The prognosis of children with hydrocephalus and congenital heart disease.

The association of congenital hydrocephalus and heart disease in children is infrequent, but may present considerable dilemmas in management. This report describes the treatment and prognosis of 11 children with both clinical problems. There were 5 males and 6 females. Hydrocephalus occurred following aqueductal stenosis in 5 children and the Dandy-Walker malformation in 3. Three children were diagnosed with idiopathic hydrocephalus. Ten children underwent cerebrospinal fluid diversion procedures for control of hydrocephalus. Five children received pharmacological therapy for cardiac disease; 4 children required surgical correction. Two children died from medical conditions; 2 families declined treatment. Follow-up from 2 to 7 years in the remaining 7 children demonstrated moderate or severe neurodevelopmental disability in 5. One child at 2 years of age showed borderline developmental disability while 1 child is developing normally at 10 years of age. Overall the occurrence of symptomatic hydrocephalus and heart disease in the perinatal period resulted in mortality or neurodevelopmental disability in 9/11 children.

Primary intraosseous meningioma. Case report.

Primary intraosseous meningioma of the skull is an uncommon lesion often confused preoperatively with a primary bone tumor of the skull. The case of an intraosseous meningioma without dural connection or association with a cranial suture is reported that was radiographically diagnosed as Paget's disease and initially treated conservatively. Persistent headache prompted a biopsy, yielding a benign, heavily ossified, and psammomatous meningioma. The lesion was treated with wide surgical excision and acrylic cranioplasty. Subsequent review of the literature has revealed 35 additional cases of purely intraosseous meningioma. An analysis of these 36 cases demonstrates a mean patient age at diagnosis of 45 years and a 2:1 female preponderance, with the majority of lesions associated with cranial sutures. Sixty-four percent of the lesions were hyperostotic on plain skull x-ray films; the rest were osteolytic or a mixture of both. There was no relationship to prior trauma. The treatment of choice is wide surgical resection followed by cranial reconstruction.

Local skin burn causes systemic (lung and kidney) endothelial cell injury reflected by increased circulating and decreased tissue factor VIII-related antigen.

Inasmuch as xanthine oxidase (XO)-derived O2* metabolites may contribute to vascular endothelial injury and Factor VIII antigen (F8Ag) is a component of endothelial cells, we hypothesized that XO-derived O2* might damage and cause distant organ endothelial cells to release F8Ag in rats subjected to skin burn. We found that serum F8Ag (ELISA) increased in the blood of rats subjected to skin burn (70 degrees C water to shaved dorsal skin for 30 seconds) but not in sham control rats (30 degrees C water). Coincidentally, F8Ag levels also decreased in lung and kidney tissue sections (immunofluorescent staining) of burned rats but not sham rats. Increases in circulating F8Ag levels and decreases in tissue F8Ag levels appeared to result from XO-derived O2* metabolites: F8Ag levels did not increase in the blood and did not decrease in the tissues of rats pretreated with allopurinol (a specific XO inhibitor, 50 mg/kg) or dimethylthiourea (DMTU) (a permeable O2* metabolite scavenger, 250 mg/kg). Lung injury as assessed by permeability studies (I125-albumin leak) paralleled changes in blood F8Ag levels in sham, burn, allopurinol-, and DMTU-treated groups. We conclude that skin burn causes a systemic vascular injury that can be inhibited by allopurinol or DMTU and is reflected by increased circulating and tissue decreased Factor VIII antigen levels. Release of Factor VIII antigen may serve as a valuable marker of distant organ injury in patients with skin burn.