Introduction Although extracorporeal shock wave lithotripsy (ESWL) is minimally invasive and highly efficient for the management of kidney stones, adverse effects have been described. Available indicators of renal function exhibit insufficient sensitivity in acute renal injury (AKI). We aimed to evaluate the severity of the kidney tissue response to ESWL injury by measuring the urinary neutrophil gelatinase-associated lipocalin (uNGAL), which can indicate AKI in its early phase. Material and methods The prospective, controlled study included 62 patients with nephrolithiasis undergoing single ESWL treatment. uNGAL level was measured before the procedure, and 6 h and 12 h after. Results The median uNGAL level increased by 126.0%, 6 h after ESWL (p <0.001). The growth rate continued and 12 h after was higher by 583.7%, compared to the pre-treatment level (p <0.001). The median value of estimated glomerular filtration rate (eGFR) dropped by 15.3% 12 h after the treatment (p <0.001). It increased by 5.0% in the period 7 days to 3 months after (p <0.001) and after 3 months it was lower by 10.1% compared to pre-ESWL values (p <0.001). uNGAL level after 12 h was significantly negatively associated with eGFR, 12 h, 7 days and 3 months after the ESWL. The sensitivity of uNGAL 12 h after ESWL was 60.6%; its specificity was 75.0%, with a positive predictive value of 74.0% and negative predictive value of 61.7%. Conclusions uNGAL appears to be a useful biomarker for the assessment and prediction of AKI. It was noticed that uNGAL had the highest predictive value 12 h after the ESWL treatment.

BACKGROUND Infantile hypertrophic pyloric stenosis (IHPS) is the most common condition requiring surgery in infancy, but the etiology of IHPS is still unclear. The study aimed to analyze the epidemiological and clinical features of the infants with IHPS in our setting and determine the yearly trends in IHPS incidence in the Sarajevo Canton between 2007 and 2016. METHODS We retrospectively analyzed epidemiologic, clinical, and operative data of all infants undergoing pyloromyotomy for IHPS over ten years in the largest tertiary care facility in Bosnia and Herzegovina. RESULTS Fifty-three IHPS patients were diagnosed, yielding an overall incidence of 1.17 per 1000 live births (1.25 and 1.09 cases in 2007-2011 and 2012-2016, respectively). IHPS was more prevalent among male infants (ratio 6.6:1, p < 0.001). The mean age at onset of symptoms was 39.6 days (range, 17-107 days). The estimated median time from symptoms onset to hospitalization was 11 days (range, 1-17 days). The mean age at diagnosis was significantly longer in premature infants compared with term infants (p = 0.003). Both first-born rank and bottle-feeding were significantly associated with IHPS (p = 0.001 and p = 0.04, respectively). No seasonal variation associated with IHPS was detected (p = 0.25). No evidence was found of differences in the incidence of IHPS related to maternal age (p = 0.24) and smoking (p = 0.59). CONCLUSION Our data indicate a declining trend and provide insights into the clinical characteristics of IHPS in Bosnia and Herzegovina. Most of the obtained results are in line with the published data and could improve the quality of local pediatric services.

Abstract Rationale: Peutz-Jeghers syndrome (PJS), a rare autosomal dominant disorder, is characterized by mucocutaneous pigmentations, hamartomatous polyps in the gastrointestinal tract, and a high risk of developing various malignancies. To the best of our knowledge, only 1 case of appendiceal carcinoid associated with PJS has been previously reported in the pediatric population. Patient concerns: We report a 7-year-old girl who was admitted for severe, intermittent abdominal pain and cramps, nausea, and vomiting. Multiple brown melanotic macules on the lips, buccal mucosa, and the tongue were noted. Diagnosis: A plain abdominal X-ray in a standing position revealed dilated intestinal loops with multiple air-fluid levels. A computed tomography scan of the abdomen showing a “coffee bean” appearance of the jejunal loop with a transition point to the duodenal loop. Axial-contrast-enhanced computed tomography scan of the abdomen showing dilated jejunum loops, filled with fluid with the swirled appearance of mesentery typical for volvulus. The diagnosis of PJS was based on clinical findings along with the histopathologic confirmation of the hamartomatous polyps. Interventions: An emergency laparotomy was performed, revealing a jejunojejunal intussusception starting 40 cm from the duodenojejunal flexure. Jejunotomy revealed that a lead-point intussusception was a necrotic hamartomatous polyp. After resecting the involved jejunal necrotic segment, including the polyp, end-to-end jejuno-jejunal anastomosis was performed. Further exploration revealed the presence of a jejunal mass 80 cm from the duodenojejunal flexure identified as another hamartomatous pedunculated polyp. The polyp was resected, and the enterotomy was then closed transversely. The grossly normal appendix was also removed. Outcomes: Clinical findings along with the histopathologically confirmed hamartomatous polyps were consistent with PJS. An appendiceal carcinoid (well-differentiated neuroendocrine tumor, European Neuroendocrine Tumor Society stage pT2) was incidentally detected during histological examination of the appendix. The patient and parents were counseled accordingly, focusing on active surveillance and control of symptoms. Two additional hamartomatous polyps (gastric and jejunal) were detected endoscopically and resected in the fourth postoperative week. A regular, 1-year follow-up and surveillance revealed no complications or recurrences. Lessons: Unusual neoplasms can occasionally be encountered in well-defined syndromes such as PJS. Therefore, active follow-up and surveillance are mandatory for all patients with PJS.

Background Encapsulating Peritoneal Sclerosis (EPS) is a rare phenomenon in paediatric patients with kidney failure treated with peritoneal dialysis (PD). This study highlights clinical challenges in the management of EPS, with particular emphasis on peri-operative considerations and surgical technique. Methods Retrospective analysis of all paediatric patients with EPS treated at the Manchester Centre for Transplantation. Results Four patients were included with a median duration of 78 months on PD. All patients had recurrent peritonitis (> 3 episodes), and all had symptoms within three months of a change of dialysis modality from PD to haemodialysis or transplant. In Manchester, care was delivered by a multi-disciplinary team, including surgeons delivering the adult EPS surgical service with a particular focus on nutritional optimisation, sepsis control, and wound management. The surgery involved laparotomy, lavage, and enterolysis of the small bowel + / − stoma formation, depending on intra-abdominal contamination. Two patients had a formal stoma, which were reversed at three and six months, respectively. Two patients underwent primary closure of the abdomen, whereas two patients had re-look procedures at 48 h with secondary closure. One patient had a post-operative wound infection, which was managed medically. One patient’s stoma became detached, leading to an intra-abdominal collection requiring re-laparotomy. The median length of stay was 25 days, and patients were discharged once enteral feeding was established. All patients remained free of recurrence with normal gut function and currently two out of four have functioning transplants. Conclusions This series demonstrates 100% survival and parenteral feed independence following EPS surgery. Post-operative morbidity was common; however, with individualised experience-based decision-making and relevant additional interventions, patients made full recoveries. Health and development post-surgery continued, allowing the potential for transplantation. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information

Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung's disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung's disease) from 264 hospitals (89 in high-income countries, 166 in middle-income countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p<0·0001 between all country income groups). Gastroschisis had the greatest difference in mortality between country income strata (nine [90·0%] of ten in low-income countries, 97 [31·9%] of 304 in middle-income countries, and two [1·4%] of 139 in high-income countries; p≤0·0001 between all country income groups). Factors significantly associated with higher mortality for all patients combined included country income status (low-income vs high-income countries, risk ratio 2·78 [95% CI 1·88–4·11], p<0·0001; middle-income vs high-income countries, 2·11 [1·59–2·79], p<0·0001), sepsis at presentation (1·20 [1·04–1·40], p=0·016), higher American Society of Anesthesiologists (ASA) score at primary intervention (ASA 4–5 vs ASA 1–2, 1·82 [1·40–2·35], p<0·0001; ASA 3 vs ASA 1–2, 1·58, [1·30–1·92], p<0·0001]), surgical safety checklist not used (1·39 [1·02–1·90], p=0·035), and ventilation or parenteral nutrition unavailable when needed (ventilation 1·96, [1·41–2·71], p=0·0001; parenteral nutrition 1·35, [1·05–1·74], p=0·018). Administration of parenteral nutrition (0·61, [0·47–0·79], p=0·0002) and use of a peripherally inserted central catheter (0·65 [0·50–0·86], p=0·0024) or percutaneous central line (0·69 [0·48–1·00], p=0·049) were associated with lower mortality. Interpretation Unacceptable differences in mortality exist for gastrointestinal congenital anomalies between low-income, middle-income, and high-income countries. Improving access to quality neonatal surgical care in LMICs will be vital to achieve Sustainable Development Goal 3.2 of ending preventable deaths in neonates and children younger than 5 years by 2030. Funding Wellcome Trust.

OBJECTIVE Appendiceal perforation has significant effects on perioperative morbidity and postoperative outcome. The present study aimed to identify possible predictive factors associated with perforated appendicitis (PA) in children at admission in the emergency department (ED). METHODS In this retrospective observational cohort study, consecutive medical records of children <18 years old with surgically and histopathologically confirmed acute appendicitis (AA) over three years (2013-2015) were analyzed. Patients were divided into two groups: PA and non-perforated appendicitis (NPA). The differences between the two groups and potential predictors of PA were explored using univariate and multivariate analyses. RESULTS During the study period, 295 patients underwent an appendectomy and had confirmatory AA diagnoses. Ninety-two patients had a PA (31.2%). In the univariate analysis, male gender, vomiting, diarrhea, fever, elevated white blood cell count (WBC) levels, and high C-reactive protein (CRP) were identified as predictors of PA. In the multivariate analysis, male gender (odds ratio [OR]: 3.133; 95% confidence interval [CI]: 1.610-6.096); vomiting (OR: 2.346; 95% CI: 1.141-4.822); diarrhea (OR: 4.549; 95% CI: 1.850-11.181); fever (OR: 3.429; 95% CI: 1.765-6.663); elevated WBC (OR: 2.962; 95% CI: 1.491-5.884) and elevated CRP (OR: 3.061; 95% CI: 1.267-7.396) were variables that predicted the PA in children. CONCLUSION Our data indicate that several clinical and biochemical parameters can reliably distinguish between pediatric PA and NPA at admission in the emergency department.

Abstract Abdominal injuries are rare in sports, but even minor traumas may result in potentially life-threatening injuries. The most frequent abdominal injury is abdominal wall contusion, following by blunt injuries of the kidneys and spleen. The cases of blunt abdominal injuries in soccer are rarely reported in the literature. We present a case of a grade IV splenic laceration in a 10-year-old soccer goalkeeper caused by the blunt abdominal trauma sustained during the gameplay. The trauma was successfully treated with a nonoperative management approach.

Purpose The acute scrotum (AS) in the pediatric population is a medical emergency. The most common causes of AS include testicular torsion (TT) and torsion of the appendix testis (TAT). Their distinction may be clinically challenging. The purpose of our study was to compare demographic and clinical characteristics of the pediatric cases of TT and TAT and thus provide clinical evidence for distinguishing these two conditions. Methods We retrospectively analyzed all children ≤ 16 years who underwent surgical exploration for AS. The patients were divided into Group 1 or TT and Group 2 or TAT groups. Results Ninety patients were included in the study (24 with TT and 66 with TAT). Patients with TT were significantly older than those with TAT (p < 0.001). The peak incidence of TT was in the age of 12–16 years (p < 0.001), whereas the peak of TAT was in the age group of 7–11 years (p < 0.001). Scrotal pain was more prevalent in patients with TAT (p = 0.02), whereas systemic signs (nausea/vomiting and abdominal pain) affected more frequently the TT patients (p = 0.003 and p < 0.001, respectively). The mean duration of symptoms was significantly longer in the TAT group than in the TT group (p < 0.001). Color-Doppler Ultrasound (CDUS) findings of absent or decreased testicular blood flow in the affected testis strongly favored the diagnosis of TT (p < 0.001). Conclusion Our data indicate that the older age, shorter duration of symptoms, systemic signs (nausea/vomiting and abdominal pain), and characteristics CDUS findings can help distinguish between the two most common acute scrotum causes.

A congenital hernia into the umbilical cord (CHUM) is often misinterpreted as a mild form of omphalocele. Herniated content in CHUM can be either the solitary intestinal loop or persistent omphalomesenteric duct (POMD) with the potential for traumatic injury in a case of inadequate examination of the umbilical cord and its clamping in the delivery room. Herein, we report a case of a male newborn with a functional bowel obstruction due to peritonitis caused by necrosis of iatrogenically clamped POMD in the CHUM. A 2-day-old full-term male infant was referred to the emergency department with a 1 day history of bilious vomiting, a gradual increase in abdominal distension, and absence of passage of meconium. The infant was born at 38 weeks’ gestation with a birthweight of 2,885 g. The baby was born following an uncomplicated pregnancy and a normal spontaneous vaginal delivery. The Apgar scores were eight at both 1 and 5 min. On physical examination, his abdomen was slightly distended by an umbilical ligation clip placed approximately 2.5 cm from the enlarged base of the umbilical cord (Fig. 1a). The stump of the umbilical cord was thickened and red. The baby was pale and dehydrated. An abdominal radiograph showed dilated small bowel loops due to small bowel obstruction (Fig. 1b). After resuscitation, a surgical exploration via a circumumbilical incision revealed a clamped POMD in the umbilical cord. The top of the POMD was crushed by the umbilical cord clamp (Fig. 1c). The POMD was resected at its base on the ileal loop. The ileal loop was closed transversely using 5-0 Vicryl by single-layer extra mucosal-interrupted sutures. The postoperative recovery was uneventful at a follow up of 8 years. Evaluation of the umbilical cord is a routine part of every newborn examination in the delivery room. Any suspected abnormal thickening of the base of the umbilical cord or any other malformations found should be further evaluated by a neonatologist or pediatric surgeon. Congenital hernia into the umbilical cord is a type of ventral abdominal wall defect in which the bowel usually herniates into the base of normally inserted umbilical cord through a patent umbilical ring. The condition results from a failure of return of intestine loops following the physiological gut herniation around 10–12 weeks of gestation. Due to similar morphologic features, characterized by coverage of eviscerated abdominal contents with a sac comprising outer amnion and inner peritoneal lining, CHUM may be easily misdiagnosed as a small omphalocele. Unlike an omphalocele, CHUM has an intact abdominal wall with adequate muscle development and a complete umbilical ring covered by a small cuff of skin about ~2.5 cm. Congenital hernia into the umbilical cord is usually not linked to chromosomal abnormalities but cases of trisomy 13 associated with CHUM have been reported in the literature. However, if missed, this condition can lead to intestinal damage by a low-placed umbilical cord clamp as it is shown in our illustrative case. Although very rare, similar complications have been reported in the literature. The prevention of inadvertent bowel injury during cord clamping at delivery is possible with increased awareness and knowledge regarding CHUM. Primary prevention includes the prenatal sonographic CHUM detection characterized by intestinal protrusion only into the base of the hernia. The most important preventive measure if the umbilical cord is broad based is the umbilical cord clamping at a safe distance from the basis (at least 5 cm from the abdominal wall). In conclusion, a careful inspection of the umbilical cord of all newborns in the delivery room is essential to identify any clinically relevant umbilical abnormality (e.g., a persistence of CHUM with POMD). This would prevent any iatrogenic gut injury during umbilical cord clamping. Although these complications are rare, they should be kept in mind when performing umbilical cord clamping.

Purpose Infantile hemangiomas (IH) are the most common benign vascular tumors of infancy. Ocular complications due to orbital IH include strabismal, deprivational, or anisometropic astigmatism, and visual loss secondary to amblyopia. Observations We report a case of a two-month-old female infant with a severe deep orbital IH. The diagnosis was established by clinical examination and magnetic resonance imaging (MRI). The patient was treated with oral propranolol for six months. Complete tumor regression was observed clinically and by MRI following the treatment. The patient showed no adverse effects or tumor recurrence at the 14-month follow-up. Conclusions and Importance Despite its benign nature, periorbital IH requires a rapid diagnosis and prompt treatment to prevent vision loss, particularly in infants. Our case confirms that oral propranolol may be regarded as a safe drug for periorbital IH with an excellent outcome.

Abstract Rationale: Pentalogy of Cantrell (POC) is an extremely rare syndrome with an estimated incidence of 1:65,000 to 200,000 live births. Its complete form includes a midline epigastric abdominal wall defect, defects affecting the lower sternum, anterior diaphragm, diaphragmatic pericardium, and various intracardiac defects. Patient concerns: We report a case of complete POC affecting only the first-born of a set of premature dizygotic twins. Diagnosis: A giant omphalocele with an eviscerated liver and bowel on prenatal, obstetric ultrasonography at 24 gestational weeks was observed. At birth, physical examination confirmed a massive (10 × 8 cm) epigastric omphalocele in which a significant part of the liver was seen. A postnatal echocardiogram revealed the presence of an ostium secundum atrial septal defect, perimembranous ventricular septal defect, and moderate pulmonary stenosis. X-ray showed an abnormal intrathoracic positioned stomach, which was confirmed with a plain x-ray of the upper intestinal tract with hydrosoluble contrast. Computed tomography (CT) scan revealed the sternum's absence and a close connection between the pericardial sac and the stomach wall. Interventions: The patient underwent surgical intervention at 18 days of age. Outcomes: Despite adequate and appropriate postoperative treatment, the baby rapidly deteriorated and died 72 hours after surgery. Lessons: POC is a complex, high-mortality syndrome whose management requires a multidisciplinary approach and meticulous planning. Despite all efforts, POC carries a poor prognosis, particularly in patients affected by its complete form.

Aim To evaluate modalities of extensor tendons repair of hand and forearm in specific anatomical zones with regard to etiological factors and presence of associated injuries of adjacent anatomical structures. Methods This cross-sectional study included 279 patients referred for extensor tendon repair of hand and forearm in specific anatomical zones. Available treatment modalities were evaluated concerning etiological factors, anatomical zones, and associated injuries. Statistical significance was analysed in the occurrence of early and late postoperative complications according to anatomical zones. Results Direct repair of extensor tendon lesions was found to be the most common modality of reconstruction, 230 (93.5%), of which blade injuries were predominant, 120 (48.7%). Direct tendon repair was mostly indicated in Zone VI and Zone III, in 55 (23.9%) and 42 (18.3%) patients, respectively. Statistically, a significant correlation was confirmed between treatment modalities, injuries in specific anatomical zones, and type of etiological factor (p<0.0001). Statistical correlation was confirmed between zones of injuries and the occurrence of early and late complications (p=0.002). Conclusion Successful postoperative recovery was correlated with the recognition of functional failure in specific zones, assessment of potential associated injuries, and selection of the most optimal modality of reconstruction.