Extensive central nervous system involvement in optic pathway gliomas in neurofibromatosis type 1

Optic pathway gliomas (OPG) in neurofibromatosis type 1 (NF1) usually remain localized to the anterior visual pathway. However, a small number can demonstrate widespread dissemination. We describe three children with NF1 OPGs and extensive central nervous system involvement. In one case, a postmortem examination revealed tumor cells extending continuously from the optic nerves to the conus medullaris. This is the most widespread NF1 OPG reported in the literature. We suggest that rapid visual deterioration in the absence of radiographic changes or increased intracranial pressure can be caused by increasing tumor infiltration within the central nervous system.     

Human herpesvirus 7 infection associated with central nervous system manifestations

The clinical features of infection with human herpesvirus 7 (HHV-7) are not well described. Exanthem subitum is the only illness that is confirmed to be caused by HHV-7. We report two children who had exanthem subitum associated with central nervous system manifestations. Two strains of HHV-7 were isolated sequentially from peripheral blood mononuclear cells and saliva of the same child who had exanthem subitum complicated with acute hemiplegia in childhood. Two strains were confirmed to be HHV-7 by means of monoclonal antibodies to human herpesvirus 6 (HHV-6) and HHV-7, polymerase chain reaction, and DNA analysis. During the convalescent period, the antibody titer to HHV-7 rose from less than 1:10 to 1:320, whereas the antibody titer to HHV-6 remained less than 1:10. Another child with exanthem subitum complicated by acute hemiplegia had serologic evidence of primary HHV-7 infection. These two cases demonstrate a new relationship between HHV-7 and central nervous system symptoms. (J PEDIATR 1996;129:301-5)     

Diagnosis and management of central nervous system infections in pediatric cancer patients

Infections of the central nervous system (CNS) are a common cause of neurological abnormalities in pediatric oncology patients apart from cancer disease of the CNS. Often symptoms do not present unequivocally, impeding a clear distinction from other differential diagnosis. Repeated neurological examinations of the patient allow selecting the necessary laboratory tests and imaging techniques. As delayed antiinfectious treatment is associated with high mortality and severe sequelae of survivors, patients do need empirical therapy using adequate doses until diagnostic results allow a more specific treatment. This article provides the recommendations of the Infectious Diseases Working Party of the German Society for Pediatric Infectious Diseases (DGPI) and the German Society for Pediatric Hematology/Oncology (GPOH) for diagnosis and treatment of infections of the CNS in immunocompromised patients.     

Renovascular hypertension in pediatric patients with neurofibromatosis

Vascular lesions in addition to pheochromocytoma are now recognized as causes of hypertension associated with neurofibromatosis. We have analyzed hospital charts of 106 pediatric patients with neurofibromatosis, eight of whom were noted to have been hypertensive on at least one occasion. Of these eight patients, five had vascular lesions involving the renal vasculature, one had obstructive uropathy and two had transient hypertension associated with traction and pain. Approximately 5% of pediatric patients with neurofibromatosis have vascular lesions causing hypertension.     

Characteristics of Lisch nodules in patients with neurofibromatosis type 1

PURPOSE: To assess the prevalence and location of Lisch nodules among patients with neurofibromatosis type 1. PATIENTS AND METHODS: This was a prospective observational case series in which 369 consecutive patients from a neurofibromatosis clinic participated. Predetermined inclusion and exclusion criteria were applied to these participants. The presence of Lisch nodules was determined by one of the authors (SMC). The percentage of patients with neurofibromatosis type 1 with Lisch nodules was calculated. Lisch nodules were characterized as being distributed superiorly, inferiorly, or equally in each eye. The percentage of eyes in each group was ascertained. RESULTS: Among patients of all ages with neurofibromatosis type 1, 63.2% had Lisch nodules, whereas 92% of postpubertal patients had Lisch nodules. Among eyes with Lisch nodules, 80% had an inferior distribution. CONCLUSIONS: This study corroborated previously reported data regarding the prevalence of Lisch nodules in patients with neurofibromatosis type 1 and found that Lisch nodules were predominantly located inferiorly on the iris. The authors propose that exposure to sunlight may be the mechanism for this distribution of hamartomatous nodules.     

Morbidity of second-look surgery in pediatric central nervous system tumors.

The extent of brain tumor resection affects survival. Second-look surgery (resection of residual tumor before radiographic progression) may improve survival by reducing the tumor burden, but the morbidity of the procedure is not known. On chart review of 280 patients with two or more brain tumor operations treated between January 1985 and June 1998, we identified 47 patients with second-look surgery. Lansky and Eastern Cooperative Oncology Group (ECOG) performance scores, as well as perioperative complications were recorded. There were 21 gliomas (6 malignant), 12 medulloblastomas, 3 craniopharyngiomas, 3 ependymomas and 8 miscellaneous tumors. Median time to second surgery was 50 days. Perioperative complications occurred in 45% of patients. There was no significant change in the mean Lansky and ECOG scores 4 and 24 weeks after surgery. Gross total resection (GTR) was achieved in 62% of patients and near total resection (NTR) in 23%, and 15% of patients had subtotal resection. GTR or NTR was achieved in 66% of medulloblastomas and 100% of gliomas. We conclude that second-look surgery by experienced pediatric neurosurgeons has an acceptable morbidity and should be considered in patients with residual tumors.     

Central nervous manifestations of neurofibromatosis in children (author's transl)]

Serious central nervous manifestation of neurofibromatosis have been reported in 11 children aged 1,5-12 years. According to the present literature we conclude: 1. Central nervous lesions of neurofibromatosis are common even in childhood. 2. About two thirds of these patients suffer from brain tumors. 3. The tumors involve the suprasellar region in many cases. 4. By this localisation endocrine manifestations, especially precocious puberty, are frequent.     

Imaging of the central nervous system in pediatrics and adolescence.

Magnetic resonance imaging (MRI) has taken its place beside computed tomography (CT) and ultrasonography in effective imaging of the central nervous system in infants and children. The major initial impact of MRI in pediatric neuroradiology has been the displacement or replacement of the more expensive and invasive procedures such as angiography, myelography, cisternography, and ventriculography. Ultrasonography remains the screening procedure for fetal and infant central nervous system abnormalities, whereas CT continues to be an effective brain screening technique after loss of the ultrasonography windows with growth of the child. The better the clinical information transmitted to the radiologist in advance of imaging of the patient, the more likely the clinician will receive valuable information in return.     

Spectrum of renal findings in pediatric fibromuscular dysplasia and neurofibromatosis type 1

Background  

Fibromuscular dysplasia (FMD) and neurofibromatosis type 1 (NF1) are the most common causes of pediatric renal artery stenosis (RAS) in western countries, and characterization of their angiographic features could aid in an accurate diagnosis and in treatment.     

Rib head protrusion into the central canal in type 1 neurofibromatosis

Background

Intraspinal rib head dislocation is an important but under-recognized consequence of dystrophic scoliosis in patients with neurofibromatosis 1 (NF1).

Objective

To present clinical and imaging findings of intraspinal rib head dislocation in NF1.

Materials and methods

We retrospectively reviewed clinical presentation, imaging, operative reports and post-operative courses in four NF1 patients with intraspinal rib head dislocation and dystrophic scoliosis. We also reviewed 17 cases from the English literature.

Results

In each of our four cases of intraspinal rib head dislocation, a single rib head was dislocated on the convex apex of the curve, most often in the mid- to lower thoracic region. Cord compression occurred in half of these patients. Analysis of the literature yielded similar findings. Only three cases in the literature demonstrates the MRI appearance of this entity; most employ CT. All of our cases include both MRI and CT; we review the subtle findings on MRI.

Conclusion

Although intraspinal rib head dislocation is readily apparent on CT, sometimes MRI is the only cross-sectional imaging performed. It is essential that radiologists become familiar with this entity, as subtle findings have significant implications for surgical management.