Navigating Inconclusive Upper-Gastrointestinal Series in Infantile Bilious Vomiting: A Case Series on Intestinal Malrotation.

BACKGROUND Bilious vomiting in a child potentially portends the dire emergency of intestinal malrotation with volvulus, necessitating prompt surgical management, with differentials including small-bowel atresia, duodenal stenosis, annular pancreas, and intussusception. Although the upper-gastrointestinal series (UGI) is the diagnostic investigation of choice, up to 15% of the studies are inconclusive, thereby posing a diagnostic challenge. CASE REPORT We report a case series of 3 children referred for bilious vomiting, whose initial UGI was inconclusive and who were eventually confirmed to have intestinal malrotation at surgery. The first child was a female born at 37 weeks with antenatally diagnosed situs inversus and levocardia, who developed bilious vomiting on day 1 of life. The duodenojejunal flexure (DJ) could not be visualized on the UGI because of faint opacification on first pass of the contrast and subsequent overlap with the proximal jejunal loops. The second child was a male born at 36 weeks, presenting at age 4 months with bilious vomiting of 2 days duration. The third child was a female born at 29 weeks, presenting with bilious aspirates on day 3 of life. UGI for all 3 showed persistent hold-up of contrast at the proximal duodenum with no opacification of the distal duodenum or small bowel.Adjunctive techniques during the UGI and ultrasound examination helped achieve a preoperative diagnosis of malrotation in these children. CONCLUSIONS Application of diagnostic adjuncts to an inconclusive initial UGI may help elucidate a preoperative diagnosis of intestinal malrotation in infantile bilious vomiting.

An Intelligent Customer-Driven Digital Solution to Improve Perioperative Health Outcomes Among Children Undergoing Circumcision and Their Parents: Development and Evaluation.

BACKGROUND: Circumcision as a common elective pediatric surgery worldwide is a stressful and anxiety-inducing experience for parents and children. Although current perioperative interventions proved effective, such as reducing preoperative anxiety, there are limited holistic solutions using mobile apps. OBJECTIVE: This paper aims to describe the development and primary evaluation of an intelligent customer-driven smartphone-based app program (ICory-Circumcision) to enhance health outcomes among children undergoing circumcision and their family caregivers. METHODS: Based on the review of the literature and previous studies, Bandura's self-efficacy theory was adopted as the conceptual framework. A multidisciplinary team was built to identify the content and develop the apps. Semistructured interviews were conducted to evaluate the ICory-Circumcision. RESULTS: The ICory-Circumcision study was carried out from March 2019 to January 2020 and comprised 2 mobile apps, BuddyCare app and Triumf Health mobile game app. The former provides a day-by-day perioperative guide for parents whose children are undergoing circumcision, while the latter provides emotional support and distraction to children. In total, 6 participants were recruited to use the apps and interviewed to evaluate the program. In total, 4 main categories and 10 subcategories were generated from content analysis. CONCLUSIONS: ICory-Circumcision seemed to lean toward being useful. Revisions to ICory-Circumcision are necessary to enhance its contents and features before advancing to the randomized controlled trial. TRIAL REGISTRATION: ClinicalTrials.gov NCT04174404; https://clinicaltrials.gov/ct2/show/NCT04174404.

Comparing the Performance of Multiple Small-Data Personalized Tacrolimus Dosing Models for Pediatric Liver Transplant: A Retrospective Study.

Tacrolimus is a potent immunosuppressant used after pediatric liver transplant. However, tacrolimus's narrow therapeutic window, reliance on physicians' experience for the dose titration, and intra- and inter-patient variability result in liver transplant patients falling out of the target tacrolimus trough levels frequently. Existing personalized dosing models based on the area-under-the-concentration over time curves require a higher frequency of blood draws than the current standard of care and may not be practically feasible. We present a small-data artificial intelligence-derived platform, CURATE.AI, that uses data from individual patients obtained once daily to model the dose and response relationship and identify suitable doses dynamically. Retrospective optimization using 6 models of CURATE.AI and data from 16 patients demonstrated good predictive performance and identified a suitable model for further investigations.Clinical Relevance- This study established and compared the predictive performance of 6 personalized tacrolimus dosing models for pediatric liver transplant patients and identified a suitable model with consistently good predictive performance based on data from pediatric liver transplant patients.

Enhanced recovery after surgery in children undergoing abdominal surgery: meta-analysis.

BACKGROUND: Enhanced recovery after surgery (ERAS) is a multimodal approach that streamlines patient processes before, during, and after surgery. The goal is to reduce surgical stress responses and improve outcomes; however, the impact of ERAS programmes in paediatric abdominal surgery remains unclear. The authors aimed to review the effectiveness of ERAS on clinical outcomes in children undergoing abdominal surgery. METHOD: CINAHL, CENTRAL, Embase, ProQuest, PubMed, and Scopus were searched for relevant studies published from inception until January 2021. The length of hospital stay (LOS), time to oral intake, time to stool, complication rates, and 30-day readmissions were measured. Meta-analyses and subgroup analyses were conducted using RevMan 5.4 with a random-effects model. RESULTS: Among 2371 records from the initial search, 111 articles were retrieved for full-text screening and 12 were included for analyses. The pooled mean difference (MD) demonstrated reduced LOS (MD -1.96; 95 per cent c.i. -2.75 to -1.17), time to oral intake (MD -3.37; 95 per cent c.i. -4.84 to -1.89), and time to stool (MD -4.19; 95 per cent c.i. -6.37 to -2.02). ERAS reduced postoperative complications by half and 30-day readmission by 36 per cent. Subgroup analyses for continuous outcomes suggested that ERAS was more effective in children than adolescents. CONCLUSION: ERAS was effective in improving clinical outcomes for paediatric patients undergoing abdominal surgery.

Percutaneous transhepatic interventional therapy of portal vein stenosis in paediatric liver transplantation: A systematic review of efficacy and safety.

PURPOSE OF THE SYSTEMATIC REVIEW: To determine the efficacy and safety of percutaneous trans-hepatic balloon and/or stent angioplasty (PTA) in the management of portal vein (PV) stenosis following paediatric liver transplantation. METHODS: Articles were included from a systematic search of Medline, Embase, Cochrane CENTRAL, ClinicalTrials.gov and International Clinical Trials Registry from inception to the 29th of August 2020. RESULTS: There were 213 paediatric liver recipients who underwent PTA for PV stenosis in 19 included studies published between 1991 and 2019. Balloon angioplasty was the initial treatment in the majority (n = 153). Primary stent placement (n = 34) was performed for elastic recoil, intimal tears and PV kinks and rescue stent placement (n = 14) for recurrent PV stenosis following primary balloon angioplasty. The technical success was 97.6%-100% overall, 97.6%-100% for balloon-angioplasty-only and 100% for primary stenting. The clinical success was 50%-100% overall, 50%-100% for balloon-angioplasty-only and 100% for primary stenting. Long-term PV patency was 50%-100% overall, 37.5%-100% for balloon-angioplasty-only and 100% for primary stenting. Primary balloon angioplasty was successful in 78% of the cases. Of the recurrent PV stenoses, 9% resolved with stent placement and one required a meso-Rex shunt. There was one re-transplantation without stenting. The complication rate was 2.6% for balloon-angioplasty-only (bleeding, liver abscess, 2 PV thromboses) and 5.9% for primary stenting (bleeding, stent-fracture). There was no procedure-related mortality. CONCLUSION: Percutaneous transhepatic balloon angioplasty may be the initial management of portal vein stenosis in paediatric liver recipients. Stent placement may be a primary option in selected cases and a reliable rescue option for recurrent portal vein stenosis following balloon-angioplasty-only.

Successful resumption of peritoneal dialysis following living donor liver transplantation in children with end-stage renal disease.

Children with ESRD in need of RRT are commonly managed by PD due to difficulty with vascular access for HD and the relatively large extracorporeal blood volume required. Major abdominal surgery may result in injury to the peritoneum and consequent adhesion, thereby resulting in a reduction in the anatomical capacity and transport capability across the peritoneal membrane. Here, we report successful resumption of PD after LDLT in two pediatric patients. The causes of ESRD were PH1 and juvenile nephronophthisis, respectively. Both patients were managed by PD prior to LDLT. PD was converted to HD starting three days before LDLT and was continued postoperatively until resumption of PD on days 13 and 28, respectively. The PD weekly Kt/V urea was maintained before and after LDLT. The patients continued to do well on PD without complications. Meticulous intra-operative techniques during LDLT allow postoperative PD resumption by preservation of peritoneal integrity with effective transport capability and without added risk of peritonitis.

Benefit of early inflow exclusion during living donor liver transplantation for unresectable hepatoblastoma.

BACKGROUND: Hepatoblastoma (HB) is a highly malignant primary liver tumor in children. Although liver transplantation (LT) is an effective treatment for unresectable HB with good long-term outcomes, post-transplant survival is mainly affected by recurrence, despite adjuvant chemotherapy. Novel strategies are needed to improve the outcomes in patients undergoing LT for unresectable HB. PATIENTS AND METHODS: Twelve children received LT for unresectable HB. In 9 patients, we applied early exclusion of hepatic inflow (hepatic artery and portal vein) and creation of a temporary portocaval shunt during LT. RESULT: There were differences in the duration of and the blood loss during operation as compared with previously reports. The estimated glomerular filtration rate was well preserved at 3, 6, and 12months and the latest follow-up after LT, and the recurrence-free survival was 88.9%. CONCLUSION: Early inflow control during LT for unresectable HB may benefit recurrence-free survival by minimizing blood loss and tumor dissemination, preserving renal function and allowing early adjuvant chemotherapy.

Reno- and splenoportal anastomosis for a retransplant patient with situs inversus.

PV thrombosis following pediatric LT is a serious complication that may lead to graft loss. LDLT poses limitations with regard to the availability of vein grafts for complex PV reconstructions. We herein report a unique reconstruction of the PV inflow in a one-yr-old boy with situs inversus undergoing re-LDLT. The inflow was derived from the SPV and the RRV. A common channel was created utilizing a donor IMV and the recipient explant LHV as vascular conduits. With the application of innovative surgical reconstructions, pre-existing portomesenteric thrombosis may be amenable to re-LDLT in the pediatric population.

Chemotherapy-induced B-cell depletion in hepatoblastoma patients undergoing ABO-incompatible living donor liver transplantation.

LT from ABO-I donors requires preconditioning regimens to prevent postoperative catastrophic AMR. NAC for HBL is known to cause myelosuppression leading to a reduction in the number and function of lymphocytes. We investigated this chemotherapy-induced myelosuppression in HBL patients listed for LT from ABO-I donors with reference to the kinetics of B, T cells, and anti-ABO blood type isoagglutinin titers. Between 2005 and 2015, of the 319 patients who underwent LDLT at our institute, 12 were indicated for unresectable HBL. Three patients with unresectable HBL who underwent LDLT from ABO-I donors are included in this study. Immunosuppression consisted of a standard regime of tacrolimus and low-dose steroids as in ABO compatible/identical LDLT. No additional preoperative therapies for B-cell depletion were used. Absolute lymphocyte counts, lymphocyte subsets (including CD20+ B cells, CD3+CD4+ T cells and CD3+CD8+ T cells), and anti-ABO blood type isoagglutinin titers were measured before LDLT and postoperatively. The median age at diagnosis was 19 months (range, 3-31 months). The median follow-up was seven months (range, 6-15 months). The median interval from the last NAC to LDLT was 33 days (range, 25-52 days). The median interval from LDLT to adjuvant chemotherapy was 28 days (range, 22-36 days). The counts of CD20+ B cells before LDLT were depleted to median 5 cells/mm(3) (range, 0-6 cells/mm(3)). There was a transient rebound in the CD20+ B cell counts on day seven (maximum of 82 cells/mm(3)) followed by a decline starting at 14 days after LDLT that was sustained for the duration of adjuvant chemotherapy. Anti-ABO blood type isoagglutinin titers were lowered to between 1:1 and 1:16 before LDLT and remained low for the duration of follow-up in this study. All of the three patients remained in good health without either acute cellular or AMR after LDLT. The B-cell depletion that occurs after cisplatin-based chemotherapy for HBL may help accomplish safe ABO-I LDLT in children without the use of additional conditioning regimens for prevention of AMR.