Jacques G. LeBlanc

Rhabdomyosarcoma arising within congenital pulmonary cysts : report of three cases

Over the past 9 months, three cases of primary pulmonary rhabdomyosarcoma have been treated at British Columbia Childrens Hospital. Two patients (aged 24 and 37 months) presented with spontaneous pneumothoraces and had cystic changes in the affected lung on chest radiograph. The third patient (aged 42 months) was evaluated for chronic cough, fever, and failure to thrive. Chest x-ray showed a large mass in the left lower lobe as well as mediastinal adenopathy. All three of these lesions originated within congenital lung cysts, one a peripheral bronchogenic cyst and the others cystic adenomatoid malformations. This report suggests that there is a significant risk for the development of rhabdomyosarcoma within malformed pulmonary tissue.

Transaxillary pleurectomy for treatment of spontaneous pneumothorax.

In the 16-year period 1962 to 1978, 409 transaxillary apical pleurectomies were carried out for definitive treatment of spontaneous pneumothorax in 362 patients. Surgical indications included recurrence (336), bilaterality of the disease (23), persistent air leak (22), and nonexpansion of the lung (10). There was 1 operative death (unsuspected brain tumor), and 3 patients required reexploration for clot removal. The average postoperative period of hospitalization was 6 days. Three hundred ten patients (86% of all patients) were contacted for follow-up 1 to 16 years after operation (average, 4.5 years). There were two documented episodes of recurrent ipsilateral pneumothorax (0.6%). Postoperative pulmonary function studies were done in 40 patients (unilateral, 29; bilateral, 11) 2 to 5 years after operation. The results indicate that there are no significant abnormalities compared with predicted values.

Giant hydatid lung cysts in the Canadian northwest: Outcome of conservative treatment in three children☆

Hydatid lung disease due to Echinococcus granulosus in the Canadian northwest and Alaska is often asymptomatic and usually benign. We reviewed the course and outcome of three children with giant hydatid lung cyst seen over a 2-year period. All were North American Indian children aged 9 to 12 years who presented with cough, fever, and chest pain. One had a rash. There was a history of exposure to domestic dogs who had been fed moose entrails in each case. Chest x-rays showed solitary lung cysts with air-fluid levels, from 6 cm to 12 cm in diameter. Aspiration of each cyst demonstrated Echinococcus hooklets and protoscolices. Serology was unhelpful, being negative in two cases. Transient pneumonitis and pneumothorax were seen as complications of needle aspiration. Two cysts gradually resolved over the following 6 months. One child returned after 9 months with a lung abscess due to superimposed infection of the cyst remnant with Haemophilus influenzae, and eventually required lobectomy. The existence of an endemic benign variant of E granulosus in Canada is not widely known, and it is important to distinguish it from the more aggressive pastoral form of the disease seen in immigrants from sheep-rearing countries. The native Canadian disease usually resolves spontaneously, does not cause anaphylaxis, and does not implant daughter cysts if spilled. Surgical treatment should be avoided except for complications such as secondary bacterial infection.

Influence of bidirectional superior cavopulmonary anastomosis on pulmonary arterial growth.

Right-sided BSCA provides for satisfactory pulmonary arterial growth in infants and children with complex congenital heart defects, and it could enhance the growth of a small right pulmonary artery. The growth of the left pulmonary artery, particularly in younger patients, needs close attention to confirm the safe role of BSCA in long-term palliation.

Double-blind placebo-controlled trial of corticosteroids in children with postpericardiotomy syndrome

SummaryThe objective of this study was to assess the efficacy of corticosteroids in hastening the recovery of children with postpericardiotomy syndrome, using a randomized double-blind placebo-controlled trial in a tertiary care referral center for pediatric cardiology and cardiac surgery. Twenty-one children, 6 months of age or older (mean age 3.9 years) with postpericardiotomy syndrome following open or closed heart surgery were administered either prednisone 2 mg/kg/day reducing to zero over 14 days (n=12) or placebo (n=9). Progress was monitored by daily clinical assessment and alternate day cross-sectional echocardiograms. The primary measures of efficacy were the number of patients in remission at 72 h and at 1 week. No difference in remission rates were found at 72 h, but at 1 week significantly more children treated with prednisone were in remission (placebo 3/9; prednisone 10/12,p=0.03). A trend to faster resolution of all symptoms and signs was seen in the prednisone-treated group but this was not associated with earlier hospital discharge. Enlargement of pericardial effusion was seen in two children treated with steroids. No complications of treatment were encountered. Prednisone hastens the recovery of children with postopericardiotomy syndrome. Pericardial effusions may increase in size despite the use of corticosteroids.

Intermediate follow-up of right ventricular outflow tract reconstruction with allograft conduits

BACKGROUND Allograft conduits are among many varieties of material used for right ventricular outflow tract reconstruction. They invariably need to be replaced due to growth of the patient or conduit failure. METHODS From June 1984 to June 1996, a total of 76 patients underwent reconstruction of the right ventricular outflow tract with an allograft conduit: 51 aortic and 25 pulmonary. The median age, weight and conduit size at surgery were 37 months (range, 0.2 to 228 months), 12.4 kg (range, 2.9 to 61.4 kg), and 17 mm (range, 8 to 26 mm), respectively. RESULTS The hospital mortality was 5.3% (4 of 76 patients) and 2 patients died at 9 and 78 months follow-up. The median follow-up was 61 months (range, 2 to 132 months). Reoperation was necessary in 22 patients (28.9%) at a median interval of 50.5 months (range, 3 to 109 months) and the median conduit size was 21 mm (range, 12 to 23 months). There was no mortality. Freedom from reoperation at 64 months was 49.5% for conduits 15 mm and smaller, and 73.3% for conduits 16 mm and larger. Analysis by age shows freedom from reoperation at 64 months of 49.4% and 74.5% for patients younger than and older than 2 years, respectively. At 54 months there was no statistical difference in freedom from reoperation between pulmonary and aortic allografts. CONCLUSION Right ventricular outflow tract reconstruction with allograft conduits results in a high reoperation rate at 4 years but provides significantly longer freedom from reoperation with conduits larger than 15 mm or in patients over 24 months of age.

Assessment of Vocal Fold Mobility Before and After Cardiothoracic Surgery in Children

OBJECTIVES To assess the incidence of vocal fold immobility (VFI) after cardiothoracic surgery in children and to determine the factors potentially associated with this outcome. METHODS Flexible laryngoscopy to assess vocal fold mobility was performed before surgery and within 72 hours after extubation in 100 pediatric patients who underwent cardiothoracic procedures. The 2 operating surgeons recorded the surgical technique and their impression of possible injury to the recurrent laryngeal nerve. The presence of laryngeal symptoms, such as stridor, hoarseness, and strength of cry, after extubation was documented. RESULTS Of 100 children included in this study, 8 had VFI after surgery. Univariate analyses showed that these 8 patients were younger and weighed less than the patients with normal vocal fold movement. Monopolar cautery was used in all patients with VFI. On univariate analysis, factors statistically significantly associated with VFI were circulatory arrest and dissection or ligation of the patent ductus arteriosus, left pulmonary artery, right pulmonary artery, or descending aorta. However, multivariate analyses failed to show these associations. CONCLUSIONS The incidence of VFI after cardiothoracic surgery in our population of children was 8.0% (8 of 100). Of several factors found to be potentially associated with VFI on univariate analysis, none were significant on multivariate analysis. This may be a result of the few patients with VFI. A larger multicenter prospective study would be needed to definitively identify factors associated with the outcome of VFI.

Pulmonary Artery Banding: Results and Current Indications in Pediatric Cardiac Surgery

The results of pulmonary artery banding in 144 patients seen from 1971 to 1984 were reviewed. Age ranged from 1 week to 4 years (median, 8 weeks) and weight, from 1.1 to 16 kg (median, 4 kg). The patients were divided into three major groups: Group 1, defects without mixing disorders (ventricular septal defect, double-outlet right ventricle [DORV], atrioventricular septal defect); Group 2, defects with mixing disorders (transposition of the great arteries, DORV, single ventricle, tricuspid atresia); and Group 3, miscellaneous (mitral atresia, left ventricular hypoplasia, truncus complex). The diagnostic group influenced survival (p = 0.0035). In Group 1, 88.8% survived, but only 64.9% survived in Groups 2 and 3 combined. The presence of patent ductus arteriosus or coarctation of the aorta had no effect on survival (p = 0.61 and p = 0.7, respectively). The clinical condition at thirty days after pulmonary artery banding was good in 35.1% and fair in 46.9% of the patients. When the data were divided into the three periods 1971 through 1974, 1975 through 1979, and 1980 through 1984, which included 28, 49, and 67 patients, respectively, a significant improvement in survival was observed from the early (64.3%) to the late period (92.5%) (p = 0.0009). Patients weighing less than 4 kg had a significantly lower survival in the period 1971 through 1974 (37.5% versus 91.67%). No significant difference in survival was detected in the late period, 1980 to 1984 (90% versus 94.6%), between patients weighing less than and those weighing more than 4 kg. Pulmonary artery banding is clinically satisfactory in small infants and children with complex anomalies.

Treatment of grafts and major vessel thrombosis with low-dose streptokinase in children.

Low-dose streptokinase infusions have been used in 8 of our patients. Five of these were newborns who had major vessel occlusion. Four babies had extensive aortic thrombosis and hypertension producing congestive aortic thrombosis and hypertension producing congestive heart failure. One baby had caval and renal vein thrombosis and was in renal failure. Two infants with cyanotic heart disease and 1 with arteritis had occluded prosthetic grafts, which were reopened completely. Two grafts were between the subclavian and pulmonary arteries (Blalock-Taussig shunt), and one was between the abdominal aorta and right renal artery. In each patient, the thrombolytic agent was delivered directly to the area of thrombosis by three or four percutaneously inserted French catheters. The dose of streptokinase used was between 50 and 100 U/kg/hr. Therapy lasted for 2 to 11 days. Major bleeding was anticipated, and it occurred in 1 patient. Low-dose streptokinase infusion is a safe and effective treatment for a variety of thrombotic problems in infants and children.

The Contegra conduit: Late outcomes in right ventricular outflow tract reconstruction

Objectives: To report the clinical outcomes (early death, late death, and rate of reintervention) and performance of the Contegra conduit as a right ventricle outflow tract implant and to determine the risk factors for early reintervention. Methods: Forty-nine Contegra conduits were implanted between January 2002 and June 2009. Data collection was retrospective. The mean age and follow-up duration of Contegra recipients was 3.5 ± 4.6 years and 4.2 ± 2.0 years, respectively. Results: There were three deaths (two early, one late), giving a survival rate of 93.9%. The rate of conduit-related reintervention was 19.6% and was most often due to distal conduit stenosis. Age at implantation of <3 months, receipt of a conduit of 12–16 mm diameter, and a diagnosis of truncus arteriosus were each significant contributors to the rate of reintervention. Conclusion: The Contegra is a cost-effective and readily available solution. However, there is a limited range of larger calibers, which means that the homograft conduit (>22 mm) remains the first choice of implant in older children. The rates of reintervention are significantly higher with a diagnosis of truncus arteriosus, age at implantation of <3 months, and implantation of conduits sized 12–16 mm.