Surgical waitlist management: Perspectives from surgeons on surgical prioritization at a paediatric hospital.

Globally exacerbated surgical waitlists have provided the opportunity to reflect on prioritization and resource allocation decisions. The unique circumstances of paediatric surgery and consequences of surgical delay prompted the study reported in this paper. As part of a larger project to attend to prioritization in our surgical waitlists, we conducted a Quality Improvement study, the purpose of which is to understand surgeon's perspectives regarding the ethical and practical realities of surgical prioritization at our institution. The study comprises semi-structured interviews with nine full-time paediatric surgeons from a variety of subspecialties conducted at our institution, which is a tertiary paediatric hospital with ten surgical subspecialties in a publicly funded healthcare system. Participants articulated how they prioritize their waitlists, and how they understand ethical prioritization. These findings resonate with the growing public concern for ethical practice in healthcare delivery and transparency in prioritization and resource allocation practices. Specifically, more transparency, consistency, and support is required in prioritization practices. This work highlights the importance of institutional dialogue regarding surgical case prioritization. Because quality improvement work is necessarily site-specific, concrete generalizations cannot be offered. However, the insights gleaned from these interviews and the process by which they were gleaned are a valuable knowledge-sharing resource for any institution that is interested in ongoing quality improvement work. The objectives here were to clarify the goals of prioritization within the institution, improve prioritization practices, and make them more ethical and transparent.

Findings in percutaneous trans-hepatic cholecysto-cholangiography in neonates and infants presenting with conjugated hyperbilirubinemia: emphasis on differential diagnosis and cholangiographic patterns.

BACKGROUND: Biliary atresia (BA) is one of the causes of conjugated hyperbilirubinemia in infants which if untreated leads to end-stage liver disease and death. Percutaneous Trans-hepatic Cholecysto-Cholangiography (PTCC) is a minimally invasive study which can be utilized in the diagnostic work-up of these patients. This study's purpose is to describe the experience with PTCC in neonates, the imaging findings encountered, and the abnormal patterns which warrant further investigation. METHODS: A 16-year single-center retrospective study of patients with persistent neonatal cholestasis (suspected BA) undergoing PTCC. Patient demographics, laboratory values, PTCC images, pathology and surgical reports were reviewed. RESULTS: 73 patients underwent PTCC (68% male, mean age 8.7 weeks, mean weight 4.0 Kg). The majority of studies were normal (55%). Abnormal patterns were identified in 33 cases, 79% were diagnosed with BA and 12% with Alagille syndrome. Non-opacification of the common hepatic duct with a narrowed common bile duct (42%) and isolated small gallbladder (38%) were the most common patterns in BA. CONCLUSION: PTCC is a minimally invasive study in the diagnostic work-up of infants presenting with conjugated hyperbilirubinemia (suspected BA). Further invasive investigations or surgery can be avoided when results are normal.

Consensus Guidelines for Perioperative Care in Neonatal Intestinal Surgery: Enhanced Recovery After Surgery (ERAS®) Society Recommendations.

BACKGROUND: Enhanced Recovery After Surgery (ERAS®) Society guidelines integrate evidence-based practices into multimodal care pathways that have improved outcomes in multiple adult surgical specialties. There are currently no pediatric ERAS® Society guidelines. We created an ERAS® guideline designed to enhance quality of care in neonatal intestinal resection surgery. METHODS: A multidisciplinary guideline generation group defined the scope, population, and guideline topics. Systematic reviews were supplemented by targeted searching and expert identification to identify 3514 publications that were screened to develop and support recommendations. Final recommendations were determined through consensus and were assessed for evidence quality and recommendation strength. Parental input was attained throughout the process. RESULTS: Final recommendations ranged from communication strategies to antibiotic use. Topics with poor-quality and conflicting evidence were eliminated. Several recommendations were combined. The quality of supporting evidence was variable. Seventeen final recommendations are included in the proposed guideline. DISCUSSION: We have developed a comprehensive, evidence-based ERAS guideline for neonates undergoing intestinal resection surgery. This guideline, and its creation process, provides a foundation for future ERAS guideline development and can ultimately lead to improved perioperative care across a variety of pediatric surgical specialties.

The role of sonography for depiction of a whirlpool sign unrelated to midgut malrotation in neonates.

BACKGROUND: The sonographic whirlpool sign of volvulus due to midgut malrotation is well recognized. However, variations of the whirlpool sign may be seen in other conditions, but this observation has received little attention in the literature. OBJECTIVE: This study presents a series of neonates with a variety of causes of congenital intestinal obstruction, all associated with a whirlpool sign (unrelated to midgut volvulus), which was correctly recognized preoperatively on sonography. We also emphasize the pivotal role of sonography in managing congenital obstruction of the intestinal tract in neonates. MATERIALS AND METHODS: This is a retrospective analysis of clinical, imaging and surgical findings in 11 neonates with congenital intestinal obstruction associated with a whirlpool sign (unrelated to midgut volvulus) that was recognized preoperatively on sonography and in whom the cause for the whirlpool was documented at surgery. RESULTS: Eleven neonates (eight male, three female) had clinical and radiographic evidence of intestinal obstruction in whom sonography depicted a whirlpool sign, which was recognized on the initial sonogram in nine and on a repeat sonogram in two. The whirlpool was located in the upper abdomen in only two, mid-abdomen in five and right lower quadrant in four. The whirlpool was only 1-2 cm in diameter. An upper gastrointestinal series in three neonates failed to depict the cause of obstruction. Contrast enema in three cases had findings suggesting the site of obstruction was in the ileum but none depicted the exact cause of the obstruction. At surgery, the whirlpool sign correlated with a segmental volvulus of the small intestine in eight neonates and with the coiled distal small intestine associated with apple-peel atresia in the other three. CONCLUSION: This study illustrates neonates in whom a whirlpool sign (unrelated to midgut volvulus) was correctly recognized on sonography before surgery. Sonography proved more useful than an upper gastrointestinal series or contrast enema in depicting the exact cause of the obstruction. Pediatric radiologists must make the effort to search throughout the entire abdomen and pelvis for a small whirlpool sign on sonography, even in the absence of midgut malrotation, in neonates with congenital intestinal obstruction. Its recognition preoperatively will facilitate a rapid diagnosis and will obviate the necessity for contrast examinations of the gastrointestinal tract, which require ionizing radiation.

The experiences of parents and caregiver(s) whose child received an organ from a living anonymous liver donor.

BACKGROUND: Anonymous living donor transplantation is a potential strategy to address the shortage of available organs for transplant. A living anonymous donor (LAD) is a donor with no biological connection and whose identity is unknown to the recipient. This study captured the lived experiences of pediatric liver transplant recipient families whose child received an organ from a LAD. METHODS: Qualitative data collection and analysis were guided by a theoretical framework of phenomenology. Data analysis highlighted themes through an inductive process of reviewing transcript paragraphs to code for significant statements that represented key concepts and captured depth of experience. RESULTS: A total of nine interviews were conducted with 10 participants. Data analysis yielded themes of emotional turbulence through their transplant journey. Pre-transplant experiences were characterized by feelings of helplessness and desperation. Receiving a LAD transplant prompted shock, relief, and acceptance of the donation. Post-transplant experiences were characterized by altered life-perspectives and varied levels of connectedness to the donor, marked by gratitude and concern for donor well-being. CONCLUSION: Anonymous donation in liver transplantation is perceived by recipient families as a remarkable gift and a viable donor option. Our preliminary findings can be used to inform strategy development regarding future delivery of care.

Analysis of surgical interruption of the enterohepatic circulation as a treatment for pediatric cholestasis.

To evaluate the efficacy of nontransplant surgery for pediatric cholestasis, 58 clinically diagnosed children, including 20 with Alagille syndrome (ALGS), 16 with familial intrahepatic cholestasis-1 (FIC1), 18 with bile salt export pump (BSEP) disease, and 4 others with low γ-glutamyl transpeptidase disease (levels <100 U/L), were identified across 14 Childhood Liver Disease Research Network (ChiLDReN) centers. Data were collected retrospectively from individuals who collectively had 39 partial external biliary diversions (PEBDs), 11 ileal exclusions (IEs), and seven gallbladder-to-colon (GBC) diversions. Serum total bilirubin decreased after PEBD in FIC1 (8.1 ± 4.0 vs. 2.9 ± 4.1 mg/dL, preoperatively vs. 12-24 months postoperatively, respectively; P = 0.02), but not in ALGS or BSEP. Total serum cholesterol decreased after PEBD in ALGS patients (695 ± 465 vs. 457 ± 319 mg/dL, preoperatively vs. 12-24 months postoperatively, respectively; P = 0.0001). Alanine aminotransferase levels increased in ALGS after PEBD (182 ± 70 vs. 260 ± 73 IU/L, preoperatively vs. 24 months; P = 0.03), but not in FIC1 or BSEP. ALGS, FIC1, and BSEP patients experienced less severely scored pruritus after PEBD (ALGS, 100% vs. 9% severe; FIC1, 64% vs. 10%; BSEP, 50% vs. 20%, preoperatively vs. >24 months postoperatively, respectively; P < 0.001). ALGS patients experienced a trend toward greater freedom from xanthomata after PEBD. There was a trend toward decreased pruritus in FIC1 after IE and GBC. Vitamin K supplementation increased in ALGS after PEBD (33% vs. 77%; P = 0.03). Overall, there were 15 major complications after surgery. Twelve patients (3 ALGS, 3 FIC1, and 6 BSEP) subsequently underwent liver transplantation. CONCLUSION: This was a multicenter analysis of nontransplant surgical approaches to intrahepatic cholestasis. Approaches vary, are well tolerated, and generally, although not uniformly, result in improvement of pruritus and cholestasis. (Hepatology 2017;65:1645-1654).

Early Posthepatoportoenterostomy Predictors of Native Liver Survival in Biliary Atresia.

OBJECTIVES: Most infants with biliary atresia (BA) require liver transplantation (LT) after hepatoportoenterostomy (HPE), including those who initially clear jaundice. The aim of the present study was to identify clinical and routine laboratory factors in infants with BA post-HPE that predict native liver survival at 2 years. METHODS: A retrospective cohort study was conducted in 217 patients with BA undergoing HPE in Sydney, Australia and Toronto, Canada between January 1986 and July 2009. Univariate and multivariate logistic regression using backwards-stepwise elimination identified variables at 3 months after HPE most associated with 2-year native liver survival. RESULTS: Significant variables (P < 0.05) on univariate analysis included serum total bilirubin (TB) and albumin at 3 months post-HPE, bridging fibrosis or cirrhosis on initial liver biopsy, ascites of <3 months post-HPE, type 3 BA anatomy, age at HPE of >45 days, change in length z scores within 3 months of HPE, and center. On multivariate analysis, TB (P < 0.0001) and albumin (P = 0.02) at 3 months post-HPE, and center (P = 0.0003) were independently associated with native liver survival. Receiver operating characteristic analysis revealed an optimal cut-off value of TB <74 µmol/L (4.3 mg/dL; area under the receiver operating characteristic curve 0.8990) and serum albumin level >35 g/L (3.5 mg/dL; area under the receiver operating characteristic curve 0.7633) to predict 2-year native liver survival. TB and albumin levels 3 months post-HPE defined 3 groups (1: TB ≤74 µmol/L, albumin >35 g/L; 2: TB ≤74 µmol/L, albumin ≤35 g/L; 3: TB >74 µmol/L) with distinct short- and long-term native liver survival rates (log-rank P < 0.001). Length z scores 3 months post-HPE were poorer for group 2 than group 1 (-0.91 vs -0.30, P = 0.0217) with similar rates of coagulopathy. CONCLUSIONS: Serum TB and albumin levels 3 months post-HPE independently predicted native liver survival in BA when controlling for center. Serum albumin level <35 g/L in infants with BA who were no longer jaundiced at 3 months post-HPE was a poor prognostic indicator. Poorer linear growth and absence of significant coagulopathy suggest a role for early aggressive nutritional therapy in this group.

Liver transplantation for children with acute liver failure associated with secondary hemophagocytic lymphohistiocytosis.

Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening systemic disease, characterized by overwhelming stimulation of the immune system and categorized as primary or secondary types. Occasionally, acute liver failure (ALF) may dominate the clinical presentation. Given the systemic nature of HLH and risk of recurrence, HLH is considered by many a contraindication to liver transplantation (LT). The aim of this study is to review our single-center experience with LT in children with secondary HLH and ALF (HLH-ALF). This is a cross-sectional, retrospective study of children with secondary HLH-ALF that underwent LT in 2005-2014. Of 246 LTs, 9 patients (3 males; median age, 5 years; range, 0.7-15.4 years) underwent LT for secondary HLH-ALF. Disease progression was rapid with median 14 days (range, 6-27 days) between first symptoms and LT. Low fibrinogen/high triglycerides, elevated ferritin, hemophagocytosis on liver biopsy, and soluble interleukin 2 receptor levels were the most commonly fulfilled diagnostic criteria; HLH genetic studies were negative in all patients. Immunosuppressive therapy after LT included corticosteroids adjusted to HLH treatment protocol and tacrolimus. Thymoglobulin (n = 5), etoposide (n = 4), and alemtuzumab (n = 2) were used in cases of recurrence. Five (56%) patients experienced HLH recurrence, 1 requiring repeat LT, and 3 died. Overall graft and patient survival were 60% and 67%, respectively. Six patients are alive and well at a median of 24 months (range, 15-72 months) after transplantation. In conclusion, LT can be beneficial in selected patients with secondary HLH-ALF and can restore good health in an otherwise lethal condition. Liver Transplantation 22 1245-1253 2016 AASLD.

Biliary complications in pediatric liver transplantation: Incidence and management over a decade.

This study analyzed how features of a liver graft and the technique of biliary reconstruction interact to affect biliary complications in pediatric liver transplantation. A retrospective analysis was performed of data collected from 2001 to 2011 in a single high-volume North American pediatric transplant center. The study cohort comprised 173 pediatric recipients, 75 living donor (LD) and 98 deceased donor (DD) recipients. The median follow-up was 70 months. Twenty-nine (16.7%) patients suffered a biliary complication. The majority of leaks (9/12, 75.0%) and the majority of strictures (18/22, 81.8%) were anastomotic. There was no difference in the rate of biliary complications associated with DD (18.4%) and LD (14.7%) grafts (P = 0.55). Roux-en-Y (RY) reconstruction was associated with a significantly lower rate of biliary complications compared to duct-to-duct reconstruction (13.3% versus 28.2%, respectively; P = 0.048). RY anastomosis was the only significant factor protecting from biliary complications in our population (hazard ratio, 0.30; 95% confidence interval, 0.1-0.85). The leaks were managed primarily by relaparotomy (10/12, 83.3%), and the majority of strictures were managed by percutaneous biliary intervention (14/22, 63.6%). Patients suffering biliary complications had inferior graft survival (P = 0.04) at 1, 5, and 10 years compared to patients without biliary complications. Our analysis demonstrates a lower incidence of biliary complications with RY biliary reconstruction, and patients with biliary complications have decreased graft survival.

Magnetic foreign body injuries: a large pediatric hospital experience.

OBJECTIVE: To examine trends in magnet-related injuries and hypothesize that changes are a result of new neodymium-iron-boron magnets that are smaller, stronger, and commonly sold in sets. STUDY DESIGN: In this retrospective chart review, we searched our institution's electronic patient record for patients less than 18 years old who were diagnosed with magnetic foreign body ingestion between 2002 and 2012. Cases were analyzed for patient, magnetic foreign body, and management characteristics. Incidence rates and case characteristics were compared between the first 8 years of the study period and the last 3. RESULTS: We identified 94 patients who met our search criteria. Of confirmed ingestions, the median age was 4.5 years and 65% were male. The incidence of visits increased between the 2002-2009 period and the 2010-2012 period by a factor of 2.94 (95% CI, 1.84-4.70), whereas the incidence of injuries involving multiple magnets increased by a factor of 8.40 (95% CI, 3.44-20.56). The volume of the magnets decreased from 878.6 mm3 to 259.8 mm3. Six cases required surgical removal of the magnets because of intra-abdominal sepsis or concern for imminent bowel perforation. CONCLUSIONS: Since 2002, there has been a significant increase in the incidence of magnetic foreign body injuries. These injuries have increasingly involved multiple, smaller magnets and required operative intervention.

Placement of an internal-external biliary drain through a bilio-enteric fistula in a neonate to re-establish antegrade bile flow after liver transplantation.

Three-month-old baby girl with history of post-liver transplant hepatic artery dissection treated with ligation after take down of the biliary anastomosis and placement of a surgical external common bile duct drain. There was persistent malfunction of this drain. A bilio-enteric fistula was noted during the later placement of an image guided percutaneous external drain. Subsequently, an internal-external biliary drain was successfully placed through this fistula. An excellent clinical and functional result was achieved.

Introduction of a Warming Bundle to Reduce Hypothermia in Neonatal Surgical Patients.

INTRODUCTION: Hypothermia in the neonatal surgical population has been linked with significant morbidity and mortality. Our goal was to decrease intra and postoperative hypothermia. INTERVENTION: In November 2021, a radiant warmer and hat were included along with standard warming methods prior to the start of General Surgery procedures to minimize episodes of hypothermia. PRIMARY OUTCOME: Core body temperature was measured pre, intra and post-operatively. METHODS: Data were prospectively collected from electronic medical records from July 2021 to March 2023. A retrospective analysis was performed. Hypothermia was defined as a temperature <36.5C. Control charts were created to analyze the effect of interventions. RESULTS: A total of 277 procedures were identified; 226 abdominal procedures, 31 thoracic, 14 skin/soft tissue and 6 anorectal. The median post-natal age was 36.1 weeks (IQR: 33.2-39.2), with a pre-surgical weight of 2.3 kg (IQR: 1.6-3.0) and operative duration of 181 min (IQR: 125-214). Hat and warmer data were unavailable for 59 procedures, both hat and warmer were used for 51 % procedures, hat alone for 29 %, warmer alone for 10 % and neither for 10 % of procedures. Over time there was a significant increase in hat utilization while warmer usage was unchanged. There was a significant increase in the mean lowest intra-operative temperature and decrease in proportion of hypothermic patients intra-operatively and post-operatively. CONCLUSIONS: The inclusion of a radiant warmer and hat decreased the proportion of hypothermic patients during and after surgery. Further studies are necessary to analyze the impact on surgical outcomes. LEVEL OF EVIDENCE: III.

Successful liver transplantation in an infant with stage 4S(M) neuroblastoma.

We report a 2.5-month-old infant with bilateral adrenal neuroblastoma, stage 4S(M), with liver metastases and chemotherapy-induced veno-occlusive disease leading to cirrhosis requiring liver transplantation. Despite unknown tumour histology and MYCN-amplification status, we proceeded with liver transplant. This decision was based on clinical suspicion that our patient was MYCN-negative due to significant tumour regression, and was supported by evidence indicating that MYCN-amplification is rare in infants with favourable-stage neuroblastoma. This is the second case report of neuroblastoma requiring liver transplantation; however, in the previously reported case, the diagnosis of neuroblastoma was not established until after transplantation. We discuss this unique case to justify the potential use of life-saving liver transplants in infants with neuroblastoma.

The intersection of pediatric surgery, climate change, and equity.

Climate change is occurring at an unprecedented rate. Recent years have seen heatwaves, wildfires, floods, droughts, and re-emerging infectious diseases fueled by global warming. Global warming has also increased the frequency and severity of surgical disease, particularly for children, who bear an estimated 88% of the global burden of disease attributable to climate change. Health care delivery itself weighs heavily on the environment, accounting for nearly 5% of global greenhouse gas emissions. Within the health care sector, surgery and anesthesia are particularly carbon intensive. The surgical community must prioritize the intersection of climate change and pediatric surgery in order to address pediatric surgical disease on a global scale, while reducing the climate impact of surgical care delivery. This review defines the current state of climate change and its effects on pediatric surgical disease, discusses climate justice, and outlines actions to reduce the climate impact of surgical services. LEVEL OF EVIDENCE: Level V.

Development of a five point enhanced recovery protocol for pectus excavatum surgery.

PURPOSE: We implemented and evaluated an Enhanced Recovery after Surgery (ERAS) protocol for Nuss procedures consisting of patient education, bowel management, pre/post-operative transitional pain service involvement, serratus anterior plane blocks and intercostal nerve cryoablation. METHODS: A 5-point ERAS protocol was implemented using multiple plan-do-study-act (PDSA) cycles. Data was collected prospectively for patients in the full ERAS protocol and retrospectively for previous patients. The primary outcome was length of stay (LOS). Secondary outcomes were opioid consumption, pain scores, protocol compliance and patient satisfaction. The impact of PDSA cycles and the ERAS protocol was quantified using statistical process control charts and Mann Whitney U test. RESULTS: A total of 53 patients were identified, 13 within the ERAS protocol and 40 prior to introduction. There was no difference in age, sex, or Haller index between the two cohorts. The median LOS was decreased by 3 days in the ERAS cohort (P = 0.00001). There was decreased opioid consumption on post-operative day 1 (1.47 vs 1.96 MME/kg, p = 0.009) and overall (3.12 vs 6.35 MME/kg, p = 0.0042) in the ERAS cohort. Median pain scores did not differ between cohorts. ERAS bundle element compliance was: education 92%, bowel management 100%, transitional pain involvement 100%, serratus block 100% and cryoablation 100%. The 1-month survey revealed that 92% of patients were satisfied with their experience. CONCLUSION: Our results demonstrate significant reduction in LOS and a trend to decreasing opioid consumption in hospital following ERAS protocol implementation and support the further application of ERAS protocols in pediatrics. LEVEL OF EVIDENCE: III - Retrospective comparative study.

Secondary impacts of the COVID-19 pandemic at a tertiary children's hospital in Canada: a mixed-methods study.

OBJECTIVES: Decisions to pause all non-essential paediatric hospital activities during the initial phase of the COVID-19 pandemic may have led to significant delays, deferrals and disruptions in medical care. This study explores clinical cases where the care of children was perceived by hospital clinicians to have been negatively impacted because of the changes in healthcare delivery attributing to the restrictions placed resulting from the COVID-19 pandemic. DESIGN AND SETTING: This study used a mixed-methods approach using the following: (1) a quantitative analysis of overall descriptive hospital activity between May and August 2020, and utilisation of data during the study period was performed, and (2) a qualitative multiple-case study design with descriptive thematic analysis of clinician-reported consequences of the COVID-19 pandemic on care provided at a tertiary children's hospital. RESULTS: Hospital-level utilisation and activity patterns revealed a substantial change to hospital activity including an initial reduction in emergency department attendance by 38% and an increase in ambulatory virtual care from 4% before COVID-19 to 67% between May and August 2020. Two hundred and twelve clinicians reported a total of 116 unique cases. Themes including (1) timeliness of care, (2) disruption of patient-centred care, (3) new pressures in the provision of safe and efficient care and (4) inequity in the experience of the COVID-19 pandemic emerged, each impacting patients, their families and healthcare providers. CONCLUSION: Being aware of the breadth of the impact of the COVID-19 pandemic across all of the identified themes is important to enable the delivery of timely, safe, high-quality, family-centred paediatric care moving forward.

I-PASS enhances effectiveness and accuracy of hand-off for pediatric general surgery patients.

BACKGROUND: I-PASS is a validated and standardized hand-off protocol shown to reduce medical error and improve hand-off efficiency in the pediatric medical population. Our aim was to evaluate the feasibility, effectiveness, accuracy and resident satisfaction of implementing I-PASS on a pediatric surgery service. METHODS: A prospective intervention Quality Improvement (QI approved) study was utilized to evaluate resident written and verbal hand-offs before and after implementation of I-PASS on a pediatric surgery service at a tertiary center. Anonymous surveys were completed by residents following each observation. Results were analyzed using T or Mann-Whitney U Tests and Chi Square. RESULTS: A total of 49 written tools and 50 verbal hand-offs were compared pre-and post I-PASS implementation. With I-PASS, increased written accuracy was observed in the documentation of the patient summary (p < 0.05). Accuracy in the verbal hand-off of illness severity, patient summary, contingency plan, action list and synthesis also improved (p < 0.05); but duration of hand-off increased (p < 0.01). Post implementation surveys of residents demonstrated an increased understanding of patient management (p < 0.05). CONCLUSION: Implementing I-PASS on a pediatric surgery service with modifications catered to surgical patients, improved the effectiveness and accuracy of written and verbal patient hand-offs and increased provider satisfaction and preparedness. LEVEL OF EVIDENCE: Level II.

The anatomic pattern of biliary atresia identified at time of Kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival.

OBJECTIVE: The goals of this study were to describe the clinical and anatomic features of infants undergoing Kasai portoenterostomy (KPE) for biliary atresia (BA) and to examine associations between these parameters and outcomes. METHODS: Infants enrolled in the prospective Childhood Liver Disease Research and Education Network, who underwent KPE were studied. Patients enrolled in a blinded, interventional trial were excluded from survival analysis. Primary endpoints were successful surgical drainage (total bilirubin less than 2 mg/dL within the first 3 months), transplant-free survival (Kaplan-Meier), and time to transplant/death (Cox regression). RESULTS: KPE was performed in 244 infants (54% female; mean age: 65 ± 29 days). Transplant-free survival was 53.7% and 46.7% at 1 and 2 years post-KPE. The risk of transplant/death was significantly lower in the 45.6% of patients who achieved successful bile drainage within 3 months post-KPE (HR: 0.08, P < 0.001). The risk of transplant/death was increased in patients with porta hepatis atresia (Ohi type II and III vs type I; HR: 2.03, P = 0.030), nonpatent common bile duct (Ohi subtype: b, c, and d vs a; HR: 4.31, P = 0.022), BA splenic malformation syndrome (HR: 1.92, P = 0.025), ascites > 20 mL (HR: = 1.90, P = 0.0230), nodular liver appearance compared to firm (HR: = 1.61, P = 0.008), and age at KPE ≥ 75 days (HR: 1.73, P < 0.002). Outcome was not associated with gestational age, gender, race, ethnicity, or extent of porta hepatis dissection. CONCLUSION: Anatomic pattern of BA, BASM, presence of ascites and nodular liver appearance at KPE, and early postoperative jaundice clearance are significant predictors of transplant-free survival.

Increasing severity of pectus excavatum is associated with reduced pulmonary function.

OBJECTIVE: To determine whether pulmonary function decreases as a function of severity of pectus excavatum, and whether reduced function is restrictive or obstructive in nature in a large multicenter study. STUDY DESIGN: We evaluated preoperative spirometry data in 310 patients and lung volumes in 218 patients aged 6 to 21 years at 11 North American centers. We modeled the impact of the severity of deformity (based on the Haller index) on pulmonary function. RESULTS: The percentages of patients with abnormal forced vital capacity (FVC), forced expiratory volume in 1 second (FEV(1)), forced expiratory flow from 25% exhalation to 75% exhalation, and total lung capacity findings increased with increasing Haller index score. Less than 2% of patients demonstrated an obstructive pattern (FEV(1)/FVC <67%), and 14.5% demonstrated a restrictive pattern (FVC and FEV(1) <80% predicted; FEV(1)/FVC >80%). Patients with a Haller index of 7 are >4 times more likely to have an FVC of ≤80% than those with a Haller index of 4, and are also 4 times more likely to exhibit a restrictive pulmonary pattern. CONCLUSIONS: Among patients presenting for surgical repair of pectus excavatum, those with more severe deformities have a much higher likelihood of decreased pulmonary function with a restrictive pulmonary pattern.

Critical review of controversial issues in the management of advanced pediatric liver tumors.

Hepatocellular carcinoma (HCC) and hepatoblastoma (HB) are the most common primary tumors of liver in children. The management of patients with locally advanced, unresectable disease or those with extra-hepatic distant metastases provides substantial challenges to pediatric oncologists, hepatologists, and surgeons. Herein, we critically debate the two sides of three specific controversies: (1) the role of chemotherapy in the treatment of advanced pediatric HCC; (2) the indications for liver transplantation in children with HCC, specifically, the appropriateness of using adult Milan criteria; and (3) the role of liver trasplantation in children with unresectable HB that present with metastatic disease. Pediatr Blood Cancer 2011;56:1013-1018. © 2010 Wiley-Liss, Inc.