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1.
Pediatr Surg Int ; 40(1): 124, 2024 May 07.
Artigo em Inglês | MEDLINE | ID: mdl-38713441

RESUMO

PURPOSE: Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a well described clinical condition, but reports are focused on microcolon and intestinal hypoperistalsis, while data on bladder management are scant. Aim of the study is to present urological concerns in MMIHS. METHODS: Retrospective evaluation of clinical data on urological management of MMIHS patients treated in the last 10 years. RESULTS: Six patients were enrolled (3 male, 3 female). Three girls had prenatal diagnosis of megacystis (1 vesicoamniotic shunt was placed). All patients had genetic diagnosis: 5 had ACTG2 gene mutations and 1 MYH11 mutation. All patients were addressed to our attention for urinary symptoms, such as urinary retention, urinary tract infections, acute renal injury. Two patients presented frequent stoma prolapses. All children underwent a complete urological evaluation, and then started a bladder management protocol (clean intermittent catheterization, via urethra or cystostomy-tube placement), with improvement of urinary infections, upper urinary tract dilation and stoma prolapses, if present. All patients had good renal function at last follow-up. CONCLUSION: We believe that MMIHS patients must be addressed soon and before onset of symptoms for a multidisciplinary evaluation, including an early assessment by a pediatric urologist expert in functional disorder, to preserve renal function at its best.


Assuntos
Anormalidades Múltiplas , Colo , Colo/anormalidades , Pseudo-Obstrução Intestinal , Bexiga Urinária , Bexiga Urinária/anormalidades , Humanos , Feminino , Estudos Retrospectivos , Masculino , Anormalidades Múltiplas/cirurgia , Colo/cirurgia , Bexiga Urinária/cirurgia , Lactente , Pseudo-Obstrução Intestinal/cirurgia , Pseudo-Obstrução Intestinal/diagnóstico , Recém-Nascido , Pré-Escolar , Mutação
2.
J Endourol ; 37(12): 1276-1281, 2023 12.
Artigo em Inglês | MEDLINE | ID: mdl-37742112

RESUMO

Objective: Thulium laser (ThL) has become popular in urology, because of its powerful action on tissue, achieving optimal ablation and hemostasis. Aim of our article was to evaluate efficacy of ThL in infants affected by posterior urethral valve (PUV) ablation. Patients and Methods: Clinical charts of 25 infants (age ≤12 months) who underwent PUV ablation were retrospectively reviewed. According to our protocol, all patients performed voiding cystourethrography and cystoscopy 6 to 8 months after initial treatment. Several factors, including age and weight at surgery, operative time, postoperative bleeding, catheterization period, postoperative urinary retention, retreatment for valve remnants, and stricture at follow-up, were evaluated. Preoperative, intraoperative, and postoperative data were analyzed. Results: Mean age at primary surgery was 4.5 months (5 days-10.5 months) and mean weight at primary surgery was 5.7 kg (2.5-10.3 kg). Mean operative time was 29.5 minutes (range 15-50 minutes). None of the patients experienced intraoperative and postoperative bleeding. In all cases, postoperative catheterization period was 1 day. Residual valves were found in 6 of 25 (24%) patients. No cases of urethral stricture were registered during follow-up (48.4 months, range: 11-95). Analyzing literature data using other techniques, complication rate of ThL PUV ablation seems lower than standard treatments (electrofulguration, cold knife) and comparable with those reported with other laser techniques. Conclusion: PUV ablation with ThL has proven to be feasible and safe in infants. Further studies are needed to define the real effectiveness of this laser technology in PUV ablation. Miniaturized instruments and ThL technology make early PUV treatment feasible also in low body weight newborns.


Assuntos
Uretra , Obstrução Uretral , Humanos , Lactente , Recém-Nascido , Uretra/cirurgia , Túlio/uso terapêutico , Estudos Retrospectivos , Obstrução Uretral/cirurgia , Lasers , Hemorragia Pós-Operatória
3.
Pediatr Surg Int ; 39(1): 226, 2023 Jul 06.
Artigo em Inglês | MEDLINE | ID: mdl-37410181

RESUMO

Anorectal malformation (ARM) is often associated to other congenital malformations, requiring a tailored management. Hypospadias' treatment in ARM is poorly described. Aim of study is to describe our experience in ARM-hypospadias patients especially in relation to occult spinal dysraphism (OSD). ARM patients treated from 1999 to 2022 were retrospectively reviewed, selecting male with hypospadias. Clinical data, hypospadias's severity, ARM sub-type (Group A: perineal fistula; Group B: urethral fistula, bladder fistula, no fistula), OSD, other associated malformations, NLUTD were evaluated. Exclusion criteria: uncomplete data. Among 395 ARMs, 222 were males, 22 (10%) had hypospadias. Two patients were excluded. Group A: 8 patients, Group-B: 12. Hypospadias: proximal 9 patients, distal 11. Neuro-urological evaluation was performed before hypospadias repair. Eleven patients (55%) had OSD. Four OSD patients presented NLUTD and underwent detethering and CIC (two via cystostomy button, two via appendicostomy); two of them had hypospadias repaired. All proximal hypospadias underwent two stages of surgery. Distal hypospadias was corrected in 4/11 cases. Hypospadias is quite common in ARM patients and its surgical management must be scheduled considering the possible OSD and NLUTD, with the possible need for intermittent catheterization. Complexity of ARM and hypospadias appears to be related to each other.


Assuntos
Malformações Anorretais , Hipospadia , Defeitos do Tubo Neural , Fístula Urinária , Humanos , Criança , Masculino , Feminino , Hipospadia/complicações , Hipospadia/cirurgia , Malformações Anorretais/complicações , Malformações Anorretais/cirurgia , Estudos Retrospectivos , Uretra/cirurgia , Defeitos do Tubo Neural/cirurgia
4.
Biomedicines ; 11(5)2023 Apr 27.
Artigo em Inglês | MEDLINE | ID: mdl-37238971

RESUMO

BACKGROUND: In the last twenty-five years, Onabotulinum Toxin A (BTX-A) has gained increasing popularity for neurogenic lower urinary tract dysfunction (NLUTD) treatment. To maintain its efficacy, repeated BTX-A intradetrusor injections are required over time, with unknown effects on the bladder wall in children. The aim of this paper is to report long-term effects on the bladder wall in children treated with BTX-A. METHODS: Children with NLUTD not responsive to anticholinergics were treated with BTX-A, according to our protocol, with bladder wall control using endoscopic cold-cup biopsy. Specimens were evaluated considering edema, chronic inflammation, and fibrosis. RESULTS: Of the 230 patients treated from 1997 to 2022, we considered only specimens obtained in patients who had received ≥5 treatments (36 children), considered as the threshold to evaluate clinical effectiveness on long-term treatment with BTX-A. Most of them had congenital NLUTD (25 patients) and detrusor overactivity (27 patients). In all, increased edema and chronic inflammation with reduced fibrosis over time was reported; these data were not statistically significant. No difference was observed between patients with congenital and acquired diseases. CONCLUSIONS: Repeated intradetrusor BTX-A injections are not related to significant histological alterations in children, similarly with adults, and repeated injections could be considered safe.

5.
Pediatr Surg Int ; 38(10): 1461-1472, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-35943565

RESUMO

PURPOSE: Caudal regression syndrome (CRS) is a rare congenital abnormality including orthopedic deformities, urological, anorectal, and cardiac malformations. The clinical spectrum of CRS varies in severity, therefore multiple surgeries and complex medical care may be required with the efforts and support of a multidisciplinary team to ensure the most accurate treatment and the best outcome. The aim of our retrospective study was to evaluate the role of a multidisciplinary treatment and the long-term outcome in patients with severe CRS. METHODS: Clinical, surgical and psychological data were collected for all patients with diagnosis of CRS, treated at our Pediatric Hospital from January 1995 to December 2020. Patients with a severe form of CRS according to Pang's classification were included in the study. All patients have been followed by a multidisciplinary team composed by urologists, neurosurgeons, neonatal and digestive surgeons, orthopedic surgeon, physiatrists and psychologists. On admission, patients were screened for CRS with sacrum, spine and legs X-ray, spine MRI, renal ultrasound, voiding cystography and urodynamic, and renography. Clinical data about bowel function were evaluated. RESULTS: During the study period, 55 patients with CRS were treated at our Institution. Six out of 55 (10.9%), presented with severe form of CRS (5 pts with type 1; 1 pt, with type 2) and represent our study group. Diagnosis of severe CRS was made at birth because of the typical deformities of the pelvis (fusion of the iliac wings), and of the lower extremities (undeveloped legs with flexion of the knees, clubfoot). All patients presented with neurogenic bladder, 4/6 (66.6%) with vesicoureteral reflux (VUR) and 2/6 (33.3%) with renal agenesia and contralateral ectopic hypoplastic kidney. Bowel symptoms occurred in 5/6 pts (83.3%). All patients were started with an earlier clean intermittent catheterization (CIC) regimen. In 3 patients oxybutynin has been effective or well tolerated, while in other 3 onabotulinumtoxin A has been used. Vesicoureteral reimplantation has been performed in 1 patient, 2 required endoscopic treatment of VUR. On long-term, 2 patients required bladder augmentation with ileum and appendicostomy (Mitrofanoff). Low adherences in CIC have been observed in three patients, mainly at puberty. Two patients presented with chronic renal failure. One patient reported suicide proposal. Regarding social life, only one is regularly performing sport activity. CONCLUSION: CRS is characterized by maldevelopment of the caudal half of the body with variable involvement of the gastrointestinal, genitourinary, skeletal, and nervous systems. Management of CRS includes a wide variety of interventions to address the full spectrum of possible anatomical abnormalities. Hence, a multidisciplinary team is also mandatory for a correct bladder and bowel management, in order to maintain continence and preserve renal function, improve quality of life and increase self-esteem.


Assuntos
Anormalidades Múltiplas , Bexiga Urinaria Neurogênica , Refluxo Vesicoureteral , Criança , Seguimentos , Humanos , Recém-Nascido , Equipe de Assistência ao Paciente , Qualidade de Vida , Estudos Retrospectivos , Bexiga Urinaria Neurogênica/etiologia
6.
Pediatr Surg Int ; 38(10): 1487-1494, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-35882671

RESUMO

PURPOSE: Urethral duplication (UD) is a rare malformation, which can be associated with other anomalies, like anorectal malformations (ARM). ARM has been described with occult spinal dysraphism (OSD). No ARM-UD-OSD combination has been reported. AIM: To share our experience and to discuss the management of ARM-UD-OSD association. METHODS: We retrospectively reviewed records of five boys with UD. Four of these had ARM-UD-OSD association. ARM was the first diagnosis in all; OSD and UD was detected during screening for associated malformation. RESULTS: All patients underwent ARM correction, 3 after colostomy. All reached fecal continence, 3 are performing bowel management. Three patients underwent UD surgical correction. Because of symptoms' worsening, 2 children had detethering surgery. At a mean follow-up of 9.5 years, all patients have normal renal function, 3 are on clean intermittent catheterization (CIC) for neurogenic bladder (1 has a cystostomy, another one an appendicostomy). CONCLUSIONS: UD and OSD should be considered in patients with ARM. Children with these conditions associated must be centralized in a third-level Center and management carefully planned; in particular, urethral reconstruction should be weighed, considering CIC could be required. Suspicion of neurogenic bladder must be present in OSD patient.


Assuntos
Malformações Anorretais , Defeitos do Tubo Neural , Bexiga Urinaria Neurogênica , Malformações Anorretais/complicações , Malformações Anorretais/diagnóstico , Malformações Anorretais/cirurgia , Criança , Humanos , Masculino , Defeitos do Tubo Neural/complicações , Estudos Retrospectivos
7.
Front Pediatr ; 10: 1057092, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36683802

RESUMO

Posterior Urethral Valves (PUV) are the most common cause of lower urinary tract obstruction. More severe forms are detected early in pregnancy (mainly type I), while other forms are usually discovered later in childhood when investigating lower urinary tract symptoms. Bladder dysfunction is common and is associated with urinary incontinence in about 55% (0%-72%). Despite the removal of the obstruction by urethral valve ablation, pathological changes of the urinary tract can occur with progressive bladder dysfunction, which can cause deterioration of the upper urinary tract as well. For this reason, all children with PUV require long-term follow-up, always until puberty, and in many cases life-long. Therefore, management of PUV is not only limited to obstruction relief, but prevention and treatment of bladder dysfunction, based on urodynamic observations, is paramount. During time, urodynamic patterns may change from detrusor overactivity to decreased compliance/small capacity bladder, to myogenic failure (valve bladder). In the past, an aggressive surgical approach was performed in all patients, and valve resection was considered an emergency procedure. With the development of fetal surgery, vesico-amniotic shunting has been performed as well. Due to improvements of prenatal ultrasound, the presence of PUV is usually already suspected during pregnancy, and subsequent treatment should be performed in high-volume centers, with a multidisciplinary, more conservative approach. This is considered to be more effective and safer. Primary valve ablation is performed after clinical stability and is no longer considered an emergency procedure after birth. During childhood, a multidisciplinary approach (pediatric urologist, nephrologist, urotherapist) is recommended as well in all patients, to improve toilet training, using an advanced urotherapy program with medical treatments and urodynamic evaluations. The aim of this paper is to present our single center experience over 30 years.

8.
J Pediatr Surg ; 56(10): 1803-1810, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34167803

RESUMO

AIM: To evaluate the correlation between sacral/vertebral anomalies and spinal cord anomalies (SCA) on MRI, in patients with anorectal malformation (ARM). METHODS: Patients with ARM consecutively treated between January 1999 and August 2019 were included. Radiological imaging of sacrum and spine were retrospectively analyzed and correlated to the presence of SCA at MRI. Fisher's exact test and X2 test were used as appropriate; p<0.05 was considered statistically significant. RESULTS: 348 patients with ARM were enrolled in the study, 147 presented SCA at MRI. 144 patients showed spinal bone anomalies, isolated vertebral and sacral anomalies were found in 17,6% and 35% respectively. Higher level of ARM was associated with a significant higher prevalence of sacral and vertebral anomalies. A significant correlation was found between the "level" of ARM and the presence of SCA (p<0.05). Sacral anomalies were significantly correlated with the presence of SCA at MRI (p<0.05). SCA were found in 70% of patients with vertebral anomalies (VA) and in 76% of patients with sacral anomalies. The presence of multiple malformations (vertebral and sacral anomalies) are strictly related to the presence of SCA. However, the absence of spinal bone anomalies does not exclude the presence of SCA. SD was the most represented type of SCA (n=94/147), of those 96% had fatty filum. Neurological or neurourological symptoms were detected in 11,5% patients (n=17) with SCA and required neurosurgical intervention. CONCLUSIONS: Our data confirm the strong relation between sacral or vertebral anomalies and SCA. However, in our series also patients without sacral/vertebral anomalies had SCA at MRI. Our results suggest that, despite the presence or absence of spinal anomalies, spinal cord MRI should be performed in all children with ARM, to allow a correct multidisciplinary follow-up and treatment. In fact, most patients with spinal bone and SCA are asymptomatic, but could develop clinical manifestations during their growth.


Assuntos
Anormalidades Múltiplas , Malformações Anorretais , Anormalidades Musculoesqueléticas , Anormalidades Múltiplas/diagnóstico por imagem , Anormalidades Múltiplas/epidemiologia , Criança , Humanos , Imageamento por Ressonância Magnética , Estudos Retrospectivos , Medula Espinal
9.
Pediatr Surg Int ; 35(2): 227-231, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30390136

RESUMO

PURPOSE: To analyze the outcome after untethering surgery in patients with spinal dysraphism (SD), with or without associated anorectal malformation (ARM). METHODS: Patients operated on for SD, with (Group A) or without (Group B) associated ARM (1999-2015), were included. The post-operative outcome was analyzed in the two groups in terms of improving of clinical symptoms (neuro-motor deficits, bladder dysfunction, bowel dysfunction) and of instrumental examinations (urodynamics, bladder ultrasound, neurophysiology). Fisher's exact test and χ2 test were used as appropriate; p < 0.05 was considered statistically significant. MAIN RESULTS: Ten patients in Group A and 24 in Group B were consecutively treated. One patient was lost at follow up. Six patients (25%) in Group B underwent prophylactic surgery. The analysis of the pre-operative symptoms in the two groups showed that a significantly higher number of patients in group A needed bowel management and presented with neuro-motor deficits, compared to group B (p = 0.0035 and p = 0.04, respectively). Group A showed a significant post-operative neuro-motor improvement as compared to group B (p = 0.002). CONCLUSIONS: Based on our results, untethering seems to be effective in neuro-motor symptoms in selected patients with ARM. In ARM patients, untethering surgery does not seem to benefit intestinal and urinary symptoms. The presence of the associated ARM does not seem to impact the medium-term outcome of patients operated for SD.


Assuntos
Malformações Anorretais/complicações , Defeitos do Tubo Neural/complicações , Defeitos do Tubo Neural/cirurgia , Disrafismo Espinal/complicações , Criança , Pré-Escolar , Constipação Intestinal/etiologia , Constipação Intestinal/cirurgia , Incontinência Fecal/etiologia , Incontinência Fecal/cirurgia , Feminino , Humanos , Masculino , Procedimentos Neurocirúrgicos , Período Pós-Operatório , Estudos Retrospectivos , Transtornos Urinários/etiologia , Transtornos Urinários/cirurgia , Urodinâmica
11.
Pediatr Surg Int ; 33(8): 843-847, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28601900

RESUMO

PURPOSE: To describe the association of anorectal malformation (ARM) and spinal dysraphism (SD) in terms of impact on the management of SD and embryogenetic implications. METHODS: Patients with SD associated with (A) or without (B) ARM were included. The two groups were further divided into operated on (A1/B1) or not (A2/B2) for SD. Groups A and B were compared for type of SD (embryogenetic classification) and prevalence of neurosurgery; Groups A1 vs. A2 for type of ARM (Wingspread classification); Groups A1 vs. B1 for age at neurosurgery, neurophysiology, and clinical symptoms. MAIN RESULTS: One hundred twenty-one patients with SD, 83 with and 38 without ARM were consecutively treated (1999-2015). Group A was associated only with SDs developing after primary neurulation, corresponding to the period of cloacal septation and organogenesis (p = 0.0007). Untethering surgery was significantly less frequent in Group A (p < 0.0001 and p = 0.04, respectively). Higher ARMs were not associated with increased risk for neurosurgery. No other significant differences were detected. CONCLUSIONS: In our series, ARMs were associated only with SD developing after primary neurulation, suggesting a single insult leading to both SD and the associated ARM. Neurosurgery is indicated less frequently in patients with ARM-associated SD, despite the similar preoperative clinical features.


Assuntos
Malformações Anorretais/complicações , Malformações Anorretais/embriologia , Disrafismo Espinal/complicações , Disrafismo Espinal/embriologia , Malformações Anorretais/diagnóstico por imagem , Criança , Desenvolvimento Embrionário , Feminino , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Procedimentos Neurocirúrgicos/métodos , Estudos Retrospectivos , Disrafismo Espinal/diagnóstico por imagem
12.
Urology ; 101: 73-79, 2017 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-27693876

RESUMO

OBJECTIVE: To define safety and effectiveness of cystostomy button in the management of bladder drainage in pediatric patients with neurogenic bladder, and report our personalized surgical technique. MATERIALS AND METHODS: This study is a retrospective analysis of patients undergoing cystostomy button placement for bladder drainage from October 2009 to December 2015. Endoscopic and open surgical techniques and medium-term complication were analyzed and indications were recorded. RESULTS: Thirty-five patients, 16 (45.7%) females and 19 (54.3%) males, underwent cystostomy button placement for bladder drainage with a mean age of 8.6 ± 4.8 years (standard deviation) and a mean follow-up time of 37 months. There were 91.4% of patients who had a neurogenic bladder; a nonobstructive urinary retention was diagnosed in the remainder of cases. A medium-term complication was mostly represented by urinary tract infection observed in 10 of 35 patients that was the most representative cause of button removal (4 of 35). Other observed complications were button leakage (n = 2), decubitus (n = 1), and bladder stone (n = 1). No postoperative complication was observed and no differences were found in terms of complications in the two surgical approaches performed. CONCLUSION: Cystostomy button is a safe and effective treatment for bladder drainage in neurogenic pediatric patients and it is also well accepted by patients and caregivers. Cystostomy button, which may avoid mechanical concerns and most of the social discomfort, should be considered an alternative method to other bladder drainage modalities.


Assuntos
Cistostomia/métodos , Endoscopia/métodos , Complicações Pós-Operatórias/epidemiologia , Bexiga Urinaria Neurogênica/cirurgia , Micção/fisiologia , Criança , Feminino , Seguimentos , Humanos , Itália/epidemiologia , Masculino , Morbidade , Estudos Retrospectivos , Taxa de Sobrevida/tendências , Resultado do Tratamento , Bexiga Urinaria Neurogênica/diagnóstico , Bexiga Urinaria Neurogênica/fisiopatologia
13.
Pediatr Surg Int ; 32(8): 729-35, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27372296

RESUMO

PURPOSE: The present study aims to identify clinical and pathological factors that can predict the risk of spinal cord anomalies (SCA) in patients with anorectal malformations (ARM), the need for neurosurgery, and to define the impact of SCA on the outcome of patients with ARM. METHODS: A 16-year retrospective analysis of all patients treated at a single tertiary children's Hospital with diagnosis of ARM. Data were collected to assess the impact of defined clinical characteristics on prevalence of SCA (detected at MRI). Children surgically treated or not for SCA were compared for age, clinical symptoms and type of anomalies at surgery or at last follow-up, respectively. Moreover, patients with intermediate/high ARMs, with or without SCA were compared for neurogenic bladder (NB), constipation, soiling and need for bowel management (BM). RESULTS: Two hundred and seventy-five children were treated for ARM in the study period, 142 had spinal MRI that showed SCA in 85. Patients with SCA had significantly higher prevalence of preterm birth (p < 0.05), cardiac anomalies (p = 0.02), vertebral anomalies (p = 0.0075), abnormal sacrum (p < 0.0001), and VACTERL association (p = 0.0233). Ten patients were surgically treated for SCA. The prevalence of neurological bladder and neuro-motor deficits, of vertebral and genital anomalies, particularly cryptorchidism, was significantly higher in the operated group (p < 0.01, for each analysis). In patients with intermediate/high ARMs, no significant difference was observed between those with or without SCA, in terms of prevalence of NB, intestinal function and need for BM. CONCLUSIONS: In patients with ARM, factors that can predict a higher prevalence of SCA and also determine an increased indication to neurosurgery may be identified. SCA by itself does not seem to affect the functional prognosis of children with intermediate/high ARM. These data may help physicians in stratifying the clinical and diagnostic pathway of patients with ARM.


Assuntos
Malformações Anorretais/epidemiologia , Medula Espinal/anormalidades , Anormalidades Múltiplas/epidemiologia , Criptorquidismo/epidemiologia , Criptorquidismo/etiologia , Feminino , Humanos , Lactente , Recém-Nascido , Itália/epidemiologia , Masculino , Estudos Retrospectivos , Bexiga Urinaria Neurogênica/epidemiologia , Bexiga Urinaria Neurogênica/etiologia
15.
Pediatr Surg Int ; 31(9): 805-8, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-26143411

RESUMO

PURPOSE: Anorectal malformations (ARM) can commonly occur in association with other congenital anomalies or as part of the combined anomaly. The present study aims to assess the outcome of patients with ARM and patients with ARM in VACTERL association. METHODS: A 12-year retrospective analysis of all patients treated at a single tertiary children's institution with a diagnosis of ARM. We identified and compared patients with ARM to those with ARM in VACTERL association (3 or more anomalies). Data were collected for both groups to assess type of ARM, urinary incontinence (UI), constipation, soiling, dietary/laxative treatment, bowel management (BM) and surgical complications. Type of lesion and clinical outcomes were classified according to Krickenbeck International classification. Patients lost to follow-up, dead or not yet toilet-trained (or <4 years old) were excluded. RESULTS: One hundred ninety-eight patients were identified, 174 enrolled in the study. Lesions were classified for each study group (VACTERL- vs VACTERL+) as perineal fistulas (36.4 vs 9.7%, p = 0.0028), rectourethral fistulas (prostatic and bulbar) (23.1 vs 38.7%, p = ns), rectovesical fistulas (3.5 vs 9.7%, p = ns), rectovestibular fistulas (19.6 vs 22.6%, p = ns), cloacal malformations (4.9 vs 9.7%, p = ns), no fistula (4.9 vs 3.2%, p=), others (7.7 vs 6.4%, p = ns). The frequency of both dietary/laxative treatment and BM, as well as surgical complications were significantly higher in patients with VACTERL. CONCLUSIONS: The coexistence of VACTERL anomalies negatively affects not only the surgical outcome but also the bowel functioning. Therefore, a dedicated follow-up is strongly recommended. Further studies are needed to assess if this has an impact on the quality of life of these patients.


Assuntos
Canal Anal/anormalidades , Anus Imperfurado/cirurgia , Esôfago/anormalidades , Cardiopatias Congênitas/terapia , Rim/anormalidades , Deformidades Congênitas dos Membros/terapia , Reto/anormalidades , Coluna Vertebral/anormalidades , Traqueia/anormalidades , Canal Anal/cirurgia , Malformações Anorretais , Feminino , Humanos , Recém-Nascido , Masculino , Reto/cirurgia , Estudos Retrospectivos , Resultado do Tratamento
16.
J Urol ; 188(3): 953-7, 2012 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-22819111

RESUMO

PURPOSE: We studied whether noninvasive urodynamic evaluation can be as effective and safe as invasive urodynamics in detecting lower urinary tract dysfunction and in preventing late onset renal failure during long-term management of boys with posterior urethral valves. MATERIALS AND METHODS: We evaluated 47 boys with posterior urethral valves using repeat urodynamics. A total of 28 patients with followup of at least 3 years and repeat evaluation of serum creatinine were included in the study. The first 14 boys in the series underwent cystometry and pressure-flow study at least every 3 years (group A), and the remaining 14 patients were monitored annually from age 5 with bladder diary, uroflowmetry, post-void residual urine on ultrasound and serum creatinine (group B). Lower urinary tract dysfunction and serum creatinine were compared (Fisher exact test and Mann-Whitney test) between groups A and B, and by stratifying patients into subgroups by age (5 to 6, 7 to 13 and older than 13 years). In all patients urodynamic diagnosis of lower urinary tract dysfunction was matched and confirmed with lower urinary tract symptoms. RESULTS: During followup the prevalence of lower urinary tract dysfunction did not differ significantly between group A (71% in boys 5 to 6, 43% in boys 7 to 13 and 85% in boys older than 13 years) and group B (36%, 43% and 60%, respectively). Late onset renal failure was observed in 2 boys in group A and 2 in group B. CONCLUSIONS: Noninvasive urodynamic evaluation seems to be as safe and effective as invasive urodynamic study in the long-term management of boys with posterior urethral valves. Based on these findings, invasive urodynamics may be reserved for cases of progressive deterioration of lower urinary tract dysfunction or renal function.


Assuntos
Uretra/anormalidades , Uretra/fisiopatologia , Bexiga Urinária/fisiopatologia , Urodinâmica , Adolescente , Criança , Pré-Escolar , Técnicas de Diagnóstico Urológico , Seguimentos , Humanos , Masculino , Fatores de Tempo
17.
J Urol ; 185(6 Suppl): 2552-7, 2011 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-21527192

RESUMO

PURPOSE: Botulinum toxin type A has gained popularity in urology. Most reported studies have been in adults at urology centers and most have addressed long-term safety. Since botulinum toxin type A treatment for neurogenic bladder dysfunction requires repeat injections, verifying that such treatment does not induce fibrosis in children seems essential. MATERIALS AND METHODS: The study was approved by the institutional review board and patients were enrolled after we obtained written consent. Patients with neurogenic bladder dysfunction not responding to conventional treatment (anticholinergics and clean intermittent catheterization) were treated with 10 IU/kg botulinum toxin type A up to a maximum of 300 IU. Endoscopic cold cup biopsies were obtained from the posterolateral bladder wall 1.5 to 2 cm above the ureteral orifice. Bladder wall findings were categorized into 3 groups, including inflammatory infiltration, edema and fibrosis. Each criterion was then graded as mild or severe and analyzed by Fisher's exact test (p <0.05). RESULTS: A total of 46 bladder wall biopsies were obtained from 40 patients 2 to 18 years old. Biopsies were evaluated in groups 1 and 2, including group 1-20 from patients with no botulinum toxin type A injection and group 2-20 after botulinum toxin type A injection. Group 2 was subdivided into group 3-10 biopsies after 1 injection and group 4-10 after multiple injections. Six patients underwent biopsy twice, that is before the first and second treatments. Histological changes were present in all biopsies. When comparing groups 1 and 2, there was no statistically significant difference in inflammation and edema. However, there was a significant difference in fibrosis between groups 1 and 4 (p <0.05) with apparently decreased fibrosis after multiple injections. CONCLUSIONS: In our experience repeat botulinum toxin type A injections into the detrusor in children do not lead to increased fibrosis in the bladder wall. This study confirms the long-term safety of botulinum toxin type A in the pediatric population.


Assuntos
Toxinas Botulínicas Tipo A/efeitos adversos , Fármacos Neuromusculares/efeitos adversos , Bexiga Urinaria Neurogênica/tratamento farmacológico , Bexiga Urinaria Neurogênica/patologia , Bexiga Urinária/efeitos dos fármacos , Bexiga Urinária/patologia , Administração Intravesical , Adolescente , Biópsia , Toxinas Botulínicas Tipo A/administração & dosagem , Toxinas Botulínicas Tipo A/uso terapêutico , Criança , Pré-Escolar , Feminino , Fibrose/induzido quimicamente , Humanos , Masculino , Fármacos Neuromusculares/administração & dosagem , Fármacos Neuromusculares/uso terapêutico
18.
J Urol ; 185(6 Suppl): 2558-62, 2011 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-21527204

RESUMO

PURPOSE: Botulinum toxin type A has revolutionized the treatment of neurogenic bladder dysfunction. The original injection technique used a rigid cystoscope and a flexible collagen needle. To date botulinum toxin type A injection techniques have not been standardized. We present our experience in pediatric patients using a new flexible injection system. MATERIALS AND METHODS: We treated 24 patients 3.8 to 17.5 years old who had neurogenic bladder dysfunction with botulinum toxin type A bladder and/or sphincter injection using a rigid cystoscope and the new N-DO™ endo-injector needle system. Another 24 patients 3.6 to 17.8 years old were treated with a 3.7Fr standard flexible needle and served as controls. Operative time, hospital stay, complications and efficacy were considered. Selection criteria and treatment were the same in the 2 groups. The 10 IU/kg dose was determined according to European Association of Urology guidelines. RESULTS: All patients received botulinum toxin type A bladder injection while 11 patients in the endo-injector group and 5 controls also received urethral injection. In the endo-injector needle and control groups average operative time was 12.4 and 17.3 minutes for the bladder, and 5.1 and 10.1 minutes for the urethra, respectively (each p <0.05). All patients were discharged home the day after the procedure. No complications were observed. Urodynamics revealed an average maximum detrusor pressure decrease of 25 and 21 cm H(2)O, and an average bladder capacity increase of 75 and 80 ml in the endo-injector and control groups, respectively (p not significant). CONCLUSIONS: While retaining efficacy, the endo-injector needle technique appears to be more rapid than the standard procedure for botulinum toxin type A injection for neurogenic bladder dysfunction. Whether patients may be treated with sedation only remains to be clarified.


Assuntos
Toxinas Botulínicas Tipo A/administração & dosagem , Fármacos Neuromusculares/administração & dosagem , Bexiga Urinaria Neurogênica/tratamento farmacológico , Administração Intravesical , Adolescente , Criança , Pré-Escolar , Desenho de Equipamento , Humanos , Injeções/instrumentação , Injeções/métodos , Agulhas
19.
Ann Vasc Surg ; 23(3): 398-409, 2009.
Artigo em Inglês | MEDLINE | ID: mdl-19427566

RESUMO

The selective blockage of platelet-derived growth factor BB (PDGF-BB), basic fibroblast growth factor (bFGF), and transforming growth factor beta1 (TGF-beta1) by specific antibodies coated into expanded polytetrafluoroethylene (ePTFE) grafts may diminish neointimal hyperplasia. Sixty pigs were divided into two groups (n = 30 each) and then further divided into five subgroups. Group 1 had a bilateral iliac artery ePTFE interposition graft precoated with Matrigel. Three subgroups (A, B, and C) received a specific monoclonal antibody against PDGF-BB, bFGF, or TGF-beta1. One (D) received all antibodies, and one served as control (nonimmune immunoglobulin G [IgG] isotypes) (E). Group 2 had a bilateral iliac artery endothelial cell (EC)-seeded ePTFE interposition graft precoated with Matrigel. Three subgroups (A, B, and C) received a specific antibody against PDGF-BB, bFGF, or TGF-beta1. One (D) received all antibodies, and one served as control (nonimmune IgG isotypes) (E). Light microscopy and immunohistochemical stain showed that neointimal hyperplasia formation was significantly reduced in subgroups D compared to the others (p < 0.05). In subgroups D, the different precoating influenced neointimal hyperplasia formation. It was more pronounced in the prosthesis precoated with EC and Matrigel (p < 0.05). In organ culture, the amount of PDGF-BB, bFGF, and TGF-beta1 release was reduced in subgroup D animals compared to the others (p < 0.05). In subgroups D, the release of PDGF-BB, bFGF, and TGF-beta1 depended on ePTFE seeding. A higher amount of these growth factors was released in the prostheses precoated with EC and Matrigel (p < 0.05), and the bromodeoxyuridine labeling index confirmed higher incorporation in this subgroup (p < 0.001). The combined use of locally administered anti-PDGF-BB, bFGF, and TGF-beta1 monoclonal antibodies reduces neointimal hyperplasia formation.


Assuntos
Anticorpos Monoclonais/administração & dosagem , Implante de Prótese Vascular/instrumentação , Prótese Vascular , Fator 2 de Crescimento de Fibroblastos/antagonistas & inibidores , Artéria Ilíaca/cirurgia , Fator de Crescimento Derivado de Plaquetas/antagonistas & inibidores , Politetrafluoretileno , Fator de Crescimento Transformador beta1/antagonistas & inibidores , Túnica Íntima/cirurgia , Animais , Becaplermina , Western Blotting , Proliferação de Células , Células Cultivadas , Materiais Revestidos Biocompatíveis , Colágeno , Combinação de Medicamentos , Células Endoteliais/metabolismo , Células Endoteliais/transplante , Ensaio de Imunoadsorção Enzimática , Feminino , Fator 2 de Crescimento de Fibroblastos/imunologia , Fator 2 de Crescimento de Fibroblastos/metabolismo , Hiperplasia , Artéria Ilíaca/metabolismo , Artéria Ilíaca/ultraestrutura , Imuno-Histoquímica , Laminina , Microscopia Eletrônica de Varredura , Modelos Animais , Técnicas de Cultura de Órgãos , Fator de Crescimento Derivado de Plaquetas/imunologia , Fator de Crescimento Derivado de Plaquetas/metabolismo , Desenho de Prótese , Proteoglicanas , Proteínas Proto-Oncogênicas c-sis , Suínos , Fator de Crescimento Transformador beta1/imunologia , Fator de Crescimento Transformador beta1/metabolismo , Túnica Íntima/metabolismo , Túnica Íntima/ultraestrutura
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