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PURPOSE: To evaluate whether X, formerly known as Twitter, is being used effectively to advance the goals of International Volunteers in Urology (IVUmed). How is X activity associated with end-user engagement? METHODS: Monthly analytics of the X account @IVUmed were reviewed between September 2014 and November 2022 using https://analytics.twitter.com/ . Outcomes included tweets, mentions, impressions, engagements, interactions, followers, and profile visits. Statistical analysis using Mann-Whitney U test and Spearman's rank-order correlation was performed. Top tweet content between December 2020 and November 2022 was also analyzed and assigned one of seven different categories: research, workshops, mission statement, educational materials, fundraising, individual spotlight, and other. RESULTS: Of @IVUmed's 1668 followers, 1334 (80.0%) were individuals. One thousand one hundred twenty-six (84.4%) individuals listed their locations with the majority (79.8%) residing in high-income countries. Tweet impressions have increased over time; they were significantly higher (p < 0.01) on average after the onset of COVID-19 in March 2020. From December 2020 to November 2022, new followers were positively correlated with tweet impressions (p < 0.01), total mentions (p < 0.01), and profile visits (p < 0.01). Profile visits were positively correlated with total tweets (p < 0.01). The content categories for monthly top tweets that proportionally garnered the most engagements were workshops (50%) and individual spotlight (29%), despite not being the most tweeted about content categories. CONCLUSION: Non-profit organizations wishing to increase their web-based outreach can benefit from increased primary X activity. While not evaluated in this study, it may also improve fundraising capabilities. Nevertheless, periodic review of account activity is important to ensure engagement of the targeted audience.
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Medios de Comunicación Sociales , Urología , Humanos , Salud Global , MercadotecníaRESUMEN
PURPOSE: Pediatric testicular torsion is a urological emergency that requires timely intervention. In 2015, quality metrics for testicular torsion were implemented in the U.S. News & World Report "Best Children's Hospitals" rankings. Our study examines and compares testicular salvage surgery rates before and after the institution of national quality metrics from a multi-institutional database. MATERIALS AND METHODS: The Pediatric Health Information System® was surveyed for all testicular torsion encounters using ICD (International Classification of Diseases), Ninth and Tenth Revisions coding from 52 hospitals between January 2010 and December 2019. Patients <1 year and ≥18 years of age were excluded. Only hospitals that reported outcomes before and after quality scoring were included. Testicular salvage surgery was defined as patients having undergone orchiopexy without concomitant orchiectomy. Age, race, distance from hospital, household income and insurance status were compared. RESULTS: A total of 890 patients (551 pre-metric and 339 post-metric) from 38 hospitals were included. The testicular salvage surgery rate was 12.5% higher in the post-metric cohort (70.9% versus 58.4%). Hospital compliance to testicular torsion quality metrics increased from 62% in 2015 to 98% in 2019. Mean age, race, distance to hospital, household income, insurance status and use of ultrasound were not statistically different between pre- and post-metric cohorts. CONCLUSIONS: Since the implementation of quality metrics, salvage surgery rates for testicular torsion increased to 70.9%, an improvement of 12.5% when compared to pre-metric outcomes. Patient factors were similar between the 2 groups. Multi-institutional prospective data are needed to validate this database study and evaluate overall testicular salvage rate.
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Torsión del Cordón Espermático , Niño , Humanos , Masculino , Orquiectomía , Orquidopexia , Estudios Prospectivos , Estudios Retrospectivos , Torsión del Cordón Espermático/cirugíaRESUMEN
INTRODUCTION: Skin entrance doses for voiding cystourethrogram (VCUG) have not been well characterized in the literature. Radiation exposure is measured as either dose area product (DAP) or air kerma, which estimates the effective dose, but does not accurately reflect absorbed dose at skin level. OBJECTIVE: The objective of this study was to measure the skin entrance dose during fluoroscopic VCUG study in pediatric patients using single point dosimeters. STUDY DESIGN: Pediatric patients undergoing fluoroscopic VCUG were prospectively enrolled in our study. Landauer NanoDot™OSLD dosimeters were affixed to the skin overlying the sacrum to measure skin entrance dose. The fluoroscopic unit was set to the following parameters: low dose setting, skin-to-source distance of 54 cm, pulsed fluoroscopy at 3 frames/sec. RESULTS: Forty-four patients with a median age of 13.6 months (IQR 3.7-42.3) were enrolled. Median fluoroscopic time was 54 s (IQR 36-72). The median values absorbed dose by dosimeter and air kerma were 0.32 mGy (IQR 0.13-0.56, range 0.01-2.9) and 0.24 mGy (IQR 0.14-0.37), respectively. There was a positive correlation between the air kerma and absorbed dose (r = 0.69, p < 0.001) and fluoroscopy time and absorbed dose mGy (r = 0.60, p < 0.001). Absorbed dose was independent of age, body mass index and body surface area (p = 0.19, p = 0.57 and p = 0.16, respectively). Median whole body effective dose was 0.04 mSv (IQR 0.02-0.7). DISCUSSION: Overall, the absorbed dose received by the dosimeter remained low at a median of 0.32 mGy (range 0.01-2.91). These values are remarkably low and well within the accepted radiation exposure norms. Our radiologists follow a strict protocol to reduce the overall radiation emitted during a VCUG. These measures include setting the x-ray source at a low dose mode, collimating to the smallest area possible, and using pulsed fluoroscopy at 3 frames/sec. Limitations to this study include a slight variability in technique of VCUG between different technicians and providers, for which we cannot control. CONCLUSION: The radiation dose absorbed at the skin entrance and uniform whole body effective dose is low for a single VCUG when utilizing strict parameters. While newer non-ionizing technologies continue to emerge, our data is encouraging and will serve as a valuable tool when educating parents regarding radiation doses associated with a VCUG.
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Cistografía , Exposición a la Radiación , Niño , Preescolar , Fluoroscopía , Humanos , Lactante , Dosis de Radiación , RadiografíaRESUMEN
INTRODUCTION: We describe the effect of a state mandated opioid consent on opioid utilization and multimodal pain use for major pediatric genitourinary surgeries. METHODS: All patients who underwent an inpatient pediatric genitourinary surgery at the Children's Hospital of Pittsburgh between August 2015 and February 2020 were identified. Inpatient and outpatient multimodal pain control utilization were assessed. Delayed prescriptions or emergency department visits within 30 days were identified and when applicable referenced against National Surgical Quality Improvement Program data. RESULTS: After accounting for a 6-month transition period following policy implementation, 462 major pediatric genitourinary procedures were identified. The frequency of discharge opiate prescriptions decreased from 68.4% prior to the transition period to 10.7% afterward(p<0.001). Morphine milligram equivalents per prescription decreased from 75(IQR 45-150) to 45(IQR 22.5-75)(p<0.001). The rate of delayed non-opioid analgesic prescriptions (6.6% vs 7.4%), delayed opioid prescriptions (1.5% vs 0.3%), or emergency department visits (11.8% vs 12.6%)(p = 0.809) remained unchanged. Data agreed with National Surgical Quality Improvement Program data in 98.5% of cases. After excluding one surgeon who departed after the transition period, regional block utilization did not change from 61/115(53%) to 147/320(45.9%)(p = 0.425). CONCLUSIONS: A state mandated opioid consent safely reduced opioid utilization for most major pediatric genitourinary surgeries. LEVEL OF EVIDENCE: Level III.
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Analgésicos Opioides , Dolor Postoperatorio , Analgésicos Opioides/uso terapéutico , Niño , Gobierno , Humanos , Consentimiento Informado , Manejo del Dolor , Dolor Postoperatorio/tratamiento farmacológico , Pautas de la Práctica en MedicinaRESUMEN
PURPOSE: Postoperative opioids are overprescribed in the United States. In November 2016 the State of Pennsylvania required an opioid consent for minors. Our hypothesis is that this mandate decreased postoperative opioid prescriptions in our division. MATERIALS AND METHODS: All patients who received a urological outpatient or minor emergency procedure from August 2015 to August 2019 were identified. Surgeries performed within 6 months after mandate implementation were excluded to account for the transition period. Perioperative data including case type were extracted by a clinical data warehouse from preexisting fields within the health record. The frequencies of postoperative prescriptions, delayed prescriptions and emergency department encounters were assessed. A multivariable logistic regression to identify predictors of opioid prescription at discharge was performed. RESULTS: A total of 4,349 patients were analyzed. The frequency of postsurgical opioid prescriptions decreased from 45.3% to 2.6% (p <0.001). The median morphine milligram equivalent decreased by 22.5 among children prescribed an opioid (p <0.001). Rates of an emergency department visits (3% vs 2.7%) or delayed nonopioid prescriptions (0.8% vs 1.2%) within 30 days of discharge were unchanged (p >0.05). Fewer patients received a delayed opioid prescription after mandate implementation (0.03% vs 0.5%, p <0.001). Female patients were less likely (OR 0.309, 95% CI 0.195-0.491; p <0.001) to receive opioids prior to but not after the mandate (OR 0.309, 95% CI 0.544-2.035; p=0.122). Increasing age was predictive of receiving an opioid before (OR 1.187, 95% CI 1.157-1.218; p <0.001) and after (OR 1.241, 95% CI 1.186-1.299; p <0.001) the mandate. CONCLUSIONS: A state mandated opioid consent for minors greatly reduced post-urological surgery opioid prescription rates without increasing rates of readmission or delayed prescriptions.
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Analgésicos Opioides/efectos adversos , Consentimiento Informado/legislación & jurisprudencia , Manejo del Dolor/normas , Dolor Postoperatorio/tratamiento farmacológico , Pautas de la Práctica en Medicina/estadística & datos numéricos , Procedimientos Quirúrgicos Urológicos/efectos adversos , Procedimientos Quirúrgicos Ambulatorios/efectos adversos , Analgésicos Opioides/normas , Niño , Preescolar , Utilización de Medicamentos/legislación & jurisprudencia , Utilización de Medicamentos/estadística & datos numéricos , Servicio de Urgencia en Hospital/estadística & datos numéricos , Tratamiento de Urgencia/efectos adversos , Tratamiento de Urgencia/métodos , Femenino , Humanos , Lactante , Consentimiento Informado/normas , Masculino , Epidemia de Opioides/prevención & control , Manejo del Dolor/métodos , Dolor Postoperatorio/etiología , Readmisión del Paciente/estadística & datos numéricos , Pennsylvania , Pautas de la Práctica en Medicina/legislación & jurisprudencia , Pautas de la Práctica en Medicina/normas , Estudios Retrospectivos , Gobierno Estatal , Procedimientos Quirúrgicos Urológicos/métodos , Urólogos/legislación & jurisprudencia , Urólogos/normas , Urólogos/estadística & datos numéricosRESUMEN
BACKGROUND: Dismembered pyeloplasty is considered the gold standard treatment for ureteropelvic junction obstruction (UPJO). Although the frequency and timing of follow up imaging after pyeloplasty is variable, renal ultrasound (RUS) is commonly utilized. With minimal training, point-of-care ultrasound (POCUS) can be easily performed by a urologist during a post-operative visit. OBJECTIVE: Our hypothesis is that POCUS is an accurate, time-saving, and cost-effective alternative to a complete retroperitoneal ultrasound (CRUS) performed by the Radiology Department after pyeloplasty. STUDY DESIGN: The clinical records of all children who underwent pyeloplasty (by any method) over a 12 month period at our institution were retrospectively reviewed. The exact timing and method (POCUS vs. CRUS) of follow up imaging was surgeon-dependent. Statistical analysis was performed to compare the time and cost of POCUS vs. CRUS. The clinical course of each patient who had each type of imaging was assessed. RESULTS: A total 45 patients were included in this analysis. Over a mean follow up period of 29 months, a total of 73 CRUS and 67 POCUS were performed. Each CRUS on average added 2 h to each patient's healthcare encounter. Had the 73 CRUS been performed as POCUS instead, this would have corresponded to $83,751 less charges to payers. There was no difference in the rate of the detection of worsening, stable, or improved hydronephrosis (HN) between either modality (p > 0.05). The recommended follow up time for observed HN was no different between CRUS and POCUS (p > 0.05). Children with worsening HN on POCUS underwent functional studies without confirmatory CRUS. Interestingly, two patients had metachronous, contralateral UPJO discovered during post-operative imaging. These were both discovered by POCUS. Nineteen (42%) patients who had attended at least one post-operative visit were eventually loss to follow-up. This occurred exclusively in those who did not have worsening ultrasound (p < 0.01). There was no difference in the loss to follow-up after POCUS (8) or CRUS (12) (p > 0.05). CONCLUSIONS: POCUS performed by a urologist is an accurate assessment of HN after pyeloplasty with time and cost savings to compared to a CRUS performed by a radiologist. POCUS is not associated with any difference in rate of detection of worsening HN or rate of loss to follow up.
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Hidronefrosis , Laparoscopía , Obstrucción Ureteral , Niño , Análisis Costo-Beneficio , Humanos , Hidronefrosis/diagnóstico por imagen , Hidronefrosis/cirugía , Pelvis Renal/diagnóstico por imagen , Pelvis Renal/cirugía , Sistemas de Atención de Punto , Estudios Retrospectivos , Resultado del Tratamiento , Obstrucción Ureteral/diagnóstico por imagen , Obstrucción Ureteral/cirugía , Procedimientos Quirúrgicos UrológicosRESUMEN
Juvenile granulosa cell tumors of the ovary are rare sex cord-stromal ovarian tumors that are typically diagnosed during the first 2 decades of life. Most patients present with precocious puberty in the early stages of disease. We present a rare case of asymptomatic uterine torsion from a 15-cm juvenile granulosa cell tumors in a 5-year-old girl with elevated inhibin B, breast development, vaginal bleeding, and a palpable right-sided abdominal mass.
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Tumor de Células de la Granulosa/diagnóstico , Neoplasias Ováricas/diagnóstico , Anomalía Torsional/diagnóstico , Útero/diagnóstico por imagen , Preescolar , Diagnóstico Diferencial , Femenino , Tumor de Células de la Granulosa/complicaciones , Tumor de Células de la Granulosa/cirugía , Humanos , Neoplasias Ováricas/complicaciones , Neoplasias Ováricas/cirugía , Ovariectomía/métodos , Enfermedades Raras , Tomografía Computarizada por Rayos X , Anomalía Torsional/etiología , Anomalía Torsional/cirugíaRESUMEN
45,X/46,XY mosaicism is one of a heterogenous group of congenital conditions known as differences (disorders) of sex development (DSD) that results in abnormal development of internal and external genitalia. Patients with DSD, particularly those with segments of the Y chromosome, are at increased risk for germ cell tumors including gonadoblastoma. Gonadoblastoma is a neoplasm comprised of a mixture of germ cells and elements resembling immature granulosa or Sertoli cells with or without Leydig cells or lutein-type cells in an ovarian type stroma. Gonadoblastoma has an increased prevalence of 15% to 40% in patients with 45,X/46,XY mosaicism and has been previously reported in patients as young as 5 months of age with that karyotype. Herein, we describe a 3-month-old child with 45,X/46,XY karyotype who was referred for the evaluation of asymmetric labia majora. Additional evaluation revealed left streak gonad and right dysplastic/dysgenetic testis. Both gonads contained foci of cells typical for gonadoblastoma as well as undifferentiated gonadal tissue, underscoring the potential for very early infantile gonadoblastoma and the spectrum of developmental anomalies associated with this karyotype.
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Trastornos del Desarrollo Sexual/diagnóstico , Disgenesia Gonadal Mixta/diagnóstico , Gonadoblastoma/diagnóstico , Trastornos del Desarrollo Sexual/genética , Trastornos del Desarrollo Sexual/patología , Femenino , Disgenesia Gonadal Mixta/genética , Disgenesia Gonadal Mixta/patología , Gonadoblastoma/genética , Gonadoblastoma/patología , Humanos , Recién Nacido , Cariotipo , Masculino , MosaicismoRESUMEN
BACKGROUND: Phalluses present inside the extrophied bladder of cloacal exstrophy (CE) newborns have been sporadically reported in the literature; this clinical entity has largely unknown origins and may represent an extremely rare anomaly of CE. OBJECTIVE: Along with nearly doubling the number of reported intravesical phalluses in the literature, this study aims to outline the common anatomic features and discuss the implications for theories of CE embryogenesis. STUDY DESIGN: The authors retrospectively identified patients with CE and a potential intravesical phallus between 1997 and 2017 at two high-volume centers. Information was obtained about karyotype, age at closure, neurologic and renal anomalies, diastasis, phallus anatomy, and phallus biopsy pathology. RESULTS: Six genotypic males met the inclusion criteria. Five of six had a diastasis greater than 6 cm. Four of six had paired corporal bodies in the intravesical phallus, one had a single corporal body, and one had a corporal-like structure. Five of six patients had a phallus located midline in the caudal aspect of the bladder; one was located midline in the bladder dome. Phallic biopsies were obtained in three of six patients. Two showed glanular and corporal tissue while the other showed vascular proliferation morphologically similar to that of erectile tissue. DISCUSSION: Previous reports suggested that a superior vesicle fissure configuration, fusion of the corporal bodies, and fused bladder plates were common findings with an intravesical phallus. With the addition of new cases, the only consistent variable between patients is a phallus located anywhere along the bladder plate that can comprise a corporal-like structure, a single corporal body, or fused corporal bodies. These findings have implications for several embryologic theories. Although this is a retrospective review with a limited number of patients, the condition is exceedingly infrequent making it only observable retrospectively over decades at high volume centers. CONCLUSIONS: The study outlined common anatomic features of the intravesical phallus in cloacal exstrophy and discussed the subsequent embryologic implications. In cloacal exstrophy newborns with presumed aphallia, meticulous inspection of the bladder plate and biopsy of any potential phallic structures can prevent resection of phallic tissue.
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Anomalías Múltiples/embriología , Anomalías Múltiples/cirugía , Extrofia de la Vejiga/embriología , Extrofia de la Vejiga/cirugía , Pene/anomalías , Pene/cirugía , Vejiga Urinaria/anomalías , Vejiga Urinaria/cirugía , Extrofia de la Vejiga/complicaciones , Preescolar , Humanos , Lactante , Masculino , Pene/embriología , Estudios Retrospectivos , Vejiga Urinaria/embriología , Procedimientos Quirúrgicos Urológicos Masculinos/métodosRESUMEN
OBJECTIVE: To describe the phenotype, referral pattern, and trends of urologic management for children with megacystis microcolon intestinal hypoperistalsis syndrome. MATERIALS AND METHODS: We performed a retrospective review of all patients with megacystis microcolon intestinal hypoperistalsis syndrome treated at a large tertiary children's medical center over a 14-year period. Our primary outcomes included the rate of urology referral, urodynamic characteristics of bladder function, and the proportion of patients managed with clean intermittent catheterization. RESULTS: Twenty-six patients were evaluated at our institution with a median age at presentation of 1.3 years (73% female, 19 of 26). Most patients presented for intestinal transplantation evaluation and were dependent on parenteral nutrition. Megacystis was diagnosed prenatally in 15 patients and postnatally in 11 patients. Twenty patients (77%, 20 of 26) were evaluated by a pediatric urologist. Six patients (23%, 6 of 26) had varying degrees of vesicoureteral reflux. Early bladder management consisted of clean intermittent catheterization in 13 patients and vesicostomy in 8 patients. CONCLUSION: Megacystis microcolon intestinal hypoperistalsis syndrome is a severe form of intestinal failure and bladder atony with substantial urologic morbidity. Parents should be counseled accordingly and prompt urologic consultation should be included in the multidisciplinary approach to these complex patients.
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Anomalías Múltiples/cirugía , Colon/anomalías , Seudoobstrucción Intestinal/cirugía , Vejiga Urinaria/anomalías , Anomalías Múltiples/genética , Colon/cirugía , Femenino , Hospitales Pediátricos , Humanos , Lactante , Seudoobstrucción Intestinal/genética , Intestinos/trasplante , Masculino , Fenotipo , Estudios Retrospectivos , Vejiga Urinaria/cirugíaRESUMEN
Capillary hemangioma is a rare benign lesion in the testicle, particularly in pediatrics. It can mimic malignancy, leading to radical orchiectomy. We present a case of a testicular hemangioma in a child, and review the literature on testicular hemangiomas in this age group. A hypervascular testicular lesion without elevated tumor markers may warrant intraoperative biopsy to direct surgical management, which may include testis-sparing surgery if amenable.
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Hemangioma/cirugía , Neoplasias Testiculares/cirugía , Niño , Hemangioma/diagnóstico por imagen , Hemangioma/patología , Humanos , Inmunohistoquímica , Masculino , Orquiectomía , Neoplasias Testiculares/diagnóstico por imagen , Neoplasias Testiculares/patología , UltrasonografíaRESUMEN
OBJECTIVE: To elucidate the trends in urologic causes for renal transplant in the pediatric population using a national database. Little is known about the specific pediatric urologic conditions and resultant trends that precede renal transplantation. MATERIAL AND METHODS: We reviewed the United Network for Organ Sharing (UNOS) database for pediatric patients (<18 years old) who underwent renal transplantation from January 1988 to September 2015. We included those patients who received a renal transplant because of a urologic condition. RESULTS: Over 27 years, 7291 of 20,213 children (36%) underwent renal transplant secondary to a urologic condition. The 2 most common indications were hypoplasia/dysplasia/dysgenesis/agenesis (HDDA, 35.1%) and congenital obstructive uropathy (COU, 25.7%). The incidence of COU has increased from 18% to 30%, and the incidence of Wilms tumor has remained relatively consistent at 1.8% per year. In addition, 68% of all urologic renal transplants were performed in men compared with women. However, a higher percentage of women required transplantation because of Wilms tumor (3.1% vs 1.2%) and chronic pyelonephritis (17.6% vs 7.9%). Overall, the majority of patients (61.5%) who underwent renal transplantation were white, 18.8% Hispanic, and 15.3% black. CONCLUSION: HDDA and COU consistently have been the most common urologic indications for renal transplantation. Both are the leading causes in men, whereas HDDA and chronic pyelonephritis are predominant in women. Higher rate of renal transplant during the ages of 11-17 years is suggestive of increased burden on poorly functioning kidneys during times of adolescent growth.
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Enfermedades Renales/cirugía , Trasplante de Riñón , Adolescente , Niño , Preescolar , Bases de Datos Factuales , Femenino , Humanos , Lactante , Trasplante de Riñón/estadística & datos numéricos , Masculino , Factores de Tiempo , Obtención de Tejidos y Órganos , Estados UnidosRESUMEN
OBJECTIVES: To identify the rate at which testicular torsion occurs in a lateral direction and identify any predictors of direction of testicular rotation and orchiectomy. MATERIALS AND METHODS: We performed a retrospective review of 104 cases of emergent scrotal exploration performed for testicular torsion by 3 pediatric urologists from 2003 to 2017. Patients with neonatal torsion, negative scrotal exploration, or exploration for presumed intermittent testicular torsion were excluded. Univariable logistic regression was performed to assess if any factors predicted direction of testicular rotation. Univariable and multivariable logistic regression was used to identify predictors of testicular salvage. RESULTS: Of 104 cases of acute testicular torsion, information regarding the direction of testicular rotation was available in 81 patients. Lateral testicular rotation occurred in 38 cases (46%). No factors were found to be predictive of direction of torsion. Orchiectomy for testicular nonviability was performed in 50 of 104 cases (48%). On univariable analysis, younger age (p = .002), absence of gastrointestinal symptoms (P = .02), time to exploration (P < .001), testicular size differential on ultrasound (p = .002), absence of hydrocele (P = .01), abnormal ultrasound echotexture (P < .001), and degree of torsion (P = .04) were associated with orchiectomy. With the exception of absence of gastrointestinal symptoms, all predictors of orchiectomy remained statistically significant on multivariable analysis. CONCLUSION: Testicular rotation occurs in a lateral direction in 46% of cases. Lateral manual detorsion should be performed only with awareness of the potential for increasing the degree of testicular rotation. New independent predictors of testicular salvage were identified.
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Orquiectomía , Tratamientos Conservadores del Órgano , Rotación , Torsión del Cordón Espermático/cirugía , Adolescente , Niño , Humanos , Masculino , Estudios Retrospectivos , Factores de Riesgo , Torsión del Cordón Espermático/patologíaRESUMEN
INTRODUCTION: Mechanical bowel preparation (MBP) has historically been the standard of care for patients undergoing reconstructive urologic surgery, including urinary diversion. To date, several studies have examined the role of mechanical bowel preparation in postoperative outcomes in pediatric patients undergoing augmentation cystoplasty. However, these patient populations have been heterogeneous in nature, with no studies dedicated to examining the role of MBP prior to reconstructive urologic surgery in pediatric patients with myelomenginoceles. Thus, our objective was to retrospectively assess perioperative measures and postoperative complications after reconstructive urologic surgery with or without mechanical bowel preparation in pediatric myelomeningocele patients. MATERIALS AND METHODS: From 2008 to 2013, 80 patients with myelomeningocele underwent reconstructive urologic surgery involving the use of bowel. Seventy patients underwent a preoperative MBP while 10 did not. Perioperative measures and postoperative complications for these two cohorts were assessed. RESULTS: Eighty patients with myelomeningocele were identified; 70 patients underwent MBP while 10 patients did not. There were no statistically significant differences in demographics or operative time. There were no statistically significant differences in postoperative outcomes including time to first bowel movement and time to tolerating diet. There was also no significant difference in overall complication rate; patients with MBP had 31/70 (44%) complications while 2/10 (20%) of those without MBP had complications (p = 0.18). CONCLUSION: There was no significant difference in perioperative measures and postoperative complications for patients who did not receive a mechanical bowel preparation. Our findings indicate that it is safe and warranted to perform a prospective, randomized study to better characterize the risks and benefits of preoperative bowel preparation for patients with myelomeningocele.
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Cuidados Preoperatorios/métodos , Vejiga Urinaria Neurogénica/cirugía , Procedimientos Quirúrgicos Urológicos/métodos , Niño , Humanos , Intestinos , Meningomielocele/complicaciones , Estudios Retrospectivos , Vejiga Urinaria Neurogénica/etiologíaRESUMEN
OBJECTIVES: To assess the optimal length of follow-up for patients undergoing both open and minimally invasive pyeloplasties to ensure prompt detection of a recurrent obstruction. There are no standard guidelines on ideal follow-up and imaging post-pediatric pyeloplasty currently. METHODS: A retrospective chart review identified 264 patients (<18 years old) who underwent pyeloplasty for ureteropelvic junction obstruction between April 2002 and December 2014. Ultrasound was obtained every 3-4 months for the first year following pyeloplasty and thereafter at discretion of treating physician. Patient characteristics including symptoms and imaging were reviewed. RESULTS: Of the 264 patients, 72% were male with mean age of 51 months and follow-up of 26.8 months. Approximately 73% followed up to 3 years. Fourteen patients (5.3%) had a recurrent obstruction. Among the failures, 85% were diagnosed and underwent successful redo pyeloplasty within 3 years. Six infants had a recurrence (43% of all unsuccessful surgeries) and were diagnosed within 3 years of the initial surgery. Patients undergoing a minimally invasive procedure were less likely to be followed for more than 3 years compared to an open procedure (p < 0.001). Patients with severe hydronephrosis preoperatively were followed longer (p = 0.031). Age at surgery and type of surgical approach (p < 0.01) were significant predictors of length of follow-up in a negative binomial regression. CONCLUSION: Based on the results, a minimum of 3 years of follow-up is necessary to detect the majority of recurrent obstructions. Those patients who have higher than average lengths of follow-up tend to be younger and/or underwent an open surgical approach.
RESUMEN
BACKGROUND: The XX male disorder of sex development (DSD) is a rare condition that is most commonly associated with the presence of the SRY gene on one of the X chromosomes due to unequal crossing-over between sex chromosomes during spermatogenesis. However, in about 20% of the XX male individuals, SRY is missing, although these persons have at least some testis differentiation. The genetic basis of genital ambiguity and the mechanisms triggering testis development in such patients remain unknown. METHODS: The proband with 46,XX SRY-negative testicular DSD was screened for point mutations by whole exome sequencing and CNVs using a high-resolution DSD gene-targeted and whole genome array comparative genomic hybridisation. The identified Xp21.2 genomic alteration was further characterised by direct sequencing of the breakpoint junctions and bioinformatics analysis. RESULTS: A unique, 80 kb microdeletion removing the regulatory sequences and the NR0B1 gene was detected by microarray analysis. This deletion disturbs the human-specific genomic architecture of the Xp21.2 dosage-sensitive sex (DSS) reversal region in the XX patient with male-appearing ambiguous genitalia and ovotestis. CONCLUSIONS: Duplication of the DSS region containing the MAGEB and NR0B1 genes has been implicated in testis repression and sex reversal. Identification of this microdeletion highlights the importance of genomic integrity in the regulation and interaction of sex determining genes during gonadal development.
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Trastornos Testiculares del Desarrollo Sexual 46, XX/genética , Cromosomas Humanos X/genética , Trastornos Ovotesticulares del Desarrollo Sexual/genética , Eliminación de Secuencia , Trastornos Testiculares del Desarrollo Sexual 46, XX/patología , Preescolar , Hibridación Genómica Comparativa , Receptor Nuclear Huérfano DAX-1/genética , Variaciones en el Número de Copia de ADN , Femenino , Disgenesia Gonadal/genética , Humanos , Masculino , Ovario/patología , Trastornos Ovotesticulares del Desarrollo Sexual/patología , Secuencias Reguladoras de Ácidos Nucleicos , Testículo/patologíaRESUMEN
Phallic reconstruction is an important part of management for congenital aphallia. Scrotal flap phalloplasty has been described in pediatric patients to create the appearance of a nonfunctioning phallus. We describe a modified scrotal flap technique with the addition of an acellular dermal matrix patch (AlloDerm) to provide additional girth and support to the phallus. The postoperative cosmetic outcome has been satisfactory and there is no documented complication over a 12-month follow-up. AlloDerm dermal matrix can be a safe addition to phallic reconstruction and its overall application needs to be further studied.