Erkrankungen des unteren Gastrointestinaltrakts

Gontard A., Hussong J., Yang S.S., Chase J., Franco I., Wright A.

Neurodevelopmental disorders (NDs) are incapacitating disorders, which begin early in life, are mainly caused by genetic and neurobiological factors, and show a tendency to persist. They are associated with higher rates of incontinence in children and adolescents, including nocturnal enuresis, daytime urinary incontinence, fecal incontinence, and constipation. Without diagnosis and treatment, they will interfere with incontinence treatment leading to less favorable outcomes. The aim of this International Children's Continence Society (ICCS) document is to provide an overview of the three most important NDs, that is, attention-deficit/hyperactivity disorder, autism spectrum disorder (ASD), and intellectual disability (ID).This consensus paper was commissioned by the ICCS. A selective, nonsystematic review was performed. Guidelines, reviews, and selected studies were included. The recommendations are consensus-based.ADHD is the most common ND with special relevance in clinical practice. ASD and ID are less common, but more severe disorders than ADHD. Basic principles of the assessment and treatment of NDs are provided. Incontinence is common among patients with NDs. Specific modifications and practical approaches in the treatment of incontinence in children with NDs are outlined.Incontinence in children and adolescents with NDs is common. Effective treatment of incontinence should be adapted and modified to the specific needs of patients with NDs. A multiprofessional approach is recommended.

Worm M., Reese I., Ballmer-Weber B., Beyer K., Bischoff S.C., Bohle B., Brockow K., Claßen M., Fischer P.J., Hamelmann E., Jappe U., Tebbe J.K., Klimek L., Koletzko B., Lange L., et. al.

Pogorelić Z., Huskić D., Čohadžić T., Jukić M., Šušnjar T.

Background: Percutaneous internal ring suturing (PIRS) is a simple and popular technique for the treatment of inguinal hernia in children. The aim of this study was to analyze the learning curves during implementation of PIRS in our department. Methods: A total of 318 pediatric patients underwent hernia repair using the PIRS technique by three pediatric surgeons with different levels of experience in laparoscopic surgery. These patients were enrolled in a prospective cohort study during the period October 2015–January 2021. Surgical times, intraoperative and postoperative complications, in addition to outcomes of treatment were compared among the pediatric surgeons. Results: Regarding operative time a significant difference among the surgeons was found. Operative time significantly decreased after 25–30 procedures per surgeon. The surgeon with advanced experience in laparoscopic surgery had significantly less operative times for both unilateral (12 (interquartile range, IQR 10.5, 16.5) min vs. 21 (IQR 16.5, 28) min and 25 (IQR 21.5, 30) min; p = 0.002) and bilateral (19 (IQR 14, 21) min vs. 28 (IQR 25, 33) min and 31 (IQR 24, 36) min; p = 0.0001) hernia repair, compared to the other two surgeons. Perioperative complications, conversion, and ipsilateral recurrence rates were higher at the beginning, reaching the benchmarks when each surgeon performed at least 30 PIRS procedures. The most experienced surgeon had the lowest number of complications (1.4%) and needed a fewer number of cases to reach the plateau. The other two surgeons with less experience in laparoscopic surgery had higher rates of complications (4.4% and 5.4%) and needed a higher number of cases to reach the plateau (p = 0.190). Conclusions: A PIRS learning curve for perioperative and postoperative complications, recurrences, and conversion rates reached the plateau after each surgeon had performed at least 30 cases. After that number of cases PIRS is a safe and effective approach for pediatric hernia repair. A surgeon with an advanced level of experience in pediatric laparoscopic surgery adopted the technique more easily and had a significantly faster learning curve.

Bielicki I.N., Somme S., Frongia G., Holland-Cunz S.G., Vuille-dit-Bille R.N.

Gastroschisis and omphalocele reflect the two most common abdominal wall defects in newborns. First postnatal care consists of defect coverage, avoidance of fluid and heat loss, fluid administration and gastric decompression. Definitive treatment is achieved by defect reduction and abdominal wall closure. Different techniques and timings are used depending on type and size of defect, the abdominal domain and comorbidities of the child. The present review aims to provide an overview of current treatments.

Adams A.D., Stover S., Rac M.W.

An omphalocele is a congenital defect in the abdominal wall characterized by absent abdominal muscles, fascia, and skin. The characteristic ultrasound appearance includes a midline defect with herniation of abdominal contents into the base of the umbilical cord. Other anatomic abnormalities are seen in approximately 50% of cases, most notably cardiac defects (19%-32%). Approximately, 50% of cases are associated with genetic and multiple malformation syndromes including trisomy 13/18, pentalogy of Cantrell and Beckwith-Wiedemann syndrome. Therefore, a thorough evaluation is recommended, including detailed anatomic survey, fetal echocardiogram, genetic counseling, and prenatal diagnostic testing. Overall prognosis depends on the size of the omphalocele, genetic studies, and associated anomalies. Early prenatal diagnosis remains important in order to provide parental counseling and assist in pregnancy management. Delivery should occur at a tertiary care center. Timing and mode of delivery should be based on standard obstetric indications with cesarean delivery reserved for large omphalocele (>5 cm) or those that involve the fetal liver. Neonatal management involves either primary or staged reduction, both of which can be associated with a prolonged neonatal hospitalization.

Rajindrajith S., Devanarayana N.M., Thapar N., Benninga M.A.

Functional fecal incontinence (FI) is a worldwide problem in children and comprises constipation-associated FI and nonretentive FI. Irrespective of pathophysiology, both disorders impact negatively on the psychological well-being and quality of life of affected children. A thorough clinical history and physical examination using the Rome IV criteria are usually sufficient to diagnose these conditions in most children. Evolving investigations such as high-resolution anorectal and colonic manometry have shed new light on the pathophysiology of functional FI. Although conventional interventions such as toilet training and laxatives successfully treat most children with constipation-associated FI, children with nonretentive FI need more psychologically based therapeutic options. Intrasphincteric injection of botulinum toxin, transanal irrigation and, in select cases, surgical interventions have been used in more resistant children with constipation-associated FI.

Ring J., Beyer K., Biedermann T., Bircher A., Fischer M., Fuchs T., Heller A., Hoffmann F., Huttegger I., Jakob T., Klimek L., Kopp M.V., Kugler C., Lange L., Pfaar O., et. al.

Chen C.B., Tahboub F., Plesec T., Kay M., Radhakrishnan K.

Autoimmune enteropathy is an extremely rare condition characterized by an abnormal intestinal immune response which typically manifests within the first 6 months of life as severe, intractable diarrhea that does not respond to dietary modification. Affected individuals frequently present with other signs of autoimmunity. The diagnosis is made based on a characteristic combination of clinical symptoms, laboratory studies, and histological features on small bowel biopsy. Autoimmune enteropathy is associated with a number of other conditions and syndromes, most notably immunodysregulation polyendocrinopathy enteropathy X-linked (IPEX) syndrome and autoimmune polyglandular syndrome type 1 (APS-1). Diagnosis and treatment is challenging, and further research is needed to better understand the pathogenesis, disease progression, and long-term outcomes of these conditions.

Alkofair B., Alruwaili A., Gai J., Harahsheh A.S.

AbstractPrevious reports have identified risk factors associated with development of post-Fontan protein-losing enteropathy. Less is known about the economic impact and resource utilisation required for post-Fontan protein-losing enteropathy in the current era. We conducted a single-centre retrospective study to assess the impact of post-Fontan protein-losing enteropathy on transplant-free survival. We also described resource utilisation and treatment variations among post-Fontan protein-losing enteropathy patients. Children who received care at our centre between 2009 and 2017 after the Fontan surgery were eligible. Initial admissions for the Fontan operative procedure were excluded. Demographics, hospital admissions, resource utilisation, medications and charges were reviewed. Patients were divided into two groups based on the presence of post-Fontan protein-losing enteropathy. Of the 343 patients screened, 147 met the eligibility criteria. Of these, 28 (19%) developed protein-losing enteropathy. After adjusting for follow-up duration, the protein-losing enteropathy group had higher number of encounters (2.15 ± 2.16 versus 1.47 ± 2.56, p 0.002), hospital length of stay (days) (25 ± 51.3 versus 11.4 ± 41.7, p < 0.0001) and total charges (2018US$) (388,489 ± 759,859 versus 202,725 ± 1,076,625, p < 0.0001). Encounters for patients with protein-losing enteropathy utilised more therapies. Among those with protein-losing enteropathy, use of digoxin was associated with slightly decreased odds for mortality and/or transplant (0.95, confidence interval 0.90–0.99, p 0.021). The 10-year transplant-free survival for patients with/without protein-losing enteropathy was 65.7/97.3% (p 0.002), respectively. Post-Fontan protein-losing enteropathy is associated with reduced 10-year transplant-free survival, higher resource utilisation, charges and medication use compared with the non-protein-losing enteropathy group. Practice variation among post-Fontan protein-losing-enteropathy patients is common. Further larger studies are needed to assess the impact of standardisation on the well-being of children with post-Fontan protein-losing enteropathy.

Lange L., Gernert S.

Bei der Diagnostik von Nahrungsmittelallergien sind viele Faktoren zu berücksichtigen. Zunächst ist es wichtig, die verschiedenen klinischen Bilder zu erkennen, die durch eine Nahrungsmittelallergie hervorgerufen werden können. Die Zuordnung zu einer Erkrankung entscheidet, welche weiteren Untersuchungen notwendig und sinnvoll sind. Bei Immunglobulin(Ig)E-vermittelten Allergien ist neben der Anamnese der Sensibilisierungsnachweis durch die Bestimmung des spezifischen IgE oder den Prick-Test ein wichtiger Pfeiler der Diagnostik. Entscheidend ist, dass der Nachweis einer Sensibilisierung gegen einen Extrakt aus einer Allergenquelle nur selten eine tatsächliche Allergie bedeutet. Ein Großteil der positiven Befunde hat keine klinische Relevanz. Die moderne komponentenbasierte Allergiediagnostik verbessert die Aussagekraft. Trotzdem ist die Diagnose oft nur mit einer Provokationstestung endgültig zu stellen. Dieser Beitrag weist auf mögliche Schwierigkeiten bei der Befundinterpretation hin.

Grabenhenrich L., Trendelenburg V., Bellach J., Yürek S., Reich A., Fiandor A., Rivero D., Sigurdardottir S., Clausen M., Papadopoulos N.G., Xepapadaki P., Sprikkelman A.B., Dontje B., Roberts G., Grimshaw K., et. al.

The prevalence of food allergy (FA) among European school children is poorly defined. Estimates have commonly been based on parent-reported symptoms. We aimed to estimate the frequency of FA and sensitization against food allergens in primary school children in eight European countries.A follow-up assessment at age 6-10 years of a multicentre European birth cohort based was undertaken using an online parental questionnaire, clinical visits including structured interviews and skin prick tests (SPT). Children with suspected FA were scheduled for double-blind, placebo-controlled oral food challenges (DBPCFC).A total of 6105 children participated in this school-age follow-up (57.8% of 10 563 recruited at birth). For 982 of 6069 children (16.2%), parents reported adverse reactions after food consumption in the online questionnaire. Of 2288 children with parental face-to-face interviews and/or skin prick testing, 238 (10.4%) were eligible for a DBPCFC. Sixty-three foods were challenge-tested in 46 children. Twenty food challenges were positive in 17 children, including seven to hazelnut and three to peanut. Another seventy-one children were estimated to suffer FA among those who were eligible but refused DBPCFC. This yielded prevalence estimates for FA in school age between 1.4% (88 related to all 6105 participants of this follow-up) and 3.8% (88 related to 2289 with completed eligibility assessment).In primary school children in eight European countries, the prevalence of FA was lower than expected even though parents of this cohort have become especially aware of allergic reactions to food. There was moderate variation between centres hampering valid regional comparisons.

Schleiger A., Ovroutski S., Peters B., Schubert S., Photiadis J., Berger F., Kramer P.

AbstractObjective:Protein-losing enteropathy is an infrequent but severe condition occurring after Fontan procedure. The multifactorial pathogenesis remains unclear and no single proposed treatment strategy has proven universally successful. Therefore, we sought to describe different treatment strategies and their effect on clinical outcome and mortality.Material and Methods:We performed a retrospective observational study. From the total cohort of 439 Fontan patients treated in our institution during the study period 1986–2019, 30 patients (6.8%) with protein-losing enteropathy were identified. Perioperative, clinical, echocardiographic, laboratory, and invasive haemodynamic findings and treatment details were analysed.Results:Median follow-up after disease onset was 13.1 years [interquartile range 10.6]. Twenty-five patients received surgical or interventional treatment for haemodynamic restrictions. Medical treatment, predominantly pulmonary vasodilator and/or systemic anti-inflammatory therapy with budesonide, was initiated in 28 patients. In 15 patients, a stable remission could be achieved by medical or surgical procedures (n = 3 each), by combined multimodal therapy (n = 8), or ultimately by cardiac transplantation (n = 1). Phrenic palsy, bradyarrhythmia, Fontan pathway stenosis, and absence of a fenestration were significantly associated with development of protein-losing enteropathy (p = 0.001–0.48). Ten patients (33.3%) died during follow-up; 5-year survival estimate was 96.1%. In unadjusted analysis, medical therapy with budesonide and pulmonary vasodilator therapy in combination was associated with improved survival.Conclusions:Protein-losing enteropathy is a serious condition limiting survival after the Fontan procedure. Comprehensive assessment and individual treatment strategies are mandatory to achieve best possible outcome. Nevertheless, relapse is frequent and long-term mortality substantial. Cardiac transplantation should be considered early as treatment option.

Bergholz R., Krebs T., Cremieux B., Georgi C., Fromm F., Boettcher M., Andreas T., Tiemann B., Wenke K., Reinshagen K., Hecher K.

This is the cumulative technical report on the operative procedures and limitations of fetoscopic bag insertion, intestinal bag placement, and bag fixation to the fetus in a series of pilot studies in an ovine model for prenatal treatment of gastroschisis. In 24 German blackhead sheep, a surgically created gastroschisis was managed by fetoscopic placement of the extruded intestines into a bag. The bag was then fastened onto the fetal abdominal wall. Different materials (sterile gloves, latex condoms, laparosopic retrieval bags) and different fixation techniques (laparoscopic staplers, interrupted and continuous sutures) have been examined. The fetuses were retrieved and evaluated at the end of gestation. Uterine bag insertion was successful in 15 of 24 (62.5%) and intestinal bag placement in 10 of 15 available fetuses (66.6%). The main factor limiting fetoscopic procedures was chorioamniotic separation (CAS). Sterilized condoms provided the most appropriate type of bags and the V-Loc™ running suture, the most expedient type of fixation, which was achieved in 9 of the 10 fetuses (complete = 2, partially = 7) by using a three port access (5 mm and 2 × 3 mm). All bags were encountered completely or partially dislocated from the fetus at the end of gestation. Fetoscopic intestinal bag placement and fixation in gastroschisis technically demanding. None of the evaluated techniques led to permanent anchorage of the bag to the fetus. The development of specially designed instruments, bags and fixation methods is required to optimize this approach.

Andrade W.S., Brizot M.L., Francisco R.P., Tannuri A.C., Syngelaki A., Akolekar R., Nicolaides K.H.

To investigate intra-abdominal bowel dilation (IABD) in the prediction of complex gastroschisis.This was a retrospective study of 174 singleton pregnancies with isolated fetal gastroschisis, resulting in live birth and with available ultrasound images from visits at both 20-22 and 30-32 weeks' gestation. IABD was measured as the greatest transverse diameter of the most dilated intra-abdominal bowel segment, by an operator blinded to postnatal outcome. The distribution of IABD measurements in those with complex and those with simple gastroschisis was determined and the best cut-off value to predict complex gastroschisis was selected using receiver-operating characteristics (ROC) curves. The area under the ROC curve (AUC), detection rate (DR), false-positive rate (FPR), positive predictive value (PPV) and negative predictive value (NPV) were determined.The study population included 39 (22.4%) cases of complex and 135 (77.6%) cases of simple gastroschisis. In the prediction of complex gastroschisis, the AUC at 20-22 weeks' gestation was 0.742 (95% CI, 0.628-0.856) and the respective value for 30-32 weeks was 0.820 (95% CI, 0.729-0.910). At the IABD cut-off of 7 mm at 20-22 weeks, DR, FPR, PPV and NPV for complex gastroschisis were 61.5%, 6.7%, 72.7% and 89.4%, respectively, and at IABD cut-off of 14 mm at 30-32 weeks, the respective values were 64.9%, 5.9%, 75.0% and 90.7%.Measurement of IABD at 20-22 or at 30-32 weeks' gestation is useful in the prediction of complex gastroschisis. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Muensterer O.J., Gianicolo E.

Inguinal hernia repair is one of the most frequent operations in pediatric surgery and is increasingly performed laparoscopically. The latter introduced new momentum in the debate on the necessity of contralateral exploration, as the rates of contralateral patent processus vaginales and metachronous inguinal hernias determine whether a routine closure would be overtreatment or useful prevention.We searched MEDLINE via PubMed, Web of Science and Scopus at the 6th of September 2017; reference lists and CrossRef were snowballed for reports citing identified studies. Eligibility criteria were age