Portal-systemic encephalopathy from a spontaneous gastrorenal shunt diagnosed by three-dimensional computed tomography and treated effectively by percutaneous vascular embolization.

A 67-year-old man with a portal-systemic shunt confirmed by three-dimensional computed tomography (3D-CT) was successfully treated by percutaneous vascular embolization. The patient had aggravated loss of memory, disorientation, and hyperammonemia. A gastrorenal shunt 16 mm in diameter was found by 3D-CT reconstructed by helical computed tomography (CT). Embolization was performed only in the shunt percutaneously through the inferior vena cava. One year after the embolization, no recurrence of portal-systemic encephalopathy and no portal hypertension have appeared, and the clinical course has been good.

Portal hypertension in children and young adults: sonographic and color Doppler findings.

Portal hypertension is a relatively uncommon pathologic condition in children and young adults in contrast with older adults. The aim of this study is to evaluate the utility of sonography and color Doppler sonography in the diagnosis of portal hypertension in children and young patients and to evaluate the sonographic pattern of each disease. We reviewed 25 such patients who were younger than 30 years old and obtained the following sonographic findings: (1) liver cirrhosis: (a) multiple intrahepatic venovenous shunts in patients with primary Budd-Chiari syndrome and (b) intrahepatic vascular narrowing and nodular coarse parenchymal texture, with multiple very-high-echo spots along the portal vein in patients with Wilson disease; (2) congenital hepatic fibrosis: marked and developed collaterals, wide periportal echogenic band, and a heterogeneous parenchymal texture comprised of multiple high echoes but without portal thrombus; and (3) extrahepatic portal thrombosis: invisible portal lumen except as an echogenic band. Sonography and color Doppler sonography are very useful in diagnosing these portal hypertensive diseases. However, there are no specific sonographic findings, and the role of sonography is limited to follow-up observation of associated secondary hepatobiliary changes in patients with congenital biliary atresia.

Hepatic involvement in Osler-Weber-Rendu disease: findings on pulsed and color Doppler sonography.

OBJECTIVE: The purpose of this study was to compare the hemodynamics of the liver vasculature as determined by Doppler sonography for patients with Osler-Weber-Rendu disease and for healthy subjects. SUBJECTS AND METHODS: Real-time color Doppler sonography and pulsed Doppler sonography were used to study 10 patients with Osler-Weber-Rendu disease and 25 healthy subjects. Vessel diameter and flow velocity in the hepatic artery and its branches as well as in the portal vein and its segmental branches were determined. Flow patterns in the hepatic veins were analyzed. The clinical manifestations of the disease were correlated with the Doppler sonographic findings. RESULTS: The hepatic artery and its branches were dilated and tortuous, and flow velocity was greater in patients than in healthy subjects; hepatic artery velocities (mean +/- SD) were 153 +/- 65.2 cm/sec versus 64.9 +/- 11.4 cm/sec (p < .005). The resistive index measured in the hepatic artery did not differ significantly between the two groups (p was not significant). No intrahepatic arteriovenous shunt sites were detected by color Doppler sonography. However, arterioportal shunts were observed in two patients, one of whom also had a portovenous shunt. A portovenous shunt and multiple shunts between hepatic veins were found in another patient. Portal venous flow was similar in both groups. No relationship between Doppler sonographic changes and clinical manifestations was found. CONCLUSION: The multiple microscopic arteriovenous shunts found in Osler-Weber-Rendu disease were not detected in our patients but resulted in dilatation and increased flow velocity in the hepatic artery. Flow velocity in the portal vein appeared to be undisturbed. Large intrahepatic shunts were easily outlined by Doppler sonography.

Color Doppler findings in small abdominal aneurysms.

Four cases of small abdominal aneurysms were assessed by color Doppler ultrasonography. The aneurysms demonstrated interesting and highly complicated internal hemodynamics, including a swirling blood flow pattern and/or a color mosaic pattern. These findings suggested the usefulness of color Doppler ultrasonography for obtaining details on internal hemodynamics, which cannot be obtained with other modalities, such as angiography or computed tomography (CT).

Macroscopic intrahepatic portal-hepatic venous shunt not associated with chronic hepatic disease.

Asymptomatic macroscopic portal-hepatic venous shunt (MPVS) without chronic hepatic disease is a rare vascular lesion. Two cases of this disorder are reported, and the usefulness of color Doppler sonography for studying hemodynamics of such small vascular lesions is stressed.

Diffuse heterotopic submucosal cystic malformation of the stomach: ultrasonographic diagnosis.

The preoperative diagnosis of diffuse heterotopic submucosal cystic malformation of the stomach is very difficult to make with upper gastrointestinal (GI) series or endoscopy. We report a case of submucosal cysts associated with an early gastric carcinoma in which ultrasonography (US) and endoscopic ultrasonography (EUS) were very useful in the preoperative diagnosis.

[Adult dermatomyositis with angiopathy and cecum perforation].

This previously healthy 43-year-old man was admitted to our hospital with a history of rash, dysphagia and severe myalgia for two months. Physical examination showed prominent edema and erythema over the face and the chest, scattered ulcerations on the trunk, and muscle atrophy most prominent proximally. Serum levels of muscle enzymes were remarkably increased. Two weeks of oral prednisolone therapy (40 mg/day) was not effective, and betamethasone intravenous pulse therapy (3 x 1000 mg) was followed by slight clinical improvement. However, 12 days after pulse therapy, he complained abdominal pain on the right lower quadrant. The surgical findings included peritonitis due to single perforation of the cecum. After operation, cyclosporine therapy was added and over the next 14 month a considerable clinical improvement was noted. Prednisolone was reduced from 80 mg to 10 mg daily. Biopsy specimens from ulcerated+ skin and perforated cecum showed prominent vascular abnormalities: arterial and venous intimal hyperplasia, occlusion of vessels by fibrin thrombi, and lymphocytic infiltration which affected veins of all sizes. The evidence strongly suggests that both skin ulcers and cecum perforation were caused by vasculitis and occlusion of vessels, which often seen in childhood dermatomyositis.

A case of solitary mastocytoma suggesting a divided form of mast cell nevus.

The case of a 10-week-old boy with a solitary mastocytoma is reported. The lesion, noticed at birth, was located in an area extending from the dorsum of the left hand to the proximal phalanx of the ring and little fingers. It appeared as a coin-sized, flattened dome-shaped, round tumor with occasional blistering. The web between the two fingers was free of cutaneous changes. The diagnosis was confirmed by the histological characteristics: a densely mastocytic infiltration into the dermis. The present case of solitary mastocytoma, referred to as "mast cell nevus", is unique because of its previously undescribed divided form.