ABSTRACT
Although tyrosine is considered indispensable during the neonatal period, its poor solubility has limited its inclusion in parenteral amino acid solutions to less than 1% of total amino acids. Dipeptides of tyrosine are highly soluble, have been shown to be well used and safe in animal models and humans, and, therefore, may be used as an effective means of providing tyrosine in the parenterally fed neonate. The goal of the present study was to determine the tyrosine requirement of the parenterally fed neonate receiving graded intakes of glycyl-L-tyrosine as a source of tyrosine. Thirteen infants receiving adequate energy (340 +/- 38 kJ. kg(-1).d(-1)) and protein (2.4 +/- 0.4 g.kg(-1).d(-1)) were randomized to receive parenteral nutrition with one of five graded levels of glycyl-L-tyrosine. The mean requirement and safe level of intake were estimated using a 1-(13)C-phenylalanine tracer and linear regression cross-over analysis that identified a break point in the response of label appearance in breath CO(2) (F(13)CO(2)) and phenylalanine oxidation to graded tyrosine intake. Based on the mean estimates of whole-body phenylalanine oxidation, the tyrosine mean requirement and safe level of intake were found to be 74 mg.kg(-1). d(-1) and 94 mg.kg(-1).d(-1), respectively. This represents 3.1 and 3.9% of total amino acids, respectively, considerably higher than levels found in present commercially available pediatric amino acid solutions. These data raise concern regarding the adequacy of aromatic amino acid intake in the parenterally fed neonate.
Subject(s)
Dipeptides/administration & dosage , Parenteral Nutrition , Phenylalanine/metabolism , Tyrosine/metabolism , Amino Acids/urine , Humans , Infant, Newborn , Phenylalanine/pharmacokinetics , Phenylalanine/urine , Tyrosine/pharmacokinetics , Tyrosine/urineABSTRACT
BACKGROUND/PURPOSE: Liver tumors that surround the three major hepatic veins traditionally have been considered unresectable. This report describes an extended atypical left hepatectomy technique for tumors around the major hepatic veins. METHODS: Three children with tumors surrounding the 3 hepatic veins underwent intraoperative evaluation for extended atypical left hepatectomy. The left hepatic artery, left branch of the portal vein, and the 3 hepatic veins are occluded with vascular clamps. Perfusion of the remaining liver is through the right hepatic artery and portal vein into the retrohepatic vena cava via the retro hepatic veins. If the liver remains soft and does not become mottled, division of the 3 hepatic veins and resection of the tumor are carried out. RESULTS: Extended atypical left hepatectomy was successful in 2 children. Bile leak occurred in 1 instance and healed spontaneously. Both patients had transiently elevated serum bilirubin and transaminase levels and an elevated prothrombin time for 2 weeks. Both survived after treatment with chemotherapy. In the third child the liver became tense and mottled, and the procedure was abandoned. CONCLUSIONS: Successful extended atypical left hepatectomy depends on the ability of the retro hepatic veins to adequately drain blood into the vena cava after interruption (clamping) of the main hepatic veins. If the liver becomes mottled and tense the procedure must be abandoned and the patient should be considered for hepatic transplantation.
Subject(s)
Hepatectomy/methods , Hepatoblastoma/surgery , Liver Neoplasms/surgery , Child, Preschool , Female , Hepatoblastoma/pathology , Humans , Liver Neoplasms/pathology , Male , Tomography, X-Ray ComputedABSTRACT
BACKGROUND/PURPOSE: For children with probable mediastinal lymphoma and a high risk of cardiorespiratory morbidity, many centers recommend delaying the diagnostic biopsy for 24 to 48 hours while corticosteroids are administered to reduce tumor size and morbidity. This study was undertaken to determine the effect of preoperative steroid use on the accuracy of the pathological diagnosis and incidence of perioperative cardiorespiratory morbidity. METHODS: From 1988 to 1998, 86 children were treated for mediastinal lymphoma. Twenty-three received steroid before biopsy (study group) because of clinical evidence of respiratory compromise, and the remaining 63 served as controls. Clinical parameters, steroid use, and detailed pathological findings obtained at initial and subsequent biopsies were reviewed. Steroid treatment was considered to have had an adverse effect on the pathological diagnosis if (1) a definitive diagnosis was delayed more that 1 month, (2) a definitive diagnosis could not be made, or (3) the extent of disease could not be staged with certainty. RESULTS: Steroid treatment had an adverse effect on the pathological diagnosis in 5 of 23 (22%) children: 1 diagnostic delay, 3 failures of a definitive diagnosis, and 1 possible failure of staging. A definitive diagnosis was made in all control patients. Perioperative survival was 100% in both groups. At biopsy, only 3 children in the steroid treatment group and 2 children in the control group had moderate, nonfatal cardiorespiratory instability. Parameters of steroid use among children who had inaccurate pathological diagnoses or cardiorespiratory morbidity were not significantly different from those who did not. CONCLUSIONS: Steroid treatment before biopsy of mediastinal lymphoma may adversely affect the pathological accuracy or cause a delay in definitive diagnosis in a minority of cases. The dose and duration of steroid use was not related to outcome. Prebiopsy steroid appears to minimize the likelihood of cardiorespiratory morbidity in high-risk patients.
Subject(s)
Biopsy , Glucocorticoids/therapeutic use , Lymphoma/diagnosis , Mediastinal Neoplasms/diagnosis , Adolescent , Child , Child, Preschool , Diagnostic Errors , Female , Humans , Lymphoma/drug therapy , Male , Mediastinal Neoplasms/drug therapyABSTRACT
Pneumatic reduction of idiopathic intussusception is successful in about 80% of cases, while 60% of the failures are reduced at surgery without resection. To determine whether delayed, repeated attempts at enema reduction of failures would reduce the need for operation in selected cases, over a 2-year period (1994-1996 inclusive), 17 infants with idiopathic intussusception underwent delayed repeat enemas 2-19 h following the first failed attempt at reduction. Clinical parameters and radiologic findings were evaluated with respect to outcome. Ten intussusceptions were successfully reduced after the second attempt in 9 and after the fourth attempt in 1. Seven children underwent a laparotomy, 5 because of failure of progressive reduction at air enema (AE). Two were taken to surgery early in the series, 1 because of perforation during a second attempt and 1 while awaiting a third reduction attempt. The 10 successful reductions all showed progressive movement of the intussusceptum on each AE; the 2 who perforated failed to show progressive reduction on their second AE. Because of these cases, the remaining 5 were referred to surgery because of failure of progressive reduction of the intussusceptum on the second attempt. At laparotomy, of the 7 unsuccessful reductions, 4 required resection and 3 had difficult manual reduction. The presence of vomiting, a mass, and/or bloody stools were not predictors of outcome. Failures had higher body temperatures (38.1 +/- 0.3 vs 37.4 +/- 0.1 degrees C, P = 0.07), heart rates (153.7 +/- 8 vs 136.9 +/- 2.1 min, P = 0.03), and longer duration of symptoms (36.8 +/- 4 vs 21.3 +/- 3.6 h; P = 0.01) than successes. Delayed repeat AEs may be safe and effective in selected cases of idiopathic intussusception, but should be considered only if significant movement of the intussusceptum is noted at each attempt. The ideal time for repeat AE reduction prior to surgery is not established, but 2-4 h appears appropriate. Pyrexia, tachycardia, and duration of symptoms greater than 36 h are relative contraindications to this course of management.
Subject(s)
Enema , Intussusception/therapy , Air , Humans , Infant , Insufflation , Retreatment , Treatment FailureABSTRACT
PURPOSE: Nonoperative management of blunt hepatic injury (BHI) has become widely accepted in hemodynamically stable children without ongoing transfusion requirements. However, late hemorrhage, especially after discharge from the hospital can be devastating. The authors report the occurrence of serious late hemorrhage and the sentinel signs and symptoms in children at risk for this complication. METHODS: Nonoperative management of hemodynamically stable children included computed tomography (CT) evaluation on admission and hospitalization with bed rest for 7 days, regardless of injury grade. Activity was restricted for 3 months after discharge. Hepatic injuries were classified according to grade, amount of hemoperitoneum, and periportal hypoattenuation. RESULTS: Over 5 years, nonoperative management was successful in 74 of 75 children. One child returned to the hospital 3 days after discharge with recurrent hemorrhage necessitating surgical control. Review of the CT findings demonstrated that he was the only child with severe liver injury in all four classifications. A second child, initially treated at an outside hospital, presented 10 days after injury with ongoing bleeding and died despite surgical intervention. Only the two children with delayed bleeding had persistent right abdominal and shoulder discomfort in the week after BHI. CONCLUSIONS: Our findings support nonoperative management of BHI. However, late hemorrhage heralded by persistence of right abdominal and shoulder pain may occur in children with severe hepatic trauma and high injury severity scores in multiple classifications.
Subject(s)
Hemorrhage/etiology , Liver Diseases/etiology , Liver/injuries , Wounds, Nonpenetrating/therapy , Adolescent , Humans , Liver/diagnostic imaging , Male , Time Factors , Tomography, X-Ray Computed , Wounds, Nonpenetrating/diagnostic imagingABSTRACT
Tyrosine is considered to be an indispensable dietary amino acid in the neonate, yet achieving adequate parenteral tyrosine intake is difficult due to its poor solubility. Increasing the supply of phenylalanine is the most common means of compensating for low tyrosine levels. Unfortunately, plasma phenylalanine concentrations are sometimes elevated in infants receiving high phenylalanine intake. This led us to study the phenylalanine and tyrosine metabolism in 16 neonates randomized to receive total parenteral nutrition with either a high or a moderate phenylalanine-containing amino acid solution. A primed, 24-h continuous stable isotope infusion of L-[1-13C]phenylalanine and L-[3,3-2H2]tyrosine was given to enable the measurement of phenylalanine and tyrosine kinetics. Results demonstrated that 1) phenylalanine hydroxylation was significantly greater in infants receiving high phenylalanine, 2) phenylalanine oxidation and percent dose oxidized was also significantly greater in infants receiving high phenylalanine, 3) apparent phenylalanine retention was greater in neonates receiving high phenylalanine, and 4) alternate catabolites of phenylalanine and tyrosine metabolism were significantly greater in infants receiving high phenylalanine compared with moderate phenylalanine. We conclude that neonates respond to increased parenteral phenylalanine intake by increasing their hydroxylation and oxidation rates. The greater oxidation of phenylalanine in infants receiving high phenylalanine in conjunction with the urinary excretion of alternate catabolites of phenylalanine and tyrosine suggests that the high phenylalanine intake may be in excess of needs. However, the lower apparent phenylalanine retention observed in infants receiving moderate phenylalanine suggests that the total aromatic amino acid level of moderate phenylalanine may be deficient for neonatal needs.
Subject(s)
Amino Acids/administration & dosage , Parenteral Nutrition, Total , Phenylalanine/administration & dosage , Phenylalanine/metabolism , Tyrosine/administration & dosage , Tyrosine/metabolism , Carbon Isotopes , Deuterium , Female , Humans , Hydroxylation , Infant Food/analysis , Infant Nutritional Physiological Phenomena , Infant, Newborn , Kinetics , Male , Nutritional Requirements , Oxidation-ReductionABSTRACT
BACKGROUND/PURPOSE: Enteric duplications can occur throughout the entire alimentary tract. When they occur in the pancreatic head, they present a formidable challenge in both diagnosis and treatment. Surgical management has ranged from simple drainage to local excision or radical resection (eg, Whipple procedure). The authors propose that with identification of the local anatomic relationships, definitive management can be achieved by complete local resection of the cyst mucosa. METHODS: The authors have treated four patients, ages 13 months to 4 years for enteric duplication cysts within the pancreatic head. RESULTS: Clinical presentations were quite varied, including pancreatitis (n = 2), gastritis (secondary to increased production of gastrin), and recurrent pleural effusion with high amylase content. Two of the four cysts had been drained initially using Roux-en-Y cystenterostomies. Our preoperative imaging studies included abdominal ultrasonography, endoscopic retrograde cholangiopancreatography (ERCP), angiography, computerized tomography (CT) or magnetic resonance imaging (MRI) scanning. When necessary intraoperatively, ultrasonography was used for cyst localization and transduodenal pancreatography to define precise ductal relationships. All four duplication cysts were completely excised, including two that communicated directly with the main pancreatic duct. One of the latter extended into the pleural cavity and required a thoracotomy for complete excision. Pathological exam of the excised cysts demonstrated gastric, duodenal, or respiratory mucosa. All four patients have remained entirely asymptomatic during a follow-up of 2 to 7 years postoperatively. CONCLUSION: The authors conclude that complete local resection of enteric duplication cysts in the pancreatic head can be performed for definitive management, avoiding the complications of more radical procedures.
Subject(s)
Pancreas/abnormalities , Pancreatic Pseudocyst/surgery , Anastomosis, Roux-en-Y , Child, Preschool , Female , Gastritis/etiology , Humans , Infant , Male , Pancreatic Pseudocyst/congenital , Pancreatic Pseudocyst/diagnosis , Pancreatitis/etiology , Pleural Effusion/etiologyABSTRACT
BACKGROUND/PURPOSE: Enterocolitis (EC) represents a serious complication after the surgical correction of Hirschsprung's disease (HD). Although previous studies have identified risk factors associated with the development of this complication before definitive repair, the factors leading to EC after pull-through have not been examined. This study was therefore designed to determine risk factors for the development of post-pull-through EC. METHODS: Patients with HD treated from 1991 through 1996 at the Hospital for Sick Children in Toronto, Canada were assessed. Risk factors were examined in three areas: patient factors (gender, age at diagnosis, age and weight at pull-through), technical factors (type of repair, number of stages, location of transition zone, previous EC), and mechanical factors. RESULTS: In 105 consecutive patients, the incidence of postoperative EC was 32%. There was no mortality. The risk of postoperative EC was significantly increased by mechanical factors related to anastomotic complications (relative risk, 2.8) and intestinal obstruction (relative risk, 3.5). This finding was not attributable to the general occurrence of any postoperative complication because the incidence of postoperative complications was equally distributed in patients with and without EC. The presence of EC significantly increased the number of hospital admissions, mean length of stay, and total treatment cost. CONCLUSION: These findings suggest the use of measures to decrease mechanical obstruction so as to decrease the incidence and impact of this potentially devastating complication.
Subject(s)
Enterocolitis/etiology , Hirschsprung Disease/surgery , Postoperative Complications , Female , Humans , Infant , Male , Risk FactorsABSTRACT
PURPOSE: The authors report a 5-year experience of inserting the Palmaz stent into infants and children who had a variety of major airway obstructions. METHODS: From 1992 to 1997, 30 balloon expandable stents (Palmaz) were inserted in the trachea (n = 18) and bronchi (n = 12) of 16 infants, ages 1 week to 26 months (median, 9 months), suffering from three types of serious airway obstruction. In group 1, 10 stents were placed in eight children for tracheal or bronchomalacia. In group II, 11 stents were inserted in four infants for stricture at the site of surgical repair of stenosis. In group III, nine stents were placed to relieve airway compression from enlarged pulmonary arteries associated with severe congenital heart disease in three children and mediastinal lymphangioma in one. Tracheal stents were 30 mm long and were expanded to 8 to 10 mm at placement. Bronchial stents were 12 to 15 mm long and were expanded to 7 to 9 mm. The nonexpanded stents were placed on an inflatable balloon catheter and were inserted into the desired position in the airway through a bronchoscope or endotracheal tube using x-ray control. They were expanded and fixed in place by inflating the balloon to its rated diameter. RESULTS: In group I, granulation tissue developed over the stents in five of eight cases. Obstructing granulations were removed by scraping or balloon compression in three and resulted in earlier than the planned removal in two. Stents have now been removed in six of eight cases. Major airway obstruction has not recurred. In group II, stents have been in place in all cases for 13 to 56 months after insertion, but in one child with three stents, two were removed for obstructive granulations 44 months after insertion. All are well. All group III patients could be extubated after stenting, but two with heart disease died after 3 and 12 months of palliation. During the course of follow-up, stents in the bronchi of two had migrated, and an additional stent was required. Autopsy in one showed full-thickness bronchial erosion but no perforation by the stent. A total 11 of 30 stents have been removed bronchoscopically in seven children without complications. Another child referred here for tracheal stent removal after laser resection of granulations died at attempted removal because the stent was "welded" into the tracheal wall by the inflammatory reaction. Manipulation of the stent completely occluded the airway. CONCLUSIONS: Airway stents can be inserted easily and safely and left in-situ for prolonged periods to relieve major airway obstruction from a variety of causes. Tissue reaction may necessitate bronchoscopic manipulation and early stent removal, and adds to the difficulty of removal.
Subject(s)
Bronchial Diseases/therapy , Stents , Tracheal Diseases/therapy , Tracheal Stenosis/therapy , Constriction, Pathologic/therapy , Equipment Design , Humans , Infant , Infant, Newborn , Stents/adverse effects , Time Factors , Treatment OutcomeABSTRACT
PURPOSE: The safety and efficacy of nonoperative management of pancreatic contusions and transections was examined by reviewing the case histories of 35 consecutive children with pancreatic injuries treated over the past 10 years. METHODS/RESULTS: Surgical exploration was performed for the management of associated injuries only. The diagnosis of pancreatic trauma was suspected in children with abdominal pain, tenderness, elevated serum amylase levels and findings consistent with pancreatic injury on abdominal ultrasound scan or computerized tomography (CT) examination. After children were diagnosed with pancreatic injury, enteral feedings were withheld and total parenteral nutrition administered until abdominal pain resolved and serum amylase levels and radiographic findings improved. Twenty-three children received diagnosis within 24 hours of injury, and in 12, the diagnosis was delayed 2 to 14 days. Hyperamylasemia was found in 27 of 35 children. Twenty-eight children sustaining pancreatic injuries were treated nonoperatively. Abdominal imaging in these children demonstrated pancreatic contusion in 14, transection in 11, and pseudocyst in three. Enteral feeding resumed an average of 15 days after injury. The average hospital stay was 21 days. Pseudocysts formed in 10 children (2 of 14 with contusion; 5 of 11 with transection; three children presented late, and the type of pancreatic injury could not be determined), whose average hospital stay was 25 days. All pseudocysts were successfully managed nonoperatively, although percutaneous aspiration or drainage was required in six children. Children underwent follow-up for an average of 10 months after injury (range, 1 to 144 months). Abdominal pain and radiological abnormalities resolved in all children before discharge from the clinic. CONCLUSIONS: Nonoperative management of pancreatic contusion and transection diagnosed radiologically is effective and safe. Pseudocysts may form after pancreatic injury, and if large or symptomatic, can be managed successfully by percutaneous drainage.
Subject(s)
Abdominal Injuries/complications , Pancreas/injuries , Wounds, Nonpenetrating/therapy , Child , Diagnostic Imaging , Humans , Pancreatic Pseudocyst/epidemiology , Pancreatic Pseudocyst/etiology , Pancreatic Pseudocyst/therapy , Parenteral Nutrition, Total , Retrospective Studies , Time Factors , Wounds, Nonpenetrating/diagnosis , Wounds, Nonpenetrating/epidemiologyABSTRACT
This series describes the occurrence of unbalanced circulatory shunting in three consecutively treated pairs of conjoined twins. The impact of renal functional changes and compensatory cardiac physiology on the timing of surgery and perioperative management are illustrated. The craniopagus set of twins presented in renal failure (1A) and cardiac failure (1B) to our hospital at age 2.5 years. Renal transplantation for anuria was performed in 1A before separation of the twins. This did not improve urine output in the hypoperfused twin, and normal renal function was not restored until the twins were separated. Two sets of omphalopagus twins were delivered and transferred to our hospital and separated during the first week of life. In each case a preoperative oliguric state in the underperfused twin reversed after separation, and a postoliguric diuresis ensued. Serum creatinine levels were similar in each pair preoperatively despite the inequality in urine volumes. In conjoined twins, assessment of renal function is necessary and should include serial measurements of urine volumes and urine creatinine clearance. Serum creatinine is not a useful marker of individual twin renal function in this situation. When disparity in renal function exists, early separation is indicated to prevent renal failure and compensatory cardiac failure.
Subject(s)
Fetofetal Transfusion/physiopathology , Kidney/physiopathology , Renal Insufficiency/physiopathology , Twins, Conjoined/surgery , Child, Preschool , Creatinine/blood , Female , Humans , Infant, Newborn , Male , Pregnancy , Twins, Conjoined/physiopathologyABSTRACT
Plasma lipoprotein composition in infants receiving fat-free parenteral nutrition reflects the endogenous synthesis and metabolism of lipids. We studied the composition of plasma lipoproteins in 49 appropriate for gestational age newborn infants after surgery who received only glucose and amino acid solutions for 5.4 +/- 0.3 days (M +/- SE). Of the infants studied, 31 were fullterm (gestational age 39.5 +/- 0.2 weeks) and 18 premature (34.3 +/- 0.7 weeks). Plasma lipid levels (total lipids, triglycerides, free cholesterol, sterol, esters, phospholipids) did not differ between term and premature infants, but triglycerides and cholesterol were markedly lower than in young, fasting adults. The contribution of triglycerides to lipoprotein lipids was strikingly low in chylomicrons (21% vs. 90% in young fasting adults) and VLDL (34 vs 60%) and the infants had a consistently lower cholesterol content of HDL (21 mg/dl vs. 45-50 mg/dl in adults) and LDL (43 mg/dl vs. 100 mg/dl). All infantile lipoproteins were enriched with phospholipids. These results are comparable to those reported for cord plasma. In premature babies, VLDL were markedly reduced and contained less triglycerides, free and esterified cholesterol than in term infants. In contrast, HDL were increased in preterm infants and carried more phospholipids. VLDL contributed to al lesser and HDL to a greater extent to plasma lipid transport in premature infants. We conclude that in premature infants hepatic synthesis of triglycerides and cholesterol and their secretion as VLDL is reduced, which may be caused by low substrate availability or an immaturity of the synthetic pathway. In premature infants, HDL appears to play a major role in transporting plasma lipids to peripheral tissues.
Subject(s)
Infant, Premature/blood , Lipoproteins/blood , Parenteral Nutrition , Cholesterol/blood , Chylomicrons/blood , Humans , Infant, Newborn , Triglycerides/bloodABSTRACT
BACKGROUND: Papillary cystic tumour of the pancreas in children is a rare tumour. METHODS: Ten patients admitted to The Hospital for Sick Children, Toronto, have been reviewed, and presentation and management are documented. RESULTS: One patient who had disseminated disease at presentation died. CONCLUSION: Excision of the tumour irrespective of size is recommended.
Subject(s)
Cystadenoma, Papillary/epidemiology , Pancreatic Neoplasms/epidemiology , Adolescent , Child , Child, Preschool , Cystadenoma, Papillary/diagnosis , Cystadenoma, Papillary/surgery , Female , Humans , Infant , Male , Pancreas/pathology , Pancreatectomy , Pancreatic Neoplasms/diagnosis , Pancreatic Neoplasms/surgery , Pancreaticoduodenectomy , Retrospective Studies , Treatment OutcomeABSTRACT
Persistent hyperinsulinemic hypoglycemia of the neonate (PHHN) usually presents in the neonate or infant. Although rare, the possibility of PHHN should be considered in all neonates who have unremitting hypoglycemia. Untreated hypoglycemia results in severe neurological disability or death. An inappropriately elevated serum insulin level and low serum ketone and free-fatty-acid concentrations in the presence of hypoglycemia confirm the diagnosis. Early diagnosis and aggressive medical management have reduced the morbidity associated with PHHN. Pancreatic resection is necessary for long-term control of hypoglycemia when medical therapy fails. Ninety-five percent pancreatectomy has been the procedure of choice at the author's institution and other pancreatic centers. However, a review of the authors' experience and of in the English-language literature has demonstrated a 33% failure rate, requiring further surgery or medical management. Furthermore, long-term follow-up showed that diabetes develops in most children who undergo 95% pancreatectomy. The high failure rate of 95% pancreatectomy and the ultimate development of diabetes in virtually all children suggest the alternative approaches should be considered.
Subject(s)
Hyperinsulinism/congenital , Hypoglycemia/congenital , Combined Modality Therapy , Humans , Hyperinsulinism/diagnosis , Hyperinsulinism/therapy , Hypoglycemia/diagnosis , Hypoglycemia/therapy , Infant , Infant, Newborn , Pancreas/pathology , Pancreatectomy , Treatment OutcomeABSTRACT
In 1986, The Hospital for Sick Children (Toronto, Canada) began to use a standard preoperative chemotherapeutic regimen for patients who had unresectable hepatoblastoma. In 1988, we extended this protocol to all children who had hepatoblastoma. Of 25 children who presented with hepatoblastoma, 22 were eligible for protocol therapy. After percutaneous biopsy, cycles of cisplatin (20 mg/m2/d for 5 days) and Adriamycin (25 mg/m2/d for 3 days) were administered every 3 weeks by continuous intravenous infusion. A CAT scan was performed after the third cycle. Surgery was undertaken if response indicated that complete resection was possible. If not, a further one to three cycles were given until response was adequate. Postoperatively, therapy was continued for a total of six cycles. Twenty of twenty-two (91%) tumors responded to chemotherapy. Over half required only three cycles. Twenty hepatic resections (6 segmentectomies, 10 lobectomies, 4 trisegmentectomies) were performed. Preoperative therapy significantly reduced the extent of resection calculated to be necessary for complete excision at an initial diagnosis of the primary tumor in all but one. In the two children with inadequate response, total hepatectomy and transplant was necessary for complete resection. No deaths or operative delays were attributed to chemotherapy toxicity. Nineteen of 22 children (87%) are alive with no evidence of disease, including both transplant patients. One death was caused by intraoperative bleeding and the other two were caused by metastatic lung disease at 22 and 26 months, respectively. Twelve children, eight with tumors that would have been unresectable before effective chemotherapy, have had follow-up for more than 5 years. This protocol of preoperative chemotherapy appears to be safe and effective for most children who have hepatoblastoma.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Hepatoblastoma/drug therapy , Liver Neoplasms/drug therapy , Chemotherapy, Adjuvant , Child , Child, Preschool , Cisplatin/administration & dosage , Doxorubicin/administration & dosage , Female , Fluorouracil/administration & dosage , Hepatoblastoma/pathology , Hepatoblastoma/surgery , Humans , Infant , Liver Neoplasms/pathology , Liver Neoplasms/surgery , Male , Survival Rate , Vincristine/administration & dosageABSTRACT
OBJECTIVE: To determine the effect of the Palmaz stent, which has been successfully used to relieve airway obstruction in a small group of children, on the normal and operated on animal trachea. DESIGN: In this experimental trial, stents were placed bronchoscopically in the thoracic tracheae of 4 groups of 50 anesthetized cats. The cats in group 1 (adults, n = 10) and group 2 (kittens, n = 10) had normal tracheae; the cats in group 3 (adults, n = 15) underwent horizontal tracheal incision and closure; and the cats in group 4 (adults, n = 15) underwent pericardial patch tracheoplasty. In group 3 and 4, the stents were inserted in 10 cats and 5 cats served as controls. Stents were inflated to a 15-mm diameter in group 1 and an 8-mm diameter in the other groups. Half of the animals with stents were killed 5 weeks after the procedure, and the others, 10 weeks after the procedure. SETTING: The Hospital for Sick Children, Toronto, Ontario. RESULTS: In group 1, 1 animal died of tracheal perforation. A mild cough was noted in 15 of the 39 cats with stents. In group 4, 3 cats had difficulty eating and lost weight. The results of esophagoscopy excluded esophageal inflammation or obstruction in these 3 animals. The results of bronchoscopy indicated a nonobstructing rim of granulation tissue at the end of the stent in 15 of 20 cats in groups 1 and 2 and 17 of 20 cats in groups 3 and 4 and at the repair site in all animals with stents from groups 3 and 4. The results of autopsy indicated the cross-sectional area at the site of the stent was greater than normal in group 1 (P < .003) and smaller than the normal trachea at the site of the tracheoplasty in group 4 controls without stents (P < .02); however, the cross-sectional area at the tracheoplasty site with the stent was not smaller (P < .13). The results of histologic examination indicated a mild inflammatory reaction, with granulation tissue in all animals with stents, but in group 1, with overexpanded stents, the reaction was more severe, with epithelial ulceration, fibrosis, and sealed-off perforations in most animals. In group 3, the tracheae with stents had significantly more inflammatory reaction, granulation tissue, and epithelial damage than the controls without stents. CONCLUSIONS: The Palmaz stent provokes an inflammatory reaction in the normal trachea and the trachea recently operated on. With the exception of the group 1 animals with overexpanded stents, this reaction is clinically insignificant. The Palmaz stent is able to maintain a normal lumen size after pericardial tracheoplasty in cats.
Subject(s)
Angioplasty, Balloon/instrumentation , Stents/adverse effects , Trachea/pathology , Animals , Bronchoscopes , Cats , Female , Granulation Tissue/pathology , Male , Radiography , Stents/statistics & numerical data , Trachea/diagnostic imaging , Trachea/surgery , Tracheitis/etiology , Tracheitis/pathologyABSTRACT
OBJECTIVE: Aortopexy is the more extended treatment for severe tracheomalacia, when it fails, reintervention and other procedures are necessary. We present our experience in the treatment of this pathology. MATERIAL AND METHODS: Tracheomalacia, bronchomalacia and tracheobroncomalacia cases during a twelve year period (1983-1995) were reviewed (type, age, symptoms, surgical procedures and results were collected). RESULTS: Nineteen patients (9 males, 10 females) presented tracheobronchomalacia in our Hospital. Diagnosis was made by bronchoscopy in all of them: nine cases of tracheomalacia, five bronchomalacias and five suprastomal malacias were found. Mean diagnosis age was twenty four months (newborn-twelve years). Ten patients were managed nonoperatively, five with anterior cricoideal suspension and two cases were treated by aortopexy; one of them, after 2 months of respiratory stridor and recurrent respiratory arrest, needed an endoscopically placed intraluminal expandable Palmaz Prothesis. This prothesis was removed two years later. In this moment, the patient is doing well without stridor or respiratory symptoms.
Subject(s)
Bronchial Diseases/surgery , Tracheal Diseases/surgery , Catheterization , Child , Child, Preschool , Endoscopy , Esophagus/diagnostic imaging , Female , Humans , Infant , Infant, Newborn , Male , Prostheses and Implants , Radiography , Tracheal Diseases/diagnostic imagingSubject(s)
Cellulose, Oxidized , Hepatoblastoma/diagnostic imaging , Liver Neoplasms/diagnostic imaging , Neoplasm Recurrence, Local/diagnostic imaging , Diagnosis, Differential , Hemostasis, Surgical , Hepatoblastoma/surgery , Humans , Infant , Liver Neoplasms/surgery , Male , Radiography , UltrasonographyABSTRACT
A 95% pancreatectomy became the treatment of choice for persistent hyperinsulinemic hypoglycemia of the neonate (PHHN, Nesidioblastosis) at the author's institution, when lesser resections failed to prevent hypoglycemia in 25% to 50% of cases. With few outcome data available in the literature, the authors reviewed their 25-year experience to assess the efficacy and the long-term consequences of this procedure. Since 1971, 27 infants underwent a 95% pancreatectomy for the treatment of PHHN. None had responded to medical treatment (glucose infusion, glucagon, octreotide, diazoxide), and two had 85% pancreatectomy that failed. The procedure consisted of resecting the pancreas including the uncinate process, leaving only the gland lying between the common bile duct (CBD) and the duodenum and a small rim of pancreas along the duodenal sweep. Hyperinsulinemia and hypoglycemia recurred in nine children (33%), all within 2 to 5 days. Seven of them were subsequently cured with near-total pancreatic resection. Partial pancreatic regrowth was evident at reoperation. In two cases hypoglycemia was controlled with diazoxide and frequent feedings because reoperation was refused. The gross anatomic findings and the histopathology were not predictive of treatment failure. Perioperative complications occurred in four of 27 children (15%) after 95% pancreatectomy and in four of seven children (57%) after near-total pancreatectomy. Clinical follow-up ranged from 0.5 to 18 years (mean, 8 years; median, 8 years). To date, diabetes has developed in 15 children (56%), nine of 20 (45%) after 95% pancreatectomy (mean age, 9.7 years) and six of seven (86%) after a near-total pancreatectomy (mean age, 1.7 years). After 95% pancreatectomy, the incidence of diabetes increased with age, developing in nine of the 13 (69%) children followed up for more than 4 years. The failure of 95% pancreatectomy to prevent hypoglycemia in one third of children with PHHN and the ultimate development of diabetes in a minimum of two-thirds, indicates that an alternative treatment strategy is needed for this disease.