The Dandy-Walker syndrome is a developmental disorders of the brain characterized by cystic deformity of the 4th ventricle and agensis of the cerebellar vermis. Other systemic anomalies and chromosomal abnormalities are associated with this syndrome. We are experienced a case in a 9 months old male infant who presented initially with frequent vomiting, low birth weight, On the physical examination, a prominent occiput, palpable mass below the right upper quadrant, pulmonary valve stenosis, congenital dislocation of the hips, ventral flexion of fingers, clubfoots and the rocker-bottom deformities of feet were present. On the chromosomal study, there were chromosomal polymorphisms in a thickened C-band of chromosome No. 1 by C-banding method. The brain CT revealed a large, thin-walled, low density mass of CSF without enhancement in the posterior fossa, showing upward displacement of cerebellar hemisphere with absent inferior vermis(or associated with dysplastic cerebellar hemisphere). A brief review of the related literatures were included in this report.
Brain ; Chromosome Aberrations* ; Clubfoot ; Congenital Abnormalities ; Dandy-Walker Syndrome* ; Dislocations ; Fingers ; Foot ; Hip ; Humans ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Male ; Physical Examination ; Pulmonary Valve Stenosis ; Vomiting

No abstract available.
Epidermal Cyst*

We report two patients with Hallervorden-Spatz disease, who were diagnosed by same MR findings of marked low signal intensity in the globus and substantia nigra. They presented with ataxic and spastic gait, intention tremor, delayed mental development, and dysarthria. They were 7 year-old male and 8 yea r-old female siblings, who were healthy until 3 years of age when they suffered from progressive symptoms. T2-weighted images showed marked low signal intensity in the globus pallidus and substantia nigra indicating an increased irondeposition, and it might suggest Hallervorden-Spatz disease.
Child ; Dysarthria ; Female ; Gait Disorders, Neurologic ; Globus Pallidus ; Humans ; Male ; Pantothenate Kinase-Associated Neurodegeneration* ; Siblings* ; Substantia Nigra ; Tremor

PURPOSE: Pediatric sedation is an important factor for obtaining the images of good quality. We performed this study to analyze the efficacy of our sedation protocol using chloral hydrate. MATERIALS AND METHODS: We collected prospectively 151 sedation records of children(1 day-15 years old), who were sedated with chloral hydrate for MR(n=112) or CT(n=39) studies. We initially administered 50mg/Kg orally(n=94) or rectally(n=57) 30 minutes before the scheduled examinations, and then administered additionali dose (second dose :25-35mg/Kg, third dose:10-15mg/Kg) to patients whom initial dose failed to sedate. RESULTS: Satisfactory sedation was achieved by initial administration in 109 patients(72%) without si difference between oral(per oral: P.O.) and rectal(per rectal: P.R.) administration. Second dose was required in 28% and third dose in 5%. MR and CT examinations required second dose in 36(32%) and 6 patients(15%), respectively. P.O. -patients vomited in 5%. P.R. -patients defecated in 22% after initial administration. There were no other serious complications. Time interval from the drug administration to the start of examinations was 33 minutes in initial-dose-group and 64 minutes in additional-dose-group. Two patients could not complete MR examination due to early arousal. Prolonged sedation, requiring more than 30 minutes for alertness after MR and CT examinations, was encountered in twenty(18%) and two patients(5%), respectively. CONCLUSION: Our protocol using chloral hydrate(P.O. or P.R.) is thought to be an effective and safe method for pediatric sedation for MR or CT imaging.
Arousal ; Child* ; Chloral Hydrate* ; Humans ; Prospective Studies

No abstract available.
Trichothiodystrophy Syndromes*

Bile* ; Choledochal Cyst* ; Humans ; Infant* ; Peritonitis*

PURPOSE: Bilateral high signal intensity in basal ganglia on Tl-weighted images is unusual. the purpose of this study is to describe the pattern of high signal intensity and underlying disease. METHODS AND MATERIALS: During the last three years, 8 patients showed bilateral high signal intensity in basal ganglia on Tl-weighted image, as compared with cerebral white matter. Authors analized the images and underlying causes retrospectively. Of 8 patients, 5 were male and 3 were female. The age ranged from 15 days to 79 years. All patient were examined by a 0.5T superconductive MRI. Images were obtained by spin echo multislice technique. RESULTS: Underlying causes were 4 cases of hepatopathy, 2 cases of calcium metabolism disorder, and one case each of neurofibromatosis and hypoxic brain injury. These process were bilateral in all cases and usually symmetric. In all cases the hyperintense areas were generally homogenous without mass effect or edema, although somewhat nodular appearence was seen in neurofibromatosis. Lesions were located in the globus pallidus and internal capsule in hepatopathy and neurofibromatosis, head of the caudate nucleus in disorder of calcum metabolism, and the globus pallidus in hypoxic brain injury. CONCLUSION: Although this study is limited by its patient population, bilateral hyperintense basal ganglia is associated with various disease entities. On analysis of hyperintense basal ganglia lesion, the knowledge of clinical information improved diagnostic accuracy.
Basal Ganglia* ; Brain ; Brain Injuries ; Calcium Metabolism Disorders ; Caudate Nucleus ; Edema ; Female ; Ganglia ; Globus Pallidus ; Head ; Humans ; Internal Capsule ; Magnetic Resonance Imaging ; Male ; Metabolism ; Neurofibromatoses ; Retrospective Studies

Differentiation between benign and malignant hepatic lesions was attempted according to the shape of the lesion, internal architecture and characteristics of signal intensity on MR images. MR images. We retrospectively analyzed 20 cases of hemangiomas, 12 cases of hepatomas and 5 cases of cysts which were diagnosed by clinical, radiological and surgical methods (33 patients). On T1-weighted, proton weighted, and T2-weighted images, most hemangiomas (70%) and cysts (100%) had smooth margin and were round or oval in shape, while hepatocellular carcinomas had irregular (67%) or lobulated (33%) borders. Ninety percent of hemangiomas and 100% of cysts had homogeneous signal intensity, but 67% of hepatocellular carcinomas were inhomogeneous. Signal intensities of hepatocellular carcinomas were more variable. However, hemangionmas were isointense or hyperintense on proton weighted images, and markedly hyperintense on T2-weighted images . In conclusion, inhomogenous, irregular border, and variable signal intensity lesions had high likelihood of malignancy, while homogenous and markedly hyperintense lesions were more likely benign.
Carcinoma, Hepatocellular ; Diagnosis* ; Hemangioma ; Magnetic Resonance Imaging* ; Protons ; Retrospective Studies

Differentiation between benign and malignant hepatic lesions was attempted according to the shape of the lesion, internal architecture and characteristics of signal intensity on MR images. MR images. We retrospectively analyzed 20 cases of hemangiomas, 12 cases of hepatomas and 5 cases of cysts which were diagnosed by clinical, radiological and surgical methods (33 patients). On T1-weighted, proton weighted, and T2-weighted images, most hemangiomas (70%) and cysts (100%) had smooth margin and were round or oval in shape, while hepatocellular carcinomas had irregular (67%) or lobulated (33%) borders. Ninety percent of hemangiomas and 100% of cysts had homogeneous signal intensity, but 67% of hepatocellular carcinomas were inhomogeneous. Signal intensities of hepatocellular carcinomas were more variable. However, hemangionmas were isointense or hyperintense on proton weighted images, and markedly hyperintense on T2-weighted images . In conclusion, inhomogenous, irregular border, and variable signal intensity lesions had high likelihood of malignancy, while homogenous and markedly hyperintense lesions were more likely benign.
Carcinoma, Hepatocellular ; Diagnosis* ; Hemangioma ; Magnetic Resonance Imaging* ; Protons ; Retrospective Studies

No abstract available.
Thyroid Gland* ; Thyroid Nodule*