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1.
J Pediatr Urol ; 2024 May 15.
Artigo em Inglês | MEDLINE | ID: mdl-38782683

RESUMO

INTRODUCTION & BACKGROUND: Standard urotherapy is a well-established treatment for children with incontinence, although it is often challenging for both child and parents, and not always successful. As an alternative, several in- and outpatient bladder training programs have shown positive results on achieving continence. However, the disadvantage is the hospital environment, which can be more stressful for the child, and also quite expensive for society. OBJECTIVE: The aim was to evaluate the outcome on achieving continence following a voiding camp, where standard urotherapy was applied during a one-week stay at a regular summer youth camp, outside the hospital. STUDY DESIGN: Retrospective analysis of 105 children with urinary incontinence, followed in an expert centre for urinary incontinence for at least one year. Data at 7 different time points, before, during and until 6 months after voiding camp were collected. RESULTS: Even though all children had regular follow-up in an expert centre for urinary incontinence for at least one year before participating voiding camp, only 15% of the children reached the recommended amount of daily fluid intake (1.5 L/day). Once minimal daily fluid intake was re-established during the voiding camp, an immediate increase in the maximum voided volume (MVV), and a decrease in the number of wet days and wet nights per week was noted. This effect on a higher MVV remained even 3 months after voiding camp. DISCUSSION: Although sufficient daily fluid intake is a well-established part of standard urotherapy, up until now there was no data that proved the positive impact of sufficient daily fluid intake on bladder volume training and achieving continence in children. CONCLUSION: Voiding camp, as an unique bladder rehabilitation program for children with incontinence, is a successful alternative treatment option. Optimizing the daily fluid intake during voiding camp had a major positive impact on bladder volume training and achieving continence in children.

2.
World J Urol ; 42(1): 34, 2024 Jan 13.
Artigo em Inglês | MEDLINE | ID: mdl-38217728

RESUMO

OBJECTIVE: To identify any self-reported differences or attitudes towards certification, publication, or practice patterns between adult urology and paediatric general surgery-trained paediatric urology providers. There are no known published differences in clinical/operative/research outcomes in either group. METHODS: An 18-item cross-sectional survey was compiled through the EAU Young Academic Urologists (YAU) office and disseminated to a trans-Atlantic convenience sample of current practising paediatric urologists. This was created using a mini-Delphi method to provide current semi-quantitative data relating to current opinions and attitudes of this cohort. RESULTS: A total of 228 respondents completed the survey, with female respondents representing 37% and 34% for urology and paediatric general surgery, respectively. Nearly 90% overall respondents felt that a full 2-year paediatric fellowship program was very important and 94% endorsed a collaborative dedicated paediatric urology on call service, with 92% supporting the joint development of transitional care. Urology managed higher numbers of bedwetting (p = 0.04), bladder bowel dysfunction (p = 0.02), endourological procedures (p = 0.04), and robotics (p = 0.04). Paediatric general surgery managed higher numbers of laparoscopic reconstruction (p = 0.03), and posterior urethral valve ablation (p = 0.002). CONCLUSION: This study represents the first time that a cross-sectional cohort of paediatric urologists from different training backgrounds were compared to assess their productivity, practice patterns and attitudes. Paediatric urology is in a unique position to have two contributing specialities, with the ability to provide optimal transitional and lifelong care. We believe that there should be a strong emphasis on collaboration and to remove any historically-created barriers under policies of equity, diversity and inclusivity.


Assuntos
Doenças Urológicas , Urologia , Adulto , Humanos , Criança , Feminino , Urologia/educação , Estudos Transversais , Urologistas , Inquéritos e Questionários
3.
J Urol ; 210(6): 899-907, 2023 12.
Artigo em Inglês | MEDLINE | ID: mdl-37747130

RESUMO

PURPOSE: Bladder and bowel dysfunction is a common but underdiagnosed pediatric entity which may represent up to 47% of pediatric urology consults. The objectives of this observational study were to determine functional 1-year outcomes following standard treatment of bladder and bowel dysfunction in both control and neuropsychiatric developmental disorder groups using validated questionnaires, and to perform an initial cost analysis. MATERIALS AND METHODS: This was a prospective observational study conducted across a number of academic European centers (July 2020-November 2022) for new bladder and bowel dysfunction patients. Parents completed a sociodemographic survey, information pertaining to prior neuropsychiatric developmental disorder diagnoses, as well as a number of validated functional scores. RESULTS: A total of 240 patients were recruited. In the control bladder and bowel dysfunction group, the baseline Dysfunctional Voiding Scoring System and Childhood Bladder and Bowel Dysfunction Questionnaire scores were 20% and 17.% lower, respectively, after 1 year compared to the neuropsychiatric developmental disorder group. The change in improvement was diminished for the neuropsychiatric developmental disorder cohort in both Dysfunctional Voiding Scoring System and Childhood Bladder and Bowel Dysfunction Questionnaire scores. The odds ratio of full symptom resolution was 5.7 in the control cohort compared to the neuropsychiatric developmental disorder cohort. A cost analysis on prescribed medications at referral led to a total cost of €32,603.76 (US $35,381.00) in the control group and €37,625.36 (US $40,830.00) in the neuropsychiatric developmental disorder group. CONCLUSIONS: This study demonstrates that pediatric patients with a neuropsychiatric developmental disorder exhibit more severe bladder and bowel dysfunction at baseline and throughout treatment with a lower overall quality of life, as well as 15.4% higher medication costs at referral. It is also important that parents' and caregivers' expectations are managed regarding higher levels of treatment resistance for functional bladder and bowel issues.


Assuntos
Enteropatias , Doenças da Bexiga Urinária , Criança , Humanos , Constipação Intestinal , Deficiências do Desenvolvimento/complicações , Estudos Prospectivos , Qualidade de Vida , Bexiga Urinária , Doenças da Bexiga Urinária/complicações , Doenças da Bexiga Urinária/terapia , Doenças da Bexiga Urinária/diagnóstico
4.
J Pediatr Urol ; 19(4): 430.e1-430.e8, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-37173199

RESUMO

INTRODUCTION: Complex urological anomalies often require continued care as patients reach adulthood. Adequate transition for adolescents with ongoing urological care needs is critical to allow for seamless care in adult hospitals. Studies have shown that this can lead to improved patient and parental satisfaction, and lower utilisation of unplanned inpatient beds and emergency department visits. There is currently no ESPU-EAU consensus on the adequate mechanism and very few individual papers examining the role of urological transition for these patients in a European setting. This study aimed to identify current practice patterns in paediatric urologists providing adolescent/transitional care, to assess their opinions towards formal transition and to look for variations in care. This has implications for long-term patient health and specialist care. METHODS: An 18-item cross-sectional survey was compiled and pre-approved through the EAU-EWPU and ESPU board offices prior to dissemination to all registered ordinary members affiliated with the ESPU. This was created using a mini-Delphi method through the EWPU research meetings to provide current semi-quantitative data relating to current opinions and attitudes of this cohort. RESULTS: A total of 172 respondents (55% paediatric general surgery; 45% urology) across 28 countries completed the survey. The majority of respondents were in practice >10 years and spent >80% time in paediatric urology. There was no formal transition process according to 50% respondents and over half of those that did have less than 1/month, with <10% using validated questionnaires. More than two-thirds respondents continued to provide care after transition, as >70% units had no designated corresponding adult service. Furthermore, 93% paediatric believe a formal transition service to be very important, using a multidisciplinary framework. A pareto chart demonstrated 10 specific conditions to be of most interest in transition to adulthood. CONCLUSION: This is the first study to assess the requirements of paediatric urologists for adequate transitional care, however due to the nature of the survey's distribution, this was a non-scientific poll based on a convenience sample of respondents. It is critical that dual-trained or adult-trained urologists with a specific interest in paediatric urology work with current paediatric urologists in a multidisciplinary fashion to facilitate early transition based on the adolescent's developmental and biopsychosocial requirements. National urological and paediatric surgical societies need to make transitional urology a priority. The ESPU and EAU should collaboratively consider developing transitional urology guidelines to allow a framework by which this can occur.


Assuntos
Cuidado Transicional , Urologia , Adulto , Humanos , Criança , Adolescente , Urologistas , Estudos Transversais , Urologia/métodos , Inquéritos e Questionários
5.
J Pediatr Urol ; 19(4): 487-488, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-37150636

RESUMO

INTRODUCTION: The bladder-Exstrophy-Epispadias complex (BEEC) contains a wide spectrum of congenital malformations. A treatment naïve refugee was referred to our center with what was identified as BEEC. MATERIALS AND METHODS: A 27-year-old female patient was referred for total incontinence since birth by the general practitioner from the refugee center. An exstrophic bladder with blind ending ureteral orifices and a second non-exstrophic bladder with two orthotopic ureters was identified, demonstrating the bladder duplication in the sagittal plane. Laparotomy was performed, dissecting the exstrophic bladder plate caudally and using it as a ventral onlay to augment the non-exstrophic bladder. A Mitchell-type bladder neck reconstruction was performed with an autologous fascia sling around the bladder neck to obtain continence. As the patient had never voluntarily voided, chances of spontaneous voiding after surgery were low. Therefore creation of a continent Mitrofanoff-type vesicostomy was additionally realized and genital reconstruction was achieved. RESULTS: 12 months post operatively, the patient was completely continent, had a bladder capacity of 250 ml, and performed self-catheterization 5 times a day. No post-operative complications were observed. CONCLUSION: Admission of political refugees can implicate challenging surgeries for congenital malformations in adults, such as BEEC. This demonstrates the importance of multidisciplinary transitional care.


Assuntos
Extrofia Vesical , Epispadia , Incontinência Urinária , Feminino , Adulto , Humanos , Bexiga Urinária/cirurgia , Extrofia Vesical/cirurgia , Extrofia Vesical/complicações , Incontinência Urinária/etiologia , Epispadia/diagnóstico , Epispadia/cirurgia , Epispadia/complicações
6.
J Pediatr Urol ; 19(1): 146-147, 2023 02.
Artigo em Inglês | MEDLINE | ID: mdl-36283947

RESUMO

INTRODUCTION: Patient/parent education and participation helps improve post-operative care. Dressing and catheter care after hypospadias surgery varies widely and young parents are keen to use available media when seeking for help, especially if surgery is done in an outpatient setting. An information video about post-operative care after hypospadias is made available through a tertiary referral hospital's website. MATERIALS AND METHODS: Hypospadias surgery is an outpatient surgery in our setting. A double diaper system is used to prevent contamination of the urinary catheter and penile bandage with stools. A video explaining how the dressing works was made, helping parents maintaining the dressing and thereby solving possible questions/problems about dressing or medications while at home. Warning signs and symptoms are mentioned in which case parents should contact the urologist: fever, continuous blood loss and lack of urinary output. RESULTS: An information folder summarizing the latter, including a link to the video is given to all parents pre-operatively, providing reassurance according to parents' feedback. CONCLUSION: Parent participation can help improve post-operative care, especially in outpatient clinic setting. This video helps parents through post-operative care after hypospadias surgery. Its availability through informative folders and hospital's website is destined to educate and reassure parents.


Assuntos
Hipospadia , Masculino , Humanos , Criança , Hipospadia/cirurgia , Pênis/cirurgia , Pais , Bandagens , Cuidados Pós-Operatórios
7.
J Pediatr Urol ; 18(5): 706-707, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-36075828

RESUMO

INTRODUCTION: Duplication of the urethra is a rare congenital anomaly in females which, if left unidentified, might cause refractory urinary incontinence. This video demonstrates its identification and treatment. PATIENT & METHODS: A 5-year-old female was referred for primary refractory diurnal incontinence without any dry moment since birth. Initial clinical examination showed permanent urine leakage from an opening cranial to the clitoris. Ultrasound did not show any bladder or kidney abnormalities. A diagnostic cystoscopy under anesthesia revealed an accessory meatus located cranially to the orthotopic meatus and to the clitoris. RESULTS: Lying in dorsal decubitus under general anesthesia, catheters were inserted in respectively the orthotopic and accessory meatus. The accessory meatus and urethra were dissected away into the bladder dome from a circumferential skin incision. Defects were closed and bladder drainage per urethram was left in situ for 10 days. P ostoperative recovery was uncomplicated. Complete resolution of the incontinence was immediately achieved at catheter removal. Daytime and night-time continence are observed with 3 years follow-up. CONCLUSION: Urethral duplication is a rare finding in females but should be excluded during the workup of primary incontinence. In girls with isolated urethral duplication, resection of the accessory urethra is the best surgical option.


Assuntos
Doenças Uretrais , Incontinência Urinária , Humanos , Feminino , Pré-Escolar , Uretra/diagnóstico por imagem , Uretra/cirurgia , Uretra/anormalidades , Bexiga Urinária/cirurgia , Incontinência Urinária/etiologia , Incontinência Urinária/cirurgia , Cistoscopia
8.
J Pediatr Urol ; 17(3): 316-325, 2021 06.
Artigo em Inglês | MEDLINE | ID: mdl-33846072

RESUMO

BACKGROUND: The variability of the urethral plate (UP) characteristics is one of the factors that influence technical choices for hypospadias correction. However, it is difficult to objectively evaluate the UP, leading to controversies in this subject, and vague terms utilized in the literature to describe its characteristics. OBJECTIVE: We aim to analyze the previously described methods used to characterize and evaluate UP quality, emphasizing the pros and cons of each system, and highlighting its possible influence on different postoperative outcomes. METHODS: We searched the databases PubMed, Embase, and Cochrane Library CENTRAL from January 1, 2000 to August 20, 2020. The following concepts were searched: urethra reconstruction/urethra replacement/urethroplasty AND hypospadias/hypospadias, AND children AND "plate" with the gray literature search. Subgroup analyses were also carried out. The quality of the involved studies was reviewed operating a modified version of the Newcastle-Ottawa Scale (NOS). RESULTS: 996 citations perceived as relevant to screening were retrieved. Thirteen studies were included comprising a total of 1552 cases. The number of patients in each study varied between 42 and 442, and the average post-surgical follow-up duration ranged between 6 months and twenty-six months. All studies used postoperative urethral stents of variable sizes and types. The impact of UP was most frequently assessed for cases treated with the tubularized incised plate (TIP) repair. CONCLUSION: The UP quality seems to play a role as an independent factor influencing postoperative outcomes of hypospadias repair. Currently used strategies for the appraisal of UP quality are highly subjective with a low index of generalizability. Various attempts to overcome these limitations exist but none was consistently accepted, leaving a wide space for creative investigation in order to obtain an objective, reproducible, precise, and well-validated tool.


Assuntos
Hipospadia , Procedimentos de Cirurgia Plástica , Criança , Humanos , Hipospadia/cirurgia , Lactente , Masculino , Stents , Resultado do Tratamento , Uretra/cirurgia , Procedimentos Cirúrgicos Urológicos Masculinos
9.
J Pediatr Urol ; 16(1): 43.e1-43.e8, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31810878

RESUMO

INTRODUCTION: The androgen receptor (AR) plays an important role in the development of male genitalia, and impaired androgen signalling has been hypothesised to underlie congenital penile malformations (CPM) such as hypospadias. Previous studies exploring the role of AR expression in the development of CPM have yielded conflicting results. OBJECTIVES: To assess AR expression in human foreskin of boys/men born with hypospadias, buried penis versus controls. STUDY DESIGN: Foreskin samples of 428 boys and men undergoing primary penile surgery (198 controls, 197 hypospadias, and 33 buried penis) were collected between October 2013 and July 2018. AR staining was performed in all samples and semi-quantitatively scored by two researchers independently, using a modified quick score (mQuicks) that assesses the proportion and intensity of AR staining in smooth muscle fibres. RESULTS: The interobserver variability of the mQuicks had a high level of agreement for the total score, as well as for the subscores. Two phases of high AR expression were observed in all groups, the first following the postnatal gonadotropin surge (i.e., mini-puberty) and the second in (pre-) puberty. No differences in AR expression were found in hypospadias or buried penis cases as compared to controls matched for age at time of surgery. DISCUSSION: This study describes the physiological evolution in AR expression in the human foreskin of boys with CPM and explains the cause of the previously reported, conflicting results. Despite the very large cohort, the limitations of this study are the low number of cases younger than six months at the time of surgery and the lack of Tanner stages to correlate with the mQuicks in adolescents. CONCLUSIONS: The mQuicks is a straightforward and informative tool to semi-quantitatively assess AR expression in the dartos tissue. In this study, AR expression in human foreskin shows a bimodal distribution in boys with CMP and controls, following physiological androgen exposure. No statistically significant difference in AR expression could be found between both groups. Whether other local mechanisms are affected by these physiological changes is currently unclear. However, strict age-matching should be considered when exploring the mechanisms underlying disturbed penile and urethral development in CMP.


Assuntos
Prepúcio do Pênis/anormalidades , Prepúcio do Pênis/metabolismo , Hipospadia/etiologia , Receptores Androgênicos/biossíntese , Criança , Pré-Escolar , Correlação de Dados , Humanos , Lactente , Masculino , Estudos Prospectivos , Receptores Androgênicos/fisiologia
10.
J Pediatr Urol ; 15(4): 322-329, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31227314

RESUMO

The investigation, management and follow-up of paediatric ureteropelvic junction obstruction is not standardized. The Young Pediatric Urology Committee of the European Society of Pediatric Urology interviewed five experts in the field on various aspects of management and compared this with published literature.


Assuntos
Gerenciamento Clínico , Laparoscopia/métodos , Procedimentos de Cirurgia Plástica/métodos , Obstrução Ureteral/cirurgia , Procedimentos Cirúrgicos Urológicos/métodos , Criança , Humanos , Pelve Renal , Imageamento por Ressonância Magnética , Ultrassonografia , Obstrução Ureteral/diagnóstico
11.
J Pediatr Urol ; 15(1): 87-88, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30473475

RESUMO

INTRODUCTION: Failure of kidney migration during embryonic life results in an ectopic kidney, with an incidence varying from 1 in 500 to 1 in 1200. Pelvic kidney can be a rare cause of recurrent urinary tract infection (UTI), warranting nephrectomy in some cases. MATERIAL AND METHODS: A 7-year-old girl with a history of recurrent UTIs and vaginal discharge was diagnosed with a dysplastic afunctional ectopic kidney located in the pelvis, with ureter draining into the cervix or vaginal wall. RESULTS: A robot-assisted approach was chosen, with side docking of the robot to allow concurrent vaginoscopic/hysteroscopic exploration. A uterus bicornis was found. The ectopic ureter was dissected toward its drainage in the vaginal wall, where it was sutured and resected. The dysplastic kidney and ureter were removed. Compared to pure laparoscopic approach, dissection deep into the pelvis toward the vaginal wall is aided by the robotic dexterity and facilitates complete resection of the structure, which avoids leaving a ureteric stump into the vaginal wall. CONCLUSIONS: Robot-assisted surgery is a safe and effective option for resection of ectopic kidneys with ectopic ureter in children. Dissection up until the vaginal wall in children is aided by robotic dexterity, making this kind of surgery the ideal indication for robotic-assisted surgery.


Assuntos
Anormalidades Múltiplas/cirurgia , Rim/anormalidades , Rim/cirurgia , Procedimentos Cirúrgicos Robóticos , Ureter/anormalidades , Ureter/cirurgia , Criança , Feminino , Humanos , Procedimentos Cirúrgicos Urológicos/métodos
12.
J Pediatr Urol ; 15(1): 71.e1-71.e6, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30473476

RESUMO

INTRODUCTION: Re-ascended testes account for a proportion of all undescended testes (UDTs); one main hypothesis relating to their etiology relates to a patent processus vaginalis peritonei. The aim was to investigate gubernacular insertion points in boys with late ascended testis as a possible guide to an alternative embryological etiology. PATIENTS AND METHODS: Patients with proven ascended testes were recruited from four different pediatric urology centers between May 2016 and September 2017. All patients were evaluated regarding their gubernacular insertion during orchidopexy. The presence of accompanying patent processus vaginalis and the association between the epididymis and testis were also documented. RESULTS: Seventy-seven children (mean age = 73.1 ± 41.2 months [range 18-176]) were enrolled into the study. A non-orthotopic gubernacular insertion point was found in 96.1% (n = 74); 34.2% (n = 26) of these were located in the groin and 63.2% (n = 48), high within the scrotum. Figure A. An open processus vaginalis peritonei was found in 35.1%. Twelve patients (15.6%) had small, dysplastic appearing testis with testis-epididymis dissociation. Boys with a higher insertion of the non-orthotopic gubernaculum (n = 48, groin) were operated earlier (mean age at surgery, 62.3 months) compared with those with a gubernacular insertion at a high scrotal site (mean age at surgery, 90.5 months; p = 0.004). Figure B. DISCUSSION: This study revealed that non-orthotopic gubernacular insertion is found in the vast majority of the ascending testis cases. Patent processus vaginalis was accompanying only 35.1% of all children and might be the cause of the ascending testis in this small subgroup of patients in line with the earlier reports [1]. In boys with ascending testes, in this population, the gubernaculum was very likely to insert non-orthotopically. In concordance with previous reports [2] and regarding the finding of a an earlier age at surgery in boys with higher inserting gubernacula, this could provide a logical explanation as to how these testes are initially palpable in the scrotum and then, during body growth are retracted to the groin. CONCLUSION: In 96.1% of the patients, a non-orthotopic gubernacular insertion was found. This points to embryologic etiology, complying well with earlier reports and further underlining the critical importance of timely diagnosis and treatment for this group of patients.


Assuntos
Criptorquidismo/embriologia , Gubernáculo/embriologia , Adolescente , Fatores Etários , Criança , Pré-Escolar , Gubernáculo/anatomia & histologia , Humanos , Lactente , Masculino , Estudos Prospectivos
13.
J Pediatr Urol ; 15(2): 176.e1-176.e7, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30581060

RESUMO

INTRODUCTION: Urethral injuries and trauma-related strictures (UITSs) in children are rare. The treatment is challenging but crucial to avoid life-long urinary complications such as recurrent stricture formation, urinary incontinence, and impotence. OBJECTIVE: The aim was to report on the surgical and functional outcome of urethroplasty for UITSs and to provide data on patient-reported outcome measures (PROMs). MATERIAL AND METHODS: Between November 2001 and October 2017, 18 male children (≤18 years; median: 13 years) underwent urethroplasty for UITSs at a single tertiary referral center. Etiology was iatrogenic in five (27.8%), perineal straddle injury in six (33.3%) and pelvic fracture urethral injury (PFUI) in seven (38.8%) patients. PFUIs and short (≤3 cm) bulbar strictures were treated by transperineal anastomotic repair (n = 15; 83.3%), whereas a long bulbar stricture and a penile stricture were treated by, respectively, a preputial skin graft and flap urethroplasty. A penetrating penile urethral injury during circumcision underwent early exploration with primary repair of the laceration. Failure was defined as need for additional urethral instrumentation. PROMs were sent to patients ≥16 years at the latest evaluation. RESULTS: Median follow-up was 57 (range: 8-198) months. No complications and grade 1, 2, and 3 were present in, respectively, 13 (72.2%), two (11.1%), one (5.6%), and two (11.1%) patients. The success rate in a tertiary referral center was 94.4%. An immediate failure was observed in a patient with a PFUI and concomitant bladder neck injury. PROMs were available in 12 patients. Four patients (33.3%) reported erectile dysfunction. Post-void dribbling (25%) and urgency (50%) were the most frequently reported complaints. All patients were satisfied after urethroplasty and stated that they would undergo the surgery again. DISCUSSION: This series corroborates the recent trend in favor of transperineal anastomotic repair for PFUI, with combined abdominoperineal approach reserved for complex situations (e.g. bladder neck injury). For anterior UITSs, adaption of the technique to the characteristics of UITSs (etiology, location, length, and quality of graft bed) yielded excellent outcomes. Future systematic use of PROMs is also needed in children to elucidate the impact of urethroplasty on the urinary and sexual function. CONCLUSION: External trauma is the most important etiology of UITSs, but iatrogenic causes should not be neglected. Urethroplasty, mainly by anastomotic repair (AR) but with the technique adapted to local stricture characteristics if necessary, has an excellent long-term success rate in experienced hands. Functional disturbances are frequent, but despite this, patient satisfaction is high after urethroplasty.


Assuntos
Uretra/lesões , Uretra/cirurgia , Estreitamento Uretral/cirurgia , Adolescente , Criança , Pré-Escolar , Humanos , Lactente , Masculino , Medidas de Resultados Relatados pelo Paciente , Estudos Retrospectivos , Resultado do Tratamento , Estreitamento Uretral/etiologia , Procedimentos Cirúrgicos Urológicos Masculinos/métodos , Ferimentos e Lesões/complicações
14.
Clin Genet ; 92(1): 99-103, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28032338

RESUMO

Steroidogenic factor 1 (encoded by SF1/NR5A1) is a transcription factor with multiple target genes involved in the development and function of multiple steroidogenic and non-steroidogenic tissues. NR5A1 mutations lead to several phenotypes, including sex reversal, spermatogenesis failure, premature ovarian failure and adrenocortical insufficiency. The implication of NR5A1 mutations in spleen development anomalies was recently highlighted. We provide new evidence of this involvement, describing a novel heterozygous non-sense NR5A1 mutation in a 46,XY-DSD with polysplenia female proband and her father, who had hypospadias and asplenia.


Assuntos
Insuficiência Adrenal/genética , Hipospadia/genética , Insuficiência Ovariana Primária/genética , Fator Esteroidogênico 1/genética , Adolescente , Insuficiência Adrenal/patologia , Criança , Feminino , Heterozigoto , Humanos , Hipospadia/patologia , Masculino , Mutação , Insuficiência Ovariana Primária/patologia , Processos de Determinação Sexual/genética , Espermatogênese/genética , Baço/crescimento & desenvolvimento , Baço/patologia
15.
J Pediatr Urol ; 13(1): 86-87, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-27887909

RESUMO

OBJECTIVE: To describe modification of the tubularized incised plate urethroplasty (TIP) for distal hypospadias, and assess its efficacy, and functional and cosmetic outcomes. METHODS: A prospective evaluation of a consecutive series of patients operated for primary distal hypospadias was conducted at a tertiary reference center. A standardized modification of the TIP (mTIP) procedure was performed on a 10 French catheter. Clinical data were collected in a dedicated database. Intraoperative variables, postoperative complications and outcomes, by means of uroflowmetries and a validated (HOPE) questionnaire, were assessed. Efficacy was evaluated with the reported complications: functional outcome was evaluated with uroflowmetries and cosmetic assessment by a validated questionnaire (HOPE). A descriptive statistical analysis was performed. RESULTS: Of the 112 boys operated between 30/09/2011 and 1/04/2014, 50 completed long-term follow-up with functional and esthetic evaluation, as required for inclusion. Median age at surgery was 25 months (range 14-156); median follow-up time was 21.5 months (range 6-48). Complications requiring re-intervention occurred in 2/50 boys. Uroflowmetry presented a bell-shaped curve in 47/50 boys, and the median HOPE score was 9.5 (range 7.6-10.0). CONCLUSION: The mTIP procedure provided satisfactory long-term functional and cosmetic outcomes, as validated by uroflowmetries and standardized questionnaire.


Assuntos
Hipospadia/cirurgia , Procedimentos de Cirurgia Plástica/métodos , Retalhos Cirúrgicos , Urodinâmica/fisiologia , Procedimentos Cirúrgicos Urológicos Masculinos/métodos , Pré-Escolar , Seguimentos , Humanos , Hipospadia/fisiopatologia , Lactente , Masculino , Estudos Prospectivos , Fatores de Tempo , Resultado do Tratamento
16.
J Pediatr Urol ; 12(4): 229.e1-7, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27346071

RESUMO

INTRODUCTION: Minimally invasive pyeloplasty (MIP) for ureteropelvic junction (UPJ) obstruction in children has gained popularity over the past decade as an alternative to open surgery. The present study aimed to identify the factors affecting complication rates of MIP in children, and to compare the outcomes of laparoscopic (LP) and robotic-assisted laparoscopic pyeloplasty (RALP). MATERIALS AND METHODS: The perioperative data of 783 pediatric patients (<18 years old) from 15 academic centers who underwent either LP or RALP with an Anderson Hynes dismembered pyeloplasty technique were retrospectively evaluated. Redo cases and patients with anatomic renal abnormalities were excluded. Demographics and operative data, including procedural factors, were collected. Complications were classified according to the Satava and modified Clavien systems. Failure was defined as any of the following: obstructive parameters on diuretic renal scintigraphy, decline in renal function, progressive hydronephrosis, or symptom relapse. Univariate and multivariate analysis were applied to identify factors affecting the complication rates. All parameters were compared between LP and RALP. RESULTS: A total of 575 children met the inclusion criteria. Laparoscopy, increased operative time, prolonged hospital stay, ureteral stenting technique, and time required for stenting were factors influencing complication rates on univariate analysis. None of those factors remained significant on multivariate analysis. Mean follow-up was 12.8 ± 9.8 months for RALP and 45.2 ± 33.8 months for LP (P = 0.001). Hospital stay and time for stenting were shorter for robotic pyeloplasty (P < 0.05 for both). Success rates were similar between RALP and LP (99.5% vs 97.3%, P = 0.11). The intraoperative complication rate was comparable between RALP and LP (3.8% vs 7.4%, P = 0.06). However, the postoperative complication rate was significantly higher in the LP group (3.2% for RALP and 7.7% for LP, P = 0.02). All complications were of no greater severity than Satava Grade IIa and Clavien Grade IIIb. DISCUSSION: This was the largest multicenter series of LP and RALP in the pediatric population. Limitations of the study included the retrospective design and lack of surgical experience as a confounder. CONCLUSIONS: Both minimally invasive approaches that were studied were safe and highly effective in treating UPJ obstruction in children in many centers globally. However, shorter hospitalization time and lower postoperative complication rates with RALP were noted. The aims of the study were met.


Assuntos
Pelve Renal/cirurgia , Laparoscopia , Procedimentos Cirúrgicos Robóticos , Obstrução Ureteral/cirurgia , Adolescente , Criança , Pré-Escolar , Europa (Continente) , Feminino , Humanos , Lactente , Masculino , Procedimentos Cirúrgicos Minimamente Invasivos , Complicações Pós-Operatórias/epidemiologia , Estudos Retrospectivos , Sociedades Médicas , Resultado do Tratamento , Procedimentos Cirúrgicos Urológicos/métodos , Urologia
17.
J Urol ; 195(1): 112-7, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26241906

RESUMO

PURPOSE: We prospectively compared buccal mucosa graft and lingual mucosa graft urethroplasty with respect to donor site morbidity and urethroplasty outcome. MATERIALS AND METHODS: Patients treated with buccal mucosa graft (29) or lingual mucosa graft (29) urethroplasty were included in the study. Oral pain and morbidity were assessed using the numeric rating scale (scale 0 to 10) as well as an in-home questionnaire administered 3 days, 2 weeks and 6 months postoperatively. RESULTS: After a mean (± SD) followup of 30 (± 13) months successful urethroplasty was achieved in 24 (82.8%) and 26 (89.7%) patients treated with buccal mucosa graft and lingual mucosa graft, respectively (p = 0.306). Median numeric rating scale after 3 days, 2 weeks and 6 months was 4, 2 and 0 for buccal mucosa graft and 6, 3 and 0 for lingual mucosa graft, respectively, with no statistical differences between the groups. At day 3 significantly more patients in the lingual mucosa graft group had severe difficulties with eating and drinking (62.1% vs 24.1%, p = 0.004) and speaking (93.1% vs 55.2%, p = 0.001), and had dysgeusia (48.3% vs 13.8%, p = 0.01). Two weeks postoperatively speech impairment was still more frequent with lingual mucosa graft (55.2% vs 13.8%, p = 0.002), whereas oral tightness was more frequent with buccal mucosa graft (41.4% vs 6.9%, p = 0.005). After 6 months 44.8% and 31% of patients treated with buccal mucosa graft and lingual mucosa graft, respectively, still reported sensitivity disorders (p = 0.279). CONCLUSIONS: The success of urethroplasty with lingual and buccal mucosa grafts was similar. Oral pain was not different after both grafts. In the early postoperative period there were differences in oral morbidity between buccal and lingual mucosa grafts. Long-term oral morbidity was not infrequent with both grafts.


Assuntos
Mucosa Bucal/transplante , Complicações Pós-Operatórias/etiologia , Coleta de Tecidos e Órgãos/efeitos adversos , Sítio Doador de Transplante/lesões , Uretra/cirurgia , Estreitamento Uretral/cirurgia , Humanos , Masculino , Estudos Prospectivos , Língua , Resultado do Tratamento , Procedimentos Cirúrgicos Urológicos Masculinos
18.
J Urol ; 193(5): 1620-4, 2015 May.
Artigo em Inglês | MEDLINE | ID: mdl-25444989

RESUMO

PURPOSE: Pathophysiological mechanisms leading to chordee in patients with hypospadias and to the hidden state of buried penis in the prepubic fat remain unclear. Resection of dartos tissue usually makes the penis straight in patients with hypospadias and corrects it in those with buried penis, suggesting a common pathophysiology related to dartos tissue. MATERIALS AND METHODS: Tissue samples from 113 children undergoing primary penile surgery for hypospadias (94 patients), epispadias (1) or buried penis (18) were collected between November 2011 and September 2013. Tissue samples from 79 children undergoing circumcision for nonmedical reasons served as controls. All samples were stained with smooth muscle actin and analyzed by the same pathologist, who was blinded to indication for surgery. Chi-square and Fisher exact tests were applied. RESULTS: Three different dartos tissue patterns were observed. Pattern I (normal) consisted of smooth muscle fibers of dartos tissue organized in a parallel configuration in the subcutaneous tissue. Pattern II was characterized by poorly developed and hypotrophic smooth muscle fibers. Pattern III was determined by randomly distributed smooth muscle fibers in the subcutaneous tissue, without parallel configuration. Pattern I was observed in 45 circumcision specimens (64%). Of buried penis cases 78% were considered abnormal (pattern II in 4 cases and III in 10, p = 0.001). Of hypospadias cases 70% were considered abnormal (pattern II in 31 cases, III in 32, and mixed II and III in 3, p < 0.001). The only epispadias case was designated pattern II. CONCLUSIONS: Congenital penile pathology (hypospadias, buried penis) is associated with structural anomalies in dartos tissue. Further research is needed to unveil the pathophysiology of the condition.


Assuntos
Epispadia/cirurgia , Hipospadia/cirurgia , Músculo Liso/anormalidades , Pênis/anormalidades , Pênis/cirurgia , Adolescente , Criança , Pré-Escolar , Humanos , Lactente , Masculino , Estudos Prospectivos , Centros de Atenção Terciária , Procedimentos Cirúrgicos Urológicos Masculinos/métodos
19.
Case Rep Urol ; 2014: 632852, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-25254137

RESUMO

Gastrointestinal stromal tumors (GISTs) are the most frequent mesenchymal tumors of the gastrointestinal tract with surgical resection remaining the cornerstone of therapy. Pararectal lesions are considered to be technically difficult and pose in some cases a challenge. We report, to the best of our knowledge, the first robotic-assisted pararectal GIST excision. A 43-year-old man was referred to our center with pararectal GIST recurrence, despite treatment with targeted therapy. Eleven years ago, he underwent extensive abdominal surgery including cystoprostatectomy with ileal neobladder diversion due to GIST resection in the rectoprostatic space. Robot-assisted surgical resection was successfully performed without the need for temporary colostomy. The postoperative course of the patient was uneventful, and the pathology report confirmed a GIST recurrence with negative surgical margins and pelvic lymph nodes free of any tumor. Robotic-assisted pelvic surgery can be extended to incorporate excision of pararectal GISTs, as a safe, less invasive surgical alternative with promising oncological results and minimal injury to adjacent structures.

20.
Case Rep Nephrol Urol ; 4(1): 12-7, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-24575117

RESUMO

Perineal urethrostomy is considered to be the last option to restore voiding in complex/recurrent urethral stricture disease. It is also a necessary procedure after penectomy or urethrectomy. Stenosis of the perineal urethrostomy has been reported in up to 30% of cases. There is no consensus on how to treat a stenotic perineal urethrostomy, but, in general, a form of urinary diversion is offered to the patient. We present the case of a young male who underwent perineal urethrostomy after urethrectomy for urethral cancer. The postoperative period was complicated by wound dehiscence with subsequent complete obliteration of the perineal urethrostomy. Revision surgery was performed with reopening of the obliterated urethral stump and coverage of the skin defect between the urethra and the perineal/scrotal skin with a meshed split-thickness skin graft. To date, this patient is voiding well and satisfied with the offered solution.

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