European Journal of Pediatric Surgery最新文献

Background Visual examination is crucial for assessing pectus carinatum (PC) severity and treatment results. This cross-sectional study evaluates the inter- and intraobserver agreement of PC deformities before and after treatment. Methods Observers examined medical photographs of patients before and after treatment. Primary outcome was inter- and intraobserver agreement on esthetic results after treatment. Secondary outcomes included inter- and intraobserver agreement on severity and symmetry before treatment, differences in esthetic results after Ravitch surgery and dynamic compression bracing (DCS-bracing), and the impact of scars, age and treatment duration on esthetic results. Results Medical photographs of 201 patients (aged 4-18) were evaluated by five surgeons and five peers. Surgeons and peers demonstrated inadequate (κ<.61) interobserver agreement on esthetic results (κ=.26, κ=.22), severity of PC (κ=.43, κ=.38) and symmetry (κ=.37, surgeons only). Agreement between surgeons and peers on esthetic results (κ=.37) and severity before treatment (κ=.54) was similarly inadequate. Surgeons and peers demonstrated inadequate intraobserver agreement on esthetic results (κ=.49, κ=.34), severity of PC (κ=.54, κ=.48) and symmetry (κ=.60, surgeons only). Deformities treated with Ravitch surgery were perceived as more severe but yielded better results. Peers, unlike surgeons, viewed scars as negatively impacting results. No relationship was found between results after treatment and treatment duration (p=.682, p=.062) or age ( p=.205, p=.527). Conclusions Subjective assessment of PC severity and esthetic results is inconsistent. 3D-scanning could help standardize treatment completion and aid patients and surgeons in determining treatment completion. The psychosocial effects of scars should be addressed when discussing treatment options.

Introduction The perineal body preserving PSARP (posterior sagittal anorectoplasty) (PPP) is a novel modification of the original PSARP for female patients with rectovestibular fistulas designed to eliminate the risk of perineal body dehiscence. This study aims to examine the outcomes following PPP. Methods A retrospective, single institution study was performed examining female patients with rectovestibular fistula who underwent PPP between 1/2020-12/2023. Exposure was done through the intended anoplasty only, there was no perineal body or posterior sagittal incision utilized. No routine post-operative dilations were performed. Post-operative outcomes, day of discharge, time to first feeding, and early stooling patterns were assessed. Results Fifteen patients underwent a PPP at a median age of six months (range 2 days-19 months) and median follow up of eleven months (range 1-36). Three (20%) patients underwent repair within the first three months of life. Five (33%) had diverting ostomies prior to referral to our team. There was no incidence of dehiscence or rectal prolapse. Two (13%) patients developed an anal stricture which required revision. Fourteen (93%) patients resumed normal feeds on post operative day one. Eleven (73%) were discharged on post operative day one. All patients were stooling spontaneously at their most recent clinical encounter with eleven (73%) utilizing laxatives. Conclusions The PPP eliminates the risk of perineal body dehiscence and has a quick return to regular diet and home. There is a 13% stricture rate which could relate to a difference in the distal rectal mobilization compared to the traditional PSARP.

Background:  Esophageal atresia (EA) is a rare disease requiring surgical repair, usually within the first days of life. Patients with EA require intensive postoperative care and often have comorbidities. There is a lack of data on the costs incurred by patients with EA during the first year of life.

Methods:  Anonymized claims data were provided by the Techniker Krankenkasse (∼10.8 million clients). Data were extracted for patients who had an inpatient diagnosis of EA (International Classification of Diseases [ICD]: Q39.0 or Q39.1) and a reconstruction of the esophageal passage in case of atresia (Operationen-und Prozedurenschlüssel [German version of ICPM, International Classification of Procedures in Medicine; OPS] 5-428.0 to 5-428.7, 5-316.1 or 5-431.0) during their first hospital stay. All patients were in their first year of life at initial hospitalization (2016-2020) and were followed up for 1 year. Costs, length of hospital stay, and duration of mechanical ventilation and differentiated OPS services were analyzed using descriptive statistics. Multiple linear regression was used to analyze the determinants of hospital costs.

Results:  A total of 119 patients with EA were included (55.5% male). The mean cost of the 1-year observation period was €89,736 ± 97,419 (range €12,755-640,154). The increasing costs of the initial hospitalization led to a disproportionate increase in the costs of the 1-year observation period. The presence of an associated malformation combined with surgical complications was associated with almost five-fold higher costs than in patients without an associated malformation and an uncomplicated course (€193,103 ± 157,507 vs. €39,846 ± 33,473). The mean duration of mechanical ventilation was 23.2 ± 43.1 days and the mean length of hospital stay was 80.3 ± 77.2 days.

Conclusion:  To our knowledge, this is the first study to investigate the costs of EA patients in the first year of life. The presence of an associated malformation combined with surgical complications was associated with almost five-fold higher costs than in patients without an associated malformation and an uncomplicated course.

Aim of the study:  Esophageal atresia (EA) is associated with impaired motor development, cardiopulmonary function, and physical activity (PA). Despite missing scientific evidence, this fact is often attributed to associated congenital heart disease (CHD). The aim of this study was to investigate PA in EA patients without CHD compared with CHD patients and healthy controls.

Methods:  In this multicenter study, EA patients aged 6 through 17 years were included. Moderate-to-vigorous PA (MVPA, minutes per week) was assessed using the standardized and validated questionnaire Motorik-Modul Physical Activity Questionnaire. EA patients were randomly matched 1:4 for gender and age with patients with CHD (n = 1,262) and healthy controls (n = 6,233). Patients born with both EA and CHD were excluded. Means and 95% confidence intervals (95% CIs) were calculated. To identify associated factors, Spearman's correlation was performed.

Main results:  Overall, 69 EA patients were matched with 276 CHD patients and 276 controls (57% male, 43% female, mean age 10,3 years, 95% CI: 9.5-11.1). Mean MVPA was reduced in EA (492 minutes, 95% CI: 387-598) and CHD patients (463 minutes, 95% CI: 416-511) compared with controls (613 minutes, 95% CI: 548-679). In subgroup analysis, MVPA was reduced further in females and older age groups with EA and CHD. For EA patients, there was no statistical association between Gross type, current symptoms, surgical approaches, and MVPA.

Conclusion:  Isolated EA and CHD were associated with reduced PA, especially in females and teenagers. To avoid additional morbidity associated with sedentary behavior, PA should be promoted during follow-up.

Introduction:  Previous assumptions suggested that the technique of approximation without osteotomy in primary exstrophy repair (PER) could only be applied in newborns and anticipated poorer outcomes. Recent studies indicated that this technique can be successfully executed not only in immediate PER but also yields favorable long-term results. Therefore, we evaluated and compared the orthopaedic and radiological long-term outcomes after pubic symphysis approximation without osteotomy in immediate and delayed PER.

Methods:  From March 2018 to December 2020, individuals with PER and approximation of the symphysis without osteotomy were recruited. Patients <12 years and with a history of orthopaedic surgery of the bony pelvis were excluded. Orthopaedic examinations and magnetic resonance imaging (MRI) of the bony pelvis including the hip joints were performed and pubic diastasis, the acetabulum angle (ACA), and the center-edge angle (CEA) were evaluated.

Results:  Twenty-nine patients were included, 11 of them had an immediate and 18 had a delayed PER. Between the two groups, no significant differences could be observed concerning hip pain (p = 0.419), mobility impairment (p = 0.543), sports impairment (p = 0.543), hip impingement (p = 1.000), leg length discrepancy (p = 0.505), and width of the pubic diastasis as measured by MRI (p = 0.401). There were also no significant differences with regard to CEA right (median 30 degrees, p = 0.976), CEA left (median 31.5 degrees, p = 0.420), ACA right (median 19 degrees, p = 0.382), and ACA left (median 17 degrees, p = 0.880).

Conclusion:  There were no significant differences in clinical orthopaedic or radiological long-term outcomes between bladder exstrophy patients after immediate and delayed bladder closure with symphysis approximation without osteotomy. Establishing core outcome sets is essential to get robust and comparable results, further advancing and substantiating our initial insights.

Background:  Necrotizing enterocolitis (NEC) is one of the main causes of acute abdomen in neonates. Surgical treatment entails important morbidity and mortality and conservative management, when possible, offers better outcomes. Post-NEC intestinal strictures are one of the main complications.

Methods: Retrospective analysis from June 2011 to November 2022 of post-NEC strictures (PNS) after conservative management of neonates diagnosed with NEC (modified Bell stage IIA or higher) at a tertiary neonatal surgery center.

Results:  Out of 219 NEC, 126 received initial conservative management (57.5%), 24 (19%) of which eventually underwent surgery for PNS. Average gestational age and weight at birth of our cohort were 31.3 ± 4.9 weeks and 1,694 ± 1,009 g.PNS diagnosis was made 38.4 ± 16.5 days after the NEC episode. 6/24 (25%) were asymptomatic and diagnosed by screening enema, 11 (46%) presented signs of intestinal obstruction before the enema could be performed and 7 (29%) after a normal previous protocol study.Median age at PNS surgery was 56 ± 17.9 days. A total of 2/3 strictures were found in cecum, ascendent, and transverse colon. Primary resection and anastomosis were performed in all cases. Feeds were restarted on postoperative day 4.3 ± 2.9. Two cases presented anastomotic complications (1 dehiscence and 1 stenosis), and no deaths were recorded.

Conclusions:  PNS is a frequent complication after conservative management. Deffered surgical treatment after the acute NEC episode is resolved allows for safer surgeries (since patients have reached hemodynamical stability and overcome septic shock), shorter resections, and favorable postoperative outcomes.

Introduction:  This study aimed to evaluate the non-neoplastic renal parenchyma in Wilms tumor (WT) and investigate its impact on nephron-sparing surgery (NSS).

Materials and methods:  The non-neoplastic renal parenchyma of WT patients was prospectively collected for pathological examination. The histology of non-neoplastic renal parenchyma was assessed from two perspectives: nephrogenic rests (NRs) and nephrons.

Results:  A total of 46 non-neoplastic renal parenchyma specimens were collected from 42 WT patients. The surgeons assessed the median proportion of non-neoplastic renal parenchyma as 30%, whereas using ellipsoid volume, it was calculated to be 27%. The Youden index of surgeons' assessment peaked at a 15% proportion of non-neoplastic renal parenchyma. The bilateral WT (BWT) group and NSS group exhibited significant differences compared with the unilateral WT group and radical nephrectomy group, respectively, with the BWT group showing a tendency toward thickened basement membrane.

Conclusion:  The presence of NRs and endogenous nephron alternations should be given due attention in WT. The probability of abnormalities is low when the proportion of non-neoplastic renal parenchyma exceeds 15%, providing pathological support for expanding the adaptation of NSS.

Introduction:  The aim of the study was to investigate the prevalence of esophageal atresia (EA), frequency of associated anomalies, and mortality from 2004 to 2017 in the Finnish population. We hypothesized the Spitz classification and the presence of other congenital malformations would predict mortality in patients with EA as well as assumed the survival to be high among patients with EA.

Materials and methods:  This retrospective, population-based study was based on the registries maintained by THL Finnish Institute for Health and Welfare and Statistics Finland. The cases were identified and classified according to the World Health Organization's International Classification of Diseases and Health Related Problems (ICD) revisions 9 and 10 (ICD-9 and ICD-10) codes and accompanying written diagnoses. Associated anomalies were classified based on the EUROCAT criteria, and minor anomalies were excluded. All statistical tests were performed as a two-sided significance level set at p < 0.05. The chi-square or Fisher's exact test was utilized for categorical variables. The change in prevalence rates during the study period was evaluated with linear regression.

Results:  In total, 337 cases with EA were identified including 295 (87.5%) live births, 17 (5.0%) stillbirths, and 25 (7.4%) terminations of pregnancy. The total prevalence for EA in Finland was 4.17/10,000 births with no significant change during the study period, p = 0.35. Neonatal mortality was 5% (n = 15) and 1-year survival was 91.5%. Mortality was associated with syndromic cases (p = 0.002). The Spitz classification predicted neonatal mortality better than cardiac anomalies alone (p < 0.001 and p = 0.6, respectively). Type C was the most common atresia type (65.9%) followed by type A (14.8%) and B (6.8%). The most common group of associated malformations were heart defects (35.0%) followed by other gastrointestinal tract malformations (15.3%) and limb anomalies (12.2%). Syndromic cases (12.2%) were associated with type A and B atresias (p = 0.001). VACTERL association was observed in 16.6% of the cases.

Conclusion:  The overall prevalence of EA remains stable and relatively high in Finland. Despite the high prevalence of co-occurring malformations, the overall survival rate is high. Spitz classification predicted neonatal survival well.

Background/purpose:  Gastroesophageal reflux disease (GERD) after lung transplantation (LuTx) can lead to chronic lung allograft dysfunction. Our aim was to assess the prevalence of GERD in pediatric LuTx recipients and to investigate the impact of medical and surgical GERD treatment on lung function.

Methods:  Ethical approval was obtained. Data of all consecutive patients who underwent LuTx from 2013 to 2023 and aged < 18 years at the time of the study were prospectively collected. A GERD diagnostic algorithm was established, including assessment of symptoms (vomiting, heartburn, regurgitation, cough, recurrent airway infections), bronchoscopy, forced expiratory volume in 1 second, and pH impedance. Further investigations included upper gastrointestinal series and esophagogastroduodenoscopy. Patients with GERD underwent medical treatment or fundoplication. Lung function was monitored.

Results:  Thirty-six patients (2 months-18 years, 50% male) were included. Twenty-nine (80%) underwent spirometry, 16 (45%) pH impedance study, and 14 (39%) esophagogastroduodenoscopy. Twenty-two (61%) had no GERD symptoms and 12 (33%) showed normal pH impedance study or esophagogastroduodenoscopy. Fourteen (39%) patients had GERD symptoms, all 9 tested symptomatic patients (25%) had pathological GERD-specific diagnostics. Three (8%) patients underwent fundoplication after a median of 1.6 years (range 1.1-5.7 years) post-LuTx without surgical complications. After a median follow-up of 2.3 years (range 1.3-2.8 years) post-fundoplication, all (n = 3) had complete remission of GERD symptoms and lung function improvement. Lung function decline was observed in 6 (67%) of the tested symptomatic patients on proton-pump inhibitors (PPIs) treatment.

Conclusion:  Over one-third of our patients presented with GERD symptoms after LuTx. Symptoms and lung function may be reliable GERD indicators. Given the high prevalence of GERD, we suggest a routine posttransplant diagnostic algorithm including pH impedance study. Eighty percent of all symptomatic patients had a lung function decline despite PPI. Fundoplication is safe and may improve long-term outcome in pediatric LuTx recipients.

Introduction:  Anorectal malformations (ARMs) are rare congenital anomalies that involve the anus, rectum, and oftentimes the genitourinary tract. The management of ARM patients is complex, and many controversies exist. To address this issue, the European Reference Network eUROGEN for rare and complex urogenital conditions aimed to develop comprehensive guidelines for the management of ARM.

Methods:  The Dutch Quality Standard for ARM served as the basis for the development of guidelines applicable on a European level. Literature was searched in Medline, Embase, and Cochrane. The ADAPTE method was utilized to incorporate the newest available evidence. A panel of 15 experts from 7 European countries assessed currency, acceptability, and applicability of recommendations. Recommendations from the Dutch Quality Standard were adapted, adopted, or rejected, and recommendations were formed considering current evidence and/or expert consensus.

Results:  Prenatal and neonatal diagnostic workup as well as postsurgical follow-up of anorectal, genitourinary tract, and neurologic system were reviewed. Seven new studies were identified. The panel adapted 13 recommendations, adopted 7, and developed 8 de novo. The availability of high-quality evidence was limited, and most recommendations were based on retrospective studies, case series, or expert opinion.

Conclusion:  Patients with ARM and their families require highly specialized and comprehensive care from the prenatal period to adulthood. This guideline provides recommendations for a comprehensive diagnostic workup of children with ARM throughout their life that is applicable on a European level.