ABSTRACT:We studied 34 Spanish children with hypomelanosis of Ito. This disease has an incidence of 1 per 1000 new patients consulting a paediatric neurological service, or 1 per 8000-10,000 unselected patients in a children's hospital. About 94% of our patients show noncutaneous abnormalities. Mental retardation (IQ below 70) was present in 64.7%; another 14.7% had an IQ between 70 and 90, usually associated with poor school performance. Four children exhibited autistic behaviour. Seizures of various types were present in 53% of cases. Other skin alterations in addition to the typical hypomelanosis were observed in 38% of our cases: caf6-au-lait spots, angiomatous nevi, nevus marmorata, nevus of Ota, Mongolian blue spot, heterochromia of the iris or hair, and other nonspecific pigmentations. Other associated disorders occur inconsistently and include macrocephaly, microcephaly, h6mihypertrophy, kyphoscoliosis, coarse facial features, genital anomalies, inguinal hernia, congenital heart disease, hypertelorism, and abnormalities of the teeth, feet and eyes. Autosomal dominant inheritance is demonstrated in some but not all cases.
Three histologically verified cases of cystic cavernous angioma of the posterior fossa are reported. The clinical history of these patients is presented, and the diagnostic and surgical problems arising from the extreme rarity of the combination of cerebellar site and cystic nature of the lesion are discussed.
Gadolinium-diethylenetriaminepenta-acetic acid (Gd-DTPA) is a chelated paramagnetic contrast agent under clinical trial for use in magnetic resonance (MR) imaging. The increased signal intensity following the intravenous infusion of contrast medium may improve the ability of MR imaging to delineate tumors. The use of this method in 15 pediatric patients with suspected brain-tumor recurrence was analyzed. All 15 patients underwent postoperative Gd-DTPA-enhanced MR imaging, and residual tumor was demonstrated in nine of them. Based on the findings of the enhanced MR studies, four patients had additional surgery, two underwent radiation therapy, and one was given immunotherapy. Continued surveillance was recommended for the remaining eight patients. In all cases the enhanced MR imaging studies were superior to the unenhanced studies in regard to the qualitative and quantitative assessment of the residual tumor. Gadolinium-DTPA-enhanced MR imaging appears to be a safe and effective means of providing an accurate postoperative assessment of residual disease in pediatric brain-tumor patients. It is as effective as contrast-enhanced computerized tomography and has the sensitivity and anatomic resolution provided by MR imaging. The most useful role of this agent was in the postoperative period, in assessing the adequacy of surgical resection. This technique is recommended as the procedure of choice in the postoperative assessment and long-term surveillance of patients with brain tumors.
A prospective study was conducted in 15 pediatric patients who had undergone resection of intracranial tumors. The object of the study was to determine the safety and efficacy of magnetic resonance (MR) imaging performed after the administration of gadolinium diethylenetriamine-pentaacetic acid (Gd-DTPA) in evaluating residual or recurrent tumor. Precontrast T1-weighted, intermediate, and T2-weighted images were obtained at a field strength of 1.5 T. Gd-DTPA was then injected intravenously in a dose of 0.1 mmol per kilogram of body weight. T1-weighted images were obtained within 5 minutes after the injection, intermediate and T2-weighted images were obtained 10 minutes after the injection, and T1-weighted images were obtained approximately 20 minutes after the injection. None of the patients experienced allergic reactions or other side effects. Physical examination findings and laboratory values were unchanged after the Gd-DTPA-enhanced examination. In six patients, contrast-enhanced images depicted tumor not suspected on nonenhanced images. In four other patients, enhanced images provided better definition of the tumor core. The images of one patient with a brain stem tumor showed no evidence of enhancement. Pre- and postcontrast images of three previously treated patients showed no evidence of tumor. Gd-DTPA appears to be a safe and effective contrast agent for MR imaging and provides a more accurate method of imaging in the follow-up of brain tumors in pediatric patients.
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