Fabry disease presenting with multiple hemorrhagic cerebral infarction. A case report.

We describe a 57-year-old woman, a heterozygote for Fabry disease who had multiple hemorrhagic cerebral infarctions. Her clinical course and radiological findings suggested cardiogenic cerebral embolus, but distinction from multiple cerebral infarction associated with Fabry disease seemed necessary. Our present case is reported with reference to the literature to introduce various types of stroke, which can develop in patients with Fabry disease.

High-flow Neonatal Macrocerebral Arteriovenous Fistulas in Hereditary Hemorrhagic Telangiectasia.

Although some cases of vein of Galen aneurysmal malformation (VGAM) present initial clinical symptoms such as cardiopulmonary disturbance in the neonatal period, pial arteriovenous fistula is very seldom present as a clinical symptom immediately after birth. A neonatal patient, the first-born to his family, presented with tachypnea postpartum. This baby had a family history of hereditary hemorrhagic telangiectasia. A cerebral MR image revealed multiple macrocerebral arteriovenous fistulas (MCAVFs), resulting in a large partially thrombosed venous pouch within the cerebral cortex. Trans-arterial embolizations of the main two fistulas were performed using N-butyl cyanoacrylate (NBCA) with tantalum powder six months after birth. Post-embolization angiography confirmed the obliteration of the fistulas and magnetic resonance imaging (MRI) revealed thrombosis and reduction in size of the venous component. His tachypnea disappeared completely. There were no neurological complications due to the treatment. The prognosis of multiple MCAVFs mainly depends on the presence of medullar signs and symptoms and a delay before treatment. Pure glue endovascular intervention, as used in our case, is considered to be the first therapeutic choice to decrease the risk of neurological consequences.

Development of diffusion-weighted image using a 0.3T open MRI.

The purpose of this study was to develop a new technique for diffusion-weighted MRI (DWI) with a low-field scanner. DWI is becoming important for assessment of acute stroke. Until recently DWI required expensive technology. We developed multishot-DWI sequence for 0.3T open type MR imager. We prospectively studied forty patients on this 0.3T MRI and compared this DWI to single-shot-DWI by 1.5T-MRI. Group A: Twenty-four patients with acute cerebral infarctions detected by 1.5T-DWI were re-examined using 0.3T-DWI within 24 hours. Sixteen patients with acute cerebral infarctions detected by 0.3T-DWI were re-examined using 1.5T-DWI within 24 hours. In 22 (92%) of 24 cases, 0.3T-DWI showed high signal. In the other two patients, motion artifact distorted 0.3T-DWI. Group B: In all 16 patients, all infarctions detected by 0.3T-DWI showed high signal on 1.5T-DWI. These preliminary data show that, as long as the patient is able to keep still, multishot-DWI can be acquired successfully on a 0.3T open type MRI system.

A case of adenocarcinoma with mixed subtypes displaying ground-glass opacity on high-resolution CT.

We report a case of adenocarcinoma with mixed subtypes with pleural dissemination and lymphatic permeation, although the CT results showed ground-glass opacity that led to the diagnosis of bronchioloalveolar carcinoma without foci of active fibroblastic proliferation.

Development of a subject-standing-type cone-beam computed tomography for chest and orthopedic imaging.

A subject-standing-type cone-beam computed tomography (CT) with high spatial resolution has been developed as a new three-dimensional imaging modality for subjects standing or sitting naturally on a turntable. A 16-in. X-ray image intensifier and charge coupled device camera acquires a 12-bit 5122-pixel projection at 60 f/s and the rotation period is 4.8 or 9.6 s for 288 or 576 projections, respectively. To reduce image noise, the system controls the X-ray pulse duration and iris-opening area through real-time analysis of the projection image. To improve CT accuracy and eliminate artifacts, the veiling glare of the image intensifier and scattered X-rays are corrected. Human chest and orthopedic studies with about 50 patients were conducted. Three-dimensional images with a spherical field of view with a diameter of 21-25 cm, 0.4- to 0.5-mm voxels and a 512(3) matrix were obtained. In coronal, sagittal and volume rendering images, the surface of arthrosis was visualized smoothly with a resolution higher than that of conventional CT. In the case of gonarthrosis, narrowing of the clearance at the surface of arthrosis was visualized clearly under body-weight burdening, which would be difficult if the subject was lying down.

[Malignant solitary fibrous tumor of the pleura: two cases and review of the literature].

We present two cases of malignant solitary fibrous tumor of the pleura (MSFT-P) and review 29 reported cases of MSFT-P. In examining the clinical and morphological features and prognosis of MSFT-P, we found that over 90% of the patients had some symptoms and one-third showed local recurrence. Chest radiographs and CT demonstrated large broad-based masses with necrosis, hemorrhage, and pleural effusion. The maximum diameter of the tumors was greater than 10 cm. Pleural effusion was seen in more than 70% of the patients, but there was only one case of pleural dissemination. All of the cases were well demarcated, and this was the most reliable finding for differentiating MSFT-P and malignant mesothelioma.

Pancreatoblastoma. Three original cases and review of the literature.

PURPOSE: To describe the characteristics of pancreatoblastoma. MATERIAL AND METHODS: We studied 3 cases of pancretoblastoma and reviewed another 59 cases. Parameters analyzed were tumor site, hemorrhage, capsule formation, necrosis, vascularity, production of alpha-fetoprotein (AFP), cystic changes and calcification. RESULTS: The diagnostic findings were as follows: pancreatic head origin (24/54, 44%), pancreatic body and tail origin (30/54, 56%), hemorrhage (16/17, 94%), capsule formation (24/26, 92%), necrosis (28/31, 90%), hypervascularity (10/14, 71%), production of AFP (19/28, 68%), cystic changes (11/16, 69%), and calcification (10/21, 48%). All neonatal cases demonstrated cystic changes. Three of them were patients with Beckwith-Wiedmann syndrome. The incidence of capsule formation and calcification was not related to the origin of the tumor. CONCLUSION: The most common features of pancreatoblastoma are hemorrhage, capsule formation and necrosis.

Longitudinal follow-up study of smoking-induced lung density changes by high-resolution computed tomography.

To evaluate the ability of high-resolution computed tomography (HRCT) to detect longitudinal changes in structural abnormalities caused by smoking, HRCT and pulmonary function tests were used to examine nonsmokers, current smokers, and past smokers annually for 5 yr. Inspiratory HRCT was taken for the upper, middle, and lower lung fields, while expiratory images were obtained for the upper and lower lung fields only. We estimated the three quantitative CT parameters including MLD (mean CT value), HIST (CT value with the most frequent appearance), and %LAA (relative area of low attenuation with CT values less than -912 HU). Most of the pulmonary function tests, excepting FEV(1), did not change annually, whereas many of the inspiratory HRCT parameters did. In nonsmokers, only %LAA in the middle or lower lung fields exhibited an annual increase. In current smokers, %LAA in the upper lung field was augmented, while inspiratory MLD or HIST in the middle or lower lung field became more positive. In past smokers, %LAA in any lung field examined increased. The annual change in %LAA in the upper lung field was larger for past smokers than nonsmokers, with little difference between past and current smokers. Expiratory CT parameters showed few annual changes in all groups. In conclusion, (1) aging increases airspace abnormalities, mainly in the lower lung field; (2) although continuous smoking worsens airspace abnormalities mainly in the upper portion of the lung, this trend does not seem to slow down even after smoking cessation; and (3) inspiratory HRCT images are superior to expiratory images for longitudinal estimation of structural abnormalities caused by aging and smoking.

A case of poorly differentiated hilar lung adenocarcinoma of an unidentified histological type.

The patient was a 74-year-old man, a physician, whose chief complaint was an unproductive cough. The shadow of a mass was seen at the hilum of the left lung, and the mediastinal lymph nodes on both sides were swollen. No histological diagnosis was obtained even after bronchoscopy, including transbronchial needle aspiration biopsy, but large-cell carcinoma of the lung was diagnosed on the basis of ultrasound-guided biopsy of a shadow in the liver suspected of being a metastatic tumor (T2N3M1, Stage IV). Two courses of chemotherapy (CBCDA + VDS) failed to gain any improvement, and the pain resulting from recurrent bone metastases was managed mainly by the administration of the best supportive care. The patient was readmitted to the hospital after development of numbness in the right upper extremity followed by complication of pneumonia and heart failure, and he passed away. Autopsy revealed a primary hilar lung tumor with a histological diagnosis of poorly differentiated adenocarcinoma.

Bronchopulmonary arterial anastomosis at the precapillary level in human lung. Visualization using CT angiography compared with microangiography of autopsied lung.

PURPOSE: To investigate the interrelationships between the bronchial and pulmonary circulations including the existence of precapillary bronchopulmonary arterial anastomoses. MATERIAL AND METHODS: CT of bronchial arteriography (BAG-CT) was performed in 10 patients and BAG-CT during a pulmonary artery block test (PA-block) in 5 patients with lung cancer. Bronchial and pulmonary circulations were evaluated in 5 autopsied normal lungs by injecting silicone rubber with different colors into the bronchial and pulmonary arteries. RESULTS: BAG-CT correlated well with the findings at silicone rubber injection into lung autopsy samples. BAG-CT demonstrated inflow of contrast medium into the pulmonary artery during PA-block in all cases, while no inflow was observed before and following reversal of PA-block. Mixed silicone rubber was observed in the lobar to subsubsegmental bronchial arteries in all cases and in the subsubsegmental pulmonary artery in one case. CONCLUSION: Precapillary bronchopulmonary arterial anastomoses may exist at the level of the lobar bronchi to the periphery. If either the pulmonary or bronchial circulation is disturbed, flow occurs inside the anastomoses to supplement the other flow, especially flow from the bronchial to the pulmonary arteries via the anastomoses, which occurs within 30 min.

Middle mesenteric artery visualized by computed tomographic angiography.

The middle mesenteric artery, a third mesenteric artery arising from the aorta that principally feeds the transverse colon, is an extremely rare anomaly. We identified a middle mesenteric artery branching into the ileocolic artery and into the right, middle, and accessory middle colic arteries. It supplied the cecum and the entire ascending and transverse colon. This anomaly was detected with computed tomographic angiography.

Congenital absence of the portal vein in a boy.

Congenital absence of the portal vein (CAPV) is a malformation that is generally thought to be limited to females. We encountered an 11-year-old boy with this malformation. In 17 previously reported cases of CAPV, 2 were male. Three male patients, including our case, were Abernethy type Ib malformation. They had no associated liver tumour and two had no additional congenital abnormality. Conversely, 13 of the 15 female patients had congenital abnormalities and 6 had liver mass lesions.

[Organ preservation in the treatment of malignant solid tumors in children].

Organ preservation is one of the requirements for maintaining the high quality of life after the treatment of malignant solid tumors in children. We analyze our recent results of renal preservation in the abdominal neuroblastoma and describe 3 cases of successful organ preservation in pelvic malignancy. Between 1982 and 1996, 29 patients with adrenal or retroperitoneal neuroblastoma, that extended into the surrounding tissues and organs and/or to the contralateral side, underwent delayed primary excision. Before 1982, 9 patients were treated and only one ipsilateral kidney was preserved. On the other hand, 13 of 20 kidneys were preserved after 1987. Adoption of cis-platinum deepened our awareness of preserving the kidney. Furthermore, we have altered our strategy since 1987 to continuing preoperative chemotherapy until the size of the tumor becomes as small as it is estimated completely resectable by our own index, which is derived from computed tomography. Kidneys are shielded during intraoperative irradiation, and the tumor relapsed from the shielded area of retroperitoneum in one patient. Four of 14 preserved kidneys became atrophic and lost their function. The bladder and the rectum were left intact at tumor resection after intensive chemotherapy in two patients with pelvic (one retroperitoneal and one vaginal) rhabdomyosarcoma. A yolk sac tumor of the vagina responded completely to chemotherapy with cis-platinum, etoposide and bleomycin. The patient has survived 7.5 years without any local and distant relapse in spite of the fact that surgery, aside from several diagnostic biopsies, was not undertaken.

[Radiological approach to the pleura and pleural cavity with CT and MRI].

We discuss the efficacy and limitations of CT and MRI in the assessment of the pleura and pleural cavity. With CT and MRI findings from 610 patients, we address six topics: 1) Normal anatomy of the pleural cavity, 2) Pleural effusion, 3) Pleural tumors, 4) Pyothorax, 5) Lung cancer, and 6) The pleura as a mirror of systemic diseases. CT showed specific findings of acute bleeding. MRI was sensitive enough for the diagnosis of subacute bleeding and chylothorax. In the evaluation of pleural tumors, CT and MRI revealed specific evidence of pedunculated localized fibrous tumors and lipomas. In long-standing pyothorax, it is important to detect any associated secondary malignancies. CT and MRI show as a mass penetrating the bony thorax. B-cell non-Hodgkin malignant lymphoma is the most commonly associated malignancy. The sensitivities for diagnosis of pleural extension of bronchogenic carcinoma are 80% with CT and 86% with MRI. The specificities are 89% with CT and 75% with MRI. To study the pleura as a mirror of systemic diseases, we used high resolution CT to examine 104 patients without evidence of the diseases mentioned above. Eighteen of those 104 patients were found to have pleural thickening, and all 18 were smokers. Of the 24 non-smokers, none had pleural thickening. The difference in the occurrence of pleural thickening between smokers and non-smokers was significant (p < 0.01).

Swallowing dysfunction and aspiration in neonates and infants.

In children, swallowing dysfunction and aspiration are common causes of recurrent pneumonia and can be fatal. The underlying mechanism is still unknown. In this study, we evaluated the swallowing function in 72 neonates and infants by fluoroscopy and followed the course of 39 patients for more than one year. The results of all the examinations were recorded on videotape or a digital imaging system. All 10 patients with neurologic disorder presented cricopharyngeal dysfunction. Of the 12 patients with near miss sudden infant death syndrome, 10 showed nasopharyngeal reflux, and 5 demonstrated cricopharyngeal dysfunction. In the 50 patients without neurologic disorder, 29 revealed cricopharyngeal dysfunction. Laryngeal elevation was not seen in 75%. Three of these 29 patients had no other abnormality. All patients without neurologic disorder recovered well. Seven patients demonstrated prolonged aspiration. Four of them had neurologic disorder, and the rest had near miss sudden infant death syndrome. Fluoroscopic examination showed two types of aspiration: one was caused not only by pharyngeal but also by lingual muscles. It was not associated with any neurologic disorders and the symptoms were transient. The other was caused by cricopharyngeal dysfunction and most of these patients had neurologic diseases and prolonged symptoms.

[Changes of peripheral airways through mechanical ventilation in acute respiratory failure].

To evaluate the morphologic changes in the peripheral airways associated with positive pressure ventilation, fine fiberoptic bronchoscopy (1.8 mm outer diameter) was performed in 12 patients who had no history of prior pulmonary failure. In 19 examinations, the main morphologic findings were paleness and bronchial dilatation in the peripheral airways where increased secretions, pigmentation and stenosis or collapse were also observed. Patients with bronchial dilatation had longer periods of mechanical ventilation (17 +/- 8 days) compared to those without dilation (10 +/- 5 days). The structural destruction in the peripheral airways observed in patients on prolonged positive pressure mechanical ventilation suggests that barotrauma may be more widespread than previously recognized.

[A study of localized inflammatory pulmonary lesions by ultra-thin bronchofiberscope].

We used a new type of endoscope developed by the authors in 10 patients with localized inflammatory pulmonary lesions in the peripheral areas which were far from the 3 branch and needed to be distinguished from lung cancer. Endoscopic findings revealed reddening, obstruction, stenosis, hypersecretion and dilation in the peripheral airway. The endoscopic findings in this disease were different from those peripheral lung cancer. The combination of thin-section CT, endoscopy and lung biopsy was the best method for determining the morphological changes in localized pulmonary lesions.

[MRI and ultrasonography of atherosclerosis of the thoracic aorta and carotid arteries in elderly hypercholesterolemic patients].

In 53 elderly participants aged more than 60 the thoracic aorta and bilateral carotid arteries were observed with noninvasive techniques, MRI and ultra-sonography, in order to elucidate the relationship between hypercholesterolemia and atherosclerosis in the elderly. Hypercholesterolemic subjects were classified as group H (serum total cholesterol (TC) greater than 220 mg/dl), group H-I (220 mg/dl less than TC less than 250 mg/dl) and group H-II (TC greater than or equal to 250 mg/dl). Atherosclerotic changes of the thoracic aorta were observed in 46% of group H, 27% of group H-I, 60% of group H-II and 37% of normolipidemic subjects (group NL). Carotid atherosclerotic changes were observed in 19% of group H, 9% of group H-I, 27% of group H-II and 18% of group NL. In group H-I, the percentages of atherosclerotic changes in both thoracic aorta and carotid arteries were lower than those in group NL. However, atherosclerotic changes of thoracic aorta and carotid arteries were detected in 43% and 29% of the subjects showing higher apo B/Apo Al ratio than 1.0 among group H-I + NL (TC less than 250 mg/dl). These changes occurred in 32% and 13% of the subjects showing lower apo B/Apo Al ratio than 1.0 among the same groups. Namely, atherosclerotic changes of the thoracic aorta and carotid arteries were observed more frequently in the subjects showing a higher apo B/Apo Al ratio than 1.0 even if their serum cholesterol values were not higher than 250 mg/dl. We should use not only the serum cholesterol value but also the apo B/Apo Al ratio as an indicator to evaluate the roles of lipids in the development of atherosclerosis.