European Journal Of Pediatric Surgery[JOURNAL]
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200 papers
Tuberous Breast and Poland Syndrome: An Underestimated Association?
Forlini V, Franchelli S, Romanini MV
Eur J Pediatr Surg · 2025 Feb · PMID 39626778 · Publisher ↗
AIM OF THE STUDY: Both Poland syndrome (PS) and tuberous breast (TB) are anomalies affecting the breast, but they are considered mutually exclusive. Our aim was to determine the possible coexistence of PS and TB and to...
AIM OF THE STUDY: Both Poland syndrome (PS) and tuberous breast (TB) are anomalies affecting the breast, but they are considered mutually exclusive. Our aim was to determine the possible coexistence of PS and TB and to discuss TB management when associated with PS. METHODS: Between 2010 and 2023, 51 female adolescent PS patients were studied at our center. Among these, we evaluated those who developed TB after puberty. PS diagnosis was made based on the hypoplasia or absence of pectoralis major muscle detected clinically and confirmed radiologically. Breast anomaly on PS side was classified with Thorax Breast and Nipple (TBN) classification: B1 corresponds to breast hypoplasia, B2 to breast aplasia. TB diagnosis was made with a clinical evaluation after puberty. TB cases were classified according to Grolleau classification. MAIN RESULTS: Among 51 postpubertal PS females, we identified 23 (45%) who developed TB. In 3 cases (13%) TB was bilateral, in 20 (87%) contralateral to PS affected side. Age at time of the first surgical procedure was 16 years. Patients were treated with breast implants, fat grafting, rigotomy, local flaps, or a combination of these. TB treatment was performed simultaneously with PS breast reconstruction when feasible, or after it. CONCLUSIONS: TB incidence in our series was almost twice than in general population. This is the first study demonstrating and quantifying this association. TB deformity must be considered while defining PS surgical path in adolescents. Fat grafting is the first surgical option to treat both anomalies. A multidisciplinary approach is needed to minimize number of surgeries and maximize cosmetic results.
Interferential Current Stimulation Enhances Rectal Motor Activity: Insights from an Isolated Perfused Porcine Model.
Martel R, Boettcher M, Klinke Petrowsky M … +4 more , Hoyos NA, Herrmann I, Magerl W, Herrmann M
Eur J Pediatr Surg · 2025 Feb · PMID 39587047 · Publisher ↗
INTRODUCTION: Interferential current (IFC) has been studied in several clinical trials for the treatment of bowel motility disorders, most often in children. However, only moderate effects are reported, and in contrast...
INTRODUCTION: Interferential current (IFC) has been studied in several clinical trials for the treatment of bowel motility disorders, most often in children. However, only moderate effects are reported, and in contrast to IFC, the so-called placebo application is indiscernible. The mechanisms and neuroanatomic points of action remain elusive. Therefore, this therapy remains being questioned. METHODS: To gain objective experimental data about IFC stimulation, we examined this method ex vivo in an isolated perfused porcine rectum including the mesorectum. To elucidate the role of plexus nerve fibers and enteric ganglia, we performed IFC stimulation also in the presence of tetrodotoxin (TTX) or hexamethonium (HXN). We applied the commonly used stimulation modes with a beat frequency sweeping between 5 and 25 Hz (IFC) and 80 and 150 Hz (IFC). We monitored intraluminal pressure and motility by online barometry and video recording, respectively. Motor activity, reflected by changes in the intraluminal pressure (cm HO·s) and longitudinal movements (pixels·s), was quantified over time as root mean squares (RMSs). RESULTS: After IFC, we observed a 30% increase in the rectal motility in the pressure changes which was sustained over 30 minutes post-stimulation ( < 0.02); only a minor effect was detected for IFC. Both TTX and HTX abolished the stimulation. This suggests neuronal modulation. CONCLUSION: IFC stimulates rectal motor activity in the isolated perfused porcine rectum. Ganglia in the enteric nervous system are modulated to allow increased activity for at least 30 minutes. Therefore, the isolated porcine rectum is a suitable tool to study the effectiveness of various IFC settings in the rectum.
Evaluating Inter- and Intraobserver Agreement on Pectus Carinatum Severity and Treatment Outcomes: A Comparison of Subjective and Objective Assessment Methods.
van Braak H, de Beer SA, Zwaveling S … +3 more , Oomen MWN, van Heurn LWE, de Jong JR
Eur J Pediatr Surg · 2025 Jun · PMID 39537133 · Full text
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 afte...
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 < 0.61) interobserver agreement on esthetic results (κ = 0.26, κ = 0.22), severity of PC (κ = 0.43, κ = 0.38), and symmetry (κ = 0.37, surgeons only). Agreement between surgeons and peers on esthetic results (κ = 0.37) and severity before treatment (κ = 0.54) was similarly inadequate. Surgeons and peers demonstrated inadequate intraobserver agreement on esthetic results (κ = 0.49, κ = 0.34), severity of PC (κ = 0.54, κ = 0.48), and symmetry (κ = 0.60, 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 ( = 0.682, = .062) or age ( = 0.205, = .527). CONCLUSIONS: Subjective assessment of PC severity and esthetic results is inconsistent. Three-dimensional 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.
The PPP - Perineal Body Preserving PSARP (Posterior Sagittal Anorectoplasty) for Anorectal Malformation with Rectovestibular Fistula in Females-Report of Early Outcomes.
Xu TO, Ryan JA, Feng C … +3 more , Badillo A, Sandler A, Levitt MA
Eur J Pediatr Surg · 2025 Apr · PMID 39515783 · Publisher ↗
INTRODUCTION: The perineal body preserving posterior sagittal anorectoplasty (PSARP) (PPP) is a novel modification of the original PSARP for female patients with rectovestibular fistulas designed to eliminate the risk o...
INTRODUCTION: The perineal body preserving posterior sagittal anorectoplasty (PSARP) (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 January /2020 and December 2023. Exposure was done through the intended anoplasty only. Perineal body or posterior sagittal incision was not utilized. No routine postoperative dilations were performed. The postoperative outcomes, day of discharge, time to first feeding, and early stooling patterns were assessed. RESULTS: A total of 15 patients underwent a PPP at a median age of 6 months (range 2 days to 19 months) with median follow-up of 11 months (range 1-36). Three (20%) patients underwent repair within the first 3 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 postoperative day 1. Eleven (73%) were discharged on postoperative day 1. All patients were stooling spontaneously at their most recent clinical encounter with 11 (73%) utilizing laxatives. CONCLUSIONS: 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 with the traditional PSARP.
An Analysis Regarding the Ultimate Outcome of Abstracts Presented at the European Paediatric Surgeons' Association Congress.
Gerçel G, Durakbaşa ÇU
Eur J Pediatr Surg · 2025 Feb · PMID 39442555 · Publisher ↗
INTRODUCTION: The objective of this study is to analyze the conversion rate of abstracts presented at the European Paediatric Surgeons' Association (EUPSA) congress into full-text publications and to conduct a thorough...
INTRODUCTION: The objective of this study is to analyze the conversion rate of abstracts presented at the European Paediatric Surgeons' Association (EUPSA) congress into full-text publications and to conduct a thorough analysis of the attributes and quality of the papers published. MATERIALS AND METHODS: Abstract books including the years 2017 to 2022 were reviewed. Searches on PubMed and Google Scholar, utilizing keywords from the titles and the author names, were conducted to trace subsequent full-text publications. A categorical analysis detected variations and trends, with a significance threshold of < 0.05. Quantitative data were presented as means ± standard deviations, whereas categorical data were represented as counts () and percentages (%). RESULTS: A total of 2,139 abstracts were presented at the EUPSA annual meetings during five consecutive congresses. The average number of presented abstracts was 427.6 ± 20.4 per year from across 63 different countries. European countries contributed the majority (71%). The presentations included both oral ( = 817, 38.2%) and poster presentations ( = 1,322, 61.8%). They predominantly focused on clinical topics (90.6%). Single-center retrospective studies were the most common study design (43.7%). Out of all abstracts presented, 1,033 (48.3%) were published within an average time interval of 1.39 ± 1.19 years after presentation. Most journals had an impact factor (IF) between 1 and 5 (74.5%). There was no significant year-to-year variation in publication rates ( = 1). Basic science studies were published in journals with significantly higher IF compared with clinical studies ( < 0.001). CONCLUSIONS: The publication rate of abstracts presented at the EUPSA annual congress stands at 48.3%, aligning with the rates observed in other similar studies. This suggests that abstracts submitted to the EUPSA congresses were evaluated and scored rigorously, adhering to international selection criteria. Furthermore, the majority of these abstracts were published in journals with moderate to high IFs, providing quantitative evidence of the scientific quality of research within the field of pediatric surgery.
Long-Term Orthopaedic and Radiological Outcomes of Symphysis Approximation without Osteotomy in Primary Bladder Exstrophy Repair.
Promm M, Hofbauer R, Brandl R … +6 more , Gossler C, Brandstetter S, Kertai M, Hofmann A, Schnabel MJ, Rösch WH
Eur J Pediatr Surg · 2025 Jun · PMID 39438003 · Publisher ↗
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 indi...
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 ( = 0.419), mobility impairment ( = 0.543), sports impairment ( = 0.543), hip impingement ( = 1.000), leg length discrepancy ( = 0.505), and width of the pubic diastasis as measured by MRI ( = 0.401). There were also no significant differences with regard to CEA right (median 30 degrees, = 0.976), CEA left (median 31.5 degrees, = 0.420), ACA right (median 19 degrees, = 0.382), and ACA left (median 17 degrees, = 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.
Pathological Insights into Non-Neoplastic Renal Parenchyma in Wilms Tumor: Implications for Nephron-Sparing Surgery.
Huang M, Shu M, Xu Z … +7 more , Wang L, Liu L, Liu J, Zhang H, Yang S, Wang C, Gao P
Eur J Pediatr Surg · 2025 Jun · PMID 39424346 · Publisher ↗
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...
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.
The Role of GERD Diagnosis and Treatment in Preventing Lung Function Decline After Pediatric Lung Transplantation.
Garrisi EC, Müller C, Ure B … +3 more , Carlens J, Dingemann J, Schukfeh N
Eur J Pediatr Surg · 2025 Feb · PMID 39419066 · Publisher ↗
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...
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 ( = 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.
ERN eUROGEN Guidelines on the Management of Anorectal Malformations Part I: Diagnostics.
Aubert O, Irvine WFE, Aminoff D … +14 more , de Blaauw I, Cascio S, Cretolle C, Iacobelli BD, Mantzios K, Midrio P, Miserez M, Sarnacki S, Schmiedeke E, Schwarzer N, Sloots C, Stenström P, Lacher M, Gosemann JH
Eur J Pediatr Surg · 2025 Apr · PMID 39393414 · Publisher ↗
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....
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.
ERN eUROGEN Guidelines on the Management of Anorectal Malformations, Part IV: Organization of Care and Communication between Providers.
Aubert O, Irvine W, Aminoff D … +14 more , de Blaauw I, Cascio S, Cretolle C, Iacobelli BD, Lacher M, Mantzios K, Midrio P, Miserez M, Sarnacki S, Schmiedeke E, Sloots C, Stenström P, Schwarzer N, Gosemann JH
Eur J Pediatr Surg · 2025 Apr · PMID 39393413 · Publisher ↗
INTRODUCTION: Being born with an anorectal malformation (ARM) can have profound and lifelong implications for patients and parents. Organization of care and communication between health care providers is an overlooked a...
INTRODUCTION: Being born with an anorectal malformation (ARM) can have profound and lifelong implications for patients and parents. Organization of care and communication between health care providers is an overlooked area of patient care. The European Reference Network eUROGEN for rare and complex urogenital conditions assembled a panel of experts to address these challenges and develop comprehensive guidelines for the management of ARM. METHODS: The Dutch Quality Standard for ARM served as the basis for the development of guidelines. 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 seven 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 all available evidence, expert consensus, and the European context. RESULTS: Aspects pertaining to organization of care, patient/parent/health care provider communication, and referral and collaboration between providers caring for ARM patients were assessed. Two new studies were identified. In total, the panel adapted 12 recommendations, adopted 7, and developed 2 de novo. The overall level of newly found evidence was considered low and most recommendations were based on expert opinion. CONCLUSION: Collaborative care and organization of care are gaining importance in the field of ARM. This guideline gives practical guidance on how to achieve better communication and collaboration between all involved parties, applicable at the European level.
Conservative Management of Necrotizing Enterocolitis in Newborns: Incidence and Management of Intestinal Strictures.
Mena R, Guillén G, Lopez-Fernandez S … +5 more , Martos Rodríguez M, Ruiz CW, Montaner-Ramon A, López M, Molino JA
Eur J Pediatr Surg · 2025 Jun · PMID 39348873 · Publisher ↗
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...
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.
Prevalence, Mortality, and Associated Anomalies in Esophageal Atresia: A Retrospective Study of Finnish Population Data (2004-2017).
Alikärri S, Helenius I, Heiskanen S … +5 more , Syvänen J, Kemppainen T, Löyttyniemi E, Gissler M, Raitio A
Eur J Pediatr Surg · 2025 Jun · PMID 39326446 · Publisher ↗
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 cl...
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 < 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, = 0.35. Neonatal mortality was 5% ( = 15) and 1-year survival was 91.5%. Mortality was associated with syndromic cases ( = 0.002). The Spitz classification predicted neonatal mortality better than cardiac anomalies alone ( < 0.001 and = 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 ( = 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.
Medical Costs of Patients Undergoing Esophageal Atresia Repair are Mainly Influenced by Associated Malformations.
Pardey N, Zeidler J, Blaser J … +4 more , Becker N, Dingemann J, Ure B, Schukfeh N
Eur J Pediatr Surg · 2025 Feb · PMID 39322202 · Publisher ↗
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...
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.
Physical Activity Levels in Children with Esophageal Atresia and Congenital Heart Disease: A Comparative Multicenter Study.
Frankenbach LM, Holler AS, Oetzmann von Sochaczewski C … +10 more , Wessel L, Muensterer OJ, Dingemann J, Widenmann A, Helm P, Siaplaouras J, Bauer U, Apitz C, Niessner C, König TT
Eur J Pediatr Surg · 2025 Jun · PMID 39313207 · Publisher ↗
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 associ...
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 ( = 1,262) and healthy controls ( = 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.
European Reference Network eUROGEN Guidelines on the Management of Anorectal Malformations, Part II: Treatment.
Aubert O, Irvine WFE, Aminoff D … +14 more , de Blaauw I, Cascio S, Cretolle C, Iacobelli BD, Mantzios K, Midrio P, Miserez M, Sarnacki S, Schmiedeke E, Schwarzer N, Sloots C, Stenström P, Lacher M, Gosemann JH
Eur J Pediatr Surg · 2025 Apr · PMID 39299249 · Publisher ↗
INTRODUCTION: Anorectal malformations (ARMs) are rare birth defects affecting the anorectum and oftentimes the genitourinary region. The management of ARM patients is complex and requires highly specialized surgical and...
INTRODUCTION: Anorectal malformations (ARMs) are rare birth defects affecting the anorectum and oftentimes the genitourinary region. The management of ARM patients is complex and requires highly specialized surgical and medical care. The European Reference Network eUROGEN for rare and complex urogenital conditions aimed to develop comprehensive guidelines for the management of ARM applicable on a European level. METHODS: The Dutch Quality Standard for ARM served as the basis for the development of guidelines. 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 seven 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 the current evidence, expert opinion, and the European context. RESULTS: Surgical and medical treatment of ARM, postoperative instructions, toilet training, and management of fecal and urinary incontinence were addressed. Seven new studies were identified. The panel adapted 23 recommendations, adopted 3, and developed 8 de novo. The overall level of newly found evidence was considered low. CONCLUSION: Treatment of ARM patients requires a multidisciplinary team and expertise about anatomical and surgical aspects of the disease, as well as long-term follow-up. This guideline offers recommendations for surgical and medical treatment of ARM and associated complications, according to the best available evidence and applicable on a European level.
ERN eUROGEN Guidelines on the Management of Anorectal Malformations Part III: Lifelong Follow-up and Transition of Care.
Aubert O, Irvine WFE, Aminoff D … +14 more , de Blaauw I, Cascio S, Cretolle C, Iacobelli BD, Lacher M, Mantzios K, Miserez M, Sarnacki S, Schmiedeke E, Schwarzer N, Sloots C, Stenström P, Midrio P, Gosemann JH
Eur J Pediatr Surg · 2025 Apr · PMID 39299248 · Publisher ↗
INTRODUCTION: Anorectal malformations (ARMs) are complex congenital anomalies of the anorectal region, oftentimes also affecting the genitourinary system. Although successful surgical correction can often be achieved in...
INTRODUCTION: Anorectal malformations (ARMs) are complex congenital anomalies of the anorectal region, oftentimes also affecting the genitourinary system. Although successful surgical correction can often be achieved in the neonatal period, many children will experience functional problems in the long term. The European Reference Network for rare and complex urogenital conditions (eUROGEN) assembled a panel of experts to address these challenges and develop comprehensive guidelines for the management of ARM. METHODS: The Dutch Quality Standard for ARM served as the foundation 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 the current evidence and/or expert consensus. RESULTS: Lifelong follow-up, integration, and transition of care were assessed. A total of eight new studies were identified. The panel adapted 18 recommendations, adopted 6, and developed 6 de novo. Overall, the level of evidence was considered low. CONCLUSION: Successful lifelong follow-up and transition of care require a dedicated team of pediatric and adult specialist and an individually tailored patient-centered approach. This guideline summarizes the best available evidence on follow-up of ARM patients and provides guidance for the development of structured transition programs.
A Survey of Preoperative, Perioperative, and Postoperative Management Practices for Testicular Torsion in Pediatric Patients among European Surgeons.
Beher A, Moreno-Alfonso JC, Garnier H … +3 more , Darici D, Salö MJ, Aubert O
Eur J Pediatr Surg · 2025 Feb · PMID 39251202 · Publisher ↗
INTRODUCTION: Management of testicular torsion varies between specialties resulting in lack of standardization. The aim of this survey was to assess pre-, peri-, and postoperative differences. METHODS: An online questi...
INTRODUCTION: Management of testicular torsion varies between specialties resulting in lack of standardization. The aim of this survey was to assess pre-, peri-, and postoperative differences. METHODS: An online questionnaire was distributed in 2023 to general and pediatric surgeons, pediatric urologists, and urologists by the Trainees of European Pediatric Surgery. MAIN RESULTS: Among 88 respondents (92% tertiary referral hospital), 38% always or usually perform sonography in case of suspected torsion respectively. In addition, 15% always attempt manual detorsion and 44% depending on the clinical presentation. Most surgeons (93%) favor a scrotal approach. Interestingly, pediatric surgeons choose a transscrotal while other specialties opt for a midline incision ( = 0.002). The majority (57%) employ a three-point fixation, while 42% opt for a two-point fixation. In case of necrotic testis, 61% puncture the testis with 52% opting for surgical removal, while 33% perform orchiectomy and 6% leave it in situ. Regarding necrotic or borderline-appearing testis, 54% take a biopsy. Furthermore, 82% perform prophylactic orchidopexy of the contralateral side during the same session. Postoperative antibiotics are always administered by 12%, while 52% do so sometimes. Most perform sonographic (58%) or clinical (57%) follow-up 3 months postoperatively. Notably, pediatric surgeons and urologists perform follow-up more frequently themselves, while general surgeons/urologists recommend follow-up by pediatricians ( = 0.002). In addition, 76% of responders affirm adhering to European Association of Urology guidelines. Unawareness of guidelines and institutional practices are reasons most commonly cited for nonadherence. CONCLUSION: Our study reveals significant disparities in the pre-, peri-, and postoperative management of testicular torsion underscoring the need for establishing standardized practices.
Rethinking Fistula Preservation in Anorectal Malformation Surgery: A Histopathological Perspective.
Agarwal P, Roy S, Pandey A … +4 more , Tyagi N, Pant N, Kumar P, Rawat J
Eur J Pediatr Surg · 2025 Feb · PMID 39151926 · Publisher ↗
OBJECTIVE: This study aims to assess the histopathological characteristics of the fistulous tissue in males with anorectal malformation (ARM) to determine its suitability for use in constructing a neoanus. METHODS: Thi...
OBJECTIVE: This study aims to assess the histopathological characteristics of the fistulous tissue in males with anorectal malformation (ARM) to determine its suitability for use in constructing a neoanus. METHODS: This prospective observational study included male ARM patients with recto-urethral fistula. All other types were excluded. The tissue specimens comprised 0.5 to 2.0 cm of the most distal part of the rectal pouch and the fistulous tissue. Hematoxylin and eosin-stained sections were prepared. Histological features, viz. internal sphincter, anal grand and crypts, ganglion cells, presence of subepithelial fibrosis, thickened nerve trunks, and metaplasia, were evaluated. RESULTS: Of 65 patients of ARM admitted, 24 met the inclusion criteria. Gross and microscopic internal sphincter was not found in any sample. The anal mucosa was visualized in all samples; however, crypts were irregular and distorted architecture was seen in 4 (16.67%) samples. Urothelial metaplasia was identified in 10 (41.67%) samples. Adequate ganglion cells were seen only in 6 (25%) samples. Significant submucosal fibrosis was seen in all samples. Thickened nerve trunks were identified in 4 (16.67%) samples. CONCLUSIONS: All normal anal histological features could not be found together in the fistula tissue. With the absence of normal features, such as internal anal sphincter muscles, and the presence of abnormal histopathological features, such as subepithelial fibrosis, thickened nerve trunks, and metaplasia, there are high chances of abnormal bowel function like constipation if the fistulous tissue is used for neoanus construction. It may have an impact on the quality of life of patients.
Analysis of Techniques in Laparoscopic Inguinal Hernia Repairs across Pediatric Age Groups: EUPSA Trainees of European Pediatric Surgery Survey.
Ulman H, Aubert O, Wiernik A … +4 more , Moreno-Alfonso JC, Gazzaneo M, Sterlin A, Saxena AK
Eur J Pediatr Surg · 2025 Feb · PMID 39117315 · Publisher ↗
AIM: This survey analyzed techniques in laparoscopic inguinal hernia repair (LIHR) across pediatric age groups. MATERIALS AND METHODS: Data were collected through an online survey for pediatric surgeons, comprising of...
AIM: This survey analyzed techniques in laparoscopic inguinal hernia repair (LIHR) across pediatric age groups. MATERIALS AND METHODS: Data were collected through an online survey for pediatric surgeons, comprising of 38 questions, conducted by the European Pediatric Surgeons' Association (EUPSA) Trainees of European Pediatric Surgery (TEPS) LIHR Working Group. RESULTS: The survey was completed by 183 surgeons from 22 countries. Seventy-seven percent of respondents had performed LIHR at least once. Regarding preferences about the patient's gender, 7% respondents perform LIHR only in selected females, 9% routinely in females, 15% in both genders with age/weight restrictions, 24% routinely in both genders, 31% in selected cases, and 14% never perform LIHR. Percutaneous internal ring suturing (PIRS) was the preferred technique in all age groups, with totally extraperitoneal and transabdominal preperitoneal repairs preferred by 9% in adolescents. The majority (59%) repaired a contralateral patent processus vaginalis if present. Hydro-dissection (21%) and additional intra-abdominal instruments (42%) were preferred more often for male patients. The distal hernia sac was left intact by most respondents (92%). Responses regarding recurrence rates varied: 40% responded that LIHR had recurrence rates comparable to open surgery, whereas 10% reported increased recurrences and hence limited its use, and 10% consider that slightly increased recurrences are outweighed by lower complication rates associated with laparoscopic methods and thus continue LIHR. CONCLUSIONS: PIRS is the preferred choice for pediatric LIHR. Surgical techniques vary and are influenced by patient gender and age. The survey gives insights into demographics, case selection, and approaches among pediatric surgeons with regard to LIHR.
Comparative Outcomes of Single-Stage versus Two-Stage Laparoscopic Fowler-Stephens Orchidopexy: A Systematic Review snd Meta-Analysis.
Fung ACH, Tsang JTW, Leung L … +2 more , Chan IHY, Wong KKY
Eur J Pediatr Surg · 2025 Feb · PMID 39079708 · Publisher ↗
INTRODUCTION: Intra-abdominal testis (IAT) remains a challenging and controversial subset within the management of cryptorchidism. While Fowler-Stephens orchidopexy (FSO) is still being advocated as the gold standard fo...
INTRODUCTION: Intra-abdominal testis (IAT) remains a challenging and controversial subset within the management of cryptorchidism. While Fowler-Stephens orchidopexy (FSO) is still being advocated as the gold standard for the treatment of this entity, there is new and conflicting evidence on the comparative outcomes between single- or two-stage laparoscopic FSO (LFSO). The aim of the study is to investigate whether staging has benefits in children receiving LFSO. METHODS: We searched the PubMed, Medline, Embase, and Cochrane Trials databases for studies comparing single- with two-stage LFSO in children from January 1, 1995 to December 31, 2023. We assessed the identified studies for quality and performed a systematic review and meta-analysis in accordance with the Preferred Reporting of Systematic Reviews and Meta-Analyses. The main outcome measures examined were success rate (in terms of the scrotal position of the testis) and testicular atrophy, which were analyzed using fixed effect models. RESULTS: We included 17 eligible studies that involved a total of 499 operated testes. The overall success rates of single- and two-stage LFSO were 79.4 and 90.3%, respectively. The overall testicular atrophy rates of single- and two-stage LFSO were 17.3 and 11%, respectively. Fixed effect model analysis showed that two-stage LFSO is significantly superior to single-stage LFSO in overall success rate (odds ratio [OR: 2.57]; 95% confidence interval [CI]: 1.50-4.39, = 0.0006) and testicular atrophy rate (OR: 0.48; 95% CI: 0.28-0.79, = 0.004). There is no heterogeneity in the reports, and the funnel plot showed no publication bias. CONCLUSIONS: Two-stage LFSO remains the first choice of operation for children with a high IAT, with a significantly higher success rate and a lower testicular atrophy rate.
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