European Journal of Pediatric Surgery最新文献

Background:  Potential underlying genetic variations of pectus excavatum (PE) are quite rare. Only one-fifth of PE cases are identified in the first decade of life and thus are of congenital origin. The objective of this study is to test if early-onset PE is more likely to be part of genetic variations than PE that becomes apparent during puberty or adolescence.

Materials and methods:  Children younger than 11 years who presented with PE to the outpatient clinic of the Department of Pediatric Surgery at our center between 2014 and 2020 were screened by two clinical geneticists separately. Molecular analysis was performed based on the differential diagnosis. Data of all young PE patients who already had been referred for genetic counseling were analyzed retrospectively.

Results:  Pathogenic genetic variations were found in 8 of the 18 participants (44%): 3 syndromic disorders (Catel-Manzke syndrome and two Noonan syndromes), 3 chromosomal disorders (16p13.11 microduplication syndrome, 22q11.21 microduplication syndrome, and genetic gain at 1q44), 1 connective tissue disease (Loeys-Dietz syndrome), and 1 neuromuscular disorder (pathogenic variation in BICD2 gene).

Conclusion:  Early-onset PE is more likely to be part of genetic variations than PE that becomes apparent during puberty or adolescence. Referral for genetic counseling should therefore be considered.

Trial registration:  NCT05443113.

Objective:  Surgical intervention in pediatric patients can cause variable degrees of psychological stress with potential consequences in the perioperative period and even in the long term, after hospital discharge in the form of behavioral changes days and months later. The aim of our study was to determine which preoperative preparation strategy reduces postoperative maladaptive behavioral changes in children undergoing ambulatory pediatric surgery.

Materials and methods:  This prospective observational study included 638 pediatric American Society of Anesthesiologists physical status I or II patients who underwent ambulatory pediatric surgery. They were grouped into four preoperative preparation groups: not premedicated (NADA), premedicated with midazolam (MDZ), parental presence during induction of anesthesia (PPIA), and parental presence during induction of anesthesia and premedicated with midazolam (PPIA + MDZ). All patients included in the study were contacted by telephone during 1 year posthospital discharge to assess the postoperative maladaptive behavioral changes using the Posthospitalization Behavior Questionnaire (PHBQ). We performed a multivariate analysis to evaluate the influence of type of preparation and behavioral changes.

Results:  Patients in the PPIA and PPIA + MDZ preparation groups presented less postoperative maladaptive behavioral changes compared to patients in the NADA and MDZ groups (odds ratio [OR]: 1.8 [1.1-2.8] and OR 2.2 [1.03-4.49]) during the first week and first month. The intensity of emergence delirium measured by the Pediatric Anesthesia Emergence Delirium (PAED) scale increases the probability of postoperative maladaptive behavioral changes (OR: 1.05 [1.006-1.103]).

Conclusion:  The presence of parents during induction of anesthesia (PPIA and PPIA + MDZ) is a very effective strategy in reducing postoperative behavioral changes. These benefits are more significant in children under 5 years of age.

Introduction:  The possibilities, recent advances, and pitfalls in the histopathologic workup of specimens submitted for the diagnosis of Hirschsprung's disease (HSCR) are presented.

Materials and methods:  A literature research was performed in the database PubMed including the years 2000 to 2024.

Results:  The pathologist is involved in the diagnosis of HSCR in three scenarios: (1) the primary diagnosis in a child with the typical clinical symptoms, (2) the intra-operative guidance of surgery in a case where the diagnosis is already established, and (3) the confirmation of the diagnosis and the documentation of the extent of the disease in the resected specimen. Identification of ganglion cells in the enteric neural plexuses excludes HSCR, and the histological confirmation of a complete absence of these ganglion cells is the gold standard for its diagnosis. However, difficulties in the detection of ganglion cells with standard stains and/or a limited amount of tissue in the specimen submitted for diagnosis make supportive histologic stains and techniques, e.g., calretinin immunohistochemistry or acetylcholinesterase histochemistry necessary for an unequivocal diagnosis of HSCR.

Conclusions:  Improving the diagnostic accuracy of this life-threating disease is an interdisciplinary task. A good communication between pathologist and clinician, as well as mutual knowledge of skills and challenges of the other discipline, is necessary for a successful diagnostic teamwork.

Introduction:  Anastomotic stricture (AS) is the second most common complication after esophageal atresia (EA) repair. We aimed to evaluate the data in the Turkish Esophageal Atresia Registry to determine the risk factors for AS development after EA repair in a large national cohort of patients.

Methods:  The data between 2015 and 2021 were evaluated. Patients were enrolled into two groups according to the occurrence of AS. Patients with AS (AS group) and without AS (non-AS group) were compared according to demographic and operative features, postoperative intubation status, and postoperative complications, such as anastomotic leaks, fistula recanalization, and the presence of gastroesophageal reflux (GER). A multivariable logistic regression analysis was performed to define the risk factors for the development of AS after EA repair.

Results:  Among the 713 cases, 144 patients (20.19%) were enrolled into the AS group and 569 (79.81%) in the non-AS group. The multivariable logistic regression showed that, being a term baby (odds ratio [OR]: 1.706; p = 0.006), having a birth weight over 2,500 g (OR: 1.72; p = 0.006), presence of GER (OR: 5.267; p < 0.001), or having a recurrent tracheoesophageal fistula (TEF, OR: 4.363; p = 0.006) were the risk factors for the development of AS.

Conclusions:  The results of our national registry demonstrate that 20% of EA patients developed AS within their first year of life. In patients with early primary anastomosis, birth weight greater than 2,500 g and presence of GER were risk factors for developing AS. When patients with delayed anastomosis were included, in addition to the previous risk factors, being a term baby, and having recurrent TEF also became risk factors.

Level of evidence:  III.

Introduction:  Abdominal adhesions following surgery can lead to complications like intestinal obstruction and pelvic pain. While no molecular therapies currently target the underlying adhesion formation process, various barrier agents exist. 4DryField® has shown promise in reducing bleeding and adhesions in adults. This study aimed to assess its effectiveness in children.

Methods:  The study examined all pediatric patients who underwent laparotomy between January 2018 and February 2022. It compared outcomes between those treated with 4DryField® and a control group. Key endpoints included surgical revision, adhesion recurrence, infections, insufficiencies, fever, C-reactive protein (CRP) levels, and time to gastrointestinal passage.

Results:  In total, 233 children had surgery for bowel adhesions. After propensity score matching, 82 patients were included in the analysis: 39 in the control and 43 in the 4DryField® group. 4DryField® did not affect the readhesion rate. Children in the treatment group had significantly more complications (47 vs. 15%, p = 0.002), more often fever, and higher CRP levels.

Conclusions:  4DryField® did not show potential in reducing adhesion formation, but it was associated with significantly more complications in pediatric patients. Thus, future prospective studies are needed to evaluate the safety and effectiveness of 4DryField® in children.

Introduction:  The T-fasteners gastrostomy (T-PEG) has become increasingly popular over recent years as an alternative to the "pull-technique" gastrostomy (P-PEG). This study aimed to compare P-PEG and T-PEG complications.

Materials and methods:  A retrospective observational study of pediatric patients who underwent percutaneous endoscopic gastrostomy (PEG) placement. P-PEG was performed using the standard Ponsky technique and was replaced after 6 months by a balloon gastrostomy under sedation. T-PEG was performed using three percutaneous T-fasteners (that allow a primary insertion of a balloon gastrostomy). The balloon was replaced by a new one after 6 months without sedation. Complications were recorded.

Results:  In total, 146 patients underwent PEG placement, 70 P-PEG and 76 T-PEG. The mean follow-up was 3.9 years (standard deviation = 9.6). Age, weight, and associated comorbidities were comparable (p > 0.05). The overall complications were 17 (24.2%) in the P-PEG group and 16 (21.0%) in the T-PEG group (p > 0.05). P-PEG was associated with more sedation for button replacement (97 vs. 2.6% [p < 0.05]). P-PEG was associated with more early tube dislodgement during the first replacement (7.2 vs. 1.4% [p = 0.092]). Two of the five dislodged gastrostomies in the P-PEG group underwent laparotomy due to peritonitis, whereas the only dislodged gastrostomy in the T-PEG group was solved endoscopically. Altogether, P-PEG was associated with more complications that required urgent endoscopy, laparotomy, or laparoscopy (18.6 vs. 6.6% [p < 0.05]).

Conclusions:  P-PEG was associated with more sedation, complications during first button replacement, and complications requiring urgent endoscopy, laparotomy, or laparoscopy compared with T-PEG.

Introduction:  Duodenal atresia (DA) is the most common atresia of the small bowel. This study aims to assess the prevalence, mortality, and associated anomalies related to DA in Finland from 2004 to 2017.

Material and methods:  A nationwide study based on registers maintained by the Finnish Institute for Health and Welfare and Statistics Finland containing data on all live births and stillbirths and terminations of pregnancy. The cases were identified based on the ICD-9 and 10 (International Classification of Diseases revisions 9 and 10) codes. Associated anomalies were classified based on the EUROCAT criteria; minor anomalies were excluded.

Results:  There were 249 DA cases including 222 (89.2%) live births, 16 (6.4%) stillbirths, and 11 (4.4%) terminations. There was no significant change in the prevalence rates between 2004 and 2017. Live birth prevalence was 2.75/10,000 and total prevalence was 3.08/10,000 births. A total of 100 (40.2%) cases were isolated, 67 (26.9%) had other multiple congenital anomalies, and 83 (33.3%) were syndromic. There were no terminations in isolated DA. Most associated anomalies were cardiac (36.1%), followed by other gastrointestinal tract anomalies (23.7%) and limb deformities/defects (7.2%). Trisomy 21 was observed in 63 cases (25.3%). Neonatal mortality was 3.6% (n = 8) and at 1 year 95.0% were alive. Both neonatal and infant mortalities were associated with cardiac anomalies (p < 0.001 and p = 0.001, respectively). All neonatal deaths had associated cardiac defect(s).

Conclusions:  The prevalence of DA in Finland remains stable and among the highest reported. DA is often associated with cardiac anomalies, which portend a high risk for mortality. Despite the burden of associated anomalies, overall survival is high.

Objective:  The objective of the study was to compare the effective rate, cure rate, adverse reaction rate, and effective time of three methods in the treatment of infantile hemangioma, hoping to provide reference for improving the clinical therapeutic effect.

Methods:  This is a retrospective study of 307 infantile hemangioma patients admitted to the Department of Oral and Maxillofacial Surgery, School of Stomatology, China Medical University, from January 2014 to October 2021. The patients were divided into three groups. In group A, timolol maleate solution was introduced by the nano-microneedle technique (97 cases); in group B, timolol maleate drops dipped in medical swabs were applied (107 cases); and group C patients took propranolol orally (103 cases). The effective rate, cure rate, adverse reaction rate, and effective time of treatment were recorded and compared.

Results:  The total effective rate, total cure rate, and total adverse reaction rate of the three groups were 87.6, 65.2, and 9.4%, respectively. The results showed that the effective and cure rates of group A were higher than those of group B (92.8 and 76.3%, respectively; p < 0.05), the adverse reaction rate of group C was higher than that of group B (17.5%, p < 0.05), and the effective time of group A was shorter than that of groups B and C. In most patients, symptoms significantly improved within 2 months (p < 0.05).

Conclusion:  Timolol maleate introduced by nanometer microneedles is an effective and safe treatment for superficial hemangioma in infants with the highest effective and cure rates, lower incidence of adverse reactions, and the shortest effective treatment time.