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The aim was to assess the results of primary anastomosis (PA) compared to enterostomy (ES) in infants with spontaneous intestinal perforation (SIP) and a weight below 1000 g. Between 2014 and 2016, enterostomy was routinely carried out on extremely low birth weight (ELBW) patients with SIP. From 2016 until 2019, all patients underwent anastomosis without stoma formation. We compared outcome and complications in both groups. Forty-two patients with a median gestational age of 24.3 weeks and a birth weight of 640 g with SIP were included. Thirty patients underwent PA; ES was performed in 12 patients. Overall in-hospital mortality was 11.9% (PA: 13.3%, ES: 8.3%). Reoperations due to complications became necessary in 10/30 patients with PA and 4/12 patients with ES. Length of stay was 110.5 days in the PA group and 124 days in the ES group. Median weight at discharge was higher in the PA group (PA: 2258 g, ES: 1880 g, p = .036). Conclusion: Primary anastomosis is a feasible treatment option for SIP in infants < 1000 g and may have a positive impact on weight gain and length of hospitalization. However, further studies on selection criteria for PA are necessary. What is Known: center dot Enterostomy (ES) and primary anastomosis (PA) are feasible treatment options in preterm infants with spontaneous intestinal perforation (SIP). center dot Stomal complications or failure to thrive due to poor food utilization can pose significant problems. What is New: center dot Primary anastomosis in case of SIP is equal to enterostomy in terms of mortality and revision rate; however, length of stay and weight gain can be presumably positively influenced. center dot Primary anastomosis is a valid treatment option even for patients weighing less than 1000 g.

Introduction Esophageal atresia (EA) is often accompanied by tracheobronchial malformations leading to stridor, recurrent bronchitis, and occasionally to life-threatening obstructive apnea after surgical repair. The aim of this study was to identify the presence of tracheomalacia in patients with EA and tracheoesophageal fistula (TEF) pre- and postoperatively and to find endoscopic correlates leading to clinical airway symptoms. Methods In a cohort of 362 patients with EA-TEF who underwent 595 tracheoscopies at the Children's Hospital of Cologne between January 1983 and December 2002, impaired tracheal lumen, localization of TEF, tracheal pulsations, and corresponding clinical symptoms were retrospectively analyzed. Results The incidence of tracheomalacia was higher in patients with EA and TEF (Gross B-D) compared with patients with EA alone (Gross A) and average tracheal collapse does not significantly change before and after surgical repair of the esophagus in all types. Patients with cyanosis while eating and obstructive apnea presented with an average tracheal collapse of 89%. The presence of respiratory symptoms such as cough, stridor, or bronchitis was not associated with a higher grade of tracheal collapse compared with patients without any airway symptoms (average tracheal collapse of 37% in symptomatic patients vs. 33% in nonsymptomatic patients). Conclusion Tracheomalacia tends to be present independently of surgical procedure. Tracheomalacia should be measured by tracheoscopy (in % of tracheal collapse). Patients with a tracheal collapse of >80%, a ventral pulsation, and obstructive apnea or cyanosis in combination, are at risk for life-threatening situations and further surgical treatment should be considered.

PURPOSE Telomere maintenance is a hallmark of high-risk neuroblastoma; however, the contribution of telomerase and alternative lengthening of telomeres (ALT) to clinical phenotypes has remained unclear. We aimed to determine the clinical relevance of telomerase activation versus ALT as biomarkers in pretreatment neuroblastoma and to assess the potential value of telomerase as a therapeutic target. MATERIALS AND METHODS The genomic status of TERT and MYCN was assessed in 457 pretreatment neuroblastomas by fluorescence in situ hybridization. ALT was examined in 273 of 457 tumors by detection of ALT-associated promyelocytic leukemia nuclear bodies, and TERT expression was determined by microarrays in 223 of these. Cytotoxic effects of telomerase-interacting compounds were analyzed in neuroblastoma cell lines in vitro and in vivo. RESULTS We detected TERT rearrangements in 46 of 457 cases (10.1%), MYCN amplification in 93 of 457 cases (20.4%), and elevated TERT expression in tumors lacking TERT or MYCN alterations in 10 of 223 cases (4.5%). ALT activation was found in 49 of 273 cases (17.9%). All these alterations occurred almost mutually exclusively and were associated with unfavorable prognostic variables and adverse outcome. The presence of activated telomerase (ie, TERT rearrangements, MYCN amplification, or high TERT expression without these alterations) was associated with poorest overall survival and was an independent prognostic marker in multi-variable analyses. We also found that the telomerase-interacting compound 6-thio-2'-deoxyguanosine effectively inhibited viability and proliferation of neuroblastoma cells bearing activated telomerase. Similarly, tumor growth was strongly impaired upon 6-thio-2'-deoxyguanosine treatment in telomerase-positive neuroblastoma xenografts in mice. CONCLUSION Our data suggest telomerase activation and ALT define distinct neuroblastoma subgroups with adverse outcome and that telomerase may represent a promising therapeutic targe

Background/purpose: The impact of abdominal topography and surgical technique on resectability and local relapse pattern of relapsed abdominal high-risk neuroblastoma (R-HR-NB) is not clearly defined. Methods: A sample of thirty-nine patients with R-HR-NB enrolled in the German neuroblastoma trials between 2001 and 2010 was analyzed retrospectively using surgical and imaging reports. We evaluated resectability and local relapse pattern within 6 standardized abdominal regions, impact of extent of the first resective surgery on overall survival (OS), and of number of operations and a higher cumulative surgical assessment score (C-SAS) on OS after the first event. Results: In the left upper abdomen, rates for tumor persistence and relapse were 45.9% and 13.5% and in the left lower abdomen 27.7% and 8.3%, respectively. OS in months did not differ between complete and incomplete first resections (median (interquartile range): 35 (45.6) vs. 40 (65.4), P = .649). Better OS after the first event was associated with repeated as compared to single surgery (47.7 (64.7) vs. 4.3 (12.5), P = .000), and with higher as compared to lower C-SAS (47.7 (64.3) vs. 7.6 (14.7), P = .002). Conclusions: OS after relapse/progression was not dependent on the extent of first resection. The number of operations was associated with better outcome after event. Type of study: Treatment study. (C) 2017 Elsevier Inc. All rights reserved.

Background Transumbilical laparoscopic-assisted appendectomy (TULAA) is fast and cost-effective since no endoloops, staplers or wound protection devices are used. We analyzed the effects of TULAA as first approach for perforated (PA) and non-perforated (NPA) appendicitis in children. Patients We performed a retrospective analysis of 181 children for whom TULAA was the first approach for appendicitis between October 2010 and March 2016. Methods Morbidity, additional laparoscopic instrument insertion (AI), conversions to open extraumbilical appendectomy (OC), and complications were evaluated. Results TULAA was initiated in 181 (87.4%) children (113 boys: 68 girls). Median age was 10.3 years (3.3-13.9 years) and BMI 16.8kg/m2 (12.4-30.8). Appendicitis was non-perforated in 157 (86.7%) and perforated in 24 (13.3%) patients. TULAA was finalized in 142 (78.5%) patients, AI were inserted in 20 (11%) and OC were performed in 19 (10.5%) patients. Duration of surgery did not exceed 20min for 12.8%, and 30min for 43.6% of patients with TULAA and NPA. The rate of wound infections did not differ between procedures (TULAA 3/142 (2.1%), AI 0 (0%), OC 1/19 (5.3%), P=1.000). Further postoperative course was uneventful in 179 (98.9%) patients. Conclusion TULAA can be used as first approach for appendicitis in all children with a low rate of complications. Extracorporeal appendix stump closure can be safely achieved in the majority of children without using laparoscopic disposable devices.

Background The incidence of invasive aspergillosis (IA) in children with hematooncological malignancies is increasing as a result of intensive treatment, immunosuppression, and extended use of broad-spectrum antibiotics. Infection of the GI tract by Aspergillus spp. is a rare and fatal complication, which often requires surgical diagnostic and therapeutic exploration. Objective The aim of this study was to determine the characteristics of symptomatic intestinal aspergillosis, diagnosis, treatment, and outcome of pediatric patientswith an underlying hemato-oncologic disease. Patients andMethods We analyzed 2,307 German patients with acute lymphoblastic leukemia (ALL) from age 1 to 17 years registered in the AIEOP-BFM ALL 2000 study from 2000 to 2006. All reported adverse events were assessed for symptoms of IA and retrospectively reviewed for any sign or proof of intestinal involvement of IA. Results In this cohort, IA was reported in 30 of 2,307 patients while intestinal involvement was documented in five patients. Four of these patients had intestinal symptoms and three patients underwent explorative laparotomy. Among clinical cases with IA, gastrointestinal manifestation of IA mostly occurred in adolescent patients (10-16 years). Symptoms varied from abdominal tenderness and pain to constipation. Intestinal aspergillosis was proven by microbiological and histopathological examination and fungal infection was observed macroscopically in the jejunal lumen during surgery. Despite the extended surgery and antifungal therapy, outcome of disseminated IA with intestinal involvement remains poor. Conclusion Surgeons should be aware of surgical complications of intestinal aspergillosis in children with hematooncological diseases requiring exploration and resection. IA is a rare event and still difficult to diagnose due to unspecific abdominal symptoms. Thus, biopsy sampling is of utmost importance to ensure diagnosis, and resection of necrotic or perforated tissue should b

Background: Although several studies have been conducted on the role of surgery in localized neuroblastoma, the impact of surgical timing and extent of primary tumor resection on outcome in high-risk patients remains controversial. Methods: Patients from the German neuroblastoma trial NB97 with localized neuroblastoma INSS stage 1-3 age > 18 months were included for retrospective analysis. Imaging reports were reviewed by two independent physicians for Image Defined Risk Factors (IDRF). Operation notes and corresponding imaging reports were analyzed for surgical radicality. The extent of tumor resection was classified as complete resection (95-100%), gross total resection (90-95%), incomplete resection (50-90%), and biopsy (<50%) and correlated with local control rate and outcome. Patients were stratified according to the International Neuroblastoma Risk Group (INRG) staging system. Survival curves were estimated according to the method of Kaplan and Meier and compared by the log-rank test. Results: A total of 179 patients were included in this study. 77 patients underwent more than one primary tumor operation. After best surgery, 68.7% of patients achieved complete resection of the primary tumor, 16. 8% gross total resection, 14.0% incomplete surgery, and 0.5% biopsy only. The cumulative complication rate was 20.3% and the surgery associated mortality rate was 1.1%. Image defined risk factors (IDRF) predicted the extent of resection. Patients with complete resection had a better local-progression-free survival (LPFS), event-free survival (EFS) and OS (overall survival) than the other groups. Subgroup analyses showed better EFS, LPFS and OS for patients with complete resection in INRG high-risk patients. Multivariable analyses revealed resection (complete vs. other), and MYCN (non-amplified vs. amplified) as independent prognostic factors for EFS, LPFS and OS. Conclusions: In patients with localized neuroblastoma age 18 months or older, especially in INRG high-risk

The self-assembly of tris(bidentate) chiral complexes, [M(4- carboxyphenyldipyrrinato)3] (M = Co(iii), Rh(iii)), with a threefold-symmetric array of peripheral carboxyl groups is detailed. Thin sheet-like aggregates are observed in methanol/water mixtures