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Appropriate coordination of smooth muscle contraction and relaxation is essential for normal colonic motility. The impact of perturbed motility ranges from moderate, in conditions such as colitis, to potentially fatal in the case of pseudo-obstruction. The mechanisms underlying aberrant motility and the extent to which they can be targeted pharmacologically are incompletely understood. This study identified colonic smooth muscle as a major site of expression of neuropilin 2 (Nrp2) in mice and humans. Mice with inducible smooth muscle-specific knockout of Nrp2 had an increase in evoked contraction of colonic rings in response to carbachol at 1 and 4 weeks following initiation of deletion. KCl-induced contractions were also increased at 4 weeks. Colonic motility was similarly enhanced, as evidenced by faster bead expulsion in Nrp2-deleted mice versus Nrp2-intact controls. In length-tension analysis of the distal colon, passive tension was similar in Nrp2-deficient and Nrp2-intact mice, but at low strains, active stiffness was greater in Nrp2-deficient animals. Consistent with the findings in conditional Nrp2 mice, Nrp2-null mice showed increased contractility in response to carbachol and KCl. Evaluation of selected proteins implicated in smooth muscle contraction revealed no significant differences in the level of α-smooth muscle actin, myosin light chain, calponin, or RhoA. Together, these findings identify Nrp2 as a novel regulator of colonic contractility that may be targetable in conditions characterized by dysmotility.
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Colo , Motilidade Gastrointestinal , Contração Muscular , Músculo Liso , Neuropilina-2 , Animais , Humanos , Camundongos , Carbacol/farmacologia , Colo/metabolismo , Colo/fisiologia , Camundongos Knockout , Contração Muscular/efeitos dos fármacos , Contração Muscular/genética , Músculo Liso/efeitos dos fármacos , Músculo Liso/metabolismo , Neuropilina-2/genética , Neuropilina-2/metabolismo , Motilidade Gastrointestinal/efeitos dos fármacos , Motilidade Gastrointestinal/genéticaRESUMO
Congenital genitourinary anomalies are among the most frequent types of birth defects in neonates. Some anomalies can be a significant cause of morbidity in infancy, while others remain asymptomatic even until adulthood and can be at times the only manifestation of a complex systemic disease. The spectrum of these anomalies results from the developmental insults that can occur at various embryologic stages, and an understanding of the formation of the genitourinary system is helpful in the evaluation and treatment of a child with a congenital genitourinary anomaly. Imaging plays an essential role in the diagnosis of congenital genitourinary anomalies and treatment planning. In this article, we highlight the embryologic and characteristic imaging features of various congenital genitourinary anomalies, demonstrate the utility of different imaging modalities in management, and review specific imaging modalities and protocols for image optimization.
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Anormalidades Urogenitais , Adulto , Criança , Diagnóstico por Imagem , Feminino , Humanos , Recém-Nascido , Imageamento por Ressonância Magnética , Masculino , Anormalidades Urogenitais/diagnóstico por imagem , Sistema Urogenital/diagnóstico por imagemRESUMO
PURPOSE: To compare the prevalence of vesicoureteral reflux (VUR), febrile urinary tract infection (fUTI), and chronic kidney disease (CKD) among patients with multicystic dysplastic kidney (MCDK) diagnosed by renal scintigraphy (RS) versus follow-up renal ultrasound (RUS) alone. METHODS: This was a retrospective review of patients seen at a tertiary care center from 2010 to 2020 with MCDK diagnosed by RS or follow-up RUS. Differences in the prevalence of VUR, fUTI, and CKD by cohort were assessed using logistic regression analysis, Pearson X2 , and Fisher's Exact tests. Temporal trends in diagnostic methods used (RUS versus RUS + RS) were evaluated using the Cochran-Armitage trend test. RESULTS: One-hundred seventy-two patients were included: 50% (n = 86) underwent RUS + RS and 50% (n = 86) underwent RUS alone to diagnose MCDK. Prevalence of VUR, fUTI, and CKD did not significantly vary between groups. Among patients who had a VCUG, 4.4% had contralateral VUR (1.7% RUS + RS group; 7.4% RUS group; p = 0.19) and 14.5% had at least one fUTI (16.3% RUS + RS group; 12.8% RUS group; p = 0.52). Females were significantly more likely to have at least one fUTI (p = 0.04). Four patients (2.3%) developed CKD, all in the RUS + RS cohort (p = 0.12). Diagnosis of MCDK by RUS versus RUS + RS did not significantly vary over time (p = 0.17). CONCLUSION: Patients with unilateral MCDK confirmed by RS versus RUS alone do not significantly vary in the prevalence of VUR, fUTI, or CKD. Renal scintigraphy studies may not be necessary in unilateral MCDK diagnosis but continue to be used.
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Rim Displásico Multicístico , Insuficiência Renal Crônica , Infecções Urinárias , Refluxo Vesicoureteral , Feminino , Humanos , Lactente , Rim/diagnóstico por imagem , Rim Displásico Multicístico/diagnóstico por imagem , Rim Displásico Multicístico/epidemiologia , Cintilografia , Insuficiência Renal Crônica/epidemiologia , Estudos Retrospectivos , Infecções Urinárias/diagnóstico por imagem , Infecções Urinárias/epidemiologia , Refluxo Vesicoureteral/diagnóstico por imagem , Refluxo Vesicoureteral/epidemiologiaRESUMO
BACKGROUND: Hydronephrosis (HN) is the most common abnormality detected on prenatal ultrasound. This study sought to stratify outcomes of patients by severity of prenatal HN with postnatal outcomes. METHODS: This was a retrospective review of patients referred to a tertiary care fetal-maternal clinic with diagnosis of prenatal HN from 2004 to 2019. HN severity was categorized as mild, moderate, or severe. Data were analyzed to determine the association between HN severity and surgical intervention. Decision for surgery was based on factors including history of multiple urinary tract infections, evidence of renal scarring, and/or reduced renal function. Surgery-free survival time was represented by the Kaplan-Meier method, and hazard ratios were calculated using the log-rank test. RESULTS: 131 kidneys among 101 infants were prenatally diagnosed with hydronephrosis; 35.9% had mild HN, 29.0% had moderate HN, and 35.1% had severe HN. 8.5% of patients with mild HN, 26.3% of patients with moderate HN, and 65.2% of patients with severe HN required surgery. Patients with severe HN were 12.2 (95% CI 6.1-24.4; p < 0.001) times more likely to undergo surgery for HN than patients with mild HN and 2.9 (95% CI 1.5-5.3; p = 0.003) times more likely to undergo surgery than patients with moderate HN. Patients with moderate HN were 4.3 times more likely to require surgery than patients with mild HN (95% CI 1.5-12.9; p = 0.01). Median age at surgery was 11.8 months among patients with mild HN (IQR 11.7-14.1 months), 6.6 months among patients with moderate HN (IQR 4.2-16.4 months), and 5.4 months among patients with severe HN (3.7-12.4 months). CONCLUSION: Among this cohort of referrals from a fetal-maternal clinic, severity of HN correlated with increased likelihood of surgical intervention. Continued assessment of patients with prenatal HN should be evaluated to best determine the role of the pediatric urologist in cases of prenatal HN.
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Hidronefrose/diagnóstico por imagem , Hidronefrose/cirurgia , Ultrassonografia Pré-Natal , Feminino , Humanos , Recém-Nascido , Masculino , Gravidez , Cuidado Pré-Natal , Estudos Retrospectivos , Índice de Gravidade de Doença , Centros de Atenção Terciária , Resultado do TratamentoRESUMO
BACKGROUND: Laparoscopic resection is the most well described minimally-invasive approach for adrenalectomy. While it allows for improved cosmesis, faster recovery and decreased length of hospital stay compared with the open approach, instrument articulation limitations can hamper surgical dexterity in pediatric patients. Use of robotic assistance can greatly enhance operative field visualization and instrument control, and is in the early stages of adoption in academic centers for pediatric populations. CASE PRESENTATION: We present a single-institution series of pediatric adrenalectomy cases. The da Vinci Xi surgical system was used to perform adrenalectomies on three consecutive patients (ages, 2-13 years) at our center. Final pathology revealed ganglioneuroblastoma (n = 2) and pheochromocytoma (n = 1). Median operating time was 244 min (range, 244-265 min); median blood loss was estimated at 100 ml (range, 15-175 ml). Specimens were delivered intact and all margins were negative. Median post-operative hospital stay was 2 days (range, 1-6 days). All patients remain disease-free at median follow-up of 19 months (range, 12-30 months). CONCLUSION: Our experience continues to evolve, and suggests that robotic surgery is safe, feasible and oncologically effective for resection of adrenal masses in well-selected pediatric patients.
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Neoplasias das Glândulas Suprarrenais/cirurgia , Adrenalectomia/métodos , Procedimentos Cirúrgicos Robóticos , Adolescente , Pré-Escolar , Feminino , Humanos , Masculino , Centros de Atenção TerciáriaRESUMO
PURPOSE: For bladder exstrophy repair it is universally accepted that successful initial surgery is paramount to achieve the optimal outcome. Gaining the necessary surgical experience is challenging due to the rarity of bladder exstrophy. We report preliminary findings of a multi-institutional collaboration created to increase experience and proficiency with the care of bladder exstrophy. MATERIALS AND METHODS: Our 3 institutions alternatively served as the host site for scheduled surgeries with observation, commentary and critique by visitors from the other sites. The technique was complete primary repair with bilateral iliac osteotomy. The timing of complete primary repair at age 1 to 3 months facilitated collaboration. We recorded patient demographics and outcomes, and the impact of this collaboration on our technique and experience. Video recording was used for real-time observation and teaching, and future analysis, editing and review. RESULTS: A total of 16 site visits occurred from February 2013 through May 2014. Complete primary repair was performed in 9 males and 7 females with bladder exstrophy. Median age at complete primary repair was 2 months (range 0.1 to 28.8). Median followup was 8.9 months (range 2.8 to 18.2). All closures were successful with no dehiscence. Complications included urethrocutaneous fistula in 2 patients, 1 episode of pyelonephritis in 3 each and urethral obstruction in 2 females, of whom 1 required clean intermittent catheterization. CONCLUSIONS: We report a multi-institutional collaboration to standardize the surgical management of bladder exstrophy. This effort increased the annual experience of each institution involved from threefold to ninefold and it has accelerated the physician knowledge base to ultimately benefit patient care.
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Extrofia Vesical/cirurgia , Procedimentos Cirúrgicos Urogenitais/métodos , Adolescente , Adulto , Criança , Pré-Escolar , Competência Clínica , Comportamento Cooperativo , Feminino , Humanos , Lactente , Masculino , Melhoria de Qualidade/organização & administração , Adulto JovemRESUMO
PURPOSE: Treating constipation in children with voiding dysfunction may improve or resolve urinary symptoms. A clinical diagnosis of constipation may not identify all patients. Abdominal radiographs (plain x-ray of the kidneys, ureters and bladder) are often used to assess constipation but no objective definition of constipation based on abdominal radiographs exists. Most abdominal radiograph rating scales use subjective criteria and our previous series showed that these scales have poor reliability. We identified reliable, objective parameters on abdominal radiograph to predict constipation. MATERIALS AND METHODS: The abdominal radiographs of 80 children 4 to 12 years old, including 40 with constipation and 40 successfully treated for constipation, were assessed for several measurable parameters. Logistic regression was used to construct a model to predict constipation status based on these abdominal radiograph parameters. Model accuracy was assessed using AUC analysis of ROC curves. RESULTS: The most predictive model included cecal diameter, total length of stool measured, stool length in the rectum, and patient age and gender. As measured by the area under the ROC curve, accuracy was excellent at 0.87. We calculated cutoffs for individual parameters on abdominal radiograph, including total stool length greater than 33.4 cm, cecal diameter greater than 3.7 cm and stool length in the rectum greater than 5.9 cm. CONCLUSIONS: We identified accurate, reliable criteria based on objective measurements on abdominal radiograph to differentiate patients with and without constipation. These criteria may be applied to objectively assess constipation status in children with urinary symptoms without a history of constipation. Further study will determine whether these criteria predict the response to treatment.
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Constipação Intestinal/diagnóstico por imagem , Radiografia Abdominal , Estudos de Casos e Controles , Criança , Pré-Escolar , Constipação Intestinal/complicações , Humanos , Sintomas do Trato Urinário Inferior/complicações , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Estudos RetrospectivosRESUMO
Urogenital sinus (UGS) is a rare anomaly characterized by a common cavity involving the reproductive and urinary tracts. We describe a patient with VACTERL syndrome who presented for urologic care at 11 years of age due to supposed "recurrent urinary tract infections" and was subsequently found to have UGS in which the vagina connected directly to the bladder. She underwent robotic UGS mobilization to disconnect the vagina from the bladder and vaginoplasty to mature the vagina to the perineum. The objective of this report is to describe the presentation, diagnosis, and management of a patient with rare high confluence UGS.
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Infecções Urinárias , Anormalidades Urogenitais , Feminino , Humanos , Genitália Feminina , Vagina/cirurgia , Bexiga Urinária/cirurgia , Rim , Infecções Urinárias/diagnóstico , Infecções Urinárias/etiologia , Anormalidades Urogenitais/complicações , Anormalidades Urogenitais/diagnóstico , Anormalidades Urogenitais/cirurgiaRESUMO
PURPOSE: Patients with spina bifida require urologic follow up with assessments of bladder and renal function. The ideal screening protocol for bladder compliance remains controversial. This study assessed associations between bladder end filling pressure (EFP) and detrusor leak point pressure (DLPP) on urodynamic studies (UDS) and findings from renal bladder ultrasounds (RBUS) and voiding cystourethrograms (VCUG). METHODS: Pediatric spina bifida patients who underwent RBUS and/or VCUG within six months of UDS at a single institution from 2015 to 2021 were retrospectively reviewed. Demographics, VCUG and RBUS results, and UDS EFP and DLPP were recorded. Multiple linear regression analyses and Pearson chi-square tests were conducted to correlate RBUS and VCUG findings with DLPP/EFP. RESULTS: 129 patients were included. In adjusted analyses, moderate to severe hydronephrosis on RBUS was significantly associated with elevations in mean DLPP/EFP (p = 0.006). Patients with round bladders had significantly lower mean DLPP/EFP than patients with elongated or conical bladders (p < 0.0001). The presence of trabeculations was associated with increased mean DLPP/EFP (p < 0.0001) and increases in severity of trabeculations were associated with higher DLPP/EFP (p < 0.0001). The presence of vesicoureteral reflux (VUR) on VCUG was not associated with significant differences in mean DLPP/EFP (p = 0.20). CONCLUSIONS: Abnormal bladder shape and trabeculations on VCUGs in spina bifida patients had significant associations with increased mean bladder pressures on UDS. Assessment of bladder shape and contour by VCUG may be a valuable screening tool in conjunction with routine RBUS in determining the need for further assessment of bladder pressures in patients with spina bifida.
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Disrafismo Espinal , Bexiga Urinaria Neurogênica , Refluxo Vesicoureteral , Humanos , Criança , Estudos Retrospectivos , Bexiga Urinária/diagnóstico por imagem , Disrafismo Espinal/complicações , Disrafismo Espinal/diagnóstico por imagem , Bexiga Urinaria Neurogênica/diagnóstico por imagem , Bexiga Urinaria Neurogênica/etiologia , Refluxo Vesicoureteral/complicações , Refluxo Vesicoureteral/diagnóstico por imagem , UrodinâmicaRESUMO
PURPOSE: Parents counseled for surgery are quoted operative risks based on published results in the literature. However, outcomes from single surgeon or single institution retrospective studies are not generalizable. We assessed whether published outcomes were perceived to be representative of personal practice by pediatric urologists. We also correlated patterns of perceptions with surgical volumes and demographic variables. MATERIALS AND METHODS: A survey of 26 questions on 8 topics was e-mailed to 269 members of the American Academy of Pediatrics Section on Urology. Topics studied included distal and single stage proximal hypospadias, pyeloplasty, ureteral reimplantation with or without tapering, bladder neck reconstruction, and single and 2-stage bladder exstrophy repair. Participants were asked whether their rates of results were lower, the same or higher than specific published outcomes on a 5-point Likert scale. Study participants were also requested to provide demographic data and to estimate their annual case volume for each condition. RESULTS: Of the 269 section members who were contacted 110 (40.9%) completed the survey. More than 80% of respondents practice pediatric urology exclusively with 50.9% reporting operative volumes of more than 400 cases per year. A greater proportion of participants reported worse outcomes than published reports for hypospadias fistula rates (distal p = 0.001, proximal p = 0.023), bladder neck repair (p = 0.018) and exstrophy repair continence rates (single and 2-stage p <0.001). Improved outcomes compared to published data were reported for ureteral reimplantation (p = 0.013) and pyeloplasty (p = 0.003). However, these findings did not correlate consistently with case volume or other demographic characteristics. CONCLUSIONS: A significant proportion of pediatric urologists perceive their personal outcomes to be different than those in the published literature, regardless of practice setting, operative volume or time in practice. In an era of pay for performance and quality improvement, publication bias can have implications for patient care, reimbursement and malpractice.
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Pediatria , Editoração , Procedimentos Cirúrgicos Urológicos , Urologia , Adulto , Criança , Humanos , Pessoa de Meia-Idade , Resultado do TratamentoRESUMO
PURPOSE: Our primary aim was to determine the prevalence of positive over-the-counter (OTC) pregnancy tests among pediatric female and male patients who had undergone augmentation enterocystoplasty. A secondary aim was to determine whether patient factors or urinalysis (UA) results were associated with false positive OTC pregnancy test results in patients with a history of augmentation enterocystoplasty. METHODS: Patients at a tertiary pediatric hospital who had previously undergone augmentation enterocystoplasty, Mitrofanoff appendicovesicostomy only, and age- and sex-matched controls were prospectively recruited. Urine samples were obtained, and two OTC pregnancy tests were conducted - OTC Test A reported the ability to detect pregnancy at hCG levels as low as 9.6 mIU/mL, while OTC Test B reported positive pregnancy test results at hCG levels ≥25 mIU/mL. A point of care (POC) pregnancy test and UA were also completed. Mann-Whitney U tests and Fisher's exact tests were used to assess whether patient factors or UA results were associated with false positive pregnancy test results. RESULTS: Fifty pediatric patients were enrolled, including 20 with augmentation enterocystoplasty, 20 age- and sex-matched controls, and 10 with Mitrofanoff appendicovesicostomy only. Among the 20 patients who had undergone augmentation cystoplasty, 15 (75.0%) had positive pregnancy test results on OTC Test A, including 11/13 (84.6%) females and 4/7 (57.1%) males. No control patients or patients with Mitrofanoff appendicovesicostomy only had a positive test. No patients had positive pregnancy test results on OTC Test B or the POC test. While patients with augmentation enterocystoplasty were significantly more likely to test positive for blood (p = 0.01), nitrate (p = 0.03), and leukocytes (p < 0.0001), these factors were not significantly associated with false positive pregnancy results. No patient factors or UA results were associated with increased likelihood of false positive OTC pregnancy test results. CONCLUSIONS: These findings indicate that sensitive OTC pregnancy tests that detect low quantities of hCG in urine may result in false positive results among patients who have undergone augmentation enterocystoplasty. Female patients of reproductive age with bladder augmentations should be counseled regarding appropriate pregnancy testing options. Additional research is needed to determine the mechanism responsible for false positive pregnancy tests in this patient population.
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Procedimentos Cirúrgicos do Sistema Digestório , Testes de Gravidez , Gravidez , Criança , Humanos , Masculino , Feminino , Procedimentos Cirúrgicos Urológicos/métodos , Anastomose Cirúrgica , IntestinosRESUMO
A 2-month-old full-term female presented with a large anterior sacral meningomyelocele resulting in transient obstructive uropathy with bilateral hydronephrosis and acute kidney injury. After initial bladder decompression and surgical resection of the meningomyelocele, there was spontaneous resolution of bladder function confirmed with urodynamics. Anterior spinal meningomyelocele (ASM) is a rare neural tube defect that may present with urinary dysfunction secondary to compression of the bladder and sacral nerve roots or congenital defects to the bladder nervous supply. Obstructive uropathy due to ASM may spontaneously resolve after surgical resection.
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A 12-year-old otherwise healthy boy presents to a local emergency room with sudden onset, sharp, continuous left testicular pain for 9 hours, associated with nausea and vomiting. Exam and ultrasound is consistent with testicular torsion. During scrotal exploration, a bell-clapper deformity of the left testicle is appreciated, with a normal gubernacular attachment of the right testicle. Bell-clapper deformity is a congenital failure of the posterior attachment of the gubernaculum to the testis, which increases testicular mobility within the tunica vaginalis and predisposes individuals to testicular torsion.
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Torção do Cordão Espermático/etiologia , Testículo/anormalidades , Criança , Humanos , Masculino , Torção do Cordão Espermático/cirurgia , Testículo/cirurgiaRESUMO
A 16-year-old female was incidentally found to have an abnormal pelvic ultrasound while undergoing evaluation for mild scoliosis. A large, thick-walled, lobular, fluid-filled structure was found at midline in the pelvis that initially resembled bladder diverticula due to its anatomical position and sonographic appearance. Voiding cystourethrogram and computed tomography later revealed a large cystic mass originating from the right ovary. This report outlines a case of a mature cystic teratoma convincingly mimicking bladder anomaly on sonography.
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Divertículo/diagnóstico , Neoplasias Ovarianas/diagnóstico , Teratoma/diagnóstico , Bexiga Urinária/anormalidades , Adolescente , Diagnóstico Diferencial , Feminino , HumanosRESUMO
INTRODUCTION/BACKGROUND: Bladder exstrophy patients have a high prevalence of inguinal hernia that often become clinically evident following bladder closure. Understanding when the bladder exstrophy patient is under greatest risk of developing an inguinal hernia following bladder closure is important, since incarceration resulting in strangulation of intra-abdominal contents can lead to significant morbidity if not addressed in a timely fashion. Although the incidence and risk factors of inguinal hernia have been reported, the timing of occurrence is not well understood. OBJECTIVE: The primary objective of this study was to assess the timing of inguinal hernia following complete primary repair of bladder exstrophy (CPRE). In addition, we aimed to evaluate possible risk factors associated with inguinal hernia, including sex, age at bladder closure and iliac osteotomy status. STUDY DESIGN: A multi-institutional retrospective review identified patients with bladder exstrophy repaired by CPRE under 6 months of age while excluding those who underwent inguinal hernia repair before or during bladder closure. Timing of inguinal hernia following bladder closure was evaluated using Kaplan-Meier methods. Cox proportional hazards model was used to investigate association of sex, age at bladder closure, and osteotomy on the risk of developing of inguinal hernia while clustering for institution. RESULTS: 91 subjects were included in our analysis with median follow-up time of 6.5 years. 34 of 53 males (64.2%) and 2 of 38 females (5.3%) underwent inguinal hernia repair. The median time to inguinal hernia was 4.7 months following closure. The greatest hazard of inguinal hernia was within the first six months following closure. In multivariate analysis, male sex was strongly associated with inguinal hernia (HR = 19.00, p = 0.0038). Osteotomy and delay in closure were not significantly associated with inguinal hernia. 7 of 36 patients (19.4%) who underwent inguinal hernia repair presented with recurrence on the ipsilateral side. DISCUSSION: Our results suggest that the greatest risk of inguinal hernia is within the first six months following bladder closure. The decreased risk of inguinal hernia after one year of follow-up may reflect anatomic stability that is reached following major reconstruction of the pelvis. While male bladder exstrophy patients are significantly more susceptible to inguinal hernias following CPRE, osteotomy and delayed bladder closure do not appear to be protective factors for inguinal hernia development following initial bladder closure. CONCLUSIONS: There is a heightened risk of inguinal hernia in the first six months following closure. The rate of recurrence following inguinal hernia repair is significantly elevated compared to the general pediatric population.
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Extrofia Vesical , Hérnia Inguinal , Extrofia Vesical/epidemiologia , Extrofia Vesical/cirurgia , Criança , Feminino , Hérnia Inguinal/epidemiologia , Hérnia Inguinal/cirurgia , Humanos , Masculino , Estudos Retrospectivos , Resultado do Tratamento , Procedimentos Cirúrgicos UrológicosRESUMO
INTRODUCTION: Infants with myelomeningocele are at risk for chronic kidney disease caused by neurogenic bladder dysfunction. Urodynamic evaluation plays a key role to risk stratify individuals for renal deterioration. OBJECTIVE: To present baseline urodynamic findings from the Urologic Management to Preserve Initial Renal function for young children with spina bifida (UMPIRE) protocol, to present the process that showed inadequacies of our original classification scheme, and to propose a refined definition of bladder hostility and categorization. STUDY DESIGN: The UMPIRE protocol follows a cohort of newborns with myelomeningocele at nine children's hospitals in the United States. Infants are started on clean intermittent catheterization shortly after birth. If residual volumes are low and there is no or mild hydronephrosis, catheterization is discontinued. Baseline urodynamics are obtained at or before 3 months of age to determine further management. Based on protocol-specific definitions, urodynamic studies were reviewed by the clinical site in addition to a central review team; and if necessary, by all site urologists to achieve 100% concurrence. RESULTS: We reviewed 157 newborn urodynamic studies performed between May 2015 and September 2017. Of these 157 infants, 54.8% were boys (86/157). Myelomeningocele closure was performed in-utero in 18.4% (29/157) and postnatally in 81.5% (128/157) of newborns. After primary review, reviewers agreed on overall bladder categorization in 50% (79/157) of studies. Concurrence ultimately reached 100% with further standardization of interpretation. We found that it was not possible to reliably differentiate a bladder contraction due to detrusor overactivity from a volitional voiding contraction in an infant. We revised our categorization system to group the "normal" and "safe" categories together as "low risk". Additionally, diagnosis of detrusor sphincter dyssynergia (DSD) with surface patch electrodes could not be supported by other elements of the urodynamics study. We excluded DSD from our revised high risk category. The final categorizations were high risk in 15% (23/157); intermediate risk in 61% (96/157); and low risk in 24% (38/157). CONCLUSION: We found pitfalls with our original categorization for bladder hostility. Notably, DSD could not be reliably measured with surface patch of electrodes. The effect of this change on future renal outcomes remains to be defined.
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Meningomielocele , Bexiga Urinaria Neurogênica , Criança , Pré-Escolar , Hostilidade , Humanos , Lactente , Recém-Nascido , Masculino , Meningomielocele/complicações , Meningomielocele/diagnóstico , Bexiga Urinaria Neurogênica/diagnóstico , Bexiga Urinaria Neurogênica/etiologia , UrodinâmicaRESUMO
A 5-week-old full-term female with high-risk right hydronephrosis was transferred to the emergency department (ED) for tachypnea. A contrast-enhanced computed tomography (CT) study demonstrated a 9.0 cm cystic lesion in the region of the previously seen hydronephrosis (Fig. 1). Urology was consulted to rule out a cystic neoplasm, which on review of imaging was more suggestive of a contained forniceal rupture. An ultrasound-guided percutaneous nephrostomy tube drained 200 cc of urine. Culture and cytology were negative. Respiratory status markedly improved. The patient was discharged with outpatient follow to evaluate for ureteropelvic junction obstruction.
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Nefropatias/diagnóstico por imagem , Ruptura Espontânea/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Diagnóstico Diferencial , Feminino , Humanos , Recém-Nascido , Doenças Renais Císticas/diagnóstico por imagemRESUMO
A 7-week-old girl presented with tachypnea and abdominal distension. Abdominal ultrasonography demonstrated a 16 cm cystic mass in the right abdomen. Contrast-enhanced T2-weighted magnetic resonance imaging revealed severe hydronephrosis of the right kidney with thinning of the renal parenchyma consistent with a massive ureteropelvic junction (UPJ) obstruction. A percutaneous nephrostomy tube was placed for decompression of her right kidney and 1270 ml of fluid was drained. The patient underwent an open pyeloplasty. Her follow-up renal ultrasonography demonstrated resolution of her severe hydronephrosis.
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INTRODUCTION: Stress urinary incontinence (SUI) is common among older multiparous females but rarely reported in active, young girls. OBJECTIVES: Our hypothesis is: physically active adolescent females develop pelvic floor laxity demonstrable on upright VCUG. Our objectives are to (1) increase awareness of SUI in young females, (2) test our hypothesis with an upright VCUG, and (3) report effectiveness of step-wise management. STUDY DESIGN: A retrospective review was performed of nulliparous girls with only SUI seen from 2000 to 2015, who were evaluated with upright voiding cystourethrography (VCUG) (bladder descent defined as ≥2 cm drop of bladder neck below pubic ramus at capacity). Data collection included level of physical activity, physical examination, BMI and Z-scores, urodynamics, management, and treatment response. Standard urotherapy (SUT) (timed voiding, proper diet, adequate fluids, bowel management) and biofeedback therapy (BFT) was initiated. Fisher exact test was used to calculate 'p' values. RESULTS: Thirty-three females (median age 15.1 years, range 5.5-20.3) were identified who underwent an upright VCUG; 20 had bladder neck descent (Fig.). Of these 20, 15 (75%) were involved in strenuous activity, whereas only three of 13 (23%) without descent engaged in intense athletics. No differences were noted in median BMI and Z-score with strenuous activity (21.1 (15.2-26.7) and 0.31 (-0.9-1.94)), respectively, versus patients without (21.3 (15.8-33.5) and 0.62 (-0.0-2.38)). Average follow-up for all was 16.6 months (range 0.4-102.2). Of 20 demonstrating bladder neck descent, three did not complete therapy and were lost to follow-up. Only six of these 17 became dry. Of the remaining 11, eight underwent surgery: Burch colposuspension (5), fascial sling (2), Coaptite to the bladder neck (1), and an artificial urinary sphincter (1). This latter girl had a failed Burch colposuspension 1 year previously. All surgical patients are dry. Of 13 without bladder descent on VCUG, five did not complete therapy and were lost to follow-up. The remaining eight were managed non-surgically; seven were fully dry at last follow-up. Overall, 13 of 25 (52%) achieved dryness. SUT and BFT were more effective in those without, than in those with bladder descent (87.5% vs. 35.3%, p = 0.0302, Fisher exact test). DISCUSSION AND CONCLUSIONS: Physically active, nulligravid girls with SUI can be efficaciously diagnosed on upright VCUG. They should be considered for non-surgical therapy but will likely require bladder neck elevating surgery. Non-surgical therapy works for those with minimal bladder descent on cystography.