugía , Pelvis Renal/cirugía , Laparoscopía/métodos , Procedimientos Quirúrgicos Robotizados/métodos , Procedimientos Quirúrgicos Urológicos/efectos adversos , Adolescente , Niño , Preescolar , Estudios de Cohortes , Femenino , Humanos , Hidronefrosis/diagnóstico , Lactante , Pelvis Renal/fisiopatología , Masculino , Pronóstico , Reoperación/métodos , Estudios Retrospectivos , Insuficiencia del Tratamiento , Resultado del Tratamiento , Procedimientos Quirúrgicos Urológicos/métodosRESUMEN
Fertility preservation has become more common for various populations, including oncology patients, transgender individuals, and women who are concerned about age-related infertility. Little attention has been paid to fertility preservation for patients with differences/disorders of sex development (DSD). Our goal in this article is to address specific ethical considerations that are unique to this patient population. To this end, we present a hypothetical DSD case. We then explore ethical considerations related to patient's age, risk of cancer, concern about genetic transmission of a DSD condition to children, co-occurring gender dysphoria, and access to experimental fertility preservation procedures. Given the limitations of current technologies, we recommend offering fertility preservation to individuals living with DSD using an informed decision-making approach that instills realistic expectations and minimizes the potential for false hope. Finally, we conclude with practical recommendations for this case based on the ethical considerations.
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Preservación de la Fertilidad , Neoplasias , Personas Transgénero , Adolescente , Niño , Toma de Decisiones , Femenino , Preservación de la Fertilidad/ética , Humanos , Neoplasias/terapiaRESUMEN
PURPOSE: While serial renal ultrasound is often used as an alternative to functional renal imaging in children followed for hydronephrosis, it is unclear whether a lack of hydronephrosis progression safeguards against loss of renal function. In this study we characterize the association between findings on serial renal ultrasound and diuretic renography in children with severe unilateral hydronephrosis. MATERIALS AND METHODS: We retrospectively reviewed imaging among patients younger than 18 years old with a history of severe unilateral hydronephrosis, 2 renal ultrasounds and 2 diuretic renograms. Each pair of renal ultrasounds was interpreted by an independent blinded diagnostic radiologist and compared to a contemporaneous diuretic renogram. Change in hydronephrosis was considered as 1) a change in hydronephrosis grade or 2) any change by radiologist interpretation. A 5% or greater change in split differential function was considered significant. Chi-square and Spearman correlation analyses were performed. RESULTS: A total of 85 children were evaluated. Increased hydronephrosis was noted in 11.8% of children by grade and 32.9% by radiologist interpretation. Split differential renal function worsened by 5% or more in 17.6% of children. Overall, 13.3% of children with stable or decreased hydronephrosis demonstrated worsening split differential function at an average of 11.8 months. When renal ultrasound and diuretic renograms were directly compared, the Spearman correlation was poor (r = 0.24, 95% CI 0.03 to 0.43). CONCLUSIONS: The overall correlation between imaging modalities was poor, and 13.3% of children with stable or decreased hydronephrosis had worsening of split differential renal function. These findings are important to consider when counseling nonoperatively managed children followed without diuretic renography.
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Hidronefrosis/diagnóstico , Pelvis Renal/diagnóstico por imagen , Adolescente , Niño , Preescolar , Progresión de la Enfermedad , Diuréticos/administración & dosificación , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Masculino , Renografía por Radioisótopo/métodos , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Ultrasonografía/métodosRESUMEN
PURPOSE: We sought to determine the presence of germ cells in the gonads of patients with disorders of sex development to establish whether preservation of germ cells for future fertility potential is possible. We hypothesized that germ cells are present but vary by age and diagnosis. MATERIALS AND METHODS: We reviewed histology from patients with disorders of sex development who underwent gonadectomy/biopsy from 2002 to 2014 at a single institution for pathological classification of the gonad, composition of gonadal stroma and germ cell presence. RESULTS: A total of 44 patients were identified and germ cells were present in 68%. The presence and average number of germ cells per mm2 were analyzed by gonad type and diagnosis. By gonad type all ovotestes, most testes, ovaries and dysgenetic testes, and 15% of streak gonads had germ cells present. By diagnosis germ cells were present in all patients with complete androgen insensitivity syndrome, Denys-Drash syndrome, SRY mutation, mixed gonadal dysgenesis, ovotesticular conditions and StAR (steroid acute regulatory protein) deficiency, in some patients with persistent müllerian duct syndrome, XO/XY Turner syndrome and disorders of sex development not otherwise specified, and in none with complete or partial gonadal dysgenesis. Germ cells were present in the gonads of 88% of patients 0 to 3 years old, 50% of those 4 to 11 years old and 43% of those older than 12 years. CONCLUSIONS: Germ cells were present in the majority of our cohort and the presence decreased with age. This novel, fertility driven evaluation of germ cell quantity in a variety of disorders of sex development suggests that fertility potential may be greater than previously thought. Further studies must be done to evaluate a larger population and examine germ cell quality to determine the viability of these germ cells.
Asunto(s)
Trastornos del Desarrollo Sexual/complicaciones , Preservación de la Fertilidad , Células Germinativas , Infertilidad/etiología , Ovario/citología , Testículo/citología , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , MasculinoRESUMEN
PURPOSE: This review provides an overview of pediatric fertility preservation. Topics covered include the patient populations who could benefit, the current state of fertility preservation options and research, and considerations related to ethics and program development. MATERIALS AND METHODS: A broad Embase® and PubMed® search was performed to identify publications discussing investigational, clinical, ethical and health care delivery issues related to pediatric fertility preservation. Relevant publications were reviewed and summarized. RESULTS: Populations who could benefit from fertility preservation in childhood/adolescence include oncology patients, patients with nononcologic conditions requiring gonadotoxic chemotherapy, patients with differences/disorders of sex development and transgender individuals. Peripubertal and postpubertal fertility preservation options are well established and include cryopreservation of oocytes, embryos or sperm. Prepubertal fertility preservation is experimental. Multiple lines of active research aim to develop technologies that will enable immature eggs and sperm to be matured and used to produce a biological child in the future. Ethical challenges include the need for parental proxy decision making and the fact that fertility preservation procedures can be considered not medically necessary. Successful multidisciplinary fertility preservation care teams emphasize partnerships with adult colleagues, prioritize timely consultations and use standardized referral processes. Some aspects of fertility preservation are not covered by insurance and out-of-pocket costs can be prohibitive. CONCLUSIONS: Pediatric fertility preservation is an emerging, evolving field. Fertility preservation options for prepubertal patients with fertility altering conditions such as cancer and differences/disorders of sex development are currently limited. However, multiple lines of active research hold promise for the future. Key considerations include establishing a multidisciplinary team to provide pediatric fertility preservation services, an appreciation for relevant ethical issues and cost.
Asunto(s)
Preservación de la Fertilidad/tendencias , Niño , Femenino , Humanos , MasculinoRESUMEN
PURPOSE: Children with bladder dysfunction resulting in increased storage pressure are at risk for renal deterioration. The current gold standard for evaluation of bladder pressure is urodynamics, an invasive test requiring catheterization. We evaluated ultrasound shear wave elastography as a novel means of assessing bladder biomechanical properties associated with increased bladder pressure. MATERIALS AND METHODS: Concurrent shear wave elastography and urodynamics were performed. Ultrasound shear wave elastography images were obtained of the anterior and posterior wall when empty and at 25%, 50%, 75% and 100% expected bladder capacity, and end fill volume. Regions of interest were confirmed by a pediatric radiologist. Bladder cohorts were defined as compliant (capacity detrusor pressure less than 25 cm H2O) and noncompliant (25 cm H2O or greater). Pearson correlation coefficients and a mixed effects model evaluated the relationship between shear wave speed and detrusor pressure, compliance and normalized compliance. An unpaired t-test was used for between cohort analyses. RESULTS: In all 23 subjects mean shear wave speed of the anterior and posterior bladder walls significantly correlated with detrusor pressure throughout filling. When comparing compliant and noncompliant bladders, mean shear wave speed and detrusor shear wave speed of the anterior wall significantly increased with filling of noncompliant bladders. Shear wave speed remained at baseline levels in compliant bladders. Mean shear wave speed of the anterior wall was significantly correlated with compliance and normalized compliance. CONCLUSIONS: Ultrasound shear wave elastography bladder measurements correlate well with bladder storage pressure, and shear wave speed measurements differ between compliant and noncompliant bladders. This is the first known study to demonstrate that shear wave elastography is promising as a bedside modality for the assessment of bladder dysfunction in children.