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Byline: M. Lopez-Santamaria (1), M. Gamez (1), J. Murcia (1), J. Diez-Pardo (1), M. Diaz (1), N. Leal (1), R. Lobato (1), L. Martinez (1), L. Hierro (2), C. Camarena (2), A. De la Vega (2), E. Frauca (2), P. Jara (2), T. Berrocal (3), C. Prieto (3), P. Cortes (3), J. Tovar (1) Keywords: Key words Biliary atresia; Hepatic portoenterostomy; Long-term follow-up; Liver transplantation Abstract: The outcome of 18 biliary atresia (BA) patients (5 male, 13 female age range 10.7--22.5 years mean 15.4+-0.7 years) treated with hepatic portoenterostomy (HPE) and jaundice-free for more than 10 years without liver transplantation (LT) is analyzed retrospectively. Eight of these patients subsequently required LT (age at LT 12.8+-0.5 years, range 10.5--15.2 years) 3 children (aged 11.6, 13.2 and 14.1 years, respectively) had episodes of gastrointestinal variceal bleeding associated with other signs of severe disease and are now candidates for LT and among the 7 asymptomatic patients (age range 11.2--22.5 years mean 15.9+-2.1 years), 5 had sonographic and biochemical signs of moderate portal hypertension (PH). In order to analyze whether the age at transplantation influences the survival of children transplanted for BA, we also reviewed the outcome of 71 BA patients transplanted at our hospital between 1986 and 1996. All the children older than 10 years at the time of LT were alive only patients younger than 10 years died following LT (n= 15). We conclude that the natural outcome of extrahepatic BA is toward PH, fibrosis, and cirrhosis, even in those cases successfully treated with HPE. In our experience, the results of sequential treatment with HPE and LT were excellent. Author Affiliation: (1) Hospital Infantil 'La Paz', Dto de Cirugia Pediatrica, Paseo de la Castellana 261, E-28046 Madrid, Spain, ES (2) Department of Hepatology, Pediatric Hospital 'La Paz', Madrid, Spain, ES (3) Department of Radiology, Pediatric Hospital 'La Paz', Madrid, Spain, ES

Aim: Aim of the study was assess the results of the treatment of High-Risk Hepatoblastoma (HRH) in a tertiary center where all liver surgery facilities, including pediatric transplantation (LT), are available. Methods: 91 primary liver tumors treated between 1991 and 2009 were retrospectively reviewed. HRHs as defined by the SIOP criteria (PRETEXT IV or any stage with venous involvement, extrahepatic disease, tumor rupture and < 100 ng/ml serum AFP) were identified and imaging and biopsies were reviewed. The treatment consisted of total removal of the tumor, involving extended hepatectomies and LT if necessary, together with SIOPEL-guided chemotherapy. Results: 23/57 hepatoblastomas were HRH (11F/12M). 17 were considered unresectable by standard techniques, 3 had extrahepatic disease, and 3 fulfilled both criteria. Mean age at diagnosis was 2.3±2.4 years. 3 children (referred after chemotheraphy) died without surgery. 4 had resections (2 left and 2 right trisegmentectomies). Primary LT was required in 15 children (7 cadaveric donors and 8 living related donor transplantations (LRDT), 2 of them with retrohepatic vena cava replacement), and 1 patient had rescue LT after recurrence. Mean follow-up was 4.8±2.9 years. 2 children who had undergone liver resection developed pulmonary metastases at 1.7 and 1.6 years postoperatively and survived after surgical treatment. 2 children with LT developed EBV-related lymphoma and leukemia respectively but survived. Event-free survival (EFS) at 1, 5, and 10 years was 78.3±8.6 % , 63.1±10.5 % , and 63.1±10.5 % , respectively. 6 children died (3 without surgery, 1 after liver resection, 1 after primary LT and 1 after rescue LT). Overall survival at 1, 5 and 10 years was 78.3±21.7 % , 73.2±26.8 % and 73.2±26.8 % . Of those with primary LT, survival at 1, 5 and 10 years was 93.3±6.4 % , 93.3±6.4 % and 93.3±6.4 % . Conclusions: Outstanding results in the treatment of HRH are possible in tertiary centers when referral is early (preferably at diagnosis) and specialized liver surgery and transplantation facilities are available. [ABSTRACT FROM AUTHOR]

Objective:  Neutrophil-to-lymphocyte ratio (NLR) is an emerging inflammatory marker in abdominal pathologies. Ileocolic intussusception (ICI) involves a progressive intestinal inflammation, and the effectiveness of nonsurgical treatment (enema) might be related to the inflammation degree, although no previous studies have investigated this relationship. Our aim is to identify predictors of the need for surgical treatment in ICI., Materials and Methods:  A single-center, retrospective, case-control study was performed in children with ICI, who were treated with initial nonsurgical management between 2005 and 2019. Patients were divided in two groups: A (effective enema) and B (need for surgery). Admission demographic and clinical and laboratory data were analyzed. Specificity and sensitivity of the different parameters as predictors of the need for surgical treatment were determined by receiver operating characteristic (ROC) curves., Results:  A total of 511 patients were included (410: group A; 101: group B), without statistically significant demographic differences. Group B presented significantly higher frequency of vomiting, bloody stools, and longer median time since symptoms onset (24 vs. 8 hours; p  < 0.001). Group B presented higher median laboratory inflammatory markers than group A: NLR (6.8 vs. 1.8; p  < 0.001), neutrophils (10,148 vs. 7,468; p  < 0.001), and C-reactive protein (CRP; 28.2 vs. 4.7; p  < 0.001). In ROC curve analysis, NLR had an area under the curve of 0.925, higher than neutrophil count (0.776; p  = 0.001), CRP (0.670; p  = 0.001), and time since symptoms onset (0.673; p  = 0.001). It was estimated a cut-off point of NLR greater than 4.52 (sensitivity: 73.2%; specificity: 94.5%)., Conclusion:  High NLR values imply a high degree of bowel inflammation and might anticipate the need for surgical treatment in ICI in children., Level of Evidence:  III., Competing Interests: None declared., (Thieme. All rights reserved.)

Background and Aims: The European Liver Transplant Registry (ELTR) has collected data on liver transplant procedures performed in Europe since 1968., Approach and Results: Over a 50-year period (1968-2017), clinical and laboratory data were collected from 133 transplant centers and analyzed retrospectively (16,641 liver transplants in 14,515 children). Data were analyzed according to three successive periods (A, before 2000; B, 2000-2009; and C, since 2010), studying donor and graft characteristics and graft outcome. The use of living donors steadily increased from A to C (A, n = 296 [7%]; B, n = 1131 [23%]; and C, n = 1985 [39%]; p = 0.0001). Overall, the 5-year graft survival rate has improved from 65% in group A to 75% in group B (p < 0.0001) and to 79% in group C (B versus C, p < 0.0001). Graft half-life was 31 years, overall; it was 41 years for children who survived the first year after transplant. The late annual graft loss rate in teenagers is higher than that in children aged <12 years and similar to that of young adults. No evidence for accelerated graft loss after age 18 years was found., Conclusions: Pediatric liver transplantation has reached a high efficacy as a cure or treatment for severe liver disease in infants and children. Grafts that survived the first year had a half-life similar to standard human half-life. Transplantation before or after puberty may be the pivot-point for lower long-term outcome in children. Further studies are necessary to revisit some old concepts regarding transplant benefit (survival time) for small children, the role of recipient pathophysiology versus graft aging, and risk at transition to adult age., (© 2021 American Association for the Study of Liver Diseases.)

Background: Mesenchymal stromal cells (MSC) have been proposed as a promising complement to standard immunosuppression in solid organ transplantation because of their immunomodulatory properties. The present work addresses the role of adipose-derived MSC (Ad-MSC) in an experimental model of acute rejection in small bowel transplantation (SBT)., Material/methods: Heterotopic allogeneic SBT was performed. A single dose of 1.5x106 Ad-MSC was intra-arterially delivered just before graft reperfusion. Animals were divided into CONTROL (CTRL), CONTROL+Ad-MSC (CTRL&#95;MSC), tacrolimus (TAC), and TAC+Ad-MSC (TAC&#95;MSC) groups. Each Ad-MSC groups was subdivided in autologous and allogeneic third-party groups., Results: Rejection rate and severity were similar in MSC-treated and untreated animals. CTRL&#95;MSC animals showed a decrease in macrophages, T-cell (CD4, CD8, and Foxp3 subsets) and B-cell counts in the graft compared with CTRL, this decrease was attenuated in TAC&#95;MSC animals. Pro- and anti-inflammatory cytokines and some chemokines and growth factors increased in CTRL&#95;MSC animals, especially in the allogeneic group, whereas milder changes were seen in the TAC groups., Conclusion: Ad-MSC did not prevent rejection when administered just before reperfusion. However, they showed immunomodulatory effects that could be relevant for a longer-term outcome. Interference between tacrolimus and the MSC effects should be addressed in further studies., (© 2021 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Introduction:  Recurrence of ileocolic intussusception (ICI) has been related to residual bowel wall edema after enema reduction. Early oral tolerance has been associated with a higher risk of re-intussusception, so an imaging test (ultrasound) has traditionally been performed before restarting oral tolerance. Our aim is to analyze the cost-effectiveness of performing a routine ultrasound in patients who remain asymptomatic after successful enema reduction., Materials and Methods:  A retrospective observational study was performed in patients with ICI who underwent a successful enema reduction between 2005 and 2019 and distributed in two groups according to whether or not a routine ultrasound was performed before restarting oral tolerance: group A (ultrasound) or B (no ultrasound). We analyzed demographic, clinical and laboratory variables, length of hospital stay, and recurrence rate., Results:  We included 366 patients who presented 373 ICI episodes (165 in group A and 208 in group B), without significant differences in gender and age. Group A patients presented a higher percentage of vomiting and bloody stools than those in group B without differences in the other clinical features studied, time of evolution, or laboratory variables. Group A presented a higher length of hospital stay than group B (36 vs. 24 hours), although it was not statistically significant ( p  = 0.30). No statistically significant differences were observed in the recurrence rate between both groups (10.3% A vs. 10.8% B; p  = 0.83)., Conclusion:  Performing routine ultrasound before restarting oral tolerance in asymptomatic patients after successful ICI reduction does not decrease the risk of re-intussusception and should not be routinely encouraged., Competing Interests: None declared., (Thieme. All rights reserved.)

Aim:  Hepatoblastoma is the most frequent hepatic tumor in children, and its initial presentation will affect treatment and prognosis. The aim of this study is to evaluate long-term results of liver transplantation in children with unresectable hepatoblastoma., Patients and Methods:  This is a retrospective review of patients with hepatoblastoma who underwent liver transplantation, analyzing risk factors, tumor presentation, treatment, and long-term survival to identify prognostic factors., Results:  Thirty-one patients underwent liver transplantation in the context of unresectable hepatoblastoma, mainly males (67%) and with risk factors such as prematurity (12.9%), maternal smoking (6.5%), and familial adenomatous polyposis (3.2%). Most frequent presentation was multifocal PRETEXT III (51.6%) and PRETEXT IV (45.2%), with metastasis at diagnosis in 12.9% and vascular involvement in 54.8%.Twenty-one patients received a living-donor (67.7%) and 10 a cadaveric graft (32.2%), at 31.7 months of age (5-125). Most transplants were primary, and only two were performed as rescue therapy after an attempt of surgical resection of the tumor.Overall survival 1 and 5 years after transplantation were 93.3% ± 4.6% and 86.4% ± 6.3%, respectively. We could not find any statistically significant differences between risk factors, tumor presentation, type of graft, or type of transplant., Conclusion:  Liver transplantation has increased hepatoblastoma survival in unresectable tumors. Probably due to these good results, we have not been able to find significant prognostic factors in this cohort., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Introduction:  There is a lack of an agreed Hirschsprung-associated enterocolitis (HAEC) definition. In 2009, a HAEC score was proposed for the diagnosis of HAEC episodes. Our aim was to apply the HAEC score on HAEC episodes to determine its diagnostic efficiency and whether it correlated to its severity., Methods:  Retrospective study of patients with HAEC admitted between 2000 and 2016. Episodes of HAEC were identified and the HAEC score was calculated. A cut-off of ≥ 10 according to Pastor et al and ≥ 4 according to Frykman et al were used. A Pearson's correlation coefficient was performed for outcome variable: length-of-stay (LOS)., Results:  Note that 21/93 (22.6%) patients with Hirschsprung's disease presented 50 HAEC episodes with a median of 2 (1-5) episodes during an 8.3-year (2-15.6) follow-up. The most common symptoms were foul-smelling (86% [43/50]) and explosive (60% [30/50]) diarrhea. Physical findings showed a distended abdomen (76% [38/50]) and fever (60% [30/50]) with dilated bowel (82% [41/50]) and rectosigmoid cut-off (80% [40/50]) identified on X-rays. Only 34% (17/50) showed abnormal laboratory findings. Patients were admitted with a median LOS of 7 days (1-28). A HAEC score of 9 (2-16) was found, and only 50% (25/50) of patients met the initial criteria (score of 10 points). However, the use of a 4-point cut-off would have allowed the diagnosis of 98% (49/50) of the patients. A positive linear correlation was found for LOS and HAEC score ( r  = 0.3, p  = 0.014)., Conclusion:  There is no standardized definition for HAEC. The initial HAEC score cut-off is restrictive and might fail to identify milder episodes. The positive correlation with LOS and thus HAEC severity might aid in patient information and anticipation of treatment., Competing Interests: None declared., (Georg Thieme Verlag KG Stuttgart · New York.)

Aim:  Extrahepatic portal vein obstruction (EHPVO) is a frequent cause of noncirrhotic portal hypertension in children. The aim of this study is to analyze long-term results after diversion surgery., Patients and Methods:  Retrospective review of EHPVO patients who underwent diversion surgery analyzing number of platelets, leukocytes, prothrombin activity, splenomegaly, and gastrointestinal bleeding 10 years after surgery., Results:  Thirty-three patients were evaluated, mostly males (64%) and presenting with gastrointestinal bleeding (46%). Mesoportal shunt (Rex) was performed in 19 patients, mesocaval in 7, distal splenorenal in 7, and proximal splenorenal in 3. While comparing mesoportal shunt to the other portosystemic shunts, an increase in platelets was found with every technique, but it was clearly higher in mesoportal shunt. The highest increase was 6 months after surgery ( p  = 0.0015) as well as prothrombin activity ( p  = 0.0003). Leukocytes level also increased without statistical significance. Spleen size (cm) and spleen size Z score (SSAZ) decreased significantly 6 months after mesoportal shunt ( p  = 0.0168). Before surgery, over 94% patients suffered gastrointestinal bleeding, which reduced significantly afterward with bleeding episodes in only four (12%) of them., Conclusion:  Diversion surgery in EHPVO, especially mesoportal shunt of Rex, improves hepatic function (prothrombin activity), reduces hypersplenism (platelets, leukocytes, and spleen size), and decreases gastrointestinal bleeding episodes., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Purpose of Review: One of the biggest successes of intestinal rehabilitation programs is that more patients achieve enteral autonomy without transplantation. Many factors are responsible of this accomplishment including new parenteral formulas, better catheter management, surgical management, and the experience of the teams. The purpose of this review is to analyze recent published papers regarding intestinal lengthening procedures trying to find out how many transplantations are avoided and for which patients., Recent Findings: A trend towards performing less intestinal transplants has been identified in the last years. The general improvement of intestinal rehabilitation accounts for this step forward. However, the role of intestinal lengthening has not been clarified., Summary: Surgical techniques for autologous reconstructive surgery are not limited to bowel lengthening. Longitudinal intestinal lengthening and tailoring and serial transverse enteroplasty offered good results in terms of intestinal adaptation, long-term survival, and subsequent need of intestinal transplantation. In recent series, less than one quarter of patients who underwent intestinal lengthening required salvage intestinal transplantation.

Aim:  Cardiac function can be impaired in patients with pectus excavatum (PE) due to anatomic and dynamic compression of the heart. Efforts for radiation dose reduction in imaging techniques have allowed cardiac magnetic resonance imaging (c-MRI) to play a major role in PE assessment. The aim of our study is to describe the findings of c-MRI 18 months after we changed the PE assessment protocol from chest computed tomography to c-MRI., Patients and Methods:  Since mid-2015 all patients with severe PE (suspected Haller's index > 3.2) were assessed with inspiratory and expiratory c-MRI. A retrospective analysis of these patients was performed evaluating the following parameters: (1) Radiologic PE indexes (Haller's, correction and asymmetry indexes; and sternal rotation) and (2) cardiac function (including left and right ventricle ejection fraction)., Results:  A total of 20 patients met the inclusion criteria. Dynamic imaging showed a significant difference during inspiration and expiration of the Haller's index 3.85 (range: 3.17-7.3) versus 5.10 (range: 3.85-10.8) ( p  < 0.05), and correction index (26.86% vs. 36.84%, respectively, p  < 0.05). The sternal rotation was 14.5 (range: 0-36). c-MRI analysis disclosed a right ventricle ejection fraction of 50.3%. (normal range: 61% [54-71%]). Echocardiographic imaging underestimated the functional repercussion of PE in all patients., Conclusion:  Initial results show that PE assessment by c-MRI allows a radiation-free image of the chest wall deformity during the entire breathing process. Also, it permitted the evaluation of the influence of sternum impingement on cardiac function. These findings allowed us a careful surgical evaluation and preoperative planning., Competing Interests: Conflict of Interest: None., (Georg Thieme Verlag KG Stuttgart · New York.)