Graft function 5-7 years after renal transplantation in early childhood.

BACKGROUND: Low recipient age is still a risk factor for graft failure after kidney transplantation (Tx). Detailed prospective reports on long-term graft function in small children after renal Tx are still lacking. METHODS: Forty-nine kidney allograft recipients who received transplants before the age of 5 years were followed prospectively. The most common disease was congenital nephrotic syndrome of the Finnish type. Twenty patients were recipients of living related donors (LRD), and 29 were cadaveric kidney (CAD) recipients. All patients received triple immunosuppression. Glomerular filtration rate (GFR), effective renal plasma flow (ERPF), sodium, urate, and potassium handling, and concentrating capacity were studied for up to 7 years after Tx. RESULTS: Patient survival 7 years after Tx was 100% for LRD and 96% for CAD recipients. Graft survival was 94% for LRD and 79% for CAD recipients (P=NS) and 89% and 83% for children >2 years and <2 years of age at Tx, respectively (P=NS). Five years after Tx, GFR was 70 vs. 64 and ERPF was 380 vs. 310 ml/min/1.73 m2 for LRD and CAD recipients, respectively (P=NS). Mean absolute GFR remained stable. GFR was lower in children who received transplants at <2 years than in children who received transplants at >2 years of age, 54 vs. 75 ml/min/1.73 m2 (P=0.02). Sodium handling remained intact, but hyperuricemia was seen in 43-67%; 17-33% showed abnormal handling of potassium; and most patients had a subnormal concentrating capacity. CONCLUSIONS: Excellent long-term graft survival and good graft function can be achieved with triple immunosuppression, even in young CAD kidney recipients.

Successful liver transplantation after induction chemotherapy in children with inoperable, multifocal primary hepatic malignancy.

BACKGROUND: The prognosis for primary epithelial liver tumor in children in whom radical surgery cannot be performed after chemotherapy is poor. Orthotopic liver transplantation has resulted in mortality up to 50%, largely as a result of problems in determining the criteria for transplantation. METHODS: We report results on liver transplantation for primary epithelial liver malignancy in five children (mean age at transplantation: 6.0 years). Only patients with inoperable residual tumor in the liver after four cycles of multidrug chemotherapy, but without extrahepatic infiltration or metastases, were considered eligible for transplantation. RESULTS: Mean follow-up was 4.6 years. Patient and graft survival was 100%, with no signs of residual or de novo malignancy. CONCLUSION: In children with inoperable primary liver malignancy with no extrahepatic tumor growth, orthotopic liver transplantation has an excellent outcome.

Spontaneous breathing and total body oxygen consumption in children recovering from open-heart surgery.

The effects of withdrawal of ventilatory support on cardiopulmonary function, oxygen consumption and carbon dioxide production were assessed in 25 infants and children within seven days (2.9 +/- 2.5 days; mean +/- SD) of an open heart operation, during weaning from ventilatory support. The average age of the patients was 3.4 +/- 3.5 years and weight 12.4 +/- 8.3 kg. Heart rate, blood pressure, arterial and central venous blood gas values, and oxyhemoglobin saturations were measured during controlled mechanical ventilation and during spontaneous breathing with continuous positive airway pressure. Simultaneously, VO2 and VCO2 were measured using indirect calorimetry. Withdrawal of ventilatory support effected an expected, significant decrease in arterial pH (7.42 +/- 0.10 to 7.37 +/- 0.06; p less than 0.001) and an increase in PaCO2 (34 +/- 6 to 40 +/- 5 mm Hg; p less than 0.0001), while arterial blood oxyhemoglobin saturation, heart rate, and blood pressure remained unchanged. A significant increase in central venous oxyhemoglobin saturation (67.9 +/- 11.9 to 74.8 +/- 8.3 percent; p less than 0.001) indicated improvement in systemic blood flow during spontaneous breathing. Average VO2 and VCO2 did not change significantly. A decrease in VO2 by more than 5 percent was seen in seven patients, an increase by more than 5 percent in nine, and a change within +/- 5 percent in nine patients. The change in VO2 was inversely related to the difference between measured and expected VO2 during mechanical ventilation (r = -0.73) and to body temperature (r = -0.69). The results indicate that factors other than the oxygen uptake by the respiratory muscles may have significant effects on total body VO2 in infants and children after open-heart surgery. Therefore, monitoring of VO2 during withdrawal of ventilatory support may not be an accurate indicator of respiratory work and oxygen cost of breathing in these patients.

Bleeding and use of blood products after heart operations in infants.

Recent studies have suggested that postoperative bleeding is decreased in pediatric heart operations if fresh whole blood instead of blood component therapy is used for postoperative transfusions. Because this is in contrast to our practice to use whole blood for only the priming of the cardiopulmonary bypass circuit and then to use blood components for additional transfusion requirements, it was our interest to analyze the bleeding complications and the use of blood products after heart operations in infants. The patient records of the 73 infants operated on in 1992 were reviewed. The chest tube drainage varied from 3 to 51 ml/kg per 6 hours (mean 10 ml/kg) and it did not correlate with any of the tested clinical or laboratory parameters. One infant underwent reoperation because of surgical bleeding. Disseminated intravascular coagulation developed in another patient. Sixty-eight patients (93%) needed red blood cell supplementation. Sixty-eight percent of patients between 1 month and 1 year old could be treated without any other postoperative transfusion except for red blood cell supplementation. In contrast, in the neonates, platelet concentrates or fresh frozen plasma, or both, were used in 61% of the patients. In addition to the known immaturity of the hemostatic system, the increased need for platelet concentrates in the neonates was attributed to longer cardiopulmonary bypass time, deeper hypothermia in association with circulatory arrest, larger dosages of heparin, and more extensive plasma dilution during cardiopulmonary bypass. In conclusion, a low rate of bleeding complications and acceptably low general blood loss can be achieved postoperatively with blood component therapy.

Fibrinolysis, antithrombin III, and protein C in neonates during cardiac operations.

Fibrinolysis and coagulation were studied in 10 neonates undergoing cardiac operations for congenital heart defects. Coagulation was activated during cardiopulmonary bypass as evidenced by highly increased prothrombin fragment 1 + 2 levels compared with preoperative values. Prothrombin fragment 1 + 2 levels remained elevated until postoperative day 3. Unlike coagulation, fibrinolysis was not activated during cardiopulmonary bypass but did show late activation on postoperative day 3, as evidenced by elevated levels of the fibrin degradation product D-dimer. Lack of fibrinolytic activation during bypass and its appearance on postoperative day 3 were partly explained by changes observed in tissue plasminogen activator and its inhibitor. During bypass, levels of tissue plasminogen activator and its inhibitor increased by 3.4-fold and 3.2-fold, respectively. In the postoperative period, levels of plasminogen activator inhibitor normalized rapidly whereas tissue plasminogen activator remained elevated, resulting in late fibrinolytic activation on postoperative day 3. In accordance with elevated prothrombin fragment 1 + 2, platelet count, antithrombin III, protein C, prothrombin, and factor VII were decreased on postoperative day 2, indicating ongoing consumptive coagulopathy. Nine patients had antithrombin III and six had protein C levels below age-specific normal ranges, consistent with an acquired deficiency state. Three had central venous thrombosis by postoperative day 4 or 5. In all three, thrombosis was preceded by antithrombin III deficiency, protein C deficiency, and highly elevated plasminogen activator inhibitor (3.7 to 37 times the mean of the other patients) on postoperative days 1 to 3. In conclusion, cardiopulmonary bypass in neonates caused rapid and profound alterations in the coagulation and fibrinolytic systems and initiated consumptive coagulopathy lasting until at least postoperative day 3. Thrombophilic abnormalities in antithrombin III, protein C, and fibrinolysis were frequently found and were associated with serious thrombotic complications.

Regional generation of free oxygen radicals during cardiopulmonary bypass in children.

Studies on free radical generation during cardiopulmonary bypass have focused mainly on the heart and the lungs. However, low pumping pressure, nonpulsatile perfusion, and hypothermia affect the entire circulation, resulting in decreased splanchnic blood flow, increased intestinal permeability, and endotoxemia. To evaluate regional phenomena, we studied 16 children undergoing cardiopulmonary bypass. Free radical production, granulocyte activation, and hypoxanthine metabolism were assessed separately in the circulations drained by the inferior and superior venae cavae, as well as in the oxygenator. Three minutes after the onset of cardiopulmonary bypass, significant gradients between the inferior vena cava and the arterial line of the oxygenator existed in malondialdehyde (+0.60 +/- 0.12 mumol/L, lactoferrin (+18.21 +/- 7.65 micrograms/L), myeloperoxidase (+53.75 +/- 16.50 micrograms/L), hypoxanthine (-0.62 +/- 0.15 mumol/L), and urate (+8.87 +/- 4.03 mumol/L). These gradients decreased in parallel with decreasing body temperature. Except for a transient gradient in malondialdehyde at 3 minutes after the onset of cardiopulmonary bypass (+0.23 +/- 0.08 mumol/L), no changes were detected between the superior vena cava and the arterial line. In the oxygenator, granulocyte activation was observed only after aortic declamping. We conclude that during cardiopulmonary bypass, significant free radical generation, granulocyte activation, hypoxanthine elimination, and urate production take place in the region drained by the inferior vena cava. In the oxygenator, granulocyte activation occurs only after aortic declamping.

Kidney function in cyclosporine-treated pediatric heart transplant recipients.

BACKGROUND: End-stage kidney disease may develop in 1% to 3% of cyclosporine-treated heart transplant recipients, and most patients show a decreased glomerular filtration rate. There are little data on kidney function in pediatric recipients, although good function is needed for their optimal development. METHODS: Kidney function was prospectively investigated in 10 children receiving triple immunosuppression (cyclosporine, azathioprine, methylprednisolone) during the first 18 months after heart transplantation. The early cyclosporine trough level target was 300 to 500 micrograms/L and 100 to 200 micrograms/L after the first year. 51Chromium-ethylenediamine tetraacetic acid, para-amino hippuric acid, lithium, and sodium clearances, measurements of serum and urinary electrolytes, and urinary concentration tests were performed. Renal biopsy specimens were obtained from four patients after 18 months. RESULTS: Heart function was good in all patients. Six patients (60%) remained rejection-free at 18 months. The mean glomerular filtration rate was 92.4 ml/min/1.73 m2 before transplantation, increased to 115 by 6 months (p < 0.05), and thereafter remained stable. The mean renal plasma flow was 487 ml/min/1.73 m2 after 18 months. Hypertension was seen in all patients at discharge but in only one at 18 months. Mild hyperuricemia was the most common sign of tubular dysfunction occurring in five patients at discharge but in only two patients at 18 months. The result of kidney histopathologic study was normal in three of four patients, and cyclosporine nephrotoxicity was not diagnosed. CONCLUSIONS: Triple immunosuppression with cyclosporine adequately protects the graft against acute rejection. It is compatible with normal glomerular function and leads to only minor tubular disturbances.

Value of C-reactive protein in reflecting the magnitude of complement activation in children undergoing open heart surgery.

The kinetics of C-reactive protein (CRP) were studied prospectively in 30 children (aged 21 days - 16 years) undergoing open heart surgery. CRP was related to the kinetics of total haemolytic complement, complement C3a and postoperative complications. Two (7%) patients died and ten (33%) had postoperative complications. The patients with complications were younger (p less than 0.035), underwent longer perfusions (p less than 0.001) and had longer aortic cross-clamping times (p less than 0.003). The mean peak CRP level after surgery (108 mg/l) was reached, on the average, in 43 h. No statistical difference in CRP concentrations was found between the complication and non-complication groups. Extensive complement activation was seen in every patient. CRP did not reflect the magnitude of complement activation induced by cardiopulmonary bypass. The patient sample was too small to draw reliable conclusions about the value of CRP in detecting postoperative complications after open heart surgery in children.

Hyperamylasemia after cardiac surgery in infants and children.

OBJECTIVE: This study was conducted to clarify the incidence of hyperamylasemia after cardiac surgery in infants and children. DESIGN AND PATIENTS: 186 infants and children operated on at Children's Hospital. Helsinki, during an 11-month period were enrolled in the study. Serum samples were taken before and on 3 consecutive days after cardiac surgery at the intensive care unit and before discharge from the hospital. MEASUREMENTS: We measured serum total amylase and serum pancreatic amylase with two different assays (1) reduction of salivary amylase from total amylase activity and (2) measurement of mass concentration with monoclonal antibodies. RESULTS: Preoperative values for both total amylase and pancreatic isoenzymes were strongly age-related. At least one of the three tests showed postoperative hyperamylasemia (> +/- 2 SD above starting values of the age group and maximal value > 3 times the individual starting value) in 64/186 (34%) patients. 22/186 (12%) patients had abnormal results in all assays. A more than tenfold rise in pancreatic amylase, suggesting pancreatitis, was found in 14 patients (8%). Mortality was 21% in this subgroup, but 5% in the rest of the patients. Hyperamylasemia was more common after 1 year of age, and after open-heart surgery, especially homograft implantation or cardiac transplantation. CONCLUSIONS: Hyperamylasemia is a common finding after cardiac surgery in pediatric patients. Amylase isoenzyme measurements are needed for clinical decision making. Age-group-related reference values are mandatory for the right interpretation of amylase values.

Prolonged granulocyte activation, as well as hypoxanthine and free radical production after open heart surgery in children.

OBJECTIVE: To investigate granulocyte activation, as well as hypoxanthine and free radical production in children during the first day after cardiopulmonary bypass. DESIGN: A prospective study of pediatric patients undergoing either cardiac surgery with a cardiopulmonary bypass or thoracotomy and extracardiac vascular surgery not requiring a cardiopulmonary bypass. SETTING: Operative and intensive care units, Children's Hospital, University of Helsinki, Finland. PATIENTS: Seven consecutive patients undergoing elective correction of a ventricular septal defect and six patients undergoing extracardiac surgery for ligation of a patent ductus arteriosus or repair a coarctation of the aorta. MEASUREMENTS AND MAIN RESULTS: Plasma concentrations of myeloperoxidase (140-334 micrograms/l preoperatively, 460-1692 micrograms/l at 0.2 h after declamping, 471-1386 micrograms/l at 0.5 h after declamping) and lactoferrin (77-258 micrograms/l preoperatively, 533-1783 at 0.2 h, 404-1482 micrograms/l at 0.5 h) as markers of granulocyte activation, and hypoxanthine (0-5.7 mumol/l preoperatively, 4.3-17.0 mumol/l at 0.2 h, 6.5-17.9 mumol/l at 0.5 h) increased in a biphasic manner at 0.2-0.5 h and 6-10 h postoperatively (all p < 0.05). Expired ethane, as an index of free radical activity, increased at 10 h postoperatively (36-119 pmol/kg per min preoperatively, 72-152 pmol/kg per min, p < 0.005). CONCLUSION: Granulocyte activation, and hypoxanthine and free radical production occur at least 10 h after cardiopulmonary bypass. In children undergoing open heart surgery, attempts to reduce free radical activity should be extended to the postoperative period.

Direct reconstruction of the pulmonary artery during the arterial switch operation: an interesting surgical option with excellent hemodynamic results.

BACKGROUND: In transposition of the great arteries, reconstruction of the neo-pulmonary artery is a challenging surgical detail during the arterial switch procedure. We present early and midterm clinical and hemodynamic results of a direct reconstruction of the pulmonary artery avoiding prosthetic and autologous material. METHODS: Between 1990 and June 1996, a total of 189 patients underwent the arterial switch procedure because of D-transposition of the great vessels. Of them, 47 underwent direct pulmonary artery reconstruction. Mean age at operation was 5.2+/-4.1 days and mean weight was 3.75+/-0.85 kg. Simple transposition of the great arteries was present in 13, transposition of the great arteries plus ventricular septal defect in 27, and more complex forms of transposition of the great arteries in 7 patients. The great vessels were side-by-side in 4 patients and in the anteroposterior position in 43 patients. The technique of direct pulmonary reconstruction includes extensive mobilization of both pulmonary artery branches into the hilum, posterior incision of the mean pulmonary artery into the bifurcation, and resuspension of the posterior commissure of the neo-pulmonary valve. A large anastomosis without any tension is then performed, using the anterior remnant aortic sinus of Valsalva to fit out the expected size of the neo-pulmonary artery. RESULTS: Early mortality was 8.5% (4/47) in this particular group of patients. Postoperative echocardiography was performed before hospital discharge, 3 to 6 months postoperatively, and after a mean follow-up of 24 months. Of the 43 survivors, 37 patients had a pressure gradient across the pulmonary valve of less than 15 mm Hg. Mild pulmonary stenosis (pressure gradient of 15 to 30 mm Hg) was present in 4 and more severe supravalvar stenosis (pressure gradient > 30 mm Hg) in 2 patients. After a mean follow-up of 36 months, there was one redo operation to enlarge the right ventricular outflow tract. CONCLUSIONS: Direct reconstruction of the neo-pulmonary artery-avoiding autologous pericardium and prosthetic material-may represent an interesting option during the arterial switch operation when the great vessels lie in the anteroposterior position. This technique is simple, and the hemodynamic midterm results are very favorable. The incidence of postoperative supravalvar pulmonary stenosis is low, and there may be considerable potential for unlimited tissue growth.

Delayed impairment of cerebral oxygenation after deep hypothermic circulatory arrest in children.

BACKGROUND: Clinical studies of deep hypothermic circulatory arrest (DHCA) have focused only on the immediate postoperative period. However, experimental findings suggest impairment of cerebral oxygenation at 2 to 8 hours after reperfusion. METHODS: In 10 children who had DHCA for heart operations, transcerebral differences of hemoglobin oxygen saturation and plasma hypoxanthine, xanthine, and lactoferrin concentrations were measured in concurrently obtained cerebral venous, arterial, and mixed venous samples up to 10 hours postoperatively. RESULTS: Compared with preoperative levels (57% +/- 7%), cerebral venous oxygen saturation was not significantly reduced until 2 hours (44% +/- 6%) and 6 hours (42% +/- 5%) after DHCA (p < 0.05). A statistically significant transcerebral (ie, cerebral vein versus artery) concentration difference of hypoxanthine was observed at 30 minutes (3.6 +/- 0.9 micromol/L), 1 hour (3.4 +/- 1.1 micromol/L), and 2 hours (3.1 +/- 0.8 micromol/L) after DHCA but not preoperatively (0.4 +/- 0.2 micromol/L). A transcerebral concentration difference of lactoferrin occurred 30 minutes after DHCA (196 +/- 70 microg/mL) but not preoperatively (16 +/- 20 microg/mL). CONCLUSIONS: Cerebral venous oxygen saturation of hemoglobin decreased as late as 2 to 6 hours after DHCA, in association with impaired cerebral energy status. Neutrophil activation in the cerebral circulation occurred 30 minutes after reperfusion.

Diminutive pulmonary artery growth following right ventricular outflow tract enlargement.

We have assessed the clinical results and angiographic pulmonary artery growth following construction of the right ventricular outflow tract in 10 children with tetralogy of Fallot or pulmonary atresia. All cases were associated with diminutive pulmonary arteries and considered unsuitable for total corrective surgery. The mean age of the children was 34 months, mean weight 10 kg and mean body surface area 0.48 m2. The right ventricular outflow tract was constructed by insertion of a patch of dura mater (5 patients), pericardium (2 patients), homograft valved conduit (2 patients) and infundibulectomy (1 patient). The ventricular septal defect was not closed. Study of the patients between 13 and 37 months postoperatively revealed an increase in mean arterial oxygen saturation of 22% (P less than 0.01) and a fall in mean haemoglobin concentration of 1.6 g% (P less than 0.05). Overall change in pulmonary artery diameter compared to that of the trachea was insignificant although in individual cases a 50% increase in diameter was shown. Palliation of symptoms was equivalent to conventional shunting procedures. Closure of the ventricular septal defect was performed successfully in 2 patients at a later date.

Malformation and stenosis of the cricoid cartilage in association with Larsen's syndrome.

Three patients with the typical features of Larsen's syndrome are described. All three developed severe respiratory symptoms caused by a congenital subglottic stenosis. Tracheotomy and treatment of the stenosis by means of laryngotracheoplasty resulted in complete collapse of the cricoid cartilage and the proximal tracheal skeleton. Lack of rigidity of the laryngeal and tracheal cartilages in patients with Larsen's syndrome could well be responsible for this failure. Surgical treatment consisted of resection of the stenotic and collapsed areas and end-to-end anastomosis. This therapy was eventually successful in all three patients.

Pancreatitis after open heart surgery in children.

Between January 1981 and March 1986, we found 54 children with abnormal serum amylase values or clinical pancreatitis after open heart surgery. Of these 33, had increased serum amylase values only, and 19 had increased serum amylase values in conjunction with clinical pancreatitis. Two patients had haemorrhagic pancreatitis identified at autopsy. The mortality was 42.9% (9/21) in patients with pancreatitis compared to 9.1% (3/33) with amylasaemia only. Pancreatitis is a serious complication after complex open heart surgery in children. The aetiology is probably of vascular origin, and routine serum amylase screening is recommended.

Lung mechanics and airway pressure transmission in infants after open heart surgery.

To evaluate the effects of positive airway pressure on lung mechanics and airway pressure transmission into the intrathoracic space, airway and intrathoracic pressure were recorded during stepwise lung inflation in 17 infants undergoing open heart surgery during the first year of life. Eleven of the 17 patients had cuffed tracheal tubes allowing calculation of lung and chest wall compliance. In 7 of these 11 patients, lung inflation to 2.5 ml/kg above functional residual capacity produced an improvement in initially low lung compliance. This volume increment required elevation of airway pressure by 4-11 cm H2O above ambient. Further lung inflation to 10 ml/kg did not effect an improvement in lung mechanics. In the remaining patients, initial lung compliance was high and remained unchanged throughout the range of lung inflation. Transmission of pressure into the intrathoracic space averaged 47 +/- 9% (mean +/- SD) and ranged from 33% to 61% when airway pressure was 10-15 cm H2O. The results indicate that the use of moderately elevated airway pressure is required to optimize lung distensibility in most infants immediately after open heart surgery. When evaluating circulatory effects of elevated airway pressure, assumption of a 50% pressure transmission is appropriate for clinical purposes. However, the observed wide range of pressure transmission warrants careful hemodynamic monitoring during continuous positive pressure breathing.

Primary mediastinal tumors in children.

From 1950-1986, a total of 159 children (age 1 day-16 years) were treated for primary mediastinal tumors at our hospital. There were 77 malignant and 82 benign tumors. Tracheal compression causing respiratory distress was a significant symptom in 45.3% (24/53) of the children under 2 years of age. The diagnosis was based on the chest X-ray and the findings at surgery. Malignant lymphoma was usually diagnosed by cervical lymph node biopsy (23/39) but the treatment protocol was non-surgical. Non-lymphatic malignant tumors were completely or partially excised in 59.0% (23/39) of the cases. There was no early or late mortality in patients with benign tumors. At follow-up (0.5-24 years; mean 6.0 years), 62.3% (48/77) of the patients with malignant tumors were alive and symptom free. About half of the mediastinal tumors in children are malignant. Mediastinal tumors in small children can cause severe respiratory symptoms demanding urgent treatment. The treatment of choice is surgery (except in lymphomas) and the results are good even in malignant tumors.

Evaluation of the relationship between preoperative risk scores, postoperative and total length of stays and hospital costs in coronary bypass surgery.

OBJECTIVES: Several risk indices have been developed for the prediction of postoperative mortality and morbidity in coronary artery bypass operations, in which the risk scores are currently recorded as routine praxis. The aim of the present study was to determine whether the risk scores can be used to predict the hospital (LOS) and postoperative (POS) lengths of stay and total costs among coronary artery bypass graft (CABG) patients. METHODS: All first-time CABG patients (n=2104) treated at Helsinki University Central Hospital during 1997-1998 were preoperatively scored using the Cleveland Clinic preoperative model. A multivariate analysis was used to evaluate the effects of the risk scores on the LOS and POS and total costs. RESULTS: The mean preoperative risk score for the patients was 1.69. The increase in preoperative risk score was associated with an increase in the LOS (0.8 days by point), and POS (with 0.55 days by point). An age over 74 years increased the LOS by an extra day. The mean total cost for the CABG procedure was 8750 euros (SD 4430 euros). The costs increased as the risk score increased. Compared with the zero risk score, a score value of 2 was associated with a 1300 euros increase in total cost and a score value of over 6 was associated with an over 7000 euros cost increase. On average, the costs increased by 6980 euros (80%) for one major complication and by 935 euros (10%) in the elderly (>74 years of age). CONCLUSIONS: The results show that increasing risk scores were associated with longer postoperative hospital lengths of stay (POS and LOS) and with increased total costs. An age over 74 years appears to be an independent risk factor in increased POS, LOS and total cost. These results may help to estimate the impact of the preoperative risk profile on the resource requirement in CABG surgery.

Iatrogenic, unexpected and other vascular rings in children.

Between May 1955 and July 1987, 33 children with a vascular ring compression syndrome were treated at this institution. There were 4 iatrogenic, 2 unexpectedly found and 27 symptomatic congenital vascular rings. Accurate diagnosis is based on a water soluble iodine contrast dye oesophagogram, bronchoscopy and angiography which are complementary examinations. All symptomatic congenital vascular rings were treated successfully without mortality, but the mortality was 50% if the lesion was iatrogenic in origin or unexpectedly found during palliative or corrective cardiac surgery.

Bronchoscopy during the first month of life.

During the 6-year period from 1984 to 1989, 196 bronchoscopies were performed on 132 neonates. The indications were grouped into four categories: (1) difficulties in artificial ventilation or failure to wean the baby from the ventilator (52); (2) other respiratory difficulties (52); (3) audible stridor (16); and (4) routine preoperative or postoperative examination of esophageal atresia patients (12). The most common finding was laryngomalacia or tracheomalacia (31). Other findings were: obstructing tracheal or bronchial granulation or stricture (23), obstructing mucous plug (22), grave tracheobronchitis (11), tracheoesophageal H-fistula (5), laryngeal perforation (3), congenital laryngeal stenosis (2), and complete laryngotracheoesophageal cleft (1). Four patients had miscellaneous pathology. The findings were normal in 30 patients. A therapeutic procedure was included in 99 of the 196 bronchoscopies. Seven serious complications occurred during the operative bronchoscopies, two of them requiring immediate pulmonary surgery. All complications were successfully managed. In contrast, no complications occurred in the 97 purely diagnostic bronchoscopies. In experienced hands, bronchoscopy of the newborn is a safe and useful examination. Complications occur when therapeutic procedures are included. Therefore, operative bronchoscopy should only be performed in conditions in which immediate thoracotomy and pulmonary surgery can be performed.