European Journal of Pediatric Surgery最新文献

Objective:  A novel modification of the cutback procedure, a posterior rectal advancement anoplasty (PRAA) for select male patients with an anorectal malformation and a rectoperineal fistula was recently described, which incised only within the limits of the sphincteric ellipse and eliminated an anterior rectal dissection and thus avoiding any possibility of a urethral injury. This report provides longer-term postoperative outcomes after PRAA.

Method:  A retrospective, single-institution study was performed examining male patients with a rectoperineal fistula between January 2020 and December 2023. PRAA was done only if the rectoperineal fistula was located within the anterior extent of the sphincteric ellipse, which was true for all patients encountered during this study period. We assessed postoperative outcomes, length of stay, time to first feeding, and early stooling patterns.

Results:  Eighteen patients underwent PRAA at a median age of 5.4 months (range 1 day-8 months) with a median follow-up of 14 months (range 4-40). Seven patients (39%) were repaired within the first month of life. Thirteen (72%) were repaired primarily and five (28%) had a diverting ostomy placed before referral. There were no instances of wound dehiscence, rectal prolapse, or urethral injury. Two (11%) patients developed an anal stricture requiring dilation or Heineke-Mikulicz anoplasty. All patients resumed feeds on postoperative day one. Median discharge was on postoperative day one (range 1-6). Fifteen (83%) were utilizing laxatives at their most recent follow-up.

Conclusion:  The PRAA avoids any potential urethral injury or perineal dehiscence has a low rate of anal stenosis (11%) and appears to be applicable to all male patients with a rectoperineal fistula. The technique allows for early return to diet and discharge and can be safely done in the neonatal period or in a delayed fashion without the need for a stoma.

Level of evidence:  Level 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 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.

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.

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.

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.

Background:  Our objective was to explore the treatment preferences for bronchopulmonary sequestration (BPS) among an international group of specialized caregivers.

Methods:  Sixty-three participants from 17 countries completed an online survey concerning the diagnostics, treatment, and follow-up. Recruitment took place among members of the Collaborative Neonatal Network for the first European Congenital Pulmonary Airway Malformation Trial Consortium and through the Association for European Pediatric and Congenital Cardiology working group database.

Results:  Most of the 63 participants were pediatric surgeons (52%), followed by pediatric pulmonologists (22%), and pediatric cardiologists (19%). The majority (65%) treated more than five cases per year and 52% standardly discussed treatment in a multidisciplinary team. Half of the participants (52%) based the management on the presence of symptoms, versus 32% on the intralobar or extralobar lesion localization. Centers with both surgical and interventional cardiac/radiological facilities (85%) preferred resection to embolization in symptomatic cases (62 vs. 15%). In asymptomatic cases too, resection was preferred over embolization (38 vs. 9%); 32% preferred noninterventional treatment, while 11% varied in preference. These treatment preferences were significantly different between surgeons and nonsurgeons (p < 0.05). Little agreement was observed in the preferred timing of intervention as also for the duration of follow-up.

Conclusions:  This survey demonstrates a variation in management strategies of BPS, reflecting different specialist expertise. Most centers treat only a handful of cases per year and follow-up is not standardized. Therefore, management discussion within a multidisciplinary team is recommended. Recording patient data in an international registry for the comparison of management strategies and outcomes could support the development of future guidelines.

Level of evidence: Level IV.

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:  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.