Cases reported "Hypertension, Portal"

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1/330. Portal hypertension due to extensive hepatic cysts in autosomal dominant polycystic kidney disease.

    Liver cysts are a well-recognized feature of autosomal dominant polycystic kidney disease (ADPKD) and occur in 77% of patients more than 60 years old. Serious sequelae, however, are rare, the two most common complications being pain and cyst infections. Portal hypertension has been reported in ADPKD due to the rare presence of congenital hepatic fibrosis. We report a case of ADPKD in a patient who had portal hypertension due to distortion of portal vein and venules by extensive hepatic cysts.
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keywords = vein
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2/330. Use of adrenal vein conduit for splenorenal shunts: a case report.

    We report a case with extrahepatic portal venous obstruction (EHPVO), who presented with recurrent bleeding following a previous devascularization procedure and needed an emergency distal spleno-renal shunt (DSRS). Due to technical difficulty because of previous scarring, the adrenal vein was used as a conduit between the splenic vein and renal vein. The shunt's patent and the patient has been bleed-free for 2 years.
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keywords = vein
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3/330. Portal hypertension secondary to arterio-portal fistulae: two unusual cases.

    A 62-year-old male presented with variceal haemorrhage. Investigation demonstrated a fistula between the left gastric artery and portal vein with a porto-systemic gradient of 35 mm Hg. Variceal bleeding was controlled by a transcatheter embolisation of the fistula, but the patient died of septicaemia three weeks later. The second patient, a 42-year-old male who presented with variceal bleeding was shown to have diffuse arterio-venous fistulae involving the right lobe of the liver with a portosystemic gradient of 25 mm Hg. In this case the variceal bleeding was successfully controlled by insertion of a transjugular intrahepatic portosystemic shunt (TIPS). The pathogenesis of portal hypertension in arterioportal fistulae and the role of interventional radiological techniques in the management of variceal bleeding in these patients is discussed.
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4/330. Epithelioid hemangioendothelioma with marked liver deformity and secondary budd-chiari syndrome: pathological and radiological correlation.

    A case of malignant epithelioid hemangioendothelioma of the liver in a 48-year-old woman with severe portal hypertension and marked deformity of the liver is presented. This woman had a history of mild liver dysfunction since the age of 30 years, and abdominal distention, esophageal varices, splenomegaly and ascites since October 1996. Imaging examinations revealed liver deformity with severe atrophy of the left lobe and the anterior segment of the right lobe. Celiac arteriography showed narrowing and upward deviation of the proper hepatic artery, and occlusion of the left and right anterior hepatic arteries. Since March 1997, hepatic venography showed stenosis in the right hepatic vein truncus. budd-chiari syndrome was clinically diagnosed. She died in June 1997. The autopsy disclosed massive tumor embolism in the left and right anterior portal branches, few in the hepatic artery, and occlusion of the left and right anterior hepatic arteries. The extensive tumor embolism resulted in portal hypertension, and atrophy of the left lobe. The anterior segment of the right lobe was probably caused by the occlusion of both the hepatic arteries and the portal veins. The posterior segment of the right lobe, without massive tumor embolism in its portal branch, appeared hypertrophic.
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keywords = vein
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5/330. Segmental portal hypertension due to splenic vein obstruction: imaging findings and diagnostic pitfalls in four cases.

    Segmental portal hypertension due to isolated splenic vein obstruction is a rare but important entity as it is the only curable cause of portal hypertension by splenectomy. Four cases are presented illustrating the radiological features of splenic vein obstruction, and the diagnostic pitfalls that arose in patients with complicated clinical presentations.
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ranking = 6
keywords = vein
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6/330. color Doppler sonography in the diagnosis of neonatal intrahepatic portosystemic shunts.

    Intrahepatic portosystemic shunts are infrequent in children. We report 3 cases of neonates who presented with jaundice during the first month of life. color Doppler sonography in the first 2 cases showed direct communication between the right portal and hepatic veins. Both infants were asymptomatic, and the shunts disappeared spontaneously. The third case involved several shunts and an aberrant medial portal vein. This patient developed heart failure and died after surgery. color Doppler sonography was useful in the diagnosis and follow-up of the shunts in all 3 cases.
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keywords = vein
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7/330. Ovarian hyperthecosis in the setting of portal hypertension.

    Hepatocellular dysfunction and perturbed portal hemodynamics alter steroid metabolism. Men with liver disease have gynecomastia, although women similarly affected rarely show virilization. We report a 10-yr-old girl with portal hypertension and shunting associated with precocious puberty and ovarian hyperandrogenism. This was one of premature twin girls; neither had clitoromegaly or genital ambiguity. In one child, neonatal respiratory problems led to umbilical vein catheterization with subsequent development of portal hypertension. Pubic hair was first noted at age 6 yr, breasts at 7 yr, and severe acne and clitoromegaly at 10 yr. Baseline sex hormones were elevated: androstenedione (A), 413 ng/dL; testosterone (T), 226 ng/dL; and estradiol (E2), 160 pg/mL. Liver transaminases were within the normal range, however, the coagulation profile was mildly abnormal. cosyntropin adrenal stimulation revealed no steroidogenic defect. dexamethasone suppression reduced A and T slightly. LH-releasing hormone stimulation produced a pubertal rise in LH and FSH. Pelvic sonography showed a large right ovary with numerous follicles. Surgical exploration revealed symmetrically enlarged ovaries with dense capsules. histology of ovarian wedge resections showed hyperthecosis; immunohistochemistry showed stromal cells expressing steroidogenic enzymes and proteins. One month postoperatively, A and T were unchanged from baseline, whereas E2 decreased to 56 pg/mL. A single dose of depot leuprolide acetate significantly reduced T. Subsequent treatment with oral contraceptives reduced T to 50 ng/dL, and cyclical menses occurred. We conclude that precocious puberty and ovarian hyperthecosis were induced in this young girl by elevated circulating levels of sex hormones, a consequence of portasystemic shunting and impaired hepatic steroid metabolism.
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keywords = vein
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8/330. Trans-anastomotic porto-portal varices in patients with gastrointestinal haemorrhage.

    AIM: Porto-portal varices are commonly seen in patients with segmental extra-hepatic portal hypertension and develop to provide a collateral circulation around an area of portal venous obstruction. It is not well recognized that such communications may also develop across surgical anastomoses and be the source of gastrointestinal haemorrhage. The possible mode of development of such communications has not been previously discussed. MATERIALS AND methods: Over a 3-year period between 1995 and 1998, porto-portal varices were demonstrated across surgical anastomoses in four patients who were referred for the investigation of acute (two), acute-on-chronic (one) and chronic gastrointestinal bleeding (one). Their medical notes and the findings at angiography were reviewed. RESULTS: Three patients had segmental portal hypertension due to extra-hepatic portal vein (one) or superior mesenteric vein (two) stenosis/occlusion. One patient had mild portal hypertension due to hepatic fibrosis secondary to congenital biliary atresia. At angiography all patients were shown to have varices crossing previous surgical anastomoses. These varices were presumed to be the cause of bleeding in three of the four patients; the site of bleeding in the fourth individual was not determined. CONCLUSIONS: Trans-anastomotic porto-portal varices are rare. They develop in the presence of extra-hepatic portal hypertension and presumably arise within peri-anastomotic inflammatory tissue. Such varices may be difficult to manage and their prognosis is poor when bleeding occurs.
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ranking = 2
keywords = vein
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9/330. Segmental portal hypertension due to a splenic echinococcus cyst.

    A 60-year-old Libyan woman developed perihilar splenic varices without other signs of portal hypertension. Plain abdominal X-ray examination showed two calcified structures in the left and right hypochondria. Ultrasound examination disclosed a 3-cm diameter, globally calcified hydatid cyst lodged in a critical location at the hilar region of the spleen. The cyst was compressing the hilar vessels which resulted in dilatation and varix formation. Another hydatid cyst measuring 5 cm in diameter, with extensive wall calcification was visualized in the right lobe of the liver. The splenic size was within normal limits. The liver revealed normal texture and size and the portal vein was of normal caliber. The patient underwent an uneventful splenectomy and was well at discharge.
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ranking = 1
keywords = vein
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10/330. Successful treatment of chronic budd-chiari syndrome with a transjugular intrahepatic portosystemic shunt.

    budd-chiari syndrome is characterized by obstruction of the hepatic venous outflow tract. Therapeutic options for chronic budd-chiari syndrome are limited. We report the case of a 28-year-old woman who presented with recurrence of chronic budd-chiari syndrome with total obstruction of all major hepatic veins. Due to worsening liver function over the course of 1 year, she had to be listed for liver transplantation. Because of therapy-refractory ascites, declining renal function and severe esophageal varices, a transjugular intrahepatic portosystemic shunt (TIPS) was placed, planned as a bridge to transplantation. Following TIPS, a marked recovery of liver function could be observed, accompanied by disappearance of ascites, esophageal varices, and normalization of kidney function. Therefore, the patient could be removed from the waiting list for liver transplantation. This case demonstrates for the first time that the use of TIPS in chronic budd-chiari syndrome may result in marked recovery of liver function.
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