Laparoscopic donor nephrectomy for the pediatric recipient population: Risk factors for adverse outcomes.

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.

Management and long-term consequences of portal vein thrombosis after liver transplantation in children.

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.

The beneficial impact of revision of Kasai portoenterostomy for biliary atresia: an institutional study.

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.

A welcome surprise: nodular fasciitis presenting as soft tissue sarcoma.

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.

Anatomic and histologic variability of epithelial apoptosis in small bowel transplants.

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.

Pectus excavatum imaging: enough but not too much.

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.

Management of small bowel volvulus around feeding Roux-en-Y limbs.

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.

Redesigning intensive care unit flow using variability management to improve access and safety.

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.

Reducing surgical site infections at a pediatric academic medical center.

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.

The role of surgical shunts in the treatment of pediatric portal hypertension.

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.

A national conference to determine research priorities in pediatric solid organ transplantation.

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.

Outcomes following liver transplantation.

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.

Improving transitions of care between the operating room and intensive care unit.

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.

High prevalence of alpha-1-antitrypsin heterozygosity in children with chronic liver disease.

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.

Financial impact of failing to prevent surgical site infections.

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.

Low-dose chemotherapy for Epstein-Barr virus-positive post-transplantation lymphoproliferative disease in children after solid organ transplantation.

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.

Pediatric liver transplantation.

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.

Pediatric liver transplantation.

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 in pediatric transplant recipients.

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.

Determination of risk factors for Epstein-Barr virus-associated posttransplant lymphoproliferative disorder in pediatric liver transplant recipients using objective case ascertainment.

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.