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1.
J Med Case Rep ; 18(1): 490, 2024 Oct 09.
Artigo em Inglês | MEDLINE | ID: mdl-39380118

RESUMO

INTRODUCTION: Triphallia, a rare congenital anomaly describing the presence of three distinct penile shafts, has been reported only once in the literature. This case report, based on an extensive literature review, describes the serendipitous discovery during cadaveric dissection of the second reported human case of triphallia, distinctly morphologically different from the previous case. CASE PRESENTATION: Despite the normal appearance of external genitalia on examination, the dissection of a 78-year-old white male revealed a remarkable anatomical variation: two small supernumerary penises stacked in a sagittal orientation posteroinferiorly to the primary penis. Each penile shaft displayed its own corpora cavernosa and glans penis. The primary penis and largest and most superficial of the supernumerary penises shared a single urethra, which coursed through the secondary penis prior to its passage through the primary penis. A urethra-like structure was absent from the smallest supernumerary penis. CONCLUSION: This case report provides a comprehensive description of the anatomical features of triphallia in a cadaver, shedding light on the morphology, embryology, and clinical implications of this anomaly. Without dissection, this anatomical variation would have remained undiscovered, suggesting the prevalence of polyphallia may be greater than expected. The single tortuous urethra present in this case, as well as the supernumerary and blind ending urethras present in many cases of penile duplication, may pose significant risk of infection, sexual dysfunction, subfertility, and traumatic catheterization. SIGNIFICANCE: These findings underscore the importance of meticulous anatomical dissections and may act as a resource for anatomists and those studying genitourinary anomalies. Although we can only speculate as to which functional implications this patient may have experienced, understanding such anatomical variations contributes to both knowledge of human anatomy and clinical management should the condition be encountered in living individuals.


Assuntos
Cadáver , Pênis , Humanos , Masculino , Pênis/anormalidades , Idoso , Uretra/anormalidades
3.
J Pediatr Urol ; 20 Suppl 1: S58-S65, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38969556

RESUMO

BACKGROUND: Posterior urethral valves (PUV) represents a heterogenous spectrum in which guidelines for management are lacking particularly for those patients facing end-stage kidney disease and transplant. In this study we aim to 1) evaluate our long term PUV pediatric transplant outcomes compared to those without lower urinary tract dysfunction and 2) assess our PUV cohort for trends in bladder management and evaluate outcomes to inform development of institutional guidelines. MATERIALS AND METHODS: A retrospective cohort analysis of all patients with a diagnosis of PUV who underwent kidney transplant from 2000 to 2023 was completed. A matched cohort of patients without lower urinary tract dysfunction was identified for comparison of graft function. Charts of PUV patients were reviewed for both sociodemographic and clinical variables. Patients were classified by bladder management at the time of transplantation into three separate groups for analysis: voiding, clean intermittent catheterization, and incontinent diversion. Primary outcomes of interest were eGFR, graft failure, and UTIs post-transplant. RESULTS: 45 patients met inclusion criteria. 69% were on dialysis prior to transplant. 51% of grafts were from a deceased donor. Bladder management consisted of voiding (62%), CIC (4 via urethra, 10 via channel) (31%), and incontinent diversion (7%). 20% underwent augmentation cystoplasty (5 = ureter, 2 = gastric, 1 = colon, and 1 = ileum) prior to or at the time of transplant. Median follow up duration was 5.4 years (3.0, 10.8). Patients on CIC had higher rates of UTI; however, we found no significant difference in graft function outcomes (eGFR, graft failure) between bladder management groups or year of transplant. VUR in the transplant kidney was associated with vesicostomy (p = 0.028). 2 of 2 gastric augments developed malignancy, one of which was cause of death. Graft failure rate was 22% in both the PUV group and matched cohort, with median interval times to failure of 6.7 years and 3.7 years, respectively (p = 0.71). There were no differences in eGFR at follow-up time points between the PUV and matched cohort. CONCLUSIONS: Patients with PUV represent a spectrum of disease with heterogeneous management before and after kidney transplant. Overall, graft function outcomes were similar when compared to matched cohort without lower urinary tract dysfunction. Patients on CIC had higher rates of UTI but without impact on graft function. Gastric augmentation cystoplasty should be avoided given risk for malignancy. Guidelines to standardize evaluation and management would be helpful for patient care and outcomes.


Assuntos
Transplante de Rim , Uretra , Humanos , Transplante de Rim/métodos , Estudos Retrospectivos , Masculino , Uretra/cirurgia , Uretra/anormalidades , Criança , Adolescente , Falência Renal Crônica/cirurgia , Feminino , Estudos de Coortes , Resultado do Tratamento , Complicações Pós-Operatórias/epidemiologia , Pré-Escolar , Melhoria de Qualidade
4.
BMC Urol ; 24(1): 154, 2024 Jul 29.
Artigo em Inglês | MEDLINE | ID: mdl-39069606

RESUMO

OBJECTIVES: To investigate the effects of bladder neck incision (BNI) and primary valves ablation on long-term kidney and bladder function in children with posterior urethral valves (PUV) and bladder neck hypertrophy (BNH). PATIENTS AND METHODS: From 1997 to 2016, a total of 1381 children with PUV were referred to our tertiary hospital. Of these patients, 301 PUV patients with bladder neck hypertrophy need concurrent BNI and valve ablation. All patients were followed up every 3-6 months on regular basis in first 2 post-surgical years and annually then after. The paired t-test and chi-square test were used to perform statistical analysis with p value < 0.05 defined as the level of significance. RESULTS: Mean age at diagnosis was 7.22 ± 2.45 months (ranging from 7 days to 15 months) with a mean follow-up of 5.12 ± 2.80 years. The incidence of hydronephrosis was decreased from 266 (88.3%) at the baseline to 73 (24.3%) patients in long-term follow-up. At baseline, 188 (62.5%) patients were diagnosed with VUR, which decreased to 20 (6.6%) individuals at the end of follow-up. Bladder and renal function were improved in follow-ups following concomitant PUV ablation and BNI. No Myogenic failure was depicted in all patients with BNH. No ureteric reimplantation was needed during the two decades follow-up. CONCLUSION: Simultaneous valve ablation with BNI may present further profits in children with PUV and BNH particularly cases of BNH with poor bladder function at the time of presentation. This method can improve the results of urodynamic and imaging studies after the surgery. We hypothesize every child with PUV presentation who has concurrent vesicoureteral reflux, CKD or persistent hydrourethronephrosis may suffer from secondary bladder neck obstruction. This secondary bladder outlet obstruction must be managed through BNI as the surgical relief.


Assuntos
Hipertrofia , Uretra , Bexiga Urinária , Humanos , Uretra/anormalidades , Uretra/cirurgia , Lactente , Masculino , Seguimentos , Bexiga Urinária/cirurgia , Recém-Nascido , Fatores de Tempo , Estudos Retrospectivos , Obstrução do Colo da Bexiga Urinária/cirurgia , Obstrução do Colo da Bexiga Urinária/etiologia , Técnicas de Ablação/métodos , Feminino , Procedimentos Cirúrgicos Urológicos/métodos , Insuficiência Renal/etiologia , Insuficiência Renal/epidemiologia
5.
Pediatr Surg Int ; 40(1): 177, 2024 Jul 06.
Artigo em Inglês | MEDLINE | ID: mdl-38969779

RESUMO

PURPOSE: We investigated the postoperative renal function in persistent cloaca (PC) patients who underwent posterior sagittal anorecto-urethro-vaginopalsty (PSARUVP) and factors influencing the renal functional outcomes. METHODS: A questionnaire survey was distributed to 244 university and children's hospitals across Japan. Of the 169 patients underwent PSARUVP, 103 patients were enrolled in the present study. Exclusion criteria was patients without data of renal prognosis. RESULTS: The present study showed that renal anomalies (p = 0.09), vesicoureteral reflux (VUR) (p = 0.01), and hydrocolpos (p = 0.07) were potential factors influencing a decline in the renal function. Approximately half of the patients had a normal kidney function, but 45.6% had a reduced renal function (Stage ≥ 2 chronic kidney disease: CKD). The incidence of VUR was significantly higher in the renal function decline (RFD) group than those in the preservation (RFP) group (p = 0.01). Vesicostomy was significantly more frequent in the RFD group than in the RFP group (p = 0.04). Urinary tract infections (p < 0.01) and bladder dysfunction (p = 0.04) were significantly more common in patients with VUR than in patients without VUR. There was no association between the VUR status and the bowel function. CONCLUSIONS: Prompt assessment and treatment of VUR along with bladder management may minimize the decline in the renal function.


Assuntos
Cloaca , Rim , Humanos , Japão/epidemiologia , Feminino , Masculino , Cloaca/anormalidades , Cloaca/cirurgia , Rim/anormalidades , Rim/cirurgia , Rim/fisiopatologia , Inquéritos e Questionários , Lactente , Vagina/cirurgia , Uretra/cirurgia , Uretra/anormalidades , Complicações Pós-Operatórias/epidemiologia , Canal Anal/cirurgia , Canal Anal/anormalidades , Reto/cirurgia , Recém-Nascido , Pré-Escolar
6.
J Pediatr Urol ; 20 Suppl 1: S18-S25, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38824105

RESUMO

INTRODUCTION: Posterior urethral valves (PUV) is a chronic condition that can adversely impact child and family health related quality of life (HRQOL). Surprisingly, to date, the impact of this condition on the QOL of children with PUV has not been studied. Thus, our aim is to evaluate this important aspect of care using validated questionnaires in our institutional PUV clinic. METHODS: From December 2022 to January 2024, the PedsQL Inventory and associated family impact module was distributed in our PUV clinic. Higher scores reflect better HRQoL and less impact on family. Children >8 years old completed the tool themselves, otherwise parents completed the tools on behalf of the child. We compared PUV scores against reported psychometric data from healthy children and other chronic illnesses. RESULTS: A total of 112 families completed the questionnaires. The total HRQoL score was 80 ± 13. Overall, we noted that the domains reflecting psychosocial impact were significantly lower than those of physical well-being (76 ± 16 vs. 89 ± 15 for psychosocial and physical impact, respectively; p < 0.01). There was a significant difference in the psychosocial emotional impact for children managed with clean intermittent catheterization (CIC) compared to those who were not (69 ± 21 vs. 81 ± 16; p = 0.005) (Table 2). In addition, we noted that children with PUV scored similar to healthy controls, but higher than children with other chronic illnesses. With respect to the impact on family, overall, parent, and family impacts were 76 ± 19, 78 ± 21, and 81 ± 21, respectively. DISCUSSION: Chronic illness has been associated with a negative impact on HRQoL in both adults and children. We noted similar findings the present study, particularly for young children and those who are maintained on CIC. To our knowledge, this is the first study to evaluate the impact of PUV on a child's HRQoL as well as the impact on the family, and to document a negative psychosocial impact of CIC in this population. However, there are some important limitations to be addressed including relying on families to complete questionnaires accurately during busy clinic visits and the lack of controls from the same environment. CONCLUSION: When compared to healthy controls, PUV patients score similarly in HRQoL assessment. In addition, children <3years of age report a negative impact on physical well being, and CIC negatively impacts the emotional well-being. These data can be used to advocate for better mental health supports and social work for these children.


Assuntos
Qualidade de Vida , Humanos , Criança , Masculino , Estudos Prospectivos , Inquéritos e Questionários , Uretra/anormalidades , Feminino , Adolescente , Pré-Escolar , Equipe de Assistência ao Paciente
9.
J Pediatr Surg ; 59(8): 1647-1651, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38734497

RESUMO

BACKGROUND: Multiple factors impact ability to achieve urinary continence in cloacal malformation including common channel (CC) and urethral length and presence of spinal cord abnormalities. Few publications describe continence rates and bladder management in this population. We evaluated our cohort of patients with cloacal malformation to describe the bladder management and continence outcomes. METHODS: We reviewed a prospectively collected database of patients with cloacal malformation managed at our institution. We included girls ≥3 years (y) of age and evaluated their bladder management methods and continence. Dryness was defined as <1 daytime accident per week. Incontinent diversions with both vesicostomy and enterovesicostomy were considered wet. RESULTS: A total of 152 patients were included. Overall, 93 (61.2%) are dry. Nearly half (47%) voided via urethra, 65% of whom were dry. Twenty patients (13.1%) had incontinent diversions. Over 40% of the cohort performed clean intermittent catheterization (CIC), approximately half via urethra and half via abdominal channel. Over 80% of those performing CIC were dry. In total, 12.5% (n = 19) required bladder augmentation (BA). CC length was not associated with dryness (p = 0.076), need for CIC (p = 0.253), or need for abdominal channel (p = 0.497). The presence of a spinal cord abnormality was associated with need for CIC (p = 0.0117) and normal spine associated with ability to void and be dry (p = 0.004) CONCLUSIONS: In girls ≥ 3 y of age with cloacal malformation, 61.2% are dry, 65% by voiding via urethra and 82% with CIC. 12.5% require BA. Further investigation is needed to determine anatomic findings associated with urinary outcomes. LEVEL OF EVIDENCE: IV.


Assuntos
Cloaca , Bexiga Urinária , Incontinência Urinária , Humanos , Feminino , Pré-Escolar , Cloaca/anormalidades , Cloaca/cirurgia , Incontinência Urinária/etiologia , Bexiga Urinária/anormalidades , Bexiga Urinária/cirurgia , Estudos Retrospectivos , Criança , Uretra/anormalidades , Uretra/cirurgia , Derivação Urinária/métodos , Cateterismo Uretral Intermitente , Resultado do Tratamento
10.
J Pediatr Urol ; 20(4): 729.e1-729.e7, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38719686

RESUMO

INTRODUCTION AND AIM: Posterior urethral valves (PUV) are often associated with bladder dysfunction. Our primary aim was to investigate bladder status following primary valves resection to gather evidence of function and to guide early clinical management. PATIENTS AND METHODS: Between July 2015-2020, we prospectively evaluated bladder function of PUV infants. Primary outcomes measured were number of voids, voided volume (VV), post-void residual (PVR) and bladder capacity (BC). Statistical comparisons and descriptive analysis were carried out between groups using 2-tails T test and Chi square test using an IBM SPSS Statistics program version 25. RESULTS: Sixty-one infants were included. Median age at resection was 28 days (5 days-11 months). In thirty-eight patients (62%) diagnosis was suspected antenatally. Vesico-ureteric reflux (VUR) was present in 16 (26%). The 4-h observational study was performed at median 4 days following primary cold-knife valve resection and 1 day (range from 1 to 12 days) after catheter removal. Infants voided an average 6 times (2-13 voids). Bladder capacity was large in 20 (33%) and small in 4 (6.5%) patients with a median ratio BC/eBC = 1.2 (0.49-22.6). Median residual urine was 9 mls but with great variability among the cohort (0-121 mls). A big variability in bladder emptying was noted between patients and between voids performed in one patient. In 13 infants (21%) average PVR was larger than the average voided volume. In 26 (42.6%) PVR was larger than VV at least once. Thirty-eight infants (62.3%) emptied their bladders almost completely at least once (with PVR <5 mls). No difference was found in PVR, Median BC/EBC and PVR/BC between infants with and without VUR (p = 0.654, P: 0.594 and p = 00.481, respectively). DISCUSSION: We presented our experience of non-invasive bladder function assessment in infants affected by PUV following primary valve resection. An interesting data is the great variability identified both between patients and between voids performed in a single patient. Average number of voids was similar to the voiding pattern in healthy newborns what reported by Gladh but variability was greater. We observed up to 13 voids in some infants and we can speculate this could reflect the presence of an overactive bladder. We observed at least 1 complete emptying in only 62% of infants and 21% of the cohort had PVR bigger than VV. In almost half of the infants (42.6%) PVR was larger than VV at least once. CONCLUSIONS: Around 40% of infants affected by PUV have abnormal bladder capacity and almost half of them have significant post void residuals following primary resection. Although controversies and limitations are present, we believe that this non-invasive study can provide valuable information to understand the dynamic of the bladder, particularly in children affected by PUV and allow early intervention in children considered "at risk". Having a non invasive way of assessing can help tailor intervention and be useful for future research into early bladder intervention and improving outcomes.


Assuntos
Uretra , Micção , Humanos , Lactente , Uretra/anormalidades , Uretra/cirurgia , Masculino , Estudos Prospectivos , Recém-Nascido , Micção/fisiologia , Fatores de Tempo , Bexiga Urinária/cirurgia , Bexiga Urinária/anormalidades , Bexiga Urinária/fisiopatologia , Urodinâmica/fisiologia
11.
J Pediatr Urol ; 20(3): 537-538, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38677981

RESUMO

INTRODUCTION: After 5 years experience with the GUD (glandular urethral disassembly) technique for distal hypospadias, we present the GUDplay technique, incorporating Thiersch-Duplay tubularization of the plate till the coronal area, disassembling the glans aggressively and refurbishing the glans. METHODS: We defined the urethral plate and designed an inverted Y incision to open the glans in two wings. The glans was entirely detached from the corpora to gain a great mobility that allowed minor cranial mobilization of the urethra and caudal rotation of the wings. In sequence, there are well-known steps: Duplay urethroplasty, spongioblasts and a Dartos flap to cover the neourethra. The glans was connected to the urethra by 6.0 PDS sutures except in the ventral meatus and the glans wings are joined in the midline. RESULTS: The 5-year-old patient had midshaft hypospadias without previous surgery. The catheter was removed after a week and the healing appears to be good. DISCUSSION: We combined principles of total glans deconstruction in association to Duplay tubularization and then lifted it up to the tip of the glans divided in two wide and mobile wings. We have treated a small series of 6 cases without complications and mean follow-up of 6.2 months.


Assuntos
Hipospadia , Pênis , Procedimentos de Cirurgia Plástica , Uretra , Procedimentos Cirúrgicos Urológicos Masculinos , Hipospadia/cirurgia , Masculino , Humanos , Procedimentos Cirúrgicos Urológicos Masculinos/métodos , Pré-Escolar , Pênis/cirurgia , Pênis/anormalidades , Procedimentos de Cirurgia Plástica/métodos , Uretra/cirurgia , Uretra/anormalidades , Escroto/cirurgia , Retalhos Cirúrgicos
12.
J Pediatr Urol ; 20(4): 726.e1-726.e7, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38653666

RESUMO

BACKGROUND: Urinary drainage for posterior urethral valves can be achieved with valve ablation (VA) or diversion by vesicostomy (VES) or cutaneous ureterostomy (CU). The effect of these interventions on long-term bladder function remains debated, and voiding symptomatology after VES or CU reversal has been poorly characterized. OBJECTIVE: The objective of this study was to examine the prevalence and scope of physician treatment patterns as a surrogate for retention or incontinence symptomatology among PUV patients undergoing primary VA or diversion by VES/CU and determine rates of progression to augmentation. STUDY DESIGN: This is a single-institution retrospective cohort study. Retention Scores (R) were calculated 1 point for: retention behavior (double/timed void), alpha-blocker, intermittent catheterization, or overnight indwelling catheter. Incontinence Scores (I) were calculated 1 point for: incontinence behavior (double/timed void), oral medication, or botulinum toxin. Patients with R score above 3 or I score above 2 were deemed to have severe retention or incontinence symptomatology respectively. End stage bladder (ESB) was defined as need for bladder augmentation. RESULTS: We identified 76 patients between 5 and 40 years old with median follow-up of 14.6 [5.0-40.4) years. There was no difference in the rates of severe retention or incontinence treatment pattern scoring between VA versus VES/CU (Figure). Rates of achieving R(1) status are similar between VA and VES/CU groups, though age of reaching R(1) was younger for those with VES/CU (4.8 years) compared to VA (6.6 years). There was no significant difference in rate of ESB by intervention category VA (9.4%) versus VES/CU (17.4%; p = 0.323). DISCUSSION: Treatment of retention symptomatology was more common than treatment of incontinence symptomatology regardless of primary management, VA or VES/CU. This study also indicates that VES/CU patients were just as responsive as VA patients to conservative treatments (behavioral changes, pharmacotherapy) for any type of bladder symptomatology as the progression to treatment of severe symptomatology and ESB were similar between cohorts. In this cohort, bladder outcomes were not associated with type of urinary diversion (VA or VES/CU). CONCLUSION: Long term bladder outcomes for valve patients demonstrated similar treatment patterns and progression to end-stage bladder regardless of diversion status. Patients went on to ESB approximately 4.4 years after diagnosis at similar rates between groups.


Assuntos
Uretra , Humanos , Estudos Retrospectivos , Masculino , Criança , Uretra/anormalidades , Uretra/cirurgia , Adolescente , Pré-Escolar , Adulto , Adulto Jovem , Resultado do Tratamento , Fatores de Tempo , Padrões de Prática Médica/estatística & dados numéricos , Seguimentos , Bexiga Urinária/cirurgia , Feminino , Estudos de Coortes , Derivação Urinária/métodos
13.
J Pediatr Urol ; 20(3): 492-496, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38280830

RESUMO

Today, prenatal diagnosis of congenital urogenital malformations is mostly dependent on anatomical variations found on imaging. However, these findings can mislead us in telling us when to intervene, and about post-natal prognosis. Since many findings are dependent on multiple assessments, delayed diagnosis can occur, leading to less optimal outcomes compared to early intervention. Analyses of fetal urinary biomarkers have been proposed as a method of finding biological changes that are predictive for diagnosis and prognosis in fetuses at risk of kidney disease. We interviewed a group of researchers that have demonstrated that by combining multiple omics traits extracted from fetal urine, the biological variability found in single omics data can be circumvented. By analyzing multiple fetal urine peptides and metabolites at single time point, the prognostic power of postnatal renal outcome in fetuses with lower urinary tract obstruction is significantly increased. In this interview, we inquired about the technical aspects of the tests, challenges, and limitations the research group have come across, and how they envision the future for multi-omics fetal analysis in the clinic.


Assuntos
Biomarcadores , Uretra , Obstrução Uretral , Humanos , Biomarcadores/urina , Feminino , Gravidez , Obstrução Uretral/embriologia , Obstrução Uretral/diagnóstico por imagem , Uretra/anormalidades , Uretra/diagnóstico por imagem , Diagnóstico Pré-Natal/métodos , Doenças Fetais/diagnóstico
14.
Medicine (Baltimore) ; 103(4): e37004, 2024 Jan 26.
Artigo em Inglês | MEDLINE | ID: mdl-38277528

RESUMO

BACKGROUND: Concomitant anterior urethral valves (AUVs) and posterior urethral valves (PUVs) is an extremely rare congenital urologic anomaly, which may be easily overlooked in the clinic. OBJECTIVE: This study assessed the prognosis of children with concomitant PUVs and AUVs. METHODS: The clinical data of inpatients with concomitant AUVs and PUVs in our hospital were collected from January 1983 to June 2022. The clinical manifestations, auxiliary inspection, and treatment were described in detail. RESULTS: In total, 6 cases of concomitant AUVs and PUVs in boys were found in our hospital, with ages ranging from 3 months to 9 years; the main clinical manifestation was abnormal urination. Four patients exhibited concomitant AUVs and PUVs preoperatively and underwent simultaneous anterior and posterior urethral valvotomy. Follow-up studies showed that 3 patients' clinical symptoms substantially improved with well-maintained renal function. One patient died of renal failure. In the other 2 patients, PUVs were initially identified and excised, but their clinical symptoms did not show substantial improvement. Following voiding cystourethrography (VCUG), the AUVs were found and obstructions were then completely relieved. However, 2 patients died of renal failure. CONCLUSIONS: If urinary symptoms cannot be substantially relieved after posterior urethral valvotomy, VCUG and cystoscopy should be repeated to shorten the interval between anterior and posterior urethral valvotomies to improve patient prognosis.


Assuntos
Insuficiência Renal , Obstrução Uretral , Criança , Masculino , Humanos , Obstrução Uretral/etiologia , Obstrução Uretral/cirurgia , Uretra/anormalidades , Micção , Prognóstico , Insuficiência Renal/complicações , Estudos Retrospectivos
15.
Urology ; 184: e243-e245, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37956764

RESUMO

Congenital urethral atresia is generally considered to be incompatible with life unless there is either a patent urachus or vesicoamniotic shunt. Here we present the case of a male neonate with anhydramnios detected at 28weeks gestation due to urethral atresia, who was born without evidence of either a patent urachus or vesicoamniotic shunt, who has survived and is not requiring respiratory support at age 5months. While this is a thought-provoking clinical case, it also highlights the importance of early and effective parental engagement in cases of complex congenital anomalies of the urinary tract.


Assuntos
Uretra , Doenças Uretrais , Recém-Nascido , Humanos , Masculino , Pré-Escolar , Uretra/cirurgia , Uretra/anormalidades
16.
BMC Pediatr ; 23(1): 445, 2023 09 07.
Artigo em Inglês | MEDLINE | ID: mdl-37679663

RESUMO

BACKGROUND: Posterior urethral valve (PUV) is the most common congenital bladder outlet obstruction in boys, causing renal damage beginning in utero. There are scarce data from Thailand regarding the long-term outcomes of PUV in boys, thus the aim of this study was to examine the presentation, clinical course, complications, outcomes and renal survival in PUV boys. METHODS: We reviewed the medical records of PUV boys treated at the Pediatric Nephrology Clinic, Prince of Songkla University, Thailand, over a 30-year-period. RESULTS: Seventy-seven PUV boys were identified, with a median age at diagnosis of 4.8 months. The most common presentations were urinary tract infection (UTI), poor urine stream and urinary dribbling in 26 (33.8%), 19 (24.7%) and 11 (14.3%) boys, respectively. Renal ultrasound results in 70 boys showed 8 (11.4%) unilateral and 56 (80%) bilateral hydronephroses. Of 72 voiding cystourethrograms, 18 (25.0%) showed unilateral and 22 (30.6%) bilateral vesicoureteral refluxes. 99mTc dimercaptosuccinic acid renal scans in 30 boys showed 12 (40%) unilateral and 8 (26.7%) bilateral renal damage. Fifty-nine (76.6%) boys had 149 UTIs; 42 (54.4%) had recurrent UTI. Forty-eight boys had valve ablation at the median age of 30.3 months. 22 boys (28.6%) developed chronic kidney disease (CKD) at a median age of 15.0 years. CONCLUSION: Of 77 PUV Thai boys, UTI was the most common presentation. Recurrence of UTI and CKD was the most common consequence. Lifelong follow-up for renal and bladder functions is essential for all PUV patients.


Assuntos
Insuficiência Renal Crônica , População do Sudeste Asiático , Uretra , Doenças Uretrais , Obstrução do Colo da Bexiga Urinária , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Instituições de Assistência Ambulatorial , Rim , Insuficiência Renal Crônica/etiologia , Tailândia/epidemiologia , Uretra/anormalidades , Uretra/cirurgia , Obstrução do Colo da Bexiga Urinária/congênito , Obstrução do Colo da Bexiga Urinária/etiologia , Obstrução do Colo da Bexiga Urinária/cirurgia , Doenças Uretrais/complicações , Doenças Uretrais/congênito , Doenças Uretrais/cirurgia , Recém-Nascido
17.
J Pediatr Urol ; 19(5): 516-518, 2023 10.
Artigo em Inglês | MEDLINE | ID: mdl-37271679

RESUMO

INTRODUCTION: Total Urogenital Mobilization (TUM) has been the standard surgical approach for the urogenital complex in Cloacal Malformations (CM) since its inception in 1997. Partial Urogenital Mobilization (PUM) in CM remains an under-utilized or under-reported option. The main anatomical difference between TUM and PUM is the division of the pubo-urethral ligaments. OBJECTIVE: We explored the feasibility of PUM in a select subset of our patients with CM and report early outcomes. STUDY DESIGN: We retrospectively reviewed prospectively collected data of all our CM patients who had primary reconstruction at our centre from 2012 to 2020. We included in our review the patients who underwent PUM. Mullerian abnormalities, spinal cord involvement, common channel length (CC), urethral length (UL), surgical reconstruction, and outcomes including urinary continence, recurrent UTI, ultrasound and preoperative DMSA/MAG3, cystovaginoscopy post-reconstruction, and post-void residuals were noted. RESULTS: Fifty-three patients had primary reconstruction, and of these, eleven had a common channel less than 3 cm. Of the eleven, only one underwent TUM. In the PUM group, two underwent filum untethering (20%). Mullerian duplication was noted in 5 patients (50%). The median CC length = 1.6 cm (range = 1.5cm-2.7 cm), and median UL = 1.5 cm (range = 1.5cm-2.5 cm). Follow-up ranged from 9 to 134months (median = 63months). Post-reconstruction all had a separate urethral and vaginal opening on examination and cysto-vaginoscopy. The continence outcomes are summarized in Fig.1. DISCUSSION: Although TUM is the most common solution for the urogenital complex in CM, a subset would be suitable for PUM, and this option is under-utilized or under-reported in literature. We presume that many who had TUM probably only needed a PUM, and therefore could report better outcomes from a bladder function aspect. It is important to differentiate the two, and outcomes should be appropriately categorized. Our default approach is a PUM in all CM with less than 3 cm common channel. Only the lateral and posterior aspects of the urogenital complex are mobilized and if the urethra did not reach a satisfactory level for easy intermittent catheterization, then we proceed to a TUM dividing the pubo-urethral ligaments. PUM avoids the potential complications related to dividing the pubo-urethral ligament in TUM. It may also avoid the need for CIC which is encountered in patients who undergo TUM. CONCLUSION: PUM is a viable alternative in cloacal malformations with good outcomes in those with a common channel under 3 cm. This of course requires appropriate patient selection and accurate categorization of interventions to understand the true outcomes.


Assuntos
Uretra , Vagina , Animais , Feminino , Humanos , Cloaca/cirurgia , Endoscopia , Estudos Retrospectivos , Uretra/cirurgia , Uretra/anormalidades , Vagina/cirurgia
18.
J Pediatr Urol ; 19(4): 397.e1-397.e7, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-37095037

RESUMO

INTRODUCTION: Hypospadias is an abnormal formation of the urethra, ventral skin, and corporal bodies. Location of the urethral meatus has historically been the phenotypic landmark that defines hypospadias. Nonetheless, classifications following location of the urethral meatus fail to consistently predict outcomes and have no correlation with the genotype. Description of the urethral plate is very subjective and difficult to reproduce. We hypothesize that the use of digital pixel cluster analysis and correlation to histological analysis can provide a novel method to describe the phenotype of patients with hypospadias. METHODS: A standardized hypospadias phenotyping protocol was developed. 1. Digital images of the anomaly, 2. Anthropometric assessment of penile dimensions (penile length, urethral plate length and width, glans width, ventral curvature), 3. Classification using the GMS score, 4. Tissue sampling (foreskin, glans, urethral plate, periurethral ventral skin) and H&E analysis by a blinded pathologist. A k-means colorimetric pixel cluster analysis was performed following the same anatomical landmark distribution as the histology samples. Analysis was performed using MATLAB v R2021b 9.11.0.1769968. RESULTS: A total of 24 patients prospectively enrolled with a standard protocol. Mean age at surgery was 16.25 months Urethral meatus was distal shaft in 7 patients, 8 coronal, 4 glanular, 3 midshaft, 2 penoscrotal. Average GMS score was 7.14 (±1.58). Average glans size was 15.71 mm (±2.33) and urethral plate width 5.57 mm (±2.06). Eleven patients underwent Thiersch-Duplay repair, 7 TIP, 5 MAGPI, and 1 a first stage preputial flap. Mean follow-up was 14.25 months ( ± 3.7 months). Two (8.3%) postoperative complications (1 urethrocutaneous fistula and 1 ventral skin wound dehiscence) were reported in the study period. Eleven (52.3%) patients with histological analysis had an abnormal pathology report. Of those, 6 (54%) had reported abnormal lymphocyte infiltration interpreted as chronic inflammation at the urethral plate. The second most common finding was hyperkeratosis visualized in the urethral plate in 4 (36.3%) and one with reported fibrosis in the urethral plate. K-means pixel analysis demonstrated a k1 mean of 64.2 for reported urethral plate inflammation vs 53.1 for non-reported urethral plate inflammation (p = 0.002) CONCLUSIONS: Current phenotyping of hypospadias using only anthropometric variables can be expanded including histological and pixel analysis correlation. Pixel clustering has a potential for a priori prediction of urethral plate quality beyond the current subjective assessment. A larger cohort will allow identification of possible predictive associations that might impact intraoperative decision-making and surgical outcomes.


Assuntos
Hipospadia , Humanos , Masculino , Hipospadia/cirurgia , Hipospadia/patologia , Projetos Piloto , Resultado do Tratamento , Procedimentos Cirúrgicos Urológicos Masculinos/métodos , Uretra/anormalidades , Complicações Pós-Operatórias/cirurgia
19.
Int J Urol ; 30(6): 526-531, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-36872075

RESUMO

OBJECTIVE: To define and classify megameatus anomalies, the parameters of a considerable number of cases were investigated and compared with those of normal children. METHODS: A total of 1150 normal babies were examined during routine nonmedical circumcision, and another 750 boys referred with hypospadias were examined during the previous 3 years. All patients were evaluated and assessed for the size, location, and configuration of the urinary meatus, and penile length and girth were measured. Children with normal size and location of the meatus were considered control group A, and 42 cases of different forms of megameatus were considered group B. Other penoscrotal, urinary, and general anomalies were examined and investigated accordingly. All data were analyzed by the SPSS 9.0.1 statistical package and compared by paired t tests. RESULTS: Forty-two uncircumcised patients aged from 1 month to 4 years (mean 18 months) were diagnosed with a urinary meatus that engrossed the whole ventral or dorsal aspects of the glans, exceeding half the width of the glans or penile girth with the complete vanishing of the glans closure in most cases. Megameatus is usually associated with the abnormal meatal position as hypospadiac, orthotopic, or epispadic. Additionally, megameatus may be associated with a normally intact or deficient prepuce. Consequently, we had four categories of megameatus, and the intact prepuce orthotopic megameatus subcategory has not been described before. Megameatus was also detected with deficient prepuce, and this was considered a hypospadiac variant. CONCLUSION: Megameatus is diagnosed precisely with penile biometry and is classified into 4 groups: hypospadiac, epispadic, and orthotopic or central, either with or without intact prepuce. This classification is applicable for expansion to other centers.


Assuntos
Circuncisão Masculina , Epispadia , Hipospadia , Masculino , Lactente , Criança , Humanos , Pênis/anormalidades , Hipospadia/cirurgia , Prepúcio do Pênis , Uretra/anormalidades
20.
Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi ; 37(2): 189-195, 2023 Feb 15.
Artigo em Chinês | MEDLINE | ID: mdl-36796815

RESUMO

Objective: Anthropometric variants in prepubertal boys with hypospadias were assigned and assessed to illustrate anatomical malformation. Methods: A total of 516 prepubertal (Tanner grade Ⅰ) boys with hypospadias who were admitted to three medical centers between March 2021 and December 2021 and met the selection criteria for primary surgery were selected. The age of the boys ranged from 10 to 111 months, with an average of 32.6 months. Hypospadias were classified according to the location of the urethral defect, 47 cases (9.11%) of the distal type (the urethral defect is in the coronal groove or beyond), 208 cases (40.31%) of the middle type (the urethral defect is in the penis body), and 261 cases (50.58%) of the proximal type (the urethral defect is at the junction or proximal side of the penis and scrotum). The following indexes were measured: penis length before and immediately after operation, reconstructed urethral length, and total urethral length. Morphological indicators of the glans area, including preoperative height and width of glans, AB, BC, AE, AD, effective AD, CC, BB, the urethral plate width of the coronal sulcus, and postoperative height and width of glans, AB, BE, and AD. In which point A is the distal endpoint of navicular groove, point B is the protuberance lateral to the navicular groove, point C is the ventrolateral protuberance of the glans corona, point D is the dorsal midline point of the glans corona, and point E is the ventral midline point of the coronal sulcus. The foreskin morphological indicators, including the foreskin width, inner foreskin length, and outer foreskin length. The scrotal morphological indicators, including the left, right, and front penile to scrotum distance. The anogenital distances, including anoscrotal distance 1 (ASD1), ASD2, anogenital distance 1 (AGD1), and AGD2. Results: The penis length of the distal, middle, and proximal types decreased successively before operation, the reconstructed urethral length increased successively and the total urethral length decreased successively, these differences were all significant ( P<0.05). The height and width of the glans of the distal, middle, and proximal types significantly decreased successively ( P<0.05), but the height/width of the glans was generally close; AB value, AD value, and effective AD value significantly decreased successively ( P<0.05); there was no significant difference in BB value, urethral plate width of the coronary sulcus, and (AB+BC)/AD value between the groups ( P>0.05). There was no significant difference in the width of glans between the groups after operation ( P>0.05); AB value and AB/BE value increased successively, and AD value decreased successively, these differences were all significant ( P<0.05). The inner foreskin length in the 3 groups significantly decreased successively ( P<0.05), while the outer foreskin length had no significant difference ( P>0.05). The left penile to scrotum distance of middle, distal, and proximal types significantly increased successively ( P<0.05). ASD1, AGD1, and AGD2 significantly decreased from distal type to proximal type successively ( P<0.05). The other indicators' differences were significant only between some groups ( P<0.05). Conclusion: The anatomic abnormalities of hypospadias can be described by anthropometric indicators, which can be used as the basis for further standardized surgical guidance.


Assuntos
Hipospadia , Procedimentos de Cirurgia Plástica , Masculino , Humanos , Lactente , Pré-Escolar , Criança , Hipospadia/cirurgia , Pênis/cirurgia , Uretra/cirurgia , Uretra/anormalidades , Prepúcio do Pênis/cirurgia , Procedimentos Cirúrgicos Urológicos Masculinos , Resultado do Tratamento
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