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1.
J Urol ; 212(1): 165-174, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38700226

RESUMO

PURPOSE: Urodynamic testing (UDS) is an important tool in the management of pediatric lower urinary tract conditions. There have been notable efforts to standardize pediatric UDS nomenclature and technique, but no formal guidelines exist on essential elements to include in a clinical report. We sought to identify ideal structure and elements of a pediatric UDS assessment based on expert consensus. MATERIALS AND METHODS: Pediatric urologists regularly performing UDS were queried using a Delphi process. Participants were invited representing varied geographic, experience, and societal involvement. Participants underwent 3 rounds of questionnaires between November 2022 and August 2023 focusing on report organization, elements, definitions, and automated electronic health record clinical decision support. Professional billing requirements were also considered. Consensus was defined as 80% agreeing either in favor of or against a topic. Elements without consensus were discussed in subsequent rounds. RESULTS: A diverse sample of 30 providers, representing 27 institutions across 21 US states; Washington, District of Columbia; and Canada completed the study. Participants reported interpreting an average number of 5 UDS reports per week (range 1-22). The finalized consensus report identifies 93 elements that should be included in a pediatric UDS report based on applicable study conditions and findings. CONCLUSIONS: This consensus report details the key elements and structure agreed upon by an expert panel of pediatric urologists. Further standardization of documentation should aid collaboration and research for patients undergoing UDS. Based on this information, development of a standardized UDS report template using electronic health record implementation principles is underway, which will be openly available for pediatric urologists.


Assuntos
Consenso , Técnica Delphi , Urodinâmica , Humanos , Criança , Urologia/normas , Pediatria/normas , Masculino , Inquéritos e Questionários
2.
J Urol ; 209(3): 582-590, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36445021

RESUMO

PURPOSE: The majority of children with unilateral renal masses suspicious for malignancy undergo radical nephrectomy, while nephron-sparing surgery is reserved for select cases. We investigated the impact of tumor size on the probability of histology. We hypothesized that pediatric small renal masses are more likely benign or non-Wilms tumor, thus potentially appropriate for nephron-sparing surgery. MATERIALS AND METHODS: The SEER (Surveillance, Epidemiology, and End Results) database was analyzed for patients aged 0-18 years diagnosed with a unilateral renal mass from 2000-2016. Statistical analysis was performed to help determine a tumor size cut point to predict Wilms tumor and assess the predictive value of tumor size on Wilms tumor histology. Additionally, a retrospective review was performed of patients 0-18 years old who underwent surgery for a unilateral renal mass at a single institution from 2005-2019. Statistical analysis was performed to assess the predictive value of tumor size on final histology. RESULTS: From the SEER analysis, 2,016 patients were included. A total of 1,672 tumors (82.9%) were Wilms tumor. Analysis revealed 4 cm to be a suitable cut point to distinguish non-Wilms tumor. Tumors ≥4 cm were more likely Wilms tumor (OR 2.67, P ≤ .001), but this was driven by the statistical significance in children 5-9 years old. From the institutional analysis, 134 patients were included. Ninety-seven tumors (72.3%) were Wilms tumor. Tumors ≥4 cm had higher odds of being Wilms tumor (OR 30.85, P = .001), malignant (OR 6.75, P = .005), and having radical nephrectomy-appropriate histology (OR 46.79, P < .001). CONCLUSIONS: The probability that a pediatric unilateral renal mass is Wilms tumor increases with tumor size. Four centimeters is a logical cut point to start the conversation around defining pediatric small renal masses and may help predict nephron-sparing surgery-appropriate histology.


Assuntos
Neoplasias Renais , Tumor de Wilms , Criança , Humanos , Recém-Nascido , Lactente , Pré-Escolar , Adolescente , Neoplasias Renais/cirurgia , Neoplasias Renais/patologia , Néfrons/cirurgia , Néfrons/patologia , Tumor de Wilms/cirurgia , Nefrectomia/métodos , Estudos Retrospectivos
3.
Pediatr Blood Cancer ; 68(10): e29226, 2021 10.
Artigo em Inglês | MEDLINE | ID: mdl-34245214

RESUMO

BACKGROUND: Two chemotherapeutic agents used widely in pediatric oncology are vincristine (VCR) and doxorubicin (DOX), which may cause neuropathy and myopathy, respectively. The study hypothesis is that neurotoxic effects of VCR and/or myotoxic effects of DOX affect bladder physiology and manifest clinically as lower urinary tract dysfunction (LUTD). PROCEDURE: Based on a priori power analysis, 161 children divided evenly by gender were recruited. Children aged 5-10 years completed the dysfunctional voiding scoring system (DVSS) survey. The study cohort comprised cancer survivors treated with VCR and/or DOX. Healthy controls were recruited from well-child clinic visits. Exclusion criteria included pelvic-based malignancy, pelvic irradiation, pre-existing LUTD, neurologic abnormalities, and treatment with cyclophosphamide/ifosfamide. DVSS scores and presence of LUTD, defined as DVSS scores above gender-specific thresholds (males ≥9, females ≥6), were compared across cohorts. RESULTS: Median DVSS scores were higher in the study cohort (6 vs. 4, p = .003). Moreover, children in the study cohort were more likely to exceed threshold scores for LUTD (38.8% vs. 21%, p = .014; OR 1.8). Subanalysis by gender revealed female cancer survivors are more likely to report LUTD than controls (57.5% vs. 30%, p = .013, OR 1.9). This did not hold true for males (20% vs. 12.2%, p = .339). CONCLUSIONS: Childhood cancer survivors who received VCR and/or DOX reported higher rates of LUTD than controls. Female cancer survivors appear more likely to suffer from LUTD than males. Further study with a positive control cohort of cancer survivors who received non-VCR, non-DOX chemotherapy is underway to elucidate the contribution of a cancer diagnosis to LUTD.


Assuntos
Sobreviventes de Câncer , Doxorrubicina , Sintomas do Trato Urinário Inferior , Neoplasias , Vincristina , Criança , Doxorrubicina/efeitos adversos , Feminino , Humanos , Masculino , Neoplasias/tratamento farmacológico , Estudos Prospectivos , Bexiga Urinária , Vincristina/efeitos adversos
5.
Pediatr Surg Int ; 35(9): 985-987, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31256297

RESUMO

AIM OF THE STUDY: Misdiagnosing a cloaca as a disorder of sex development may lead to inappropriate testing, treatment, and negative emotional consequences to families. We were impressed by the fact that a significant number of patients suffering from a cloaca were referred to us with the diagnosis of a "disorder of sex development" previously referred as "ambiguous genitalia" or "intersex". On re-evaluation, none of them truly had a disorder of sex differentiation. This prompted us to conduct the following retrospective review to try to find the cause of the misdiagnosis and the way to prevent it. METHODS: A retrospective review of our colorectal database was performed to identify the total number of patients with cloacas and the number initially diagnosed as "ambiguous genitalia, intersex"/disorder of sex development. The external appearance of their genitalia and unnecessary testing or treatment received were recorded. MAIN RESULTS: A total of 605 patients with cloacas were identified. Of these, 77 (12.7%) were referred to us with the diagnosis of "ambiguous genitalia" and 13 of them (17%) went on to receive an intervention that was not indicated: karyotyping (10), steroids (3), and ovarian biopsy (1). The karyotype result in all patients was XX. The misdiagnosis was triggered by the external appearance of the perineum, simulating a case of virilization with a hypertrophic clitoris, but was simply prominent labial skin. Careful examination of the perineal structure allowed us to determine that it consisted of folded skin with no evidence of corpora. CONCLUSION: Patients born with a cloaca are at risk for mismanagement from being erroneously labeled as disorders of sex development. The diagnosis of a cloacal anomaly is a clinical one. The practitioner must distinguish between phallus-like clitoral hypertrophy and a normal clitoris with prominent labial skin.


Assuntos
Cloaca/anormalidades , Erros de Diagnóstico/prevenção & controle , Transtornos do Desenvolvimento Sexual , Bases de Dados Factuais , Feminino , Genitália/anormalidades , Humanos , Períneo/anormalidades , Estudos Retrospectivos
6.
Pediatr Surg Int ; 35(9): 1005-1008, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31278478

RESUMO

PURPOSE: The literature about ectopic ureters in anorectal malformations is limited. Repair of an anorectal malformation may require dissection near the normal or abnormal insertion of the ureters. Knowledge of the presence and location of ectopic ureters may prevent intraoperative injury. We aim to describe the incidence and location of ectopic ureters in patients with anorectal malformations and to characterize associated renal anomalies. METHODS: This is an IRB-approved retrospective study of patients with anorectal malformations and ectopic ureters identified in our colorectal database. RESULTS: Of 2283 patients with anorectal malformation, 79 (3.5%) had ectopic ureter(s). Of those, 29% had bilateral ectopic ureters. Nearly all (87%) of bilateral ectopic ureters occurred in females. Ectopic ureters most commonly inserted into the bladder neck (33%), vagina (15%), or urethra (13%). Renal dysfunction was noted in a high proportion of patients. The majority (59%) of ectopic ureters were associated with dysfunction of the ipsilateral kidney. Interestingly, 29% of patients with unilateral ureteral ectopia had an abnormal contralateral kidney. Only 22 patients (28%) had two normal kidneys, and 5 (6%) had documented renal failure with 2 (2.5%) requiring renal transplant. This compares to a transplant rate of 0.6% among anorectal malformation patients without ectopic ureter. CONCLUSIONS: The incidence of ectopic ureter is 3.5% among anorectal malformation patients. Cloaca and recto-bladder neck fistula are the types of anorectal malformation with higher incidence. Ureteral ectopia seems to confer an increased risk of renal failure. Identifying ectopic ureters is important for surgical planning and monitoring renal function vigilantly.


Assuntos
Malformações Anorretais/cirurgia , Ureter/anormalidades , Pré-Escolar , Bases de Dados Factuais , Feminino , Humanos , Rim/anormalidades , Masculino , Estudos Retrospectivos
8.
J Pediatr Urol ; 2024 May 28.
Artigo em Inglês | MEDLINE | ID: mdl-38866647

RESUMO

BACKGROUND: The transfer of pediatric patients with testicular torsion from community hospitals to pediatric centers can be a time and resource-intensive step toward emergent surgical intervention. OBJECTIVE: We sought to describe trends of patient transfer in our state and compare clinical outcomes and health system costs between patients transferred and treated primarily at a pediatric center. STUDY DESIGN: This retrospective cohort study compared patients aged 1-18 years who presented directly to a pediatric center to those transferred for acute testicular torsion from 2018 to 2023. Exclusion criteria included age <1 year, non-urgent surgery, and admission from clinic. Patient age, BMI, Tanner stage, ASA class, insurance coverage, and presentation time were covariates. Group characteristics and times from symptom onset to initial ED presentation to surgery were compared via two-sided Student's t-tests. Clinical outcomes (orchiectomy, testicular atrophy) were compared via Fisher's exact tests. Costs from transferring hospitals were estimated from costs at our institution, and medical transport costs were extrapolated from contract prices between transport agencies and the pediatric center to compare total episode-of-care cost. RESULTS: A total of 133 cases (37 primaries, 96 transfers) met inclusion criteria. Transfers increased over the study period (67%-75%). There were no significant differences in age, Tanner stage, ASA score, BMI, or time of day of presentation between groups. Median transfer distance was 12 miles (IQR 7-22) and time was 1 h (IQR 1-2). More than half of cases (53%) were transferred due to hospital policy regarding surgical treatment of minors, and 25% due to lack of urology coverage. Time from initial ED site to OR was nearly doubled for the transfer group (median 4.5 vs 2.5 h, p = 0.02). Despite a higher rate of orchiectomy in the primary group (43 vs 22%, p = 0.01), this difference was not significant after stratification by symptom duration. The estimated average cost of care for patients transferred was twice that of primary patients ($15,082 vs $6898). DISCUSSION: Transfer of pediatric patients in our state for testicular torsion has increased in recent years. Hospital policies and local urology coverage are primary drivers of patient transfer which nearly doubled time to surgical intervention and more than doubled cost of care. Clinical outcomes were driven by delayed presentation. CONCLUSION: Transfer of pediatric patients for testicular torsion nearly doubles time to surgical intervention and more than doubles cost of care. Restrictive hospital policies and gaps in rural hospital urology coverage present opportunities to improve the quality and efficiency of care for these children.

9.
J Pediatr Urol ; 19(3): 277-283, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-36775718

RESUMO

BACKGROUND: Although hypospadias outcomes studies typically report a level or type of repair performed, these studies often lack applicability to each surgical practice due to technical variability that is not fully delineated. An example is the tubularized incised plate (TIP) urethroplasty procedure, for which modifications have been associated with significantly decreased complication rates in single center series. However, many studies fail to report specificity in techniques utilized, thereby limiting comparison between series. OBJECTIVE: With the goal of developing a surgical atlas of hypospadias repair techniques, this study examined 1) current techniques used by surgeons in our network for recording operative notes and 2) operative technical details by surgeon for two common procedures, tubularized incised plate (TIP) distal and proximal hypospadias repairs across a multi-institutional surgical network. STUDY DESIGN: A two-part study was completed. First, a survey was distributed to the network to assess surgeon volume and methods of recording hypospadias repair operative notes. Subsequently, an operative template or a representative de-identified operative note describing a TIP and/or proximal repair with urethroplasty was obtained from participating surgeons. Each was analyzed by at least two individuals for natural language that signified specified portions of the procedure. Procedural details from each note were tabulated and confirmed with each surgeon, clarifying that the recorded findings reflected their current practice techniques and instrumentation. RESULTS: Twenty-five surgeons from 12 institutions completed the survey. The number of primary distal hypospadias repairs performed per surgeon in the past year ranged from 1-10 to >50, with 40% performing 1-20. Primary proximal hypospadias repairs performed in the past year ranged from 1-30, with 60% performing 1-10. 96% of surgeons maintain operative notes within an electronic health record. Of these, 66.7% edited a template as their primary method of note entry; 76.5% of these surgeons reported that the template captures their operative techniques very or moderately well. Operative notes or templates from 16 surgeons at 10 institutions were analyzed. In 7 proximal and 14 distal repairs, parameters for chordee correction, urethroplasty suture selection and technique, tissue utilized, and catheter selection varied widely across surgeons. CONCLUSION: Wide variability in technical surgical details of categorically similar hypospadias repairs was demonstrated across a large surgical network. Surgeon-specific modifications of commonly described procedures are common, and further evaluation of short- and long-term outcomes accounting for these technical variations is needed to determine their relative influence.


Assuntos
Hipospadia , Procedimentos de Cirurgia Plástica , Urologia , Criança , Masculino , Humanos , Lactente , Hipospadia/cirurgia , Resultado do Tratamento , Uretra/cirurgia , Procedimentos Cirúrgicos Urológicos Masculinos/métodos , Estudos Retrospectivos
10.
J Pediatr Rehabil Med ; 16(4): 605-619, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-38073338

RESUMO

PURPOSE: This study aimed to analyze organ system-based causes and non-organ system-based mechanisms of death (COD, MOD) in people with myelomeningocele (MMC), comparing urological to other COD. METHODS: A retrospective review was performed of 16 institutions in Canada/United States of non-random convenience sample of people with MMC (born > = 1972) using non-parametric statistics. RESULTS: Of 293 deaths (89% shunted hydrocephalus), 12% occurred in infancy, 35% in childhood, and 53% in adulthood (documented COD: 74%). For 261 shunted individuals, leading COD were neurological (21%) and pulmonary (17%), and leading MOD were infections (34%, including shunt infections: 4%) and non-infectious shunt malfunctions (14%). For 32 unshunted individuals, leading COD were pulmonary (34%) and cardiovascular (13%), and leading MOD were infections (38%) and non-infectious pulmonary (16%). COD and MOD varied by shunt status and age (p < = 0.04), not ambulation or birthyear (p > = 0.16). Urology-related deaths (urosepsis, renal failure, hematuria, bladder perforation/cancer: 10%) were more likely in females (p = 0.01), independent of age, shunt, or ambulatory status (p > = 0.40). COD/MOD were independent of bladder augmentation (p = >0.11). Unexplained deaths while asleep (4%) were independent of age, shunt status, and epilepsy (p >= 0.47). CONCLUSION: COD varied by shunt status. Leading MOD were infectious. Urology-related deaths (10%) were independent of shunt status; 26% of COD were unknown. Life-long multidisciplinary care and accurate mortality documentation are needed.


Assuntos
Hidrocefalia , Meningomielocele , Feminino , Humanos , Meningomielocele/complicações , Meningomielocele/cirurgia , Estudos Retrospectivos , Causas de Morte , Derivação Ventriculoperitoneal/efeitos adversos , Hidrocefalia/cirurgia
11.
Sci Rep ; 12(1): 5113, 2022 03 24.
Artigo em Inglês | MEDLINE | ID: mdl-35332157

RESUMO

Vincristine (VCR) is one of the most common chemotherapy agents used in pediatric oncology. Despite the well-known VCR-induced peripheral neuropathy, potential impacts of VCR on lower urinary tract (LUT) function remain poorly defined. We investigated the effects of systemic VCR exposure in childhood on LUT function by using juvenile mice treated with VCR (4 mg/kg) or saline and evaluated at 5 weeks later. VCR induced a decreased urinary frequency with increased functional bladder capacity and non-void contractions. There were no changes in detrusor contractility between the groups. VCR exposure caused sexual dimorphic changes; in females, increased intravesical pressure at micturition and downregulations of a major player in bladder afferent firing, Htr3b, in the bladders, and Cav1.2 in the lumbosacral dorsal root ganglia (Ls-DRG), while male mice displayed increases in bladder compliance and detrusor activity, upregulations of IL-2, Trpa1 and Itga1 in the bladders and neuroinflammation-related genes, P2×4, P2×7, IL-2 and CD68 in the Ls-DRG. These results suggest that that systemic VCR exposure caused sensory neuropathy via sex-dimorphic mechanisms, leading to altered LUT function. These changes might clinically present as gender-specific signs or symptoms of LUT dysfunction, and follow-up urological assessment may be of benefit for pediatric cancer patients treated with VCR.


Assuntos
Doenças do Sistema Nervoso Periférico , Bexiga Urinária , Animais , Feminino , Humanos , Interleucina-2/efeitos adversos , Masculino , Camundongos , Doenças do Sistema Nervoso Periférico/induzido quimicamente , Micção , Vincristina/efeitos adversos
12.
J Pediatr Urol ; 18(4): 502.e1-502.e9, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35810140

RESUMO

BACKGROUND: Management of the neurogenic bladder is variable, complex, and often requires a demanding bladder care regimen which may present caregiver burdens that are unique among chronic disease. While research into patient quality of life is increasing, parallel study of the caregiver experience is scant. Existing research primarily comprises survey data using validated instruments originally developed for non-urologic conditions, such as dementia. These surveys may detect high caregiver burden and decreased quality of life amongst caregivers but are limited in their ability to understand the underlying causes. OBJECTIVE: To characterize the experience of those caring for children with neurogenic bladders, with a focus on unexpected burdens and challenges. METHODS: In light of limited existing research, a qualitative research methodology was selected to explore the caregiver experience. Semi-structured phone interviews were conducted with primary caregivers of children with neurogenic bladder, all of whom were patients in the pediatric urology department of a single tertiary pediatric referral center. Purposive sampling was used to ensure diverse representation. Interviews were recorded, transcribed, and professionally translated if needed. Transcripts were analyzed using a team-based inductive grounded-theory approach, facilitated by ATLAS. ti software. Member-checking focus groups were held to validate the results. RESULTS: Twenty-five caregivers were interviewed (20 in English, 5 in Spanish), at which point thematic saturation was reached. Three primary themes emerged surrounding the topic of unexpected challenges: 1. High caregiver burden, 2. Challenges with catheterization and supplies, 3. Urinary tract infections. Member-checking focus groups validated the thematic analysis and provided additional insights into mitigating factors for these challenges. A child's independence with his or her health care regimen was cited as particularly important for decreasing caregiver burden. DISCUSSION: Caregivers of children with neurogenic bladder report their role is more difficult than they anticipated it would be. Catheterization represents a particularly burdensome task, and recurrent infections are an unexpected and persistent medical challenge. Understanding unexpected challenges that caregivers face will help pediatric urologists target modifiable factors to decrease caregiver burden, address current gaps in counseling and expectation-setting, and set the stage for more complete shared decision-making. CONCLUSIONS: This study represents an initial qualitative characterization of the experience caring for a child with neurogenic bladder. This is a key first step in understanding how caregivers make decisions for their children and their families. This initial study is foundational to a larger project to create a decision aid for caregivers of children with neurogenic bladder.


Assuntos
Cuidadores , Bexiga Urinaria Neurogênica , Humanos , Criança , Masculino , Feminino , Cuidadores/psicologia , Bexiga Urinaria Neurogênica/terapia , Bexiga Urinaria Neurogênica/psicologia , Qualidade de Vida , Pesquisa Qualitativa , Inquéritos e Questionários
14.
Urol Case Rep ; 34: 101452, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33194552

RESUMO

We report the case of a 4 year old female with severe traumatic brain injury who developed bilateral obstructing ureteral stones after hypertonic saline treatment. She developed calcium phosphate stones after two weeks of hypertonic saline therapy, and was successfully treated with ureteral stents and ureteroscopy. She has remained stone-free since that time. We postulate that an incomplete type 1 renal tubular acidosis made her intolerant to the acid and sodium load of the saline, and discuss other lithogenic factors of her presentation.

15.
J Pediatr Urol ; 17(1): 84.e1-84.e8, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33229228

RESUMO

BACKGROUND: With increasing awareness of the opioid epidemic, there is a push for providers to minimize opioid prescriptions. Enhanced Recovery After Surgery (ERAS) is a comprehensive multidisciplinary perioperative protocol that includes minimization of opioid analgesia in favor of non-opioid alternatives and regional analgesia. While ERAS protocols have consistently been shown to decrease inpatient opioid utilization, the impact on opioid prescribing practices and use after discharge in pediatric surgical patients is unclear. OBJECTIVE: This study aims to assess the impact of an ERAS protocol on outpatient opioid prescription patterns after pediatric lower urinary tract reconstructive surgery. We hypothesize that implementation of an ERAS protocol leads to fewer outpatient opioid prescriptions as measured by number and total quantity of oral morphine milligram equivalents by body weight per patient. METHODS: All patients who underwent bladder augmentation, creation of a continent catheterizable channel, bladder neck reconstruction or closure, or revision of prior reconstructive procedures at our tertiary care facility between 2011 and 2017 were reviewed. Patients were divided into pre-ERAS and ERAS cohorts based on whether surgery occurred before or after ERAS implementation. The Colorado Prescription Drug Monitoring Program was used to track filling of postoperative opioid prescriptions for patients covered by the database. RESULTS: A total of 167 urologic reconstructive surgeries were analyzed, including 83 before ERAS and 84 after ERAS implementation. Patients in the ERAS cohort received and filled more outpatient opioid prescriptions at time of discharge (82.6% historical vs 93.9% ERAS, p = 0.015; 76.1% vs 57.9%, p = 0.012). There were no differences in prescription total morphine milligram equivalents normalized to body mass, total days supplied, or 90-day opioid prescription refill rates. DISCUSSION: We found an unexpected increase in postoperative outpatient opioid prescriptions following implementation of an ERAS protocol for lower urinary tract reconstructive surgery. Possible reasons include worry about pain crisis at home in the setting of decreased hospital length of stay in the ERAS cohort or generalized upward drift in opioid prescribing patterns over time. ERAS protocols in other subspecialties reveal mixed findings but consistently suggest standardization of outpatient opioid prescribing patterns leads to a decrease in opioid prescriptions. CONCLUSIONS: Patients received more, not fewer, outpatient opioid prescriptions following major urologic reconstructive surgery after implementation of an ERAS protocol. Purposeful efforts should be made to standardize opioid prescriptions at discharge based on meaningful clinical criteria.


Assuntos
Analgésicos Opioides , Recuperação Pós-Cirúrgica Melhorada , Criança , Humanos , Manejo da Dor , Dor Pós-Operatória/tratamento farmacológico , Dor Pós-Operatória/prevenção & controle , Padrões de Prática Médica
16.
Urology ; 137: 168-172, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31794814

RESUMO

Neurofibromatosis-1 has a known increased risk of malignancy with rhabdomyosarcoma occurring in up to 6% of patients. Here we report on an 8-year-old male with a history of Neurofibromatosis-1 and previously treated stage 3, group III bladder/prostate embryonal rhabdomyosarcoma (diagnosed at 18 months old) who presented with penile swelling concerning for priapism. Imaging and subsequent biopsy confirmed embryonal rhabdomyosarcoma of the penile corporal bodies. Penile rhabdomyosarcoma is exceedingly rare, with less than 15 case reports in the literature. Our patient received chemoradiation per D9803 with organ preserving local control and is doing well 3 months after treatment.


Assuntos
Quimiorradioterapia/métodos , Recidiva Local de Neoplasia , Neurofibromatose 1 , Neoplasias Penianas , Priapismo/diagnóstico , Neoplasias da Próstata , Rabdomiossarcoma Embrionário , Neoplasias da Bexiga Urinária , Biópsia/métodos , Criança , Diagnóstico Diferencial , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Recidiva Local de Neoplasia/diagnóstico , Recidiva Local de Neoplasia/patologia , Estadiamento de Neoplasias , Neurofibromatose 1/patologia , Neurofibromatose 1/terapia , Neoplasias Penianas/diagnóstico , Neoplasias Penianas/tratamento farmacológico , Neoplasias Penianas/patologia , Neoplasias Penianas/radioterapia , Pênis/diagnóstico por imagem , Pênis/patologia , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Neoplasias da Próstata/patologia , Neoplasias da Próstata/terapia , Rabdomiossarcoma Embrionário/patologia , Rabdomiossarcoma Embrionário/terapia , Resultado do Tratamento , Neoplasias da Bexiga Urinária/patologia , Neoplasias da Bexiga Urinária/terapia
17.
Urology ; 137: 164-167, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31733273

RESUMO

Pediatric extrarenal malignant rhabdoid tumors (MRTs) are rare, aggressive tumors with a poor prognosis (20% 5-year survival). There are currently fewer than 10 published case reports of primary MRT of the bladder. We report the case of an 18-month-old female with an isolated MRT of the bladder which was initially misdiagnosed as an inflammatory myofibroblastic tumor on biopsy. We review the history, tumor biology, histology, and current management of extrarenal MRT, along with lessons learned from the difficulty with the patient's initial diagnosis.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Biópsia/métodos , Cistectomia/métodos , Neoplasias de Tecido Muscular , Radioterapia/métodos , Tumor Rabdoide , Neoplasias da Bexiga Urinária , Diagnóstico Diferencial , Erros de Diagnóstico , Feminino , Humanos , Hidronefrose/diagnóstico , Hidronefrose/etiologia , Lactente , Neoplasias de Tecido Muscular/diagnóstico , Neoplasias de Tecido Muscular/patologia , Prognóstico , Tumor Rabdoide/complicações , Tumor Rabdoide/diagnóstico , Tumor Rabdoide/patologia , Tumor Rabdoide/cirurgia , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento , Ultrassonografia/métodos , Bexiga Urinária/patologia , Neoplasias da Bexiga Urinária/complicações , Neoplasias da Bexiga Urinária/diagnóstico , Neoplasias da Bexiga Urinária/patologia , Neoplasias da Bexiga Urinária/cirurgia
18.
J Pediatr Surg ; 55(7): 1334-1338, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31128844

RESUMO

BACKGROUND/PURPOSE: While many children with renal tumors require long term venous access (VA) for adjuvant chemotherapy, certainly not all do. This study develops and tests a VA decision tree (DT) to direct the placement of VA in patients with renal tumors. METHODS: Utilizing data readily available at surgery a VADT was developed. The VADT was tested retrospectively by 2 independent reviewers on a historic cohort. The ability of the VADT to appropriately select which patients would benefit from VA placement was tested. RESULTS: 160 patients underwent renal tumor surgery between 2005 and 2018. 70 (43.8%) patients met study criteria with median age of 45.1 months (range 1.1-224); 73% required VA. Using the VADT, VA placement was "needed" in 67.1% of patients and "deferred" in 32.9%. Interrater reliability was very high (kappa = 0.97, 95% CI 0.91-1, p < 0.001). The sensitivity and specificity of the VADT to correctly decide on VA placement were 0.92 (0.8-0.98) and 1 (0.79-1). Using the VADT, no patient would have undergone unnecessary VA placement. In reality, 4.3% of patients had an unnecessary VA placed which required a subsequent removal. CONCLUSIONS: These preliminary data support the continued study of this VADT to guide intraoperative decisions regarding VA placement in patients with renal tumors. LEVEL OF EVIDENCE: III - Study of diagnostic test.


Assuntos
Cateterismo , Neoplasias Renais/tratamento farmacológico , Neoplasias Renais/cirurgia , Adolescente , Criança , Pré-Escolar , Árvores de Decisões , Humanos , Lactente , Rim/cirurgia , Estudos Retrospectivos
19.
J Pediatr Urol ; 16(4): 492.e1-492.e9, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32680626

RESUMO

INTRODUCTION: The COVID-19 pandemic has led to an unprecedented need to re-organise and re-align priorities for all surgical specialties. Despite the current declining numbers globally, the direct effects of the pandemic on institutional practices and on personal stress and coping mechanisms remains unknown. The aims of this study were to assess the effect of the pandemic on daily scheduling and work balances, its effects on stress, and to determine compliance with guidelines and to assess whether quarantining has led to other areas of increased productivity. METHODS: A trans-Atlantic convenience sample of paediatric urologists was created in which panellists (Zoom) discussed the direct effects of the COVID-19 pandemic on individual units, as well as creating a questionnaire using a mini-Delphi method to provide current semi-quantitative data regarding practice, and adherence levels to recently published risk stratification guidelines. They also filled out a Perceived Stress Scale (PSS) questionnaire to assess contemporary pandemic stress levels. RESULTS: There was an 86% response rate from paediatric urologists. The majority of respondents reported near complete disruption to planned operations (70%), and trainee education (70%). They were also worried about the effects of altered home-lives on productivity (≤90%), as well as a lack of personal protective equipment (57%). The baseline stress rate was measured at a very high level (PSS) during the pandemic. Adherence to recent operative guidelines for urgent cases was 100%. CONCLUSION: This study represents a panel discussion of a number of practical implications for paediatric urologists, and is one of the few papers to assess more pragmatic effects and combines opinions from both sides of the Atlantic. The impact of the pandemic has been very significant for paediatric urologists and includes a decrease in the number of patients seen and operated on, decreased salary, increased self-reported stress levels, substantially increased telemedicine usage, increased free time for various activities, and good compliance with guidelines and hospital management decisions.


Assuntos
Adaptação Psicológica/fisiologia , Betacoronavirus , Infecções por Coronavirus/epidemiologia , Fidelidade a Diretrizes/estatística & dados numéricos , Pneumonia Viral/epidemiologia , Estresse Psicológico/epidemiologia , Inquéritos e Questionários , Urologistas/psicologia , Adulto , COVID-19 , Criança , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Pandemias , SARS-CoV-2 , Estresse Psicológico/psicologia
20.
Urology ; 130: 151-154, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30807774

RESUMO

Due to the rarity of Wilms tumor (WT) and the relative urgency with which pediatric renal tumors are treated, there is little reported data on the natural history and growth of WTs. Historical reports of estimated doubling times of WTs were based on time to disease recurrence after initial diagnosis and treatment, and were published before the current advancements in molecular biomarker testing. We compare 2 cases of WT with sequential imaging, and postulate how the growth parameters of these tumors may be associated with differing chromosomal traits.


Assuntos
Neoplasias Renais/patologia , Tumor de Wilms/patologia , Adolescente , Pré-Escolar , Humanos , Neoplasias Renais/diagnóstico por imagem , Neoplasias Renais/cirurgia , Masculino , Fatores de Tempo , Carga Tumoral , Tumor de Wilms/diagnóstico por imagem , Tumor de Wilms/cirurgia
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