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1.
Prenat Diagn ; 44(2): 124-130, 2024 02.
Artigo em Inglês | MEDLINE | ID: mdl-36919753

RESUMO

OBJECTIVE: We evaluate survival of fetuses with severe Lower Urinary Tract Obstruction (LUTO) based on bladder morphology. We hypothesize that fetuses with a "floppy" appearing bladder on initial prenatal ultrasound will have worse infant outcomes than fetuses with full/rounded bladders. METHOD: We retrospectively reviewed all cases of LUTO evaluated in our fetal center between January 2013 and December 2021. Ultrasonographic assessment, renal biochemistry, and bladder refilling contributed to a "favorable" or "unfavorable" evaluation. Bladder morphology on initial ultrasound was classified as "floppy" or "full/rounded." Vesicoamniotic shunting was offered for favorably evaluated fetuses. Baseline demographics, ultrasound parameters, prenatal evaluations of fetal renal function, and infant outcomes were collected. Fetuses diagnosed with severe LUTO were included in analysis using descriptive statistics. The primary outcome measured was survival at 6 months of life. RESULTS: 104 LUTO patients were evaluated; 24 were included in analysis. Infant survival rate at 6 months was 60% for rounded bladders and 0% for floppy bladders (p = 0.003). Bladder refill adequacy was lower in fetuses with floppy bladders compared with rounded bladders (p value < 0.00001). CONCLUSION: We propose that bladder morphology in fetuses with severe LUTO may be a prognostication factor for predicting infant outcomes and provides a valuable, noninvasive assessment tool.


Assuntos
Doenças Fetais , Obstrução Uretral , Gravidez , Lactente , Feminino , Humanos , Bexiga Urinária/diagnóstico por imagem , Estudos Retrospectivos , Obstrução Uretral/diagnóstico por imagem , Obstrução Uretral/cirurgia , Ultrassonografia Pré-Natal , Doenças Fetais/diagnóstico por imagem , Feto
2.
Fetal Diagn Ther ; 47(8): 587-596, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32146466

RESUMO

OBJECTIVE: To determine postnatal neurodevelopmental (ND) outcomes for children with congenital lower urinary tract obstruction (LUTO) based on disease severity. METHODS: Twenty male infants with LUTO were classified prenatally as Stage 1 (normal amniotic fluid and renal function; n = 5), Stage 2 (signs of obstruction with preserved renal function; n = 13), and Stage 3 (signs of severe renal damage; n = 2). ND status was assessed using the Developmental Profile-3 test in 5 developmental domains (physical, adaptive behavior, social-emotional, cognitive, and communication). Each domain was considered to be delayed if standard scores were 2 or more SD below the mean. ND outcomes were compared between cases with an expected normal renal function (LUTO Stage 1) and those with impaired renal function (LUTO Stages 2 and 3). Results from cases with Stage 2 were also compared to those from Stage 3. ORs were calculated to predict risk for adverse ND outcome for each domain considering prenatal and postnatal factors. RESULTS: Gestational age (GA) at the diagnosis of LUTO was similar between both groups (Stage 1: 24.85 ± 7.87 vs. Stages 2 and 3: 21.4 ± 4.31 weeks; p = 0.24). Twelve of 15 cases with Stage 2 or 3 underwent vesicoamniotic shunt placement compared to none of Stage 1 fetuses (p < 0.01). No differences in GA at delivery were detected between the groups (37.9 ± 1.6 vs. 35.1 ± 3.6 weeks; p = 0.1). One of the infants in the Stage 2 and 3 groups received a kidney transplant during follow-up. One case (20%) from Stage 1 group required dialysis during the first 6 months of life, and 1 case from Stage 2 to 3 group required it during the first 6 months (p = 1.0), whereas 3 additional cases needed dialysis from 6 to 24 months (p = 0.6). Mean age at Developmental Profile 3 (DP-3) testing was 20.3 ± 12.3 months (Stage 1: 11.2 ± 8.6 vs. Stages 2 and 3: 23.4 ± 13.4 months; p = 0.07). Fifteen of the 20 patients (75%) had no ND delays. Of the 5 patients with ND delays, 4 received dialysis. No differences in ND outcomes between infants with LUTO Stage 1 and those with Stages 2 and 3 were detected except for a trend toward better physical development in Stage 1 (102.6 ± 11.6 vs. 80.7 ± 34.9; p = 0.05). Infants diagnosed with LUTO Stage 3 showed significantly lower adaptive scores than those diagnosed with Stage 2 (Stage 2: 101.9 ± 22.3 vs. Stage 3: 41.5 ± 30.4; p = 0.04) and a nonsignificant trend for lower results in physical (85.8 ± 33.0 vs. 47.5 ± 38.9; p = 0.1) and socio-emotional (94.7 ±17.9 vs. 73.5 ± 13.4; p = 0.1) domains. Infants who received dialysis showed 15-fold increased risk (95% CI 0.89-251) for delayed socio-emotional development (p = 0.06). Diagnosis of fetal renal failure increased the risk for delays in the adaptive domain 30-fold (95% CI 1.29-93.1; p = 0.03). Infants with abnormal renal function had 19 times (95% CI 1.95-292) increased risk for delays in the physical domain (p = 0.03). CONCLUSIONS: While most patients with LUTO do not exhibiting ND delays, our results support the importance of ND monitoring, especially in severe forms of LUTO, as increased severity of this condition may be associated with poorer ND outcomes.


Assuntos
Rim/diagnóstico por imagem , Malformações do Sistema Nervoso/diagnóstico por imagem , Obstrução Uretral/congênito , Adolescente , Adulto , Líquido Amniótico , Feminino , Idade Gestacional , Humanos , Recém-Nascido , Masculino , Gravidez , Estudos Retrospectivos , Índice de Gravidade de Doença , Ultrassonografia Pré-Natal , Obstrução Uretral/diagnóstico por imagem , Adulto Jovem
3.
Genet Med ; 19(4): 412-420, 2017 04.
Artigo em Inglês | MEDLINE | ID: mdl-27657687

RESUMO

PURPOSE: To investigate the utility of whole-exome sequencing (WES) to define a molecular diagnosis for patients clinically diagnosed with congenital anomalies of kidney and urinary tract (CAKUT). METHODS: WES was performed in 62 families with CAKUT. WES data were analyzed for single-nucleotide variants (SNVs) in 35 known CAKUT genes, putatively deleterious sequence changes in new candidate genes, and potentially disease-associated copy-number variants (CNVs). RESULTS: In approximately 5% of families, pathogenic SNVs were identified in PAX2, HNF1B, and EYA1. Observed phenotypes in these families expand the current understanding about the role of these genes in CAKUT. Four pathogenic CNVs were also identified using two CNV detection tools. In addition, we found one deleterious de novo SNV in FOXP1 among the 62 families with CAKUT. The clinical database of the Baylor Miraca Genetics laboratory was queried and seven additional unrelated individuals with novel de novo SNVs in FOXP1 were identified. Six of these eight individuals with FOXP1 SNVs have syndromic urinary tract defects, implicating this gene in urinary tract development. CONCLUSION: We conclude that WES can be used to identify molecular etiology (SNVs, CNVs) in a subset of individuals with CAKUT. WES can also help identify novel CAKUT genes.Genet Med 19 4, 412-420.


Assuntos
Variações do Número de Cópias de DNA , Sequenciamento do Exoma/métodos , Predisposição Genética para Doença/genética , Anormalidades Urogenitais/diagnóstico , Refluxo Vesicoureteral/diagnóstico , Adolescente , Criança , Pré-Escolar , Feminino , Fatores de Transcrição Forkhead/genética , Fator 1-beta Nuclear de Hepatócito/genética , Humanos , Lactente , Peptídeos e Proteínas de Sinalização Intracelular/genética , Masculino , Proteínas Nucleares/genética , Fator de Transcrição PAX2/genética , Linhagem , Polimorfismo de Nucleotídeo Único , Proteínas Tirosina Fosfatases/genética , Proteínas Repressoras/genética , Anormalidades Urogenitais/genética , Refluxo Vesicoureteral/genética , Adulto Jovem
4.
Pediatr Nephrol ; 31(4): 605-12, 2016 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-26525197

RESUMO

BACKGROUND: The aim of this study was to identify predictors of 'intrauterine fetal renal failure' in fetuses with severe congenital lower urinary tract obstruction (LUTO). METHODS: We undertook a retrospective study of 31 consecutive fetuses with a diagnosis of LUTO in a tertiary Fetal Center between April 2013 and April 2015. Predictors of 'intrauterine fetal renal failure' were evaluated in those infants with severe LUTO who had either a primary composite outcome measure of neonatal death in the first 24 h of life due to severe pulmonary hypoplasia or a need for renal replacement therapy within 7 days of life. The following variables were analyzed: fetal bladder re-expansion 48 h after vesicocentesis, fetal renal ultrasound characteristics, fetal urinary indices, and amniotic fluid volume. RESULTS: Of the 31 fetuses included in the study, eight met the criteria for 'intrauterine fetal renal failure'. All of the latter had composite poor postnatal outcomes based on death within 24 h of life (n = 6) or need for dialysis within 1 week of life (n = 2). The percentage of fetal bladder refilling after vesicocentesis at time of initial evaluation was the only predictor of 'intrauterine fetal renal failure' (cut-off <27 %, area under the time-concentration curve 0.86, 95 % confidence interval 0.68-0.99; p = 0.009). CONCLUSION: We propose the concept of 'intrauterine fetal renal failure' in fetuses with the most severe forms of LUTO. Fetal bladder refilling can be used to reliably predict 'intrauterine fetal renal failure', which is associated with severe pulmonary hypoplasia or the need for dialysis within a few days of life.


Assuntos
Sintomas do Trato Urinário Inferior/etiologia , Insuficiência Renal/etiologia , Obstrução Uretral/etiologia , Obstrução do Colo da Bexiga Urinária/etiologia , Sistema Urinário/anormalidades , Anormalidades Urogenitais/complicações , Anormalidades Urogenitais/diagnóstico , Anormalidades Múltiplas , Feminino , Doenças Fetais , Mortalidade Hospitalar , Humanos , Sintomas do Trato Urinário Inferior/diagnóstico , Sintomas do Trato Urinário Inferior/mortalidade , Sintomas do Trato Urinário Inferior/terapia , Pulmão/anormalidades , Pneumopatias/complicações , Masculino , Mortalidade Perinatal , Valor Preditivo dos Testes , Prognóstico , Insuficiência Renal/diagnóstico , Insuficiência Renal/mortalidade , Insuficiência Renal/terapia , Terapia de Substituição Renal , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Ultrassonografia Pré-Natal , Obstrução Uretral/diagnóstico , Obstrução Uretral/mortalidade , Obstrução Uretral/terapia , Obstrução do Colo da Bexiga Urinária/diagnóstico , Obstrução do Colo da Bexiga Urinária/mortalidade , Obstrução do Colo da Bexiga Urinária/terapia , Sistema Urinário/diagnóstico por imagem , Sistema Urinário/fisiopatologia , Anormalidades Urogenitais/mortalidade , Anormalidades Urogenitais/terapia
5.
Prenat Diagn ; 36(8): 720-5, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27247093

RESUMO

OBJECTIVE: To identify factors associated with fetal shunt dislodgement in lower urinary tract obstruction (LUTO). METHODS: We conducted a retrospective study of 42 consecutive fetuses with a diagnosis of LUTO in a tertiary fetal center between April 2013 and November 2015. Possible factors associated with prenatal shunt dislodgment were evaluated in fetuses who underwent shunt placement, including gestational age at diagnosis, gestational age at procedure, presence of 'keyhole sign', initial fetal bladder volume and wall thickness, prenatal ultrasonographic renal characteristics, amniotic fluid volume, presence of ascites prior to shunting, and type of fetal shunt. RESULTS: Nineteen (46.3%) fetuses underwent shunt placement at a median gestational age of 19 (range: 16.3-31.1) weeks. Shunt dislodgement occurred in 10 (52.6%) patients. A total of 35 procedures were performed; among which 16 (45.7%) were repeat procedures. The only prenatal factor associated with shunt dislodgement was the type of the shunt; Kaplan-Meier analysis indicated that the Rocket was associated with increased likelihood of remaining orthotopic (p = 0.04). CONCLUSION: Fetal shunt dislodgement occurs in approximately half of the patients and appears to be associated with the type of the shunt. Future research is necessary to develop better shunt systems and to investigate different fetal therapeutic approaches. © 2016 John Wiley & Sons, Ltd.


Assuntos
Drenagem/instrumentação , Doenças Fetais/cirurgia , Feto/cirurgia , Migração de Corpo Estranho/epidemiologia , Falha de Prótese , Obstrução Uretral/cirurgia , Bexiga Urinária/cirurgia , Adulto , Feminino , Doenças Fetais/diagnóstico por imagem , Idade Gestacional , Humanos , Gravidez , Estudos Retrospectivos , Fatores de Risco , Ultrassonografia Pré-Natal , Obstrução Uretral/diagnóstico por imagem , Adulto Jovem
6.
J Urol ; 194(2): 512-6, 2015 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-25701546

RESUMO

PURPOSE: Proximal hypospadias repair using a staged approach is a complex reconstructive operation with the potential for significant complications requiring repeat surgery. We report outcomes of staged hypospadias repair using transposed preputial skin flaps and factors predictive of postoperative complications. MATERIALS AND METHODS: We retrospectively analyzed patients who underwent staged proximal hypospadias repair using transposed preputial skin flaps between 2002 and 2013. Patient demographics, operative details, complications, reoperations and factors predictive of complications were reviewed. RESULTS: A total of 56 patients were identified with a mean age of 14.1 months (median 14.3) at first stage. Mean followup was 38.6 months (median 34.1). Complications requiring additional unplanned operation(s) were observed in 38 patients (68%), including fistulas in 32 (57%), diverticula in 8 (14%), meatal stenosis in 5 (9%), urethral stricture in 8 (14%) and glans dehiscence in 3 (5%). In addition, redo first stage repair was performed in 4 patients (7%). Since some patients had more than 1 complication, the total number of complications is greater than the number of patients undergoing a redo operation. On univariate analyses the use of small intestinal submucosa was significantly associated with an increased risk of fistula (91% vs 49%, p = 0.02) and urethral diverticulum (64% vs 24%, p = 0.04). Incision of the tunica albuginea of the corpora was associated with an increased likelihood of fistula (77% vs 44%, p = 0.03). Finally, patients with glans dehiscence were significantly younger at first stage (5.8 vs 14.8 months, p = 0.01). CONCLUSIONS: The reoperation rate for complications in children undergoing staged hypospadias repair using transposed preputial skin flaps is higher than previously reported.


Assuntos
Prepúcio do Pênis/transplante , Hipospadia/cirurgia , Procedimentos de Cirurgia Plástica/métodos , Complicações Pós-Operatórias/epidemiologia , Transplante de Pele/métodos , Retalhos Cirúrgicos , Seguimentos , Humanos , Incidência , Lactente , Masculino , Prognóstico , Estudos Retrospectivos , Texas/epidemiologia , Fatores de Tempo , Cicatrização
7.
J Urol ; 193(2): 662-6, 2015 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-25167992

RESUMO

PURPOSE: We assessed outcomes in children with ureterocele after transurethral incision at our institution between 2001 and 2014, focusing on end points of vesicoureteral reflux, improvement of hydronephrosis and need for second surgery. MATERIALS AND METHODS: We performed chart reviews of 83 patients, collecting information including age at transurethral incision, gender, renal anatomy, ureterocele location, indication for transurethral incision, and preincision and postincision vesicoureteral reflux and hydronephrosis status. Patients were divided into those with single system and duplex system ureteroceles, and intravesical and extravesical location for analysis. Statistical evaluations were performed with the Wilcoxon rank test and Fisher exact test. RESULTS: Transurethral incision was performed at a mean age of 34.2 months in patients with single system ureterocele and 8.9 months in those with duplex system ureterocele (p <0.0001). Cure rates (improvement of hydronephrosis and absence of vesicoureteral reflux) were 55.6% in patients with single system ureterocele and 14.9% in those with duplex system ureterocele (p = 0.0031). Rates of de novo reflux into the ureterocele moiety were 27.8% for patients with single system ureterocele and 56.2% for those with duplex system ureterocele (p = 0.0773). Patients with single system ureterocele required significantly fewer second surgeries (3.8%) than those with duplex system ureterocele (73.7%, p <0.0001). CONCLUSIONS: Patients with single system ureterocele may benefit from endoscopic incision. Transurethral incision was definitive in all such patients except 1 in our study. Although most patients with duplex system ureterocele will need a second operation, transurethral incision remains of value in this population in instances of sepsis or bladder outlet obstruction, or to facilitate planned reconstruction when the child is older.


Assuntos
Ureterocele/cirurgia , Ureteroscopia , Pré-Escolar , Feminino , Instalações de Saúde , Humanos , Hidronefrose/etiologia , Hidronefrose/cirurgia , Lactente , Masculino , Reoperação , Estudos Retrospectivos , Resultado do Tratamento , Ureterocele/complicações , Refluxo Vesicoureteral/etiologia , Refluxo Vesicoureteral/cirurgia
8.
Pediatr Endocrinol Rev ; 12(4): 373-87, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-26182482

RESUMO

Androgen insensitivity syndrome (AIS) is an undervirilization syndrome in individuals with 46, XY karyotype. The undervirilization can be complete feminization or incomplete virilization with grades of ambiguity. AIS is caused by mutations in the androgen receptor, resulting in resistance to the physiologic activities of androgens. Differing degrees of resistance lead to three phenotypes: a complete form with female-appearing external genitalia, a partial form with a wide range of virilization, and a mild form with only minor undervirilization. AIS presents different challenges depending on whether resistance is complete or partial. Challenges include sex assignment, which impacts other medical decisions such as gonadectomy, hormonal replacement, and other surgical interventions. This review describes medical, psychosocial, and ethical concerns for each stage of development in complete and partial AIS, from the neonatal period to adulthood. These aspects of care should be addressed within an ethical framework by a multidisciplinary team, with the patients and families being the stakeholders in the decision-making process. We use the GRADE system when appropriate to appraise the existing evidence and provide recommendations and guidelines for management of AIS and appropriate transition of patients from pediatric to adult care.


Assuntos
Síndrome de Resistência a Andrógenos/terapia , Adolescente , Adulto , Síndrome de Resistência a Andrógenos/fisiopatologia , Síndrome de Resistência a Andrógenos/psicologia , Androgênios/uso terapêutico , Criança , Pré-Escolar , Revelação , Transtornos do Desenvolvimento Sexual , Estrogênios/uso terapêutico , Feminino , Genitália , Gônadas/cirurgia , Humanos , Lactente , Recém-Nascido , Consentimento Livre e Esclarecido , Masculino , Neoplasias/etiologia , Fenótipo , Puberdade , Fatores de Risco , Procedimentos de Readequação Sexual , Fatores de Tempo
9.
Pediatr Endocrinol Rev ; 12(1): 46-54, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-25345085

RESUMO

Hypospadias is a common congenital malformation in males, the cause of which may be genetic, hormonal, or environmental, although it usually is idiopathic or possibly multifactorial. Determining the optimal diagnostic testing and management remains a challenge. Hypospadias is corrected with surgery, and androgen therapy often is used preoperatively as an adjunctive therapy, although its use, timing, and effectiveness are unclear. No standardized approach has been established for the diagnostic testing for hypospadias or for preoperative androgen therapy. We reviewed current literature and used the Grading of Recommendation, Assessment, Development, and Evaluation (GRADE) system to assess the quality of evidence and provide recommendations for a diagnostic testing algorithm from an endocrine and genetic perspective and for the optimal use of preoperative androgen therapy. These recommendations are an important step towards standardizing the use of diagnostic testing and the management of patients with hypospadias.


Assuntos
Androgênios/uso terapêutico , Hipospadia/diagnóstico , Hipospadia/terapia , Humanos , Hipospadia/genética , Masculino , Resultado do Tratamento
10.
J Urol ; 186(4 Suppl): 1601-5, 2011 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-21855921

RESUMO

PURPOSE: Cryptorchidism is a common finding in infants and young boys. Early repair lessens the extent of testicular injury. We hypothesized that anatomical and socioeconomic factors affect the timing of consultation and treatment for boys with cryptorchidism. MATERIALS AND METHODS: Under an institutional review board approved protocol we reviewed the records at a single institution of children who underwent exploration for unilateral or bilateral cryptorchidism. Demographic and anatomical factors were recorded. RESULTS: The median age of 677 boys at consultation and surgery was 20.3 and 28.9 months, respectively. Median age at consultation for boys with nonpalpable and palpable testicles was 12.3 and 20.9 months, respectively (p = 0.03). Boys with a concomitant penile anomaly had a younger median age at consultation than boys without a penile anomaly (8.5 vs 20.3 months, p <0.01). Demographic factors did not vary with respect to time to consultation and surgery (p >0.05). Multivariate analysis showed that abdominal site and concomitant penile anomaly were associated with earlier time to consultation (p = 0.02 and <0.01, respectively). CONCLUSIONS: The timing of consultation for boys with undescended testicles does not vary in regard to race, language or insurance type at this tertiary care institution. Instead, anatomical factors influenced age at consultation for boys with cryptorchidism. This suggests that in some geographic regions access to care is not restricted for minorities or noncommercially insured children.


Assuntos
Efeitos Psicossociais da Doença , Criptorquidismo/diagnóstico , Diagnóstico Precoce , Pênis/anatomia & histologia , Encaminhamento e Consulta/economia , Testículo/anatomia & histologia , Fatores Etários , California/epidemiologia , Pré-Escolar , Criptorquidismo/epidemiologia , Criptorquidismo/cirurgia , Seguimentos , Humanos , Incidência , Lactente , Cobertura do Seguro/estatística & dados numéricos , Masculino , Orquidopexia/métodos , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Fatores Socioeconômicos , Fatores de Tempo
11.
J Urol ; 186(4 Suppl): 1693-8, 2011 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-21862074

RESUMO

PURPOSE: Recent comparisons of the impact of the surgical approach on pediatric pyeloplasty outcomes have generally incorporated a form of internal ureteral drainage. We hypothesized that the surgical approach does not affect outcomes in children who undergo unstented pyeloplasty and stenting offers no long-term benefit in those with pediatric pyeloplasty. MATERIALS AND METHODS: After receiving institutional review board approval we examined the records of all children who underwent initial pyeloplasty from December 2001 to December 2009. We compared unstented and stented pyeloplasties, and each surgical approach in the unstented group. RESULTS: During the study period 367 pyeloplasties were performed, including 231 unstented and 136 stented procedures. When comparing unstented to stented pyeloplasties, there was no difference in the complication or failure rate. Of unstented pyeloplasties 71, 67 and 93 were done using a transperitoneal laparoscopic approach, a flank approach and dorsal lumbotomy, respectively. There were 5 failures, of which 2 were laparoscopic, 2 used a flank approach and 1 used dorsal lumbotomy (p = 0.51). A total of 31 patients, including 10 treated with a laparoscopic approach, 3 with a flank approach and 18 with dorsal lumbotomy (p = 0.02), required second procedures, of which 24 were temporary drainage for a urine leak. Univariate analysis of factors associated with secondary procedures in the unstented pyeloplasty group showed that only surgical approach was significant (p = 0.05). CONCLUSIONS: In pediatric pyeloplasty there is no significant difference in outcome between stented and unstented repairs. In unstented repairs complications may vary by surgical approach. Regardless of the approach unstented pyeloplasty is safe and effective in the pediatric population.


Assuntos
Pelve Renal/cirurgia , Obstrução Ureteral/cirurgia , Derivação Urinária/métodos , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Laparoscopia , Masculino , Complicações Pós-Operatórias , Estudos Retrospectivos , Stents , Fatores de Tempo , Resultado do Tratamento , Procedimentos Cirúrgicos Urológicos/métodos
12.
Eur J Obstet Gynecol Reprod Biol ; 262: 118-123, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34010724

RESUMO

OBJECTIVES: To evaluate a novel scoring system that combines several prenatal parameters for selecting ideal candidates for fetal intervention, and for predicting postnatal survival in patients with severe fetal lower urinary tract obstruction (LUTO). METHODS: We retrospectively reviewed all cases of severe LUTO evaluated for fetal intervention in a single large fetal center between January 2013 and December 2017. A scoring system for determining fetal candidacy for intervention was retrospectively developed based on postnatal outcomes. The proposed scoring system included fetal urinary biochemistry, renal ultrasound parameters, initial bladder volume, and degree of bladder refill. Relevant demographic characteristics, ultrasound reports and laboratory results were reviewed. Receiver operating characteristic (ROC) curves were used to select the cut-off values for initial bladder volume and degree of bladder refill and to evaluate the performance of the scoring system in predicting postnatal death. RESULTS: Of the 79 LUTO patients evaluated, 31 were eligible for the study. The overall 6-month postnatal survival was 64.5 % (20/31). A scoring system (0-8) was suggested with 2 points for unfavorable biochemistry, 4 points for ultrasound evidence of dysplastic kidneys, 1 point for inadequate initial bladder volume and 1 point for inadequate bladder refill. Scores>3 (N = 7) were associated with 0 % 6-month survival. The ROC curve for predicting postnatal mortality showed area under curve (AUC) of 0.82 (95 % CI 0.65-0.99). Subgroup analysis within subjects who underwent fetal intervention (N = 22) also confirmed the significance of the distribution of the scoring system between groups who survived and those who did not after adjustment for GA at delivery (p = 0.01). CONCLUSION: We propose a novel scoring system for antenatal evaluation of patients with severe LUTO which may be useful in selecting those candidates most appropriate for intervention and in counseling parents about predicted postnatal outcome.


Assuntos
Doenças Fetais , Obstrução Uretral , Feminino , Humanos , Gravidez , Estudos Retrospectivos , Ultrassonografia Pré-Natal
13.
J Urol ; 184(4 Suppl): 1589-93, 2010 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-20728107

RESUMO

PURPOSE: Parental decision making in children with vesicoureteral reflux has potentially become more complex with the evolution of ethnic diversity in the United States, the Internet, the publication of contradictory clinical data and the emergence of minimally invasive surgery. We performed a cross-sectional study of parental management for pediatric vesicoureteral reflux. MATERIALS AND METHODS: We administered a 26-item questionnaire to parents of children with vesicoureteral reflux seen at Texas Children's Hospital urology offices or undergoing antireflux surgery at that institution. Univariate and multivariate analysis was done on patient disease characteristics, demographics, predicted reflux duration, surgery success rate, antibiotic cessation, complication risk, financial considerations, urologist recommendations, Internet information, friend recommendations, and postoperative voiding cystourethrography, renal ultrasound and recovery. RESULTS: Enrolled in the study were 15 boys and 49 girls with a mean age of 3.5 years and a mean reflux grade of 2.8. Of the cases 37 were bilateral. Parents chose endoscopic treatment in 38 children, open ureteroneocystostomy in 9, antibiotic prophylaxis in 14 and observation without antibiotics in 3. Univariate analysis suggested that Hispanic parents rated ultrasound and financial considerations as more important than white parents (p <0.05). Multivariate analysis revealed that differences seen on univariate analysis may have been due to an association between race and income. Finally, 93.6% of parents rated urologist opinion as very or extremely important. CONCLUSIONS: Data indicate that the parents of our patients highly value the opinion of the pediatric urologist when choosing treatment for their children with vesicoureteral reflux. Despite social changes the physician-parental relationship remains critical. Differences in parental decision making may be linked to associations between race and income.


Assuntos
Tomada de Decisões , Pais/psicologia , Refluxo Vesicoureteral/terapia , Pré-Escolar , Estudos Transversais , Feminino , Hispânico ou Latino , Humanos , Renda , Masculino , Inquéritos e Questionários , População Branca
14.
J Urol ; 184(4 Suppl): 1743-7, 2010 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-20728168

RESUMO

PURPOSE: Testicular torsion is a true urological emergency. We determined whether a delay in treatment due to hospital transfer or socioeconomic factors would impact the orchiectomy rate in children with this condition. MATERIALS AND METHODS: We retrospectively evaluated the records of boys seen at a single institution emergency department who proceeded to surgery for a diagnosis of acute testicular torsion from 2003 to 2008. Charts were reviewed for transfer status, symptom duration, race, insurance presence or absence and distance from the hospital. Orchiectomy specimens were evaluated for histological confirmation of nonviability. RESULTS: We reviewed 97 records. The orchiectomy rate in patients who were vs were not transferred to the emergency department was 47.8% vs 68.9%, respectively (p = 0.07). Symptom duration was greater in the orchiectomy group with a mean difference of 47.9 hours (p <0.01). The mean transfer delay was 1 hour 15 minutes longer in the orchiectomy group (p = 0.01). Boys who underwent orchiectomy were 2.2 years younger than those who avoided orchiectomy (p = 0.01). Multivariate analysis showed that symptom duration and distance from the hospital were the strongest predictors of orchiectomy. CONCLUSIONS: Data suggest that torsion is a time dependent event and factors that delay time to treatment lead to poorer outcomes. These factors include distance from the hospital and the time delay associated with hospital transfer.


Assuntos
Tratamento de Emergência/estatística & dados numéricos , Orquiectomia/estatística & dados numéricos , Transferência de Pacientes , Torção do Cordão Espermático/cirurgia , Doença Aguda , Adolescente , Criança , Humanos , Masculino , Estudos Retrospectivos , Fatores Socioeconômicos , Fatores de Tempo
15.
J Pediatr Surg ; 2020 Jan 09.
Artigo em Inglês | MEDLINE | ID: mdl-31955989

RESUMO

PURPOSE: Robot-assisted laparoscopic extravesical ureteral reimplantation has previously been described as a viable minimally invasive option to open surgery. However, concerns for robotic surgery have been raised owing to assumed higher costs and heterogeneous clinical outcomes. We hypothesized that similar hospital charges and clinical outcomes occur when comparing open and robotic cases in matched cohorts. MATERIALS AND METHODS: Open and robotic reimplantation cases from 2013 to 2015 for primary vesicoureteral reflux were matched by age using 1:1 nearest neighbor matching. The matched cohorts were analyzed and compared for their direct itemized hospital charges per surgical case, complications, and clinical outcomes. RESULTS: There were 38 patients in each group after age-matching the 135 patients. Operating room charges were higher for the robotic group compared to the open group (p=0.002), whereas pharmacy and laboratory costs were lower for the robotic group. However, there were no significant differences in total overall charges between the open and robotic groups with cystoscopy or without cystoscopy (p=0.345, p=0.533), since the median hospital stay length was shorter for the robotic group (p<0.001). Clinical success rates were identical for the two groups (open 94.8% vs robotic 94.8%). There were also no significant differences in number of complications between the two cohorts. CONCLUSIONS: This is the first age-matched study comparing hospital charges and clinical outcomes of pediatric open and robotic reimplantation. While operating room charges were higher for the robotic cohort, lower hospitalization charges led to comparable overall hospital charges, as well as equivalent clinical outcomes for both cohorts. LEVEL OF STUDY: Level III (Retrospective comparative study) TYPE OF STUDY: Retrospective Study.

16.
J Urol ; 182(1): 280-4; discussion 284-5, 2009 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-19450839

RESUMO

PURPOSE: Laparoscopic orchiopexy is a safe operation. However, the bladder can be injured during creation of the transperitoneal tunnel for the cryptorchid testis. We reviewed our experience with this complication. MATERIALS AND METHODS: We searched the operative notes of patients who had undergone laparoscopic orchiopexy between August 15, 2002 and October 1, 2008, and identified bladder injuries and their treatment. RESULTS: A total of 93 patients underwent laparoscopic orchiopexies for 101 undescended testes during the study interval, with 3 procedures resulting in bladder injuries. The 3 operations varied with regard to whether the injury was recognized intraoperatively or postoperatively, and repaired in an open or laparoscopic fashion. CONCLUSIONS: Bladder injury during laparoscopic orchiopexy is a rare but serious complication that can be managed by an open or laparoscopic approach. We recommend placement of a urethral catheter and syringe assisted drainage of all urine from the bladder at the beginning of the operation, careful perivesical dissection particularly in children with prior inguinal surgery, filling and emptying of the bladder during the procedure, and maintaining a high index of suspicion especially when hematuria is observed.


Assuntos
Criptorquidismo/cirurgia , Complicações Intraoperatórias/etiologia , Laparoscopia/efeitos adversos , Doenças da Bexiga Urinária/etiologia , Bexiga Urinária/lesões , Procedimentos Cirúrgicos Urológicos Masculinos/efeitos adversos , Pré-Escolar , Estudos de Coortes , Criptorquidismo/diagnóstico por imagem , Cistoscopia , Seguimentos , Humanos , Incidência , Lactente , Complicações Intraoperatórias/epidemiologia , Complicações Intraoperatórias/cirurgia , Laparoscópios/efeitos adversos , Laparoscopia/métodos , Masculino , Procedimentos Cirúrgicos Minimamente Invasivos/efeitos adversos , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Radiografia , Sistema de Registros , Medição de Risco , Testículo/cirurgia , Fatores de Tempo , Resultado do Tratamento , Doenças da Bexiga Urinária/epidemiologia , Doenças da Bexiga Urinária/cirurgia , Procedimentos Cirúrgicos Urológicos Masculinos/métodos
17.
Urology ; 127: 97-101, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30817958

RESUMO

OBJECTIVE: To determine the safety and efficacy of advanced practice provider (APP)-performed newborn circumcisions (NBCs), we reviewed outcomes of NBCs performed by pediatric urologists and APPs. We hypothesize comparable clinical outcomes between the groups. METHODS: All urology performed NBCs during a 5-year period were reviewed, including time surrounding implementation of the APP-led clinic. Return to emergency department (ED) rates, return to operating room (OR) rates, and intraprocedure bleeding requiring intervention were reviewed. Fisher exact and Mann-Whitney testing were utilized. RESULTS: There were no statistically significant differences in rates of intraprocedure bleeding, return to ED in 30days, return to OR for revision or other related penile surgery, or the overall number of patients with complications between the groups. Thirteen patients had complications in the APP cohort, compared to 8 in the urologist cohort. There was a difference in age and weight, with urologists performing NBCs on older and heavier patients. There was no difference in clinical outcomes between children over and under 10 pounds (4.5 kg). There was a significant difference in the need for revision circumcision when comparing children older vs younger than 30days (1.9% vs 0%, P = 0.034). CONCLUSION: An APP-led NBC clinic is both safe and feasible. The widely used age and weight cutoffs for NBC need to be further evaluated, as there was no significant difference in clinical outcomes. This practice design provides pediatric urologists more time to focus on the most complex patients, both in the clinic and OR.


Assuntos
Instituições de Assistência Ambulatorial/normas , Procedimentos Cirúrgicos Ambulatórios/métodos , Circuncisão Masculina/métodos , Circuncisão Masculina/estatística & dados numéricos , Urologistas/normas , Fatores Etários , Circuncisão Masculina/etnologia , Estudos de Coortes , Pessoal de Saúde , Humanos , Lactente , Recém-Nascido , Masculino , Dor Pós-Operatória/epidemiologia , Dor Pós-Operatória/fisiopatologia , Segurança do Paciente , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/fisiopatologia , Padrões de Prática Médica , Estudos Retrospectivos , Medição de Risco , Estatísticas não Paramétricas , Resultado do Tratamento , Estados Unidos
18.
J Pediatr Surg ; 54(4): 820-824, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30049573

RESUMO

BACKGROUND: Patients with a prenatal diagnosis of lower urinary tract obstruction (LUTO) may undergo prenatal interventions, such as vesicoamniotic shunt (VAS) placement, as a temporary solution for relieving urinary tract obstruction. A recent FDA communication has raised awareness of the potential neurocognitive adverse effects of anesthesia in children. We hypothesized as to whether a prenatal LUTO staging system was predictive of the number of anesthesia events for prenatally diagnosed LUTO patients. METHODS: We retrospectively reviewed the prenatal and postnatal clinical records for patients with prenatally diagnosed LUTO from 2012 to 2015. Patients were stratified by prenatal VAS status and by LUTO disease severity according to Ruano et al. (Ultrasound Obstet Gynecol. 2016). RESULTS: 31 patients were identified with a prenatal LUTO diagnosis, and postnatal records were available for 21 patients (seven patients in each stage). When combining prenatal and postnatal anesthesia, there was a significant difference in the number of anesthesia encounters by stage (1.6, 3.7, and 6.7 for Stage I, II, and III respectively, p = .034). Upon univariate analysis, higher gestational age (GA) at birth was associated with a decreased number of anesthesia events in the first year (p = .031). CONCLUSIONS: The majority of infants with prenatally diagnosed LUTO will undergo postnatal procedures with general anesthesia exposure in the first year of life. Patients with higher prenatal LUTO severity experienced a higher number of both prenatal and postnatal anesthesia encounters. In addition, higher GA at birth was associated with fewer anesthesia encounters in the first year. LEVEL OF EVIDENCE: This is a prognostic study with Level IV evidence.


Assuntos
Anestesia Geral/estatística & dados numéricos , Obstrução do Colo da Bexiga Urinária/cirurgia , Procedimentos Cirúrgicos Urológicos/estatística & dados numéricos , Pré-Escolar , Feminino , Idade Gestacional , Humanos , Lactente , Recém-Nascido , Masculino , Gravidez , Diagnóstico Pré-Natal/estatística & dados numéricos , Prognóstico , Estudos Retrospectivos , Sistema Urinário/anormalidades , Sistema Urinário/cirurgia
19.
Urology ; 123: 198-203, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30031832

RESUMO

OBJECTIVE: To evaluate the management and clinical outcomes of nonfunctioning upper pole moieties treated with either upper pole heminephrectomy or upper pole preservation with lower ureteral reconstruction at a single tertiary institution. METHODS: After Institutional Review Board (IRB) approval, patients with duplicated systems undergoing upper pole heminephrectomy, ureteroureterostomy, or common sheath ureteral reimplantation from 2012-2017 were identified. Only patients with a nonfunctioning upper pole moiety on ultrasound or renal scan were included. Patients undergoing upper pole heminephrectomy were compared to those undergoing upper pole preservation with respect to demographics, anatomic variations preoperatively, and postoperative outcomes. RESULTS: Twenty-seven (57%) patients underwent upper pole preservation with lower ureteral reconstruction; 20 (43%) patients underwent upper pole heminephrectomy. Patients undergoing lower ureteral reconstruction were older (1.63 vs 2.76 years, P = .018) and more commonly presented with lower pole vesicoureteral reflux (67% vs 25%, P = .008). No significant difference in postoperative complications was seen between the two groups. After ureteroureterostomy, one patient developed new onset symptomatic reflux to the upper pole requiring intravesical reimplantation. In the heminephrectomy group, 4 of 11 patients with ureteroceles had ureterocelectomy with concomitant lower pole reimplantation. After heminephrectomy, two additional patients required further interventions: ureterocele excision and transurethral polyp excision. CONCLUSION: For patients with nonfunctional upper poles, lower tract reconstruction is a safe alternative to upper pole heminephrectomy. No significant difference in outcomes was seen. Considering that nearly 1 of 3 of patients with upper pole heminephrectomy required additional lower urinary tract procedures, pursuing upper pole preservation with lower urinary tract reconstruction may be favorable.


Assuntos
Nefrectomia , Avaliação de Resultados em Cuidados de Saúde , Ureter/anormalidades , Ureter/cirurgia , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Procedimentos Cirúrgicos Urológicos/métodos
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