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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.

Background Abdominal cocoon or "encapsulating peritoneal sclerosis" (EPS) is an uncommon and rare cause of intestinal obstruction. Only a few cases have been reported in paediatric patients. Typically, EPS is described as the primary form in young adolescent girls from tropical and subtropical countries because of viral peritonitis due to retrograde menstruation or a history of peritoneal dialysis. Most patients are asymptomatic or present with abdominal pain, which is likely to occur secondary to subacute bowel obstruction. Findings at imaging, such as ultrasound, computed tomography, and magnetic resonance imaging, are often nonspecific. When diagnosed, EPS is characterized by total or partial encasement of the bowel within a thick fibrocollagenous membrane that envelopes the small intestine in the form of a cocoon because of chronic intraabdominal fibroinflammatory processes. The membrane forms a fibrous tissue sheet that covers, fixes, and finely constricts the gut, compromising its motility. Case summary We present a case of EPS in a 12-year-old boy 8 wk after primary surgery for resection of symptomatic jejunal angiodysplasia. There was no history of peritoneal dialysis or drug intake. Conclusion In this report, we sought to highlight the diagnostic, surgical, and histopathological characteristics and review the current literature on EPS in paediatric patients.

BACKGROUND In order to avoid consequences of total splenectomy, partial splenectomy (PS) is increasingly reported. The purpose of this study was to compare perioperative outcomes of laparoscopic PS (LPS) and open PS (OPS) in children and adolescents. AIM To compare perioperative outcomes of patients with LPS and OPS. METHODS After institutional review board approval, a total of 26 patients that underwent LPS or OPS between January 2008 and July 2018 were identified from the database of our tertiary referral center. In total, 10 patients had LPS, and 16 patients underwent OPS. Blood loss was calculated by Mercuriali’s formula. Pain scores, analgesic requirements and complications were assessed. The Wilcoxon rank sum test was used for comparison. To compare categorical variables, Fisher’s exact test was applied. RESULTS LPS was performed in 10 patients; 16 patients had OPS. Demographics (except for body mass index and duration of follow-up), indicating primary disease, preoperative spleen size and postoperative spleen volume, perioperative hematological parameters, postoperative pain scores, analgesic requirements, adverse events according to the Clavien-Dindo classification and the comprehensive complication index, median time from operation to initiation of feeds, median time from operation to full feeds, median time from operation to mobilization and median length of hospital stay did not differ between LPS and OPS. Median (range) operative time (min) was longer in LPS compared to the OPS group [185 (135-298) vs 144 (112-270), respectively; P = 0.048]. Calculated perioperative blood loss (mL of red blood cell count) was higher in the LPS group compared to OPS [87 (-45-777) vs -37 (-114-553), respectively; P = 0.039]. CONCLUSION This is the first study that compared outcomes of LPS and OPS. Both operative approaches had comparable perioperative outcomes. LPS appears to be a viable alternative to OPS.

The biology of cancer stem cells (CSCs) of pediatric cancers, such as hepatoblastoma, is sparsely explored. This is mainly due to the very immature nature of these tumors, which complicates the distinction of CSCs from the other tumor cells. Previously, we identified a CSC population in hepatoblastoma cell lines expressing the CSC markers CD34 and CD90, cell surface Vimentin (csVimentin) and binding of OV-6. In this study, we detected the co-expression of the immune escape factor PD-L1 in the CSC population, whereas the other tumor cells remained negative. FACS data revealed that non-CSCs give rise to CSCs, reflecting plasticity of CSCs and non-CSCs in hepatoblastoma as seen in other tumors. When we treated cells with cisplatin and decitabine, a new CD34+/lowOV-6lowCD90+ population emerged that lacked csVimentin and PD-L1 expression. Expression analyses showed that this new CSC subset shared similar pluripotency and EMT features with the already-known CSCs. FACS results further revealed that this subset is also generated from non-CSCs. In conclusion, we showed that hepatoblastoma CSCs express PD-L1 and that the biology of hepatoblastoma CSCs is of a plastic nature. Chemotherapeutic treatment leads to another CSC subset, which is highly chemoresistant and could be responsible for a poor prognosis after postoperative chemotherapy.

Hirschsprung’s disease (HD) is a congenital disorder, characterized by aganglionosis in the distal part of the gastrointestinal tract. Despite complete surgical resection of the aganglionic segment, both constipation and fecal incontinence persist in a considerable number of patients with limited treatment options. There is growing evidence for structural abnormalities in the ganglionic bowel proximal to the aganglionosis in both humans and animals with HD, which may play a role in persistent bowel dysfunction. These abnormalities include: (1) Histopathological abnormalities of enteric neural cells; (2) Imbalanced expression of neurotransmitters and neuroproteins; (3) Abnormal expression of enteric pacemaker cells; (4) Abnormalities of smooth muscle cells; and (5) Abnormalities within the extracellular matrix. Hence, a better understanding of these previously unrecognized neuropathological abnormalities may improve follow-up and treatment in patients with HD suffering from persistent bowel dysfunction following surgical correction. In the long term, further combination of clinical and neuropathological data will hopefully enable a translational step towards more individual treatment for HD.

Introduction Colonic atresia (CA) is a very rare disease. Two entities of CA can be differentiated: primary CA and CA with abdominal wall defects (AWD). This study aimed to investigate the differences between these two entities, especially for long-term outcomes. Materials and Methods Data from the major health insurance company (AOK) were analyzed. Sixty-two patients with ICD-10 codes Q42.1–2 and Q42.8–9 (atresia of the colon and rectum) who underwent surgery within 10 days after their first admission between 2007 and 2016 were obtained. Results Twenty-nine patients had an AWD, and 33 patients had primary colonic atresia (PCA). Significant differences between patients with PCA and AWD were found regarding prematurity but not for other concomitant malformations. Ostomy was the initial therapy of choice for 87.9% (29/33) of patients with PCA and 65.5% (19/29) with AWD. Central venous access was significantly more often in patients with AWD. Overall, patients with CA have an excellent short-term outcome. The 1-year mortality was 3% (1/33) in PCA and 6.9% (2/29) in AWD. Delayed management did not lead to higher mortality. Higher number of admission days and higher hospital costs were related to gastrostomy and short bowel in the long term. Conclusion The long-term outcome of CA is related to short bowel and need for initial gastrostomy but not related to AWD, prematurity, or associated anomalies.

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.

Introduction The majority of pediatric surgeons and hepatologists recommend the centralization of biliary atresia (BA) treatment within experienced liver units. We aimed to investigate whether voluntary self-restriction and acceptance of the need for this change in practice changed the BA referral policy in Germany during the last decade. Materials and Methods In cooperation with pediatric surgeons, gastroenterologists or hepatologists, and pediatric liver transplant units, the 2-year follow-up data of infants with BA born in Germany between 2010 and 2014 were collected using www.bard-online.com or pseudonymized data transfer. Results were compared with our previous analysis of the outcome data of infants with BA born between 2001 and 2005 in Germany. Result Overall, 173 infants with BA were identified, of whom 160 underwent Kasai portoenterostomy (KPE; 92.5%) and 13 (7.5%) underwent primary liver transplantation at 21 German centers. At 2-year follow-up, overall survival was 87.7% (vs. 81.9% in 2001–2005 [p = 0.19]), survival with native liver post-KPE was 29.2% (vs. 22.8% in 2001–2005 [p = 0.24]), and jaundice-free survival with native liver post-KPE was 24.0% (vs. 20.1% in 2001–2005 [p = 0.5]). Compared with the 2001–2005 analysis, all criteria showed improvement but the differences are statistically not significant. Conclusion Our observation shows that KPE management requires improvement in Germany. Centralization of BA patients to German reference liver units is not yet mandatory. However, European and national efforts with regard to the centralization of rare diseases support our common endeavor in this direction.

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 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.

Purpose The COVID-19 pandemic has led to an unprecedented expansion of telemedicine services worldwide. This study aimed to explore the practice of telemedicine in Pediatric Surgery in Germany, the impact of the pandemic on its development and parents’ and surgeons’ experiences with telemedicine. Methods The study is a cross-sectional analysis using three surveys between 6/2020 and 10/2020: (1) all Pediatric Surgery departments of Germany reported whether they provide telemedicine services. (2) Members of the German Society of Pediatric Surgery and (3) families who participated in an outpatient visit by telephone or video with the Department of Pediatric Surgery and Pediatric Urology of the University Hospital Frankfurt completed an anonymous survey on their experience with telemedicine. Results 21% of the Pediatric Surgery departments in Germany provided telemedicine, of which 57% started due to the pandemic. The lack of physical examination and face-to-face contact seem to be the major limitations to surgeons and parents. 48% of the parents answered that telemedicine is equal to or better than traditional appointments, while 33% thought that telemedicine is worse. Conclusions This study shows that families and doctors alike have had positive experiences with telemedicine and most will continue to use this format after the pandemic.

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.

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.

Anorectal malformations (ARM) represent a spectrum of rare malformations originating from a perturbated development of the embryonic hindgut. Approximately 60% occur as a part of a defined genetic syndrome or within the spectrum of additional congenital anomalies. Rare copy number variations (CNVs) have been associated with both syndromic and non-syndromic forms. The present study represents the largest study to date to explore the contribution of CNVs to the expression of ARMs. SNP-array-based molecular karyotyping was applied in 450 individuals with ARM and 4392 healthy controls. CNVs were identified from raw intensity data using PennCNV. Overlapping CNVs between cases and controls were discarded. Remaining CNVs were filtered using a stringent filter algorithm of nine filter steps. Prioritized CNVs were confirmed using qPCR. Filtering prioritized and qPCR confirmed four microscopic chromosomal anomalies and nine submicroscopic CNVs comprising seven microdeletions (del2p13.2, del4p16.2, del7q31.33, del9p24.1, del16q12.1, del18q32, del22q11.21) and two microduplications (dup2p13.2, dup17q12) in 14 individuals (12 singletons and one affected sib-pair). Within these CNVs, based on their embryonic expression data and function, we suggest FOXK2, LPP, and SALL3 as putative candidate genes. Overall, our CNV analysis identified putative microscopic and submicroscopic chromosomal rearrangements in 3% of cases. Functional characterization and re-sequencing of suggested candidate genes is warranted.

The European Union of Medical Specialists (UEMS) Section and Board of Pediatric surgery was founded more than 40 years ago. Since then major activities have been related to the improvement of quality of care of pediatric surgery in Europe. Remarkable success was achieved in the development of pediatric surgery as an independent specialty all over Europe. Other major successful activities of the UEMS Section and Board of Pediatric Surgery consisted of the development of a high-quality European examination and delineating a minimal common standard in pediatric surgery training in the form of European training requirements. Recommendations drawn from examination experience support that candidates who achieve weaker passes in part 1 may wish to consider more practice before attempting part 2 due to the weak correlation between the two scores. It may be helpful for candidates to consider having some experience working in an English-speaking clinical setting, if not truly fluent in English, to improve their chances of being successful in the part 2 examination. Other achievements of the Section were accreditation of the training centers in Europe and European Census in pediatric surgery project. All the aforementioned activities led to standardization and harmonization of pediatric surgery, as well as contributed to increasing quality of pediatric surgical service throughout Europe.

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.

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.

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.

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.

We report a case of a 2-day-old neonate with bilious vomiting and abdominal distension. A small bowel obstruction with ileal perforation due to a misplaced clamping of the umbilical cord was apparent before laparotomy. This complication was a sequala after clamping the cord too close to the abdominal wall in a case where there was a hernia into the cord with intestinal content. A herniation of abdominal contents due to an omphalocele minor or a hernia must be taken into consideration during the inspection of the umbilical cord before clamping.

Purpose This study aims to define the extent of additional surgical procedures after abdominal wall closure (AWC) in patients with gastroschisis (GS) and omphalocele (OC) with special focus on gastrointestinal related operations. Methods A retrospective chart review was performed including all operations in GS and OC patients in the first year after AWC (2010–2019). The risk for surgery was calculated using the one-year cumulative incidence (CI). Results 33 GS patients (18 simple GS, 15 complex) and 24 OC patients (12 without (= OCL), 12 OC patients with liver protrusion (= OCL +)) were eligible for analysis. 43 secondary operations (23 in GS, 20 in OC patients) occurred after a median time of 84 days (16–824) in GS and 114.5 days (12–4368) in OC. Patients with complex versus simple GS had a significantly higher risk of undergoing a secondary operation (one-year CI 64.3% vs. 24.4%; p = 0.05). 86.5% of surgical procedures in complex GS and 36.3% in OCL + were related to gastrointestinal complications. Complex GS had a significantly higher risk for GI-related surgery than simple GS. Bowel obstruction was a risk factor for surgery in complex GS (one-year CI 35.7%). Conclusion Complex GS and OCL + patients had the highest risk of undergoing secondary operations, especially those with gastrointestinal complications.

Summary 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.

The fast-evolving nature of the coronavirus disease 2019 (COVID-19) pandemic has led to unprecedented clinical, logistical, and socioeconomical challenges for health-care systems worldwide. While several studies have analyzed the impact on the presentation and management of acute appendicitis (AA) in the adult population, there is a relative paucity of similar research in pediatric patients with AA. To date, there is some evidence that the incidence of simple AA in children may have decreased during the first lockdown period in spring 2020, whereas the number of complicated AA cases remained unchanged or increased slightly. Despite a worrying trend toward delayed presentation, most pediatric patients with AA were treated expediently during this time with comparable outcomes to previous years. Hospitals must consider their individual capacity and medical resources when choosing between operative and non-operative management of children with AA. Testing for severe acute respiratory syndrome coronavirus type 2 is imperative in all pediatric patients presenting with fever and acute abdominal pain with diarrhea or vomiting, to differentiate between multisystem inflammatory syndrome and AA, thus avoiding unnecessary surgery. During the further extension of the COVID-19 crisis, parents should be encouraged to seek medical care with their children early in order that the appropriate treatment for AA can be undertaken in a timely fashion.

Introduction Inguinal hernias are repaired using either open or minimally invasive surgical techniques. For both types of surgery it has been demonstrated that a higher annual surgeon volume is associated with a lower risk of recurrence. This present study investigated the volume-outcome implications for recurrence operations, surgical complications, rate of chronic pain requiring treatment, and 30-day mortality based on the hospital volume. Materials and methods The data basis used was the routine data collected throughout the Federal Republic of Germany for persons insured by the Local General Sickness Fund “AOK” who had undergone inpatient inguinal hernia repair between 2013 and 2015. Complications were recorded by means of indicators. Hospitals were divided into five groups on the basis of the annual caseload volume: 1–50, 51–75, 76–100, 101–125, and ≥ 126 inguinal hernia repairs per year. The effect of the hospital volume on the indicators was assessed using multiple logistic regression. Results 133,449 inguinal hernia repairs were included. The incidence for recurrence operations was 0.95%, for surgical complications 4.22%, for chronic pain requiring treatment 2.87%, and for the 30-day mortality 0.28%. Low volume hospitals (1–50 and 51–75 inguinal hernia repairs per year) showed a significantly increased recurrence risk compared to high volume hospitals with ≥ 126 inguinal hernia repairs per year (odds ratio: 1.53 and 1.24). No significant correlations were found for the other results. Conclusions The study gives a detailed picture of hospital care for inguinal hernia repair in Germany. Furthermore, it was noted that the risk of hernia recurrence decreases in line with a rising caseload of the treating hospital.

Background: Congenital duodenal obstruction (CDO) can be complete (CCDO) or incomplete (ICDO). To date there is no outcome analysis available that compares both subtypes. Aim: To quantify and compare the association between CCDO and ICDO with outcome parameters. Methods: We retrospectively reviewed all patients who underwent operative repair of CCDO or ICDO in our tertiary care institution between January 2004 and January 2017. The demographics, clinical presentation, preoperative diagnostics and postoperative outcomes of 50 patients were compared between CCDO (n = 27; atresia type 1-3, annular pancreas) and ICDO (n = 23; annular pancreas, web, Ladd´s bands). Results: In total, 50 patients who underwent CDO repair were enrolled and followed for a median of 5.2 and 3.9 years (CCDO and ICDO, resp.). CCDO was associated with a significantly higher prenatal ultrasonographic detection rate (88% versus 4%; CCDO vs ICDO, P < 0.01), lower gestational age at birth, lower age and weight at operation, higher rate of associated congenital heart disease (CHD), more extensive preoperative radiologic diagnostics, higher morbidity according to Clavien-Dindo classification and comprehensive complication index (all P ≤ 0.01). The subgroup analysis of patients without CHD and prematurity showed a longer time from operation to the initiation of enteral feeds in the CCDO group (P < 0.01). Conclusion: CCDO and ICDO differ with regard to prenatal detection rate, gestational age, age and weight at operation, rate of associated CHD, preoperative diagnostics and morbidity. The degree of CDO in mature patients without CHD influences the postoperative initiation of enteral feeding.

ZusammenfassungQualitätssicherung mit Routinedaten (QSR) ist ein relativ neues Verfahren zur Bewertung der Ergebnisqualität, das sich von der externen Qualitätssicherung und der Qualitätssicherung über Registerdaten im methodischen Ansatz unterscheidet. Seitens des Wissenschaftlichen Instituts der AOK (WIdO) wird QSR seit 2011 für den Bereich Cholelithiasis bei AOK-Patienten vorgehalten. Nach Einführung eines Expertenpanels im Jahr 2013 wurden zahlreiche Veränderungen vorgenommen, die die Indikatorraten für „Transfusion/Blutung“, „Sonstige Komplikationen“ sowie den Gesamtindikator verringert, aber die risikoadjustierten Qualitätsunterschiede zwischen Kliniken keineswegs aufgehoben haben.

Aim: To evaluate the occurrence and severity of enterostomy complications in newborns suffering from different intestinal disorders. Methods: A 10-year retrospective cohort study (2008-2017) investigated newborns that underwent enterostomy formation and reversal for different intestinal disorders. Only infants less than 28 d old at the time of enterostomy creation were included in the study (corrected age was applied in the cases of preterm neonates). The patients were divided into two groups according to their underlying diseases. Group 1 included infants suffering from necrotizing enterocolitis (NEC), whereas Group 2 included newborns diagnosed with intestinal disorders other than NEC, such as meconium obstruction, anorectal malformation, focal intestinal perforation, ileus, intestinal atresia and volvulus. The primary outcome measure was enterostomy-related morbidity. The data were analyzed statistically using Pearson’s χ2 test or Fisher’s exact test for categorical variables and the Wilcoxon-Mann-Whitney U-Test for continuous variables. Results: In total, 76 infants met the inclusion criteria and were evaluated for enterostomy-related complications. Neither group showed significant differences regarding gender, gestational age, weight at birth or weight at enterostomy formation. Infants suffering from NEC (Group 1) were significantly older at enterostomy formation than the neonates of Group 2 [median (range), 11 (2-75) d vs 4 (1-101) d, P = 0.004)]. Significantly more ileostomies were created in Group 1 [47 (92.2%) vs 16 (64.0%), P = 0.007], whereas colostomies were performed significantly more often in Group 2 [2 (3.9%) vs 8 (32.0%), P = 0.002]. The initiation of enteral nutrition after enterostomy was significantly later in Group 1 infants than in Group 2 infants [median (range), 5 (3-13) vs 3 (1-9), P < 0.001]. The overall rate of one or more complications in patients of both groups after enterostomy formation was 80.3%, with rates of 86.3% in Group 1 and 68.0% in Group 2 (P = 0.073). Most patients suffered from two complications (23.7%). Four or more complications occurred in 21.6% of the infants in Group 1 and in 12.0% of the infants in Group 2 (P = 0.365). Following enterostomy closure, at least one complication was observed in 26.0% of the patients (30.6% in Group 1 and 16.7% in Group 2, P = 0.321). The occurrence of complications was not significantly different between neonates with NEC and infants with other intestinal disorders. 48 (65.8%) patients required no treatment or only pharmacological treatment for the complications that occurred [Clavien-Dindo-Classification (CDC) < III], while 25 (34.2%) required surgery to address the complications (CDC ≥ III). Early reversal of the enterostomy was performed significantly more often (P = 0.003) and the time to full enteral nutrition after closure was significantly longer [median (range), 7 (3-87) d vs 12 (5-93) d, P = 0.006] in infants with a CDC grading ≥ III than in infants with a CDC grading < III. Conclusion: Complications occur in almost all infants with enterostomies. The majority of these complications are minor and do not require surgical treatment. There is a clear trend that neonates with NEC have a higher risk for developing complications than those without NEC.

Neurogenic/neuropathic bowel dysfunction (NBD) is common in children who are affected by congenital and acquired neurological disease, and negatively impacts quality of life. In the past, NBD received less attention than neurogenic bladder, generally being considered only in spina bifida (the most common cause of pediatric NBD). Many methods of conservative and medical management of NBD are reported, including relatively recently Transanal Irrigation (TAI). Based on the literature and personal experience, an expert group (pediatric urologists/surgeons/gastroenterologists with specific experience in NBD) focused on NBD in children and adolescents. A statement document was created using a modified Delphi method. The range of causes of pediatric NBD are discussed in this paper. The various therapeutic approaches are presented to improve clinical management. The population of children and adolescents with NBD is increasing, due both to the higher survival rate and better diagnosis. While NBD is relatively predictable in producing either constipation or fecal incontinence, or both, its various effects on each patient will depend on a wide range of underlying causes and accompanying comorbidities. For this reason, management of NBD should be tailored individually with a combined multidisciplinary therapy appropriate for the status of the affected child and caregivers.