Redo nephron-sparing surgery in stage V pediatric renal tumors - A report from the SIOP/GPOH study group for renal tumors.

PURPOSE: Nephron-sparing Surgery (NSS) is the surgical treatment of choice in children with bilateral renal tumors or in syndromatic patients. With an increasing role of this surgical approach, there is also an increased number of tumor relapses after NSS. Aim of this study was to evaluate a second ("Redo-") NSS in children with relapsed renal tumors. MATERIALS AND METHODS: We retrospectively analysed patients undergoing Redo-NSS for relapsed kidney tumors between 2009 and 2021 at our institution, which represents a national reference center of the SIOP/GPOH renal tumor study group. RESULTS: Nine patients (5 girls, 4 boys) underwent Redo-NSS with resection of 15 lesions. Mean age at surgery was 58 months (12-137), mean operative time for Redo-NSS was 195 min (137-260). R0 resection status was achieved in all children. Two patients had second relapses, one of them was resected via NSS, the other child underwent tumor nephrectomy. Two patients with anaplastic relapses died from combined second relapses. Thus, 7/9 patients are alive without evidence of disease, an impaired renal function was observed in one child. Mean follow-up after Redo-NSS was 35 months (6-49). CONCLUSIONS: In renal tumor relapses, Redo-NSS can be performed with satisfactory oncological and functional results. Occurrence of diffuse anaplasia should possibly refrain from this approach. Further evaluation in international multicenter analyses are necessary for a definitive determination of Redo-NSS.

Trap-Door Thoracotomy and Clamshell Thoracotomy as Surgical Approaches for Neuroblastoma and Other Thoracic Tumors in Children.

Solid tumors of the cervicothoracic junction, the posterior mediastinum, or bilateral dorsal thoracic tumors represent a challenge in pediatric surgical oncology. The aim of this study was to evaluate trap-door thoracotomy and clamshell thoracotomy as surgical approaches. A single-center retrospective study of children with solid tumors in these specific localizations was performed. From 2015 to 2023, 26 children (17 girls; 9 boys) were treated at a median age of 54 months (range 8-229). Tumor resection was performed for neuroblastoma (n = 11); metastatic disease (n = 7); malignant rhabdoid tumor (n = 4); Ewing sarcoma (n = 1); inflammatory myofibroblastic tumor (n = 1); rhabdomyosarcoma (n = 1); and neurofibroma (n = 1). The surgical goal of macroscopic complete excision was achieved in all of the 14 children who underwent trap-door thoracotomy and in 11 of the 12 children who underwent clamshell thoracotomy. There were no major complications. At a median follow-up of 8 months (range 0-60), the disease was under local control or in complete remission in 66.7% of the children. In conclusion, surgical resection of solid tumors of the cervicothoracic junction in children can be performed safely and successfully with trap-door thoracotomy and with clamshell thoracotomy for posterior mediastinal or bilateral dorsal thoracic tumors.

Vascular rehabilitation in children with chronic intestinal failure reduces the risk of central-line associated bloodstream infections and catheter replacements.

BACKGROUND & AIMS: Children with chronic intestinal failure (IF) require a long-term central venous catheter (CVC) for provision of parenteral nutrition. Vascular, mechanical and infectious complications such as central line-associated bloodstream infections (CLABSI) may lead to progressive loss of venous access sites. Handling and management of CVCs therefore play an important role. Our vascular rehabilitation concept (VRC) is a core component of our intestinal rehabilitation program (IRP) and consists of an education program, optimization of skin care, catheter fixation and lock solution, and the use of hybrid technique for catheter placement. Aim of this study is to analyse the effectiveness of our VRC on CLABSI rates and need for CVC replacements. METHODS: Retrospective analysis of all children treated in our IRP that were followed up between 2018 and 2023. RESULTS: A total of 117 children with chronic IF could be included for analysis of 248864 catheter days (CD). 91 patients were referred from other hospitals (127117 CD before and 89359 CD after entry into our IRP). Children receiving primary care at our IRP (32388 CD) showed a significantly lower CLABSI and line replacement rate than patients referred from external centers (p < 0.001). After entering our IRP, CLABSI rates and need for CVC replacements per 1000 CD decreased significantly in referred patients: 1.19 to 0.26 and 1.77 to 0.59, respectively (p < 0.001). CONCLUSION: Management of paediatric chronic IF patients by an IRP with a vascular rehabilitation concept significantly lowers the rate of CLABSI episodes and the need for catheter replacements.

Incidence of Isolated Biliary Atresia during the COVID Lockdown in Europe: Results from a Collaborative Project by RARE-Liver.

BACKGROUND: Biliary atresia (BA) is a rare cholangiopathy where one of the proposed aetiological mechanisms is an infectious viral trigger. Coronavirus disease-19 (COVID) lockdown restrictions were implemented to reduce the transmission of infections. Strictness of lockdown varied across European countries. This study aimed to investigate if there was an association between strictness of lockdown and change in isolated BA (IBA) incidence in Europe. METHODS: We approached European centres involved in the European Reference Network RARE-LIVER. We included IBA patients born between 2015 and June 2020. We calculated the number of IBA patients born per centre per month. The Stringency Index (SI) was used as lockdown strictness indicator. The association between percentage change of mean number of IBA patients born per month and the SI was assessed. RESULTS: We included 412 IBA patients from thirteen different centres. The median number of patients per month did not change: 6 (1-15) pre-lockdown and 7 (6-9) during lockdown (p = 0.34). There was an inverse association between SI and percentage change in IBA (B = -0.73, p = 0.03). Median age at Kasai portoenterostomy (days) did not differ between time periods (51 (9-179) vs. 53 (19-126), p = 0.73). CONCLUSION: In this European study, a stricter COVID-lockdown was seemingly accompanied by a simultaneous larger decrease in the number of IBA patients born per month in the lockdown. Results should be interpreted with caution due to the assumptions and limitations of the analysis.

Results after skin traction for femur shaft fractures in children below the age of four years.

PURPOSE: Nonsurgical management has been identified as the treatment of choice for femoral shaft fractures in children below four years of age. For various reasons, the surgical approach has become increasingly popular in recent years. The aim of this study is to report results after vertical skin traction and analyze the benefits of this technique as well as to point out advantages compared with surgery in this age group. METHODS: The authors performed a retrospective data analysis, including all patients with femoral shaft fractures below the age of four who were treated with vertical skin traction at our institution between January 2006 and December 2016. RESULTS: Skin traction for a femoral shaft fracture was performed for 36 patients (mean age 1.6 years; 1 day-3.5 years). The mean duration of traction was 18.5 days (14-30). Complications included soft tissue affections (n = 5), which all healed spontaneously. Consolidation was observed in all fractures. Initial axial deviations and shortening did not change during traction until consolidation (p > 0.05), and no relevant torsion deformity occurred (p = 0.01). Patients gained full weight-bearing within 12.3 days (7-40) following end of traction. At the final follow-up, after a mean of 29.3 months (12-192), leg-length discrepancy (mean 7.1 mm; 5-20) was found on radiograms in nine cases, and axial deviations (mean 7.7°; 5-25) were documented in seven cases. None of the patients had limitations in daily activities or sports. CONCLUSION: Skin traction is a technically easy, safe, and non-invasive treatment modality for femoral shaft fractures in children below the age of four years. Strong results are obtained benefited from a high potential of growth-related correction, and in principle no anesthesia is needed. A prolonged hospitalization and socio-economic factors maintain the ongoing debate in comparison with the surgical approach. LEVEL OF EVIDENCE: Level III, retrospective.

Gastric Transposition for Repair of Long-Gap Esophageal Atresia: Indications, Complications, and Outcome of Minimally Invasive and Open Surgery.

BACKGROUND: Gastric transposition (GT) is a possible option for esophageal replacement in long-gap esophageal atresia (LGEA). The present study aims to report and compare indications and outcome of laparoscopic-assisted GT (LAGT) versus open (OGT) GT for LGEA repair. METHODS: Retrospective single-center analysis of all LGEA patients undergoing GT between 2002 and 2021. RESULTS: Thirty-one children with LGEA underwent GT. Of these, 19 underwent LAGT (mean weight at surgery 5.6 kg; mean age 167 days) and 12 underwent OGT (6.1 kg; 233 days). Indications for OGT were previous surgery (n = 7), associated severe cardiac malformations (n = 4), and a simultaneous resection of a choledochal cyst (n = 1). The conversion rate was 1. The two procedures (LAGT/OGT) differed in anesthetic time (308/350 min), duration of ventilation (5.1/5.3 days), hospital stay (34/32 days), and complications (22/15). None of the differences reached statistical significance. Outcome was also comparable: completely oral nutrition uptake in 66%/73%, slow weight gain in the low centiles in both groups, no patient developed dumping syndrome, symptomatic reflux was seen in 1 patient after OGT. CONCLUSION: In our cohort, LAGT for repair of LGEA provided similar outcomes as open surgery. The minimally invasive approach preserves thoracal structures, prevents additional thoracotomy or laparotomy, and is faster. To realize LAGT, a postpartal treatment concept including gastrostomy placement via a microincision to minimize adhesions is essential. The open surgical approach should be considered in cases of previous extensive surgical attempts of EA correction causing severe adhesions as well as associated anomalies or genetic syndromes causing hemodynamic instability.

Analysis of NPM1 splice variants reveals differential expression patterns of prognostic value in acute myeloid leukemia.

Mutations of the nucleophosmin-1 (NPM1) gene in cytogenetically normal (CN) acute myeloid leukemia (AML) identify a group of patients with more favorable prognosis. NPM1 encodes three main alternatively spliced isoforms R1(B23.1), R2(B23.2), and R3(B23.3). The expression of splice variants R1, R2 and R3 were higher in AML patients compared to normal cells of healthy volunteers (HVs), although RNA-seq analysis revealed enhanced R2 expression also in less differentiated cells of HVs as well as in AML cells. The variant R2, which lacks exons 11 and 12 coding for the nucleolar localization domain, might behave similar to the mutant form of NPM1 (NPM1mut). In accordance, in CN-AML high R2 expression was associated with favorable impact on outcome. Moreover, functional studies showed nucleolar localization of the eGFP-NPM1 wildtype and cytoplasmic localization of the eGFP-NPM1 mut protein. While the eGFP-NPM1 R2 splice variant localized predominantly in the nucleoplasm, we also could detect cytoplasmic expression for the R2 variant. These results support a unique biological consequence of R2 overexpression and in part explain our clinical observation, where that high R2 variant expression was associated with a better prognosis in CN-AML patients.