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The aim was to explore factors associated with intestinal tissue levels of anti-TNF alpha (anti-TNF), anti-TNF antibodies, and cytokines in pediatric patients with Crohn Disease (CD). In a prospective exploratory study of CD patients undergoing ileocecal resection or colonoscopy between 6/2020 and 1/2023, we analysed tissue levels of anti-TNF, anti-TNF antibodies, and cytokines (TNF-α, IL-17, IL-1β, IFN-γ) from intestinal biopsies. Mixed-effects regression models, adjusted for potential confounders, were used. Data from 27 CD patients (18 females, 66.7%) were analysed. Fourteen (52%) received adalimumab (ADA) and thirteen received infliximab (IFX), with a median therapy duration of 17 (IQR 4.5-41.5) months. Higher levels of free anti-TNF were found in macroscopically inflamed tissue compared to non-inflamed tissue (β = 3.42, 95% CI 1.05-6.10). No significant association was found between serum and tissue anti-TNF levels (β= -0.06, 95% CI - 0.70-0.58). Patients treated longer with anti-TNF had increased IL-17 levels (β = 0.19, 95% CI 0.05-0.33), independent of disease duration and age. IFN-γ levels were linked with both follow-up duration and anti-TNF length. Our study shows significantly higher free drug levels in inflamed tissue. Long-term anti-TNF treatment has been linked to increased IL-17 levels, suggesting a possible impact on the cytokine response pathway. We did not observe a relationship between serum and tissue anti-TNF levels., Competing Interests: Declarations. Competing interests: The authors declare no competing interests., (© 2025. The Author(s).)

Introduction:  Postoperative constipation (PC) in patients with imperforate anus and perineal fistula (PF) has been reported in up to 60%. Histological studies of PF revealed innervation anomalies which seem to be one of the reasons for PC. Perioperative histologically controlled fistula resection (PHCFR) allows appropriate resection of PF and pull-down normoganglionic rectum at the time of posterior sagittal anorectoplasty (PSARP)., Materials and Methods:  A total of 665 patients with anorectal malformations underwent surgery between 1991 and 2021. Of these, 364 presented PF; 92 out of them (41 F) were studied. Patients with sacral and spinal cord anomalies, neurological disorders, and cut-back anoplasty were excluded. PSARP was done on all patients. Hematoxylin-eosin staining and NADH Tetrazolium-reductase histochemical method were used. Four and more ganglion cells in the myenteric plexus represented a sufficient length of the resection. The continence was scored according to the modified Krickenbeck scoring system. Final scores ranged from 1 to 7 points. Values are given as median., Results:  A total of 65 (70.7%) patients presented an aganglionic segment in PF, and 27 patients presented hypoganglionosis. The median length of the resected fistula was 25 mm (interquartile range [IQR]: 20-30). The median total continence score was 7 (IQR: 6-7). Post-op constipation was observed in 6/92 (6.5%) patients., Conclusion:  PHCFR diminished PC to 6.5% of patients., Competing Interests: None declared., (Thieme. All rights reserved.)

Purpose: Children diagnosed with Crohn's disease (CD) often undergo ileocecal resection (ICR) during childhood. Anastomotic recurrence is a frequent finding following this procedure. Data addressing the effect of the anastomosis type on disease recurrence are scarce in the pediatric population. The Kono-S anastomosis has shown promise in reducing endoscopic, clinical, and surgical recurrence rates in adults. We aimed to report our experience with Kono-S anastomosis in children, focusing on its feasibility and postoperative complications., Methods: We retrospectively analyzed pediatric CD patients who underwent ICR with Kono-S anastomosis between August 2022 and May 2023. Data on demographics, clinical characteristics, surgery, hospitalization, and follow-up including colonoscopy were collected. Complications were classified using the Clavien-Dindo classification., Results: Twelve patients (7 females, 58.3%) were included. Six (50%) of the patients had the B3 luminal form of the disease (according to Paris classification). Median surgery duration was 174 (interquartile range [IQR] 161-216) minutes. Anastomosis creation took a median of 62 (IQR, 54.5-71) minutes. Median hospitalization length was 6 (IQR 4-7) days. No short- or mid-term complications were observed. Median follow-up duration was 9.5 (IQR 6.8-12) months., Conclusion: According to our results, Kono-S anastomosis is safe and feasible in pediatric CD patients, with no observed postoperative complications. These findings support the potential benefit of using Kono-S anastomosis as a treatment approach in children with CD., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To investigate potential early risk factors for anastomotic stricture formation and assess the predictive role of post-operative esophagrams., Methods: A retrospective study of patients with esophageal atresia with distal fistula (EA/TEF) operated between 2011 and 2020. Fourteen predictive factors were tested for stricture development. Esophagrams were used to calculate early (SI1) and late (SI2) stricture index (SI = anastomosis diameter/upper pouch diameter)., Results: Of 185 patients operated for EA/TEF in the 10-year period, 169 patients met the inclusion criteria. Primary anastomosis was performed in 130 patients and delayed anastomosis in 39 patients. Stricture formed in 55 patients (33%) within 1 year from anastomosis. Four risk factors showed strong association with stricture formation in unadjusted models: long gap (p = 0.007), delayed anastomosis (p = 0.042), SI1 (p = 0.013) and SI2 (p < 0.001). A multivariate analysis showed SI1 as significantly predictive of stricture formation (p = 0.035). Cut-off values using a receiver operating characteristic (ROC) curve were 0.275 for SI1 and 0.390 for SI2. The area under the ROC curve demonstrated increasing predictiveness from SI1 (AUC 0.641) to SI2 (AUC 0.877)., Conclusions: This study identified an association between long gap and delayed anastomosis with stricture formation. Early and late stricture indices were predictive of stricture formation., (© 2023. The Author(s).)

Pectus excavatum is the most common chest wall deformity in the Czech Republic. This chest deformity is typically characterized by a wall depression with sternal rotation. If the excavation of the chest wall does not cause any physical or psychological problems, the patient does not need any specific treatment. However, if the deformity is painful, affects the function of the lungs, heart or results in psychological problems, we can propose an appropriate treatment for the specific age category of the patient. Up to 10 years, we choose a procedure that includes targeted exercises and rehabilitation; in the age group of 10-15 years, we can add to the exercises the vacuum bell therapy according to the patient's wishes and compliance; and in the age category of 16 years and above, the patient can be offered a surgical solution. The Nuss operation (so-called MIRPE - minimally invasive repair of pectus excavatum) is the gold standard in surgical treatment; during this surgery, a patient-shaped bar is inserted retrosternally into the patient's chest under thoracoscopic control and is left for 3 years. The aim of this article is to describe the most common modern methods used in the treatment of patients with pectus excavatum, supplemented by a historical overview.

Introduction: Pyeloplasty for congenital hydronephrosis (pyeloureteral junction obstruction, PUJO) can be performed as an open, laparoscopic or robotic procedure in children. The aim of this study was to analyze a pilot cohort of pediatric patients operated robotically., Methods: A retrospective analysis of the cohort of patients 3 to 18 years of age who underwent primary robotic pyeloplasty between December 2018 (which is when the robotic program was launched at the University Hospital Motol) and June 2021. Patients already operated on for PUJO in the past were excluded. The indication criteria, clinical symptoms, operation time, hospitalization length and complications were evaluated from the medical records., Results: During this period, we operated on 28 children aged 3 to 17 years (median 8); 2 patients were excluded. Of the 26 children analyzed, 6 (23%) were asymptomatic before surgery, 17 (65%) reported pain, 2 (8%) had acute pyelonephritis before the surgery, and 1 (4%) suffered from nephrogenic hypertension. The most common etiology of PUJO was an aberrant vessel in 22 (85%) patients. The time from incision to skin closure was 106 to 201 minutes (median 142 minutes). Patients were hospitalized for 3 to 5 days (median 4) after the surgery and followed up for 1 to 30 months (median 6). Twenty-four of the 26 patients showed regression of the pelvic dilatation according to the postoperative ultrasound scan and no symptoms of PUJO. Seven (27%) had postoperative complications requiring a procedure under general anesthesia (all Clavien-Dindo 3b); of these, 2 were re-operated., Conclusion: Robotic pyeloplasty is an alternative to laparoscopic and open surgery. It is a safe and successful (92%) method. We believe that the higher rate of complications (27%) in our cohort is due to the fact that this is a new technique and more experience is needed.

Introduction: Within the development of laparoscopy, the single incision laparoscopy method has been recently introduced in paediatric surgery. The aim of this study was to evaluate the initial experience with single incision laparoscopic appendectomy at the Department of Paediatric Surgery, 2nd Faculty of Medicine, Charles University and Motol University Hospital, Prague., Methods: A consecutive cohort of paediatric patients (18 years) undergoing laparoscopic single incision surgery for uncomplicated appendicitis between 4/30/2019 and 4/30/2021 was retrospectively evaluated. The following parameters were monitored: patients demographic characteristics, perioperative course, surgery duration, number and method of surgery conversions, length of postoperative hospitalization, postoperative complications classified according to Clavien-Dindo classification, occurrence of incisional hernias, and length of follow-up., Results: In all, 160 patients (72 (45%) females) with uncomplicated appendicitis were included in the study. The median age at the time of surgery was 12 (IQR: 9.914.9) years. The median duration of surgery was 50 (IQR: 3860) minutes. Only one conversion to multiport laparoscopy and no conversion to open surgery were reported. The median postoperative hospital stay was 2 (IQR: 23) days. We observed 5 intra-abdominal and 9 wound complications., Conclusion: Single incision laparoscopic appendectomy is a safe, effective and rapidly adoptable method for the treatment of uncomplicated appendicitis in children.

Introduction: The incidence of Crohns disease in the paediatric population has been increasing and requires surgical treatment in addition to conservative therapy. While surgical treatment used to be the last step after the failure of all conservative therapies, nowadays it is a standard part of complex treatment. Surgery can enter the treatment process at any stage of the disease and, with a proper indication, timing and preoperative optimization, it can induce immediate remission in patients. On the other hand, with inadequate or improper preoperative preparation and indication, surgical treatment can cause serious or even life-threatening complications. The spectrum of patients undergoing surgery is changing in the era of biological therapy. The aim of this review was to summarize the current knowledge of the impact of biological (anti-TNF alpha) therapy on the development of postoperative complications in children and adolescents operated for Crohns disease., Methods: We present a review based on literature available in MEDLINE-PubMed and Embase databases., Conclusion: According to current knowledge, no association was found between biological treatment in the preoperative period and the development of postoperative complications in paediatric patients. Surgical treatment of paediatric patients with Crohns disease is one of standard treatment modalities.

Introduction:  The number of patients with necrotizing pneumonia has increased in recent years. The aim of this study is to review the incidence, management, and outcome of pediatric necrotizing pneumonia requiring surgical therapy and to prove that lung resection results in favorable development of patients. We hypothesize that overall lung function in children after lung resection does not differ from that of the healthy population., Materials and Methods:  A retrospective tertiary referral center study with a prospective follow-up spirometric study of patients with necrotizing pneumonia managed between January 2010 and December 2019 was performed., Results:  The study cohort consisted of 1,295 patients admitted to the pediatric department for community-acquired pneumonia; 47 patients developed necrotizing pneumonia, 36 of whom underwent parenchymal lung resection. A 5-year rise in the occurrence of necrotizing pneumonia requiring resection was 77%, with a significant increase in the last 3 years ( p  < 0.05). The median age at the time of surgery was 32.5 (interquartile range [IQR]: 32.25) months. Streptococcus pneumoniae was the most prevalent pathogen (83%), although 53.3% of these patients were vaccinated against the agent. In 67% of patients, preresection procedures were performed: drainage of pneumothorax (17%), drainage of empyema (46%), drainage of empyema with use of alteplase (25%), and thoracoscopic decortication (12%). Surgical procedures included lobectomy (72.2%), wedge resection (13.9%), bilobectomy (8.3%), and pneumonectomy (5.6%). The postoperative complication was bronchopleural fistula in three patients. There were two (5.5%) postoperative deaths due to multiple organ failure. The follow-up spirometry was performed 43.3 (median, IQR 23.8-66.7) months after surgical intervention. Normal lung function was detected in 35 (64.8%) patients, restrictive pattern in 6 (11.1%) patients, obstructive pattern in 11 (20.4%) patients, and combined in 2 (3.7%) patients., Conclusion:  The number of patients with necrotizing pneumonia requiring resection has increased significantly in the last 3 years ( p  < 0.05). Aggressive surgical treatment results in significant clinical improvement in most cases and favorable lung function outcome. Long-term follow-up showed normal spirometry in 64.8% of cases., Competing Interests: None declared., (Thieme. All rights reserved.)

Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) is the most common congenital malformation of the upper digestive tract. This study represents the first genome-wide association study (GWAS) to identify risk loci for EA/TEF. We used a European case-control sample comprising 764 EA/TEF patients and 5,778 controls and observed genome-wide significant associations at three loci. On chromosome 10q21 within the gene CTNNA3 (p = 2.11 × 10 -8 ; odds ratio [OR] = 3.94; 95% confidence interval [CI], 3.10-5.00), on chromosome 16q24 next to the FOX gene cluster (p = 2.25 × 10 -10 ; OR = 1.47; 95% CI, 1.38-1.55) and on chromosome 17q12 next to the gene HNF1B (p = 3.35 × 10 -16 ; OR = 1.75; 95% CI, 1.64-1.87). We next carried out an esophageal/tracheal transcriptome profiling in rat embryos at four selected embryonic time points. Based on these data and on already published data, the implicated genes at all three GWAS loci are promising candidates for EA/TEF development. We also analyzed the genetic EA/TEF architecture beyond the single marker level, which revealed an estimated single-nucleotide polymorphism (SNP)-based heritability of around 37% ± 14% standard deviation. In addition, we examined the polygenicity of EA/TEF and found that EA/TEF is less polygenic than other complex genetic diseases. In conclusion, the results of our study contribute to a better understanding on the underlying genetic architecture of ET/TEF with the identification of three risk loci and candidate genes., Competing Interests: The co-author C.M.B. declares the following interests: Shire (grant recipient, Scientific Advisory Board member), Idorsia (consultant), Lundbeckfonden (grant recipient), Pearson (author, royalty recipient), and Equip Health Inc. (Clinical Advisory Board). All other co-authors declare no competing interests., (© 2022 The Authors.)

Background/purpose: The available literature on congenital isolated H-type tracheoesophageal fistula (TEF) is limited, and preferred approach varies among centers (cervicotomy, thoracotomy, thoracoscopy). We aimed to present one of the biggest case series of thoracoscopic approach for congenital isolated TEF and to assess the method's feasibility and outcomes., Methods: Retrospective review of thoracoscopic TEF repair experiences at 2 European university pediatric surgery centers., Results: 9 boys and 3 girls were involved in the study (age 5 days-4 years), 8 of them were newborns (mean birth weight 3013 g, mean gestational age 39 weeks). The most common presenting symptoms were desaturations on feeding in neonates and recurrent respiratory tract infections in older children. The diagnoses were established on contrast study and confirmed with rigid bronchoscopy. The fistulas were located at or below the thoracic inlet; the fistulas were 2 cm above the carina to half the height of the trachea. All patients underwent successful thoracoscopic TEF repair. There were no conversions. The postoperative course was uneventful in all but one who had rethoracoscopy for prolonged postoperative chylothorax. All patients had satisfactory vocal cord function. One patient required reoperation for fistula recurrence 8 months after primary surgery., Conclusion: Thoracoscopic approach for isolated H-type TEF seems to be possible as a procedure of choice with satisfactory results and all benefits of minimally invasive procedure., Level of Evidence: IV (case series)., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Over a twenty-year period, we performed 255 ERCP procedures in infants aged up to 1 year. ERCP was indicated in cholestatic infants with suspicion of biliary obstruction. The most common diagnosis was biliary atresia (48%), choledochal cysts (13%), and choledocholithiasis (4%). The procedure complication rate was 13.7%. Hyperamylasemia occurred in 12.9%. More severe complications were rare-0.8% of ERCP procedure. There were no cases of postprocedural pancreatitis or death. Our study has proved that ERCP is a safe and reliable method in this age group. Its high specificity and negative predictive value for extrahepatic biliary atresia can prevent unnecessary surgeries in patients with normal bile ducts or endoscopically treatable pathologies., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2021 Jan Stovicek et al.)

Introduction: Ileocolic intussusception is one of the most frequent causes of acute abdomen in infants and toddlers. The purpose of this study was to evaluate the treatment of ileocolic intussusception in the Czech Republic in 2019., Methods: Fourteen departments of paediatric surgery participated in this multicentre retrospective study. A SurveyMonkey Inc. questionnaire was used for anonymous data collection of patients with ileocolic intussusception., Results: In 2019, ileocolic intussusception was diagnosed in 162 patients; median age was 30 months (3 months to 9.5 years). Contrast enema was indicated in 133 patients, primary surgery in 14 patients and spontaneous reduction was found in 15 patients. All departments using contrast enema had a success rate of 89.5%; 12 departments approached the contrast enema procedure under general anaesthesia (including myorelaxation at 7 departments) and 2 departments under sedation. Contrast enema under general anaesthesia with/without myorelaxation was significantly more successful than contrast enema under sedation (108/113, 95.6% vs 11/20, 55%; p.

Introduction: Sinus pilonidalis (SP) is an acquired inflammatory disease, which is relatively common in the paediatric population. Surgery is indicated in symptomatic patients. In 2017, minimally invasive pilonidal sinus treatment (EPSiT) was adapted to the paediatric population., Aim: To evaluate the first experience with minimally invasive endoscopic treatment of SP (PEPSiT) in children and adolescents in the Czech Republic., Material and Methods: A retrospective review of all consecutive paediatric patients who underwent PEPSiT from November 2018 to February 2020. The monitored parameters were demographics, perioperative course of the disease, surgery, length of hospitalisation, postoperative complications, healing, disease recurrence, and follow-up., Results: Seventeen patients were enrolled in the study. The median age at surgery was 17.1 years (range: 12.5-18). The subjects comprised 76% males, and the median body mass index was 25.6 kg/m 2 (range: 17-30.3 kg/m 2 ). Thirteen patients underwent previous surgical treatment (76%) under local anaesthesia. The median duration of PEPSiT was 50 min (range: 30-85 min). The subjective evaluation of pain by patients on the VAS scale was 0 on the day of discharge. There were no postoperative complications up to the 30 th postoperative day. Two disease recurrences were successfully managed by re-PEPSiT. By the end of follow-up, 14/15 patients had healed. Two patients are still within 3 months of surgery, which is too soon to definitively evaluate possible recurrence of the disease., Conclusions: These preliminary results show that PEPSiT is a highly promising method. It is safe and well-tolerated by patients (short hospital stay, quick return to normal life, low pain and analgesic consumption). Two recurrences of disease were treated by re-PEPSiT., Competing Interests: The authors declare no conflict of interest., (Copyright: © 2020 Fundacja Videochirurgii.)

Purpose: To evaluate the impact of surgeon-assessed extent of primary tumor resection on local progression and survival in patients in the International Society of Pediatric Oncology Europe Neuroblastoma Group High-Risk Neuroblastoma 1 trial., Patients and Methods: Patients recruited between 2002 and 2015 with stage 4 disease > 1 year or stage 4/4S with MYCN amplification < 1 year who had completed induction without progression, achieved response criteria for high-dose therapy (HDT), and had no resection before induction were included. Data were collected on the extent of primary tumor excision, severe operative complications, and outcome., Results: A total of 1,531 patients were included (median observation time, 6.1 years). Surgeon-assessed extent of resection included complete macroscopic excision (CME) in 1,172 patients (77%) and incomplete macroscopic resection (IME) in 359 (23%). Surgical mortality was 7 (0.46%) of 1,531. Severe operative complications occurred in 142 patients (9.7%), and nephrectomy was performed in 124 (8.8%). Five-year event-free survival (EFS) ± SE (0.40 ± 0.01) and overall survival (OS; 0.45 ± 0.02) were significantly higher with CME compared with IME (5-year EFS, 0.33 ± 0.03; 5-year OS, 0.37 ± 0.03; P < .001 and P = .004). The cumulative incidence of local progression (CILP) was significantly lower after CME (0.17 ± 0.01) compared with IME (0.30 ± 0.02; P < .001). With immunotherapy, outcomes were still superior with CME versus IME (5-year EFS, 0.47 ± 0.02 v 0.39 ± 0.04; P = .038); CILP was 0.14 ± 0.01 after CME and 0.27 ± 0.03 after IME ( P < .002). A hazard ratio of 1.3 for EFS associated with IME compared with CME was observed before and after the introduction of immunotherapy ( P = .030 and P = .038)., Conclusion: In patients with stage 4 high-risk neuroblastoma who have responded to induction therapy, CME of the primary tumor is associated with improved survival and local control after HDT, local radiotherapy (21 Gy), and immunotherapy.

Purpose: Analysis of surgical management and survival of pediatric patients with gastric tumors treated at our institution., Methods: A retrospective study of patients with primary gastric tumors treated between 1993 and 2018 was conducted., Results: Eight patients, five girls and three boys, were diagnosed with gastric tumors at an average age of 10.4 years (1 day-15.4 years). Surgical management included Billroth type I procedure in five and tumor excision in three patients. Histology revealed gastrointestinal stromal tumor (GIST) in four patients and one of each of schwannoma, myofibroblastic tumor, hamartoma and teratoma. Microscopically clear margins were reported in six patients. Repeated local recurrence occurred in three patients (2 × GIST, 1 × myofibroblastic tumors) who consequently underwent three, four and six reoperations. One of these patients had liver metastases, which were managed with ligation of the hepatic arteries. This patient was also diagnosed with a lung hamartoma, which was treated with a lobectomy. Survival rate was 100% with a median follow-up of 8.6 years (7 months-25.5 years)., Conclusions: Gastric tumors are rare in children and represent a management challenge. Repeated recurrence of GISTs and myofibroblastic tumors remains frequent even after complete resection and may necessitate multiple surgeries, therefore patients require a lifelong follow-up.

Background: Appendicitis is one of the most common diagnoses in pediatric populations. Although new recommendations for the treatment of pediatric appendicitis were published, management varies among different institutions., Objectives: To determine current practices in 4 (n = 4) representative pediatric surgical departments in Central Europe., Material and Methods: One department from each of the 4 countries was surveyed using an online questionnaire. Questions focused on preoperative, operative and postoperative practices in 2018, particularly those related to antibiotic (ATB) therapy and laparoscopy., Results: A total of 519 appendectomies were performed, among which 413 (79.6%) were laparoscopic appendectomies (LAs), with a conversion rate of 5.1%. Appendectomy, as an elective procedure, was performed in 43 (8.3%) patients. One-quarter (129 patients) had complex appendicitis and 72.3% of these were operated laparoscopically. In 3 departments, ATB prophylaxis was administered, based on the decisions of the operating surgeon. One department used standard ATB prophylaxis (metronidazole). Whenever phlegmonous appendicitis was detected, ATB were administered therapeutically in 2 departments. Two other departments administered ATB based on surgeon decision. The choice of ATB was not standardized. If complex appendicitis was detected, all sites administered ATB therapeutically. The type of ATB treatment was standardized in complex cases in 2 departments. Thirty-four complications (6.6%) at surgical sites were recorded - 4.1% (16/390) after uncomplicated and 14% (18/129) after complex appendicitis. Thirty-two occurred after acute surgeries and 26 of these followed laparoscopic procedures. Postoperatively, intra-abdominal abscesses occurred in 3.5% of laparoscopic and in 2.9% of open appendectomy (OA) cases., Conclusions: This questionnaire study showed that treatment outcomes for appendicitis in children in Central Europe are comparable with data reported in the literature. Laparoscopic appendectomy is the predominant surgical method, but there is a little consensus for ATB treatment in the management of appendicitis at our 4 pediatric surgical departments.

Necrotizing enterocolitis (NEC) is a severe gastrointestinal disease affecting mainly preterm newborns. It is characterized by unexpected onset and rapid progression with specific diagnostic signs as pneumatosis intestinalis or gas in the portal vein appearing later in the course of the disease. Therefore, we analyzed diagnostic and prognostic potential of the markers of early NEC pathogenesis, such as excessive inflammatory response (serum amyloid A (SAA)) and gut epithelium damage (intestinal and liver fatty acid-binding protein (I-FABP and L-FABP, respectively) and trefoil factor-3 (TFF-3)). We used ELISA to analyze these biomarkers in the urine of patients with suspected NEC, either spontaneous or surgery-related, or in infants without gut surgery (controls). Next, we compared their levels with the type of the disease (NEC or sepsis) and its severity. Already at the time of NEC suspicion, infants who developed NEC had significantly higher levels of all tested biomarkers than controls and higher levels of I-FABP and L-FABP than those who will later develop sepsis. Infants who will develop surgery-related NEC had higher levels of I-FABP and L-FABP than those who will develop sepsis already during the first 6 hours after the abdominal surgery. I-FABP was able to discriminate between infants who will develop NEC or sepsis and the SAA was able to discriminate between medical and surgical NEC. Moreover, the combination of TFF-3 with I-FABP and SAA could predict pneumatosis intestinalis , and the combination of I-FABP, L-FABP, and SAA could predict gas in the portal vein or long-term hospitalization and low SAA predicts early full enteral feeding. Thus, these biomarkers may be useful not only in the early, noninvasive diagnostics but also in the subsequent NEC management., Competing Interests: The authors declare that they have no conflicts of interests., (Copyright © 2020 Stepan Coufal et al.)

Introduction: The miniinvasive approach is a trend in pediatric surgery nowadays. The new surgical technique called percutaneous internal ring suturing (PIRS) is a promising method bringing all the benefits of miniinvasive surgery., Methods: Prospective study of patients operated on using the PIRS technique from 01 January 2018 to 01 January 2020 at the Department of Pediatric Surgery, 2nd Faculty of Medicine, Charles University, University Hospital Motol., Results: 73 patients (25 boys and 48 girls) were operated on using PIRS. The median age was 68 months. 90 % of operations were performed by the same team of surgeons. During the procedure there were found 53 right-sided and 38 left-sided inguinal hernias. In 18 cases the hernia was bilateral, but only in 13 cases was this diagnosis made before the operation. A non-absorbable stitch was used in 57 cases to close the internal ring of the inguinal canal, and a non-absorbable monofilament in 16. The median operating time was 34 minutes. There were 3 recurrences (3.3 %) in our study.  Conclusion: In our initial study, the PIRS technique proved to be a safe alternative method to the open inguinal hernia surgery. This method provides the benefit of allowing to revise the contralateral inguinal canal as a prevention of a metachronous inguinal hernia. The cosmetic results were excellent.

Introduction:  The incidence of Crohn's disease (CD) within the pediatric population is increasing worldwide. Despite a growing number of these patients receiving anti-tumor necrosis factor α therapy (anti-TNF-α), one-third of them still require surgery. There is limited data as to whether anti-TNF-α influences postoperative complications. We evaluated postoperative complications in patients who were or were not exposed to anti-TNF-α therapy in our institutional cohort., Materials and Methods:  A retrospective review of CD patients who underwent abdominal surgery between September 2013 and September 2018 was performed. The patients were divided into two groups based on whether they were treated with anti-TNF-α within 90 days before surgery. Thirty-day postoperative complications were assessed using Clavien-Dindo classification (D-C); this examination included surgical site infections (SSIs), stoma complications, intra-abdominal septic complications, non-SSIs, bleeding, ileus, readmission rate, and return to the operating room. Mann-Whitney U -test, Fisher's exact test, and multivariate logistic regression analyses were used for statistical analysis., Results:  Sixty-five patients (41 males) with a median age of 16 years (range: 7-19) at the time of operation were identified. The most common surgery was ileocecal resection in 49 (75%) patients. Forty-three (66.2%) patients were treated with anti-TNF-α preoperatively. Seven patients (11%) experienced postoperative complications. There was no statistically significant difference in postoperative complication in patients who did or did not receive anti-TNF-α before surgery (D-C minor 2.3% vs. 4.6%, p  = 1; D-C major 7% vs. 9.1%, p  = 1)., Conclusion:  The use of anti-TNF-α in pediatric CD patients within the 90 days prior to their abdominal surgery was not associated with an increased risk of 30-day postoperative complications., Competing Interests: None declared., (Georg Thieme Verlag KG Stuttgart · New York.)

Ingestion of a foreign body is a frequent diagnosis in the pediatric population. In a small percentage of cases, foreign bodies themselves are strong magnets, and swallowing of multiple magnetic bodies can lead to serious complications in the gastrointestinal tract. Two consecutive case reports of patients who swallowed two magnetic beads are presented. In both cases, the abdominal radiograph described two magnets in contact, one in the area of the left hypochondrium and one in the right hypogastrium. Attempts of endoscopic localization and removal were unsuccessful. Due to the failure of magnet progression, laparoscopic revision of the abdominal cavity was indicated in both patients on the 25th and 4th day after swallowing. Using the magnetic forces between the magnets and the laparoscopic instruments, the foreign bodies were localized in the appendix of the first patient and in the cecum of the other one. The magnets were extracted together with the removal of the appendix in both patients. This is one of the first articles describing the successful extraction of foreign magnetic bodies from the gastrointestinal tract via laparoscopic appendectomy., Competing Interests: Conflict of Interest None declared.

Introduction: Wandering spleen is a rare cause of recurrent abdominal pain in children that is difficult to diagnose. Splenopexy should be preferred to splenectomy., Case Report: The patient, a 4 years old girl on growth hormone therapy, was referred to the Department of Paediatric Surgery for an assessment of episodes of abdominal colic and frequent vomiting. Sonography did not indicate any pathology, gastrointestinal contrast study showed an altered shape of the gastric outlet, and upper endoscopy diagnosed a prepyloric membrane. Open surgery was indicated based on these examinations, revealing a large wandering spleen and dilated stomach and small bowel loops oppressed by the long splenic suspensory ligaments. Pyloroplasty was performed with the antral membrane excision and shortening of the long gastrosplenic ligament and placement of the spleen into the left hypochondrium. No complications occurred in the postoperative period. Four months later the patient was hospitalised again for abdominal colic pain. Surgical revision of the abdomen revealed the wandering spleen in the mesogastrium. Even though the spleen was the cause of the second surgery, we decided to preserve the spleen. Partial splenectomy was done, fixing the large spleen into an extraperitoneal pocket in the left hypochondrium. The postoperative course was uneventful. Blood flow to the spleen is regularly monitored by Doppler ultrasound. Vaccination was indicated at the office of haematology according to the scheme for splenectomy patients; however, prophylactic antibiotic therapy was not needed given the good function of the preserved part of the spleen., Conclusion: Extraperitoneal fixation of the spleen seems to be a good choice for patients with a wandering spleen, particularly for children and adolescents.

Introduction:  Standard coagulation tests (activated partial thromboplastin time [aPTT] and prothrombin time [PT]) are used for the assessment of coagulation profile in critically ill pediatric patients undergoing invasive interventions such as insertion of central venous catheter, tonsillectomy, laparotomy, etc. However, these tests do not reflect the profile of whole blood coagulation. Rotational thromboelastometry (ROTEM) as a point of care (POC) viscoelastic test may serve as an alternative method. Due to its ability to assess coagulation profile of the whole blood, it might yield normal results despite prolonged aPTT/PT results. The aim of this study was to find out if there was any severe bleeding during or after invasive procedures if ROTEM test was normal despite prolonged values of aPTT/PT in pediatric patients., Materials and Methods:  We retrospectively analyzed data for the years 2015 to 2017 for pediatric patients with prolonged values of aPTT or PT and normal ROTEM tests-internal thromboelastometry (INTEM) (assessing internal pathway of coagulation) and external thromboelastometry (EXTEM) (assessing external pathway of coagulation)-and we looked for severe bleeding during or after invasive procedures., Results:  In 26 pediatric patients (children from 2 months to 17 years old), we found that INTEM and EXTEM tests showed normal coagulation despite prolonged values of aPTT ratio with a median of 1.47 (minimum 1.04 and maximum 2.05), international normalized ratio with a median of 1.4 (minimum 0.99 and maximum 2.10), and PT ratio with a median of 1.30 (minimum 0.89 and maximum 2.11). In these patients, no severe bleeding was observed during interventions or postoperatively., Conclusion:  Our data support using thromboelastometry method as an alternative coagulation test for the assessment of coagulation profile in pediatric patients undergoing surgical or other invasive procedures, especially using it as a POC test. All invasive procedures in our study were performed without severe bleeding despite prolonged values of PT/aPTT with normal ROTEM results. It seems that ROTEM assessment of coagulation may lead to decreased administration of fresh frozen plasma and shorten time of patient preparation for intervention., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Aim of Study: Duodenum-preserving resection of the pancreatic head (DPRPH) with Roux-en-Y pancreatojejunostomy is a procedure used to remove focal pathological lesions of the pancreatic head. Although predominantly used in adult patients, it is both safe and effective in children. The aim of this study was to review our experience with this procedure, with focus on its indications, complications and long-term outcomes., Methods: A retrospective analysis of pediatric patients who underwent DPRPH between 1994 and 2015 was performed. Patient files were reviewed for demographic, diagnostic, operative and histological details, postoperative complications. Patients were contacted telephonically and sent questionnaires to determine long-term outcomes., Results: The study cohort consists of 21 patients, 14 girls and 7 boys, with an average age of 11.72 years (range 3 months to 18.6 years), who underwent DPRPH with end-to-end anastomosis of the jejunum to the pancreatic body (Roux-en-Y anastomosis). In four cases the head and also part of the body of the pancreas was resected. In the remaining 17 cases, only the head of the pancreas was resected. Indications for DPRPH were solid pseudopapillary tumor of the pancreas (n = 10), trauma (n = 8), pancreas divisum (n = 1), focal congenital hyperinsulinism (n = 1) and pancreatic cyst (n = 1). The length of follow-up ranged from 1 to 22 years (average 9.66). One patient developed a biliary fistula, which closed spontaneously within 2 weeks after stent insertion. A recurrence of abdominal pain was reported in two patients, occurring at 7 months after the operation in one patient and at 1 year in the other. Pancreatic endocrine insufficiency did not occur in any of the 21 patients. Seven patients currently require a low fat diet, five of which need pancreatic enzyme supplementation. An additional two patients need enzyme supplementation without dietary restriction., Conclusion: DPRPH is a safe and effective procedure for the treatment of large focal pathological lesions of the pancreatic head in children. As a less invasive procedure than pancreatoduodenectomy, it is more appropriate for the developing child.

Background: Endoscopic retrograde cholangiopancreatography (ERCP) is seldom used in children, and published series have limited numbers of pediatric patients. The aim of this retrospective observational study was to assess the efficacy and safety of pediatric ERCP in a large group of children., Methods: Data were evaluated from 626 children with biliopancreatic disorders admitted to University Hospital Motol, Prague, between January 1999 and January 2018. Clinical data were obtained by retrospective evaluation of our database of pediatric ERCP procedures and from clinical records., Results: We performed 856 ERCPs on 626 pediatric patients; of these procedures, 59% were therapeutic and 41% were diagnostic. We achieved 96% technical success. Indications for ERCP and pathological findings differed in different age groups. The main role of ERCP was in excluding biliary atresia in those aged less than one year. In children aged 1 to 6 years, the most frequent diagnoses were choledochal cyst followed by choledocholithiasis. In children aged 7 to 12 years and 13 to 19 years, the most frequent diagnoses were choledocholithiasis followed by pancreatic pathology. The overall complication rate found in this study was similar to rates observed in adult populations., Conclusions: Our study shows the efficacy and safety of diagnostic and therapeutic ERCP in a large series of infants and children with technical success and complication rates comparable to those in adults. Our data show that ERCP had different roles in different age groups of children., Competing Interests: The authors have declared that no competing interests exist.

Background/purpose: We analyzed the capacity of urinary Intestinal fatty acid-binding protein (I-FABP) to quantify the degree of mucosal injury in neonates with gastroschisis (GS) and to predict the speed of their clinical recovery after surgery., Methods: In this prospective study, we collected urine during the first 48h after surgery from neonates operated between 2012 and 2015 for GS. Neonates with surgery that did not include gut mucosa served as controls for simple GS and neonates with surgery for intestinal atresia served as control for complex GS patients. The I-FABP levels were analyzed by ELISA., Results: Urinary I-FABP after the surgery is significantly higher in GS newborns than in control group; I-FABP in complex GS is higher than in simple GS. I-FABP can predict subsequent operation for ileus in patients with complex GS. Both ways of abdominal wall closure (i.e. primary closure and stepwise reconstruction) led to similar levels of I-FABP. None of the static I-FABP values was useful for the outcome prediction. The steep decrease in I-FABP after the surgery is associated with faster recovery, but it cannot predict early start of minimal enteral feeding, full enteral feeding or length of hospitalization., Conclusion: Urinary I-FABP reflects the mucosal damage in gastroschisis but it has only a limited predictive value for patients' outcome., Competing Interests: The authors have declared that no competing interests exist.

Introduction:  A retrospective study was performed to evaluate the clinical features, diagnostic methods, and treatment alternatives in children with pancreas divisum (PD)., Materials and Methods:  Patients who underwent treatment for PD between 1999 and 2014 at our department were evaluated for sex, age, presenting symptoms, physical examination findings, biochemical markers, diagnostic methods, treatment modalities, and results of treatment during follow-up., Results:  Seven patients who underwent treatment of symptomatic PD were included in the study. The median for follow-up period was 8 years (from 26 months to 16 years). Male-to-female ratio was 4:3 and the median age at presentation was 11 years (2-14 years). Presenting symptoms were recurrent episodic epigastric pain. Pancreatitis was documented by elevated amylase or lipase levels. Endoscopic retrograde cholangiopancreatography (ERCP) was the method of diagnosis of PD in all patients. Five patients had complete PD and two had incomplete variants. Three patients improved after ERCP papillotomy. In three patients, papillotomy was unsuccessful but they have only mild episodes of pancreatitis. One patient presented at the age of 4 years with recurrent pancreatitis. She was treated surgically by duodenum-preserving resection of the pancreatic head (DPRPH) because of severe recurrent pancreatitis occurring even after ERCP papillotomy. The patient is 26 months after operation without any reported problems., Conclusion:  Patients with symptomatic PD are indicated for ERCP papillotomy attempt. If there is not improvement after ERCP, then recurrent bouts of severe pancreatitis are considered as an indication for surgical procedure. DPRPH is a safe and feasible surgical alternative., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Purpose: To compare anthropometric data (body mass index [BMI]) in patients without lithiasis to patients with symptomatic simple cholelithiasis or choledocholithiasis., Methods: We retrospectively reviewed data from 147 patients undergoing laparoscopic cholecystectomy between 2001-2015. Complete growth data from 98 patients was compared with anthropometric data from the population of the Czech Republic and a control group (BMI of 100 consecutive patients without biliary stones in abdominal ultrasound who were admitted to a surgical department for suspected appendicitis)., Results: The BMI of 75 children with simple cholelithiasis and 23 with choledocholithiasis was compared to the standard Czech pediatric population and to the control group. The median age (simple cholelithiasis and choledocholithiasis) was 16 years, and 35 patients (24%) had a family history of gallstones. Types of lithiasis included multiple (n = 120), solitary (n = 11), and sludge (n = 10). Five cases had polyps and one had gallbladder dysplasia. Patients with simple cholelithiasis had significantly higher BMI compared to the control group without cholelithiasis (p<0.0001) and the standard Czech population (p = 0.03). Patients with choledocholithiasis had a mean BMI significantly higher than that of the general population (p = 0.001) and the control group (p = 0.0001). Patients with choledocholithiasis had significantly higher BMI than those with simple cholelithiasis (p = 0.03)., Conclusion: Patients with cholelithiasis had significantly higher BMI than the general population, and patients with choledocholithiasis had significantly higher BMI than patients with simple lithiasis. Elevated BMI is a risk factor for developing choledocholithiasis. ERCP and early laparoscopic cholecystectomy in patients with choledocholithiasis offer equivalent outcomes in patients with simple cholelithiasis.

Introduction: Surgical diseases of the pancreas in children are not common and may be associated with significant morbidity and potential mortality. A multidisciplinary approach is essential for correct diagnosis, surgical strategy and postoperative as well as follow-up care., Method: Retrospective analysis of patients operated on due to a pathological lesion of the pancreas focused on diagnostics, operating procedures, postoperative complications, and long-term results. Between 1991 and 2016, eighty-nine children were treated in our department for a pathologic lesion of the pancreas. 39 of them were boys and 50 were girls., Results: Mean age of the patients was 9.3 years (1 month-18.4 years). Patients were followed from the operation to the age of 19, after which they were referred for follow-up to adult specialists. The indications for surgery were trauma in 34 children, solid pseudopapillary tumor in 23 children, biopsy in 10, hyperinsulinism in 8, chronic pancreatitis in 4, pancreatic cyst in 3, insulinoma in 3, carcinoma in 2, and serous cystadenoma and pancreas divisum in one patient. The most frequent procedures performed on the pancreas were distal pancreatectomy in 35 cases, the duodenum-preserving pancreatic head resection in 23 cases, pseudocystogastroanastomosis in 11 cases, 9095% pancreatic resection in 5 cases, Whipple operation in two cases, Puestow procedure in one case, tumor enucleation in one case, and tumor biopsy for cancer in one case. In 5 patients after major pancreatic injury, ERCP and papillotomy with insertion of a stent into the pancreatic duct was performed. 3 patients died, one after a polytrauma with severe pancreatic injury and two patients with pancreatic cancer., Conclusion: Pancreatic surgery in children is not a common operation, and individual as well as institutional experience remains limited. After more than 20 years of experience with pancreatic surgery, we believe that close cooperation with surgeons, pediatric gastroenterologists, radiologists, anesthesiologists, intensivist, pathologists and ERCP specialists is necessary for successful diagnosis and treatment of pancreatic disease in children.Key words: pancreas pancreatic surgery in children duodenum preserving head resection of the pancreas.

Introduction: Gastroschisis is the most common malformation among abdominal wall defects with the incidence of 1:45000 live birth neonates. Postoperative care for these patients is very difficult due to the risk of abdominal compartment syndrome. The identification of markers that correlate with bowel damage in gastroschisis may prevent this serious postoperative complication., Methods: Review of the literature., Conclusion: Prenatal ultrasound prediction of bowel damage followed by an appropriate surgical strategy is a possibility of optimizing care and outcomes in gastroschisis patients. The dynamics of I-FABP is a promising biomarker for the elimination of abdominal compartment syndrome during postoperative care.Key words: gastroschisis preformed silo intraabdominal dilatation I-FABP.

Background and Study Aims: The aim of this study was to assess the significance and safety of endoscopic retrograde cholangiopancreatography (ERCP) in diagnosing and treating bile duct injuries in children., Patients and Methods: Fourteen pediatric patients, with traumatic or postoperative bile duct injury, in which ERCP was performed, were retrospectively evaluated., Results: We performed 46 ERCP and 12 endoscopic papillotomies in children with suspected bile duct injuries. A bile stent was primarily inserted in 13 patients and there were 20 replacements. Endoscopic treatment of bile leakage without need for bile duct sutures or reconstruction was successful in 85.7%. Post ERCP complications included cholangitis and recurrent bleeding, which occurred only in two patients each., Conclusions: ERCP and endoscopic bile stent insertion is a highly effective, minimally-invasive treatment for bile duct injury and should be included as part of the therapeutic procedures in pediatric patients with suspected bile duct injury.

Aim: To evaluate long-term quality of life and somatic growth of patients with gastroschisis and compare them with the general population., Methods: We performed a questionnaire survey of the quality of life of our patients treated between 2004-2012., Results: A questionnaire was sent to our 56 patients with gastroschisis, 38 mothers of patients (68%) responded to the questionnaire. 33 of 38 mothers claim that the quality of life of their child is very good, 4 of them responded that it is good. 1 mother confessed that the quality of life was very poor. Anthropometric data show comparable results with the standard population except for patients of 1 year of age who still have lower weight (P<0.001) and body height in the 5th percentile and patients of 3 years of age who are also significantly thinner. 13% of patients in our study group have gastrointestinal problems. 9 patients (24%) attend follow-up at the neurological center (Attention Deficit Hyperactivity Disorder n=6, mental retardation n=1, dysarthria n=2), however, overall intellectual abilities are within normal range. 7 patients underwent surgery for umbilical (n=3) or inguinal hernia (n=4), 2 patients were operated on for undescended testicles, 3 patients were operated on for an adhesive ileus. 92% of mothers are very satisfied with the cosmetic result of the scar., Conclusion: The study has shown that the majority of patients after operation of gastroschisis have a very good quality of life without limitation in comparison with the general population. The presented anthropometric data confirm that the development of patients with gastroschisis is favourable.

Objectives: Respiratory morbidity in congenital diaphragmatic hernia (CDH) is associated with high mortality and adverse outcome. Accurate prenatal diagnosis is essential for prognosis and potential treatment in utero. The aim was to evaluate the prenatal ultrasound findings in assessing the respiratory prognosis in fetuses with isolated left-sided CDH., Methods: We retrospectively analyzed the medical records of 59 prenatally diagnosed left-sided CDH cases managed at a tertiary perinatal center., Results: Survival rate in the study group was 73% (43/59). We found no statistically significant relationship between survival and the presence of polyhydramnios, gestational age at diagnosis, lung-to-head ratio (LHR) and observed/expected LHR (O/E LHR) values, gestational age at birth and birth weight. Intrathoracic liver herniation was a statistically significant parameter adversely affecting survival (37.2% in survivors, 68.8% in non-survivors, p = 0.031) and logistic regression confirmed this relationship. The presence of pneumothorax and severe pulmonary hypertension were significantly associated with mortality (82% non-survivors versus 15% in survivors, p = 0.0001)., Conclusion: Intrathoracic liver herniation seems to be a reliable parameter in the prediction of survival and neonatal respiratory morbidity in fetuses with isolated left-sided CDH. In contrast, we found no significant correlation between perinatal outcome and LHR, O/E LHR values, birth weight and gestational age.

Chest injuries in children are part of polytrauma resulting from high-energy violence, most often caused by traffic accidents. Blunt chest injuries (95%) are significantly more frequent than penetrating injuries (5%). Lung contusion, rib fracture, pneumothorax or haemothorax, are the more common injuries, but tracheobronchial rupture, cardiac or diaphragmatic injuries may also occur. The anterior X-ray image remains the basic examination method for isolated chest injuries. CT trauma scan with a contrast medium is done in polytraumatized children. Blunt injuries of intra-thoracic organs in haemodynamically stable children are treated mostly conservatively (85%) under full monitoring at the ICU. Surgical treatment is necessary in a minority of patients. Mortality and morbidity of patients with chest injury depend on the actual combination of multiple body systems injury. The severity of total injury can be predicted using objective scoring systems (Abbreviated Injury Scale=AIS; Injury Severity Score=ISS). Overall mortality ranges from 6 to 20%. Mortality is high but this is mainly due to associated head injuries.Key words: multiple trauma thoracic trauma - paediatric lung contusion Injury Severity Score=ISS.

Introduction: Biliary cysts with an abnormal pancreatobiliary junction are one of the most common pancreatobiliary malformations. The main symptom is cholestasis; endoscopic retrograde cholangiopancreatography (ERCP) plays a key role in diagnosis., Aim: Retrospective evaluation of ERCP performed to diagnose abnormalities of the pancreatobiliary junction., Material and Methods: We retrospectively evaluated ERCP performed to diagnose abnormalities of the pancreatobiliary junction, mainly choledochal cysts, in 112 children between 1990 and 2011., Results: We performed 112 examinations of 50 children with abnormal pancreatobiliary junction and choledochal cysts (15 males and 35 females, average age: 5 years, range: 1 month - 15 years). Cysts were associated with a common channel in 37 (74%) cases, were not associated with a common channel in 9 (18%) cases, and in 3 (6%) cases the common channel lacked cysts. We performed endoscopic papilla sphincterotomy on 33 (66%) patients; endoscopic drainage was performed 62 times, including 17 patients without papilla sphincterotomy. In 15 (30%) cases, we only performed sphincterotomy. Extraction of lithiasis was done in 2 (4%) cases. Both ERCP and magnetic resonance cholangiopancreatography (MRCP) were performed on 13 patients. There was concordance of the choledochal cyst and of the type of cyst in both methods; however, common channels could not be observed by MRCP. There was 1 serious complication (perforation after sphincterotomy) and 11 (9%) mild adverse events., Conclusions: When performed at an expert centre, ECRP is a safe and reliable procedure for children with choledochal cysts and/or an abnormal pancreatobiliary junction. Endoscopic retrograde cholangiopancreatography remains a reasonable alternative because MRCP has a limited ability to diagnose the precise anomalies of the pancreatobiliary junction and has no therapeutic capabilities., Competing Interests: The authors declare no conflict of interest.

Background  Delayed surgery after stabilization of infants with congenital diaphragmatic hernia (CDH) is an accepted strategy. However, the evidence favoring delayed versus immediate surgical repair is limited. We present an extremely rare case of a very low-birth-weight infant with prenatally diagnosed left-sided CDH and unexpected transmural bowel perforations developing within the postnatal stabilization period. Case Report  A neonate born at 31st week of gestation with a birth weight of 1,470 g with antenatally diagnosed left-sided CDH presented with bowel dilation leading to transmural bowel perforations on the 2nd day of life. Meconium pleuroperitonitis resulted in severe systemic inflammatory response syndrome, pulmonary hypertension, multiple organ failure, and death. Conclusion  In neonates with CDH deteriorating under standard postnatal management, intestinal perforation, and early surgical intervention should be considered.

Selective lung intubation is a necessary prerequisite for the completion of most interventions comprising thoracotomy and thoracoscopy. In paediatric care, our site uses Univent tubes for children up to the age of three years and double-lumen tubes (DLT) for children from 6-8 years of age. In younger children, we usually use regular endotracheal intubation, with the lung being held in the hemithorax position being operated on using a surgical retractor. The article presents the analysis of 860 thoracic surgeries, of which 491 comprised selective intubation (Univent 57 cases, DLT 434 cases). The use of the aforementioned devices is connected with certain complications. Univent tube can be connected with intraoperative dislocation of the obturating balloon (29.8%) and balloon perforation (5.2%). DLT insertion may be connected with failure of tube fitting. In 84 cases we had to repeat DLT insertion (20.6%). In 8 cases we were not able to insert DLT at all (1.8%). Standard use of selective intubation methods in paediatric patients from two years of age improved the conditions for surgical interventions (Tab. 2, Fig. 2, Ref. 19).

Background and Study Aims: Trauma is one of the most common causes of morbidity and mortality in the pediatric population. The diagnosis of pancreatic injury is based on clinical presentation, laboratory and imaging findings, and endoscopic methods. CT scanning is considered the gold standard for diagnosing pancreatic trauma in children., Patients and Methods: This retrospective study evaluates data from 25 pediatric patients admitted to the University Hospital Motol, Prague, with blunt pancreatic trauma between January 1999 and June 2013., Results: The exact grade of injury was determined by CT scans in 11 patients (47.8%). All 25 children underwent endoscopic retrograde cholangiopancreatography (ERCP). Distal pancreatic duct injury (grade III) was found in 13 patients (52%). Proximal pancreatic duct injury (grade IV) was found in four patients (16 %). Major contusion without duct injury (grade IIB) was found in six patients (24%). One patient experienced duodeno-gastric abruption not diagnosed on the CT scan. The diagnosis was made endoscopically during ERCP. Grade IIB pancreatic injury was found in this patient. One patient (4%) with pancreatic pseudocyst had a major contusion of pancreas without duct injury (grade IIA). Four patients (16%) with grade IIB, III and IV pancreatic injury were treated exclusively and nonoperatively with a pancreatic stent insertion and somatostatine. Two patients (8%) with a grade IIB injury were treated conservatively only with somatostatine without drainage. Eighteen (72 %) children underwent surgical intervention within 24 h after ERCP., Conclusion: ERCP is helpful when there is suspicion of pancreatic duct injury in order to exclude ductal leakage and the possibility of therapeutic intervention. ERCP can speed up diagnosis of higher grade of pancreatic injuries.

Necrotizing enterocolitis (NEC) is severe disease of gastrointestinal tract, yet its early symptoms are nonspecific, easily interchangeable with sepsis. Therefore, reliable biomarkers for early diagnostics are needed in clinical practice. Here, we analyzed if markers of gut mucosa damage, caspase cleaved cytokeratin 18 (ccCK18) and intestinal fatty acid-binding protein (I-FABP), could be used for differential diagnostics of NEC at early stage of disease. We collected paired serum (at enrollment and week later) and urine (collected for two days in 6 h intervals) samples from 42 patients with suspected NEC. These patients were later divided into NEC (n = 24), including 13 after gastrointestinal surgery, and sepsis (n = 18) groups using standard criteria. Healthy infants (n = 12), without any previous gut surgery, served as controls. Both biomarkers were measured by a commercial ELISA assay. There were no statistically significant differences in serum ccCK18 between NEC and sepsis but NEC patients had significantly higher levels of serum and urinary I-FABP than either sepsis patients or healthy infants. Urinary I-FABP has high sensitivity (81%) and specificity (100%) and can even distinguish NEC from sepsis in patients after surgery. Urinary I-FABP can be used to distinguish NEC from neonatal sepsis, including postoperative one, better than abdominal X-ray.

Alagille syndrome may mimic biliary atresia in early infancy. Since mutations in JAG1 typical for Alagille syndrome type 1 have also been found in biliary atresia, we aimed to identify JAG1 mutations in newborns with proven biliary atresia (n = 72). Five biliary atresia patients with cholestasis, one additional characteristic feature of Alagille syndrome and ambiguous liver histology were single heterozygotes for nonsense or frameshift mutations in JAG1. No mutations were found in the remaining 67 patients. All "biliary atresia" carriers of JAG1 null mutations developed typical Alagille syndrome at the age of three years. Our data do not support association of biliary atresia with JAG1 mutations, at least in Czech patients. Rapid testing for JAG1 mutations could prevent misdiagnosis of Alagille syndrome in early infancy and improve their outcome.

Aims: The aim of the study was to analyze lung growth and abnormality of infant pulmonary function tests (IPFT) in congenital diaphragmatic hernia (CDH) survivors younger than three years of age with respect to unfavorable prognostic factors., Methods: Thirty high-risk CDH survivors at the age of 1.32±0.54 years, body weight 9.76±1.25 kg were examined using IPFT: tidal breathing analysis, baby resistance/compliance, whole baby body plethysmography and rapid thoraco-abdominal compression. Gore-Tex patch was used in 13% of patients (GORE group). Pulmonary hypertension was diagnosed and managed in 13% (iNO group). Standard protocols and appropriate reference values were used and obtained data were statistically analysed., Results: High incidence of peripheral airway obstruction (70%), increased value of functional residual capacity (FRCp) 191.3±24.5 mL (126.5±36.9 % predicted; P < 0.0005), increased value of effective airway resistance (Reff) 1.71±0.93 kPa.L(-1).s (144.4±80.1 % predicted; P < 0.01) and decreased specific compliance of the respiratory system (Crs/kg) 14.1±2.3 mL.kPa.kg(-1) (i.e., 76.1±20.1 % predicted, P < 0.0005) was noted in infants with CDH in comparison with reference values. Increased value of FRCp was found in GORE group (165.7±51.9 versus 120.4±31.2, P < 0.02) and in iNO group (183.1±52.6 versus 117.8±25.7 mL; P < 0.0005)., Conclusion: A high incidence of peripheral airway obstruction, an increased value of FRCp and decreased specific compliance of the respiratory system was noted in infants with CDH. Unfavorable prognostic factors (Gore-Tex patch, pulmonary hypertension) correlate with more severe alteration of pulmonary function in infants.

Purpose: The aim of the study was to identify which prenatal ultrasonographic findings in fetuses with gastroschisis correlate with complicated postnatal outcome., Methods: Ultrasound findings at the 30th week of pregnancy and medical reports were statistically analyzed to identify independent prenatal ultrasonographic predictors of postnatal outcome., Results: Completed prenatal data were gathered from 64 pregnancies. Prenatal intra-abdominal bowel dilatation (cutoff 10 mm) correlated with the presence of atresia (p < 0.01), longer administration of parenteral nutrition, extended hospital stay (median 53 vs. 21 days; 68 vs. 36 days, both p < 0.05), and greater number of additional surgical procedures (p < 0.05). Infants with antenatal presence of thickened bowel wall (greater than or equal to 3 mm) required longer administration of parenteral nutrition (median 34 vs. 20 days; p < 0.01) and prolonged stay (median 44 vs. 37 days; p < 0.05). Presence of oligohydramnion (amniotic fluid index below 8 cm) was connected with longer administration of parenteral nutrition in newborns (median 30 vs. 16 days; p < 0.05)., Conclusion: The isolated presence of oligohydramnion with amniotic fluid index below 8 cm, thickened bowel wall equal to or more than 3 mm and the prenatal intra-abdominal dilatation with 10 mm cutoff had significant predictive value for the adverse postnatal outcome of patients with gastroschisis.

The aim of study was to introduce technical innovation of MIRPE which reduces the risk of cardiac injury. Modification of MIRPE method with semiflexible thoracoscope and sternum elevating technique has been used. Volkmann bone hook has been inserted percutaneously to the sternum. The hook elevates the sternum forward and enlarges the retrosternal space for safer passage of thoracoscopically guided introducer. Using semiflexible thoracoscope allows better view from various angles via one site of insertion. During the period 2005-2012, the MIRPE was performed on 29 girls and 151 boys; the mean age at the time of surgery was 15.9 years (range 13-18.7 years). The mean Haller index was 4.7 (range 2.7-20.5). The most common complication was pneumothorax (3.3 %) and the incidence of bar displacement was 2 %. The most serious complication was cardiac perforation when inserting Lorenz introducer. This occurred in a 16-year-old girl; she required urgent sternotomy with right atrial repair and recovered well. External elevation of sternum with the hook was used since this case. Subsequent 113 patients underwent surgery without any serious complications. Technical innovation using semiflexible thoracoscope and hook elevation of the sternum reduces the risk of cardiac injury. The hook opens the anterior mediastinum space effectively and makes the following dissection relatively safe and straightforward.

Introduction: The aim of this article is a retrospective analysis of the results of three methods used for the repair of pectus excavatum between 1991 and 2012., Material and Methods: 508 patients were operated on at the Department of Paediatric Surgery of the Second Faculty of Medicine, Charles University, and the Teaching Hospital in Motol between 1991 and 2012. 16 patients were older than 19 years (19-28 years). The average age of paediatric and adolescent patients was 14.2 years (5-19 years range). Boys (408) prevailed over girls (82), the ratio being 4.9:1. Open Holcomb technique was used to operate on 87 patients (17%), the Welch method was applied in 208 patients (41%). Minimally invasive repair of pectus excavatum (MIRPE) was used in 213 patients (42%) during the entire period of time., Results: As for the 295 patients operated on by the open Holcomb and Welch method, the outcome was excellent in 239 (81%) of them. A favourable result with a small deformity remaining occurred in 44 patients (15%). Unfavourable result was found in 7 patients (2.5%): in these children, a deep excavation occurred 3-5 years postoperatively. In 5 patients (1.5%) we evaluated the result as recurrent chest wall deformity. The minimally invasive method (MIRPE) produced an excellent result in 182 out of 213 patients (85%). A favourable outcome was observed in 27 (22%), unfavourable result in 1 (0.5%) patient., Conclusion: In the last 15 years, minimally invasive repair of pectus excavatum has become popular worldwide. Due to very favourable cosmetic results and a relatively small, gradually decreasing number of complications, the proportion of patients requiring surgery to correct their deformity has risen sharply.