Nephrometry Scoring Systems for Nephron Sparing Surgery in Children.

The surgical decision to attempt nephron-sparing surgery (NSS) in children with renal tumors can be difficult. In adults, nephrometric tools are used for decision-making. More than 90% of low-complexity tumors are eligible for NSS, and high-complexity tumors often require total nephrectomy. We retrospectively applied those nephrometric tools [Radius, Exophytic, Nearness to the sinus or collecting system, Anterior/posterior, Location relative to polar lines (RENAL), Preoperative Aspects and Dimensions Used for an Anatomical classification (PADUA), and Renal Tumor Invasion Index (RTII) scoring systems] to the preoperative imaging of children operated for renal tumors in our institution from 2015 to 2019 and correlated them with the type of surgery. The scores were assessed by 2 independent surgeons and 1 radiologist. Forty-four tumors were removed, including 16 NSS, 38 after neo-adjuvant chemotherapy, and 6 upfront surgeries, in 30 children. More than 50% of patients in the low and medium-risk population for RENAL, PADUA, and RTII scores, and ~15% in the high-complexity categories underwent NSS. Tumors removed through NSS were significantly less complex according to each score. Interobserver reliability was good for 3 scores. The application of the RENAL, PADUA, and RTII was able to accurately classify most of the pediatric tumors, according to their complexity. These scores could help increase the indications of NSS in renal tumor surgery.

Biliary drainage surgery before or after 3 months of life versus primary liver transplantation in children with biliary atresia: comparative cohort study.

BACKGROUND: Early biliary drainage surgery (BDS; Kasai) is associated with longer transplant-free survival in biliary atresia. However, evidence is lacking on whether an age limit can be established at which liver transplantation should be performed as first-line treatment for children with a delayed diagnosis of biliary atresia. The aim of the current study was to compare the outcome of a large cohort of children with biliary atresia who underwent BDS after 90 days of life with those who underwent early BDS (before 90 days) and those who did not receive BDS and were directly referred for primary liver transplantation. METHODS: All patients with biliary atresia treated at Bicêtre, Paris-Saclay University Hospital between 1995 and 2017 were analysed in this STROBE-compliant study. Three groups were defined: BDS before 90 days of life (early BDS); BDS after 90 days of life (late BDS); and patients without BDS who were referred for primary liver transplantation (no BDS). Patient characteristics, overall survival, and native liver survival were compared. RESULTS: Of 424 children with biliary atresia, 69 patients (16 per cent) were older than 90 days when they underwent BDS. Twenty-five patients had no BDS and were referred for primary liver transplantation (6 per cent). The main reason for not performing BDS was manifest portal hypertension (18/25). Two- and 5-year transplant-free survival were significantly higher in patients with late BDS compared with no BDS (53.5 versus 12.0 per cent respectively for 2-year data and 30.4 versus 4.0 per cent respectively for 5-year data, P < 0.001). Five- and 10-year overall survival did not differ between early BDS (92 and 91 per cent respectively), late BDS (88 and 83 per cent respectively) and no BDS (80 and 80 per cent respectively, P = 0.061). CONCLUSION: Age alone should not routinely be considered a contraindication to BDS in patients older than 90 days. Liver transplantation in infancy (less than 12 months) could be avoided in 88 per cent of cases with late diagnosis of biliary atresia and is delayed significantly even when BDS is performed after 3 months. Overall survival is at least equal to patients who are referred for primary liver transplantation.

Nephron Sparing Surgery in Bilateral Wilms Tumors With Botryoid Growth Pattern.

Botryoid growth pattern (BGP) is a polypoid mass growing into the renal pelvis, rarely seen in bilateral Wilms tumors where it represents a surgical challenge. We report our experience of nephron sparing surgery in 3 patients with BGP in bilateral Wilms tumor. Surgical en bloc removal was performed after calyx opening with no complications. The histology of the BGP was Intralobar Nephrogenic Rest in all cases while all Wilms tumors were of intermediate risk. One patient early recurred. At a follow-up of 9 months, 22 and 23 years, all patients were alive with a moderate renal insufficiency and hypertension.

Evaluation of the nephron-sparing surgery formula in Wilms tumors.

AIM: Definitions of nephron-sparing surgery (NSS) procedures in Wilms tumor (WT) are not clear. The new UMBRELLA protocol offers a formula (NSS(X)-SRM(n)-PRM(n)-RRP(n%)) to better define the different NSS parameters. We aimed to assess the advantages and limits of this new formula. METHODS: This retrospective monocentric study included patients operated by NSS for WT from 1975 to 2018. We reviewed the medical records and applied the NSS formula to all included patients. RESULTS: Eighty kidneys were operated on 56 patients at a mean age of 19.2 months (4 days-7.5 years), with 49 partial nephrectomies and 31 tumorectomies. The assessment of the surgical resection margins (SRM) showed a doubt in six cases and one tumor breach. An intact pseudocapsule along the resection margin with no renal parenchyma was found in four cases at pathological resection margins (PRM) assessment, whereas a tumor breach was described in seven cases. Among the six patients with a surgical doubt, only one had a pathological stage III. There were no surgical doubts in the seven patients with tumor breach at pathology. At a mean follow-up of eight years (15 days-28.6 years), eight patients had elevated blood pressure levels. Ten had proteinuria. These two parameters were significantly increased in patients with a remaining renal parenchyma (RRP) of less than half of the initial total renal parenchyma. The serum creatinine level was normal for all except two patients. CONCLUSION: The new NSS formula described all the crucial elements of NSS. RRP seemed essential for the evaluation of long-term renal function.

Uncommun etiology of pediatric Hematuria: urethral lymphangioma.

We report a case of urethral cystic lymphangioma. Hematuria is the revealing symptom. Im our knowledge, is the third case described in literature.