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Background: Injuries from multiple magnet ingestions in the pediatric population have been increasing in both incidence and morbidity. This trend will likely continue after a 2017 court ruling that overturned a ban on the sale of magnet sets marketed as "adult desk toys." Depending on the arrangement of the ingested magnets in the gastrointestinal tract, the consequences can range from benign to life threatening., Objective: This review of cases aims to help clinicians recognize this pathology and help them appreciate the unique management of this type of foreign body ingestion., Discussion: Several cases are presented that individually illustrate an arm of the most comprehensive management algorithm, proposed by the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition. The management is largely driven by the clinical appearance of the child as well as information obtained through abdominal radiographs. Imaging variables that factor into management include the location of the magnets, the number of magnets, and the progression of magnets on serial radiographs., Conclusion: This article uses cases and illustrative medical imaging to describe the most common scenarios and their management. This is especially relevant considering recent U.S. court rulings that overturned the U.S. Consumer Product Safety Commission's ban on the sale of toys containing multiple miniature magnets., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Rationale and Objectives: Traditional assessments in radiology residency focus on the Medical Expert CanMEDS role and typically rely upon a single or limited static images. We designed an Emergency Radiology Simulator that aimed to assess the breadth of competencies required across Medical and NonMedical Expert domains., Material and Methods: An online simulator with typical emergency cases was administered in October 2015 to Post Graduate Year (PGY) 2-5 residents in Radiology. Residents provided preliminary reports, which were graded for style and content. The simulation also included prioritization, protocoling, counseling, and handover exercises geared to assess NonMedical Expert roles., Results: Fourty eight residents participated in the simulation. Level of resident was 11 PGY-2, 17 PGY-3, 13 PGY-4, and 7 PGY-5. There was a significant difference in resident performance between PGY-2 residents and those more senior in terms of the Medical Expert role (findings, diagnosis, recommendations, and clinical relevance of reports). Differences in performance between PGY levels were not seen in the NonMedical Expert roles (prioritization, protocoling, counseling, and handover)., Conclusion: Simulation provides an opportunity to assess radiology resident performance across multiple domains. PGY-2 residents performed worse on the Medical Expert domains, although performance did not significantly vary between the other years. This may suggest that competence in Emergency Radiology is achieved early in residency, possibly related to the importance placed on developing skills related to on-call performance during the PGY-2 year. The simulator should be extended to other areas of Radiology, in order to assess the ability to discriminate performance in other subspecialties., (Copyright © 2018 The Association of University Radiologists. Published by Elsevier Inc. All rights reserved.)

Background: To examine whether SMOFlipid prevents progression of intestinal failure-associated liver disease (IFALD) in parenteral nutrition (PN)-dependent infants with early IFALD (conjugated bilirubin 17-50 µmol/L, 1-3 mg/dL)., Study Design: Pilot multicenter blinded randomized controlled trial comparing SMOFlipid with Intralipid. Patients received the trial lipid for up to 12 weeks, unless they achieved full enteral tolerance sooner. The primary clinical outcome was the serum conjugated bilirubin., Results: Twenty-four infants (mean age, 6 weeks) participated in the trial (13 Intralipid and 11 SMOFlipid). At the time of trial enrollment, patients in both groups were receiving 90% of their calories by PN. Mean duration on trial was 8 weeks and did not differ according to treatment ( P = .99). At trial conclusion, patients who received SMOFlipid had a lower conjugated bilirubin than those who received Intralipid (mean difference, -59 µmol/L; P = .03). Patients receiving SMOFlipid were also more likely to have a decrease in serum conjugated bilirubin to 0 µmol/L than those in the Intralipid group over the entire observation period (hazard ratio, 10.6; 95%; P = .03). The time to achievement of full enteral tolerance did not differ statistically (hazard ratio, 1.3; P = .59) between the groups. There was no significant difference in safety outcomes between the groups., Conclusions: Compared with Intralipid, SMOFlipid reduces the risk of progressive IFALD in children with intestinal failure. This trial was registered at clinicaltrials.gov as NCT00793195.

Evidence-based medicine requires strong scientific evidence upon which to base treatment. In rare diseases, study populations are often small, and thus this evidence is difficult to accrue. Investigators, though, should be creative and develop a flexible toolkit of methods to deal with the problems inherent in the study of rare disease. This narrative review presents alternative clinical trial designs for studying treatments of rare diseases, including cross-over and n-of-1 trials, randomized placebo-phase design, enriched enrollment, randomized withdrawal design, and classes of adaptive designs. Examples of applications of these designs are presented along with their advantages and disadvantages. Additional analytical considerations such as Bayesian analysis, internal pilots, and use of biomarkers as surrogate outcomes are further discussed. A framework for selecting appropriate clinical trial design is proposed to guide investigators in the process of selecting alternative designs for rare diseases. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

Objective: To determine expert beliefs regarding the probability of intestinal failure-associated liver disease (IFALD) with novel lipid-based approaches (lipid minimization/ω-3 lipids) in managing IFALD to facilitate Bayesian analyses of clinical trials of these therapies., Study Design: Structured interviews were conducted using a validated approach to belief elicitation with 60 intestinal failure (IF) experts from across North America. Participants were asked to estimate, in an average population of infants referred for management of IF with early IFALD, the probability of advanced IFALD at 3 months following referral in each of 3 scenarios: (1) conventional lipid, (2) ω-3 lipids, and (3) lipid minimization. Probability distributions of the risk of advanced IFALD with each strategy were developed. Distributions of the elicited treatment effect for the novel approaches, relative to conventional lipid, were calculated., Results: Median duration of experience of participants managing patients with IF was 8.5 (range, 2-35) years. The median probability of advanced IFALD using conventional lipid was 32.5%; ω-3 lipids, 17.5%; and lipid minimization, 13%. The median of the elicited treatment effects relative to conventional lipid was a relative risk of 0.53 for the ω-3 lipid and 0.45 for lipid minimization., Conclusions: There was consistent expert opinion that the novel lipid-based approaches are superior to conventional therapy, with similar estimates of treatment efficacy for the 2 approaches. The distributions of the elicited treatment effects can be used as prior distributions in Bayesian analyses of clinical trials of these novel strategies., (© 2013 American Society for Parenteral and Enteral Nutrition.)

Objective: To investigate how consensus is operationalized in Delphi studies and to explore the role of consensus in determining the results of these studies., Study Design and Settings: Systematic review of a random sample of 100 English language Delphi studies, from two large multidisciplinary databases [ISI Web of Science (Thompson Reuters, New York, NY) and Scopus (Elsevier, Amsterdam, NL)], published between 2000 and 2009., Results: About 98 of the Delphi studies purported to assess consensus, although a definition for consensus was only provided in 72 of the studies (64 a priori). The most common definition for consensus was percent agreement (25 studies), with 75% being the median threshold to define consensus. Although the authors concluded in 86 of the studies that consensus was achieved, consensus was only specified a priori (with a threshold value) in 42 of these studies. Achievement of consensus was related to the decision to stop the Delphi study in only 23 studies, with 70 studies terminating after a specified number of rounds., Conclusion: Although consensus generally is felt to be of primary importance to the Delphi process, definitions of consensus vary widely and are poorly reported. Improved criteria for reporting of methods of Delphi studies are required., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Evidence based medicine requires strong scientific evidence upon which to base treatment. Because the available study populations for rare diseases are small, this evidence is difficult to accrue. Investigators need to consider a flexible toolkit of methods to deal with the problems inherent in the study of rare disease. We present some potential solutions in this paper.

Historically, intestinal failure-associated liver disease (IFALD) has been the greatest contributor to the morbidity experienced by children with intestinal failure. Although the cause of IFALD is multifactorial, recently much attention has been devoted to the critical role that intravenous lipid emulsions play in the development of IFALD. This attention has prompted an interest in alternate approaches to the provision of intravenous lipid in children with IFALD. The 2 approaches that have been advanced are that of lipid minimization and alternate intravenous lipid emulsions, including those containing ω-3 fatty acids. This article examines the rationale and current evidence for these approaches in children with intestinal failure. Our overall finding is that although these alternate approaches show significant promise, they have primarily been studied in uncontrolled settings, mainly in children with advanced IFALD. As such, we believe that there remains a lack of definitive evidence for their efficacy. Furthermore, important safety parameters remain to be evaluated, including the effect of these therapies on growth and development. Therefore, there is currently insufficient evidence to support the use of these novel therapies as standard of care in children with no or early IFALD with the goal of preventing the progression of liver disease.

Purpose of Review: To review the literature on graft type in pediatric liver transplantation, with a specific focus on publications since 2010., Recent Findings: Due to the limited availability of whole livers for transplantation, the majority of pediatric patients will receive a technical variant graft (live donor, reduced, split). Although the outcomes of these grafts may be inferior to whole organs, the detrimental impact needs to be balanced with the impact of technical variants on improved access and survival to transplantation. Vigilance in detecting and managing posttransplant complications is critical in ensuring the optimal outcome. Infants under 5 kg pose a particular challenge in terms of graft selection with hyperreduced and monosegment grafts proposed for this population. Grafts from donors after cardiac death show promise in expanding the donor pool. However, experience in pediatric patients with these grafts is quite limited, particularly in young children who form the majority of pediatric transplant recipients. Auxiliary transplantation is proposed as a strategy for children presenting with fulminant hepatic failure and for children with metabolic diseases., Summary: The majority of children will receive a technical variant graft, with graft choice being largely determined by organ availability.

Purpose: The purpose of the study was to compare outcomes after partial vs complete fundoplication in patients with prior esophageal atresia repair., Methods: All patients undergoing fundoplication following esophageal atresia repair at a tertiary care pediatric hospital from 1987 to 2006 were retrospectively reviewed. All children had at least 1 year of follow-up postfundoplication., Results: Of 47 children, 31 (66%) had a partial fundoplication and 16 (34%) had complete fundoplication. Demographics, presence of tracheoesophageal fistula, early complications of esophageal atresia repair, gastroesophageal reflux symptoms before fundoplication, and operative details of fundoplication were statistically similar between groups, except for the frequency of hiatus repair during fundoplication (23% vs 69%, P = .004). Patients were followed for a median of 4.98 years (range, 1-17.8 years). Postfundoplication symptoms of vomiting (39% vs 31%), dysphagia (45% vs 38%), retching (10% vs 25%), abnormal findings on barium study, and need for reoperation (19% vs 13%) were not statistically different between groups. However, a greater proportion of children undergoing partial fundoplication achieved long-term symptom- and medication-free recovery (52% vs 13%, P = .012)., Conclusions: Our data suggest that partial fundoplication is associated with a greater likelihood of symptom- and medication-free recovery than complete fundoplication in children with previously repaired esophageal atresia., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background: The utility and efficacy of the laparoscopic approach to the management of inflammatory bowel disease (IBD) in children are not clearly known., Methods: We conducted a retrospective descriptive cohort study of children with a diagnosis of IBD who underwent a laparoscopic or laparoscopy-assisted procedure at a quaternary pediatric referral center between 1999 and 2007., Results: One-hundred thirty-six children underwent 154 operations (85 small bowel/ileocolic and 69 colorectal) over the 8 years of the study. Median age was 14.8 years (range = 1.8-18.8). The diagnosis was Crohn's disease in 83, ulcerative colitis in 50, and indeterminate colitis in 3. Median time to regular diet was 5 days (range = 1-19), and median postoperative stay was 7 days (range = 1-70). Seven patients undergoing a small bowel/ileocolic resection (8.2%) were converted to an open procedure. Overall morbidity for the small bowel/ileocolic procedures was 27.1%. The conversion rate during subtotal colectomy (STC) was 7.1% (3/42), and it was 0% for the 22 patients who underwent ileal pouch-anal anastomosis (IPAA) procedures. Overall morbidity associated with STC was 62.8%, and following IPAA it was 63.6%. Sixteen percent (7/69) of those who underwent a colorectal procedure developed a late postoperative bowel obstruction with three patients requiring operative intervention., Conclusion: A laparoscopic approach is feasible with a low conversion rate in most children with IBD. Despite superior cosmesis, perioperative morbidity is similar to that seen with open procedures. Laparoscopic colorectal IBD procedures are associated with an unexpectedly high incidence of postoperative bowel obstruction, although the rates are comparable to those seen with open surgery.

Purpose: We sought to examine in a multiple variable model the impact of residual colonic length on time to intestinal adaptation in a cohort of infants with short bowel syndrome., Method: Infants with a surgical diagnosis of short bowel syndrome who underwent operation 90 days or younger were included in this analysis. Univariate Cox proportional hazards models for time to full-enteral feeds were developed. Predictors significant at the .2 level were entered into a stepwise multiple variable Cox proportional hazards model., Results: A total of 106 infants were included in the cohort (70 adapted). Predictors meeting the criteria for the multiple variable model were as follows: multidisciplinary management (P = .045), Serial Transverse Enteroplasty Procedure (P = .057), percent small bowel (P < .001), percent large bowel (P < .001), preserved ileocecal valve (P = .001), number of septic (P < .001), and central line complications (P < .001). The final model included the following: multidisciplinary management (hazard ratio [HR], 1.932; 95% confidence interval [CI], 1.137-3.281), percent small bowel (HR, 1.028; 95% CI, 1.02-1.04), and septic events (HR, 0.695; 95% CI, 0.6-0.805)., Conclusions: The colon does not seem to play a significant role in intestinal adaptation. However, in addition to highlighting the importance of residual small bowel length, our model highlights the benefit of multidisciplinary intestinal rehabilitation and reduction of septic complications in achieving intestinal adaptation., (Copyright (c) 2010 Elsevier Inc. All rights reserved.)

Purpose: Although evidence suggests that parenteral omega-3 lipid emulsions (O-3LEs) may be beneficial in treating advanced parenteral nutrition (PN)-associated liver disease, our objective was to determine if O-3LEs are justified in those with early liver disease., Methods: This is a retrospective analysis of prospectively collected data on all surgical neonates, who received more than 1 day of PN postoperatively between 2001 and 2004 with observation through 2005 (era before O-3LE introduction). We examined the proportion of those who developed mild and advanced liver dysfunction., Results: Of the 292 infants in the cohort, 104 (36%) developed mild liver dysfunction (conjugated bilirubin, 34 micromol/L [cBili34]) after a mean of 22 days. Thirty-one (30%) of the cBili34 patients reached a serum conjugated bilirubin of 100 micromol/L, and 13 (13%) developed liver failure. Of these, 4 underwent transplantation, and 5 died of hepatic disease. Overall, 86 of the cBili34 patients (83%) were weaned off PN., Conclusion: With more than 80% of cBili34 patients being weaned from PN without adverse hepatic sequelae, it is difficult, in the absence of definitive evidence of efficacy and safety for O-3LEs together with increased costs, to justify the routine use of O-3LEs in this low-risk population outside formal research protocols., (Copyright (c) 2010 Elsevier Inc. All rights reserved.)

Intestinal failure associated liver disease (IFALD) is one of the most common and devastating complications in infants with intestinal failure. Although multifactorial, its pathophysiology is clearly related to the administration of parenteral nutrition (PN), with a recent focus on the role of PN lipid emulsions. This paper will review the evidence for the use of omega-3 fatty acid PN lipid emulsions, which are proposed to have efficacy in the treatment of IFALD. Mechanisms explaining their effects will be considered as will future research directions.

Parenteral nutrition associated liver disease is the most common complication of pediatric short bowel syndrome (SBS). There is emerging evidence that the disease may be reversed with the use of parenteral lipid emulsions derived from fish-oils, which contain significant concentrations of omega-3 fatty acids (w3FA). This paper will review the rationale for the use of parenteral lipid emulsions containing w3FA in SBS and the evidence for their efficacy. Given the promising results and apparent safety of these emulsions, we shall also consider what the current role for PN lipid emulsions containing w3FA in children with SBS should be.

Purpose: The purpose of the study was to describe the mechanisms of injury and causes of death in children dying in a modern, integrated trauma system., Method: Records of all children (<16 years of age) who died in Ontario from 2001 through 2003 after blunt or penetrating trauma were obtained from the Chief Coroner. Demographics and the nature and causes of injury and the causes of death were recorded. Estimates of the mortality rate were determined using census data., Results: There were 234 injury deaths (222 blunt, 12 penetrating) over the 3 years. Thirty (13%) resulted from intentional injury. The median age was 10 (range, 0-15.9) years; 62% were male. Sixty-eight percent resulted from incidents involving motor vehicles (passenger, pedestrian, or cyclist). Most (74%) died at the scene; only 5% survived for more than 24 hours. Devastating craniocervical injury (Abbreviated Injury Scale 5 or 6) was present in 84% and was the only life-threatening injury in 40%. The annual mortality rate averaged 3.2 per 100,000 children., Conclusions: In a modern, integrated trauma system, most pediatric injury deaths occur at the scene from severe head injuries. In this population, strategies to reduce the death rate from pediatric trauma must focus on primary and secondary injury prevention.

Background: Existing data on pediatric intestinal length (IL) are limited because most studies report postmortem values. Using prospective data, appropriate norms for IL were developed., Method: The IL measurements, using a silk suture on the antimesenteric border, were prospectively made on patients between 24 weeks of gestational age and 5 years of age undergoing laparotomy. Patients with gastrointestinal malformations or those above or below 2 SDs for growth parameters were excluded. A curve fitting process was applied to determine the best model for IL (small bowel and colon separately) from among postconception age, weight, and height at surgery., Results: One hundred eight patients participated in this study. Highly predictive (R(2) > 0.8) models for IL were determined for all predictor variables (postconception age, weight, and height) examined suggesting that all of these variables are excellent predictors determinants of IL. Although all models had statistically similar properties, the model using height had the best performance across the full range of the variable., Conclusion: Although age, weight, nor height was definitely superior for the prediction of IL, we propose that until external validations of our models occur, height at surgery be used for the prediction of expected small intestinal and colon length in infants.

Absence or altered distribution of the interstitial cells of Cajal (ICCs) has been described in association with intestinal pseudoobstruction in adults. We report the first pediatric case with regional absence of ICCs in the distal small bowel and colon associated with intestinal pseudoobstruction. This report highlights that abnormalities of the ICCs in intestinal pseudoobstruction should be considered early in the diagnostic workup of children with intestinal pseudoobstruction.

Background: Infants with short bowel syndrome (SBS) frequently have proximal enterostomies with the colon excluded from intestinal continuity. Restoring intestinal continuity may create difficulty with perineal soiling. We propose creation of a distal sigmoid colostomy as an option for select infants with SBS., Methods: This study involves the descriptive case series of all children with SBS who received a distal sigmoid colostomy., Results: Eight infants (mean age, 70 days) received creation of a distal colostomy. In 1, placement of the colostomy was done at the initial surgery for SBS, 2 at the time of intestinal lengthening, and 5 during another procedure. Median length of small bowel was 58 cm. At 60 days postprocedure, median gain in enteral tolerance was 21% (P = .09), with median stoma output of 125% enteral intake. There were no complications directly attributable to the distal colostomy., Conclusion: Establishment of a distal sigmoid colostomy is an excellent management strategy for select children with SBS. It provides the benefits of restoring bowel continuity such as increased fluid and electrolyte absorption, improved energy absorption from fermentation of carbohydrate to short-chain fatty acids, and the theoretical enhancement of glucagonlike peptide 2 secretion that may optimize intestinal adaptation-all while protecting the perineal skin and simplifying quantification and collection of stool.

Parenteral nutrition associated liver disease (PNALD) is the major source of morbidity and mortality in children with short bowel syndrome (SBS). There is emerging evidence that omega-6 fatty acids (omega6FA) within the parenteral solution play a major role in PNALD and their effects may be reversed or ameliorated by substitution with omega-3 fatty acids (omega3FA). This paper reviews the mechanisms whereby omega3FAs may influence PNALD by improving bile flow, inhibiting steatosis, and having immunomodulatory effects. The early clinical experience with omega3FAs in SBS and PNALD is briefly reviewed and the implications of such, and future directions are considered.

Meckel's diverticulum has previously been reported to be present in patients with Crohn's disease. However, the finding is typically an incidental one, and involvement of the diverticulum in the disease process is uncommon. We report a case of an adolescent with known Crohn's disease who presented with symptoms thought to be due to terminal ileitis. At the time of laparoscopy the inflammation was found to be due to involvement of the Meckel's diverticulum with Crohn's disease.

Purpose: To examine the results of the multimodal management of patients with endangering hepatic hemangioma associated with systemic compromise., Methods: Retrospective descriptive case series of children with endangering hepatic hemangioma managed at our institution between January 1996 and June 2006., Results: Six children (5 females) presented with endangering hepatic hemangioma with systemic effects during the index time period. Mean age at presentation was 1.9 months (range, 2 days to 4 months). All patients received medical treatment, and all patients also underwent hepatic embolization, with a median number of procedures of 2 (range, 1-6). Two patients died, one of sepsis and one of progressive liver failure in a child presenting with advanced liver disease owing to neonatal hemochromatosis that was unrecognized at the time. The remaining 4 patients all recovered and were discharged. With mean postprocedure follow-up of 2.11 years (range, 0-6.2 years), all remain well., Conclusion: Multimodal management of endangering hepatic hemangioma is a strategy that deserves consideration in the management of these patients. Although the strategy requires further evaluation as to its safety and efficacy, the procedure has the potential to decrease the need for liver transplantation because of treatment failures in this population.

Objective: To examine the outcome of technical variant liver transplant techniques relative to whole organ liver transplantation in pediatric liver transplant recipients., Background: Technical variant liver transplant techniques comprising split, reduced, and live-donor liver transplantation evolved to address the need for timely and size appropriate grafts for pediatric recipients., Methods: Analysis of data from the Studies of Pediatric Liver Transplantation (SPLIT) registry, a multicenter database of 44 North American pediatric liver transplant programs. The outcome (morbidity and mortality) of each of the technical variants were compared with that of whole organ recipients., Results: Data were available on 2192 transplant recipients (1183 whole, 261 split, 388 reduced, and 360 live donor). Recipients of all technical variant graft type were significantly younger than whole organ recipients, but on average spent 2.3 months less on the waiting list. Thirty-day post-transplant morbidity was increased for each type of technical variant relative to whole organ (45.1% whole, 66.7% split, 65.5% reduced, 51.9% live-donor). Biliary complications (30 day: 7.5% whole, 18.8% split, 16% reduced, 17.5% live-donor) and portal vein thrombosis (30 day: 3.6% whole, 8% split, 8% reduced, 7.5% live-donor) were more common in all technical variant types. Graft type was an independent predictor of graft loss (death or retransplantation) in a multivariate analysis. Split and reduced (relative risk = 1.74 and 1.77, respectively) grafts had a worse outcome when compared with whole organ recipients., Conclusions: Technical variant techniques expand the pediatric donor pool and reduce time from listing to transplant, but they are associated with increased morbidity and mortality.

Purpose: This study was conducted to examine the preoperative factors predictive of subsequent intervention for gastroesophageal reflux (GER) in children with congenital diaphragmatic hernia (CDH)., Methods: We conducted a retrospective cohort study on children who underwent repair of a CDH between January 1, 1995, and December 31, 2002 with follow-up continuing to September 1, 2005. Excluded in the study were children who died during their first admission, or who underwent fundoplication at the time of CDH repair. Univariate and multivariate logistic regressions were performed to examine preoperative factors predictive of subsequent intervention (fundoplication or gastrojejunal tube placement)., Results: Of 86 children, 13 underwent intervention (fundoplication, 10; gastrojejunal tube, 3) for GER. Univariate predictors included the following: right-sided CDH, use of nonconventional ventilation, liver within the chest, and patch closure of the CDH. However, only liver within the chest and patch closure of the CDH were significant predictors in a multiple variable analysis. The positive and negative predictive values of the multivariate model were 69.2% and 87.7%, respectively., Conclusions: Infants with CDH who have liver within the chest or require patch closure of their hernia are at increased risk for subsequent intervention for GER. These children may represent a subpopulation that would benefit from fundoplication at the time of CDH repair.

Purpose: The purpose of the study was to compare the outcomes in children undergoing thoracoscopic versus open resection of congenital lung lesions., Methods: Retrospective review of 12 consecutive children (<3 years of age) undergoing thoracoscopic resection of a congenital lung lesion between 2004 and 2005 was performed. Intraoperative and early postoperative results were compared with randomly selected age- and sex-matched (2:1) patients undergoing thoracotomy between 2000 and 2005., Results: Twelve children underwent thoracoscopic resection and were compared with 24 that underwent thoracotomy. Seventy five percent of the lesions in both groups were congenital cystic adenomatoid malformations. There were no major intraoperative complications. Two thoracoscopic procedures were converted to a thoracotomy. Perioperative outcomes including operative time, length of stay, duration and volume of chest tube drainage, and dose and duration of intravenous opioids were similar for the procedures. However, children undergoing thoracoscopic procedures were less likely (odds ratio = 0.07) to have received adjunctive regional anesthesia. Overall morbidity was 33% thoracoscopic and 25% open (P = .70)., Conclusion: Thoracoscopic resection is a safe and feasible alternative to open resection of congenital lung lesions. Examination of long-term advantages of the thoracoscopic approach such as decreased risk of chest wall deformity and scoliosis and improved cosmesis will require longer follow-up.

Purpose: To examine predictors of a false-positive (FP) result on contrast enema (CE) for the diagnosis of Hirschsprung disease (HD)., Methods: Retrospective analysis, over a 5-year period (1999-2004), of infants (<6 months of age) with suspected HD undergoing rectal biopsy following abnormalities identified on CE (transition zone [TZ], abnormal rectosigmoid ratio, microcolon, retained contrast, or mucosal irregularity)., Results: One hundred twenty-nine patients underwent rectal biopsy following an abnormal CE. The FP rate was 48.5% (66 with HD). Age below 30 days (OR, 3.4; 95% CI, 1.1-10.3), female sex (OR, 3.4; 95% CI, 1.6-7.3), and absence of TZ (OR, 6.3; 95% CI, 2.6-15.3) were independently associated with an increased risk for FP on multiple variable logistic regression. A history of bilious emesis decreased the probability of FP (OR, 0.2; 95% CI, 0.06-0.5)., Conclusions: Transition zone, sex, age, and bilious emesis are important predictors of FP in those with suspected HD and CE abnormalities. With 100% incidence of FP, infants younger than 30 days with neither bilious emesis nor a TZ and female infants younger than 30 days with these features may represent a subpopulation in whom rectal biopsy can be avoided.

Background: A multidisciplinary intestinal rehabilitation team has been in place at our institution for 3 years. Our goal was to compare the preliminary outcomes of neonates with short bowel syndrome before with those after the establishment of our formalized intestinal failure program (Group for the Improvement of Intestinal Function and Treatment [GIFT])., Methods: We conducted a retrospective analysis of our intestinal failure registry comparing preGIFT (1997-1999) with GIFT (2003-2005) outcomes., Results: Fifty-four patients (mean gestational age = 33.5 weeks) were included in the GIFT cohort, and 40 patients (mean gestational age = 30.7 weeks) formed the preGIFT cohort. Overall mortality rates (33.3% vs 37.5%, P = .84) were equivalent in the 2 cohorts, although fewer patients died of liver failure after the establishment of the GIFT. Among those with liver failure, the mortality in the preGIFT group was 9/10 as compared with that of 6/13 in the GIFT group (P = .03). The decrease in liver-related deaths was partly attributable to earlier referral for and increased survival to transplant (4 for the GIFT group vs 0 for the preGIFT group)., Conclusions: Analysis of the preliminary outcomes of the GIFT program suggests that the natural history of neonatal short bowel syndrome remains unaltered to date despite a coordinated approach to care. However, improved communication and integration with the transplant service have resulted in earlier assessment, increased rates of transplantation, and decreased mortality from liver failure.

Background: Image-guided balloon dilatation has been used in adults as an alternative to standard surgical treatment of intestinal stricture. The experience in children is limited. We report our results with this procedure in the management of both congenital and acquired intestinal stenosis in children., Materials and Methods: A retrospective analysis was done of children younger than 2 years of age who underwent balloon dilatation of small and large intestinal stenosis between 1994 and 2003., Results: Eleven children underwent dilatation during the study period. Two of these children had congenital duodenal stenosis, and this represents the first report of nonoperative management of this condition. Three children underwent dilatation of small bowel strictures and 6 had dilatation of colonic and rectal strictures. Necrotizing enterocolitis was the most common (6/9) etiology of stricture. Ten of 11 patients did not require subsequent operative management although 3 children required further dilatations. The mean follow-up was 36.5 months (range, 13 days-103 months). One patient underwent a subsequent dilatation that was unsuccessful, and required operative resection of a 5-cm stricture. There was one complication, a small leak that was managed nonoperatively., Conclusion: Image-guided balloon dilatation holds promise as an alternative to surgical treatment in children with congenital or acquired stenosis of the small or large bowel, and should be considered in select patients with short strictures.

Background: The role of isolated liver transplantation in infants with parenteral nutrition-associated cholestasis (PNAC) associated with short bowel syndrome (SBS) is unclear., Methods: We performed a retrospective descriptive study of infants with PNAC and SBS who received an isolated liver transplant at our institution. Review of the literature was also performed., Results: Three infants (aged 7, 8, and 13 months) with SBS and PNAC received an isolated liver transplant. Etiology of SBS was necrotizing enterocolitis, gastroschisis, and volvulus. Two patients with pretransplant small bowel length of 40 and 80 cm, who were receiving 65% and 79% of intake enterally, demonstrated good graft function (bilirubin, 0 and 7 micromol/L) at 41 and 58 months posttransplant. Despite full tolerance of enteral feeds, both remain on parenteral nutrition (PN) (4 and 7 nights per week) for poor weight gain. One child with 25 cm of small bowel, who received 65% of energy enterally pretransplant, died 7 months posttransplant from PNAC. A literature review revealed 22 cases of isolated liver transplant for PNAC associated with SBS. Overall survival was 77%, with 76% of survivors demonstrating independence from PN., Conclusions: Isolated liver transplantation is an acceptable option for select infants with PNAC associated with SBS when further intestinal adaptation and freedom from PN are anticipated.

A congenital cystic adenomatoid malformation (CCAM) is a congenital lung lesion that, when associated with hydrops, is thought to have a dismal prognosis without fetal intervention. The authors report a case of a fetus with a CCAM associated with ascites as the only manifestation of hydrops, which was diagnosed at 19 weeks gestation. The CCAM regressed without intervention between 23 and 29 weeks of gestation with resolution of the ascites. The baby was born asymptomatic at term, and the infant underwent elective resection of the CCAM at 1 year of age. A review of the literature from 1980 to 2000 yielded 9 other cases of spontaneous resolution of hydrops associated with CCAM, suggesting that although rare, this outcome is a possibility in fetuses diagnosed with the condition. Because most fetuses diagnosed with hydrops undergo termination of pregnancy, the true incidence of hydrops resolution is potentially higher than predicted. This has important implications for prenatal counseling particularly in centers in which fetal intervention is not available.

Fibrinolytic agents have been used successfully in the management of loculated empyema; however, their use in the treatment of intraabdominal abscesses is limited. The authors describe the case of a 4-week-old girl with intraabdominal abscesses secondary to intestinal perforation that were not amenable to percutaneous drainage, but were managed successfully with intracavitary administration of tissue-plasminogen activator. This case represents the first report in a human, in which tissue-plasminogen activator was used to facilitate percutaneous drainage of an intraabdominal abscess. It is also the first time a fibrinolytic agent has been used for this purpose in a child.

Background/purpose: Live-donor liver transplantation (LDLT) has developed to address the critical shortage of cadaveric organs that accounts for 20% of children who die while awaiting for a liver transplant in Ontario each year. This report reviews the outcome of the pediatric recipients of LDLT at the authors' center., Methods: The charts of all children who received a LDLT between June 1996 and March 2002 were reviewed retrospectively., Results: Thirteen children (mean age, 3.6 years) underwent LDLT. All donors were parents except for one cousin. Ten grafts were left-lateral segments, 2 were right lobes, and 1 was a left lobe. Three patients required a SILASTIC((R)) (Dow Corning, Midland, MI) patch for delayed abdominal wall closure. Patient and graft survival rate was 100% with a median follow-up of 376 days. Major postoperative complications included biliary leaks (n = 2), biliary strictures (n = 1), portal vein thrombosis (n = 1), and hepatic venous complications (n = 1). There were no cases of hepatic artery thrombosis. Ten of 12 children became Positive for Epstein-Barr virus (EBV), and 3 of these patients had readily treatable post-transplant lymphoproliferative disorder., Conclusions: LDLT is an acceptable alternative to cadaveric transplantation for children with end-stage liver disease., (Copyright 2003 Elsevier Inc. All rights reserved.)

Background: Neurologically impaired children with gastroesophageal reflux (GER) usually are treated with a fundoplication and gastrostomy (FG); however, this approach is associated with a high rate of complications and morbidity. The authors evaluated the image-guided gastrojejunal tube (GJ) as an alternative approach for this group of patients., Methods: A retrospective review of 111 neurologically impaired patients with gastroesophageal reflux was performed. Patients underwent either FG (n = 63) or GJ (n = 48). All FGs were performed using an open technique by a pediatric surgeon, and all GJ tubes were placed by an interventional radiologist., Results: The 2 groups were similar with respect to diagnosis, age, sex and indication for feeding tube. Patients in the GJ group were followed up for an average of 3.11 years, and those in the FG group for 5.71 years. The groups did not differ statistically with respect to most complications (bleeding, peritonitis, aspiration pneumonia, recurrent gastroesophageal reflux [GER], wound infection, failure to thrive, and death), subsequent GER related admissions, or cost. Children in the GJ group were more likely to continue taking antireflux medication after the procedure (P <.05). Also, there was a trend for GJ patients to have an increased incidence of bowel obstruction or intussusception (20.8% v 7.9%). Of the FG patients 36.5% experienced retching, and 12.7% experienced dysphagia. Eighty-five percent of patients in the GJ group experienced GJ tube-specific complications (breakage, blockage, dislodgment), and GJ tube manipulations were required an average of 1.68 times per year follow-up. Nine patients (14.3%) in the FG group had wrap failure, with 7 (11.1%) of these children requiring repeat fundoplication. In the GJ group, 8.3% of patients went on to require a fundoplication for persistent problems. A total of 14.5% of GJ patients had their tube removed by the end of the follow-up period because they no longer needed the tube for feeding., Conclusions: Image-guided gastrojejunal tubes are a reasonable alternative to fundoplication and gastrostomy for neurologically impaired children with GER. The majority can be inserted without general anesthesia. This technique failed in only 8.3% patients, and they subsequently required fundoplication. A total of 14.5% of GJ patients showed some spontaneous improvement and had their feeding tube removed. Each approach, however, still is associated with a significant complication rate. A randomized prospective study comparing these 2 approaches is needed., (Copyright 2002 by W.B. Saunders Company.)

Background: Laparoscopic-assisted ileocolic resection for Crohn disease has been reported as an acceptable alternative to the open procedure in adults. We evaluated our experience with this procedure in the adolescent population., Methods: All adolescents undergoing ileocolic resection for Crohn disease during a 3-year period were retrospectively reviewed. Intraoperative and early postoperative results were analyzed, comparing those undergoing the laparoscopic-assisted approach with those having open resection., Results: Eleven patients (mean age, 15.6 years) underwent open and 12 patients (mean age, 16.5 years) underwent laparoscopic-assisted resection. None had undergone previous resection. The two groups did not differ with respect to time from diagnosis to surgery, indications for surgery, preoperative medical therapy, operative time, or length of intestine resected. One patient in the laparoscopic-assisted group was converted to an open procedure. There were no intraoperative complications in either group. Although no statistically significant differences were noted for number of days on narcotic, total dosage of narcotic, and time to regular diet, patients undergoing laparoscopic-assisted resection were discharged 2.2 days earlier (5.4 vs. 7.6; P < 0.05). There was one wound infection and one intraabdominal abscess in the open resection group, and a single patient in the laparoscopic-assisted group with postoperative fever and a wound infection., Conclusions: Laparoscopic-assisted ileocolic resection is a safe alternative to open surgery in adolescent patients with Crohn disease.

Objective: To determine whether comorbid anxiety alters response to methylphenidate (MPH) in children with attention-deficit hyperactivity disorder (ADHD)., Method: Ninety-one children with ADHD were assessed for anxiety. Children were randomly assigned to receive MPH or placebo, titrated to a dose of 0.7 mg/kg, while side effects were minimized. Measures of side effects and behavioral response were obtained from parents and teachers before treatment, after titration to optimal dose, and after 4 months of treatment. These measures, dose of drug, and rate of adherence to assigned medication assignment were compared for nonanxious (ADHD- ANX) and anxious ADHD children (ADHD+ ANX)., Results: Rates of adherence to original medication assignment did not differ between the groups. ADHD+ ANX on both MPH and placebo titrated to a lower dose at the end of titration, although the dose of drug did not differ among the groups after 4 months of treatment. No differential response to MPH between ADHD+ ANX and ADHD- ANX was noted at end-titration or at 4 months on any side effect or behavioral measures., Conclusions: Comorbid anxiety does not appear to influence development of side effects or behavioral response to MPH when dose is titrated as in standard clinical practice.