Kathleen P. Heidelberger

Aortic aneurysm after patch aortoplasty repair of coarctation: A prospective analysis of prevalence, screening tests and risks

Twenty-nine children were evaluated prospectively for the presence of an aortic aneurysm at the repair site 1 to 19 years after patch aortoplasty repair of coarctation of the aorta. In each child, noninvasive evaluation included a chest X-ray film, computed tomography of the chest and two-dimensional echocardiography. The presence and size of an aortic aneurysm were determined quantitatively by measuring the ratio of the diameter of the thoracic aorta at the repair site to the diameter of the aorta at the diaphragm (aortic ratio). An aortic ratio of greater than or equal to 1.5 was judged abnormal and was shown to be significantly greater than the aortic ratio of a normal control group. An aortogram was obtained in each child if any noninvasive screening test was found to be abnormal. As assessed by the aortogram, the prevalence of aortic aneurysm was 24% in this patient group. The sensitivity of echocardiography and chest computed tomography for detecting an aneurysm was 71% and 66%, and the specificity 76% and 85%, respectively. The chest X-ray film was 100% sensitive and 68% specific in determining the presence of an aneurysm. Although the data are not statistically significant, they suggest that children undergoing patch aortoplasty as the primary procedure (rather than a reoperation after earlier resection), and children in whom a Dacron patch is utilized may be at increased risk for aneurysm formation.(ABSTRACT TRUNCATED AT 250 WORDS)

Hodgkin's disease in children

Relatively few studies of Hodgkins disease (H.D.) in children have used the histologic criteria of the Rye classification. In our study, 55 cases of H.D. in children of 15 years and younger were histologically classified according to the Rye scheme. The cases were divided into 3 groups: 1. all patients, 2. those in their first decade of life, and 3. those aged 11 through 15. The majority of children were boys (62%). Nodular sclerosis was the most frequent histologic type seen in all children (49%) and in the third group (57%), whereas no preponderant histologic type was seen in the younger age group. Most cases of lymphocytic predominance were clinical Stage I, and the bulk of the long‐term survivors had this histologic type. Although seen in all clinical stages at the time of diagnostic biopsy, most cases of nodular sclerosis were Stage II. Prognosis of patients with nodular sclerosis was considerably worse than that of children with lymphocytic predominance. The results of our study are compared to those of other investigators.

Aneurysmal bone cysts in young children

We reviewed seven young children (< or = 10 years) with aneurysmal bone cysts. There were four girls and three boys. Six had involvement of the long bones and one had involvement of the clavicle. The average age was 5.5 years (range 2.9-10.6 years). Initial treatment was curettage and bone grafting. There were recurrences in five of the seven children (71%). This represented 100% of children with radiographically aggressive or active lesions. The recurrences appeared rapidly, at an average of 8 months from the first procedure. The mitotic index of the initial lesion did not correlate with that of the recurrent lesion. Surgical management of the recurrences must be handled individually, but repeat curettage and grafting is only recommended when surgical resection is not possible. This high rate of recurrence in radiographically aggressive or active aneurysmal bone cysts in young children should be considered when planning treatment, and in the preoperative counseling of parents.

Conjugated hyperbilirubinemia in infancy associated with parenteral alimentation

Liver biopsy was performed to exclude anatomic obstruction of the biliary tract in five prematurely born infants who had developed conjugated hyperbilirubinemia during intravenous alimentation with a protein hydrolysate. Each was being treated after having undergone a segmental intestinal resection for necrotizing enterocolitis. Bacterial and viral infections, metabolic disorders, and isoimmune hemolytic disease were excluded as possible causes of jaundice. Light microscopic and ultrastructural analysis disclosed cholestasis and hepatocellular injury without significant inflammatory reaction. Jaundice abated following permanent discontinuation of parenteral alimentation. The jaundice and cholestasis are interpreted to be hepatotoxic effects because of (1) their temporal relationship to the treatment and (2) the presence of hepatocellular damage.

Enterocolitis associated with Hirschsprung's disease: A clinical histopathological correlative study

Enterocolitis associated with Hirschsprungs disease (HEC) remains a major source of morbidity and even mortality, both before and after definitive surgical treatment. This study was undertaken to investigate whether histopathologic mucosal changes, in the absence of clinical manifestations of HEC, could predict the subsequent development of this complication. The clinical data and histopathology of 25 patients who eventually developed clinical HEC were compared with a control group of 25 age- and sex-matched patients with Hirschsprungs disease (HD) but with no clinical HEC either preoperatively or postoperatively. The histopathologic findings of tissue obtained by rectal biopsy or during laparotomy were graded from 0 to V according to severity and compared with the eventual clinical course. This study showed that (1) histopathologic findings of HEC appear to predict the clinical development, but not the severity, of future episodes of clinical HEC; (2) a histological grade of > or = II (> or = 2 crypt abscesses per high power field) should raise suspicion for subsequent occurrence of HEC, whereas a grade of > or = III (multiple crypt abscesses) places a child at high risk for development of clinical HEC; and (3) contrary to what is generally thought, histopathologic changes of HEC occur in both ganglionic and aganglionic segments. Based on these findings, it is recommended that histopathologic documentation of HEC and its grade should be an integral part of the tissue diagnosis of HD.

Osteoid osteoma and osteoblastoma of the spine

Eleven patients with spinal osteoid osteoma and six patients with spinal osteoblastoma treated between 1975 and 1990 were reviewed to characterize the tumors as they affect the spine and to define the important differences between the two tumors. All patients with cervical osteoid osteoma presented with pain, limited range of motion of the neck, and torticollis. Four osteoblastomas had soft-tissue components in the epidural space, necessitating dissection of the tumor from the dura. No soft-tissue component was found in any of the osteoid osteomas. Our results were similar to a metaanalysis of the clinical, radiographic, and surgical findings of all published cases of spinal osteoid osteoma and osteoblastoma. Important features that have not been emphasized in the literature are the high incidence of torticollis with cervical lesions and the frequent association of epidural invasion with osteoblastoma. Surgeons treating osteoblastoma of the spine should be prepared to dissect tumor from the dura.

Diagnosis and management of congenital cystic disease of the lung in children

Pulmonary sequestration, congenital adenomatoid malformation (CAM), congenital lobar overinflation (CLO), and bronchogenic cysts are four congenital lesions that may present as abnormal cystic areas within the pleural cavity in early life. They share similar clinical and embryologic characteristics, are frequently difficult to diagnose, and all require surgical treatment. From December 1974 to January 1985, 22 patients were operated upon on the Pediatric Surgical Service for congenital cystic disease of the lung. There were eight females and 14 males, ranging in age from 1 day to 18 years; 12 were under 6 months of age, and 14 were under one year. There were 7 CAMs, 5 extralobar pulmonary sequestrations, 5 intralobar pulmonary sequestrations, 3 bronchogenic cysts, and 3 cystic lobes caused by CLO. One patient had bilateral pulmonary sequestrations, intralobar on one side and extralobar on the other, with both sharing a common systemic artery arising from the infradiaphragmatic aorta. All except two asymptomatic patients presented with either progressive respiratory distress or recurrent pulmonary infections, and had cystic changes noted on chest roentgenograms. The differentiation of these congenital lesions from resolvable inflammatory cysts is important. Diagnosis was aided by selective utilization of barium contrast studies, ultrasonography, computed tomography, and arteriography. Operation consisted of resection alone of the extralobar pulmonary sequestrations and bronchogenic cysts, and total lobectomy in CAM, intralobar sequestration, and CLO. One infant with CAM died 1 day postoperatively from bilateral hypoplastic lungs. The other 21 patients are alive and well with follow-up ranging from 1 month to 9 years, with a mean of 3.5 years.(ABSTRACT TRUNCATED AT 250 WORDS)

The Influence of a Restrictive Atrial Septal Defect on Pulmonary Vascular Morphology in Patients with Hypoplastic Left Heart Syndrome

Hypoplastic left heart syndrome (HLHS) with a restrictive atrial septal defect (ASD) is a form of congenital heart disease with considerable morbidity and mortality. This morphologic analysis assesses the pulmonary vasculature in this patient population. Pulmonary arteries, the persistence of high-resistance fetal arterioles, pulmonary veins, and lymphatics from multiple lung sections from each of five patients with HLHS and a restrictive ASD were compared to those of five patients with HLHS and nonrestrictive ASD. Lung sections from each patient were qualitatively graded in severity of pathology from 0 to 3 for each of the structures described previously, with the pathologist blinded to the status of the ASD. Patients with a restrictive ASD exhibited more significant pulmonary venous thickening and lymphatic dilatation (p = 0.02), with a tendency toward persistence of high-resistance fetal vessels (p = 0.2), compared to patients with a nonrestrictive ASD. These findings imply that patients with HLHS and a restrictive ASD possess pulmonary vascular abnormalities that place them at higher risk for the current surgical interventions available compared to patients with a nonrestrictive ASD.

Prolonged extracorporeal life support of pediatric and adolescent cardiac transplant patients

Options for mechanical support of pediatric patients with severe heart failure who are awaiting transplantation or have undergone transplantation are limited. This report examines 3 patients placed on extracorporeal life support (ECLS) while awaiting transplantation and 3 patients who underwent transplantation and suffered subsequent heart failure due to rejection or postoperative myocardial dysfunction. The overall survival rate was 2 of 6. The 2 surviving patients had a failing transplanted heart. There were no survivors among the patients placed on ECLS as a bridge to transplantation. In each case a contraindication to transplantation developed before a donor heart could be obtained. The mean time of ECLS support was 147.5 hours (range, 70 to 370 hours). The ECLS circuit did not affect cyclosporin levels or antirejection therapy. Extracorporeal life support can be used to support pediatric cardiac transplant patients with biventricular failure due to acute rejection or postoperative dysfunction. Although the results have been discouraging, ECLS may still have a role as a bridge to transplantation. However, complications can develop during ECLS that may preclude transplantation.

Stent redilation in canine models of congenital heart disease: Pulmonary artery stenosis and coarctation of the aorta

In a canine puppy model, pulmonary artery stenosis was created by banding the left pulmonary artery to 30-40% of its original diameter. Animals underwent right heart catheterization and angiography 1-2 mo later, and Palmaz P308 stents were implanted. Stent redilation was performed 3-5 mo later. One mo postredilation, the animals were restudied and sacrificed. Coarctations of the aorta were created by transverse aortic incision and longitudinal repair. P308 stent implantation was performed 2-3 mo later. Stent redilation was performed after 6-10 mo, and the animals were restudied and sacrificed 1-2 mo later. Stent implantation was performed in 6 puppies with pulmonary artery stenosis, as 2 animals developed postoperative pulmonary arterial hypoplasia, precluding stenting. The stenosis diameter increased from 4.8 +/- 0.5 mm to 7.4 +/- 0.6 mm (mean +/- SE) following stenting (P = 0.005), and increased further to 9.2 +/- 0.7 mm following redilation (P < 0.001). There were no significant vessel tears or ruptures. Coarctation stenting was performed in 8 animals. The coarctation was dilated from 5.8 +/- 0.9 mm to 9.8 +/- 0.6 mm (P < 0.001), and to 13.5 +/- 0.5 mm at redilation (P = 0.002). Redilation could not be performed in 1 animal. Aortic rupture and death occurred in 2 of 7 animals at redilation. Stent implantation and redilation in experimental pulmonary artery stenosis appears safe and effective. Though stent implantation for coarctation of the aorta appears safe, there was a 28% aortic rupture rate at stent redilation in this model.