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.
Cardiac Catheterization* ; Cardiac Catheters* ; Cardiovascular Diseases* ; Child* ; Cineangiography* ; Heart Defects, Congenital ; Humans

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

The congenital cystic adenomatoid malformation (CCMA) of the lung is a rare variant of congenital cystic lung disease consisted of one or usually multiple interconnecting cysts in the pulmonary parenchyma lined by cuboidal and columnar epithelium. This diease present clinically in three ways: 1) stillborn or perinatal death, 2)progress respiratory distress in the newborn, and 3)acute and chronic pulmonary infections in the older infant and child. The onset of symptoms, which are cyanosis, tachypnea, and other forms of respiratory distress, usually occurs at or shortly after birth, This manifestations are related to compression of the remained normal ung by expansion of the cysts. We have experienced a case of congenital cystic adenomatoid malformation of the lung in a 1-day-old male infant who had tachypnea. A right upper lobectomy was done with satisfactory postoperative courses clinically and radiologically at 8 months of age. A brief review of the related literature is presented.
Child ; Cyanosis ; Cystic Adenomatoid Malformation of Lung, Congenital* ; Epithelium ; Humans ; Infant ; Infant, Newborn ; Lung Diseases ; Lung* ; Male ; Parturition ; Tachypnea

Lung agensis is a rare developmental anomaly. It can range from total bronchial and parechymal agensis to mild pulmonary parenchymal hypoplasia of one or both lungs. A case of lobar agenesis of the left upper lung in a 15-year-old boy is presented. The patient had mild exertional dyspnea. Pulmonary angiography revealed the absence of the left upper pulmonary artery and vein. Bronchography showed no branching of bronchus to the left upper lobe. Intravenous pyelography revealed incomplete duplication of the right urinary tract.
Abnormalities, Multiple/*radiography ; Adolescent ; Bronchography ; Cardiac Catheterization ; Dyspnea/etiology ; Heart Murmurs/etiology ; Humans ; Lung/*abnormalities ; Male ; Pulmonary Artery/*abnormalities/radiography ; Pulmonary Veins/*abnormalities ; Tomography, X-Ray Computed ; Urinary Tract/*abnormalities ; Urography

PURPOSE: Joubert syndrome presents neonatal respiratory abnormalities and other clinical manifestations. Pathologically the patients show hypoplasia or agenesis of cerebellar vermis and other intracranial anomalies. Our purpose is to evaluate the clinical manifestations and MR findings of Joubert syndrome. MATERIALS AND METHODS: Among the patients presenting with clinical stigmata of Joubert syndrome and agenesis of vermis on MR imaging, eight patients who did not satisfied the criteria of Dandy-Walker malformation, tectocerebellar dysraphia and rhombencephalosynapsis were selected. MR findings and clinical manifestation were analyzed. RESULTS: On MR imaging, agenesis of the cerebellar vermis (all cases), hypoplasia of the cerebellar peduncle (6cases), fourth ventricular contour deformity(6cases), tentorial elevation (4cases), deformity of the lateral ventricles (4cases), dysgenesis of the straight sinus (3cases) were demonstrated. Other findings were abnormalities of corpus callosum (3cases), falx anomalies (3 cases), occipital encephalomeningocele (2 cases) and fluid collection in posterior cranial fossa (2cases). Clinical manifestations were developmental delay (5cases), abnormal eyeball movement (3cases), hypotonia (2 cases), neonatal rerspiratoy abnormality (2cases), etc. CONCLUSION: Joubert syndrome showed various clinical manifestations and intracranial anomalies. MR imaging is an useful modality in detection of the cerebellar vermian agenesis and other anomalies of the patients.
Christianity ; Congenital Abnormalities ; Corpus Callosum ; Cranial Fossa, Posterior ; Dandy-Walker Syndrome ; Humans ; Lateral Ventricles ; Magnetic Resonance Imaging* ; Muscle Hypotonia

Multiple pterygium (Escobar) syndrome is a rare disorder manifested by growth retardation, facial and genital anomalies, and widespread musculo-skeletal deformities. This disorder was originally described by Bussiere in 1902. And then in 1976, Gorlin et al. described an autosomal recessive disorder characterized by congenital arthrogryposis, skin webs across joints and unusual faces. It was fully delineated as a distinct entity by Escobar et al. in 1978 and an alternative name, Escobar syndrome. In this report, we present an multiple pterygium syndrome that manifested by arthrogryposis, pterygium in shoulder, elbow, wrist, severe deviation of lower leg and ankle, webbed neck, microphthalmia, low set ears, small mouth and high arched palate, and genital anomalies.
Ankle ; Arthrogryposis ; Congenital Abnormalities ; Ear ; Elbow ; Joints ; Leg ; Microphthalmos ; Mouth ; Neck ; Palate ; Pterygium* ; Shoulder ; Skin ; Wrist

PURPOSE: To analyze the clinical and radiological manifestations of pulmonary infection in patients with AIDS. MATERIALS AND METHODS: We reviewed the medical records and analyzed retrospectively analysed the chestradiographs (n=24) and CT scans (n=11) of 26 patients with AIDS who had been followed up at our institute from1987 to June 1998. Pulmonary infections were confirmed by sputum smear and culture (n=18), pleuralexamination(n=3), bronchoalveolar lavage (n=3), autopsy (n=4), transbronchial lung biopsy (n=1) or clinicalhistory (n=9). The study group included 23 men and three women aged 25-54 (average 35.2) years. We correlated theradiologic findings with CD4 lymphocyte counts. RESULT: Pulmonary infections included tuberculosis (n=22),Pneumocystis carinii pneumonia (n=9), cytomegalovirus (n=3), and unidentified bacterial pneumonia (n=2).Radiologically pulmonary tuberculosis was classified as primary tuberculosis (n=11 ; mean CD4 counts: 41.3cells/mm3) and post-primary tuberculosis (n=11 ; mean CD4 counts: 251.3 cells/mm3). CT findings of tuberculosisincluded lymphadenitis (n=6), bronchogenic spread (n=5), large consolidation (n=4), esophago-mediastinal fistula(n=2), and cavity (n=1). Tuberculosis in AIDS responded rapidly to anti-TB medication with complete or markedresolution of lesions within three months. Radiologic findings of Pneumocystis carinii pneumonia included diffuseground glass opacities, cysts, and reticular opacities. CONCLUSION: Tuberculosis was the most common infection inpatients with AIDS in Korea, and this is attributed to the high prevalence of tuberculosis. Radiological findingsvaried with CD4+ cell count, showing those of primary tuberculosis as a patient's CD4+ cell count decreased.Pulmonary tuberculosis in AIDS responded rapidly to anti-Tb medication.
Autopsy ; Biopsy ; Bronchoalveolar Lavage ; CD4 Lymphocyte Count ; Cytomegalovirus ; Female ; Glass ; Humans ; Inpatients ; Korea ; Lung ; Lymphadenitis ; Male ; Medical Records ; Pneumonia ; Pneumonia, Bacterial ; Pneumonia, Pneumocystis ; Prevalence ; Retrospective Studies ; Sputum ; Tomography, X-Ray Computed ; Tuberculosis ; Tuberculosis, Pulmonary

PURPOSE:We performed this study to evaluate the clinical significance and temporal changes of pleural effusion developed after the resection of hepatoma. MATERIALS AND METHODS: We reviewed retrospectively follow-up chest radiographs of 97 patients who had undergone operation for hepatoma and had no radiologically demonstrable postoperative complications. The duration of pleural effusion was classified into five groups and the amount of pleural effusion at one week after operation was graded into four groups. Statistical significance of the relationship between the duration, amount of pleural effusion and five factors, which are location and size of tumor, age of the patients, methods of operation, and preoperative liver function, was studied respectively. RESULTS:Pleural effusion was developed in 63.9% (62/97) and the mean duration was 2.5 weeks. In 92% (52/56), pleural effusion disappeared spontaneously within four weeks. Patients who had hepatoma in upper portion of the right lobe developed more frequent pleural effusion which persisted longer, and was larger in amount at one week after operation(p<0.05). There were no statistically significant differences between pleural effusion and the other four factors. CONCLUSION:Pleural effusion following hepatoma surgery should not be regarded as a sign of post-operative complication, as it invariably disappears spontaneously within four weeks. Development of pleural effusion is considered to be caused by local irritation and disturbance of lymphatic flow at the diaphragm.
Carcinoma, Hepatocellular* ; Diaphragm ; Follow-Up Studies ; Humans ; Incidence* ; Liver ; Pleural Effusion* ; Postoperative Complications ; Radiography, Thoracic ; Retrospective Studies

Antiphospholipid syndrome is a disease producing vascular thrombosis with antiphospholipid antibody and usually associated with systemic lupus erythematosus. It is called primary antiphospholipid syndrome(PAPS) if it does not have the clinical features of collagen vascular disorder. Ocular manifestations of antiphospholipid syndrome include retinal vascular disorder, anterior ischemic optic neuropathy, and amaurosis fugax. Ocular manifestations are much less common in primary antiphospholiped syndrme than antiphospholipid syndrome associated with systemic lupus erythematosus. We experienced two cases of non-ischemic central retinal vein occlusion which were associated with primary anti phospholipid syndrome in two female patients, who complained sudden decrease of visual acuity in one eye. We report the cases with review of the literatures.
Amaurosis Fugax ; Antibodies, Antiphospholipid ; Antiphospholipid Syndrome* ; Collagen ; Female ; Humans ; Lupus Erythematosus, Systemic ; Optic Neuropathy, Ischemic ; Retinal Vein* ; Retinaldehyde ; Thrombosis ; Visual Acuity