Efficacy and Safety of Tacrolimus or Infliximab Therapy in Children and Young Adults With Acute Severe Colitis.

INTRODUCTION: One-third of children and young adults admitted for management of acute severe colitis (ASC) fail intravenous corticosteroids. Infliximab (IFX) or tacrolimus (TAC) is often used to prevent urgent colectomy in these patients. However, no prior studies have reviewed the outcome of pediatric patients with ASC who were treated with either IFX or TAC. METHODS: We retrospectively identified 170 pediatric patients with ASC admitted to our institution who did not respond to intravenous corticosteroids and were subsequently treated with either IFX or TAC. We compared 6-month colectomy rates, time to colectomy, improvement in disease activity indices, and adverse effects. RESULTS: The mean age of patients in the IFX (n = 84) and TAC (n = 86) groups were 14 and 13.8 years, respectively. The median study follow-up time was 23 months. The rate of colectomy 6 months from rescue therapy was similar whether patients received IFX or TAC (22.6% vs 26.7%, respectively, P = 0.53). The mean decline in Pediatric Ulcerative Colitis Activity Index scores from admission to discharge in those treated with IFX (31.9) or TAC (29.8) was similar (P = 0.63). Three patients treated with IFX experienced infusion reactions. Six patients treated with TAC experienced changes in renal function or electrolytes, and 4 patients reported neurologic symptoms. CONCLUSIONS: There were no significant differences in the likelihood of colectomy 6 months after initiating IFX or TAC rescue therapy. Efficacy of both agents was comparable. The types of adverse effects differed by therapy. These data support the use of either TAC or IFX in children with ASC refractory to intravenous corticosteroids.

Serrated Polyposis Syndrome in a Young Adolescent Patient.

Serrated polyps are pathological neoplastic lesions in the colon with subtle gross morphology leading to underreporting during colonoscopy. While detection rates are increasing in average-risk adult screening colonoscopy, the rate of detection during pediatric colonoscopy is unknown. Serrated polyposis syndrome is characterized by the presence of multiple serrated polyps in the colon and an increased risk of developing colorectal cancer. Cancer prevention relies on early recognition, endoscopic clearance of all polyps > 5 mm, and continued interval surveillance or prophylactic colectomy. We report the diagnosis and management of serrated polyposis syndrome in a young adolescent patient and highlight the subtle features of serrated polyps that may go unrecognized leading to underreporting in childhood.

Triple gallbladder with heterotopic gastric mucosa: a case report.

BACKGROUND: Triple gallbladder is a rare congenital anomaly of the biliary tract that can be associated with heterotopic tissue. Gallbladder triplication results from the failure of rudimentary bile ducts to regress during embryological development, and can be difficult to distinguish from Todani type II choledochal cysts and biliary duplication cysts. CASE PRESENTATION: A 2-year-old patient presented to our institution with intermittent abdominal pain for 1 year. She had elevated transaminases with imaging concerning for a choledochal cyst. After assessment with magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography, she was diagnosed with a gallbladder multiplication and a common bile duct stricture. She underwent laparoscopic cholecystectomy, which confirmed the diagnosis of triple gallbladder. One of the three gallbladders demonstrated heterotopic gastric mucosa on final pathology, including at the cystic duct margin. Follow up testing with a technetium 99 m scan demonstrated a subtle focus of increased activity in the right upper abdomen at the expected location of the common bile duct, concerning for the presence of residual gastric mucosa. The patient remains well without abdominal pain. CONCLUSIONS: We describe the first case of heterotopic gastric mucosa in a triple gallbladder in a young patient presenting with chronic abdominal pain. We also demonstrate the safety and feasibility of laparoscopic cholecystectomy in young children with triple gallbladder. Finally, we propose an interdisciplinary approach to the management of common bile duct strictures in the setting of ectopic acid secretion, involving a combination of medical management, endoscopic intervention, and possible salvage laparoscopic Roux-en-Y hepaticojejunostomy.

Safety of delayed surgical repair of omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex in infants with significant comorbidities.

PURPOSE: Management of infants with OEIS complex is challenging and not standardized. Expeditious surgery after birth has been recommended to limit soilage of the urinary tract and optimize intestinal function. However, clinical instability secondary to comorbidities is common in this population and early operation carries risk. We sought to define the risk/benefit profile of delaying repair. METHODS: All newborn patients with OEIS managed by our institution between Sep 2017 and Oct 2019 were reviewed. Comorbidities were evaluated, including cardiopulmonary pathologies and associated malformations. RESULTS: Ten patients with OEIS were managed. Patients underwent early (2 patients, repair at 0-2 days) or delayed (6 patients, repair at 6-87 days) first-stage exstrophy repair. Two patients died prior to repair (progressive respiratory failure, severe genetic anomalies). Repairs were delayed secondary to cardiac conditions, neurosurgical interventions, medical disease, and/or delayed transfer. Delayed repair patients had longer lengths of stay and use of parenteral nutrition. No patients experienced urinary tract infections prior to repair. CONCLUSIONS: Delaying first-stage exstrophy repair to allow physiologic optimization is safe. All repaired patients were discharged home, without parenteral nutrition or supplemental oxygen.

Air contrast enema reduction of single and recurrent ileocolic intussusceptions in children: patterns, management and outcomes.

BACKGROUND: There is no consensus as to when surgical intervention should be considered for recurrent ileocolic intussusceptions in a stable patient after previous successful air contrast enema. OBJECTIVE: To review the patterns of ileocolic intussusceptions, air contrast enema success rates, and pathologic lead point rates in patients with and without recurrence to evaluate whether treatment outcomes depend on the number and timing between episodes. MATERIALS AND METHODS: We retrospectively reviewed 683 children with air contrast enema performed for ileocolic intussusception between January 2000 and May 2018. Recurrent intussusceptions were separated into mutually exclusive categories: short-term only (≤7 days between episodes) and long-term (>7 days between episodes) intussusceptions. Long-term recurrences included both long-term only and long- and short-term intussusceptions. RESULTS: Of the 683 patients, 606 (89%) had at least 1 successful air contrast enema. Of the 606, 115 (19%) had recurrent intussusceptions after successful reduction. The air contrast enema success rate for a single intussusception was 86% (491/568) and for recurrent intussusceptions was 96% (110/115) (P=0.004). Single and recurrent intussusceptions had similar pathologic lead point rates (3.5% vs. 4.3%; P=0.593). Short-term and long-term recurrences did not differ in air contrast enema success rates (96% vs. 95%). Long-term recurrences had higher pathologic lead point rate compared to short-term only (13% vs. 0%; P=0.003). Of short-term recurrences, 99% (76/77) were ≤5 intussusceptions; 92% had successful air contrast enema without surgery. CONCLUSION: The majority of recurrent intussusceptions were successfully treated by air contrast enema. Short-term recurrences have lower pathologic lead point rates, suggesting that a higher surgical threshold may be plausible relative to long-term recurrences. In the appropriate clinical context, repeat air contrast enemas are a safe option for short-term recurrences, which can be attempted at least five times, potentially precluding the need for surgical intervention.

Technique and perioperative management of left pneumonectomy in neonatal piglets.

BACKGROUND: Although commonly performed in adult swine, unilateral pneumonectomy in piglets requires significant modifications in the surgical approach and perioperative care because of their smaller size and limited physiological reserve. METHODS: Nineteen neonatal piglets underwent a left pneumonectomy. They were allowed 5-7 d of preoperative acclimation and nutritional optimization. Preoperative weight gain and laboratory values were obtained before the time of surgery. A "ventro-cranial" approach is adopted where components of the pulmonary hilum were sequentially identified and ligated, starting from the most ventral and cranial structure, the superior pulmonary vein. The principle of gentle ventilation was followed throughout the entire operation. RESULTS: The median age of the piglets at the time of surgery was 12 (10-12) d. The median preoperative weight gain and albumin level were 20% (16-26%) and 2.3 (2.1-2.4) g/dL, respectively. The median operative time was 59 (50-70) min. Five of the first nine piglets died from complications, two from poor preoperative nutritional optimization (both with <10% weight gain and 2 g/dL for albumin), one from an intubation complication, one from intra-operative bleeding, and one in the postoperative period from a ruptured bulla. No mortality occurred for the next 10 cases. CONCLUSIONS: Successful outcomes for unilateral pneumonectomy in piglets require special attention to preoperative nutritional optimization, gentle ventilation, and meticulous surgical dissection. Preoperative weight gain and albumin levels should be used to identify appropriate surgical candidates. The "ventro-cranial" approach allows for a technically straightforward completion of the procedure.

Surgical Management of Crohn Disease in Children: Guidelines From the Paediatric IBD Porto Group of ESPGHAN.

The incidence of Crohn disease (CD) has been increasing and surgery needs to be contemplated in a substantial number of cases. The relevant advent of biological treatment has changed but not eliminated the need for surgery in many patients. Despite previous publications on the indications for surgery in CD, there was a need for a comprehensive review of existing evidence on the role of elective surgery and options in pediatric patients affected with CD. We present an expert opinion and critical review of the literature to provide evidence-based guidance to manage these patients. Indications, surgical options, risk factors, and medications in pre- and perioperative period are reviewed in the light of available evidence. Risks and benefits of surgical options are addressed. An algorithm is proposed for the management of postsurgery monitoring, timing for follow-up endoscopy, and treatment options.

Defining Practice Readiness in Pediatric Surgery Fellowship: A Qualitative Analysis.

INTRODUCTION: While competency-based training is at the forefront of educational innovation in General Surgery, Pediatric Surgery training programs should not wait for downstream changes. There is currently no consensus on what it means for a pediatric surgery fellow to be "practice-ready". In this study, we aimed to provide a framework for better defining competency and practice readiness in a way that can support the Milestones system and allow for improved assessment of pediatric surgery fellows. METHODS: For this exploratory qualitative study, we developed an interview guide with nine questions focused on how faculty recognize competency and advance autonomy among pediatric surgery fellows. Demographic information was collected using an anonymous online survey platform. We iteratively reviewed data from each interview to ensure adequate information power was achieved to answer the research question. We used inductive reasoning and thematic analysis to determine appropriate codes. Additionally, the Dreyfus model was used as a framework to guide interpretation and contextualize the responses. Through this method, we generated common themes. RESULTS: A total of 19 pediatric surgeons were interviewed. We identified four major themes from 127 codes that practicing pediatric surgeons associate with practice-readiness of a fellow: skill-based competency, the recognition and benefits of struggle, developing expertise and facilitating autonomy, and difficulties in variability of evaluation. While variability in evaluation is not typically included in the concept of practice readiness, assessment and evaluation were described by study participants as essential aspects of how practicing pediatric surgeons perceive practice readiness and competency in pediatric surgery fellows. Competency was further divided into interpersonal versus technical skills. Sub-themes within struggle included personal and professional struggle, benefits of struggle and how to identify and assist those who are struggling. Autonomy was commonly stated as variable based on the attending. CONCLUSION: Our analysis yielded several themes associated with practice readiness of pediatric surgery fellows. We aim to further refine our list of themes using the Dreyfus Model as our interpretive framework and establish consensus amongst the community of pediatric surgeons in order to define competency and key elements that make a fellow practice-ready. Further work will then focus on establishing assessment metrics and educational interventions directed at achieving such key elements.

Malrotation: Management of Disorders of Gut Rotation for the General Surgeon.

In this article, we aim to provide the general surgeon with a clinical blueprint to navigate disorders of gut rotation. We emphasize that bilious emesis in a newborn is malrotation with volvulus until proven otherwise. Although an upper GI series can establish the diagnosis, surgical intervention should not be delayed until the child is ill-appearing. Following detorsion, the key steps are to broaden the mesentery, fully Kocherize the duodenum, and mobilize the cecum. If nonviable bowel is encountered, the principles of damage control can be applied to children. Every effort should be made to preserve bowel length.

Hindgut Duplication in an Infant with Omphalocele-Exstrophy-Imperforate Anus-Spinal Defects (OEIS) Complex.

Introduction The congenital anomaly of omphalocele, cloacal exstrophy, imperforate anus, and spinal abnormalities (OEIS complex) is rare but well recognized. Hindgut duplications are also uncommon and are not known to be associated with OEIS. We describe a neonate with OEIS who was found to have fully duplicated blind-ending hindguts. Case Report A premature infant boy with OEIS underwent first-stage closure on day of life 6, which included excision of the omphalocele sac, separation of the cecal plate and bladder halves, tubularization of the cecal plate, hindgut rescue with end colostomy, and joining of the bladder halves. Cecal plate inspection revealed two hindgut structures that descended distally, one descended midline into the pelvis along the sacrum and the second laterally along the left border of the sacrum. Both lumens connected to the cecal plate and had separate mesenteries. In an effort to maximize the colonic mucosal surface area, the hindgut segments were unified through a side-to-side anastomosis, creating a larger caliber hindgut. The cecal plate was tubularized and an end colostomy was created. Bowel function returned and he was discharged home on full enteral feeds. Discussion This case represents a cooccurrence of two extremely rare and complex congenital anomalies. The decision to unify the distinct hindguts into a single lumen was made in an effort to combine the goals of management for both OEIS and alimentary duplications. The hindgut is abnormal in OEIS and should be assessed carefully during repair.

Combined en bloc liver-double kidney transplantation in an infant with IVC thrombosis.

We report a case of a pediatric en bloc liver-double kidney transplant in a patient with IVC thrombosis below the renal veins. The patient is an 11-month-old girl diagnosed with congenital nephrotic syndrome at two months of age. Multifocal liver masses were identified on routine ultrasound at eight months of age. Alpha fetoprotein level was 55 319. Biopsy confirmed hepatoblastoma. CT scan confirmed multiple lesions in both lobes, which would require liver transplantation for resection. She was also found to have thrombosis of her infrarenal IVC secondary to multiple central lines. She was listed for combined liver-kidney transplant and began chemotherapy. After four cycles of chemotherapy, she underwent bilateral nephrectomies followed by a combined en bloc liver-double kidney transplant from a size matched donor. In order to provide adequate venous outflow from the kidneys in the absence of a recipient infrarenal IVC, the donor liver and kidneys were procured en bloc with a common arterial inflow via the infrarenal aorta and common outflow via the suprahepatic IVC. Kidney transplantation in the absence of adequate recipient venous drainage may require unusual vascular reconstruction techniques. This case demonstrates a novel approach in patients who may require combined liver-kidney transplantation.

Quality of dictated feedback associated with SIMPL operative assessments of pediatric surgical trainees.

BACKGROUND: SIMPL is a workplace-based operative performance assessment tool which allows for dictated feedback (DF). To better understand the value of DF, we sought to characterize the type and quality of DF generated during SIMPL evaluations. METHODS: Thematic analysis of DF from SIMPL assessments between June 2017 and December 2018 at a single pediatric surgery fellowship program was performed. Comments were categorized as specific, encouraging or corrective. Categories were combined to determine DF quality as effective, mediocre or ineffective. RESULTS: Of 781 SIMPL assessments (21 faculty, 5 trainees), 451 (57%) had DF. Most comments were encouraging (93%) and specific (65%). Only 21% were corrective, 17% had entrustment features, and 8% had an explicit learning plan. Feedback quality was deemed mediocre (45%), ineffective (33%) and effective (21%). CONCLUSION: SIMPL dictated feedback was mostly encouraging and specific. To improve quality, feedback should incorporate learning plans as well as corrective and entrustment features.

Aligning Family-Clinician Expectations During Pediatric Surgical Informed Consent: Development and Implementation of an Innovative Communication Skills Workshop.

INTRODUCTION: Aligning expectations during the informed consent process before a child's surgery is an important element of good communication that benefits both surgical staff and families. We developed and evaluated a 2-hour pilot interprofessional workshop to improve the communication and relational skills of pediatric surgeons and nurse practitioners. METHODS: Focus groups with families identified key challenges in the process of informed consent. An interprofessional team, including parents whose children had experienced complex surgeries, developed the workshop collaboratively. A realistic simulation with professional actors portraying parents allowed surgical staff to practice communication skills and receive feedback about the parent perspective. Participants completed a postworkshop evaluation to determine whether the workshop met its objectives and whether they would change practice. RESULTS: Five key themes identified for the workshop included customize communication; align expectations; share clinical uncertainty; recognize/attend to emotions; and identify team members. Thirty-five clinicians participated in a workshop, and 89% completed evaluations. Three-quarters reported the learning to be valuable, and 64% were likely to change practice. Eighty-seven percent would recommend the workshop to other colleagues, and 58 to 74% felt more prepared to achieve each of eight specific skills. DISCUSSION: An innovative workshop for pediatric surgical practitioners to align family-clinician expectations can help improve clinician communication skills and comfort with informed consent. Keys to workshop development included involving parents to identify themes and participate as workshop co-faculty; enlisting leadership and recruiting surgical champions; and using pre-existing meetings to ease scheduling challenges of busy practitioners. Booster sessions may facilitate the desired cultural changes.

A Surgical View of Anatomy: Perspectives from Students and Instructors.

As curricular emphasis on anatomy in undergraduate medical education continues to evolve, new approaches to anatomical education are urgently needed to prepare medical students for residency. A surgical anatomy class was designed for third- and fourth-year medical students to explore important anatomical relationships by performing realistic surgical procedures on anatomical donors. Under the guidance of both surgeons and anatomists, students in this month-long elective course explored key anatomical relationships through performing surgical approaches, with the secondary benefit of practicing basic surgical techniques. Procedures, such as left nephrectomy, first rib resection for thoracic outlet syndrome, and carotid endarterectomy, were adapted from those used clinically by multiple surgical subspecialties. This viewpoint commentary highlights perspectives from students and instructors that suggest the value of a surgical approach to anatomical education for medical students preparing for procedure-oriented residencies, with the goals of: (1) describing the elective at the authors' institution, (2) promoting similar efforts across different institutions, and (3) encouraging future qualitative and quantitative studies of similar pedagogic efforts.

Pulmonary manifestations of immune dysregulation in CTLA-4 haploinsufficiency and LRBA deficiency.

OBJECTIVE: The primary immunodeficiency syndromes of cytotoxic T lymphocyte-associated protein 4 (CTLA-4) haploinsufficiency and lipopolysaccharide-responsive and beige-like anchor protein (LRBA) deficiency present with multisystem immune dysregulation. The aim of this study was to characterize and compare the pulmonary manifestations of these two diseases. METHODS: We retrospectively analyzed the pulmonary clinical, radiologic, and histopathologic characteristics of six patients with CTLA-4 haploinsufficiency and four patients with LRBA deficiency with pulmonary involvement followed at a large tertiary care center. RESULTS: Chronic respiratory symptoms were more frequent in patients with LRBA deficiency versus CTLA-4 haploinsufficiency (3/4 vs. 1/6). Cough was the most common respiratory symptom. Abnormalities in pulmonary exam and pulmonary function testing were more frequent in LRBA deficiency (4/4, 2/4) compared to CTLA-4 haploinsufficiency (1/6, 2/6). Chest computed tomography (CT) findings included mediastinal lymphadenopathy (4/4 in LRBA deficiency vs. 1/4 in CTLA-4 haploinsufficiency), pulmonary nodules (4/4, 3/4), ground-glass opacification (4/4, 3/4), and bronchiectasis (3/4, 1/4). Lymphocytic inflammation, concentrated bronchovasculocentrically and paraseptally, was the predominant pathologic finding and was observed in all patients who had lung biopsies (N = 3 with LRBA deficiency; N = 3 with CTLA-4 haploinsufficiency). CONCLUSION: Despite phenotypic overlap amongst these diseases, LRBA deficiency demonstrated greater severity of pulmonary disease, indicated by respiratory symptoms, pulmonary exam, and intrathoracic radiologic findings. Chest CT was the most sensitive indicator of pulmonary involvement in both disorders. Lymphocytic inflammation is the key histologic feature of both disorders. Pediatric pulmonologists should consider these disorders of immune dysregulation in the relevant clinical context to provide earlier diagnosis, comprehensive pulmonary evaluation and treatment.

A risk adjustment method for newborns undergoing noncardiac surgery.

OBJECTIVE: To develop a risk adjustment method for in-hospital mortality in newborns undergoing noncardiac surgery. SUMMARY OF BACKGROUND DATA: Understanding variation in outcomes is critical to guide quality improvement. Reliable outcome assessments need risk adjustment to allow comparisons. METHODS: Infants or=20 cases in the data set were placed into 4 risk categories by in-hospital mortality rates. Clinical variables were added to the model to better predict mortality; areas under the receiver-operator characteristic (ROC) curves were compared. The final model was validated in the KID 2003 database. RESULTS: Among 6103 eligible cases in the KID 2000, 5117 (83.8%) could be assigned to a risk category. Mortality rates were 0.2% in risk category 1, 2.5% in category 2, 6.4% in 3, and 18.4% in 4. The odds of mortality increased in each risk category relative to category 1 (P < 0.001 for each). In multivariable models adjusting for risk category, the clinical variables most predictive of in-hospital death were serious respiratory conditions and necrotizing enterocolitis. The area under the ROC curve for the full model including clinical risk factors was 0.92 in the KID 2000. The model was validated using data for KID 2003 and showed excellent discrimination (ROC = 0.90). CONCLUSION: This validated method provides a means of risk adjustment in groups of newborns undergoing noncardiac surgery, and should allow for comparative analyses of in-hospital mortality.

Prospective study of health-related quality of life and restorative proctocolectomy in children.

PURPOSE: Although restorative proctocolectomy has become the standard surgical treatment for ulcerative colitis and familial adenomatous polyposis, there are no prospective studies in children of the impact of this intervention on health-related quality of life. METHODS: A prospective study of health-related quality of life in children with ulcerative colitis or familial adenomatous polyposis undergoing restorative proctocolectomy was performed. Patients and their parents who agreed to participate completed standardized health-related quality-of-life surveys (Medical Outcomes Study Short Form-36 and Child Health Questionnaire Parent Form) within one month before colectomy and approximately one year after completion of their surgery. RESULTS: Of the 60 patients who agreed to participate, 44 completed surveys at the appropriate time periods allowing comparison. The parents of 28 of these subjects also completed paired surveys. Before colectomy, patients with ulcerative colitis had substantially lower health-related quality-of-life scores, which were also mirrored in parental surveys. Following surgery patients with ulcerative colitis had significant improvement in 7 of 8 Medical Outcomes Study Short Form-36 patient subscales and all 6 corresponding Child Health Questionnaire Parent Form parental subscales of health-related quality of life. Patients with familial adenomatous polyposis showed improvement in the bodily pain subscale alone, whereas their corresponding parental surveys only showed improvement in the mental health subscale. CONCLUSION: There are clearly significant adverse affects on health-related quality of life in children with ulcerative colitis that dramatically improved following restorative proctocolectomy.

Assessment of Operative Autonomy and Readiness for Independent Practice Among Pediatric Surgery Fellows.

PURPOSE: The purpose of this study was to evaluate the implementation of the System for Improving and Measuring Procedural Learning (SIMPL) "app" in a pediatric surgery training program. SIMPL is a novel workplace-based operative performance assessment platform which allows case by case assessment of trainees using three scales (autonomy, performance, and case complexity) and takes seconds to complete. METHODS: A pediatric surgery-specific procedural taxonomy for SIMPL was developed from the ACGME procedural library. SIMPL was piloted and implemented in a single pediatric surgery training program. A descriptive analysis of the operative assessments was undertaken. RESULTS: In the year following SIMPL implementation, 565 (39% of cases) assessments were completed by 21 faculty for 3 fellows for 148 unique procedures within a median of 8.5 h. Recorded feedback accompanied 61% of assessments. 2nd year fellows were more likely to be deemed autonomous (81% ratings) and practice ready (84%) vs. 1st year fellows (50%; p < 0.001 both), with improvements over time. CONCLUSION: Within pediatric surgery, this is the first implementation of a workplace-based operative performance assessment "app". With its ease of use, SIMPL drastically increased the volume of operative evaluations and diversified the mix of cases evaluated while SIMPL ratings demonstrated expected and graduated performance improvements over time. TYPE OF STUDY: Education. LEVEL OF EVIDENCE: N/A.

Analyzing a follow-up needs assessment: Increased use of internet-based APSA educational programs by pediatric surgeons.

PURPOSE: Needs assessment is a critical component of educational program design. Follow-up is important for improvement. Two electronic educational programs, Exam-based Pediatric surgery Educational Reference Tool (ExPERT) and Pediatric Surgery Not a Textbook (NaT), offered by the American Pediatric Surgical Association (APSA) have been functional for over three years, allowing for follow-up assessment. METHODS: A 22-question survey was distributed via email to APSA members. Questions included practice demographics, learning preferences and APSA material use. Mann-Whitney analysis was performed (p<0.05). RESULTS: 294 members responded. 43% were in academic practice with a pediatric surgery fellowship. Top preferences for obtaining/maintaining medical knowledge were national meetings (27%), ExPERT (24%), and the NaT (20%). Comparatively, in a 2014 assessment, electronic programs were less desired (16%). Cost was cited by >1/3 for not subscribing to ExPERT or NaT. Question discussions were often read regardless of response. >86% would subscribe to APSA resources if there were no CME requirement. The most frequently cited knowledge gap was fetal therapy (30%). CONCLUSIONS: This is the first publication documenting increased acceptance of electronic educational platforms for pediatric surgeons. Well-utilized and valued, the data justify and encourage continued development of electronic educational resources. Room for improvement exists in affordability, knowledge gaps, and individualizing curriculum development. LEVEL OF EVIDENCE: IV.

Restorative proctocolectomy and ileal pouch-anal anastomosis in children.

PURPOSE: This study was designed to evaluate the results of restorative proctocolectomy with distal rectal mucosectomy and ileal pouch-anal anastomosis in children. METHODS: This study is a retrospective review of 100 consecutively referred children (<18 years old) who underwent reconstruction with a J-pouch of ileum and preservation of the transitional anorectal epithelium by the same two-surgeon team. Temporary diverting ileostomy was used. The main outcome measures were daytime and nocturnal fecal continence, bowel movements per day, and complications including pouchitis, ileoanal stricture, or postoperative small-bowel obstruction. RESULTS: Average age of the 100 children (48 males/52 females) was 13.2 years (range, 2.95-17.99). All 25 children with familial adenomatous polyposis had proctocolectomy and reconstruction performed simultaneously. Of 75 children with ulcerative colitis, 50 (67%) had their colectomy followed by reconstruction after an interval ranging from 2 months to 4.4 years. Median postoperative follow-up was 2.6 years. Daytime fecal continence was achieved in 98 children, although 4 reported rare accidents. Nighttime continence was achieved in 93 children, of whom 14 reported rare accidents. The average frequency of bowel movements was 5.43/day (+/-2.22). Only one child with polyposis had pouchitis. Of 75 children with ulcerative colitis, 35 had symptoms consistent with pouchitis; of these 35 children, 10 required prolonged treatment. The most frequent postoperative complication was ileoanal stricture requiring operative dilatation and/or anoplasty (18 children). Bowel obstruction requiring surgery occurred in 18 children. One child eventually required pouchectomy for probable Crohn's disease. CONCLUSIONS: Excellent results can be achieved with restorative proctocolectomy in children with respect to fecal continence and stool frequency. However, with ulcerative colitis, a substantial risk of pouchitis remains.