European Journal of Pediatric Surgery最新文献

Introduction:  Parkes Weber's syndrome (PWS) is a rare genetic disorder characterized by overgrowth and vascular malformations, primarily affecting the extremities. While PWS is known to be associated with arteriovenous and capillary malformations, the potential involvement of lymphatic malformations (LMs) has not been previously reported. The objective of this study is to investigate the presence of lymphatic anomalies in PWS patients and their role in the development of limb asymmetry.

Materials and methods:  This is a retrospective study of patients diagnosed with PWS in a Vascular Anomalies Center from 1994 to 2020. Clinical data were obtained from medical records including diagnostic imaging, lymphoscintigraphy, and genetic testing. The Institutional Review Board and Ethics Committee have approved this study.

Results:  A total of 16 patients aged 18 interquartile range 14.7 years diagnosed with PWS were included (50% female). Six of the 16 patients with PWS had clinical and imaging data suggestive of LM (37.5%) and 3 of them had genetic variants in RASA1 (2/3) or KRAS (1/3). Limb asymmetry was greater in patients with isolated PWS (2.6 ± 0.8 cm) than in the PWS-lymphatic anomalies population (2 ± 0.7 cm), although not significant (p = 0.247). One in 6 patients with PWS-LM required amputation (16.6%) versus 1 in 10 in isolated PWS (10%).

Conclusion:  Lymphatic anomalies may be present in a significant number of patients with PWS and could have a role in limb asymmetry and outcomes. It is paramount to investigate their existence and distinguish them from true overgrowth.

Introduction:  Coexistent congenital duodenal obstruction and esophageal atresia (EA) is known to have significant morbidity and mortality. Management strategies are not well-defined for this association. The data from the Turkish EA registry is evaluated.

Materials and methods:  A database search was done for the years 2015 to 2022.

Results:  Among 857 EA patients, 31 (3.6%) had congenital duodenal obstruction. The mean birth weight was 2,104 (± 457) g with 6 babies weighing less than 1,500 g. Twenty-six (84%) had type C EA. The duodenal obstruction was complete in 15 patients and partial in 16. Other anomalies were detected in 27 (87%) patients. VACTERL-H was present in 15 (48%), anorectal malformation in 10 (32%), a major cardiac malformation in 6 (19%), and trisomy-21 in 3 (10%). Duodenal obstruction diagnosis was delayed in 10 (32%) babies for a median of 7.5 (1-109) days. Diagnosis for esophageal pathologies was delayed in 2. Among 19 babies with a simultaneous diagnosis, 1 died without surgery, 6 underwent triple repair for tracheoesophageal fistula (TEF), EA, and duodenal obstruction, and 3 for TEF and duodenal obstruction in the same session. A staged repair was planned in the remaining 9 patients. In total, 15 (48%) patients received a gastrostomy, the indication was long-gap EA in 8. Twenty-five (77%) patients survived. The cause of mortality was sepsis (n = 3) and major cardiac malformations (n = 3).

Conclusion:  Congenital duodenal obstruction associated with EA is a complex problem. Delayed diagnosis is common. Management strategies regarding single-stage repairs or gastrostomy insertions vary notably depending on the patient characteristics and institutional preferences.

Objective:  This article tests the protective effect of a commercially available mixture of hyaluronic acid, chondroitin sulfate, and poloxamer 407 on the damage caused by the exposure of esophageal mucosa to button batteries in an animal model.

Methods:  Experimental study. Sixty porcine esophageal samples were distributed in three groups: control (CG), exposure (EG), and exposure-protection (EPG). In EG and EPG, one CR2032 button battery per sample was inserted, both were subdivided into 2-, 4-, 6-, and 24-hour exposure subgroups, with subsequent battery removal. EPG samples were irrigated with the solution 1 hour after battery exposure. Esophageal pH and final voltage of the battery were measured.

Results:  pH in CG remained stable. No significant differences in pH at 1 hour were found between EG and EPG. In EPG, the pH of the mucosa exposed to the anode was lower than in GE at 2 hours (12.44 vs. 11.89, p = 0.203) and 4 hours (13.78 vs. 11.77, p < 0.0001). In the cathode pH was significantly higher in EG at 2 hours (2.5 vs. 4.11, p < 0.0001), 4 hours (2.33 vs. 4.78, p < 0.0001), and 6 hours (2.17 vs. 2.91, p < 0.0001). Significant voltage reduction at 1 hour was found in EG compared to EPG (0.48 vs. 1.08 V, p = 0.004).

Conclusion:  Exposure to hyaluronic acid solution buffers the acidification on the side exposed to the cathode and basification on the anode. This effect can be maintained up to 3 to 5 hours, even after stopping its application. Our results suggest that a solution containing hyaluronic acid could be used as an esophageal protector after accidental ingestion of button batteries.

Objective:  Nonoperative management (NOM) for simple acute appendicitis (SAA) is an acceptable mode of treatment in healthy children. Previous studies of NOM routinely excluded young children (< 5 years); however, the effect of age on NOM failure has not been directly assessed. Efficiency of NOM in young adults is questionable. Therefore, adolescents may also be at greater risk of NOM failure. Our aim was to investigate the effect of age on NOM failure.

Methods:  This is a retrospective analysis of children with SAA who received NOM between January 1, 2019, and June 30, 2021, at our institution. NOM failure was defined by subsequent appendectomy. Age was assessed as a continuous variable, and we also compared different age subgroups.

Results:  In this study, 151 children were included (60% male), mean age 11.2 ± 3.2 years (range: 5-17). Overall, 66 children (44%) failed NOM, 90% of them within the first year (median 7 weeks). Ten percent of the cohort were younger than 6 years of age and 33% of them failed NOM (p = 0.39). Per 1 year increase in age, the odds of NOM failure increased by 12% (p = 0.027). Children over 14 years of age had 2.46 times higher odds to fail NOM (p = 0.03). These higher odds remained after adjusting for appendiceal diameter and appendicolith. Linear regression showed a decrease by a factor of 12 at the time of NOM failure with every 1-year increase in age (β = -12, p = 0.09).

Conclusion:  The risk of NOM failure in children increases with age; therefore, age should be considered when deciding on the optimal management of SAA, especially in adolescents. Effectiveness of NOM in children younger than 6 years is noninferior to older children and therefore should not be excluded.

Objective:  Etiology of vocal cord paralysis (VCP) and laryngeal dysfunction may be congenital or surgical trauma of recurrent and superior laryngeal nerves. We assessed the incidence, risk factors, and morbidity of VCP after repair of esophageal atresia (EA).

Methods:  Medical records of 201 EA patients from 2000 to 2022 were reviewed for this retrospective study. Postrepair vocal cord examination (VCE) included awake nasolaryngeal fiberoscopy by otolaryngologist or laryngoscopy under spontaneous breathing anesthesia. Before 2017, postoperative VCE was performed in symptomatic patients only and routinely after 2017.

Main results:  Overall, VCE was performed on 79 (38%) patients (52 asymptomatic), whereas 122 asymptomatic patients underwent no VCE. VCP was diagnosed in 32 of 79 patients (right 12, left 10, and bilateral 10; symptomatic 25 and asymptomatic unilateral 7) corresponding with extrapolated overall VCP incidence of 16 to 24% among 201 patients including asymptomatic ones. Ten patients (bilateral VCP 8 and left VCP 2) required tracheostomy. Of 10 patients with bilateral VCP, three underwent laryngotracheal expansion surgery (left VC lateralization in one and laryngoplasty in two with acquired subglottic stenosis), three remained tracheostomy dependent, three were off tracheostomy, and one died of complications after redo esophageal reconstruction. All patients with unilateral VCP managed without tracheostomy. Cervical dissection or ostomy formation was a major risk factor of VCP.

Conclusion:  Repair of EA is associated with a considerable risk of VCP and associated morbidity. Cervical EA surgery significantly increased the risk of VCP. Bilateral VCP may eventually require laryngotracheal expansion surgery.

Objective Survival of neonates with esophageal atresia (EA) is relatively high and stable, resulting in increased attention to optimizing care and longer-term morbidity. This study aimed to reach consensus on a quality indicator set for benchmarking EA care between hospitals, regions, or countries in a European clinical audit.

Methods Using an online Delphi method, a panel of EA health care professionals and patient representatives rated potential outcome, structure, and process indicators for EA care identified through systematic literature and guideline review on a nine-point Likert scale in three questionnaires. Items were included based on predefined criteria. In rounds 2 and 3, participants were asked to select the five to ten most essential of the included indicators.

Results An international panel of 14 patient representatives and 71 multidisciplinary health care professionals representing 41 European hospitals completed all questionnaires (response rate: 81%), eventually including 22 baseline characteristics and 32 indicators. After ranking, 10 indicators were prioritized by both stakeholder groups. In addition, each stakeholder group highly prioritized one additional indicator. Following an additional online vote by the other group, these were both added to the final set.

Conclusion This study established a core indicator set of twenty-two baseline characteristics, eight outcome indicators, one structure indicator, and three process indicators for evaluating (quality of) EA care in Europe. These indicators, covering various aspects of EA care, will be implemented in the European Pediatric Surgical Audit to enable recognition of practice variation and focus EA care improvement initiatives.

Introduction:  The aim of the study is to review the continence and volitional voiding rate in a single center cohort of exstrophy-epispadias patients following Young-Dees-Leadbetter bladder neck reconstruction and to explore factors which predict continence.

Materials and methods:  Children who underwent Young-Dees-Leadbetter bladder neck reconstruction as a final stage of repair in a large single low-volume center in a small-population country between 1997 and 2019 were included. Demographic and clinical details were extracted from the patients' charts. The primary end point was continence and volitional voiding. Patients were categorized as incontinent, socially continent (daytime dry intervals > 3 hours, wet nights) and fully continent (daytime dry intervals > 3 hours, dry nights).

Results:  The study cohort included 27 patients whose median age at reconstruction was 5 years, and median follow-up was 7.8 years (interquartile range [IQR] 6-11.2). The cohort included 24 classic exstrophy patients (89%, 17 males and 7 females) and 3 isolated complete epispadias patients (11%, 1 male and 2 females). Nine (33%) patients achieved full continence and social continence was achieved by nine (33%) patients, for an overall social continence rate of 67%. Preoperative bladder capacity of 110 mL or more was associated with achieving social continence (odds ratio = 6.4, p = 0.047). The overall volitional voiding rate was 67%.

Conclusion:  Young-Dees-Leadbetter bladder neck reconstruction yielded rates of 33% for full continence and 67% for social continence and volitional voiding. These rates are comparable to those of large high-volume centers. A preoperative capacity of 110 mL or more was the sole predictor of social continence.

This study aimed to describe the available dressings and their management in patients undergoing hypospadias repair and compare postoperative outcomes with and without dressing and between the different dressing types. A comprehensive electronic literature search of PubMed, Embase, and Cochrane Library was conducted to obtain studies, published in the period 1990 to 2021, reporting on the dressing used following hypospadias surgery. All information regarding the dressing was considered as primary endpoints, whereas surgical outcomes were assessed as secondary outcomes. Thirty-one studies containing 1,790 subjects undergoing hypospadias repair were included. Dressings were divided into three categories: nonadherent to the wound, adherent to the wound, and glue-based dressings. Most authors preferred to remove/change the dressing in the ward and the median time of removal/change was 6.56 postoperative days. The dressing removal appeared as the most frequent factor generating parental anxiety. The median rate of wound-related complications was 8.18%, of urethroplasty complications 9.08% and of reoperations 8.18%. Meta-analysis of outcomes showed higher risk of reoperations using conventional dressing, with no differences in urethroplasty and wound-related complications rates between conventional and glue-based dressings. Furthermore, the use of dressing reported increased risk of wound-related complications compared with no dressing, without significant differences regarding occurrence of urethroplasty complications and reoperations. The current evidence confirmed that there is no difference in outcomes of hypospadias repair depending on a certain dressing type. To date, surgeon's preference remains the main factor determining the choice for a specific dressing or for no dressing at all.

Background:  Normalization of metabolic alkalosis is an important pillar in the treatment of infantile hypertrophic pyloric stenosis (IHPS) because uncorrected metabolic alkalosis may lead to perioperative respiratory events. However, the evidence on the incidence of respiratory events is limited. We aimed to study the incidence of peroperative hypoxemia and postoperative respiratory events in infants undergoing pyloromyotomy and the potential role of metabolic alkalosis.

Materials and methods:  We retrospectively reviewed all patients undergoing pyloromyotomy between 2007 and 2017. All infants received intravenous fluids preoperatively to correct metabolic abnormalities close to normal. We assessed the incidence of perioperative hypoxemia (defined as oxygen saturation [SpO₂] < 90% for > 1min) and postoperative respiratory events. Additionally, the incidence of difficult intubations was evaluated. We performed a multivariate logistic regression analysis to evaluate the association between admission or preoperative serum pH values, bicarbonate or chloride, and peri- and postoperative hypoxemia or respiratory events.

Results:  Of 406 included infants, 208 (51%) developed 1 or more episodes of hypoxemia during the perioperative period, of whom 130 (32%) experienced it during induction, 43 (11%) intraoperatively, and 112 (28%) during emergence. About 7.5% of the infants had a difficult intubation and 17 required more than 3 attempts by a pediatric anesthesiologist. Three patients developed respiratory insufficiency and 95 postoperative respiratory events were noticed. We did not find a clinically meaningful association between laboratory values reflecting metabolic alkalosis and respiratory events.

Conclusions:  IHPS frequently leads to peri- and postoperative hypoxemia or respiratory events and high incidence of difficult tracheal intubations. Preoperative pH, bicarbonate, and chloride were bad predictors of respiratory events.