Joerg Fuchs

Surgery of cavoatrial tumor thrombus in nephroblastoma: a report of the SIOP/GPOH study.

Resection of a Wilms tumor extending through the inferior vena cava into the right atrium represents a challenge to the pediatric surgeon. Exact preoperative diagnosis is essential to identify the tumor and its in travascular extension. To achieve a complete excision of the tumor cardiopulmonary bypass and hypothermia may be required. The feasibility of a complete resection is important as it guides subsequent therapy such as chemotherapy and radiation.

Perioperative Outcome of Patients with Esophageal Atresia and Tracheo-esophageal Fistula Undergoing Open Versus Thoracoscopic Surgery

INTRODUCTION Thoracoscopic approach for repair of esophageal atresia (EA) and tracheo-esophageal fistula (TEF) has become a standard procedure in many pediatric surgical centers. Thoracoscopic surgery in a newborn is demanding from both the surgeon and the patient. The potential benefits for the newborn are still discussed by neonatologists, pediatric intensive care physicians, and also parents. The aim of our investigation was to clearly define perioperative outcome and complication rates in children undergoing thoracoscopic versus open surgery for EA and TEF repair. PATIENTS AND METHODS We reviewed the records of 68 newborns undergoing surgery for EA and TEF between March 2002 and February 2010. Patient data of open versus thoracoscopic approach were compared regarding operating time, intraoperative as well as postoperative pCO(2)max values, postoperative ventilation time, and complications. Specific patient data are reported with the median and range. Data analysis was done with the JMP(®) 7.0.2 statistical software (SAS Institute, Cary, NC). RESULTS For the 68 patients, the mean gestational age was 35 weeks (28-41), the median birth weight was 2720 g (1500-3510 g) in the thoracoscopic group and 2090 g (780-3340 g) in the open group. There were 36 girls and 32 boys. Thirty-two children had associated anomalies. Twenty-five children were undergoing a thoracoscopic procedure. In 8 cases, the operation was converted to open thoracotomy. Another 32 children received a thoracotomy. In 11 newborns, a cervical esophagostomy was performed because of long-gap EA and these patients were excluded from the study. Operating time was 141 minutes (77-201 minutes) in the thoracoscopic group and 106 minutes (48-264 minutes) in the thoracotomy group, with significant difference (P=.014). Values of pCO(2)max during operation were 62 mm Hg (34-101 mm Hg) in the thoracoscopic group and 48 mm Hg (28-89 mm Hg) in the open group, with significant difference (P=.014). Postoperative ventilation time was 3 days (1-51 days) in all groups, with no significant difference (P=.79). Early complications were noticed in 9 children undergoing thoracoscopy and in 8 patients of the thoracotomy group, again with no significant difference (P>.05). CONCLUSION Thoracoscopic repair of EA with TEF is justified because of a comparable perioperative outcome to open surgery, competitive operating times, decreased trauma to the thoracic cavity, and improved cosmesis despite skeptical considerations. Complication rates are not higher than in children operated on through a thoracotomy. However, a learning curve has to be taken into account and large experience in minimal invasive surgery is mandatory for this procedure. Larger series have to be expected for a more objective evaluation of perioperative as well as long-term outcomes. To our opinion, the thoracoscopic approach appears to be favorable and could be a future standard.

Laparoscopic Surgery on Upper Urinary Tract in Children Younger Than 1 Year: Technical Aspects and Functional Outcome

PURPOSE Minimally invasive procedures are increasingly important in pediatric urology. However, experience is still limited with minimally invasive operations on the upper urinary tract in infants. We analyzed 3 minimally invasive procedures (pyeloplasty, heminephroureterectomy and nephrectomy) in children younger than 1 year. MATERIALS AND METHODS We analyzed 67 children (mean +/- SD age 5.1 +/- 2.9 months) undergoing minimally invasive pyeloplasty in 26 patients (group 1), heminephroureterectomy in 18 (group 2) or nephrectomy in 23 (group 3) with regard to technical aspects, surgical outcome and complications. Preoperative and postoperative ultrasound and mercaptoacetyltriglycine renal scan were statistically evaluated in groups 1 and 2. RESULTS Mean +/- SD patient weight was 6.4 +/- 1.8 kg and mean +/- SD operative time was 113.2 +/- 41.6 minutes. Conversion to open surgery was necessary in 1 pyeloplasty. One complication (missed intraoperative bowel perforation) occurred. No blood transfusion was required in any child. After pyeloplasty there were improved tracer clearances (mercaptoacetyltriglycine scan) and improved morphologies of the pyelon (ultrasound) in all patients. In groups 1 and 2 there was no statistical difference between preoperative and postoperative partial function of the affected kidney. Mean +/- SD followup was 32.5 +/- 19.8 months. CONCLUSIONS Minimally invasive procedures on the upper urinary tract in children younger than 1 year are technically challenging, and require expertise of the surgeon and the entire team. Given these assumptions, such procedures can be safely performed with excellent functional outcomes.

Surgical aspects in the treatment of patients with unilateral wilms tumor: a report from the SIOP 93-01/German Society of Pediatric Oncology and Hematology.

Objective:To assess surgical aspects in the treatment of children with unilateral Wilms tumor based on data from the Cooperative Tumor Study SIOP 93-01 of the German Society of Pediatric Hematology and Oncology. Summary Background Data:Although multiple international study trials exist for the treatment of nephroblastoma, the impact of surgical details and the outcome of the patients have not yet been described comparing different approaches of the trials. Methods:Treatment results of SIOP 93-01 of the German Society of Pediatric Hematology and Oncology were analyzed regarding frequencies of operations by surgeons and hospitals, surgical approaches, and operating subspecialties. Special attention was given to surgical complications, postoperative tumor stages and event-free survival. Results:Data sets from 757 of 1020 registered patients were received for evaluation. A unilateral Wilms tumor was observed in 512 of 757 children. Median follow-up was 4.8 years (1.2–10.7). Event-free survival rates were comparable for frequencies of operation by surgeons and hospitals, surgical approaches, and surgical specialties. Intraoperative tumor rupture rates were 12% in primarily operated patients (protocol violations) versus 3.2% in patients after preoperative chemotherapy. There were 7% intraoperative ruptures for hospitals and surgeons performing 1 operation per year, and 3% when more than 4 operations per year were carried out. Sampling of hilar lymph nodes was often incomplete for all surgical subspecialties. Conclusion:While the event-free survival for all groups is equal, there may be some long-term complications as a result of the more intensified therapy required for patients who suffer intraoperative ruptures. This will be defined only with longer term studies of late effects of the more intensified therapy. There is, however an increased rate of complications and ruptures associated with the use of midline laparotomy rather than a transverse or thoracoabdominal incision.

The 'Effects of Transfusion Thresholds on Neurocognitive Outcome of Extremely Low Birth-Weight Infants (ETTNO)' Study: Background Aims, and Study Protocol

Background: Infants with extremely low birth weight uniformly develop anemia of prematurity and frequently require red blood cell transfusions (RBCTs). Although RBCT is widely practiced, the indications remain controversial in the absence of conclusive data on the long-term effects of RBCT. Objectives: To summarize the current equipoise and to outline the study protocol of the ‘Effects of Transfusion Thresholds on Neurocognitive Outcome of extremely low birth-weight infants (ETTNO)’ study. Methods: Review of the literature and design of a large pragmatic randomized controlled trial of restrictive versus liberal RBCT guidelines enrolling 920 infants with birth weights of 400–999 g with long-term neurodevelopmental follow-up. Results and Conclusions: The results of ETTNO will provide definite data about the efficacy and safety of restrictive versus liberal RBCT guidelines in very preterm infants.

The activity of γδ T cells against paediatric liver tumour cells and spheroids in cell culture

Chemoresistance and advanced tumour stage at time of diagnosis are the major reasons for poor treatment results in hepatoblastoma (HB) and paediatric hepatocellular carcinoma (HCC). Positive results with transplantation of liver and bone marrow revealed the impact of the immune system on the treatment of liver malignancies.

Perioperative Outcome of Patients with Congenital Diaphragmatic Hernia Undergoing Open Versus Minimally Invasive Surgery

PURPOSE The aim of this study was to evaluate outcome of patients with congenital diaphragmatic hernia (CDH) undergoing open versus minimally invasive surgery. SUBJECTS AND METHODS Patient records of 33 children undergoing surgery for CDH between March 2002 and September 2008 were reviewed. Patient data were compared regarding operating time, intraoperative maximum CO(2) partial pressure (pCO(2 max)) values, postoperative ventilation time, complications, and recurrences. RESULTS Median age at time of operation was 4 days (range, 0-1017 days), and median weight was 3800 g (range, 2000-13,200 g). Laparotomy was performed in 12 children. Seventeen patients underwent thoracoscopic repair, and four children had a laparoscopic approach. Operating time was significantly longer (P=.004) in the minimally invasive group. Median values of pCO(2 max) during operation were not significantly different (P=.25) in the minimally invasive surgery group. The pCO(2 max) values in the postoperative course were significantly lower (P=.013) in the minimally invasive group, whereas median ventilation times postoperatively were significantly longer (P=.024) in the open surgery group. CONCLUSIONS Median values of pCO(2 max) in the postoperative course were significantly lower in the minimally invasive surgery group. In addition, postoperative ventilation time was shorter when children underwent minimally invasive surgery. In conclusion, minimally invasive surgery seems to offer advantages for selected patients with CDH.

Surgical management of extremely low birth weight infants with neonatal bowel perforation: a single-center experience and a review of the literature.

Background: Necrotizing enterocolitis (NEC) and focal intestinal perforation (FIP) are major causes of morbidity in infants with extremely low birth weight (ELBW). Objective: To evaluate the surgical procedures applied, and the survival and long-term outcome of ELBW infants with NEC and FIP in a single-center study. Methods: Inborn and outborn ELBW infants (<1000 g) with NEC and FIP were analyzed retrospectively from 2002 to 2007. Data collected include surgical procedures, survival as well as complications, length of partial parenteral nutrition and hospital stay. The short-term and long-term outcomes after 2–7 years were assessed and compared with a matched control group. Results: Out of 280 ELBW infants, 28 underwent surgery, 19 because of FIP and 9 for NEC. Fourteen infants in the FIP group were treated with primary laparotomy and 5 with peritoneal drainage (PD). In the NEC group, only 1 infant was treated with PD. PD was used for unstable patients and was always followed by secondary laparotomy after stabilization. Five of 28 (18%) surgically treated ELBW infants and 4 (14%) matched controls died. The following complications occurred in the surgical group: complete (n = 1) or minor wound dehiscence (n = 4), stoma prolapse (n = 5), parastomal hernia (n = 2), stoma fistula (n = 1), and wound infection (n = 2). Dependency on parenteral nutrition was significantly shorter in infants with FIP, while there were no differences in time to stoma closure and length of hospital stay between those with FIP and those with NEC. Eleven of 23 (47.8%) surviving patients with FIP or NEC showed developmental delay, compared with 9 of 24 (37.5%) in the controls. Conclusions: The management of EBLW infants with NEC and FIP remains challenging. Our treatment approach was associated with low mortality. Developmental delay seems to be caused by extreme prematurity rather than NEC- or FIP-related bowel perforation.

Differential expression of glutamine synthetase and cytochrome P450 isoforms in human hepatoblastoma.

Carcinogenesis is often linked to aberrant activation of Wnt/β-catenin signalling, in many cases caused by activating CTNNB1 mutations (encoding β-catenin). Recently, β-catenin was established as a decisive regulator of hepatic glutamine synthetase (GS) and cytochrome P450 (CYP) expression in mouse hepatocarcinogenesis. This study was aimed to analyse the connection of β-catenin signalling and GS/CYP expression in human paediatric tumours. Samples from 23 paediatric tumours were analysed for activating mutations in CTNNB1. Protein expression of the model β-catenin target GS and of various CYP isoforms was analysed and correlated with CTNNB1 mutational status and histological findings. Activating CTNNB1 mutations were frequent in hepatoblastoma (80%) and nephroblastoma (31%). In CTNNB1-mutated hepatoblastoma, expression of GS was only detected in tumour areas with epithelial, not with mesenchymal differentiation. Particularly high expression of glutamine synthetase was found in hepatoblastoma cells directly neighbouring a mesenchymal-type tumour area or stroma cells, associated with above-average cell proliferation. GS expression was not observed in CTNNB1-mutated nephroblastoma. Hepatoblastoma with activated β-catenin expressed different CYPs relevant for the metabolism of cytostatic drugs, but with high interindividual variance and heterogeneity within a single tumour. GS and different CYPs are co-expressed in hepatoblastoma with activated β-catenin. Moreover, other factors like histological subtype of tumour cells and cell-cell-interactions at the borders between different areas of the tumours affect expression of these β-catenin target genes. Analysis of CYP expression in resected tumour tissue might be useful for the selection of appropriate cytostatics for post-operative chemotherapy.

Functional outcome after laparoscopic dismembered pyeloplasty in children

OBJECTIVE Laparoscopic or retroperitoneoscopic pyeloplasty for ureteropelvic junction obstruction in children has become a routine procedure. The aim of this study was to evaluate functional outcome for patients who had undergone a laparoscopic dismembered pyeloplasty. PATIENTS AND METHODS Seventy children underwent a laparoscopic dismembered pyeloplasty. Median follow up was 24 months (1-48). We reviewed differential renal function (DRF) and tracer clearance with diuretic renography before as well as 3 and 12 months after operation. Ultrasound was used to determine the grade of hydronephrosis pre- and postoperatively. RESULTS Median age at operation was 20 months (1-178). Median operating time was 140 min (95-220). Mean DRF could be preserved with no significant difference (P>0.05). All patients showed a significant improvement in tracer clearance on diuretic renography postoperatively (P<0.0001). Ultrasound examinations postoperatively showed a diminished grade of hydronephrosis without significance (P=0.657). CONCLUSION In terms of preservation of DRF the laparoscopic approach is as effective as open surgery. Tracer clearance is significantly improving. Operating times for laparoscopic pyeloplasty are competitive. We conclude that laparoscopic pyeloplasty requires extensive experience in laparoscopic pediatric urology but might replace the open surgical procedure as gold standard in the operative treatment of ureteropelvic junction obstruction.