A Novel Standard for Systematic Reporting of Neuroblastoma Surgery: The International Neuroblastoma Surgical Report Form (INSRF): A Joint Initiative by the Pediatric Oncological Cooperative Groups SIOPEN∗, COG∗∗, and GPOH∗∗∗.

OBJECTIVE: To create the first structured surgical report form for NBL with international consensus, to permit standardized documentation of all NBL-related surgical procedures and their outcomes. SUMMARY OF BACKGROUND DATA: NBL, the most common extracranial solid malignant tumor in children, covers a wide spectrum of tumors with significant differences in anatomical localization, organ or vessel involvement, and tumor biology. Complete surgical resection of the primary tumor is an important part of NBL treatment, but maybe hazardous, prone to complications and its role in high-risk disease remains debated. Various surgical guidelines exist within the protocols of the different cooperative groups, although there is no standardized operative report form to document the surgical treatment of NBL. METHODS: After analyzing the treatment protocols of the SIOP Europe International Neuroblastoma Study Group, Children's Oncology Group, and Gesellschaft fuer Paediatrische Onkologie und Haematologie - German Association of Pediatric Oncology and Haematology pediatric cooperative groups, important variables were defined to completely describe surgical biopsy and resection of NBL and their outcomes. All variables were discussed within the Surgical Committees of SIOP Europe International Neuroblastoma Study Group, Children's Oncology Group, and Gesellschaft fuer Paediatrische Onkologie und Haematologie - German Association of Pediatric Oncology and Haematology. Thereafter, joint meetings were organized to obtain intercontinental consensus. RESULTS: The "International Neuroblastoma Surgical Report Form" provides a structured reporting tool for all NBL surgery, in every anatomical region, documenting all Image Defined Risk Factors and structures involved, with obligatory reporting of intraoperative and 30 day-postoperative complications. CONCLUSION: The International Neuroblastoma Surgical Report Form is the first universal form for the structured and uniform reporting of NBL-related surgical procedures and their outcomes, aiming to facilitate the postoperative communication, treatment planning and analysis of surgical treatment of NBL.

Aortopexy as treatment for tracheo-bronchomalacia in children: an 18-year single-center experience.

OBJECTIVES: To define the factors influencing the outcome of aortopexy as management of tracheo-bronchomalacia. DESIGN: A retrospective, single-center, observational, cohort study. SETTINGS: Surgical services in a tertiary care hospital. PATIENTS: One hundred five children who underwent an aortopexy for tracheo-bronchomalacia between 1990 and 2008. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Comorbidity (categorized into three groups), surgical approach, and location of malacia were reviewed and analyzed in relation to mortality, need for a second intervention, complications, time to extubation after surgery, intensive care unit stay, and clinical improvement. Median age at surgery was 24 wks (1 wk-541 wks). Two surgical approaches were used: median sternotomy (n = 46) and left anterior parasternal (n = 59). Long-term (>2 yrs) follow-up was available for 73 patients (median = 7.0 yrs [2-18 yrs]); 73% were asymptomatic, 18% had minor symptoms, and 9% needed either ventilation or tracheostomy. The overall mortality rate was 9%, of which one-third was airway-related. Multivariable analysis revealed that major comorbidities were a significant risk factor both for mortality and the need for further procedures (re-do surgery, tracheostomy, internal stents) in contrast to surgical approach and involvement of the bronchus. Intensive care unit stay and days of ventilation after surgery were also significantly higher in patients with major comorbidities. CONCLUSIONS: Aortopexy proved to be an effective treatment for most cases of tracheo-bronchomalacia, but major comorbidity was associated with an adverse outcome. Our data suggest that aortopexy should be considered in most cases of severe tracheo-bronchomalacia.

Neuroblastoma of the Urinary Bladder in an Infant.

As it originates from neural crest cells, Neuroblastoma (NBL) can arise anywhere along the sympathetic chain. However, its occurrence in the urinary bladder (UB) is extremely rare. We present a case of an incidentally diagnosed pelvic NBL arising from the dome of the UB in a 7-month-old infant. The mass was treated with surgical excision only after being classified as a very low risk group according to the International Neuroblastoma Risk Group staging system. The patient was disease free after 5 years of follow-up. Although rare, we suggest that NBL should be considered in the differential diagnosis of UB masses in children and investigated accordingly.

Gastrointestinal complications associated with the surgical treatment of heart disease in children.

BACKGROUND/PURPOSE: The gastrointestinal system is prone to complications following heart surgery. We sought to determine the incidence and factors associated with gastrointestinal complication after cardiac surgery in children. METHODS: A retrospective review of patients aged <16years that underwent cardiac surgery between 2009 and 2013. Primary outcome was occurrence of gastrointestinal complication within 30days. Multivariable logistic regression was performed to identify variables related to occurrence of gastrointestinal complication. Patients with gastrointestinal complication were matched with controls and postoperative lengths of stay compared. RESULTS: Eight hundred eighty-one children underwent 1120 cardiac surgical procedures. At time of operation, 18% were neonates and 39% were infants. Cardiopulmonary bypass was used in 79%. Of 1120 procedures, 31 (2.8% [95% CI 2.0-3.9%]) had gastrointestinal complication. Necrotizing enterocolitis accounted for 61% of complications. Of patients with gastrointestinal complication, 87% survived to hospital discharge. Gastrointestinal complication was associated with preoperative co-morbidity (OR 2.2 [95% CI 1.02-4.8]) and univentricular disease (OR 2.5 [95% CI 1.1-5.5]). Neonates had the highest risk of gastrointestinal complication. Patients with gastrointestinal complications had longer hospital stays than controls (median difference, 13days [95% CI 3-43]). CONCLUSIONS: Serious gastrointestinal complications are uncommon but associated with longer hospital stay. Neonates with univentricular disease and preoperative comorbidity are at highest risk. TYPE OF STUDY: Prognosis study. LEVEL OF EVIDENCE: II.

Aortopexy for persistent tracheal obstruction after double aortic arch repair.

Persistent respiratory symptoms often occur after double aortic arch (DAA) repair but rarely require a second operation. We report 4 children with severe respiratory problems (failure to extubate, 2; severe respiratory distress, 2) caused by severe tracheomalacia and tracheal compression after DAA repair, treated by anterior aortopexy. Aortopexy proved effective and safe in improving symptoms and provides a simple treatment option for children with severe malacia or tracheal compression after DAA repair.

Patterns of expression of retinoic acid receptor beta 2 (RAR-beta2)-LacZ reporter gene in the embryonic foregut.

Vitamin A and its active form retinoic acid (RA) are essential for normal embryonic development. Maternal vitamin A deficiency in experimental animals is known to produce various developmental anomalies including foregut malformations and lung hypoplasia. However, there is a little known about the role of RA receptors in the developing foregut. Our aim was to study the pattern of expression of retinoic acid receptor beta 2 (RAR-beta2) in the region of the foregut during the early stages of embryonic development. Normal mouse embryos homozygous for the lacZ-fused RAR-beta2 promoter transgene were studied to detect the expression of RAR-beta2 in the embryonic foregut. Transverse and sagittal sections of the embryos were taken at the region of the foregut to observe for The normal pattern of expression of RARbeta2-LacZ between 9.5-12.5 days post conception. RAR-beta2-LacZ was expressed in the foregut tube on 9.5 and 10.5 dpc, mainly in the oesophageal part and maximally in the region of tracheo-oesophageal fold formation. This expression faded on day 11.5, and was not seen on 12.5 dpc. The change of RAR-beta2 expression between 9.5-11.5 dpc coincided with the time of tracheo-esophageal separation of the foregut. Our study has shown a possible RA-driven genetic activity during embryonic foregut development.

Midgut malrotation and associated Hirschsprung's disease: a diagnostic dilemma.

Malrotation and Hirschsprung's disease (HSD) are rarely reported together. We report our experience with three patients who presented during the neonatal period in whom the association resulted in diagnostic difficulty. In this report, we focus on the clinical presentation, diagnosis and appropriate management.

In vitro effects of adriamycin: a dose-response study.

The in vivo effects of adriamycin (ADR) on the mouse and rat embryos are well described in the literature. However, there is a lack of knowledge about the in vitro effects of ADR. The aim of this study was to investigate the effects of ADR on the developing mouse embryo and to identify a dose of ADR, which could be used for further studies of ADR effects in vitro. CD1 mouse embryos were collected at day 8.5 post conception. They were cultured in the presence of different doses of ADR (0, 125, 250, 375 and 500 microM). After 24 h, the culture was stopped and the embryos (n = 77) were scored morphologically using the Brown-Fabro scoring system and the mean score for each organ was calculated. Dose-response plots were generated and the effective dose 50 (ED50) for each organ was identified from the plots. The effects of ADR on the developing embryo were found to be dose related and there is a dose-response relationship in most of the plots. The dose-response plots were found to be parallel for some organs. A dose of 250 microM ADR was identified as the appropriate dose for further in vitro studies. The effects of ADR on the embryos were dose related and there is a dose-response relationship in most of the developing systems. The presence of parallel dose-response plots for some regions is suggestive of similar mechanism of action of ADR on these regions. A dose of 250 microM of ADR was identified for the first time in the literature and could be used for further studies of the effects of ADR on the mouse foregut in vitro.