Complete surgical resection improves outcome in INRG high-risk patients with localized neuroblastoma older than 18 months

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 patients harboring MYCN amplification, extended surgery of the primary tumor site improved local control rate and survival with an acceptable risk of complications

Retrospective analysis of relapsed abdominal high-risk neuroblastoma

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

Tracheoscopic Findings and Their Impact on Respiratory Symptoms in Children with Esophageal Atresia

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

Does metabolic alkalosis influence cerebral oxygenation in infantile hypertrophic pyloric stenosis?

Background: This pilot study focuses on regional tissue oxygenation (rSO(2)) in patients with infantile hypertrophic pyloric stenosis in a perioperative setting. To investigate the influence of enhanced metabolic alkalosis (MA) on cerebral (c-rSO(2)) and renal (r-rSO(2)) tissue oxygenation, two-site near-infrared spectroscopy (NIRS) technology was applied. Materials and methods: Perioperative c-rSO(2), r-rSO(2), capillary blood gases, and electrolytes from 12 infants were retrospectively compared before and after correction of MA at admission (T1), before surgery (T2), and after surgery (T3). Results: Correction of MA was associated with an alteration of cerebral oxygenation without affecting renal oxygenation. When compared to T1, 5-min mean (+/- standard deviation) c-rSO2 increased after correction of MA at T2 (72.74 +/- 4.60% versus 77.89 +/- 5.84%; P = 0.058), reaching significance at T3 (80.79 +/- 5.29%; P = 0.003). Furthermore, relative 30-min c-rSO2 values at first 3 h of metabolic compensation were significantly lowered compared with postsurgical states at 16 and 24 h. Cerebral oxygenation was positively correlated with levels of sodium (r = 0.37; P = 0.03) and inversely correlated with levels of bicarbonate (r = -0.34; P = 0.05) and base excess (r = -0.36; P = 0.04). Analysis of preoperative and postoperative cerebral and renal hypoxic burden yielded no differences. However, a negative correlation (r = -0.40; P = 0.03) regarding hematocrite and mean r-rSO(2), indirectly indicative of an increased renal blood flow under hemodilution, was obtained. Conclusions: NIRS seems suitable for the detection of a transiently impaired cerebral oxygenation under state of pronounced MA in infants with infantile hypertrophic pyloric stenosis. Correction of MA led to normalization of c-rSO(2). NIRS technology constitutes a promising tool for optimizing perioperative management, especially in the context of a possible diminished neurodevelopmental outcome after pyloromyotomy. (C) 2017 Elsevier Inc. All rights reserved

Primary anastomosis as a valid alternative for extremely low birth weight infants with spontaneous intestinal perforation

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

Update on Transumbilical Single-Incision Laparoscopic Assisted Appendectomy (TULAA) - Which Children Benefit and What are the Complications?

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