Increased Musculoskeletal Deformities And Decreased Lung Volume In Patients After Ea/Tef Repair - A Real-Time Mri Study.

OBJECTIVE: This study aims to assess morphological and functional postoperative changes after open or minimally invasive (MIS) repair of esophageal atresia (EA) compared to healthy controls by thoracic real-time MRI. SUMMARY BACKGROUND DATA: Musculoskeletal deformities and pulmonary morbidity are common in children after EA repair. The real-time MRI is a novel technique that provides ultrafast, high-quality images during spontaneous breathing, without sedation even in young children. METHODS: Children aged 3-18 years were prospectively examined with a 3 Tesla MRI. Musculoskeletal deformities, static thoracic cross-sectional areas (CSA) at three different levels and lung volumes, as well as dynamic right-to-left ratio of CSA of hemithoraces and lung volumes during forced breathing were evaluated. RESULTS: 72 children (42 open, 8 MIS, 22 controls) were recruited. In the EA group, rib fusions and adhesions (78%, P<0.01) and scoliosis (15%, P=0.32) were found after thoracotomy, but not after MIS. Mean right-to-left ratio of CSA and lung volumes were lower after EA repair compared to controls (P <0.05), indicating impaired thoracic and lung development. The number of thoracotomies was a significant risk factor for smaller thoracic volumes (P<0.05). CONCLUSIONS: For the first time, morphological changes and thoracic motility after EA repair were visualized by dynamic real-time MRI. Children after EA repair show decreased right-sided thoracic and lung development compared to controls. Open repair leads to significantly more musculoskeletal deformities. This study emphasizes that musculoskeletal morbidity following a thoracotomy in infancy is high.

Outcomes of detrusorectomy in neurogenic bladders: A systematic review and meta-analysis.

INTRODUCTION: This systematic review and meta-analysis aims to assess the outcomes of detrusorectomy in children with neurogenic bladder (NB). MATERIALS AND METHODS: A search was performed in PUBMED, EMBASE and the Cochrane Library database in August 2023. The following search terms were used: "detrusorectomy", "detrusorotomy", "auto-augmentation". The two main primary outcomes were improvement in bladder capacity and bladder compliance after intervention. Outcomes were dichotomized into favorable and unfavorable. The secondary outcomes were the effect of postoperative bladder cycling on bladder compliance and bladder capacity and complications. RESULTS: 258 references were screened for inclusion, of these 242 were excluded. 8 of the remaining 16 studies were included for the qualitative and quantitative analysis. All studies were retrospective case series studies (165 patients). Median follow-up time varied between studies (1.75-11.1 years), while two studies reported a mean follow-up time of 8.1 years. Using a random effects meta-analysis, the overall rate for unchanged or improved bladder capacity was 95% (Proportion[CI]: 0.95 [0.61; 1.00]). The overall rate for improved bladder compliance after detrusorectomy was 67% (Proportion[CI]: 0.67 [0.26; 0.92)) (Summary Fig). In the bladder cycling group improved compliance was found in 89% of patients (Proportion[CI]: 0.89 [0.41; 0.99]), whereas it was 21% in the non-cycling group (Proportion[CI]: 0.21 [0.04; 0.61]) (p = 0.0552). Bladder cycling did not affect bladder capacity as the overall rate for unchanged or improved bladder capacity was 98% (Proportion[CI]: 0.98 [0.35; 0.92]) in the cycling and 73% (Proportion[CI]: 0.73 [0.46; 0.90]) in the non-cycling group (p > 0.05). Overall complications were encountered in 16 (9.7%) patients, with major complications (stones, bladder perforations) detected only in 5(3%) patients. DISCUSSION: Detrusorectomy leads to an improved bladder compliance in 67% of children. As such, detrusorectomy proves to be a viable procedure to enhance bladder compliance or to cure overactivity. Importantly, the beneficial effect of detrusorectomy on bladder compliance seems to be long-lasting. Bladder capacity remained unchanged or improved in almost all patients undergoing detrusorectomy. Postoperative bladder cycling was effective in improving bladder compliance outcome compared to the non-cycling group. Proper patient selection is the key to good postoperative outcomes. CONCLUSIONS: Detrusorectomy enhances bladder compliance in pediatric neurogenic bladders. Postoperative bladder cycling improves bladder compliance and the overall complication rate of the procedure is low. Therefore, detrusorectomy should be considered a valuable therapeutic option in the comprehensive management of neurogenic bladders in children.

Analysis of Techniques in Laparoscopic Inguinal Hernia Repairs across Pediatric Age Groups: EUPSA Trainees of European Pediatric Surgery Survey.

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.

A Survey of Preoperative, Perioperative, and Postoperative Management Practices for Testicular Torsion in Pediatric Patients among European Surgeons.

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 (p = 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 (p = 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.

ERN eUROGEN Guidelines on the Management of Anorectal Malformations, Part IV: Organization of Care and Communication between Providers.

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.

ERN eUROGEN Guidelines on the Management of Anorectal Malformations Part I: Diagnostics.

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.

European Reference Network eUROGEN Guidelines on the Management of Anorectal Malformations, Part II: Treatment.

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.

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.

European Paediatric Surgeons' Association Survey on the Adherence to EAU/ESPU Guidelines in the Management of Undescended Testes.

INTRODUCTION: The aim of this study was to assess the adherence to the European Association of Urology (EAU)/European Society for Pediatric Urology (ESPU) 2016 guidelines in the management of undescended testes (UDT). MATERIALS AND METHODS: An online questionnaire was sent in 2023 to members of the European Paediatric Surgeons' Association (EUPSA). RESULTS: Among 157 members, 46 and 44% perform orchidopexy before 12 and 18 months, respectively. In total, 92% recommend conservative management of retractile testes and 58% offer close follow-up. In case of nonpalpable testes, 78% favor laparoscopy and 18% ultrasonography. If a peeping testicle is identified at laparoscopy, 76% perform a single-stage orchidopexy. In case of a high testicle, a staged procedure is preferred (84%). Management of blind-ending spermatic vessel is heterogenous with a majority ending the operation, followed by exploration of the inguinal canal and removal of the testicular nubbin with optional fixation of the contralateral testis. Only a minority recommends hormonal therapy to improve fertility potential in bilateral UDT. A majority (59%) discuss testis removal in UDT in postpubertal boys. In addition, 77% declare following the EAU/ESPU guidelines. Unawareness of guidelines was the most common reason cited for nonadherence. International guidelines were found to have the greatest influence on clinical practice; however, personal experience and institutional practice seem to play an important role. CONCLUSION: Most recommendations of the EAU/ESPU guidelines are being followed by EUPSA members; however, personal and institutional practice impact decision making. Hormonal therapy in bilateral UDT, management of vanishing testes, and UDT in postpubertal boys could be improved.

Impact of the COVID-19 Pandemic on Pediatric Surgical Scientific Formats: An ERNICA Survey on Challenges and Future Directions.

INTRODUCTION: The aims of this survey were to assess the experiences of the transition from in-person to virtual meetings among European pediatric surgeons since the beginning of the coronavirus disease 2019 (COVID-19) pandemic and to identify preferences for future formats. MATERIALS AND METHODS: An online questionnaire was circulated among members of the European Reference Network for rare Inherited and Congenital Anomalies Network (ERNICA) in 2022. Two time periods were compared (3 years before the COVID-19 pandemic vs. the year 2021). RESULTS: A total of 87 pediatric surgeons from 16 countries completed the survey. In addition, 27% of respondents were trainees/residents and 73% consultants/lead surgeons. Consultants participated in significantly more in-person congresses prior to the COVID-19 pandemic compared with trainees (5.2 vs. 1.9; p < 0.001). A significant increase in attendance of virtual meeting was reported during 2021 compared with pre-COVID-19 (1.4 vs. 6.7; p < 0.001). Consultants had experienced significantly less absenteeism thanks to virtual meetings compared with trainees (42/61 vs. 8/23; p < 0.05). Most surgeons considered virtual meetings more economic (82%), practical (78%), and family-friendly (66%). However, a majority reported missing social events (78%). The communication among attendees or between attendees and speakers or scientific faculty was deemed worse. A minority (14%) experienced equalized representation of trainees and consultants at virtual meetings. Most respondents (58%) agreed that future meeting strategies should focus on offering virtual formats. Regarding future congresses, respondents prefer hybrid (62%) over in-person (33%) or virtual (6%) formats. CONCLUSION: According to European pediatric surgeons, virtual learning formats hold multiple advantages and should be continued. Improved technology must meet the challenges, particularly to enhance communication, equal representation, and networking between attendees.

Writing a Case Report in Pediatric Surgery: A Comprehensive Guideline.

An effective case report delivers clear and valuable clinical or surgical information to the medical community. Case reports dealing with pediatric surgical issues raise the medical community's awareness of rare diseases, unusual presentations of common disorders, or novel surgical or nonsurgical treatment approaches. Thus, case reports contribute substantially to medical advance by sharing remarkable or unexpected findings. For this reason, case reports should be prepared with vigilance, and current conventions on good medial writing practice should be observed. This guideline aims to assist clinicians and surgeons in the successful publishing of a compelling case report in pediatric surgery that is read and understood by the intended audience.

Resection of an Extraovarian Noniuteinized Thecoma in a 14-Year-Old Girl.

Thecomas are rare benign sex cord-stromal tumors that account for less than 1% of all ovarian tumors. They usually affect postmenopausal women and become symptomatic with abnormal bleeding. In adolescents, less than 10 cases have been reported so far, mainly with symptoms of hormonal disbalance. Extraovarian thecomas represent an even rarer entity, with only two cases described so far, none of them in the pediatric population. We report the case of a 14-year-old girl who presented with sudden-onset abdominal pain, dysuria, and fever, as well as highly elevated serum inflammation parameters. Ultrasound and magnetic resonance imaging (MRI) revealed a large, inhomogeneous pelvic mass (16 cm × 9 cm × 13 cm) with indistinct margins, suggestive of an infiltrative malignant teratoma or sarcoma. Laparoscopy confirmed a large mass of unknown origin. In contrast to the infiltrative character seen on preoperative MRI, the tumor could be easily exteriorized and resected after conversion to laparotomy. Ovaries, fallopian tubes, and uterus remained unaffected. Histopathology revealed a benign nonluteinized thecoma. The postoperative course and 19-month follow-up were uneventful.

SmartPAN: In vitro and in vivo proof-of-safety assessments for an intra-operative predictive indicator of postoperative pancreatic fistula.

Pancreatic surgery is complicated by untreated fluid leakage, but no tenable techniques exist to detect and close leakage sites during surgery. A novel hydrogel called SmartPAN has been developed to meet this need and is here assessed for safety before trials on human patients. First, resazurin assays were used to test the cytotoxic effects of SmartPAN's active bromothymol blue (BTB) indicator and its solution of phosphate-buffered saline (PBS) on normal (HPDE: human pancreatic duct epithelial) or carcinomic (FAMPAC) human pancreatic cells. Cells incubated with BTB showed no significant reduction in cell viability below threshold safety levels. However, PBS had a mild cytotoxic effect on FAMPAC cells. Second, SmartPAN's pathological effects were evaluated in vivo by applying 4-ml SmartPAN to a porcine (Sus scrofa domesticus) model of pancreatic resection. There were no significant differences in macroscopic and microscopic pathologies between pigs treated with SmartPAN or saline. Third, measurements using HPLC-MS/MS demonstrate that BTB does not cross into the bloodstream and was eliminated from the body within 2 days of surgery. Overall, SmartPAN appears safe in the short term and ready for first-in-human trials because its components are either biocompatible or quickly neutralized by dilution and drainage.

Virtual Education in Pediatric Surgery during the COVID-19 Era: Facing and Overcoming Current Challenges.

The novel coronavirus disease 2019 (COVID-19) pandemic has impacted our way of living in an unprecedented manner. Medical professionals at all levels have been forced to adapt to the novel virus. The delivery of surgical services and the subsequent learning opportunities for surgical residents have especially been disrupted and the pediatric surgical community has not been exempted by this. This article highlights the challenges imposed by the pandemic and outlines the various learning modalities that can be implemented to ensure continued learning opportunities throughout the pandemic and beyond. Furthermore, it aims to show how the utilization and expansion of technologies maintain and further increase the communication, as well as the exchange of and access to knowledge among peers. Virtual education-, application-, and simulation-based learning and social media, as well as telemedicine and online conferences, will play a considerable role in the future of surgical specialties and surgical education.

SmartPAN: A novel polysaccharide-microsphere-based surgical indicator of pancreatic leakage.

Postoperative pancreatic fistula is a major surgical complication that can follow pancreatic resection. Postoperative pancreatic fistula can develop as a consequence of leaking pancreatic fluid, which calls for an intraoperative indicator of leakage. But suitable indicators of pancreatic leakage have yet to be found. This study details the evidence-based development and early efficacy assessments of a novel pancreatic leakage indicator (SmartPAN), following the IDEAL framework of product development. We developed 41 SmartPAN prototypes by combining indicators of pancreatic fluid with a polysaccharide-microsphere matrix. The prototypes were assessed in vitro using porcine (Sus scrofa domesticus) pancreatic tissue and ex vivo with human pancreatic fluid. From these initial tests, we chose a hydrogel-based compound that uses the pH indicator bromothymol blue to detect alkali pancreatic fluid. This prototype was then assessed in vivo for usability, effectiveness and reliability using a porcine model. Treatment groups were defined by SmartPAN-reaction at initial pancreatic resection: indicator-positive or negative. Indicator-positive individuals randomly received either targeted closure of leakage sites or no further closure. We assessed SmartPAN's reliability and effectiveness by monitoring abdominal drainage for amylase and with relaparotomy after 48 h. SmartPAN responses were consistent between both surgical procedures and conformed to amylase measurements. In conclusion, we have developed the first surgery-ready indicator for predicting the occurrence of pancreatic leakage during pancreatic resection. SmartPAN can enable targeted prophylactic closure in a simple and reliable way, and thus may reduce the impact of postoperative pancreatic fistula by guiding peri- and post-operative management.