Choledochal cysts in children: How to Diagnose and Operate on.

OBJECTIVE: To identify the best mode for diagnosing and treating the patients with choledochal cysts. METHODS: A retrospective study was performed with medical records of patients diagnosed with choledochal cysts from January 1994 to December 2017. In all cases, the diagnosis was based on ultrasound examination. All the patients underwent cyst resection and were divided in two groups: bile enteric anastomosis in the high portion of the common hepatic duct or in the dilated lower portion. RESULTS: Eighty-one cases were studied. The age of presentation was 4 y 2 mo ± 4 y 1 mo, and the age for the surgical treatment was 5 y 5 mo ± 4 y 6 mo. In 61 cases, US was the only image examination performed. There were 67 cases of Todani type I (82.7%), 13 cases of type IV (16.0%) and one case of type III (1.2%). Nine patients (29.0%) in the first period and 2 patients (4.0%) in the second period presented with postoperative complications (p=0.016). CONCLUSION: In patients with choledochal cysts, US is the only necessary diagnostic imaging. Performing the bile enteric anastomosis in the lower portion of the common hepatic duct is safer and has a lower risk of complications.

Choledochal cysts in children: How to Diagnose and Operate on

OBJECTIVE: To identify the best mode for diagnosing and treating the patients with choledochal cysts. METHODS: A retrospective study was performed with medical records of patients diagnosed with choledochal cysts from January 1994 to December 2017. In all cases, the diagnosis was based on ultrasound examination. All the patients underwent cyst resection and were divided in two groups: bile enteric anastomosis in the high portion of the common hepatic duct or in the dilated lower portion. RESULTS: Eighty-one cases were studied. The age of presentation was 4 y 2 mo ± 4 y 1 mo, and the age for the surgical treatment was 5 y 5 mo ± 4 y 6 mo. In 61 cases, US was the only image examination performed. There were 67 cases of Todani type I (82.7%), 13 cases of type IV (16.0%) and one case of type III (1.2%). Nine patients (29.0%) in the first period and 2 patients (4.0%) in the second period presented with postoperative complications (p=0.016). CONCLUSION: In patients with choledochal cysts, US is the only necessary diagnostic imaging. Performing the bile enteric anastomosis in the lower portion of the common hepatic duct is safer and has a lower risk of complications.

Current management of biliary atresia based on 35 years of experience at a single center.

OBJECTIVE: The prognosis of patients with biliary atresia undergoing Kasai portoenterostomy is related to the timing of the diagnosis and the indication for the procedure. The purpose of the present study is to present a practical flowchart based on 257 children who underwent Kasai portoenterostomy. METHODS: We conducted a retrospective cohort study of patients who underwent Kasai portoenterostomy between 1981 and 2016. RESULTS: During the first period (1981 to 2009), 230 infants were treated, and the median age at the time of surgery was 84 days; jaundice was resolved in 77 patients (33.5%). During the second period, from 2010 to 2016, a new diagnostic approach was adopted to shorten the wait time for portoenterostomy; an ultrasonography examination suggestive of the disease was followed by primary surgical exploration of the biliary tract without complementary examination or liver biopsy. Once the diagnosis of biliary atresia was confirmed, a portoenterostomy was performed during the same surgery. During this period, 27 infants underwent operations; the median age at the time of surgery was 66 days (p<0.001), and jaundice was resolved in 15 patients (55.6% - p=0.021), with a survival rate of the native liver of 66.7%. CONCLUSION: Primary surgical exploration of the biliary tract without previous biopsy was effective at improving the prognostic indicators of patients with biliary atresia undergoing Kasai portoenterostomy.

Current management of biliary atresia based on 35 years of experience at a single center

OBJECTIVE: The prognosis of patients with biliary atresia undergoing Kasai portoenterostomy is related to the timing of the diagnosis and the indication for the procedure. The purpose of the present study is to present a practical flowchart based on 257 children who underwent Kasai portoenterostomy. METHODS: We conducted a retrospective cohort study of patients who underwent Kasai portoenterostomy between 1981 and 2016. RESULTS: During the first period (1981 to 2009), 230 infants were treated, and the median age at the time of surgery was 84 days; jaundice was resolved in 77 patients (33.5%). During the second period, from 2010 to 2016, a new diagnostic approach was adopted to shorten the wait time for portoenterostomy; an ultrasonography examination suggestive of the disease was followed by primary surgical exploration of the biliary tract without complementary examination or liver biopsy. Once the diagnosis of biliary atresia was confirmed, a portoenterostomy was performed during the same surgery. During this period, 27 infants underwent operations; the median age at the time of surgery was 66 days (p<0.001), and jaundice was resolved in 15 patients (55.6% - p=0.021), with a survival rate of the native liver of 66.7%. CONCLUSION: Primary surgical exploration of the biliary tract without previous biopsy was effective at improving the prognostic indicators of patients with biliary atresia undergoing Kasai portoenterostomy.

Living donor liver transplantation in children: should the adult donor be operated on by an adult or pediatric surgeon? Experience of a single pediatric center.

BACKGROUND/PURPOSE: Living donor liver transplantation has become a cornerstone for the treatment of children with end-stage hepatic dysfunction, especially within populations or countries with low rates of organ utilization from deceased donors. The objective is to report our experience with 185 living donors operated on by a team pediatric surgeons in a tertiary center for pediatric liver transplantation. METHODS: Retrospective analysis of medical records of donors of hepatic grafts for transplant undergoing surgery between June 1998 and March 2013. RESULTS: Over the last 14 years, 185 liver transplants were performed in pediatric recipients of grafts from living donors. Among the donors, 166 left lateral segments (89.7%), 18 left lobes without the caudate lobe (9.7%) and 1 right lobe (0.5%) were harvested. The donor age ranged from 16 to 53 years, and the weight ranged from 47 to 106 kg. In 10 donors, an additional graft of the donor inferior mesenteric vein was harvested to substitute for a hypoplastic recipient portal vein. The transfusion of blood products was required in 15 donors (8.1%). The mean hospital stay was 5 days. No deaths occurred, but complications were identified in 23 patients (12.4%): 9 patients experienced abdominal pain and severe gastrointestinal symptoms and 3 patients required reoperations. Eight donors presented with minor bile leaks that were treated conservatively, and 3 patients developed extra-peritoneal infections (1 wound collection, 1 phlebitis and 1 pneumonia). Eight grafts (4.3%) showed primary dysfunction resulting in recipient death (3 cases of fulminant hepatitis, 1 patient with metabolic disease, 1 patient with Alagille syndrome and 3 cases of biliary atresia in infants under 1 year old). There was no relation between donor complications and primary graft dysfunction (P=0.6). CONCLUSIONS: Living donor transplantation is safe for the donor and presents a low morbidity. The donor surgery may be performed by a team of trained pediatric surgeons.

Effects of the administration of pentoxifylline and prednisolone on the evolution of portal fibrogenesis secondary to biliary obstruction in growing animals: immunohistochemical analysis of the expression of TGF-β and VEGF

OBJECTIVE: During the neonatal and infancy periods, some chronic liver diseases may lead to progressive hepatic fibrosis, which is a condition that can ultimately result in the loss of organ function and severe portal hypertension necessitating hepatic transplantation. In a previous report, pharmacological interventions were demonstrated to modulate hepatic fibrosis induced by bile duct ligation in young rats. The administration of pentoxifylline or prednisolone, or the combination of both, resulted in reduced fibrogenesis in portal spaces. The objectives of the present study were to evaluate the expression of transforming growth factor β and vascular endothelial growth factor after bile duct ligation in young rats and to assess the effect of those same drugs on cytokine expression. METHODS: In this experimental study, 80 young rats (21 or 22 days old) were submitted either to laparotomy and common bile duct ligation or to sham surgery. The animals were allocated into four groups according to surgical procedure, and the following treatments were administered: (1) common bile duct ligation + distilled water, (2) sham surgery + distilled water, (3) common bile duct ligation + pentoxifylline, or (4) common bile duct ligation + prednisolone. After 30 days, a hepatic fragment was collected from each animal for immunohistochemical analysis using monoclonal antibodies against transforming growth factor β and vascular endothelial growth factor. Digital morphometric and statistical analyses were performed. RESULTS: The administration of pentoxifylline reduced the transforming growth factor β-marked area and the amount of transforming growth factor β expressed in liver tissue. This effect was not observed after the administration of prednisolone. There was a significant reduction in vascular endothelial growth factor expression after the administration of either drug compared with the non-treatment group. CONCLUSIONS: The administration of pentoxifylline to cholestatic young rats resulted in the diminished expression of transforming growth factor β and vascular endothelial growth factor in liver tissue. The administration of steroids resulted in the diminished expression of vascular endothelial growth factor only. These pathways may be involved in hepatic fibrogenesis in young rats submitted to bile duct ligation and exposed to pentoxifylline or prednisolone.

[Videosurgery in infancy and childhood: state of the art. Experience with 1408 procedures in the Instituto da Criança "Pedro de Alcântara"]. / Videocirurgia na criança: estado da arte. Experiência com 1408 procedimentos no Instituto da Criança "Pedro de Alcântara"

The videosurgery in Pediatric Surgery has a large field of applications unfortunately still underexplored. There are few services that routinely use this techinic , and Brazilian articles published are scarce. The Institute of Children's Hospital of the Faculty of Medicine, University of São Paulo, has been using for fifteen years the videosurgery which is now the first choice of treatment, among other diseases as gastroesophageal reflux, the cholecystolithiasis, the nonpalpable undescended testicles and megaesophagus. In this article we report our experience in laparoscopic pediatric surgery, acquired with 1408 surgical procedures, to present this useful method, and beneficial to a large number of situations and still underused in Pediatric Surgery.

Videocirurgia na criança: estado da arte. Experiência com 1408 procedimentos no Instituto da Criança "Pedro de Alcântara" / Videosurgery in infancy and childhood: state of the art. Experience with 1408 procedures in the Instituto da Criança "Pedro de Alcântara"

A videocirurgia em Cirurgia Pediátrica encontra um imenso campo de aplicações ainda, infelizmente, pouco explorado. São poucos os serviços que utilizam rotineiramente essa via de acesso e são escassas as referências nacionais publicadas. O Instituto da Criança do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (ICr) há uma década e meia a utiliza e hoje a videocirurgia é a via de primeira escolha para tratar, entre outras doenças, o refluxo gastresofagiano, a colecistopatia calculosa, o testículo não palpável e o megaesôfago. Neste artigo relataremos a experiência em videocirurgia pediátrica do ICr, adquirida com 1408 pacientes operados, para divulgar e popularizar esta via de acesso, útil e benéfica para um grande número de situações e ainda subutilizada em Cirurgia Pediátrica.

The videosurgery in Pediatric Surgery has a large field of applications unfortunately still underexplored. There are few services that routinely use this techinic , and Brazilian articles published are scarce. The Institute of Children's Hospital of the Faculty of Medicine, University of São Paulo, has been using for fifteen years the videosurgery which is now the first choice of treatment, among other diseases as gastroesophageal reflux, the cholecystolithiasis, the nonpalpable undescended testicles and megaesophagus. In this article we report our experience in laparoscopic pediatric surgery, acquired with 1408 surgical procedures, to present this useful method, and beneficial to a large number of situations and still underused in Pediatric Surgery.

Effects of the administration of pentoxifylline and prednisolone on the evolution of portal fibrogenesis secondary to biliary obstruction in growing animals: immunohistochemical analysis of the expression of TGF-ß and VEGF.

OBJECTIVE: During the neonatal and infancy periods, some chronic liver diseases may lead to progressive hepatic fibrosis, which is a condition that can ultimately result in the loss of organ function and severe portal hypertension necessitating hepatic transplantation. In a previous report, pharmacological interventions were demonstrated to modulate hepatic fibrosis induced by bile duct ligation in young rats. The administration of pentoxifylline or prednisolone, or the combination of both, resulted in reduced fibrogenesis in portal spaces. The objectives of the present study were to evaluate the expression of transforming growth factor ß and vascular endothelial growth factor after bile duct ligation in young rats and to assess the effect of those same drugs on cytokine expression. METHODS: In this experimental study, 80 young rats (21 or 22 days old) were submitted either to laparotomy and common bile duct ligation or to sham surgery. The animals were allocated into four groups according to surgical procedure, and the following treatments were administered: (1) common bile duct ligation + distilled water, (2) sham surgery + distilled water, (3) common bile duct ligation + pentoxifylline, or (4) common bile duct ligation + prednisolone. After 30 days, a hepatic fragment was collected from each animal for immunohistochemical analysis using monoclonal antibodies against transforming growth factor ß and vascular endothelial growth factor. Digital morphometric and statistical analyses were performed. RESULTS: The administration of pentoxifylline reduced the transforming growth factor ß-marked area and the amount of transforming growth factor ß expressed in liver tissue. This effect was not observed after the administration of prednisolone. There was a significant reduction in vascular endothelial growth factor expression after the administration of either drug compared with the non-treatment group. CONCLUSIONS: The administration of pentoxifylline to cholestatic young rats resulted in the diminished expression of transforming growth factor ß and vascular endothelial growth factor in liver tissue. The administration of steroids resulted in the diminished expression of vascular endothelial growth factor only. These pathways may be involved in hepatic fibrogenesis in young rats submitted to bile duct ligation and exposed to pentoxifylline or prednisolone.

Effects of the administration of pentoxifylline and prednisolone on the evolution of portal fibrogenesis secondary to biliary obstruction-an experimental study in growing animals.

BACKGROUND: Many chronic liver diseases lead to progressive hepatic fibrosis, a condition that can ultimately result in loss of organ function and severe portal hypertension necessitating hepatic transplantation. Within the last few decades, studies have been conducted to demonstrate the possibility of drug modulation of hepatic fibrogenesis. Regarding biliary obstruction, it has been suggested that administration of corticosteroids could promote better late outcomes for children with biliary atresia submitted to Kasai's portoenterostomy. Models used to test potential antifibrogenic drugs such as pentoxifylline (PTX) have not included growing animals. METHODS: In this experimental study, 119 young rats (21st or 22nd days) were submitted to laparotomy and common bile duct ligation (CBDL) or to sham surgery (SHAM). Animals were allocated into 5 groups, according to surgical procedure, and administered the following solutions: (1) CBDL + distilled water, (2) SHAM + distilled water, (3) CBDL + PTX, (4) CBDL + prednisolone (PRED), and (5) CBDL + PTX + PRED (PTX + PRED). Each group was further divided into 2 subgroups according to the length of the experiment (15 or 30 days). At the end of the defined period, animals were weighed, and a hepatic fragment was collected from each one for analyses. RESULTS: The PTX animals exhibited increased weight gain compared to animals in the PRED or PTX + PRED groups. Animals from the 3 therapeutic groups (PTX, PRED, and PTX + PRED) showed diminished collagen-filled area in portal spaces. Total portal space area was increased in the PTX group. CONCLUSIONS: Hepatic fibrosis induced by bile duct ligation in young rats could be modulated by pharmacologic interventions. Administration of PTX or PRED, or the combination of both, resulted in diminished collagen-filled areas in portal spaces.

Efeitos da administração de pentoxifilina e prednisolona na evolução da fibrose portal secundária à obstrução biliar: estudo experimental em animais em crescimento / Effects of administration of pentoxifylline and prednisolone on evolution of portal fibrosis secondary to biliary obstruction experimental study in growing animals

INTRODUÇÃO: Diversas doenças crônicas do fígado resultam em desenvolvimento de fibrose do parênquima, condição que pode culminar em perda de função do órgão e hipertensão portal grave com indicação de transplante. Nas últimas décadas, alguns estudos têm buscado demonstrar a possibilidade de modulação farmacológica do processo de fibrogênese hepática. Particularmente com relação às obstruções biliares, tem-se sugerido que a administração de corticosteróides pode promover melhora no prognóstico tardio das crianças portadoras de atresia das vias biliares submetidas a portoenterostomia (cirurgia de Kasai). Nenhum trabalho experimental foi realizado com o objetivo de verificar essa hipótese e os modelos descritos para estudo de outras drogas com potencial antifibrogênico, como a pentoxifilina, não incluíram animais jovens. MÉTODOS: Foram operados 119 ratos jovens (21º ou 22º dias de vida), submetidos a laparotomia com ligadura do ducto biliar comum (LDBC) ou cirurgia simulada (CS). Os animais foram divididos em 5 grupos, conforme o procedimento cirúrgico e a medicação administrada: 1. LDBC + água destilada; 2. CS + água destilada; 3. LDBC + pentoxifilina (PTX); 4. LDBC + prednisolona (PRED); 5. LDBC + pentoxifilina + prednisolona (PTX+PRED). Cada grupo foi dividido em 2 subgrupos conforme a duração do experimento (15 ou 30 dias). Ao final do período, os animais foram pesados e submetidos a retirada de um fragmento de fígado, coletado para análise histológica através das colorações de hematoxilina-eosina e de picrossírius. As lâminas coradas pelo picrossírius foram utilizadas para análise quantitativa (morfometria digital) da área preenchida por colágeno e da área total dos espaços-porta. Após a obtenção desses valores, foi calculada a fração de área de colágeno de cada grupo. RESULTADOS: Os animais do grupo PTX apresentaram maior ganho de peso do que os dos grupos PRED e PTX+PRED. Os animais dos 3 grupos terapêuticos (PTX, PRED e PTX+PRED) apresentaram maior ganho...

INTRODUCTION: Many chronic liver diseases lead to progressive establishment of hepatic fibrosis, condition that can ultimately result in loss of organ function and severe portal hypertension demanding hepatic transplantation. Within the last decades, some studies have been conducted in order to demonstrate the possibility of drug modulation of hepatic fibrogenesis. Particularly related to biliary obstruction, it has been suggested that administration of corticosteroids could promote better late outcomes for biliary atresia children submitted to Kasais portoenterostomy. There is no published experimental study related to that issue, and described models used to test potential antifibrogenic drugs, such as pentoxifylline, have not included growing animals. METHODS: In this experimental study, 119 young rats (21st or 22nd days) were submitted to laparotomy and bile duct ligation (BDL) or sham surgery (SHAM). Animals were divided into 5 groups, according to surgical procedure and administered solution: 1. BDL + distilled water; 2. SHAM + distilled water; 3. BDL + pentoxifylline (PTX); 4. BDL + prednisolone (PRED); 5. BDL + pentoxifylline + prednisolone (PTX+PRED). Each group was further divided into 2 subgroups according to the length of the experiment (15 or 30 days). At the end of the defined period, animals were weighed and one hepatic fragment was collected from each one for histological analysis using hematoxylin-eosin and Sirius red stains. Sirius red stained slides were examined for quantitative analysis (digital morphometry) of collagen-filled area and portal space total area. RESULTS: PTX group animals presented increased weight gain compared to PRED or PTX+PRED groups. Animals from the 3 therapeutic groups (PTX, PRED and PTX+PRED) showed diminished collagen-filled area in portal spaces. Total portal space area was increased...

Endovascular treatment of stenosis between hepatic vein and inferior vena cava following liver transplantation in a child: a case report.

The liver transplantation technique advances have allowed the endovascular treatment of stenosis between hepatic vein and inferior vena cava, and this has become an established and widely acceptable method for the treatment of patients with end-stage liver disease. However, in spite of the advances in the surgical technique of liver transplantation there is relatively still a high incidence of postoperative complications, especially those related to vascular complications. One technical variant of orthotopic liver transplantation is the piggyback technique with conservation of the recipient vena cava, which is anastomosed to the graft hepatic veins. As a consequence of the increased number of liver transplants in children, there is a higher demand for endovascular treatment of vascular stenosis, such as those at the level of the hepatic veins. This leads to more consistent experience of endovascular treatment of the surgical vascular complications following liver transplantation. This article describes the case of a child submitted to liver transplantation with reduced graft (left lateral segment) who presented stenosis of the anastomosis between the hepatic vein and IVC 6 months later which was successfully treated by PTA.