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.

Hepatoportal sclerosis in childhood.

The clinical, angiographic, and pathologic features of hepatoportal sclerosis in five children are presented. Hepatosplenomegaly with preservation of liver function and esophageal varices were demonstrated. Histologically, portal alterations were consistent and prominent and included subintimal sclerosis of the intrahepatic venous branches and telangiectases. Angiographic patterns were variable, but all exhibited intrahepatic portal venous obstruction. In three patients, there was an association with extrahepatic portal vein obstruction at different sites. Our data suggest that intrahepatic portal vein thrombosis could be the primary disorder in hepatoportal sclerosis, which may vary in extent and site, involving the intrahepatic branches of the portal vein with or without the involvement of the portal trunk. Combined clinical, angiographic, and pathologic data can contribute to a better understanding of hepatoportal sclerosis in children.

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.

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.

Effect of branched-chain amino acids and insulin on postinjury protein catabolism in growing animals.

Muscle proteolysis continues to occur in hypercatabolic states despite the administration of carbohydrates and proteins. Recent clinical and experimental studies have demonstrated that, under catabolic conditions, treatment with either branched-chain amino acids (BCAA) or insulin may decrease negative nitrogen balance. However, the use of BCAA-enriched solutions to inhibit muscle proteolysis has never been tested in growing animals. A study was therefore undertaken to assess the effectiveness of such solutions, with or without insulin, as compared to a more balanced amino acid solution, in preventing or diminishing postinjury protein catabolism in growing animals. Fifteen-day-old rabbits, exposed to standard moderate trauma in the form of crushing the muscle mass of one rear thigh, received one of two amino acid formulations--a balanced amino acid solution (18.8% BCAA) or a 35% BCAA-enriched solution--for 96 hr. Insulin was given to subgroups of both series. The results indicate that: (1) nitrogen balance in nontraumatized animals is clearly superior when balanced amino acids are administered; (2) BCAA-enriched solutions may decrease postinjury muscle protein catabolism; (3) after trauma, insulin also has a nitrogen-conserving effect, which is demonstrated when it is combined both with BCAA-enriched (35%) and balanced amino acid (18.8%) solutions. However, a better nitrogen balance is achieved when insulin is associated with the balanced amino acid solution.

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.

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.

Distal splenorenal shunt in children.

The distal splenorenal end-to-side anastomosis (Warren shunt) decompresses esophageal varices while maintaining high portal hypertension and avoiding reduction of portal venous blood inflow to the liver. The Warren shunt was performed in seven consecutive patients with portal hypertension, including post-necrotic cirrhosis, portal thrombosis, and schistosomiasis, all with recurrent esophageal bleeding. Five shunts remained patent and two thrombosed. There was no mortality. If long-term follow-up evaluations indicate its effectiveness in preventing esophageal hemorrhage, the distal selective splenorenal shunt would be the more physiologic and safer procedure in children with portal hypertension.

Effect of insulin on protein catabolism after injury in young animals.

Injury is followed by a negative nitrogen balance, muscle protein breakdown and loss of body mass. The intensity of these changes correlates with trauma severity. Reversion of the catabolic state has long been attempted. Recently, it has been shown that the use of insulin might inhibit protein catabolism after severe trauma. The present study was undertaken to assess the effect of insulin on post-injury catabolism in young rabbits submitted to parenteral nutrition. Concomitantly, changes in liver and skeletal muscle nitrogen contents were also studied to elucidate the mechanism of insulin's anabolic effect after trauma. We found that only in catabolic states did insulin produce a significant nitrogen-sparing effect, probably due to the capacity of the hormone to inhibit muscle proteolysis.

Evolution with age of the acetylcholinesterase activity in rectal suction biopsy in Hirschsprung's disease.

The acetylcholinesterase (AChE) activity in Hirschsprung's disease was analyzed for histochemical patterns according to age. Its evolutional nature was also assessed. Two hundred thirty children (0 to 16 years of age) with acute or chronic obstipation were submitted to rectal suction biopsy for AChE histochemical staining. One hundred nineteen of them showed abnormal AChE activity. Retrospective analysis of those cases permitted us to establish three histochemical patterns: Pattern I, thick nerve trunks present only in the submucosa and, characteristically, absence of AChE activity in the lamina propria. This pattern was predominant in newborn up to 3 months of age and was designated the newborn pattern. Pattern II, thin nerve fibers in the muscularis mucosae and submucosa with a clear infiltration in the lamina propria. This pattern was mainly seen in children older than 1 year of age and was called classical pattern. Pattern III, an intermediate pattern showing morphologic characteristics of the two previous patterns with predominance of one or another, according to the age. A prospective study was also made in 15 children with Hirschsprung's disease who were submitted to suction rectal biopsies at different ages. In this study, a clear evolutional character of the AChE activity was observed from the newborn pattern to the intermediate and finally to the classical pattern.

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)

Must fat emulsions and parenteral nutrition solutions be given through separate circuits?

To test the stability of fat emulsions with parenteral nutrition (PN) solutions, 10% soybean fat emulsion (FE) was suspended in PN solutions containing amino acids, electrolytes, vitamins, and different glucose concentrations. Six different mixtures were prepared and analyzed after 15 minutes or 24 hours, under transmission electron microscope: 10% fat emulsion (FE); FE + PN solution with 5% glucose (15 minutes); FE + PN solution with 5% glucose (24 hours); FE + PN with 12.5% glucose (24 hours); FE + PN with 25% glucose (24 hours); and FE + pure glucose 50% (24 hours). The addition of FE to PN solutions containing 5% to 25% glucose did not cause coalescence, aggregation, or confluence of the liposomes or damage to their surface in the first 24 hours after admixture. However, the suspension of fat emulsion in a 50% glucose solution coalesced liposomes and the droplets were nearly all aggregated. We conclude that mixtures of FE with PN solutions with glucose are stable for at least 24 hours and are suitable for clinical use.

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 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)

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.

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.

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)

[Gastroesophageal reflux in children: analysis of 34 consecutive cases]. / Refluxo gastroesofagiano na crianca: análise de 34 casos consecutivos.

We studied 34 consecutive children from 6 m to 10 yr presenting gastroesophageal reflux (GER) with or without hiatal hernia. There was a high incidence of stenosis: 28 (80%) children presented with stenosis of the esophagus from mild to severe degree and 20 of them (70%) developed shortened or Barrett's esophagus. This data suggest that in our serie the diagnosis of GER has been delayed or medical therapy has been inadequate or excessively prolonged. The surgical treatment of GER with or without stenosis is the Nissen fundoplication. Our first cases of shortened esophagus were treated by intrathoracic Nissen fundoplication, which was considered an extensive and major risk surgery. Lately, for these cases we have been utilizing the gastro-fundoplication without opening of the chest. This is an easier surgery, through which we could reach good results without serious postoperative complications.

[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.