Simultaneous adrenocortical carcinoma and ganglioneuroblastoma in a child with Turner syndrome and germline p53 mutation.

The predisposition to malignancy that is dominantly inherited in Li-Fraumeni syndrome is associated with germline mutations of the tumour suppressor gene p53. Although second malignant neoplasms have been described in children with p53 mutations, the synchronous occurrence of two embryologically different tumours in these children has not been reported. A 20 month old girl with failure to thrive and congenital heart defects was found to have unilateral adrenal masses which, at surgical removal, proved to be an adrenocortical carcinoma and a ganglioneuroblastoma. Further investigation showed a germline p53 mutation and Turner syndrome. It remains to be determined what effect the 45,X chromosomal complement may have on the expression of neoplasms seen in patients with p53 germline mutations.

Patent ductus arteriosus ligation: are we doing better?

Limitation on health care resource use is stimulating critical evaluation of previous preoperative standards. We retrospectively reviewed the clinical and hospital financial records of all children admitted for patent ductus arteriosus ligation from July 1984 to April 1994 for age, perioperative length of stay, readmissions for postoperative surgical problem, and hospital charges adjusted to 1994 dollars. Patients with an isolated patent ductus arteriosus, greater than 3 months of age, without preoperative or postoperative complications were included in this study and stratified into two groups based on date of operation. Group I had operation before January 1, 1991, and group II had operation on or after January 1, 1991. Comparison of these two groups revealed a significant difference in perioperative length of stay (group I, 3.9 +/- 1.2 days [mean +/- standard deviation]; group II, 2.7 +/- 0.9 days; p < 0.0001) and in hospital charges (group I, $8,700 +/- $2,100; group II, $6,600 +/- $1,000; p < 0.0001). These data support the premise that children older than 3 months undergoing elective ligation of a patent ductus arteriosus have been treated with improved efficiency and less charge without an increase in postdischarge morbidity. Health care policy decisions have forced us to evaluate the standards of perioperative care more critically.

A resurgence of acute rheumatic fever in a mid-South children's hospital.

A resurgence of acute rheumatic fever (ARF) has been reported in many areas of the United States in recent years. We retrospectively reviewed the medical records of inpatients with a new diagnosis of ARF from 1982 through 1988 at a children's hospital that serves a six-state referral area in the mid-South. Thirty patients were identified, 21 of whom were seen in 1987 (13) and 1988 (8). The rate of new cases of ARF per 1000 hospital discharges (0.7) was significantly greater for 1987 and 1988 than it was (0.15) from 1982 through 1986. Patients with recently diagnosed ARF were predominantly from nonurban areas, and polyarthritis was the most common recent major manifestation. Reasons for the resurgence of ARF in the US, including the mid-South, are unclear, but our experience serves to support recently published guidelines for the diagnosis and management of streptococcal pharyngitis in light of this resurgence of ARF.

Pediatric digital subtraction angiography: intraarterial and intracardiac applications.

Intraarterial digital subtraction angiography (IA-DSA) was performed in 80 pediatric patients. Forty-four underwent arterial injections with digital filming techniques. Of the 130 injections, DSA imaging was good or excellent in 121, often allowing resolution of vessels 1 mm in size. Thirty-six of the 80 patients underwent cardiac evaluation with intra-cardiac injections. Improved contrast resolution with IA-DSA allowed the use of smaller catheters (3-4F) and smaller amounts of contrast material. Immediate availability of subtracted images (no film processing delay) resulted in shorter total procedure time. Outpatient studies are possible. IA-DSA provides several notable advantages for the pediatric patient.

Soft-tissue swelling in two neonates during prostaglandin E1 therapy.

Two small neonates, one with hypoplastic right heart syndrome, and the other with tetralogy of Fallot and pulmonary atresia, were treated with intravenous prostaglandin E1 (Prostyn VR, Upjohn, Kalamazoo, Michigan) for 96 and 33 days, respectively. They developed limited cortical hyperostosis, but marked soft tissue swellings in all extremities. The changes were noted after PGE1 treatment for four weeks and one week, respectively. A skin biopsy was performed in one patient and revealed edematous changes and arteriolar wall abnormalities. We add this peripheral hard edema to the list of complications of PGE1 therapy, and speculate as to its pathophysiology.

An unusual complication of a conduit-mounted homograft: valve leaflet fixation in the open position.

Surgical management of left ventricular outflow tract obstruction in patients with complete transposition of the great arteries and an intact ventricular septum is difficult. Direct resection of subpulmonary stenosis has not produced satisfactory results. Thus, relief of stenosis has been accomplished with valved conduits from the left ventricle to the pulmonary artery. Initially, conduit tissue valves were porcine in nature. More recently, human-origin conduit tissue valves have been employed. In this report, we describe an unusual complication in a child with complete transposition in whom a homograft valve conduit was placed from the left ventricle to the pulmonary artery to relieve subpulmonary stenosis. The leaflets became fixed in an open position. Thus, the conduit was converted to a functionally nonvalved communication. The subpulmonary stenosis regressed, allowing egress from both the natural outflow tract and the conduit.

Modified Shumacker repair of transposition of the great arteries. Surgical and angiocardiographic considerations.

Sixteen patients with transposition of the great arteries, 8 days to 2 1/2 years of age, underwent intra-atrial transposition of venous return by the modified Shumacker technique. A bipedicle flap of right atrial wall was used to direct vena caval return to the mitral valve, and a hinged viable flap of pericardium was used to form the lateral wall of the pulmonary venous pathway. Thirteen patients survived operation and are well to date. Two of the three nonsurvivors were less than 1 month of age at operation, and the third had an associated inlet ventricular septal defect and a straddling tricuspid valve. Early postoperative chest radiographs were compared with preoperative films in each patient. Postoperative findings included minor right pleural effusion in four, minor pneumothorax in two, and paralyzed right hemidiaphragm in one. Two patients required a permanent pacemaker because of the development of sick sinus syndrome 2 and 4 years postoperatively. Each preoperative angiocardiogram was reviewed and compared with postoperative studies in nine patients. The latter demonstrated absence of caval gradients in each, trivial baffle leaks in four, a moderate baffle leak in one, small residual ventricular septal defects in two, and mild tricuspid regurgitation in two patients.