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BACKGROUND: Despite advances in infection-control practices, surgical site infections (SSIs) remain a substantial cause of morbidity, mortality, and increased costs among hospitalized patients. METHODS: We used a matched cohort design to compare costs and length of stay for 16 pediatric patients with an SSI with those of 16 matched control patients who had a similar operative procedure during the same time period but in whom an SSI did not develop. RESULTS: On average, length of stay was increased by 10.6 days (P = .02) and costs were increased by $27,288 (P = .01) for each patient with a potentially preventable SSI. On the day of the surgical procedure, costs between study patients and matched controls differed by only 1.4%. By day 3, however, costs were 36% higher for patients with an SSI. CONCLUSIONS: While matching study patients and control patients requires significant time from financial and clinical staff, this approach and the resulting data analysis strengthened and focused our efforts to prevent future SSIs and aligned initiatives to reduce SSIs with the business case for quality. [ABSTRACT FROM AUTHOR]

Background: Portal diversion by surgical shunt plays a major role in the treatment of medically refractory portal hypertension. We evaluate our center's experience with surgical shunts for the treatment of pediatric portal hypertension., Methods: All patients who underwent surgical shunt at a single institution from 2008 to 2017 were reviewed. The primary outcome was intervention-free shunt patency., Results: In this study, 34 pediatric patients underwent portal shunt creation. The median age was 7.7 years (interquartile range 4.3-12.0). Twenty-nine patients (85%) had prehepatic portal hypertension and 5 patients (15%) had intrahepatic portal hypertension. The primary manifestations of portal hypertension were esophageal varices (97%) and gastrointestinal bleeding (77%). Eighteen patients (53%) underwent meso-Rex bypass, 10 patients (29%) underwent splenorenal shunt, and 6 patients (18%) underwent mesocaval shunt. Outcomes were notable for minimal wound complications (9%), rebleeding events (12%), and mortality (3%). In the postoperative setting, 10 patients (29%) experienced a shunt complication (occlusion or stenosis), 4 of which occurred in the early postoperative period and required urgent intervention. The 1-year and 5-year "primary patency" patency rates were 71% and 66%, respectively., Conclusion: Children suffer significant morbidity from the sequelae of portal hypertension. Our experience reinforces the feasibility of surgical shunts as an effective treatment option associated with low rates of morbidity and mortality., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Transitions of care between individual providers or teams of providers have a high potential for errors due to the incomplete transfer of critical information and the need for ongoing care. The transition from the operating room (OR) to the intensive care unit (ICU) is a particularly dangerous time for critically ill children. Hand-offs of care between the OR and ICU teams during this key transition period require detailed communication of complete and accurate patient information at a time when the patient is perhaps most vulnerable from a physiologic standpoint. Improving the safety of transitions from the OR to the ICU is an active area of investigation, though there are a few notable best practices that are commonly employed in a number of centers. These best practices include having the appropriate personnel at the bedside for the hand-off, the use of scripts and the "sterile cockpit rule", the use of checklists, double verification of post-operative orders, and maintaining an overall safety culture., Competing Interests: Conflicts of Interest: The authors have no conflicts of interest to declare.

Kidney transplantation is the optimal treatment of ESRD in children. Some studies have reported inferior outcomes in recipients of LDN allografts who are ≤ 5 yr of age. We performed a retrospective review of pediatric recipient outcomes of 110 LDN allografts at our institution and examined predictors of adverse outcomes. Subgroup analysis was performed by dividing recipients into three age categories: 0-5 yr, 6-17 yr, and ≥ 18 yr. There was no significant difference between incidences of DGF or ARE between groups. Kaplan-Meier analysis demonstrated 100% allograft survival in 0- to 5-yr-old recipients, nearly reaching statistical significance (p = 0.07) for outcome superior to that of the two older age groups. Pretransplant HD was associated with increased risk of DGF (p = 0.05). Significant risk factors for ARE were recipient weight >15 kg (p = 0.033) and multiple renal arteries (p = 0.047). Previous ARE was associated with an increased risk of allograft failure (p = 0.02). LDN is not associated with increased risk of DGF, ARE, or allograft failure in the youngest recipients. These findings support an aggressive pursuit of preemptive transplantation even in the youngest pediatric allograft recipients., (© 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background: Surgical site infections (SSIs) are preventable events associated with significant morbidity and cost. Few interventions have been tested to reduce SSIs in children., Methods: A quality improvement collaboration was established in Ohio composed of all referral children's hospitals. Collaborative leaders developed an SSI reduction bundle for selected cardiac, orthopedic, and neurologic operations. The bundle was composed of 3 elements: prohibition of razors for skin preparation, chlorhexidine-alcohol use for incisional site preparation, and correct timing of prophylactic antibiotic administration. The incidence of SSIs across the collaborative was compared before and after institution of the bundle. The association between 1 of the bundle elements, namely correct timing of antibiotic prophylaxis, and the proportion of centers achieving 0 SSIs per month was measured., Results: Eight pediatric hospitals participated. The proportion of months in which 0 SSIs per center was recorded was 56.9% before introduction of the bundle, versus 81.8% during the intervention (P < .001). Correct timing of preoperative prophylactic antibiotics also significantly improved; 39.4% of centers recorded correct timing in every eligible surgical procedure per month ("perfect timing") before the intervention versus 78.7% after (P < .001). The achievement of 0 SSIs per center in a given month was associated with the achievement of perfect antibiotic timing for that month (P < .003)., Conclusions: A statewide collaborative of children's hospitals was successful in reducing the occurrence of SSIs across Ohio., (Copyright © 2014 by the American Academy of Pediatrics.)

The surgical consent serves as a key link in preventing breakdowns in communication that could lead to wrong-patient, wrong-site, or wrong-procedure events. We conducted a quality improvement initiative at a large, urban pediatric academic medical center to reliably increase the percentage of informed consents for surgical and medical procedures with accurate safety data information at the first point of perioperative contact. Improvement activities focused on awareness, education, standardization, real-time feedback and failure identification, and transparency. A total of 54,082 consent forms from 13 surgical divisions were reviewed between May 18, 2011, and November 30, 2012. Between May 2011 and June 2012, the percentage of consents without safety errors increased from a median of 95.4% to 99.7%. Since July 2012, the median has decreased slightly but has remained stable at 99.4%. Our results suggest that effective safety checks allow discovery and prevention of errors., (Copyright © 2014 AORN, Inc. Published by Elsevier Inc. All rights reserved.)

Background: Bed capacity management is a critical issue facing hospital administrators, and inefficient discharges impact patient flow throughout the hospital. National recommendations include a focus on providing care that is timely and efficient, but a lack of standardised discharge criteria at our institution contributed to unpredictable discharge timing and lengthy delays. Our objective was to increase the percentage of Hospital Medicine patients discharged within 2 h of meeting criteria from 42% to 80%., Methods: A multidisciplinary team collaborated to develop medically appropriate discharge criteria for 11 common inpatient diagnoses. Discharge criteria were embedded into electronic medical record (EMR) order sets at admission and could be modified throughout a patient's stay. Nurses placed an EMR time-stamp to signal when patients met all discharge goals. Strategies to improve discharge timeliness emphasised completion of discharge tasks prior to meeting criteria. Interventions focused on buy-in from key team members, pharmacy process redesign, subspecialty consult timeliness and feedback to frontline staff. A P statistical process control chart assessed the impact of interventions over time. Length of stay (LOS) and readmission rates before and after implementation of process measures were compared using the Wilcoxon rank-sum test., Results: The percentage of patients discharged within 2 h significantly improved from 42% to 80% within 18 months. Patients studied had a decrease in median overall LOS (from 1.56 to 1.44 days; p=0.01), without an increase in readmission rates (4.60% to 4.21%; p=0.24). The 12-month rolling average census for the study units increased from 36.4 to 42.9, representing an 18% increase in occupancy., Conclusions: Through standardising discharge goals and implementation of high-reliability interventions, we reduced LOS without increasing readmission rates.

Portal vein thrombosis (PVT) occurs in ≤12% of pediatric recipients of liver transplantation (LT). Known complications of PVT include portal hypertension, allograft loss, and mortality. The management of PVT is varied. A single-center, case-control study of pediatric LT recipients with portal vein (PV) changes after LT was performed. Cases were categorized as early PVT (if PVT was detected within 30 days of transplantation) or late PVT (if PVT was detected more than 30 days after transplantation or if early PVT persisted beyond 30 days). Two non-PVT control patients were matched on the basis of the recipient weight, transplant indication, and allograft type to each patient with PVT. Thirty-two of the 415 LT recipients (7.7%) received 37 allografts and developed PVT. In comparison with control patients, a higher proportion of patients with PVT had PVT present before LT (13.3% versus 0%, P = 0.01). Patients with early PVT usually returned to the operating room, and 9 of 15 patients (60%) had PV flow restored. Patients with late PVT had lower white blood cell (4.9 [1000/μL] versus 6.8 [1000/μL], P < 0.01) and platelet counts (140 [1000/μL] versus 259 [1000/μL], P < 0.01), an elevated international normalized ratio (1.2 versus 1.0, P < 0.001), and more gastrointestinal bleeding (25% versus 8.3%, P = 0.03) compared to controls. Patients with PVT were also less frequently at the expected grade level (52% versus 88%, P < 0.001). The patient survival rates were 84%, 78%, and 78% and 91%, 84%, and 79% for cases and controls at 1, 5, and 10 years, respectively. The allograft survival rates were 90%, 80%, and 80% for cases and 94%, 89%, and 87% for controls at 1, 5, and 10 years, respectively. In conclusion, patients with early and late PVT had preserved allograft function, and there was no impact on mortality. Patients diagnosed with early PVT often underwent operative interventions with successful restoration of flow. Patients diagnosed with late PVT experienced variceal bleeding, and some required portosystemic shunting procedures. Academic delays were also more common. In late PVT, the clinical presentation dictates care because the optimal management algorithm has not yet been determined. Multi-institutional studies are needed to confirm these findings and improve patient outcomes., (Copyright © 2013 American Association for the Study of Liver Diseases.)

Objective: To determine whether portoenterostomy (PE) revision in patients afflicted with biliary atresia (BA) is a viable treatment option and, if so, identify which patients may benefit., Background: BA, the most common cause of neonatal liver disease, results in biliary tract obstruction and hepatic fibrosis. Kasai PE is the initial surgical intervention performed and, if successful, restores drainage and preserves the native liver. Portoenterostomy failure warrants liver transplantation, but because of complications related to transplantation, treatment strategies to salvage the native liver may be beneficial. Using uniformly applied criteria, we have revised PEs to delay or avoid transplantation., Methods: A retrospective review of medical records of patients diagnosed with BA since 1983 was performed. Patient demographics, symptoms, indications for revision, laboratory values, and outcomes were recorded. A cohort of patients who underwent revision after initial PE was identified. Survival rates were assessed using the Kaplan-Meier method. For patients who required transplantation, operative data from the revised PE cohort were compared with those from the unrevised PE cohort. A Cox proportional hazards model was used to determine covariates predictive of a favorable outcome., Results: Of 181 children who underwent PE, 24 underwent revision. Adequate biliary drainage, as evidenced by normalized conjugated bilirubin levels, was achieved in 75% of revised patients. Overall survival in patients who underwent revision, regardless of transplantation, was 87%. Among patients who underwent PE revision, 46% have survived with their native liver., Conclusion: Experience at our center suggests that with appropriate patient selection, PE revision may delay the need for liver transplanation yielding encouraging patient outcomes.

Purpose: Multiple visits for the evaluation, treatment, and follow-up of straightforward surgical problems are inconvenient, can result in lost work for the parents, and missed school for the child. We hypothesized that with proper previsit screening, patients with select diagnoses can be evaluated in an outpatient clinic setting and undergo operation the same day., Methods: Criteria were developed to identify straightforward referrals to our surgical practice for umbilical, epigastric, or inguinal hernias. Scripting was created to offer families the option of consultation and, if indicated, surgical treatment on the same day. Data collected included number of patients, cases performed, insurance status, and consultation reimbursement and surgical fees. Families were surveyed postoperatively., Results: Sixty-one patient candidates participated. The diagnosis and indication for surgery were confirmed in 56 (92%), of which 50 underwent repair the day of their consultation. Seventy-two percent of patients had commercial insurance, whereas 28% had Medicaid. The preoperative consultation fee was reimbursed in 39 (78%) of 50 encounters (57% Medicaid, 86% commercial). All surgical cases were reimbursed. Patient and family satisfaction was high., Conclusions: We conclude that it is feasible to provide same-day evaluation and service for straightforward pediatric hernias with acceptable financial reimbursement and high parent satisfaction., (Copyright © 2012 Elsevier Inc. All rights reserved.)

A 15-year-old boy presented with a growing mass on the anterior chest wall. History, clinical examination, and preoperative imaging studies were consistent with soft tissue sarcoma. He underwent open biopsy, and the intraoperative pathology diagnosis of nodular fasciitis resulted in performance of a lesional excision, rather than a potentially morbid wide resection. Nodular fasciitis is a rare but important soft tissue lesion, which can be easily confused with sarcoma. The possibility of benign etiologies for soft tissue masses should be considered when planning surgical options, even when preoperative imaging studies suggest more aggressive lesions.

Background: Patients' inability to take oral nutrition calls for alternative feeding. In selected pediatric patients, traditional feeding tubes are not tolerated and jejunal feeding tubes can be obstructive. One option is a Roux-en-Y feeding limb. Our institution noted complications secondary to small bowel volvulus around this limb. Goals of this study were to review patients who experienced volvulus after Roux-en-Y creation, and to identify factors contributing to this complication., Methods: Institutional review board approval was obtained for a retrospective chart review. 25 patients were identified as having a Roux-en-Y jejunal feeding limb. Five developed volvulus. Factors documented included age, time to complication, revision, and outcome., Results: Average age at limb creation was not statistically significant between those with or without volvulus. Mean time to obstruction was 228 +/- 117 days post-limb creation. Average limb length was 18.7 +/- 7 cm in patients with volvulus, 14 +/- 2.3 cm in patients without. 3 of 5 patients presenting with volvulus were discharged home after revision; two patients died., Conclusion: There is no definitive way to prevent small bowel volvulus around Roux-en-Y feeding limbs. No predictors of volvulus were identified. Once revised, no recurrences were observed. While this complication is uncommon, it has potentially catastrophic outcomes requiring early intervention.

Background: Pectus excavatum (pectus) is a common congenital deformity of the chest wall resulting in a diminished anterior-posterior dimension. Chest CT has become a common study for preoperative assessment. CT evaluation was initially described using a single CT image; it is now common to perform a CT of the entire chest to evaluate pectus., Objective: To evaluate the efficacy of chest radiographs compared to chest CT in identifying additional clinically significant abnormalities in the preoperative evaluation of children with pectus., Materials and Methods: We reviewed the chest CT scans of 209 children and young adults who had been evaluated for possible surgical repair of pectus. Additional abnormalities were categorized as (1) incidental, (2) potentially significant, and (3) findings that affected the decision to perform surgery. Chest radiographs were reviewed for category 3 findings., Results: Seventy-six scans showed additional abnormalities, five in group 2 and two in group 3. Both group 3 findings, a vascular ring and an acute pneumonia, were identified on chest radiographs., Conclusion: Conventional radiographs identified clinically important findings in children and young adults evaluated for pectus surgery. Radiation risks and medical costs might be substantially decreased by obtaining a chest radiograph and using a limited CT technique when a CT scan is ordered for the purpose of obtaining a Haller index.

Surveillance ileoscopies are performed regularly immediately post-transplantation to prevent allograft rejection. We investigated whether variability in apoptosis exists between proximal and distal intestinal limbs of double-barreled ileostomies, and if detection varies according to number of biopsies taken and sections prepared for evaluation. We retrospectively analyzed endoscopy/pathology reports of patients who underwent simultaneous proximal and distal ileoscopies during surveillance. We re-reviewed three sections of selected biopsies for the presence of apoptotic bodies and viral inclusions. Seven patients underwent 26 endoscopies in which both distal and proximal limbs were investigated simultaneously. Apoptosis was identified in each limb simultaneously in 21/26 cases (81%). Of the discrepant results, 3/5 (60%) revealed apoptosis in the proximal limb with normal distal limb and 2/5 (40%) had apoptotic bodies identified in the distal limb and normal proximal biopsies. Re-reviewing discrepant biopsies, two patients had at least one piece of mucosa without apoptosis and apoptotic bodies were seen in only 47% of sections. Histologic variability exists between proximal and distal limbs of double-barreled ileostomies and detection of apoptosis increases with number of pieces obtained and sections examined. Investigating both limbs with adequate sample size and rigorous processing may have clinical implications., ((c) 2009 Wiley Periodicals, Inc.)

Background: Poor flow of patients into and out of the ICU can result in gridlock and bottlenecks that disrupt care and have a detrimental effect on patient safety and satisfaction, hospital efficiency, staff stress and morale, and revenue. Beginning in 2006, Cincinnati Children's Hospital Medical Center implemented a series of interventions to "smooth" patient flow through the system., Methods: Key activities included patient flow models based on surgical providers' predicted need for intensive care and predicted length of stay; scheduling the case and an ICU bed at the same time; capping and simulation models to identify the appropriate number of elective surgical cases to maximize occupancy without cancelling elective cases; and a morning huddle by the chief of staff, manager of patient services, and representatives from the operating room, pediatric ICUS, and anesthesia to confirm that day's plan and anticipate the next day's needs., Results: New elective surgical admissions to the pediatric ICU were restricted to a maximum of five cases per day. Diversion of patients to the cardiac ICU, keeping patients in the postanesthesia care unit longer than expected, and delaying or canceling cases are now rare events. Since implementation of the operations management interventions, there have been no cases when beds in the pediatric ICU were not available when needed for urgent medical or surgical use., Discussion: A system for smoothing flow, based on an advanced predictive model for need, occupancy, and length of stay, coupled with an active daily strategy for demand/capacity matching of resources and needs, allowed much better early planning, predictions, and capacity management, thereby ensuring that all patients are in suitable ICU environments.

Tracheobronchial separation (TBS) due to blunt chest trauma in children is extremely rare. We report the case of a 14-year-old boy who fell 20 feet and developed respiratory distress, bilateral pneumothoraces, pneumomediastinum, and subcutaneous emphysema. Computed tomography imaging at the initial institution failed to detect tracheobronchial disruption, and the patient was managed conservatively. The patient's worsening condition prompted bronchoscopic examination which revealed complete separation of the right main bronchus from the trachea. Single-lung ventilation was instituted using a fabricated extra-long nasotracheal tube due to the patient's large size and mandibular fracture, and the airway was primarily anastamosed with an open thoracotomy approach. The clinical features of tracheobronchial separation as well as anesthetic, clinical and surgical management of this rare but life-threatening injury are described.

Background: Surgical site infections (SSIs) remain a substantial cause of morbidity, mortality, increased length of stay, and increased hospital costs. Cincinnati Children's Hospital Medical Center (CCHMC) used reliability science to dramatically reduce the rate of surgical site infections., Methods: Key activities included the development and implementation of strategies to enhance the proportion of patients who receive timely antibiotic administration, a pediatric surgical site infection-prevention bundle, and procedure-specific pediatric surgical site infection-prevention bundles. Measures are presented in monthly reports and annotated control charts that are shared with the improvement team and organizational leadership and that are also posted on the hospital's patient safety intranet site., Results: The Class I and II SSI rate decreased from 1.5 per 100 procedure days at baseline to 0.54 per 100 procedure days, a 64% reduction. The process has remained stable (within control limits) since August 2007. There were 33 fewer surgical site infections in fiscal year (FY) 2006 than in FY 2005, and 21 fewer in FY 2007 than in FY 2006. By December 2007, 91% of eligible same-day surgery patients received antibiotics within 60 minutes before a procedure, and 94% of patients undergoing inpatient surgery received antibiotics within 60 minutes prior to incision., Discussion: Pediatric surgical patients can now expect a safer, more efficient experience with CCHMC's care system and reduced variation in care across CCHMC's surgeons and procedures. Sharing data on individual and collective provider performance was important in recruiting provider support. Examining data on any failures each day allowed assessment and correction, facilitating rapid-cycle improvement. Making the right thing to do the easy thing to do facilitated the behavior changes required.

Purpose: Infants with multiple cutaneous hemangiomas often present with hepatic hemangiomas. They can follow a benign clinical course or require complex management. We reviewed our experience in the management of hepatic hemangiomas., Methods: We performed a retrospective review of patients (1996-2007) with hepatic hemangiomas treated in our institution., Results: Twenty-six patients were diagnosed with hepatic hemangiomas as follows: 8 focal, 12 multiple, and 6 diffuse lesions. Nineteen (73%) patients had associated cutaneous hemangiomas. Sixteen patients had multiple and 3 patients had single cutaneous hemangiomas. All patients with multiple or diffuse liver lesions were screened for heart failure and hypothyroidism. Congestive heart failure developed in 4 patients, 3/4 of these patients had diffuse lesions. Two patients required thyroid replacement because of elevated thyroid-stimulating hormone. Because of progression of disease, 9 patients required steroid treatment. Two patients were treated with vincristine and 3 patients received alpha-interferon because of poor response to steroid treatment. Two patients went on to surgical resection for failed response to medical management and worsening heart failure (left lobectomy, liver transplant). Both patients had uncomplicated postoperative courses. Five patients had a previously undescribed constellation of rapidly involuting cutaneous hemangiomas (gone by 3 months, glut-1-negative) with associated liver lesions also resolving at a faster pace (mean resolution of cutaneous hemangiomas, 1.9 vs 7.9 months; P = .001; liver, 5.8 vs 25.3 months; P = .004). All patients in our series survived., Conclusion: Patients with multiple cutaneous hemangiomas should be screened for hepatic lesions. Patients with diffuse or multifocal liver hemangiomas should be screened for congestive heart failure and hypothyroidism. A subgroup of rapidly involuting cutaneous hemangiomas have a significantly shorter time for involution of hepatic lesions. The status of cutaneous lesions can be used as indicators for the liver hemangiomas.

As the field of Liver Transplantation has matured, survival alone is no longer an acceptable single metric of success. This chapter explores the impact of the PELD system for donor organ allocation, surgical modification of donor organs, living donation, and long-term transplant-related complications on overall quality of life and outcome. Strategies to improve survival, overall outcome, and health-related quality of life in long-term recipients are outlined.

The need for evidence-based practice guidelines requires high quality, carefully controlled clinical research trials. This multidisciplinary conference attempted to: identify urgent clinical and research issues, identify obstacles to performing clinical trials, develop concepts for organ-specific and all-organ research and generate a report that would serve as a blueprint for future research initiatives. A few themes became evident. First, young children present a unique immunologic environment which may lead to tolerance, therefore, including young children in immunosuppression withdrawal and tolerance trials may increase the potential benefits of these studies. Second, adolescence poses significant barriers to successful transplantation. Non-adherence may be insufficient to explain poorer outcomes. More studies focused on identification and prevention of non-adherence, and the potential effects of puberty are required. Third, the relatively naive immune system of the child presents a unique opportunity to study primary infections and alloimmune responses. Finally, relatively small numbers of transplants performed in pediatric centers mandate multicenter collaboration. Investment in registries, tissue and DNA repositories will enhance productivity. The past decade has proven that outcomes after pediatric transplantation can be comparable to adults. The pediatric community now has the opportunity to design and complete studies that enhance outcomes for all transplant recipients.

Objective: Alpha-1-antitrypsin (A1AT) deficiency is the most common genetic cause of liver disease in children; however, the role of polymorphic heterogeneity in the A1AT gene as a modifier of other forms of pediatric liver disease is not clear. We hypothesized that non-M A1AT allele variants are more common in children with chronic liver disease than in the general population., Methods: A retrospective, single-center study was performed in which A1AT phenotypes were obtained by reviewing charts of children with chronic liver disease. Chi-square analysis was used to compare allele frequencies in the population of children with liver disease with published epidemiologic data and to compare allele frequencies among disease subgroups., Results: The frequency of A1AT Z and other alleles was increased in children with chronic liver disease (n = 241) when compared with the published reference database (P < 0.001). This increase remained significant when the population was divided into disease subsets: biliary atresia (n = 67) and other liver disease (n = 174) (P < 0.001 for both). Among children with biliary atresia referred for liver transplant evaluation, the presence of a non-M allele was associated with a lower mean age at transplant listing than the MM phenotype (235 vs 779 days, P = 0.036) and more frequent loss of native liver by 24 months of age (90% vs 65%, P = 0.04)., Conclusions: A1AT non-M alleles are more frequent in children with chronic liver disease than in the general population. We speculate that these non-M alleles may act as genetic modifiers in pediatric liver disease in general and modulate disease progression in children with biliary atresia in particular.

Purpose: Total parenteral nutrition (TPN) has prolonged survival in children with intestinal failure; however, end-stage liver disease owing to TPN-induced cholestasis (ESLD-TPN) may preclude its use. ESLD-TPN is an indication for isolated liver transplantation (ILT) or multivisceral transplantation (MVT). Isolated liver transplantation for ESLD-TPN should only be considered in patients who have the potential for enteral autonomy., Methods: We retrospectively reviewed the records of patients with ESLD-TPN who underwent ILT (n = 7) or MVT (n = 5) between 1994 and 2005. The median age at the time of transplantation was 10.0 months. Intestinal failure followed necrotizing enterocolitis (n = 3), gastroschisis (n = 3), gastroschisis with volvulus (n = 3), gastroschisis with atresia (n = 1), malrotation (n = 1), and megacystis microcolon intestinal hypoperistalsis syndrome (n = 1)., Results: Isolated liver transplant patients had a median length of small bowel of 70 cm and tolerated a median of 50% of enteral calories. The median length of small bowel in patients who underwent MVT was 29 cm, and none tolerated more than 30% of goal enteral feeds. Reduced-size (n = 5) and whole-liver (n = 2) allografts were used for patients undergoing ILT. Patients undergoing MVT received liver-small bowel-pancreas (n = 4) or liver-small bowel-pancreas-colon (n = 1). Overall patient survival was 57% in ILT (median follow-up = 25.1 months); 3 survivors are TPN independent, and the fourth patient requires TPN 3 days/wk. Patient survival was 40% after MVT (median follow-up = 13.0 months); 1 MVT patient died of abuse 16.9 months after transplant and was TPN independent at the time of death. Both survivors are TPN independent. Bilirubin levels are within normal range in all survivors., Conclusion: Isolated liver transplantation for ESLD-TPN in the setting of intestinal failure is a viable option in patients who have the potential for enteral autonomy. Multivisceral transplantation is the only alternative in patients without the potential for intestinal recovery. Survival can be achieved in patients with ESLD-TPN, but mortality remains high for both procedures.

Liver transplantation has become the accepted standard of care in the treatment of a child with a failing liver. Advances in the management of critical care and immunosuppression along with the development of innovative operative procedures have improved outcome such that 5-year survival rates of 80% to 90% are expected following liver transplantation. Organ allocation schemes have evolved in an effort to better stratify recipient risk thereby more appropriately distributing deceased donor grafts. A persistent shortage of appropriate donors continues to contribute to patient mortality. The consequences of long-term immunosuppression have become increasingly apparent such that health care providers need to be aware of the side effects of chronic immunosuppression. New strategies need to be defined to minimize the need of continuous immunosuppression. The continued success of pediatric liver transplantation will require multi-disciplinary health care teams comprised of general pediatricians, pediatric hepatologists, transplant surgeons, and transplant coordinators who focus on the complex needs of the transplant recipient.

Malignancy is a well defined complication of chronic immunosuppression. Post transplant malignancies appear to be related to cumulative doses of immunosuppression, and in pediatric patients, acute infection of previously naive patients. The most commonly encountered malignancy in this age population is Post Transplant Lymphoproliferative Disorder (PTLD). PTLD is not a single entity but rather represents a continuum of disease. Treatment of PTLD should be initiated with immunosuppression reduction. Standard dose chemotherapy leads to significant morbidity. With the introduction of anti-CD20 antibody treatment with rituximab, chemotherapy has become second line therapy. The occurrence of solid malignancy appears to be associated with chronic immunosuppression. These cancers include those of skin, gynecologic organs, and the rectum, all of which appear to have the strongest association with viral mediators. Several strategies have been postulated to minimize the occurrence of malignancy. These include ganciclovir prophylaxis for the prevention of PTLD and the use of mychophenolic acid and TOR inhibitor maintenance to diminish the incidence of PTLD and solid malignancies. This leaves transplant physicians with several new and novel immunosuppressive agents with uncertain oncologic potentials that will need to be examined over the next decade.

Objective: To determine the prevalence and identify variables associated with renal dysfunction in long-term survivors of pediatric liver transplantation., Study Design: Data from 117 patients who survived>or=3 years after liver transplantation were analyzed. Demographic and clinical information was obtained from chart review and from a clinical care database. The dependent variable was renal function as determined by measured glomerular filtration rate (mGFR). Univariate and multivariate analyses were performed to identify independent variables associated with renal dysfunction (mGFR<70 mL/min per 1.73 m2)., Results: The average time since liver transplant was 7.6+/-3.4 years (range, 3 to 14.6 years). When the last available mGFR for all patients was analyzed, renal dysfunction was present in 32%. In the univariate analysis, mGFR at 1 year after transplant, cyclosporine immunosuppression, and time since transplant were significant; the second two were strongly collinear. Using multiple logistic regression modeling excluding time since transplant, cyclosporine and mGFR at 1 year after transplant were strongly associated with renal dysfunction., Conclusions: Renal dysfunction is a common complication in children who survive liver transplantation. Our observations are of critical importance because children may live long enough to move from a stage of renal insufficiency characterized by asymptomatic decreased GFR to symptomatic end-stage renal disease.

Unlabelled: During repair of an esophageal atresia with tracheoesophageal fistula (EA/TEF), a right-sided aortic arch (RAA) presents a technical challenge. We reviewed our experience with EA/TEF focusing on the impact of RAA., Methods: A retrospective review of patients with EA/TEF at our institution from 1990 to 2004 was performed. Of 61 patients, 53 had a left aortic arch (LAA) and 8 had RAA., Results: The mean gestational age and birth weight were similar between patients with LAA and RAA. There were more vascular rings in patients with RAA than with LAA (37.5% vs 3.8%, P = .009). A preoperative echocardiogram correctly identified the location of the arch in all with LAA but only 62.5% of those with RAA. Operative complications occurred in 25% of patients with RAA and 11% of patients with LAA., Conclusions: Management of a child with RAA and EA/TEF is technically challenging. Preoperative identification of the great vessel anomalies may impact operative decision making. Therefore, careful diagnostic evaluation of a patient with an aortic arch that cannot be easily defined by ECHO should be considered before definitive repair.

In the past two decades, pediatric liver transplantation has become the state-of-the-art operation with anticipated success and limited mortality. The future success of pediatric liver transplantation will require thoughtful solutions to the delicate balance of risk to donors and recipients, the complex needs of the acute postoperative patient, and the long-term challenges of chronic immunosuppression in these previously unsalvageable patients.

Background: Although surgery has been the mainstay of treatment of chondrogladiolar pectus carinatum (PC), several authors have advocated the benefits of nonoperative approaches to induce chest wall remodeling. Based on our initial success with compression bracing, we have integrated this modality into our treatment algorithm., Method: We reviewed the charts of all patients treated for PC at our pediatric hospital between 1997 and 2004. Patients were managed with observation, operative repair, and orthotic bracing that provides continuous anteroposterior sternal compression. The brace was worn for 14 to 16 hours per day until linear growth was complete or for a minimum of 2 years., Results: One hundred patients were diagnosed with PC. Fifty-seven patients had no treatment and were monitored. Twenty-nine patients were fitted with a brace. Of these 29 patients, 3 were noncompliant, resulting in a compliance rate of 90%. Of the remaining brace patients, all have had positive outcomes with no observed complications. Seventeen patients underwent surgical repair. Their outcomes were also positive with no major complications., Conclusion: Our findings clearly demonstrate that compression bracing is a safe and effective treatment for children with chondrogladiolar PC. We currently offer this approach as a first-line treatment, reserving surgery for patients who are noncompliant and those who fail the nonoperative modality.

Purpose: To evaluate the efficacy of a low-dose chemotherapy regimen in children with Epstein-Barr virus (EBV) -positive, post-transplantation lymphoproliferative disease (PTLD) after organ transplantation who have experienced failure with front-line therapy for PTLD., Patients and Methods: Eligible patients received cyclophosphamide (600 mg/m2 intravenous for 1 day) and prednisone (2 mg/kg orally for 5 days) every 3 weeks for six cycles., Results: Thirty-six patients treated on study were assessable for analyses. Front-line therapies for PTLD before study entry included immune suppression reduction or withdrawal (n = 36), antiviral therapy (n = 33), surgical resection (n = 8), rituximab (n = 2), and interferon alfa (n = 1). Reasons for failure of front-line therapy included progressive disease (PD; n = 33) and persistent disease with concurrent allograft rejection (n = 3). Thirty patients (83%) had stage III to IV disease, 92% had extranodal disease, and 75% had > or = three sites of disease. The overall response rate was 83% (75% complete response + 8% partial response). The relapse rate was 19%, with only one of five relapsed patients alive and disease-free. Four patients presented with fulminant, disseminated PTLD; only one of these four patients achieved a response, and all four died of PD. Two patients died of treatment-related toxicity. Three patients (8%) experienced allograft loss, but two of the three patients are alive and disease-free after a second transplantation. The 2-year overall, relapse-free, and failure-free (without PTLD and with functioning original allograft) survival rates were 73%, 69%, and 67%, respectively., Conclusion: This low-dose chemotherapy regimen is effective for children with EBV-positive, nonfulminant PTLD who have experienced treatment failure with front-line therapy, and this study represents the largest series of PTLD patients treated prospectively with a uniform chemotherapy regimen.

Objective: To apply the principles of statistical process control (SPC) to manage calcineurin inhibitor (CNI) blood levels. We hypothesized that the use of SPC would increase the proportion of CNI blood levels in the target range., Study Design: The study population consisted of 217 patients more than 3 months after liver transplantation. After demonstration of proof of concept using the rapid cycle improvement process, SPC was applied to the entire population. The change package included definition of target ranges for CNI, implementation of a web-based tool that displayed CNI blood levels on a control chart, and implementation of a protocol and a checklist for management of CNI blood levels. The principal outcome measure was the proportion of CNI blood levels in the target range., Results: In the pilot study, the proportion of CNI blood levels in the target range increased from 50% to 85%. When the protocol was spread to the entire population, the proportion of drug levels in the target range increased to 77% from 50% (P < .001), whereas the range of CNI levels decreased. The rate of allograft rejection did not change., Conclusions: Utilization of SPC increased the proportion of CNI blood levels in target range. These observations may be applicable to the care of other chronic healthcare problems.

Tailgut cyst, or retrorectal cystic hamartoma, is a rare congenital lesion found in the presacral space. The lesion has been infrequently reported in the literature. We report the MRI findings of a tailgut cyst in a 2-year-old girl who presented with a sacral dimple and skin discoloration.

Objective: To compare the direct health care cost of living donor liver transplantation (LDLT) with that of cadaver donor liver transplantation (CDLT) in children and identify predictors of cost., Study Design: All 16 children who underwent LDLT from January 1997 through January 2002 at Cincinnati Children's Hospital Medical Center comprised the study population. They were matched for age, diagnosis, and nutritional status with 31 children who received CDLT during the same era. A historic cohort analysis was performed., Results: There was no difference in the 1-year mortality rates between both groups. Costs associated with graft retrieval contributed 15.3% and 31% of the initial transplant cost for LDLT and CDLT, respectively. Mean cost of care in the first year was 60.3% higher for LDLT than CDLT (P=.01). Multivariate analysis identified biliary complications and insurance status as predictors of cost for initial transplantation (R(2)=0.57), whereas biliary complications and pediatric end stage liver disease scores were identified as predictors of cost of care in the first year after transplantation (R(2)=0.77)., Conclusions: The comprehensive cost of LDLT in the first year after transplantation is higher than cadaveric transplantation. This must be balanced against the time spent and care needs of patients on the waiting list.

Our primary goal was to assess health related quality of life (HRQOL) at transplantation and 1 yr after transplantation in pediatric liver transplant patients aged less than 5 years. We conducted a prospective longitudinal study of HRQOL in pediatric liver transplant recipients, aged less than 5 years to define the impact of liver transplantation on HRQOL and identify factors that predict HRQOL after transplantation. The infant toddler health status questionnaire (ITHQ) was completed at the time of listing for liver transplantation and at 6 and 12 months after liver transplantation. The primary outcome measures were the subscale scores that comprise ITHQ. The mean age (+/-s.e.m.) of the enrolled patients (n = 45) at transplantation was 1.4 (+/-1.2) yr. Thirty-eight (84%) of the enrolled patients completed the study. The highest mean baseline scores of 78.6 (+/-3.3) were for global mental health (GlobalMH). ITHQ subscale scores increased steadily after transplantation. The greatest increase was in the first 6 months after transplant. At 1 yr after transplantation, there were significant increases in all of the ITHQ subscale scores except for GlobalMH. ITHQ subscales were similar for patients who received LDLT compared with those who received cadaver donor liver transplantation (CDLT) at baseline and a year after transplant. Time elapsed as transplantation was a significant predictor of functional health in all of the models generated. Scores for general health (GH), global health (GGH), parental time-impact (PT) and parental time-emotion (PE) were higher for male children. Family cohesion (FC) improved with time elapsed since transplant and increased number of inpatient days. HRQOL improves after transplantation in all of our patients irrespective of the donor type. Functional health scores were higher in patients with normal serum bilirubin at 1 yr post-transplant. Assessment of HRQOL should be an integral part of care for liver transplant patients and their caregivers.

As survival rates following liver transplantation have increased, health care providers must assess the impact of transplantation on dimensions other than traditional medical measures. Hearing impairment may adversely impact social, emotional, cognitive, academic, and speech and language development. We hypothesized that children who undergo liver transplantation are at risk for hearing impairment due to exposure to ototoxic drugs. We conducted a review of 74 children who had undergone liver transplantation between December 1996 and September 2000 at Cincinnati Children's Hospital Medical Center. Hearing was assessed at discharge by an audiologist using age and developmentally appropriate techniques. The principal outcome measure was sensorineural hearing impairment. Independent variables were age at transplantation, United Network for Organ Sharing (UNOS) status at transplantation, primary diagnosis, post-transplant length of hospital stay, days of treatment with aminoglycosides, and days of treatment with loop diuretics. Eleven of 74 children (15%) had sensorineural hearing loss, of whom four had severe to profound hearing loss. Multivariate analyses showed that the adjusted relative risk for hearing loss in patients with hepatoblastoma was 66 and that there was a 5% increase risk for hearing loss for each additional day of hospitalization. Age at transplantation, UNOS status, and days of treatment with loop diuretics or aminoglycosides did not achieve significance in the model. Sensorineural hearing impairment occurs in a subset of pediatric patients following liver transplantation. Patients with hepatoblastoma or those who experience prolonged hospitalization after transplantation are at increased risk. Our observations are of particular importance for pediatric liver transplant recipients since the median age at transplantation is 12-18 months, a critical period for language acquisition.

Background: Previous studies have suggested an increased risk of Epstein-Barr virus-associated posttransplant lymphoproliferative disorder (EBV-PTLD) in patients receiving tacrolimus for immunosuppression. We hypothesized that after correction for confounding variables, immunosuppression with tacrolimus is not associated with an increased risk of EBV-PTLD., Methods: Potential cases of EBV-PTLD, identified by chart review, were independently ascertained by three clinicians and defined using published criteria. Agreement in diagnosing EBV-PTLD was measured using Kappa coefficients. Unadjusted and adjusted relative risk estimates were determined using proportional hazards regression., Results: Twenty-three cases of EBV-PTLD were identified in 221 patients, a proportion of 10.4% (95% confidence interval [CI]: 6.4%-14.4%). Multivariable analysis revealed that immunosuppression with tacrolimus was associated with an increased risk of EBV-PTLD (relative risk 3.10: 95% CI: 1.21-7.92), as was age at transplantation as a continuous variable (parameter estimate -0.15, P=0.03). Kappa coefficients in diagnosing EBV-PTLD and subclassifying as neoplastic and non-neoplastic EBV-PTLD were 0.73 (95% CI: 0.54-0.93) and 0.54 (95% CI: 0.40-0.68), respectively. Patients with neoplastic PTLD demonstrated a lower probability of survival than patients with non-neoplastic PTLD and non-cases., Conclusions: Immunosuppression with tacrolimus and young age at transplantation are associated with an increased risk of EBV-PTLD in children undergoing liver transplantation, although we cannot exclude detection bias as an explanation for this observed increase. Good agreement between observers can be achieved using previously published criteria for defining EBV-PTLD. Patients with neoplastic EBV-PTLD may have a worse prognosis, and thus identification of risk factors for the development of this subtype of the disorder may be more important.

We sought to estimate the prevalence of reduced bone mass, defined by lumbar spine bone mineral density (LS-BMD) (z-score < -2.0), and to determine the factors associated with LS-BMD after liver transplantation in children and adolescents. LS-BMD z-scores were measured in a sample of subjects who had undergone liver transplantation in childhood or adolescence using dual energy x-ray absorptiometry (DXA). One hundred nine patients underwent DXA. The mean age at transplantation was 4.3 years (median, 1.8 years), and mean duration since transplantation was 6.2 years (median, 5.8 years). The mean weight z-score was -0.130 (SD, 1.26). The mean LS-BMD z-score was -0.243 (SD, 1.27). Eight patients, or 7.3% (95% confidence interval [CI], 2.4% to 12.2%), had reduced bone mass. Compared with those without reduced bone mass, subjects with reduced bone mass were more likely to have been treated for rejection at least once (87.5% versus 51.5%; P =.07), and had greater cumulative exposure to prednisone during the year before DXA (92.3 versus 26.2 mg/kg/y; P =.001). Multiple linear regression determined that LS-BMD z-score was positively associated with time since transplantation and weight z-score and negatively associated with cumulative prednisone exposure. Serum 25-OH vitamin D was measured and reduced (< 15 ng/mL) in 5 of 87 patients, one of whom had reduced bone mass. In conclusion, the prevalence of reduced bone mass was 7.3% in our population. Only certain patients appear to be at risk for low BMD, including those with a history of rejection. Screening for reduced bone mass may be appropriate for these patients.

The goals of the present study were (1) to measure health-related quality of life (HRQOL) in pediatric liver transplantation (LT), (2) to identify demographic and clinical factors that correlate with HRQOL, and (3) to compare two instruments that have been used to measure HRQOL in children and adolescents. We conducted a single-center cross-sectional study of 77 pediatric LT recipients ages 5 to 18 years, all of whom had had LT at least 6 months previously. We used the Child Health Questionnaire Parent Form 50 (CHQPF50) and the PedsQL4.0 to determine measured dimensions of physical and psychosocial health from the parents' perspective. Individual scale scores range from 0 to 100, with higher scores reflecting better health. Data on demographics, clinical status at transplantation, posttransplantation clinical course, and graft function were collected to identify predictors of posttransplantation HRQOL. Fifty-three percent of the liver transplant recipients had biliary atresia, 78% were white, and 61% were female. The mean age at LT was 3.8 +/- 3.6 years, and the range of time since LT was 1 to 15 years. HRQOL in pediatric liver transplant recipients was lower than that reported for healthy children but similar to that for children with other chronic illness. Age at transplantation, the time elapsed since transplantation, hospitalizations within the previous year, maternal education, and race were significant predictors of physical health. Age at transplantation and maternal education predicted psychosocial function. HRQOL was decreased in a population of pediatric liver transplant recipients compared with the general population and similar to that for children with chronic illness. Prospective longitudinal studies will permit us to define predictors of HRQOL at different periods of time after transplantation. The information gained from this study will help us to better define expectations and the clinical course after liver transplantation to patients and their families.

Children (defined as under 18 yr of age) account for approximately 12.5% of all liver transplants in the United States. Even though the annual number of liver transplantation procedures remains relatively constant, the population of long-term survivors of liver transplantation has grown. Presently, the population of long-term survivors of liver transplantation is 10-fold greater then the number of transplantations carried out each year. For long-term survivors of liver transplantation, the goal is to maintain graft function and wellness while decreasing the morbidity associated with long-term immunosuppression. The primary diagnosis leading to liver transplantation in children do not recur in the allograft. Consequently, many of the complications of liver transplantation, both early and long term, relate to the need for immunosuppression. Children may be at increased risk to develop significant end-organ damage as a result of increased serum lipid levels, elevated blood pressure, altered glucose metabolism, decreased renal function, cancer, and diminished bone accretion that occur as a result of immunosuppressive therapy or complications of therapy. As survival rates have increased, health care providers have begun to assess health-related quality of life. We will review our current knowledge of long-term outcome following pediatric liver transplantation in children.

Therapeutic options for children with portal hypertension now include a broad range of pharmacologic, endoscopic, and surgical procedures. Thoughtful application of all of these options can improve quality of life by decreasing the complications of portal hypertension and can decrease mortality by preventing the consequences of variceal hemorrhage. The development of portal hypertensive gastropathy following palliative procedures such as endoscopic sclerotherapy and band ligation may limit their long-term success in children. The excellent results now obtained with selective portosystemic shunts and liver transplantation assure that definitive surgical treatments will continue to be important components in the treatment of children with portal hypertensive complications or progressive liver disease. Evolving procedures, such as TIPS, represent excellent short-term life-preserving techniques to stabilize critically ill patients while awaiting liver transplantation. Their role in the future, long-term management of children is yet to be defined.

Prostaglandin E1 (PGE1) and N-acetylcysteine (NAC) have been used as single agents to decrease reperfusion injury and improve outcome after solid-organ transplantation (Tx). We hypothesized that combined treatment with NAC and PGE1 would be safe and reduce reperfusion injury. We therefore carried out a pilot study to assess the safety of this drug combination and gain information regarding the efficacy of treating pediatric liver transplant recipients with NAC and PGE1. The pilot study took the form of an open-label study incorporating 25 pediatric liver transplant recipients (12 children in the treatment group and 13 children as controls). NAC (70 mg/kg) was given intravenously over 1 h following reperfusion and then every 12 h for 6 days. PGE1 (0.4 mg/kg/h) was given as a continuous intravenous infusion for 6 days, starting after the first NAC dose. The primary outcome was the safety of combined treatment with NAC and PGE1. Patient survival, graft survival, allograft rejection within the first 90 days after Tx, peak post-transplant serum alanine aminotransferase (ALT) concentration, post-transplant length of hospitalization, and post-operative complications were secondary outcomes. Post-operative complications occurred at similar rates in both control and treated groups. No complications or adverse events occurred in the treated group as a result of study drugs. The 3-month patient survival rate was 100% for both groups. For the group treated with NAC and PGE1, peak serum ALT was lower and median length of stay was shorter but the differences did not reach statistical significance. The proportion of patients with allograft rejection was not significantly different between the two groups. However, rejection was more severe in the control group than in the treated group. In summary, infusions of NAC and PGE1 were safely administered to pediatric liver transplant recipients. However, a randomized controlled study is needed to determine the efficacy of treatment with NAC and PGE1.

Objective: Efforts to decrease the cost of orthotopic liver transplantation (OLT) must address the impact of specific interventions on clinical outcome. We hypothesized that an intervention designed to decrease the length of hospitalization would reduce costs without jeopardizing clinical outcome. We further sought to identify predictors of length of stay and cost for hospitalization after liver transplantation., Methods: The study group included 47 children who underwent OLT from September 1996 to April 1999, and the control group included 36 children who underwent OLT from March 1994 to August 1996. The intervention was a transition to home program in which patients were discharged to a family living center when they met established clinical criteria and their families met predefined educational goals. We analyzed patients who survived 3 months after OLT., Results: For the intervention group, the mean length of stay, total costs, and surgical costs were 29%, 36%, and 34% lower, respectively. Organ type, height z score, race, hepatic artery thrombosis, early allograft rejection, and participation in the transition to home program predicted length of stay and total costs., Conclusion: An early discharge program based on defined criteria can be used to decrease length of stay and cost after OLT without jeopardizing clinical outcome.

Background: Chronic intrahepatic cholestasis is associated with severe pruritus that is often refractory to maximal medical management and leads to significantly impaired quality of life. The hypothesis in this study was that partial external biliary diversion (PEBD) can substantially improve intractable pruritus secondary to intrahepatic cholestasis with subsequent improvement of functional quality of life., Methods: Parents' and/or patients' clinical rating of pruritus, growth percentiles, biochemical parameters, and liver biopsies performed before and after surgery were compared in a retrospective medical record review., Results: Eight children underwent PEBD from 1990 through 1997. Complete follow-up data were available for seven patients. Before surgery, all patients had intense pruritus, which was not responsive to maximal medical therapy. Specimens obtained in preoperative liver biopsies showed moderate (n = 1), minimal (n = 6), or no (n = 1) portal fibrosis. After PEBD, all patients received ursodeoxycholic acid (10-15 mg/kg/dose two to three times daily) until resolution of pruritus. Of the seven patients with complete follow-up data, six had complete resolution of pruritus and sustained resolution up to 8 years after surgery. The patient with mild to moderate residual pruritus was the youngest to undergo PEBD. Growth improved from below the 5th percentile before surgery to the 5th through the 25th percentiles for five of six patients with more than 6 years' follow-up. All families reported improved quality of life, defined by school attendance and ability to resume normal activity with peers. There has been no clinical evidence of progression of liver disease., Conclusion: Partial external biliary diversion is effective in the long-term treatment of pruritus refractory to medical therapy and provides a favorable outcome in a select group of patients with chronic intrahepatic cholestasis without cirrhosis.

Background: Liver transplantation (LT) remains a high-risk operation, especially during the first months after LT when technical complications and preexisting illness exert their influence on survival. However, there are late deaths. The authors have reviewed their experience to identify factors impacting on long-term survival., Methods: A total of 150 patients who had undergone liver transplantation over an 11-year period were reviewed. Thirty-three patients died after LT (22%). Of these, 18 of 33 (55%) died in the first 3 postoperative months. One hundred thirty-two patients survived beyond 3 months, and 15 patients (11%) suffered late deaths. This review concentrates on the latter group., Results: The primary cause of death was sepsis in 11 of 15 (73%). In two, sepsis complicated retransplantation in chronically debilitated patients. Two additional patients had late-presenting postoperative complications (bile leak or abscess, intestinal obstruction with perforation). In two cases, pneumocystis carinii pneumonia occurred; noncompliance or unplanned discontinuation of prophylaxis was directly responsible. Multiple organ system failure from presumed immunoincompetence developed in four patients; one had undergone bone marrow transplantation for aplastic anemia (AA) after fulminant hepatic failure (FHF). Lymphoproliferative disease (LPD) was the cause of death in 3 of 15 cases (20%). In only three cases was the cause of death related to the patient's primary disease (chronic hepatitis, Alper's syndrome or seizures, and AA with FHF). Pretransplant diagnosis, and UNOS status at the time of LT did not influence the long-term survival., Conclusions: Long-term survival in patients who have undergone LT was compromised by immunosuppressive complications and sepsis. Early mortality factors, such as UNOS status, age at LT, primary diagnosis, and technical complications do not predict late deaths. In children who adhere to their medical regimen and have good initial allograft function, late postoperative infection, especially with Ebstein-Barr virus, accounts for most of the late mortality. Improved and decreased immunosuppression may further improve these long-term results.

Results show that the use of sequential surgical treatment, employing Kasai portoenterostomy in infancy, followed by selective liver transplantation for children with progressive hepatic deterioration yields improved overall survival. All children with successful Kasai portoenterostomy procedures who do not require OLT are survivors. Using newer transplant techniques, the 5-year survival rate for children who receive transplants with a primary diagnosis of biliary atresia was 82%. This yields an overall survival rate of 86% in this entire study population. Limited donor availability and increased complications after liver transplantation in infants less than 1 year of age mitigate against the use of primary liver transplantation without prior portoenterostomy for infants with biliary atresia. At present, these two operative procedures should be used as sequential and complementary modes of treatment rather than as competitive procedures. When biliary atresia is not recognized in infancy and established cirrhosis has resulted, primary transplantation should be offered as the initial surgical treatment.