Use of a new vertical traction device for early traction-assisted staged closure of congenital abdominal wall defects: a prospective series of 16 patients.

PURPOSE: Abdominal wall closure in patients with giant omphalocele (GOC) and complicated gastroschisis (GS) remains to be a surgical challenge. To facilitate an early complete abdominal wall closure, we investigated the combination of a staged closure technique with continuous traction to the abdominal wall using a newly designed vertical traction device for newborns. METHODS: Four tertiary pediatric surgery departments participated in the study between 04/2022 and 11/2023. In case primary organ reduction and abdominal wall closure were not amenable, patients underwent a traction-assisted abdominal wall closure applying fasciotens®Pediatric. Outcome parameters were time to closure, surgical complications, infections, and hernia formation. RESULTS: Ten patients with GOC and 6 patients with GS were included. Complete fascial closure was achieved after a median time of 7 days (range 4-22) in GOC and 5 days (range 4-11) in GS. There were two cases of tear-outs of traction sutures and one skin suture line dehiscence after fascial closure. No surgical site infection or signs of abdominal compartment syndrome were seen. No ventral or umbilical hernia occurred after a median follow-up of 12 months (range 4-22). CONCLUSION: Traction-assisted staged closure using fasciotens®Pediatric enabled an early tension-less fascial closure in GOC and GS in the newborn period.

Tissue Shrinkage of Resected Specimens in Hirschsprung's Disease: Why Pediatric Surgeons Think the Bowel Specimen was Longer Than Indicated in the Pathology Report.

BACKGROUND: Hirschsprung disease (HD) is an aganglionosis of variable length starting at the rectosigmoid colon with surgery as sole therapeutic option. The length of the resected bowel segment is a crucial information for the treating surgeons and influences the prognosis of the patient. It is often artificially altered due to post operative tissue shrinkage. The objective of this study is to quantify the extent tissue shrinkage of HD specimens. MATERIAL AND METHODS: Colorectal HD specimens were measured at the time of surgery and at the time of cut-up, either fresh or after formalin fixation and statistically analyzed. RESULTS: Sixteen colorectal specimens were included. Following formalin fixation the specimen length decreased by 22.7% (P < .001). Without formalin fixation the specimens shrank by an average of 24.9% (P = .05). There was no significant difference in the extent of tissue shrinkage with or without formalin fixation (P = .76). CONCLUSION: This study showed that there is significant tissue shrinkage in HD specimens. The 2 different cohorts revealed that tissue shrinkage is mostly caused by tissue retraction/alteration after organ removal but also to a lesser extent by fixation with formalin. Surgeons and (neuro-)pathologists should be aware of the sizeable shrinking artifact to avoid unnecessary confusion.

Interstitial cells of Cajal: clinical relevance in pediatric gastrointestinal motility disorders.

Interstitial cells of Cajal (ICCs) are pacemaker cells of gastrointestinal motility that generate and transmit electrical slow waves to smooth muscle cells in the gut wall, thus inducing phasic contractions and coordinated peristalsis. Traditionally, tyrosine-protein kinase Kit (c-kit), also known as CD117 or mast/stem cell growth factor receptor, has been used as the primary marker of ICCs in pathology specimens. More recently, the Ca2+-activated chloride channel, anoctamin-1, has been introduced as a more specific marker of ICCs. Over the years, various gastrointestinal motility disorders have been described in infants and young children in which symptoms of functional bowel obstruction arise from ICC-related neuromuscular dysfunction of the colon and rectum. The current article provides a comprehensive overview of the embryonic origin, distribution, and functions of ICCs, while also illustrating the absence or deficiency of ICCs in pediatric patients with Hirschsprung disease intestinal neuronal dysplasia, isolated hypoganglionosis, internal anal sphincter achalasia, and congenital smooth muscle cell disorders such as megacystis microcolon intestinal hypoperistalsis syndrome.

Development of a Bowel Management Scoring Tool in Pediatric Patients with Constipation.

OBJECTIVE: To develop a reliable and valid scoring tool, the Pediatric Bowel Management Scoring Tool (PBMST), to better guide management of constipation in pediatric patients. STUDY DESIGN: The project comprised 2 stages, development of the questionnaire and construction of the bowel management score. Two questionnaires were created, one for children aged 8-18 years to self-report and one parent proxy-report for children aged 4-8 years. Questions regarding physical symptoms (n = 6), emotional aspects (n = 2), social activities/school (n = 1), and treatment (n = 1) were included. Patients (or parents of patients) with symptoms of constipation completed the questionnaire. The reproducibility of each question was computed using the Cohen weighted kappa coefficient (κ). A bowel management score was developed using logistic regression analysis, assessing the associations between the questions and impact on self-reported quality of life (QoL). Questions with adequate reproducibility and significantly associated with QoL were incorporated into the score. RESULTS: The questionnaire was completed by 385 patients. Six questions met the inclusion criteria and were incorporated into the score: stool shape (range, 0-3 points), anorectal pain (0-4 points), abdominal pain (0-3 points), frequency of fecal incontinence (0-3 points), assistance of caregivers (0-3 points), and interference with social activities (0-6 points). Differences in bowel management scores among patients reporting no, little, some, or major impact on QoL were statistically significant (P < .001). CONCLUSIONS: The newly developed and validated PBMST is a reliable tool for evaluating bowel management strategies in children with constipation.

Outcome of esophageal atresia: inborn versus outborn patients.

Esophageal atresia (EA) is a rare congenital disease which is usually not of the detected prenatally. Due to the lack of prenatal diagnosis, some newborns with EA are born outside of specialized centers. Nevertheless, centralized care of EA has been proposed, even if a clear volume-outcome association in EA management remains unconfirmed. Furthermore, whether outcomes differ between outborn and inborn patients with EA has not been systematically investigated. Therefore, this single-center, retrospective study aimed to investigate EA management and outcomes with a special focus on inborn versus outborn patients. The following data were extracted from the medical records of infants with EA from 2009 to 2019: EA type, associated anomalies, complications, and long-term outcome. Patients were allocated into inborn and outborn groups. Altogether, 57 patients were included. Five patients were excluded (referral before surgery, loss of data, death before surgery [n = 1], and incorrect diagnosis [diverticulum, n = 1]). Among all patients, the overall survival rate was 96%, with no mortalities among outborn patients. The overall hospitalization period was shorter for outborn patients. The median follow-up durations were 3.8 years and 3.2 years for inborn and outborn patients, respectively. Overall, 15% of patients underwent delayed primary anastomosis (long-gap atresia [n = 4] and other reasons [n = 4]). Early complications included three anastomotic leakages and one post-operative fistula; 28% of patients developed strictures, which required dilatation, and 38% of patients showed relevant gastroesophageal reflux, which required fundoplication, without any differences between the groups. The two groups had comparable low mortality and expected high morbidity with no significant differences in outcome. The outborn group showed nonsignificant trends toward lower morbidity and shorter hospitalization periods, which might be explained by the overall better clinical status.

Incidence of penalty corner injuries in international field hockey.

Field hockey penalty corners (PCs) put players at potential risk for injuries. This study evaluated the incidence of PC-related injuries with special regards to head injuries. Video sequences provided by the Fédération Internationale de Hockey (FIH) of 295 matches in 11 elite field hockey tournaments (2015-2016) were analysed for overall injuries and PC-related injuries in detail. In total, 13.9% of all match injuries (49/352 injuries) occurred in relation to a PC, accounting for 7.1 PC-related injuries per 1000 player match hours (95% CI 4.4-9.9). There were 11 head injuries afflicting six defenders and five attackers. One head injury was related to a direct hit by a ball (drag flick). This study provides the first data on PC-related injuries in elite field hockey. The recorded numbers and mechanisms of PC-related injuries should be used as a baseline for further studies and potential rule adaptations.

Rates of Complications After Appendectomy in Children and Adolescents: Pediatric Surgical Compared to General Surgical Hospitals.

BACKGROUND: Appendectomies in children and adolescents are performed in Germany in pediatric surgical (PS) or general surgical hospitals (GS). The aim of this study is to evaluate whether the surgery in a PS or GS hospital has an influence on the postoperative course after appendectomy in children and adolescents. MATERIALS AND METHODS: Nationwide routine data from children and adolescents aged 1-17 y insured by the Local Health Insurance Fund who underwent appendectomy between 2014 and 2016 were analyzed (cohort study). Descriptive statistics were calculated both overall and in the two groups (PS and GS). Patients were additionally examined by age (1-5, 6-12, and 13-17 y), treatment (laparoscopic, open surgical, and conversion), and appendicitis type (nonacute: K36/K37/K38/R10, acute simple: K35.30/K35.8, and acute complex: K35.2/K35.31/K35.32). The influence of surgeon specialization on 90-d secondary surgery and 90-d general complications was assessed by multiple logistic regression. RESULTS: Altogether, 25,065 patients who underwent surgery in 83 PS and 906 GS hospitals were included. Logistic regression analysis revealed that PS was associated with a reduced risk of interventions in the 1-5- and 6-12-y age groups (odds ratio: 0.44, 0.62). Acute complex appendicitis, comorbidities, and open surgery significantly increased the risk for reintervention. PS was associated with an increased risk for complications in the 13-17-y age group (odds ratio: 1.66). CONCLUSIONS: PS and GS hospitals provided safe appendectomies in children and adolescents with low reintervention and complication rates. PS hospitals demonstrated advantages for patients in the 1-5- and 6-12-y age groups and GS hospitals for patients 13-17 y.

Influence of COVID-19 confinement measures on appendectomies in Germany-a claims data analysis of 9797 patients.

PURPOSE: COVID-19 pandemic had multiple influences on the social, industrial, and medical situation in all affected countries. Measures of obligatory medical confinement were suspensions of scheduled non-emergent surgical procedures and outpatients' clinics as well as overall access restrictions to hospitals and medical practices. The aim of this retrospective study was to assess if the obligatory confinement (lockdown) had an effect on the number of appendectomies (during and after the period of lockdown). METHODS: This retrospective study was based on anonymized nationwide administrative claims data of the German Local General Sickness Fund (AOK). Patients admitted for diseases of the appendix (ICD-10: K35-K38) or abdominal and pelvic pain (ICD-10: R10) who underwent an appendectomy (OPS: 5-470) were included. The study period included 6 weeks of German lockdown (16 March-26 April 2020) as well as 6 weeks before (03 February-15 March 2020) and after (27 April-07 June 2020). These periods were compared to the respective one in 2018 and 2019. RESULTS: The overall number of appendectomies was significantly reduced during the lockdown time in 2020 compared to that in 2018 and 2019. This decrease affects only appendectomies due to acute simple (ICD-10: K35.30, K35.8) and non-acute appendicitis (ICD-10: K36-K38, R10). Numbers for appendectomies in acute complex appendicitis remained unchanged. Female patients and in the age group 1-18 years showed the strongest decrease in number of cases. CONCLUSION: The lockdown in Germany resulted in a decreased number of appendectomies. This affected mainly appendectomies in simple acute and non-acute appendicitis, but not complicated acute appendicitis. The study gives no evidence that the confinement measures resulted in a deterioration of medical care for appendicitis.

Video Analysis of Acute Injuries in Elite Field Hockey.

OBJECTIVE: With this study, we aim to determine the value of video assessment of acute injuries in field hockey. DESIGN: Retrospective video analysis for acute injuries. SETTING: Professional field hockey. PARTICIPANTS: Seven women and men's field hockey tournaments (World League, Champions Trophy) in 2015. INTERVENTIONS: Not applicable. OUTCOME MEASURES: An injury was defined as any new musculoskeletal complaint leading to time stoppage and medical attention. The outcome measures were time at play, location on the pitch, type of injury, injured body part, and injury mechanism. RESULTS: A total of 190 injuries occurred in 179 matches (55/73 female and 135/106 male matches). There were 0.75 injuries per match for women [confidence interval (CI, 0.31-1.13)] and 1.27 injuries per match for men (CI, 0.88-1.61). The number of injuries per 1000 player match hours was 34.20 for women (CI, 14.04-51.29) and 57.89 for men (CI, 29.78-73.12). The highest injury frequency occurred within the third quarter and within the circle. Contusion was the most common injury type (90.9% in women and 96.3% in men). The leading causes of injuries were hits by the ball or stick and collisions with another player. The head/face was the most affected body part in both sex groups (32.7% in women and 29.6% in men). CONCLUSIONS: Video analysis revealed data on injury incidence within the same range of previous reports in elite field hockey. Its advantages, such as slow motion and repetitive play of injury scenes, could contribute valuable information if incorporated into the injury recording process.

Dermatofibrosarcoma protuberans in children and adolescents: Primary and Relapsed disease-Experience of the Cooperative Weichteilsarkomstudiengruppe (CWS).

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade tumor. Little is known about best treatment of primary and relapsed disease (RD). METHODS: Treatment and outcome of 40 patients with DFSP prospectively registered within the CWS-96 and -2002P trials and the registry SoTiSaR (1996-2016) were analysed. RESULTS: Median age was 8 years (range, 0.64-17.77). Fluorescence in situ hybridization analysis to detect COL1A1-PDGFB fusion genes was positive in 86% (12/14) of evaluated patients. Primary resection was performed in all patients. Patients had IRS group I (n = 28), II (n = 9), and III (n = 2); not available (n = 1). To achieve complete remission (CR), a secondary resection was performed in 18 patients resulting in microscopically complete (R0, n = 34/40) and microscopically incomplete (R1, n = 5/40) resection. All patients achieved CR. The 5-year event-free survival (EFS) and overall survival was 86% (±12; CI, 95%) and 100% (±0; CI, 95%), respectively. R0 resection/IRS I was significantly favorable for the 5-year EFS. Local relapse occurred after a median time of 1.1 years (range, 0.04-5.1) in 15% (6/40) after CR. All patients with RD underwent resection and achieved CR. Three patients had fibrosarcomatous DFSP, two were alive after R0 resection. CONCLUSION: Complete surgical resection is mandatory to prevent relapse of DFSP.

Cholinergic innervation and ganglion cell distribution in Hirschsprung's disease.

BACKGROUND: The diagnostic gold standard of Hirschsprung's disease (HD) is based on the histopathological assessment of colorectal biopsies. Although data on cholinergic innervation and ganglion cell (GC) distribution exist, only few studies have examined these two key features together. We assessed the pattern of cholinergic innervation and the amount of GCs in colorectal specimens of 14 HD patients. METHODS: We established a semi-quantitative score for cholinergic innervation using acetylcholinesterase (AChE) enzyme histochemistry and quantitatively analyzed the number of GCs via NADH tetrazolium reductase (NADH) enzyme histochemistry. We examined both the entire length of the resected specimens as well as defined areas of the transition zone of both pathological and healthy appearing segment. RESULTS: High AChE score values were associated with absence of GCs, and AChE scores were inversely correlated with the number of GCs. Nevertheless, we observed several cases in which one of the two features revealed a normal distribution pattern, whereas the other still displayed pathological features. CONCLUSIONS: Our data support the need for transmural colon biopsies, to enable the best evaluation of both cholinergic innervation and GCs for a reliable assessment of HD.

[Kidney Cysts and Cystic Nephropathies in Children - A Consensus Guideline by 10 German Medical Societies]. / Nierenzysten und zystische Nierenerkrankungen bei Kindern (AWMF S2k-Leitlinie).

This consensus-based guideline was developed by all relevant German pediatric medical societies. Ultrasound is the standard imaging modality for pre- and postnatal kidney cysts and should also exclude extrarenal manifestations in the abdomen and internal genital organs. MRI has selected indications. Suspicion of a cystic kidney disease should prompt consultation of a pediatric nephrologist. Prenatal management must be tailored to very different degrees of disease severity. After renal oligohydramnios, we recommend delivery in a perinatal center. Neonates should not be denied renal replacement therapy solely because of their age. Children with unilateral multicystic dysplastic kidney do not require routine further imaging or nephrectomy, but long-term nephrology follow-up (as do children with uni- or bilateral kidney hypo-/dysplasia with cysts). ARPKD (autosomal recessive polycystic kidney disease), nephronophthisis, Bardet-Biedl syndrome and HNF1B mutations cause relevant extrarenal disease and genetic testing is advisable. Children with tuberous sclerosis complex, tumor predisposition (e. g. von Hippel Lindau syndrome) or high risk of acquired kidney cysts should have regular ultrasounds. Even asymptomatic children of parents with ADPKD (autosomal dominant PKD) should be monitored for hypertension and proteinuria. Presymptomatic diagnostic ultrasound or genetic examination for ADPKD in minors should only be done after thorough counselling. Simple cysts are very rare in children and ADPKD in a parent should be excluded. Complex renal cysts require further investigation.

Decentralized surgery of abdominal wall defects in Germany.

PURPOSE: Neonatal surgery for abdominal wall defects is not performed in a centralized manner in Germany. The aim of this study was to investigate whether treatment for abdominal wall defects in Germany is equally effective compared to international results despite the decentralized care. METHODS: All newborn patients who were clients of the major statutory health insurance company in Germany between 2009 and 2013 and who had a diagnosis of gastroschisis or omphalocele were included. Mortality during the first year of life was analysed. RESULTS: The 316 patients with gastroschisis were classified as simple (82%) or complex (18%) cases. The main associated anomalies in the 197 patients with omphalocele were trisomy 18/21 (8%), cardiac anomalies (32%) and anomalies of the urinary tract (10%). Overall mortality was 4% for gastroschisis and 16% for omphalocele. Significant factors for non-survival were birth weight below 1500 g for both groups, complex gastroschisis, volvulus and anomalies of the blood supply to the intestine in gastroschisis, and female gender, trisomy 18/21 and lung hypoplasia in omphalocele. CONCLUSIONS: Despite the fact that paediatric surgical care is organized in a decentralized manner in Germany, the mortality rates for gastroschisis and omphalocele are equal to those reported in international data.

Major abdominal wall defects in the low- and middle-income setting: current status and priorities.

Major congenital abdominal wall defects (gastroschisis and omphalocele) may account for up to 21% of emergency neonatal interventions in low- and middle-income countries. In many low- and middle-income countries, the reported mortality of these malformations is 30-100%, while in high-income countries, mortality in infants with major abdominal wall reaches less than 5%. This review highlights the challenges faced in the management of newborns with major congenital abdominal wall defects in the resource-limited setting. Current high-income country best practice is assessed and opportunities for appropriate priority setting and collaborations to improve outcomes are discussed.

Evaluation of the C-MAC Miller Video Laryngoscope Sizes 0 and 1 During Tracheal Intubation of Infants Less Than 10 kg.

STUDY OBJECTIVE: Video laryngoscopy has primarily been developed to assist in difficult airways. Using video laryngoscopy in pediatric airway management is an up-and-coming topic. The aim of the presented study was to compare the intubation conditions obtained when using the C-MAC video laryngoscope with Miller blades sizes 0 and 1 for standard direct laryngoscopy and indirect laryngoscopy in children weighing less than 10 kg. DESIGN: This was a prospective study. SETTING: The study was performed in a university hospital. PATIENTS: Following ethical approval, 86 infants weighing less than 10 kg and undergoing surgery under general anesthesia were studied prospectively. INTERVENTION: Indirect and direct laryngoscopy either with C-MAC Miller blade size 0 or size 1. MEASUREMENTS: First, direct laryngoscopy was performed, and the best obtained view was graded without looking at the video monitor. A second investigator blinded to the view obtained under direct laryngoscopy graded the laryngeal view on the video monitor. Time to intubation, intubation conditions, and intubation attempts were recorded. RESULTS: In infants less than 10 kg, intubation conditions were excellent. There were no significant differences between the use of Miller blade 0 or 1 in reference to Cormack-Lehane grade, time to intubation, time to best view, or intubation attempts. Comparing direct and indirect intubation conditions using either Miller blade 0 or 1 revealed that the use of indirect laryngoscopy provided a significantly better view (P < 0.05) of the vocal cords. In 3 infants weighing more than 8 kg, the Miller blade 0 was described as too short and narrow for intubation. CONCLUSIONS: Both devices allowed for an excellent visualization of the vocal cords.

[Quality Assurance Using Administrative/Routine Data for Cholelithiasis - Changes Over Time and Current Status]. / Qualitätssicherung mit Routinedaten bei Cholelithiasis ­ Veränderungen und aktueller Stand.

Quality assurance using administrative/routine data (QSR) is a relatively new measure to assess outcome quality. This approach is methodologically distinct from external quality assurance, as well as from quality assurance based upon registries. Since 2011, QSR for cholelithiasis within AOK patients has been overseen by the Scientific Institute of the AOK (WIdO). Following the introduction of an expert panel in 2013, numerous changes were put into place, whereby the indicator rates for transfusion/bleeding, various complications as well as the overall indicator were reduced. Interestingly, the risk adjusted quality differences between hospitals remained solid.

Crizotinib in ALK+ inflammatory myofibroblastic tumors-Current experience and future perspectives.

Inflammatory myofibroblastic tumor (IMT) and its subtype epithelioid inflammatory myofibroblastic sarcoma (EIMS) are rare soft-tissue tumors. As about 50% of IMT and 100% of EIMS contain activating rearrangements of the anaplastic lymphoma kinase (ALK) gene, targeted kinase inhibition of ALK by compounds such as crizotinib is a potential treatment option. We performed a literature review and analyzed a total of 30 patients with IMT/EIMS treated with crizotinib. A total of 12 patients achieved complete or partial remission. As preliminary data are promising, a prospective study evaluating crizotinib treatment in patients with unresectable/multifocal ALK+ IMT/EIMS is warranted.

Laparoscopic versus open surgery for the repair of congenital duodenal obstructions in infants and children.

BACKGROUND: Laparoscopic repair of congenital duodenal obstruction (LCDO) was described more than 15 years ago. However, studies comparing outcomes of LCDO with open repair (OCDO) are rare. Standardized assessments of complications using the Clavien-Dindo classification (CDC) and the comprehensive complication index (CCI) are not available. METHODS: All patients undergoing OCDO or LCDO between 2004 and 2017 were identified from the institutional database by retrospective analysis. Postoperative outcomes were assessed, including all complications using the CDC and the CCI. RESULTS: Forty-seven consecutive patients were identified; 27 patients underwent LCDO and 20 patients had OCDO. Both groups did not differ regarding demographics, associated congenital anomalies, intraoperative pathologic findings, and operative procedures. LCDO was associated with a longer operative time [mean (SD), 202 (89) vs. 112 (41) min, P < 0.0001], shorter time to initiation of feeds [median (range), 1 (0-4) vs. 3 (1-12) days, P = 0.0027], and shorter time to full feeds [mean (SD), 8.2 (4.1) vs. 12.2 (6.4) days, P = 0.0243] compared to OCDO. Shorter length of postoperative hospital stay in LCDO group was achieved for patients without cardiac anomalies [mean (SD), 9.4 (3.1) days in LCDO group vs. 17.2 (9.4) days in OCDO, P = 0.0396] and patients without other anomalies [median (range), 12 (3-38) days in LCDO group vs. 21 (7-31) days in OCDO, P = 0.0460]. LCDO was associated with a lower CCI [median (range) 0 (0-39.7) vs. 4.3 (0-100), P = 0.0270]. CONCLUSIONS: Despite a longer operative time for LCDO, a number of advantages of LCDO over OCDO were recognized comparing both approaches in the repair of congenital duodenal obstruction. Such advantages include a lower morbidity, reduced time to initiation and completion of full enteral feeds, and shorter length of postoperative hospitalization for patients without concomitant cardiac anomalies and for patients without other anomalies when operated laparoscopic. In view of the present results, LCDO, performed in selected patients, appears to represent a viable alternative to OCDO.

Radical Surgery Improves Survival in Patients with Stage 4 Neuroblastoma.

BACKGROUND: Neuroblastoma (NBL) is the most common extracranial solid tumor in children. Despite a good overall prognosis in NBL patients, the outcome of children with stage 4 disease, even with multimodal intensive therapy, remains poor. The role of extended surgical resection of the primary tumor is in numerous studies controversial. The aim of this study was to retrospectively analyze the impact of radical surgical resection on the overall- and event-free survival of stage 4 NBL patients. METHODS: We retrospectively analyzed patient charts of 40 patients with stage 4 NBL treated in our institution between January 1990 and May 2012. All clinical and pathological findings of stage 4 NBL patients were included. Extent of surgery was assessed from the operation records and was classified as non-radical (tumor biopsy, partial 50-90% resection) or radical (near-complete >90% resection, complete resection). Overall- (OS) and event-free (EFS) survival was assessed using the Kaplan-Meier analysis and log-rank test. A multivariate Cox regression analysis was used to demonstrate independency. RESULTS: In total, 29/40 patients were operated radically (>90% resection), whereas 11 patients received subtotal resection or biopsy only. OS and EFS were significantly increased in patients with radical operation compared with non-radical resection (p = 0.0003 for OS, p = 0.004 for EFS; log-rank test). A multivariate Cox regression analysis revealed radical operation as a significant and independent parameter for OS and EFS. CONCLUSIONS: Our data indicate that radical (over 90% resection) surgery improves OS and EFS in stage 4 NBL patients.

Consensus Review of Best Practice of Transanal Irrigation in Children.

Pediatric patients with either functional or organic bowel dysfunction may suffer from constipation and fecal incontinence and represent a complex group in whom management is often difficult. Many noninvasive and invasive treatments have been proposed, with variable efficacy and adverse effects. Transanal irrigation (TAI) is now an accepted alternative, in both children and adults, for bowel dysfunction that has not responded to conservative and medical therapies. There is, however, still some uncertainty about the use of TAI in pediatric populations. Hence, a group of specialists from different nations and pediatric disciplines, all with long-standing experience of bowel management in children, performed a literature search and had round table discussions to determine the best-practice use of TAI in the pediatric patient population. Based on these findings, this article provides best-practice recommendations on indications, patient selection, important considerations before treatment, patient and family training, treatment regimens, troubleshooting, and practical aspects of TAI. We conclude that careful patient selection, a tailored approach, directly supervised training, and sustained follow-up are key to optimize outcomes with TAI in children with functional or organic bowel dysfunction.