Unusual features in four canine meningiomas.

Several subtypes of canine meningioma are recognized. This report describes four canine meningiomas with previously unreported features. The four affected dogs were of different breeds. Three of the affected dogs were male and aged 7-10 years. In one dog, age and gender were not recorded. Meningiomas were located intracranially (three dogs) or within the vertebral canal (one dog). Two meningiomas resembled gemistocytic astrocytomas, while one had focal features of a rhabdoid tumour; these three meningiomas also contained amyloid deposits. The fourth tumour, a secretory meningioma, was rich in amianthoid fibres (i.e. unusual collagen deposits containing giant collagen fibres). All of these features are also described in human meningiomas.

Large hepatic mesenchymal hamartoma leading to mid-trimester fetal demise.

This report describes a fetus with a large multiloculated cystic liver mass. Two small abdominal cysts were seen on ultrasound at 19 weeks of gestation but the patient was referred to us at 23 weeks, after the mass had grown to 8.0 x 5.6 x 7.0 cm, displacing intra-abdominal organs, heart and diaphragm. There was a small amount of ascites but no hydrops. There was polyhydramnios and a thick hyperechoic placenta. After detailed sonograms and MRI suggested the diagnosis of cystic mesenchymal hamartoma of the liver, cyst decompression was favored and consent was obtained. Unfortunately, absence of fetal cardiac activity was noted on the day of the planned intervention. Autopsy confirmed the diagnosis and demonstrated placental changes consistent with mesenchymal stem villous hyperplasia of the placenta. Large fetal cystic abdominal masses that compress the heart, lungs and other organs may benefit from prenatal decompression. This is the first report of cystic hamartoma of the liver apparent on second-trimester sonography, and the fourth time such a lesion is associated with fetal or neonatal death out of 11 cases diagnosed prenatally.

Pericardial hemangioma presenting as thoracic mass in utero.

Pericardial hemangiomas are rare lesions. We present the case of an infant who was referred to our fetal diagnosis and treatment group for the presence of a left thoracic mass, pleural effusion, and mediastinal shift on fetal ultrasound. The characteristics of the lesion suggested the presence of a pulmonary sequestration. A chest radiograph done at birth was normal. At 2 weeks of age, an enhancing lesion of the left pericardium was identified on chest CT. A cardiac MRI demonstrated enhancement of the mass on T2-weighted images. The patient underwent thoracoscopic assessment of the mass for diagnostic purposes. Multiple lesions were identified along the left pericardium and diaphragm. A frozen section biopsy revealed a hemangioma. The natural history for hemangiomas is gradual regression; however, they may increase acutely in size and cause symptoms prior to involution. Investigations should be performed to identify the involvement of other organs. This case illustrates the need to closely follow all patients with prenatally diagnosed thoracic masses with CT imaging, even when they are asymptomatic and have a normal chest radiograph at birth.

Single ventricle with systemic obstruction in early life: comparison of initial pulmonary artery banding versus the Norwood operation.

OBJECTIVES: The outcomes of initial pulmonary artery banding (PAB)+/-coarctation repair are compared with the Norwood operation in newborns with single ventricle (SV) and systemic obstruction (SO). METHODS: Between January 1987 and July 2000, 22 patients (median age, 12 days) with SV and aortic arch obstruction (AAO), subaortic stenosis (SAS), or both underwent surgery. Two initial surgical approaches were used: PAB+/-coarctation repair (group I, seven patients); Norwood type operation (group II, 15 patients). RESULTS: The overall mortality was 32% (seven of 22 patients). There was no late mortality. The mortality in group I was 43% versus 27% in group II. Recently, there has been no mortality following the Norwood operation in the last eight patients operated since 1995. Of the survivors, nine patients have undergone the Fontan operation and four patients have had the bidirectional Glenn (BDG) with no deaths. There was one repair of supravalvar aortic stenosis at the time of BDG in group II as opposed to eight reinterventions for SAS and/or AAO in four patients in group I (P=0.01). CONCLUSIONS: PAB+/-coarctation repair for SV and SO is associated with a high mortality and a high reoperation rate for SAS or recurrent AAO. Although the Norwood operation was also associated with a high mortality early on, it can now be performed with excellent outcome. This improvement, combined with a low reintervention rate for SAS or AAO, suggests that the Norwood operation is likely to emerge as the procedure of choice for SV and SO.

Isolated ductus arteriosus aneurysm in the fetus and infant: a multi-institutional experience.

OBJECTIVES: The purpose of this study was to describe the clinical characteristics and outcome and to elucidate the pathogenesis of ductus arteriosus aneurysm (DAA). BACKGROUND: Ductus arteriosus aneurysm is a rare lesion that can be associated with severe complications including thromboembolism, rupture and death. METHOD: We reviewed the clinical records, diagnostic imaging studies and available histology of 24 cases of DAA, diagnosed postnatally (PD) in 15 and antenatally (AD) in 9 encountered in five institutions. RESULTS: Of PD cases, 13 presented at <2 months, and all AD cases were detected incidentally after 33 weeks of gestation during a late trimester fetal ultrasound study. Of the 24, only 4 had DAA-related symptoms and 6 had associated syndromes: Marfan, Smith-Lemli-Opitz, trisomies 21 and 13 and one possible Ehlers-Danlos. Three had complications related to the DAA: thrombus extension into the pulmonary artery, spontaneous rupture, and asymptomatic cerebral infarction. Six underwent uncomplicated DAA resection for ductal patency, DAA size or extension of thrombus. In the four examined, there was histologic evidence of reduced intimal cushions in two and abnormal elastin expression in two. Five of the 24 died, with only one death due to DAA. Of 19 survivors, all but one remain clinically asymptomatic at a median follow-up of 35 months; however, two have developed other cardiac lesions that suggest Marfan syndrome. A review of 200 consecutive third trimester fetal ultrasounds suggests an incidence of DAA of 1.5%. CONCLUSIONS: Ductus arteriosus aneurysm likely develops in the third trimester perhaps due to abnormal intimal cushion formation or elastin expression. Although it can be associated with syndromes and severe complications, many affected infants have a benign course. Given the potential for development of other cardiac lesions associated with connective tissue disease, follow-up is warranted.

Hemolytic anemia after atrioventricular septal defect repair without synthetic material.

We report a rare case of severe hemolytic anemia following repair of a congenital heart defect without the use of prosthetic material. A review of the literature, diagnosis, and management are described. Although this is an unusual complication following congenital heart surgery, a high index of suspicion must be maintained and a possible mechanical cause should be sought and corrected.

Single-stage repair of aortic arch obstruction and associated intracardiac defects with pulmonary homograft patch aortoplasty.

OBJECTIVE: Intracardiac malformations associated with coarctation and aortic arch hypoplasia have traditionally been repaired in 2 stages, with a high mortality rate. We review our experience with single-stage biventricular repair of intracardiac defects associated with aortic arch hypoplasia by means of pulmonary homograft patch aortoplasty. METHODS: Between October 1988 and October 1997, 39 of 40 consecutive patients underwent single-stage biventricular repair for aortic arch obstruction and associated intracardiac defects. The median age at operation was 17 days and the mean weight was 3.71 +/- 1.09 kg. Nineteen patients had either dextrotransposition of the great arteries or the Taussig-Bing anomaly. Sixteen patients had multiple left-sided obstructive lesions (2 cases of critical aortic stenosis, 3 of subaortic stenosis and ventricular septal defect, and 11 of hypoplastic left heart complex). One patient had an associated complete atrioventricular septal defect. Four patients had only an associated ventricular septal defect. Through a median sternotomy, the hypoplastic aortic arch was enlarged with a pulmonary homograft patch in 36 patients. In 4 patients an extended end-to-end anastomosis was performed. RESULTS: There were 2 early deaths (5%) and 2 late deaths (5%). One late death was not cardiac related. The mean follow-up time was 36 months (range 1 month-9 years). The recoarctation rate after pulmonary homograft patch aortoplasty was 8. 3%, but after exclusion of those patients with associated left-sided obstructive lesions this decreased to 0%. No aneurysm formation in the aorta has occurred. The actuarial survival at 8 years is 89% +/- 10%. CONCLUSIONS: Single-stage biventricular repair of aortic arch obstruction and associated intracardiac defects can achieve excellent survival. We recommend pulmonary homograft patch aortoplasty because it achieves complete relief of anatomic afterload with a tension-free anastomosis and low incidence of recoarctation.

Biventricular repair in neonates with hypoplastic left heart complex.

BACKGROUND: Multiple obstructions in the left heart-aorta complex have been associated with poor survival. No consensus exists as to whether these patients will have a favorable outcome with biventricular repair where most advocate a univentricular approach. METHODS: Since late 1988, all 11 neonates seen with hypoplastic left heart complex, which includes aortic arch obstruction, underwent biventricular repair. All patients had antegrade aortic flow and no intrinsic aortic or mitral stenosis. Elimination of the extracardiac afterload was achieved by extensive ascending aorta and aortic arch reconstruction with a pulmonary homograft patch. All intracardiac shunts were eliminated to fully preload the left heart. The median age at first operation was 7 days and the mean weight, 3.59+/-0.49 kg. The echocardiographic variables used to evaluate the left heart-aorta complex were reviewed, and the preoperative and postoperative measurements were compared. RESULTS: There were two early deaths. Four patients had six reoperations for left ventricular outflow tract obstruction, 2 of whom have required prosthetic valve replacement (1, aortic and mitral; 1, aortic), and 2 patients had three reoperations for recurrent coarctation. There was one late death at 3 years from pulmonary hypertension. Mean follow-up was 44+/-35 months. The 8 current survivors are all in New York Heart Association class I or II. The actuarial survival rate at 8 years is 63%, and the freedom from reoperation at 3 years is 25%. CONCLUSIONS: We have successfully achieved biventricular repair in most of the patients with hypoplastic left heart complex, a subset of patients with hypoplastic left heart syndrome. Some growth of the left ventricular structures was already observed at the time of hospital discharge. However, reoperation, particularly for left ventricular outflow tract obstruction, appears likely. Increasing experience will more accurately define predictive criteria for the feasibility of biventricular repair.

Congenital heart defects in Sotos syndrome.

Sotos syndrome is a relatively common overgrowth syndrome with characteristic physiognomy. We report on 3 patients with congenital heart defects out of 14 Sotos syndrome patients studied clinically and or by echocardiography. Review showed another 17 patients with variable cardiac defects, mostly closure defects, making an overall incidence of approximately 8%.

One-stage midline unifocalization and complete repair in infancy versus multiple-stage unifocalization followed by repair for complex heart disease with major aortopulmonary collaterals.

BACKGROUND: Patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries have traditionally required multiple unifocalization staging operations before undergoing complete repair. Recently, the feasibility of a single-stage unifocalization and repair was demonstrated by Hanley. In this report, we describe our experience with each approach. METHODS AND RESULTS: Since 1989, 11 of 12 patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries have undergone complete surgical correction. The first seven patients were subjected to staged bilateral unifocalizations, with repair being achieved in six (group I). The last five patients have undergone a single-stage midline unifocalization and repair via a sternotomy (group II). Four of these were infants (2 weeks to 9 months) and one was 13 years old. All patients in group I had tetralogy of Fallot, whereas in group II three patients had tetralogy of Fallot, one patient had double-outlet right ventricle, and one patient had complete atrioventricular canal and transposition. In group I, the median age at the first operation was 43 weeks. Complete repair was performed at a median age of 3.5 years, with a mean number of 3.3 operations required. In group II, only one operation was required to achieve complete repair at a median age of 28 weeks. The postoperative right ventricular/left ventricular pressure ratio was 0.49 in group I and 0.45 in group II. One intraoperative death and one late death occurred in group I and no early or late deaths in group II. Currently, four patients in group I and all five patients in group II are alive and well. CONCLUSIONS: Early intervention with both surgical approaches can lead to complete biventricular repair in most patients. Because the single-stage midline unifocalization and repair can achieve a completely repaired heart in infancy with one operation, it is currently our approach of choice.

Effect of inflammation on the rabbit hepatic cytochrome P-450 isoenzymes: alterations in the kinetics and dynamics of tolbutamide.

In order to determine the effect of inflammation on the kinetics and dynamics of tolbutamide, two groups of seven and nine New Zealand rabbits received 50 mg/kg tolbutamide before and 48 hr after the production of an inflammatory reaction generated by the s.c. administration of turpentine in both hind legs. Tolbutamide in plasma and its two major metabolites (hydroxytolbutamide) and carboxytolbutamide in urine were assayed by high-performance liquid chromatography. The influence of inflammation on hepatic cytochrome P-450 was assessed by 1) determining the hepatic concentration in cytochrome P-450 and b5, 2) characterizing the activity of tolbutamide hydroxylase, 3) isolating hepatic microsomal protein bands by sodium dodecyl sulfate-polyacrylamide gel electrophoresis and 4) measuring the concentrations of hepatic cytochrome P-450 isozymes LM-2 and LM-3c. The inflammatory reaction induced a marked decrease in tolbutamide total body clearance, secondary to a reduction in its metabolic clearance. Concerning the metabolites, hydroxytolbutamide metabolic rate constant and the fraction of the dose recovered in urine as carboxytolbutamide were diminished. Tolbutamide hypoglycemic response was not significantly affected by the inflammatory process. The Vmax of tolbutamide hydroxylase was reduced from 14.6 +/- 2.3 to 5.6 +/- 1.4 nmol/mg/60 min (P less than .05), and the Km remained unchanged. The concentration of hepatic cytochrome P-450 was reduced in turpentine-treated rabbits, whereas the cytochrome b5 concentration remained the same in both groups. The systemic inflammation also reduced the content in the 48, 52, 54 and 60 kDa protein bands from hepatic microsomes and the concentration of the LM-3c form.(ABSTRACT TRUNCATED AT 250 WORDS)

Effect of congestive heart failure on the intrinsic metabolic capacity of the liver in the dog.

The intrinsic metabolic capacity of the liver at end-stage heart failure in the pacing overdrive dog model of congestive heart failure was evaluated ex vivo. Congestive heart failure was induced in seven adult mongrel dogs (20-30 kg) by cardiac electrical pacing at a frequency of 240 stimuli/min until the development of overt heart failure; seven other dogs served as controls. The animals were then anesthetized and the right ventricular papillary muscles and samples from the left lateral hepatic lobes were collected. The degree of myocardial dysfunction as well as the total amount and the activities of cytochromes P-450 were evaluated. Tension, maximum rate of tension rise, and Vmax were significantly lower (40-60%) in the paced than in the control dogs, indicating a marked myocardial dysfunction. Moreover, significant decreases in total cytochrome P-450 (0.31 +/- 0.04 vs. 0.53 +/- 0.03 nmol/mg of microsomal protein, p less than 0.01) and in the intensity of four different electrophoretic protein bands (molecular masses of 46, 48, 50, and 59 kDa) occurred in the dogs with congestive heart failure. The decrease in total cytochrome P-450 was accompanied by a significant reduction in aminopyrine N-demethylase activity (1.74 +/- 0.25 vs. 2.91 +/- 0.40 nmol/min/mg of microsomal protein, p less than 0.05). Immunoblot analysis using antibodies to two different dog liver phenobarbital-inducible cytochromes P-450 demonstrated that PBD-1 (a P-450IIIA) was not affected by congestive heart failure, whereas PBD-2 (a P-450IIB) was markedly decreased.(ABSTRACT TRUNCATED AT 250 WORDS)

The importance of congenital hypertrophy of the retinal pigment epithelium in familial adenomatous polyposis.

We describe a family with familial adenomatous polyposis (FAP) and congenital hypertrophy of the retinal pigment epithelium (RPE). Three of five members with FAP showed flat, well-demarcated, round to oval pigmented patches of congenital hypertrophy of the RPE. We stress the importance of congenital hypertrophy of the RPE as a clinical marker in identifying patients with FAP since they are at risk for cancer.