Urological outcome with the use of salvage continent vesicostomy in pediatric reconstructive surgery. A single tertiary center experience.

PURPOSE: To report our experience and results in terms of complications, reoperation rate and urinary continence with the Salvage Continent Vesicostomy (SCV) technique in pediatric patients with history or need of enterocystoplasty and absent appendix. METHODS: Retrospective review of all patients with a history of a continent catheterizable channel surgery performed in our institution between June 2016 and January 2022. Only patients with a SCV surgery with a minimum 6-month post operative follow up were included in this cohort and divided in group 1 (history of previous bladder augmentation) and group 2 (simultaneous bladder augmentation and SCV). Primary outcome of the study was to assess both continence and postoperative complication rates. Early complications were assessed using the Clavien-Dindo classification (I-V). Late complications were focused on the need of further subfascial revision. RESULTS: 84 patients with a history of a continent stoma creation surgery were identified. In 20 of them (12 males) a SCV was performed. The mean age at surgery was 10.38 (range 4.87-15.6) years and the median postoperative follow-up time was 32 (range 6-64) months. Eleven patients were included in Group 1, while 9 patients in Group 2. Early complications occurred in 4 patients (20%), two of them required a re-intervention (Clavien-Dindo IIIb). Stoma subfascial revision was further required in 2 patient (10%). Continence rate at last follow up was 95%. CONCLUSIONS: In our early experience, the salvage continent vesicostomy has proven to be a simple continent stoma technique with acceptable both continence and complication rates that can be used in selected patients with a history or need of bladder augmentation and absent appendix.

Nephro-urological outcomes of a proactive management of children with spina bifida in their first 5 Years of life.

INTRODUCTION: The proactive management of spina bifida (SB), especially of its severe form, myelomeningocele (MMC), has contributed to decreasing chronic kidney disease (CKD). The objective of this study is to present the evolution of 5-year-old patient with MMC followed from birth with a proactive approach. MATERIAL AND METHODS: This retrospective study included 55 cases with MMC of up to 5 years of age. All of them were admitted at birth and followed by a multidisciplinary group, with a proactive approach: CIC and anticholinergics. In the same group, the variables were compared within the first year and the within the fifth year of life. Chronic kidney disease (CKD) was defined by: alterations on renal DMSA scintigraphy; alterations in microalbuminuria/creatininuria ratio, proteinuria 24 hs and decrease in glomerular filtration rate (GFR) calculated with Schwartz bedside equation. RESULTS: Although overactivity, UTI and VUR decreased throughout the first 5 years (49, 9 and 12%), reduced cystometric capacity, DLPP >40 cm of water and end-filling pressure (Pdet) >20 cm of water increased (41, 27 and 61%). All patients at 5 years of age required CIC. Reduced cystometric capacity and VUR were more significant with abnormal DMSA (36%) at 5 years old ( p: 0.03). Proteinuria and CKD increased to 25% and 49%. Similarly, the need for enalapril increased from 10% to 27%. The microalbuminuria/creatininuria ratio was pathological in 27.3%. 48 patients (87%) remained unchanged on DMSA scan and the other 7 underwent modifications (4 new cases with altered DMSA) over time. Of the 32 normal DMSA cases without changes, 81% did not present proteinuria and 88% continued to respond favorably to oxybutynin. GFR <90 ml/min/1.72m 2 was found in only 3 cases with abnormal DMSA. There was a RR 1.91 (IC95% 1.15-3.16) greater of renal compromise in cases that were anticholinergic-resistant compared to non-refractory cases. DISCUSSION: Over time, some patients suffered loss of bladder wall compliance, despite the proactive approach. There is an association between abnormal renal DMSA, reduced bladder capacity, and VUR at 5 years of age. Although proteinuria, CKD and enalapril requirement increased over 5 years, almost 90% did not show changes in renal DMSA status. CONCLUSIONS: Over time, some patients suffered loss of bladder wall compliance. Hence, even if a proactive approach is followed since birth, it is essential to continue with the ongoing monitoring of the renal status and thus avoid greater renal deterioration.

"Epstein-Barr virus associated smooth muscle tumour as an unusual cause of ureteric graft obstruction in a child".

BACKGROUND: Epstein-Barr virus (EBV) is a DNA virus with oncogenic potential, especially in immunocompromised patients. EBV can promote smooth muscle proliferation, resulting in EBV-associated smooth muscle tumors (EBV-SMT). METHODS: We report a case of a 10-year-old child with end-stage renal disease secondary to hypoplastic crossed and fused kidneys who underwent kidney transplantation. EBV serology was unknown for the donor and negative for the recipient; three months after he had a primary EBV infection. Two years after the transplantation, percutaneous nephrostomy was performed because of a drop in the estimated glomerular filtration rate and severe dilatation of the graft. Nephrography showed contrast enhancement of the pelvis of the graft kidney and proximal ureter, with a clear blockage at the level of the mid ureter and no passage towards the bladder. A 1.5-cm tumor was found causing intraluminal compression of the mid ureter. RESULTS: Complete resection of the tumor and distal ureter was performed leaving a short proximal ureter. A tension-free uretero-ureteroanastomoses was achieved using the native ureter. There were no surgical complications. Histologic evaluation showed spindle-shaped muscle cells, moderate pleomorphism, and inflammatory infiltration. Immunohistochemical staining was positive for muscle-specific actin. Epstein-Barr encoding region (EBER) in situ hybridization was positive, confirming the diagnosis of EBV-associated SMT. CONCLUSIONS: EBV-SMT is an exceedingly rare oncological entity that may develop in either the graft or any other organ. The clinical findings are location related. EBV seroconversion following transplantation might be a risk factor for the development of SMT in solid organ recipients.

Lipoblastoma retroperitoneal gigante: Caso dínico / Giant retroperitoneal lipoblastoma: Case report

El lipoblastoma forma parte de los tumores derivados del tejido adiposo. Es una neoplasia benigna que se puede presentar de forma localizada o difusa, que afecta a niños en la primera infancia y que, generalmente, se localiza en el tronco y las extremidades. Estos tumores son poco frecuentes en la edad pediátrica, pero cuando uno de ellos se presenta, constituye un desafío que se debe resolver rápidamente por el riesgo de malignidad que implican otras lesiones neoplásicas, de similares características. El diagnóstico preoperatorio es dificultoso, ya que los métodos por imágenes no aportan información específica que permita diferenciarlos de otros tumores, como los liposarcomas. El diagnóstico definitivo es anatomopalógico y, en casos dudosos, es necesaria la confirmación citogenética. Se presenta el caso de un niño de 14 meses de edad con diagnóstico de lipoblastoma de localización retroperitoneal de gran tamaño.

Lipoblastoma is part of tumors derived from adipose tissue. It is a benign neoplasm that can be localized or diffuse, affecting children in early childhood and usually located in the trunk and extremities. These tumors are uncommon in the pediatric age, but when one of them is present, it is a challenge that we must resolve quickly due to the risk of malignancy that involves other similar neoplastic lesions. The preoperative diagnosis is difficult, because the imaging methods do not provide specific information that allows us to differentiate them from other tumors, such as liposarcomas. The definitive diagnosis is anatomopathological and, in doubtful cases, cytogenetic confirmation is necessary. We present the case of a 14-month-old boy with diagnosis of large sized lipoblastoma of retroperitoneal localization.

[Giant retroperitoneal lipoblastoma. Case report]. / Lipoblastoma retroperitoneal gigante. Caso dínico.

Lipoblastoma is part of tumors derived from adipose tissue. It is a benign neoplasm that can be localized or diffuse, affecting children in early childhood and usually located in the trunk and extremities. These tumors are uncommon in the pediatric age, but when one of them is present, it is a challenge that we must resolve quickly due to the risk of malignancy that involves other similar neoplastic lesions. The preoperative diagnosis is difficult, because the imaging methods do not provide specific information that allows us to differentiate them from other tumors, such as liposarcomas. The definitive diagnosis is anatomopathological and, in doubtful cases, cytogenetic confirmation is necessary. We present the case of a 14-month-old boy with diagnosis of large sized lipoblastoma of retroperitoneal localization.

El lipoblastoma forma parte de los tumores derivados del tejido adiposo. Es una neoplasia benigna que se puede presentar de forma localizada o difusa, que afecta a niños en la primera infancia y que, generalmente, se localiza en el tronco y las extremidades. Estos tumores son poco frecuentes en la edad pediátrica, pero cuando uno de ellos se presenta, constituye un desafío que se debe resolver rápidamente por el riesgo de malignidad que implican otras lesiones neoplásicas, de similares características. El diagnóstico preoperatorio es dificultoso, ya que los métodos por imágenes no aportan información específica que permita diferenciarlos de otros tumores, como los liposarcomas. El diagnóstico definitivo es anatomopalógico y, en casos dudosos, es necesaria la confirmación citogenética. Se presenta el caso de un niño de 14 meses de edad con diagnóstico de lipoblastoma de localización retroperitoneal de gran tamaño.