Imaging considerations of central nervous system manifestations in pediatric patients with neurofibromatosis type 1.

CT and MRI were used in a prospective study of the central nervous system (CNS) manifestations in 41 consecutive children with neurofibromatosis type 1 (NF-1). Gadolinium-DTPA was used in 15 patients. MRI was more effective than CT in delimiting the extension of the optic pathway glioma and in evaluating associated cerebral malformations. MRI visualized lesions generally undetected by CT, in the form of iso- or hyperintense foci with respect to the cerebral cortex in T2-weighted sequences. Well-delimited lesions of high signal intensity were observed in the globus pallidus (22 cases), the internal capsule (6 cases), corpus callosum (2 cases), anterior commissure (1 case) and semioval center (2 cases). Poorly defined hyper- or isointense areas were also observed affecting the cerebellar white matter (21 cases) and brain stem (17 cases). None of these lesions showed Gadolinium-DTPA enhancement, and were of no clinical significance. MRI has displaced CT in the initial diagnosis of patients with NF-1. Periodic annual MRI controls are only justified in patients with MRI changes to evaluate the progression or stabilization of the lesions.

Fine-needle aspiration biopsy in children: experience in 70 cases.

Results of 70 fine-needle aspiration biopsies (FNAB) were evaluated retrospectively in 61 pediatric patients. Over a period of 9 months all mass lesions suspected being malignant were aspirated. Twelve of the 70 aspirations were performed in children having known tumours, in order to exclude recurrence or metastasis. The others were carried out to obtain a diagnosis. Satisfactory specimens were obtained from 58 (83%). There were 21 benign diagnoses, 36 malignant diagnoses, and 1 with suspected malignancy. Correlation of histologic and cytologic diagnoses was possible in 45 cases. The diagnostic sensitivity and specificity were 95% and 80%, respectively. We have found FNAB more accurate in the diagnosis of malignancies than in benign lesions. The results suggest that this is a useful technique for obtaining a first diagnosis of malignancy, as well as for excluding recurrence or metastatic disease.

Radiological Diagnosis of Periventricular and Subcortical Leukomalacia.

Nine newborn infants with histories of perinatal asphyxia are presented. The pneumoencephalographic findings which led to the diagnosis are typical and constant. They include marked subcortical atrophy with rounded, dilated, and undisplaced lateral ventricles. Cystography with 3 cc of air demonstrated multiple subcortical and pareventricular cavities, without communication with the ventricular system, but with the typical honeycomb appearance of paraventricular and subcortical leukomalacia described in postmortem findings. The CT findings are typical, and provide the location of the cavities as well as their density.

The wide spectrum of the asphyxiating thoracic dysplasia.

Seven cases of A. T. D. are presented. Radiological findings were extraordinarily diverse. The prognosis of the disease is difficult for each individual case, because of the frequent pulmonary complications and cystic renal lesions are not always directly related to the severity of the bone changes. The authors believe that the term "thoracic-pelvic-phalangeal dysplasia" proposed by Langer, is the most adequate, since it points to the key factors in the diagnosis of the disease.

Intracranial calcification in a neonate with the Sturge Weber syndrome and additional problems.

The neonate in this report had severe encephalotrigeminal angiomatosis with intracranial calcification, cranial hemiatrophy, microcephaly and generalised severe cerebral atrophy. Such findings are not common in the newborn with this syndrome.

[Cavernous hemangioma of the liver (author's transl)]. / Hemangioma cavernoso hepático.

A three-month old asymptomatic infant was incidentally found to have an abdominal mass. Through standard radiological and vascular procedures it was defined as being of hepatic origin, vascular etiology and of benign prognosis. Differences among cavernous hemangioma, hepatoma, metastasis and hemangio-endothelioma are summarized. The importance of angiography is emphasized as an essential procedure previous to the surgical evaluation and therapy.