Living related donor liver transplantation in children.

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.

Hepatic artery graft in pediatric liver transplantation: single-center experience with 58 cases.

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.

Orthotopic liver transplantation in biliary atresia: a single-center experience.

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.

Rex shunt for acute portal vein thrombosis after pediatric liver transplantation in children with biliary atresia.

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.

Multiple clinical presentations of lymphoproliferative disorders in pediatric liver transplant recipients: a single-center experience.

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.

Sirolimus in pediatric liver transplantation: a single-center experience.

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.

"Rex shunt" for the treatment of portal vein thrombosis after pediatric liver transplantation: a case report.

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.

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.

[Extracorporeal membrane oxygenation (ECMO) in a neonate with respiratory distress due to meconium aspiration syndrome: Effect of the administration of exogenous surfactant] / Circulação extracorpórea prolongada (ECMO) em recém-nascido com insuficiência respiratória por Síndrome de Aspiração Meconial: efeitos da administração de surfactante exógeno.

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.

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.

The effects of prenatal intraamniotic surfactant or dexamethasone administration on lung development are comparable to changes induced by tracheal ligation in an animal model of congenital diaphragmatic hernia: studies of lung glycogen content, elastic fiber density, and collagen content.

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.

The effects of prenatal intraamniotic surfactant or dexamethasone administration on lung development are comparable to changes induced by tracheal ligation in an animal model of congenital diaphragmatic hernia.

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.

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)

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.

[Acute abdomen in childhood caused by intestinal angiostrongylus infection: a case report]. / Abdome agudo na infância por angiostrongilíase intestinal: relato de um caso.

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.