Pediatric liver transplantation: fourteen years of experience at the children institute in São Paulo, Brazil.

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-chimeric anti-IL2-R monoclonal antibody in pediatric liver transplantation: comparative study.

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.

Prognostic factors associated with congenital cystic adenomatoid malformation of the lung.

This study presents 18 cases of prenatally diagnosed congenital cystic adenomatoid malformation (CCAM) to identify potential factors that could predict prognosis. Comparisons of prenatal parameters were made between fetuses that survived and those that died perinatally. It was found that microcystic lesion, bilateral lung involvement and hydrops were each highly correlated with poor prognosis, while neither polyhydramnios nor mediastinal shift was significantly associated with had outcome. Fetal interventions were indicated only in two of the surviving cases: a thoracocentesis and a cysto-amniotic shunt. A therapeutic amniocentesis was performed in one case of polyhydramnios. The diagnosis of CCAM was histologically confirmed in all cases by necropsy or by postnatal lobectomy.

Prophylactic sclerotherapy in children with esophageal varices: long-term results of a controlled prospective randomized trial.

BACKGROUND/PURPOSE: Experience using endoscopic prophylactic sclerotherapy (PS) is restricted to adult patients and has led to conflicting results. There has not been a randomized, controlled study on the use of PS in children. The purpose of this study is to evaluate prospectively the value of PS to prevent the first hemorrhage from esophageal varices in children with portal hypertension and to assess the effect of PS on survival rate. METHODS: In a controlled, prospective, computer-based randomized trial, the effectiveness of PS was analyzed in 100 consecutive children allocated to a group receiving sclerotherapy (n = 50) or to a control group (n = 50) subjected only to regular clinical and endoscopic examinations. Clinical characteristics in both groups were similar. The minimum follow-up period was at least 18 months after the cessation of the sessions of sclerotherapy. RESULTS: After a median follow-up of 4.5 years, PS eliminated the esophageal varices in 47 of 50 (94%) patients but only 38 (76%) of them do not present upper digestive hemorrhage. Before complete obliteration of the varices, upper gastrointestinal bleeding occurred in 12 patients (24%). Six children (12%) had gastric varices, 3 of 6 of whom (50%) bled. Congestive hypertensive gastropathy was observed to occur in 8 (16%) patients, 4 of 8 of which (50%) had hemorrhagic episodes. Two patients bled from undetermined cause. In the control group, only 29 (58%) children remained free from esophageal variceal bleeding and 26 (52%) from any upper gastrointestinal bleeding (P<.05). During the follow-up period, the development of gastric varices was observed in 5 (10%) patients (P>.05) and of congestive hypertensive gastropathy in only 3 (6%) patients (P<.05), but none of them bled. PS does not improve survival rate. CONCLUSIONS: In children with cirrhotic and noncirrhotic portal hypertension, PS reduces the overall incidence of bleeding from esophageal varices that were eradicated in 94% of cases. The source of bleeding has been different in each group, being predominantly from esophageal varices in the control group and from the stomach in the prophylaxis group. When applied with appropriate technique, PS is a safe procedure with a low incidence of minor complications. PS does not change the incidence of gastric varices but increases the development of congestive hypertensive gastropathy. PS increases the risk of bleeding from the naturally formed gastric varices and from congestive hypertensive gastropathy. PS does not affect survival rate.

The effects of early and delayed immunosuppression in experimental tracheal transplantation with omentopexy.

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.

Métodos de prevención de las alteraciones estructurales pulmonares en la hernia diafragmática congénita. Estudio experimental con cirugía fetal / Preventive methods of the lung alterations in congenital diaphragmatic hernia. Experimental study with fetal surgery

La hipoplasia pulmonar es la causa de muerte en el 30 al 50 por ciento de los recién nacidos con hernia diafragmática congénita. La ligadura traqueal en el feto, previene esta alteración, estimulando el crecimiento de la vía aérea. El objetivo principal de este trabajo es investigar un método de prevención de la hipoplasia pulmonar, basado en la administración intraamniótica de drogas, pasible de ser utilizado en la especie humana. Fueron operadas 36 conejas en el día 24 de gestación. En el primer grupo de fetos se creó una hernia diafragmática. En otros tres grupos además de la hernia diafragmática se efectuaron tres procedimientos adicionales: a) ligadura traqueal b) administración intraamniótica de dexametasona c) administración intraamniótica de surfactante. Se tomó como grupo control a 14 fetos, no operados, en el día 30, se efectuó una cesarea y se procedió a canular la tráquea de todos los animales para medir los parámetros funcionales (curvas de histéresis y de compliance). Los pulmones fueron recolectados para estudios biométricos: peso, volúmen e histología (evaluación cualitativa) y estudios histomorfométricos: densidad de septos alveolares, número de alvéolos por mm y evaluación de la densidad de fibras elásticas y colágenas. Se observó que la hernia diafragmática provocó una disminución significativa en el peso y volúmen de ambos pulmones, con caída de la histéresis y la complacencia, espesamiento de los septos alveolares, disminución del contenido en fibras elásticas y aumento del colágeno. La administración de surfactante y corticoides intraamnióticos produjeron efectos similares a los de la ligadura traqueal, corrigiendo parcialmente las alteraciones pulmonares en los fetos con hernia diafragmática, presentando el surfactante efectos más acenntuados. Concluímos que la administración intraamniótica de surfactante o dexametasona pueden subsistir a la ligadura traqueal como método experimental de prevención de la hipoplasia pulmonar, presente en los fetos con hernia diafragmática congénita

Management of biliary atresia in the liver transplantation era: a 15-year, single-center experience.

BACKGROUND/PURPOSE: The aim of this study was to address the perioperative aspects of hepatoportoenterostomy (HPE) for biliary atresia (BA), through the study of a 15-year, single-center experience of the management of this disease. METHODS: One hundred twenty-seven patients were divided into three groups, depending on the variant of HPE performed: group A (n = 53) underwent HPE with external diversion of the Roux-en-Y anastomosis; group B (n = 54) underwent HPE with a long (35 to 40 cm) Roux-en-Y anastomosis, without diversion; and group C (n = 20) underwent the same kind of HPE as group B, but with a modified, "super extensive" dissection of the porta hepatitis. Eleven children in group B had an intussusception type antireflux valve in the Roux-en-Y loop. The porta hepatitis of 105 children was histologically classified in types I to III and correlated with rate of postoperative bile flow and age at surgery. Liver transplantation was performed after HPE in 20 patients. RESULTS: Overall, biliary drainage was achieved in 72.5% of the children after HPE and 26.8% of all patients became jaundice free. Porta hepatitis type III was associated with a significantly higher rate of biliary drainage post-HPE then types I and II. There was no difference in the rate of bile drainage, relative number of jaundice-free patients, and mean number of episodes of cholangitis per year among surgical groups A, B, C. In group A, 43.7% of the patients had complications related to the stoma. The actuarial survival of children who underwent HPE followed by liver transplantation was 85%. CONCLUSIONS: (1) There is no correlation between type of porta hepatis and age at surgery for BA; (2) type III porta hepatis is associated with higher rates of bile drainage post-HPE; (3) children older than 16 weeks with BA should still be considered for HPE; (4) in these older patients, factors other than the type of porta hepatis, possibly the degree of liver damage, play a role in the lower rate of bile drainage observed; (5) external diversion of the Roux-en-Y in HPE is not beneficial and is detrimental because of stoma-related complications; (6) an antireflux valve in the Roux-en-Y loop does not reduce the incidence of cholangitis post-HPE; (7) Surgical reexploration does not restore biliary drainage; and (8) sequential therapy with HPE followed by liver transplantation remains the treatment of choice for BA.

Liver biopsy in neonatal cholestasis: a review on statistical grounds.

The aim of this study was to improve the accuracy of the histopathologic diagnosis in the differential diagnosis between obstructive and nonobstructive forms of neonatal cholestasis, using this clinical situation as a model for a mathematical approach. The study was blind, and we performed it in two steps. In the first step, 49 histologic parameters were visually estimated and were scored on a scale of 0 to 4+ in 100 liver biopsy specimens obtained between 1980 and 1985 from 78 patients with neonatal cholestasis. Forty-eight of these 100 specimens were from patients with final diagnosis of obstructive cholestasis (Group I), and 52 were from patients with nonobstructive cholestasis (Group II). The age range was 3 to 24 weeks (median, 12.5 wk). Twelve histologic variables were selected by chi 2 and Fisher's exact test (P < .05). Next, a series of combinations among these variables were submitted to statistical analysis by logistic regression method, defining a six-variable model that had the most powerful predictive value to classify the type of cholestasis. The variables were portal ductal proliferation, bile plugs in portal bile ductules, portoportal bridges, neutrophils, hepatocyte swelling, and multinucleated giant hepatocytes. The score obtained by this model correspond to the probability of a case belonging to Group I. The accuracy, sensitivity, and specificity rates were 94.0%. In the second step, the model was applied to a new sample of 74 needle-liver biopsy specimens obtained between 1990 and 1995, 45 from patients in Group I and 29 from patients in Group II. The age range was 3 to 15 weeks (median, 8 wk). The accuracy, sensitivity, and specificity rates were 90.5%, 100%, and 75.9%, respectively. In our diagnostic routine, this score has been systematically reported and has been helpful in orienting the therapeutic decision in this group of patients.

Resuscitation of the injured child.

Unintentional injury is the leading cause of death for children less than 14 years of age. Optimal injury control includes prevention, acute care, and rehabilitation. When prevention efforts fail, a dedicated well-trained team must be prepared to resuscitate and treat the injured child. This article presents an approach to resuscitation that emphasizes the primary and secondary survey. Early and aggressive airway control with cervical spine protection is stressed, followed by a rapid assessment of ventilatory mechanics and circulatory status. Clinical indicators of inadequate tissue perfusion are described, with priorities concerning intravenous access highlighted. Initial resuscitation steps, complemented by laboratory and radiological assessment, occur before the secondary survey. The secondary survey completes the early resuscitation phase and consists of a systematic and complete physical examination. Resuscitation priorities specific to the multiply-injured child are also discussed. Finally, the importance of rehabilitation and prevention efforts are included.

Esophagocoloplasty in children: surgical technique, with emphasis on the double blood supply to the interposed colon, and results.

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)

Teniposide plus cytarabine as intensification therapy and in continuation therapy for advanced nonlymphoblastic lymphomas of childhood.

PURPOSE AND METHODS: Thirty-nine consecutive children (age, 2 to 11 years) with nonlymphoblastic (NL) lymphomas were treated uniformly with chemotherapy based on the LNH-II-85 protocol. The protocol consisted of a remission-induction phase that lasted 30 days and started with cyclophosphamide (CTX) 1.2 g/m2 on day 1, followed by vincristine (VCR) 1.5 mg/m2 on days 3, 10, 17, and 24, daunomycin (DAUNO) 60 mg/m2 on days 12 and 13, and prednisone 40 mg/m2/d for 30 days. If a complete remission was achieved, an intensification regimen was given that consisted of eight courses of teniposide (VM-26) 165 mg/m2 plus cytarabine (ARA-C) 300 mg/m2 every 4 days according to bone marrow tolerance. A continuation phase was subsequently started, with alternating courses of thioguanine (6-TG) 300 mg/m2/d for 4 days plus CTX 1.2 g/m2 on day 5; hydroxyurea 2.5 g/m2/d for 4 days plus DAUNO 45 mg/m2 on day 5; VCR 1.5 mg/m2 plus methotrexate (MTX) 120 mg/m2 (24 hours apart); mercaptopurine (6-MP) 500 mg/m2/d for 4 days plus MTX 40 mg/m2; and VM-26 plus ARA-C for 3 courses (4 days apart), by the end of 48 weeks. CNS prophylaxis consisted of intrathecal administration of MTX, ARA-C, and dexamethasone according to age, administered three times during remission induction and every 6 weeks afterwards. RESULTS: By the end of the analysis in July 1991, 38 of 39 patients had attained a complete remission and 36 were event-free survivors. Two failures that occurred after completion of therapy were second malignancies (acute lymphocytic leukemia and acute nonlymphocytic leukemia). CONCLUSION: These results are significantly better than those obtained with less intensive former regimens performed in our institution before the availability of VM-26. The favorable impact of an intense consolidation phase with VM-26 is remarkably exemplified by three additional patients not included in this study whose families withdrew them from therapy after the intensification phase, all three of whom have been in remission.

Surgically produced congenital diaphragmatic hernia in fetal rabbits.

Animal models of congenital diaphragmatic hernia (CDH) still are indispensable for the evolution of knowledge related to this disease and to fetal surgery in general. The lamb has provided the most reliable experimental design thus far. Considering the possible advantages of using rabbits (rather than lambs) namely lower costs, no need of special veterinary facilities, smaller body size, year-round availability, higher number of fetuses per pregnancy, and shorter gestational period, a successful model of CDH was developed in fetal rabbits. Sixteen pregnant New Zealand rabbits underwent hysterotomy and fetal operation. Group 1 (6 does) underwent surgery on gestational day 20 and group 2 (10 does) on gestational day 24 or 25. The normal full gestation time is 31 to 33 days. In group 1, the CDH was created by transabdominal puncture and dilatation of the fetal diaphragm. In group 2, the CDH was created through open thoracotomy, either left or right. The fetuses were delivered by cesarean section on gestational day 30. The overall fetal survival rate was 0 for group 1 and 70% for group 2. All operated fetuses in group 2 that were born alive had CDH. The histological morphometric examinations (radial alveolar count after sustained lung expansion) of the normal and operated fetuses in group 2 showed pulmonary hypoplasia in all the lungs on the same side as the CDH (statistical analysis was performed using the Neuman-Keuls test and analysis of variance; the significance level was set at .05).(ABSTRACT TRUNCATED AT 250 WORDS)

Treatment of portal hypertension in children.

The management of children with portal hypertension (PH) has substantially changed owing to the good results and broader application of both endoscopic sclerotherapy and orthotopic liver transplantation (OLT). Since the introduction of sclerotherapy for the treatment of bleeding esophageal varices, the number of surgical procedures has sharply decreased. Until the early 1980s, however, the treatment of choice of bleeding esophageal varices was based on different variations of two main types of open surgery: devacularization and transection operations and portosystemic shunts. The experience with nonshunt procedures is limited in the pediatric population. Literature reports from the last 25 years have emphasized a number of restrictions related to portosystemic shunts in small subjects. However, portosystemic shunts, selective or not, can be performed even in very young subjects with high rates of success. From 1974 to 1984 the distal splenorenal shunt (DSRS) was the procedure of choice for the treatment of children with variceal bleeding in our institution. Forty-two children underwent DSRS during this period. Since 1985, when endoscopic variceal sclerotherapy (EVS) replaced DSRS as the first therapeutic option in our service, this shunt has been performed in only 8 children in whom EVS has failed, none of them during the last 2 years. In this cohort of 50 cases of DSRS, the shunt patency has increased from 71% in the first 7 patients to 95% thereafter. There has been no perioperative mortality. From 1985 to April 1993, 107 children were submitted to EVS sessions for the treatment of esophageal varices bleeding.(ABSTRACT TRUNCATED AT 250 WORDS)

Compressöes do esôfago e da traquéia por anomalias do arco aórtico na infância / Compression of esophagus and trachea by aortic arch anomalies in childhood

De fevereiro de 1985 a novembro de 1992, 14 crianças portadoras de malformaçöes do arco aórtico foram atendias no Serviço de Cirurgia Pediátrica do Instituto da Criança da Faculdade de Medicina da Universidade de Säo Paulo, compreendendo sete pacientes portadores de artéria subclávia direita anômala, quatro portadores de duplo arco aórtico e três portadores de arco aórtico à direita com presença de ligamento arterioso O diagnóstico preciso da malformaçäo foi feito apenas através de esofagograma e traqueobroncoscopia. O tratamento cirúrgico foi realizado através de toracotomia póstero-lateral esquerda em todos os casos, a qual proporcionou uma excelente exposiçäo das anomalias. Näo houve óbito intra-operatório. Ocorreram três óbitos no pós-operatório tardio, decorrentes de lesöes pulmonares crônicas (2) e traqueomalácia grave (1), que se tornou sintomática apenas após a correçäo cirúrgica da anomalia. As principais complicaçöes ocorridas estäo diretamente relacionadas à demora no diagnóstico das malformaçöes. Os autores concluem que a existência dessa anomalias deve ser sempre suspeitada em crianças com sintomas respiratórios de origem indeterminada, sendo o diagnóstico feito de forma simples, basicamente através do esofagograma e da traqueoscopia

[Compression of esophagus and trachea by aortic arch anomalies in childhood]. / Compressões do esôfago e da traquéia por anomalias do arco aórtico na infância.

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.

Long-term stability of lipid emulsions with parenteral nutrition solutions.

The stability of the mixture of peripheral vein parenteral nutrition (PN) solution with 10% lipid emulsions (Intralipid or Lipofundin MCT) was tested during a prolonged period of refrigerated storage. The analysis included gross visual examination of the bottle, pH determination, and examination by electron microscope. The mixtures of fat emulsions with PN solution demonstrated no physical instability or pH alteration. Examination under electron microscope revealed no alterations after 4 wk, but the surface layer of fat globules was disrupted after 10 and 18 wk. This study demonstrates that complete nutritive mixtures can be prepared and stored in refrigeration for at least 4 wk before clinical use.

[Liver transplantation in children: initial experience of the Instituto da Criança of the Hospital das Clínicas of Universidade de São Paulo]. / Transplante de fígado em criança: experiência inicial do Instituto da Criança do Hospital das Clínicas da Universidade de São Paulo.

The initial experience at the Instituto da Criança do Hospital das Clínicas--School of Medicine of São Paulo University with liver transplantation in children is presented. A staff experienced in the management of children, including pediatric surgeons, hepatologists, critical care specialists, anesthesiologists, and other has been joined to draw therapeutic protocols. Afterward, more than 100 experimental liver transplant were performed in animals of medium size (dogs and pigs). From September, 1989 to July 1991, 12 liver transplants were performed on 9 children (3 retransplants) ranging in age from 2.5 to 17 years, being 5 boys and 4 girls. The donors had been selected according to the ABO blood group and body weight. Just once a blood A+ recipient received a liver from a blood O+ donor. The regular postoperative immunosuppression consisted of triple therapy with cyclosporin, prednisone and azathioprine. The postoperative stay in the Intensive Care Unit ranged from 3 to 24 days, according to the necessity of ventilatory support. These was no intraoperative mortality, arterial or venous thromboses, or early biliary complications. The overall survival is 78% (7/9). Primary non-function of the graft was the cause of death in two of our children. Although the number of cases is still small our results are comparable to those of the best liver transplant centers in the world.

Total gastric transposition: an alternative to esophageal replacement in children.

Total gastric transposition was performed in 21 children as an alternative procedure for esophageal replacement. The age at the operation ranged from 15 months to 11 years. Half of the children were less than 2 years old. Fifteen patients had esophageal atresia. The stomach was passed toward the neck either through the esophageal bed (6 cases, with concomitant blunt esophagectomy without thoracotomy) or the retrosternal route (15 cases). There was one death in the early postoperative period secondary to an anastomotic leak and acute mediastinitis in a case of pharyngogastric anastomosis. Three other patients developed cervical leak with spontaneous closure but this ultimately led to a late anastomotic stricture (more than 6 months) requiring endoscopic dilatation. Only one child needed more than three attempts of endoscopic dilatation. None of these patients required surgical revision. The mean follow-up was 60 months (range, 10 to 122 months). Despite bulky atonic intrathoracic stomach occurring in some children, only two patients developed regurgitation and symptoms of poor gastric emptying. There were neither early nor late respiratory problems. Excellent and good functional outcome were achieved in 85% and 15% of the patients, respectively. Two patients have not undergone a weight catch-up phase. The majority of the children have been between the 20th and 80th percentile for weight. Five children remain below the 20th and two below the 5th percentile. The remarkably low morbidity and mortality combined with satisfactory functional results indicate that the total gastric transposition is a safe and easy alternative surgical procedure for esophageal replacement in children.

The endoscopic and surgical management of portal hypertension in children: analysis of 123 cases.

Since 1973, 178 children with portal hypertension (PH) have been seen at Instituto da Criança of the University of São Paulo Medical School. Fifty-five of these children were excluded from this analysis for various reasons, including no treatment required, death before treatment, or incomplete data. From the remaining 123 children with esophageal varices, only 96 (76.1%) of them had at least one episode of upper gastrointestinal hemorrhage. Eighty-eight children were submitted to injection sclerotherapy; 26 treated prophylactically, and 62 for treatment of previous bleeding. Eleven (42.3%) children from the prophylactic group bled from esophageal varices during the treatment. They were all successfully managed thereafter. Satisfactory results were achieved in 53 (85.4%) children in the therapeutic group. Twenty-eight (45.1%) children had at least one episode of bleeding after beginning of sclerotherapy, 19 of whom eventually had successful control of the variceal bleeding. From 1973 to 1984, distal splenorenal shunt (DSS) was the procedure of choice for the treatment of bleeding esophageal varices. Forty-two children have undergone DSS during this period. Only one child was shunted prophylactically. Since 1985, injection sclerotherapy has been the first choice for the treatment and only seven children with sclerotherapy failure have since been treated by DSS. Characteristically these children had very similar splenoportographic pattern with huge esophageal and gastric varices and deviation of portal vein blood flow toward the left gastric vein.(ABSTRACT TRUNCATED AT 250 WORDS)