Epithelioid hemangioendothelioma of the liver associated with thrombocytopenia and coagulopathy.

Epithelioid hemangioendothelioma of the liver is a rare vascular neoplasm with intermediate malignant potential. The prognosis is highly unpredictable. We report the case of a 59-year-old woman who had the tumor radically resected, but multiple metastases of the liver developed associated with thrombocytopenia and consumption coagulopathy, as observed in Kasabach-Merritt syndrome. The patient did not respond to any treatment and the behavior of the tumor was very aggressive. The patient died 15 months after radical resection of the tumor.

[A case of intimal dissection of the aorta during transfemoral angiography].

A 54-year-old man underwent right transfemoral angiography because of left renal hematuria. During angiography, dissection of abdominal aorta and thoracic aorta was encountered. It was initiated by intramural catheter passage at the bifurcation of the internal and external iliac artery. Transaxillary aortography about one month after the first angiography showed occlusion of the dissecting space in the thoracic aorta and existence of dissecting space in the abdominal aorta. Communicating orifices between the true space and the false space existed not only at the bifurcation of internal iliac artery and external iliac artery, but also at the abdominal aorta near the left renal artery. CT 1.5 months after the first angiography did not demonstrate more improvement. Surgery was performed. It was impossible to sew up and close the orifices of the space because of the fragility of the intima. Surrounding abdominal aorta and common iliac artery were wrapped near orifices with a dacron graft. A follow-up CT obtained 3 months postoperatively showed that the dissecting space in the abdominal aorta had disappeared. Wrapping was very useful to promote organization of the dissecting space.

[Isolated iliac aneurysms].

An isolated iliac aneurysm is an uncommon entity with a relative incidence to abdominal aortic aneurysm of 0.9 to 1.9 percent. Analysis of 16 cases with 20 isolated iliac aneurysms experienced at our vascular service makes the basis of this report. Seventeen aneurysms involved the common iliac artery, two the internal iliac artery and one the external iliac artery. As we experienced 341 abdominal aortic aneurysms during the same period, relative incidence of isolated iliac aneurysm to abdominal aortic aneurysm was 4.7 percent. Fifteen of the 16 cases were male; isolated iliac aneurysms had significantly higher preponderance for the male. Six of the cases were presented with ruptured aneurysms, giving a rupture rate of 37.5 percent. This rupture rate was significantly higher than that of abdominal aortic aneurysms of 11.1 percent. Operation was performed on 15 patients; the remaining one died before surgery. Although there was no operative death among the elective cases, three patients in the ruptured group died postoperatively giving a mortality rate of 60 percent. The least diameter of the ruptured aneurysms was 3.5 centimeter. Among the 16 patients 5 presented with a ruptured aneurysm and 3 had no symptoms related to the aneurysm. Five patients complained of a pulsatile abdominal mass. Two of the ruptured aneurysms were associated with an iliac arteriovenous fistula. Both of them involved the common iliac artery. Although emergency operation was performed on one of them, he died of acute renal failure due to preoperative dehydration and hypovolemia. The another one was operated on electively after intensive medical treatment for heart failure and the patient survived.(ABSTRACT TRUNCATED AT 250 WORDS)

[Successful surgical management of extracranial high internal carotid aneurysm. A case report].

A 76 year-old woman visited our university hospital with a complaints of dysphagia of 3 months duration and was discovered to have a right internal carotid aneurysm protruding to the right side of the larynx. A day prior to admission, dysarthria and left hemiparesis occurred with sudden onset. These ischemic attacks spontaneously remitted within 48 hours. A carotid arteriogram showed a saccular aneurysm located high in the right internal carotid artery. Four days after admission, aneurysmectomy with end-to-end anastomosis was carried out with the temporary internal shunt under the induced hypertension. There was no sign of ischemic brain damage after the operation. But she suffered from dysphagia due to the NO. 9, 10, and 12 cerebral nerve palsies, which took 5 months to recover completely. Despite several difficulties in the operation for a highly located internal carotid aneurysm, we emphasize in this report the necessity of aneurysmectomy and the establishment of vascular continuity with the aid of internal shunt to prevent the ischemic brain damage.

[Renovascular hypertension with a solitary kidney associated with retrocaval ureter: a case report].

A case of renovascular hypertension in a patient with a congenitally solitary kidney associated with retrocaval ureter is reported. Aortorenal bypass with autogenous saphenous vein graft and concomitant mobilization of the ureter by division and reanastomosis of the inferior vena cava were performed. Pathogenesis of the stenotic lesion of the renal artery was fibromuscular dysplasia. Postoperative improvement of hypertension was not so good as expected. Soon after administration of nifedipine (20mg/day) was started, blood pressure decreased down to 130/80mmHg and had been well controlled. Problems regarding renovascular hypertension with a solitary kidney and surgical treatment of retrocaval ureter are discussed.

[Emergency perfusion lung scan for pulmonary embolism].

Six cases of pulmonary embolism were reported. As pulmonary embolism often becomes critical condition, early diagnosis and medical treatment is need. Perfusion lung scan for pulmonary embolism may be useful for a diagnosis and evaluation of the degree of its disease and therapeutic value.

Effect of copper and diethyldithiocarbamate combination therapy on the macular mouse, an animal model of Menkes disease.

Menkes disease (MD) is a neurodegenerative disorder characterized by a copper deficiency in the brain. It is caused by the defective intestinal absorption of copper resulting from a deficiency of a copper-transporting ATPase, ATP7A. This gives rise to an accumulation of copper in the intestine. The copper deficiency in the brain of MD patients cannot be improved by copper injections, because the administered copper accumulates at the blood-brain barrier and is not transported across to the neurons. To resolve this problem, we investigated the effect of a combination therapy of copper and sodium diethyldithiocarbamate (DEDTC), a lypophilic chelator, in an animal model of MD, the macular mouse. Four-week-old macular mice treated with 50 mug of CuCl2 on the 7th day after birth were used. Experimental mice were given a subcutaneous injection of CuCl2 (4 microg) and an intraperitoneal injection of DEDTC (0.2 mg/g body weight) twice a week for 4 weeks and then sacrificed. Copper concentrations and cytochrome-c oxidase activity in the brains of treated mice were higher than those of control macular mice, which received only copper or saline. The ratios of brain noradrenaline to dopamine and of adrenaline to dopamine were also increased by the treatment, suggesting that the activity of dopamine beta-hydroxylase, a copper-dependent enzyme, was improved by the treatment. Liver and renal function tests showed no abnormalities in the treated mice, although copper concentrations in the kidneys of treated mice were higher than those of control macular mice. These results suggest that DEDTC facilitates the passage of copper across the blood-brain barrier and that the combination therapy of copper and DEDTC may be an effective treatment for the neurological disturbances suffered by patients with MD.