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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.

IRI is closely related to sepsis in ITx setting. Complete understanding of the mechanisms involved in IRI development may improve outcomes. Ortothopic ITx without immunosuppression was performed in order to characterize IRI-associated mucosal damage. Twenty pigs underwent ITx. Two groups were assigned to different CI times: G1: 90 min and, G2: 180 min. Euro-Collins was used as preservation solution. Jejunal fragments were collected at donor laparotomy, 30 min, and 3 days after reperfusion. IRI assessment involved: histopathologic analysis, quantification of MPO-positive cells through immunohistochemical studies, quantification of epithelial apoptotic cells using TUNEL staining, and quantification of IL-6, ET-1, Bak, and Bcl-XL genes expression by RT-PCR. Neutrophilic infiltration increased in a similar fashion in both groups, but lasted longer in G2. Apoptosis detected by TUNEL staining increased and anti-apoptotic gene Bcl-XL expression decreased significantly in G1, 3 days after surgery. Endothelin-1 and IL-6 genes expression increased 30 min after the procedure and returned to baseline 3 days after surgery. In conclusion, IL-6 and ET-1 are involved precociously in the development of intestinal IRI. Apoptosis was more frequently detected in G1 grafts by TUNEL-staining and by RT-PCR., (© 2011 John Wiley & Sons A/S.)

Background/purpose: The introduction of the piggyback technique for reconstruction of the liver outflow in reduced-size liver transplants for pediatric patients has increased the incidence of hepatic venous outflow block (HVOB). Here, we proposed a new technique for hepatic venous reconstruction in pediatric living-donor liver transplantation., Methods: Three techniques were used: direct anastomosis of the orifice of the donor hepatic veins and the orifice of the recipient hepatic veins (group 1); triangular anastomosis after creating a wide triangular orifice in the recipient inferior vena cava at the confluence of all the hepatic veins (group 2); and a new technique, which is a wide longitudinal anastomosis performed at the anterior wall of the inferior vena cava (group 3)., Results: In groups 1 and 2, the incidences of HVOB were 27.7% and 5.7%, respectively. In group 3, no patient presented HVOB (P = .001). No difference was noted between groups 2 and 3., Conclusions: Hepatic venous reconstruction in pediatric living-donor liver transplantation must be preferentially performed by using a wide longitudinal incision at the anterior wall of the recipient inferior vena cava. As an alternative technique, triangulation of the recipient inferior vena cava, including the orifices of the 3 hepatic veins, may be used., (Copyright © 2011 Elsevier Inc. All rights reserved.)

PRES is a neuroclinical and radiological syndrome that results from treatment with calcineurin inhibitor immunosuppressives. Severe hypertension is commonly present, but some patients may be normotensive. We report herein two children who received liver transplants, as treatment for biliary atresia in the first case and for Alagille's syndrome in the second one. In the early postoperative, both patients presented hypertension and seizures. In both cases, the image findings suggested the diagnosis of PRES. The CT scan showed alterations in the posterior area of the brain, and brain MRI demonstrated parietal and occipital areas of high signal intensity. Both children were treated by switching the immunosuppressive regimen and controlling arterial blood pressure. They displayed full recuperation without any neurologic sequelae. Probably, the pathophysiology of PRES results from sparse sympathetic innervation of the vertebrobasilar circulation, which is responsible for supplying blood to the posterior areas of the brain. In conclusion, all liver-transplanted children who present with neurological symptoms PRES should be considered in the differential diagnosis, although this is a rare complication. As treatment, we recommend rigorous control of arterial blood pressure and switching the immunosuppressive regimen., (© 2010 John Wiley & Sons A/S.)

Background: Extrahepatic portal vein thrombosis (EHPVT) is an important cause of portal hypertension in children. Rex shunt has been used successfully to treat these patients., Methods: We report our experience in 19 infants and children (5 months to 14 years) with HPVT eligible for a mesenteric-portal surgical shunt with left internal jugular vein autograft. Eight children had idiopathic EHPVT, nine had post-umbilical catheterization EHPVT, one had portal vein agenesis, and one had posttransplant EHPVT., Results: It was possible to perform the Rex shunt in all patients except for 8 of 9 cases in the post-umbilical catheterization EHPVT group. A Warren procedure was performed in 4 of those patients and a proximal splenorenal shunt in 1. Current follow-up ranges from 3 to 26 months. Shunt thrombosis occurred in one patient with portal vein agenesis and associated cardiac anomaly. Portal hypertension has significantly improved after surgery. None of our patients have experienced new bleeding episodes until now., Conclusions: The Rex shunt should be considered in the treatment of children with idiopathic EHPVT experiencing repeated gastrointestinal bleeding episodes refractory to endoscopic treatment. Nevertheless, the role of this operation for children with post-umbilical catheterization EHPVT is yet to be clearly evaluated., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Introduction: The use of arterial grafts (AG) in pediatric orthotopic liver transplantation (OLT) is an alternative in cases of poor hepatic arterial inflow, small or anomalous recipient hepatic arteries, and retransplantations (re-OLT) due to hepatic artery thrombosis (HAT). AG have been crucial to the success of the procedure among younger children. Herein we have reported our experience with AG., Methods: We retrospectively reviewed data from June 1989 to June 2010 among OLT in which we used AG, analyzing indications, short-term complications, and long-term outcomes., Results: Among 437 pediatric OLT, 58 children required an AG. A common iliac artery interposition graft was used in 57 cases and a donor carotid artery in 1 case. In 38 children the graft was used primarily, including 94% (36/38) in which it was due to poor hepatic arterial inflow. Ductopenia syndromes (n = 14), biliary atresia (BA; n = 11), and fulminant hepatitis (n = 8) were the main preoperative diagnoses among these children. Their mean weight was 18.4 kg and mean age was 68 months. At the mean follow-up of 27 months, multiple-organ failure and primary graft nonfunction (PNF) were the short-term causes of death in 9 children (26.5%). Among the remaining 29 patients, 2 (6,8%) developed early graft thrombosis requiring re-OLT; 5 (17%) developed biliary complications, and 1 (3.4%) had asymptomatic arterial stenosis. In 20 children, a graft was used during retransplantation. The main indication was HAT (75%). BA (n = 15), ductopenia syndromes (n = 2), and primary sclerosing cholangitis (n = 2) were the main diagnoses. Their mean weight was 16.7 kg and age was 65 months. At a mean follow-up of 53 months, 7 children died due to multiple-organ failure or PNF. Among the remaining 13 patients, 3 developed biliary complications and 1 had arterial stenosis. No thrombosis was observed., Conclusion: The data suggested that use of an AG is useful alternative in pediatric OLT. The technique is safe with a low risk of thrombosis., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background/purpose: Posttransplantation portal vein thrombosis (PVT) can have severe health consequences, and portal hypertension and other consequences of the long-term privation of portal inflow to the graft may be hazardous, especially in young children. The Rex shunt has been used successfully to treat PVT patients since 1998. In 2007, we started to perform this surgery in patients with idiopathic PVT and late posttransplantation PVT. Herein we have reported our experience with this technique in acute posttransplantation PVT., Methods: Three patients of ages 12, 15, and 18 months underwent cadaveric (n = 1) or living donor (n = 2) orthotopic liver transplantation (OLT). All patients had biliary atresia with portal vein hypoplasia; they developed acute PVT on the first postoperative day. They underwent a mesenteric-portal surgical shunt (Rex shunt) using a left internal jugular vein autograft (n = 2) or cadaveric iliac vein graft (n = 1) on the first postoperative day., Results: The 8-month follow-up has confirmed shunt patency by postoperative Doppler ultrasound. There have been no biliary complications to date., Conclusions: The mesenteric-portal shunt (Rex shunt) using an autograft of the left internal jugular or a cadaveric vein graft should be considered for children with acute PVT after OLT. These children usually have small portal veins; reanastomosis is often unsuccessful. In addition, this technique has the advantage to avoid manipulation of the hepatic hilum and biliary anastomosis. Although this study was based on a limited experience, we concluded that this technique is feasible, with great benefits to and low risks for these patients., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Introduction: Biliary atresia (BA) is the leading indication for orthotopic liver transplantation (OLT) among children. However, there are technical difficulties, including the limited dimensions of anatomical structures, hypoplasia and/or thrombosis of the portal vein and previous portoenterostomy procedures., Objective: The objective of this study was to present our experience of 239 children with BA who underwent OLT between September 1989 and June 2010 compared with OLT performed for other causes., Methods: We performed a retrospective analysis of patient charts and analysis of complications and survival., Results: BA was the most common indication for OLT (207/409; 50.6%). The median age of subjects was 26 months (range, 7-192). Their median weight was 11 kg (range, 5-63) with 110 children (53.1%) weighing ≤10 kg. We performed 126 transplantations from cadaveric donors (60.8%) and 81 from living-related donors (LRD) (39.2%). Retransplantation was required for 31 recipients (14.9%), primarily due to hepatic artery thrombosis (HAT; 64.5%). Other complications included the following: portal vein thrombosis (PVT; 13.0%), biliary stenosis and/or fistula (22.2%), bowel perforation (7.0%), and posttransplantation lymphoproliferative disorder (PTLD; 5.3%). Among the cases of OLT for other causes, the median age of recipients was 81 months (range, 11-17 years), which was higher than that for children with BA. Retransplantation was required in 3.5% of these patients (P < .05), mostly due to HAT. The incidences of PVT, bowel perforation, and PTLD were significantly lower (P < .05). There was no significant difference between biliary complications in the 2 groups. The overall survival rates at 1 versus 5 years were 79.7% versus 68.1% for BA, and 81.2% versus 75.7% for other causes, respectively., Conclusions: Children who undergo OLT for BA are younger than those engrafted for other causes, displaying a higher risk of complications and retransplantations., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Objective: The objective of this study was to report our experience with pediatric orthotopic liver transplantation (OLT) with living related donors., Methods: We performed a retrospective chart analysis of 121 living related donor liver transplantations (LRDLT) from June 1998 to June 2010., Results: Indications were biliary atresia (BA; n = 81), primary sclerosing cholangitis (n = 5), α-1 antitrypsin deficiency (n = 4); cholestasis (n = 9), fulminant hepatic failure (n = 8), autoimmune hepatitis (n = 2), Alagille syndrome (n = 4), hepatoblastoma (n = 3), tyrosinemia (n = 2), and congenital hepatic fibrosis (n = 3). The age of the recipients ranged from 7-174 months (median, 22) and the weights ranged from 6-58 kg (median, 10). Forty-nine children (40.5%) weighed ≤10 kg. The grafts included the left lateral segment (n = 108), the left lobe (n = 12), and the right lobe (n = 1). The donors included 71 mothers, 45 fathers, 2 uncles, 1 grandmother, 1 grandfather, and 1 sister with a median age of 29 years (range, 16-53 ys) and a median weight of 68 kg (range, 47-106). Sixteen patients (12.9%) required retransplantation, most commonly due to hepatic artery thrombosis (HAT; n = 13; 10.7%). The other complications were biliary stenosis (n = 25; 20.6%), portal vein thrombosis (PVT; n = 11; 9.1%), portal vein stenosis (n = 5; 4.1%), hepatic vein stenosis (n = 6; 4.9%), and lymphoproliferative disorders (n = 8; 6.6%). The ultimate survival rate of recipients was 90.3% after 1 year and 75.8% after 3 years. Causes of early death within 1 month were HAT (n = 6), PVT (n = 2), severe graft dysfunction (n = 1), sepsis (n = 1), and intraoperative death in children with acute liver failure (n = 2). Causes of late deaths included lymphoproliferative disease (n = 3), chronic rejection (n = 2), biliary complications (n = 3), and recurrent disease (n = 3; hepatoblastoma and primary sclerosing cholangitis)., Conclusions: Despite the heightened possibility of complications (mainly vascular), LRDLT represented a good alternative to transplantation from cadaveric donors in pediatric populations. It was associated with a high survival ratio., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Posttransplantation lymphoproliferative disorder (PTLD) is a serious complication following solid organ transplantation that has been linked to Epstein-Barr virus (EBV) infection. The aim of this article was to describe a single-center experience with the multiplicity of clinical presentations of PTLD. Among 350 liver transplantations performed in 303 children, 13 survivor children displayed a histological diagnosis of PTLD (13/242 survivors; 5.4%). The age at diagnosis ranged from 12 to 258 months (median, 47), and the time from transplantation ranged from 1 to 84 months (median, 13). Ten of these children (76.9%) were EBV-naïve prior to transplantation. Fever was present in all cases. The clinical signs at presentation were anemia (92.3%), diarrhea and vomiting (69.2%), recurrent upper airway infections (38.4%), Waldeyer ring lymphoid tissue hypertrophy (23.0%), abdominal mass lesions (30.7%), massive cervical and mediastinal adenopathy (15.3%), or gastrointestinal and respiratory symptoms (30.7%). One child developed fulminant hepatic allograft failure secondary to graft involvement by PTLD. Polymorphic PTLD was diagnosed in 6 patients; 7 had the diagnosis of lymphoma. Treatment consisted of stopping immunosuppression as well as starting intravenous gancyclovir and anti-CD20 monoclonal antibody therapy. The mortality rate was 53.8%. The clinical presentation of PTLD varied from fever of unknown origin to fulminant hepatic failure. The other symptoms that may be linked to the diagnosis of PTLD are pancytopenia, tonsil and adenoid hypertrophy, cervical or mediastinal lymph node enlargement, as well as abdominal masses. Despite numerous advances, the optimal treatment approach for PTLD is not completely known and the mortality rate is still high.

Primary tracheal malignant neoplasms are very rare. Histologically, squamous cell and adenoid cystic carcinomas are the most common types of malignant primary tracheal tumors when all age groups are studied. In the past 5 years, we treated 2 children with tracheal mucoepidermoid carcinoma. Herein we report both cases and review the literature on the subject with particular emphasis on diagnosis and surgical management.

Background: Endoscopic sclerotherapy (ES) has been the standard treatment for children with idiopathic extrahepatic portal vein obstruction (EHPVO). Portosystemic shunts are indicated when variceal bleeding cannot be controlled by ES. Recently, mesenteric left portal vein bypass was indicated as a surgical intervention and preventative measure for hepatic dysfunction in children with long-term EHPVO. Nevertheless, there is a lack of published data confirming the extent of hepatic dysfunction, hypersplenism, and physical development in children with long-term follow-up., Method: We retrospectively verified the long-term outcomes in 82 children with EHPVO treated with ES protocol, focusing on mortality, control of bleeding, hypersplenism, and consequent hepatic dysfunction., Results: Of the children, 56% were free from bleeding after the initiation of ES. The most frequent cause of rebleeding was gastric varices (30%). Four patients had recurrent bleeding from esophageal varices (4.6%). Four patients underwent surgery as a consequence of uncontrolled gastric varices. There were no deaths. Most patients showed good physical development. We observed a mild but statistically significant drop in factor V motion, as well as leukocyte and platelet count., Conclusion: Endoscopic sclerotherapy is an efficient treatment for children with EHPVO. The incidence of rebleeding is low, and there was no mortality. Children develop mild liver dysfunction and hypersplenism with long-term follow-up. Only a few patients manifest symptoms of hypersplenism, portal biliopathy, or liver dysfunction before adolescence.

Background and Purpose: Late portal vein thrombosis (PVT) can be extremely well tolerated, although portal hypertension and other consequences of the long-term deprivation of portal inflow to the graft may be hazardous, especially in young children. Recently, the "Rex shunt" has been used successfully to treat these patients. We now report the initial experience with this novel technique., Methods: A 3-year-old girl with PVT at 7 months after whole organ cadaveric liver transplant displayed portal hypertension with an episode of gastrointestinal bleeding, requiring a mesenteric-portal surgical shunt ("Rex shunt") using a left internal jugular vein autograft., Results: Upon current follow-up of 6 months, postoperative Doppler ultrasound confirmed shunt patency. Endoscopic status was significantly improved after surgery with resolution of portal hypertension. There was no recurrence of bleeding., Conclusions: The mesenteric-portal shunt ("Rex shunt"), using a left internal jugular vein autograft, should be considered for children with late PVT after liver transplantation. Although this is an initial experience, we may conclude that this technique is feasible, with great potential benefits and low risks for these patients.

Background and Aims: Liver transplantation (OLT) in children has seen significant improvements in recent years. Long-term immunosuppressive strategies have focused on avoiding the risks of long-term immunosuppression, particularly nephrotoxicity, de novo malignancy and late infections. Since its introduction in renal transplantation in 1999, sirolimus (SRL) has been used by an increasing number of liver transplant centers. The aim of this study was to review the experience using SRL in pediatric liver transplant recipients at a single center., Methods: Between 1989 and 2006, 318 children underwent OLT including 13 who were converted to SRL therapy because of tacrolimus-related side effects. The indications were posttransplant lymphoproliferative disease (PTLD; n = 11), nephrotoxicity (n = 1), and de novo autoimmune hepatitis (n = 1). One patient with PTLD previously concurrently displayed chronic rejection. SRL dosages ranged between 0.4 and 5 mg/d. The median duration of follow-up was 18 months., Results: PTLD recurred in 1 patient. There were no episodes of acute rejection. One child developed hyperlipidemia that resolved with diet and medication., Conclusions: Conversion from tacrolimus to SRL in selected pediatric liver transplant recipients is safe. Children with PTLD may benefit from immunosuppression with SRL after liver transplantation.

Background: Upper airway stenosis (UAS) after endotracheal intubation is a common problem in children. Most literature refers to a surgical treatment for these lesions. Laryngotracheal reconstruction and cricotracheal resection are used for low- and high-grade stenosis, but decannulation is not always possible immediately after surgery., Purpose: The aim of this study was to verify the feasibility and results of endoscopic dilatations for treatment of subglottic stenosis., Method: The study encompassed a 12-year retrospective analysis of patients treated for UAS in a tertiary center. All children were symptomatic at the time of the endoscopic diagnosis. The stenosis was graded according to the Myer-Cotton criteria. Endoscopic dilatation was initiated immediately after the diagnosis. Children with grade IV stenosis underwent surgery., Results: Children with tracheal stenosis and no involvement of the subglottic area did not respond to endoscopic dilatations and underwent surgery. There were 45 children with grade I stenosis, 12 with grade II, 7 with grade III, and 4 with grade IV lesions. Patients with grade I, II, and III stenosis were all treated by endoscopic dilatations alone and were decannulated when asymptomatic. The average time for decannulation was 18.90 +/- 26.07 months for grade I, 32.5 +/- 27.08 months for grade II, and 27.57 +/- 20.60 months for grade III stenosis (P < .01, for grade II vs grade III)., Conclusion: Grade I, II, and III subglottic stenoses can be safely managed by early endoscopic dilatations with a high rate of success and low rate of morbidity but require a significantly long period of treatment.

HAT is the main cause of graft loss in pediatric living-related LTx. Revascularization of the graft by thrombectomy and re-anastomosis has been reported to be effective for graft salvage in cases of HAT and should be attempted when potential donors are not available for emergency re-transplantation. Immediate complications secondary to revascularization attempts in cases of HAT are not described. Late complications are mainly related to biliary tree ischemia. We report a case of child who experienced intimal hepatic artery dissection, which extended into intra-hepatic branches of the artery after a thrombectomy with a Fogarty balloon catheter in an attempt to restore arterial flow after HAT. This complication led to acute deterioration of the graft and the need for emergency re-transplantation.

Background/purpose: Esophagocoloplasty and gastric transposition are 2 major methods of esophageal substitution in children. The purpose of this study is to review the authors' experience with these 2 techniques and compare the complications of these operations to determine whether 1 method emerges superior to the other., Methods: A total of 149 children underwent surgery: 115 children underwent esophagocoloplasty, and 34 children underwent gastric transposition. Most patients (113-75.8%) had long-gap esophageal atresia. The operative technique of esophagocoloplasty consisted of the interposition of the transverse colon maintained by a double vascular pedicle based on the left colic vessels and the marginal paracolic arcade. Gastric transposition was performed according to classical technique. The transposition of colon and stomach was performed using blunt mediastinal dissection in all patients without thoracotomy. Complications and mortality of the 2 groups of patients were compared. These complications were classified as minor (cervical anastomosis leak, abdominal evisceration, diarrhea, strictures, and reflux to the interposed viscera) and major (necrosis of transposed viscera, dehiscence of an intra-abdominal or intrapleural suture, torsion of transposed viscera, delayed gastric emptying requiring reoperation, and cologastric anastomosis stricture)., Results: There were 2 graft necrosis, 1 (0.8%) in the esophagocoloplasty group and another (2.9%) in the gastric transposition group. Patients who underwent esophagocoloplasty experienced a greater incidence of minor complications (P = .001) and less major complications in comparison with the gastric transposition group (P = .001). All minor complications were treatable and had no consequences. No difference was noted between the 2 groups with regard to the mortality rate (0.9% and 5.9%, respectively, P > .05)., Conclusions: Esophagocoloplasty and gastric transposition are satisfactory means of esophageal substitution in children. Considering the incidence of major postoperative complications, esophagocoloplasty must be the first choice for esophageal replacement in children.

Few studies have evaluated the long-term use of MMF in liver transplanted children with renal dysfunction. The aim of this study is to report the experience of a pediatric transplantation center on the efficacy and security of long-term use of a MMF immunosuppressant protocol with reduced doses of CNIs in stable liver transplanted children with renal dysfunction secondary to prolonged use of CsA or Tac. Between 1988 and 2003, 191 children underwent OLT and 11 patients developed renal dysfunction secondary to CNIs toxicity as evaluated by biochemical renal function parameters. The interval between liver transplantation and the introduction of the protocol varied from one to 12 yr. Renal function was evaluated by biochemical parameters in five phases: immediately prior to MMF administration; 3, 6, 12 and 24 months after the introduction of MMF. Among the patients, nine of them (82%) showed improvement of renal function parameters in comparison with the pretreatment values. The two patients that did not show any improvement were patients in whom the interval of time between OLT and the introduction of MMF was longer. All parameters of liver function remained unchanged. No episodes of acute or chronic rejection or increases in infection rates during the period were detected. Two patients developed transitory diarrhea and leukopenia that were reverted with reduction of MMF dosage. In conclusion, in liver transplanted pediatric patients with CNI-induced chronic renal dysfunction, the administration of MMF in addition to reduced doses of CNIs promotes long-term improvement in renal function parameters with no additional risks.

Recent reports suggest that the technique of abdominal closure in neonates with anterior abdominal wall defects (AWD) correlates with the outcome. The aim of this study is to analyze factors related to mortality and morbidity, according to the technique of abdominal closure of these neonates. Retrospective analysis of charts from 76 consecutive neonates with AWD treated in a single institution. They were divided according to the type of abdominal wall closure: group I: primary closure, group II: silo followed by primary closure and group III: silo followed by polypropylene mesh. Outcome was analyzed separately for neonates with gastroschisis and omphalocele. There were 13 deaths (17.1%). Mortality for neonates with isolated defects was 9.6%. Mortality rate was similar in all groups for either neonates with gastroschisis or omphalocele. Postoperative complications were not significantly different among groups except for a prolonged time of hospitalization in group III. Mortality rate is not correlated with the type of abdominal closure. Neonates with primary closure or with other methods of abdominal wall closure had similar rate of postoperative complications. Neonates with mesh closure of the abdomen have prolonged hospitalization. The use of a polypropylene mesh is a good alternative for neonates whose primary closure or closure after silo placement is not possible.

Over a 15-yr period of observation, among the 205 children who underwent liver transplantations, one of them developed a particular type of late graft dysfunction with clinical and histological similarity to autoimmune hepatitis. The patient had alpha1-antitrypsin deficiency and did not previously have autoimmune hepatitis or any other autoimmune disease before transplantation. Infectious and surgical complications were excluded. After repeated episodes of unexplained fluctuations of liver function tests and liver biopsies demonstrating reactive or a biliary pattern, without any corresponding alteration of percutaneous cholangiography, a liver-biopsy sample taken 4 yr after the transplant showed active chronic hepatitis progressing to cirrhosis, portal lymphocyte aggregates, and a large number of plasma cells. At that time, autoantibodies (gastric parietal cell antibody, liver-kidney microsomal antibody, and anti-hepatic cytosol) were positive and serum IgG levels were high. Based on these findings of autoimmune disease, a diagnosis of 'de novo autoimmune hepatitis' was made. The treatment consisted of reducing the dose of cyclosporine, reintroduction of corticosteroids, and addition of mycophenolate mofetil. After 19 months of treatment, a new liver-biopsy sample showed marked reduction of portal and lobular inflammatory infiltrate, with regression of fibrosis and of the architectural disruption. At that time, serum autoantibodies became negative. The last liver-biopsy sample showed inactive cirrhosis and disappearance of interface hepatitis and of plasma cell infiltrate. Presently, 9 yr after the transplantation, the patient is doing well, with normal liver function tests and no evidence of cirrhosis. Her immunosuppressive therapy consists of tacrolimus, mycophenolate mofetil, and prednisolone. In conclusion, the present case demonstrates that de novo autoimmune hepatitis can appear in liver-transplant patients despite appropriate anti-rejection immunosuppression, and triple therapy with tacrolimus, mycophenolate mofetil, and prednisolone could sustain the graft and prevent retransplantation.

The classical method for arterial reconstruction in pediatric living donor liver transplantation using left lateral segment consists of end-to-end anastomosis between the donor left hepatic artery and the recipient right hepatic artery. In the present case, an intra-operative hepatic artery thrombosis occurred because of extensive intima wall dissection of the recipient hepatic artery. The patient was a 6-yr-old boy with fulminant hepatic failure, who underwent living donor partial liver transplantation with left lateral segment from his father. The graft was irrigated by a left hepatic artery and an accessory left hepatic artery from gastric artery, both arteries with diameter of <2 mm. These arteries were anastomosed to the recipient right and left hepatic arteries, respectively. Before performing the bile duct reconstruction it was noted that these anastomoses were occluded by clots of blood. An extensive subintimal dissection of the recipient hepatic artery was the cause of this problem. The creation of a new anastomosis by using a more proximal part of this artery without subintimal dissection was judged impossible. Then, the right gastroepiploic artery was mobilized and an anastomosis was performed with the donor left hepatic artery in an end-to-end fashion. Arterial blood flow to the graft was established successfully and the patient's postoperative recovery was excellent. Fifteen days after the transplantation, an angiotomography demonstrated a good hepatic arterial blood flow. The patient is now alive and well, 4 months after the transplantation. In conclusion, the method of hepatic graft arterialization described here is an important option for patients who undergo living donor or split liver transplantation.

In pediatric patients submitted to living related liver transplantation, hepatic venous reconstruction is critical because of the diameter of the hepatic veins and the potential risk of twisting of the graft over the line of the anastomosis. The aim of the present study is to present our experience in hepatic venous reconstruction performed in pediatric living related donor liver transplantation. Fifty-four consecutive transplants were performed and two methods were utilized for the reconstruction of the hepatic vein: direct anastomosis of the orifice of the donor left or left and middle hepatic veins and the common orifice of the recipient left and middle hepatic veins (group 1-26 cases), and wide triangular anastomosis after creating a wide triangular orifice in the recipient inferior vena cava at the confluence of all the hepatic veins with an additional longitudinal incision in the inferior angle of the orifice (group 2-28 cases). In group 1, eight patients were excluded because of graft problems in the early postoperative period and five among the remaining 18 patients (27.7%) presented stricture at the site of the hepatic vein anastomosis. All these patients had to be submitted to two or three sessions of balloon dilatations of the anastomoses and in four of them a metal stent had to be placed. The liver histopathological changes were completely reversed by the placement of the stent. Among the 28 patients of the group 2, none of them presented hepatic vein stenosis (p = 0.01). The results of the present series lead to the conclusion that hepatic venous reconstruction in pediatric living donor liver transplantation must be preferentially performed by using a wide triangulation on the recipient inferior vena cava, including the orifices of the three hepatic veins. In cases of stenosis, the endovascular dilatation is the treatment of choice followed by stent placement in cases of recurrence.

This study reports the 14-year experience of a single center on 206 liver transplantations from living and cadaveric donors performed in 179 pediatric patients. Biliary atresia (57.2%) and fulminant hepatitis (9.8%) were the most frequent indications. The mean age of the recipients was 3 years, 7 months (9 months to 18 years) and mean weight was 14 kg (7 to 57 kg). The allografts were distributed as 82 (39.8%) whole cadaveric, 76 (36.9%) reduced-size cadaveric, 46 (22.3%) living donor liver transplants, and 2 (0.9%) ex situ split livers. The waiting periods were 25 days for living donors and 2.5 years for cadaveric donors (P <.001). Twenty-seven children were retransplanted with hepatic artery thrombosis the most frequent indication. The postoperative complications were: primary nonfunction (12.2%), biliary stenosis (28.8%), hepatic artery thrombosis (12.2%), portal vein stenosis (4.9%), hepatic vein stenosis (6.9%), and lymphoproliferative disorder (5.9%). The diagnosis of biliary stenosis was obtained by liver biopsy and transhepatic cholangiography and treated by balloon dilatation, although four children (3.9%) required a redo hepaticojejunostomy. The venous stenoses were percutaneously dilated with five-children (4.9%) requiring venous stents. The incidence of hepatic vein stenosis was 15.6% among living donor and 2.5% in cadaveric liver transplantation (P <.05). The overall 5-year patient and graft survivals were 70.2% and 65.1%. Liver transplantation provides excellent long-term survival. The use of grafts from living donors decreases the waiting periods but increases the incidence of hepatic vein stenosis.

Basiliximab is a monoclonal antibody that binds to the alpha subunit (CD(25)) of the interleukin-2 receptor of activated T lymphocytes. The advantage of basiliximab in organ transplantation is the reduce possibility to calcineurin inhibitor dosages to avoid nephrotoxicity. Basiliximab has significantly reduced the incidence of acute rejection (AR) in renal transplant recipients; however, the results are uncertain in liver transplantation (LT). The objective of this investigation was to assess the effect of basiliximab to prevent AR in the first 6 months after pediatric LT. From March 2000 to October 2001, 32 recipients of a primary orthotopic cadaveric or living donor LT were given basiliximab by intravenous bolus injection on the day of transplantation (day 0) and on day 4. Four children who received one dose were excluded from the study. The rate and the intensity of AR episodes, the incidence of chronic rejection, serum creatinine level, incidence of infections, adverse side effects, and daily oral dosage of cyclosporine (Neoral) to maintain the target blood level of 850 to 1000 mg/dL at C2, 2 hours after the administration, were analyzed in the remaining 28 recipients. Results were compared to those obtained from a matched historical group (n = 28) of similar age, weight, and hepatic diseases distribution. None of the analyzed parameters was statistically significant (P >.05) except for the daily oral dose of cyclosporine (7 to 13 mg/kg/dose, P <.05). In our series, the addition of basiliximab to the immunosuppressive therapy did not reduce the incidence of AR in pediatric LT.

Objective: To evaluate the prognostic factors importance to survival in neonatal period of newborns with congenital diaphragmatic hernia treated at Hospital das Clínicas, School of Medicine of Universidade de São Paulo, and to compare the outcome with data published in medical literature. So that the results of this study might allow updating family counseling and guiding changes in clinical management of our department., Methods: Retrospective study of 27 consecutive newborns with congenital diaphragmatic hernia admitted to the Pediatric Surgery Department of Instituto da Criança, School of Medicine of Universidade de São Paulo, from April 1991 to January 2002, and statistical comparison with medical literature metanalysis data., Results: Of 27 patients, 15 were born at our institution and 12 were admitted by transference after birth. Twelve (44%) have had congenital diaphragmatic hernia diagnosed prenatally and 23 (85%) were full-term newborns. Most patients presented early respiratory distress and needed intubation at delivery room. Six newborns presented criteria for indication of extracorporeal membrane oxygenation. Twenty patients (74%) were submitted to operative repair and seven (26%) died without the minimal clinical stabilization necessary for surgical procedure (five of these patients reached criteria for indication of extracorporeal membrane oxygenation). The postoperative mortality was 25% (5/20). The overall survival of neonatal period was 56% (15/27). The survival of patients that were born at our hospital was 33% (4/12), and the survival of the newborns admitted by transference was 73% (11/15). Severe respiratory distress, early indication to mechanical ventilation and severe hypoxemia (post-ductal pO2 < 100 mmHg despite all efforts) were identified as predictors of bad outcome with statistical significance., Conclusion: Our high mortality rate of newborns with congenital diaphragmatic hernia is statistically similar to that described in international publications. In the group of non-responsive patients to standard treatment available, the use of extracorporeal membrane oxygenation should be able to reduce mortality. The impact of this therapeutical strategy in the overall survival depends on other factors that were not analyzed in the present study. Family counseling of patients' parents on congenital diaphragmatic hernia in our department may follow the same patterns referred in world medical literature.

Purpose: The aim of this study was to evaluate the outcome of children with pure esophageal atresia (EA) managed by the strategy of delayed primary anastomosis and to find parameters to identify which of these children will have satisfactory esophageal growth to permit a successful primary esophageal anastomosis., Methods: Sixteen children with pure EA were treated by delayed anastomosis strategy from June 1992 to May 2001 at the Instituto da Criança-University of São Paulo Medical School. The treatment consisted of creating an early feeding gastrostomy, continuous upper pouch suction, and periodic radiologic/endoscopic assessments of the gap between the superior and inferior esophageal pouches. The first assessment was done 4 weeks after the initial gastrostomy. Subsequent assessments were done, when necessary, 4 to 10 weeks after the first one. Children with the gap between the pouches corresponding to 2 vertebral bodies or less were considered for primary repair., Results: The waiting period ranged from 8 to 14 weeks. Five children were considered for primary repair (31.3%). All of them had an end-to-end esophageal anastomosis performed without tension. Eleven children (68.7%) did not show significant esophageal growth after the waiting period and were not considered for primary esophageal anastomosis. Eight of them (50%) showed an entirely intraabdominal distal pouch at the first assessment., Conclusions: (1) Infants with an exclusively intraabdominal distal pouch at the first evaluation will not reach sufficient elongation of the pouches and should be considered early as a candidate for esophageal replacement. (2) Combined radiologic/endoscopic assessment and the measurement of the gap in terms of vertebral bodies is a practical and reliable method to predict the feasibility of primary anastomosis. (3) Using these criteria, primary anastomosis could be accomplished in less than a third of cases., (Copyright 2002, Elsevier Science (USA). All rights reserved.)

Objective: To present the study carried out by the Pediatric Surgery Department of Instituto da Criança at the Medical School of Universidade de São Paulo regarding the diagnosis and treatment of children with aortic arch abnormalities and to define the role of complementary exams for diagnosis., Methods: Retrospective study of 22 patients with diagnosis of tracheoesophageal compression treated at Instituto da Criança from 1985 to 2000, analyzing pre- and postoperative clinical data, diagnostic exams and outcome., Results: The most frequent diagnosis was right aberrant innominate artery (10 cases), followed by double aortic arch (7 cases) and right aortic arch (5 cases). Respiratory symptoms (86%) and early manifestation (76% since the neonatal period) were predominant. Nevertheless, most cases (60%) had the definitive diagnosis established only after 1 year of life. The most relevant examination for the diagnosis was the esophagogram. The correction of all the anomalies was carried out through left postero-lateral thoracotomy. There were no surgical complications. The outcome was worse in patients with delayed treatment. All children remained symptomatic for up to 6 months, although they had significant improvement in the postoperative period., Conclusions: The diagnosis of vascular rings should be considered in children with early respiratory symptoms and in the wheezing baby with difficult control. The diagnosis may be established just through the esophagogram. Other image studies add few information and they are unnecessary in most cases. Less severe symptoms may persist for variable periods.

OBJECTIVES: To present the clinical outcome of a newborn with severe respiratory distress secondary to meconium aspiration syndrome and treated by extracorporeal membrane oxygenation (ECMO); and to present the effect of the use of exogenous surfactant in this case and the cost of the procedure. METHODS: Case report of a newborn with meconium aspiration syndrome and treated at the neonatal ICU of the Instituto da Criança Prof. Pedro de Alcantara, Hospital das Clínicas of the Universidade de São Paulo. RRESULTS: ECMO was carried out for 5 days with no clinical or mechanical complications. On the 4th day of ECMO, we administered porcine exogenous surfactant; a significant improvement in lung compliance was observed and the newborn was decannulated shortly after that. Treatment costs were compatible with the situation of healthcare in Brazil for treatment of critically ill newborn patients. CONCLUSIONS: ECMO is indicated in cases of neonatal respiratory distress not responding to other treatments. The technique should be made available in neonatal Intensive Care Units (ICUs) of tertiary hospitals according to well-established protocols. The use of exogenous surfactant apparently allowed for earlier decannulation of the patient and should be considered in similar cases. The treatment costs do justify the organizing of ECMO teams in this type of ICUs.

Background/purpose: Best results in experimental tracheal allotransplantation are obtained when metachronous revascularization by omentopexy and immunosuppression are used. Nevertheless, this method of revascularization implies in a 4-day period of ischemia to the graft. The aim of this study was to assess the influence of the 4-day period of ischemia on host sensitization as well as the effect of early or delayed immunosuppression on the outcome of the grafts., Methods: Thirty rabbits were submitted to tracheal allotransplantation and divided according to position of the graft (orthotopic or heterotopic transplants) and the initiation of immunosuppression (early or delayed). The quality of the revascularization was evaluated by the identification of Indian ink, perfused through the abdominal aorta, inside the submucosal vessels. The outcome of the grafts was evaluated by histological analysis according to a semiquantitative scale of alterations., Results: Grafts were better revascularized in heterotopic position. Grafts with late immunosuppression presented good outcome only when heterotopically positioned. No significant differences were observed in grafts placed heterotopically or orthotopically when immunosuppression was initiated early after the transplant., Conclusions: Transient ischemia produced by metachronous revascularization is not the single factor responsible for the histological alterations observed in tracheal allografts. These alterations probably also are produced by the activation of immune responses. This activation is more intense in more ischemic grafts, but can be suppressed by early administration of immunosuppression.

Background/purpose: A new noninvasive therapeutic strategy, which consisted of prenatal intraamniotic administration of porcine surfactant or dexamethasone, was previously used to prevent the functional and structural immaturity of lungs associated with congenital diaphragmatic hernia (CDH), and its effects on lung development were comparable with the changes induced by tracheal ligation (TL). The purpose of this study is to verify if this novel therapeutic modality has any effect in the elevated concentration of lung glycogen and altered contents of lung elastic fiber and collagen promoted by CDH., Methods: A pilot study was performed to investigate in the rabbit model if the infused drugs in the amniotic cavity were aspirated by the CDH and non-CDH fetuses, and if there was correspondence between lung immaturity and high glycogen concentration in lung tissue. Experimental groups consisted of 50 pregnant rabbits that underwent surgery on gestational day 24 or 25 to create left-sided diaphragmatic hernias in 56 fetuses, which were divided in groups according to the procedures: CDH (n = 12), CDH plus TL (n = 16), CDH plus intraamniotic administration of Curosurf (40 mg, n = 12), and CDH plus intraamniotic administration of dexamethasone (n = 16). On gestational day 30, the fetuses were delivered by cesarean section, and 28 normal unoperated fetuses served as controls. The lungs were weighed and submitted to biochemical determination of glycogen, morphometric evaluation of elastic fibers, and colorimetric analysis of collagen., Results: In all CDH and non-CDH fetuses of the pilot study, the amniotic content was massively aspirated into the lungs and trachea. There was an increase in lung glycogen content of fetuses at 24 days' gestation in comparison with 20-day gestational age fetuses, followed by a decrease in the near full-term fetuses. In the fetuses of the experimental groups, CDH decreased the lung weight to body weight ratios of lungs ipsilateral to the hernia. These changes were reversed by TL but not by intraamniotic administration of surfactant or dexamethasone. Lung glycogen concentrations in the lungs of CDH fetuses were significantly higher than those in the control group. These changes were reversed by intraamniotic administration of surfactant but not by dexamethasone administration or TL. In the lungs ipsilateral to the hernia, surfactant administration promoted a significant decrease in glycogen content to levels lower than control lungs. CDH promoted a decrease in the linear density of elastic fibers in both lungs, ipsilateral and contralateral to the hernia. This alteration was partially corrected by TL and surfactant administration, although dexamethasone administration had no effect. The concentrations of collagen in both lungs were increased significantly by CDH, and these alterations could not be reversed by TL. In the lungs ipsilateral to the hernia, intraamniotic administration of surfactant or dexamethasone promoted a significant decrease in the lung concentration of collagen but not to control levels., Conclusions: The positive effects of intraamniotic surfactant or dexamethasone administration on lung maturity of fetuses with CDH were observed. This therapy may be a substitute for TL.

Background/purpose: Lung surfactant deficiency contributes to the pathophysiology of congenital diaphragmatic hernia (CDH) and the high neonatal mortality rate. Acceleration of lung surfactant system maturation by prenatal administration of hormones has been described in animal models of CDH. However, in utero tracheal ligation (TL) is the best method to accelerate lung growth and reverse the pulmonary hypoplasia associated with CDH. Although this method offers promise, its application in humans is limited. The aim of this study was to investigate a new noninvasive therapeutic strategy, that is, the prenatal intraamniotic administration of exogenous porcine surfactant or dexamethasone, and compare it with the effects of TL in an animal model of CDH., Methods: Twenty-four pregnant New Zealand rabbits underwent surgery on gestational day 24 or 25 to create CDH in 26 fetuses. Five groups of animals were studied: (1) Control, nonoperated fetuses (n=14), (2) CDH (n=6), (3) CDH plus TL (n 6), (4) CDH plus intraamniotic administration of Curosurf (40 mg; n=6), and (5) CDH plus intraamniotic infusion of dexamethasone (0.4 mg; n=8). On gestational day 30, the fetuses were delivered by cesarean section. Functional studies (lung hysteresis curves and lung distensibility), weight and volume of lungs, histopathologic and histomorphometric analysis of lungs were performed., Results: The authors demonstrated that the hysteresis curve of CDH animals was shifted downward in comparison with controls. The analyses of curves standardized for lung weight indicated that intraamniotic administration of surfactant or dexamethasone improved lung compliance in comparison with controls and CDH fetuses, but TL had no effect on this parameter. Lung distensibility (maximum lung volume at 32 cm of water pressure per gram of lung) was reduced by CDH, but this parameter was increased by intraamniotic administration of drugs and not by TL (P< .05). CDH decreased the weight and volume of lungs (P< .05), and these changes were reversed only by TL, which prevented the herniation of the liver from the abdomen to the thorax. Histologically, CDH lungs treated with TL or intraamniotic administration of drugs demonstrated structural patterns similar to those of controls. Histomorphometric studies proved that CDH promoted significant thickening of septa walls (P< .05), and all the therapeutic methods could reverse this alteration to control values. The alveolar number per area in control lungs, CDH, and CDH plus TL lungs were similar, but in CDH plus surfactant and CDH plus dexamethasone lungs, the decreased number per area (P< .05) demonstrated that the alveolar airspace was increased., Conclusion: From these data the authors conclude that intraamniotic surfactant or dexamethasone administration is capable of preventing pulmonary hypoplasia in fetuses with CDH, and thus, this method may be a substitute for TL.

The description of certain surgical technical modifications of pediatric esophagocoloplasty and their impact on morbidity and mortality rates are presented. Seventy children, aged 12 to 120 months (mean, 52.3 +/- 39.5), were divided in two groups. Group 1 (40 patients), which represents a historical group, underwent esophagocoloplasty by the conventional technique. Group 2 (30 patients) had the following modifications to the operation: (1) preservation of the double blood supply to the interposed colon, based on the left colic vessels and left paracolic arcade, via the sigmoid vessels; (2) low cologastric anastomosis, performed at the lowest level of the anterior antrum; (3) in cases of retrosternal transposition (25 patients), fixation of the inferior border of the liver to the diaphragm and anterior abdominal wall; and (4) complete section of the left anterior muscles, behind the colon. Five patients in group 2 were supposed to undergo surgical correction of a congenital cardiac anomaly and had the colon transposed through the posterior mediastinum, on the original esophageal bed. The incidence of graft necrosis, gastrocolic reflux, esophagocolic anastomotic leak, and dysphagia are compared between the groups; the survival rates also were compared. Statistical analysis was performed using the Fisher-Yates' test, with significance set at .05. Groups 1 and 2 had the following complication rates, respectively: graft necrosis, 12.5% and 0% (P < .05); gastrocolic reflux, 20.0% and 0% (P < .05); dysphagia, 9.5% and 0% (P < .05); and esophagocolic anastomosis leak, 28.5% and 33.3% (not significant). The mortality rate was 17.5% for group 1 and 3.5% for group 2 (P < .05).(ABSTRACT TRUNCATED AT 250 WORDS)

From February 1985 to November 1992, 14 children with aortic arch anomalies and tracheal and/or esophageal compression were treated at the Pediatric Surgery Division of the São Paulo University School of Medicine. There were 3 cases of double aortic arch, 3 of right aortic arch with ligamentum arteriosum and 4 of aberrant right subclavian artery. Accurate diagnosis was based only on barium esophagoradiogram and, eventually, tracheobroncoscopy. The surgical approach was made through a left posterolateral thoracotomy that allowed an adequate exposure of the malformations. There was no operative mortality. There were 3 late post-operative deaths, 2 of them caused by chronic pulmonary lesions owing to delayed diagnosis. The other child died from complications of a severe tracheomalacia which became symptomatic only after the corrective surgery. The diagnosis of the aortic arch anomalies should be early suspected in children with undetermined respiratory symptoms and can be easily made basically by esophagoradiogram and tracheoscopy.

A case of acute abdomen in a one-year-old infant with abdominal angiostrongyliasis is reported. The main clinical and pathologic features of this disease are discussed. The diagnosis is made only through a biopsy that shows maturing stages of the worm within the intestinal wall and/or mesentery. Neither eggs nor larvae have been found in human feces. Treatment has been by surgical resection of the inflammatory mass. The prevalence of this illness is underestimated because of the difficult diagnosis. The main purpose of the present article is to attract attention to this diagnostic possibility as a cause of abdominal pain and acute abdomen in childhood.