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1/26. Emergency and elective surgical treatment of portal hypertension. A review of 23 years' experience.

    A retrospective review of surgical treatment for portal hypertension during a 23-year period in a regional unit is reported and the immediate and subsequent management of patients with bleeding oesophageal varices is discussed. Fifty-four patients with recurrent varix haemorrhage uncontrolled by conservative methods have been treated by oesophageal transection with a mortality of 22.2% (26.6% for cirrhotic patients). Thirty-two per cent of the cirrhotics were alive after 2 years. Only a minority (12%) of the survivors were considered suitable for a subsequent shunt procedure. Therapeutic portacaval anastomosis has been performed on 65 patients with a 51.2% 5-year survival (43-5% for cirrhotic patients). Further haemorrhage due to shunt thrombosis occurred in 5-3% of cases. The frequent occurrence of portal-systemic encephalopathy, increasing with duration of time following a shunt, is emphasized. The high morbidity and mortality in the poor-risk cirrhotic indicated that this type of patient is unsuitable for a portal-systemic shunt and is better treated by medical means.
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ranking = 1
keywords = haemorrhage
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2/26. Bleeding portal-hypertensive gastropathy managed successfully by partial splenic embolization.

    The use of partial splenic embolization to decrease portal pressure and reduce gastric bleeding from portal-hypertensive gastropathy, a complication of liver cirrhosis, is described. A 62-year-old man with hepatic cirrhosis secondary to hepatitis c and documented portal hypertension was admitted with hypersplenism and bleeding esophageal varices. Endoscopic ligation successfully controlled acute bleeding, but blood loss continued over the next 45 days. Bleeding secondary to portal-hypertensive gastropathy was diagnosed endoscopically. The patient's poor surgical status precluded a portosystemic shunt procedure, so partial splenic embolization was performed radiologically by the injection of Gelfoam squares. Splenic volume decreased 50% following partial embolization. Over 3 weeks, the hemoglobin concentration increased from 8.5 g/dL to 9.8 g/dL, and the platelet count increased from 41,000 to 90,000/microL. Repeat endoscopy found no gastric bleeding 18 days post-procedure. Partial splenic embolization is a radiologic procedure which can be performed safely in patients too ill to undergo portosystemic shunt. This report documents its successful use to manage hypersplenism and reduce portal pressure in a cirrhotic patient with portal-hypertensive gastropathy and hypersplenism.
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ranking = 0.30720632965795
keywords = blood loss
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3/26. Acute visual loss in a child with autosomal recessive polycystic kidney disease: case report and review of the literature.

    Acute visual loss secondary to ischemic optic neuropathy in children is extremely rare. The causes are usually hypotension or anemia. We describe the clinical course of a 9-year-old boy with a functional renal transplant who presented to the emergency room hemodynamically stable after waking up with complete bilateral loss of vision (no light perception). Examination showed that he had suffered massive nocturnal blood loss from esophageal varices secondary to portal hypertension. The patient's end-stage renal disease was secondary to autosomal recessive polycystic kidney disease (ARPKD), an entity comprised of renal cysts and hepatic fibrosis. Ophthalmologic findings in ARPKD are rarely cited in the literature. A literature search revealed 3 other cases of sudden visual loss reported in nonophthalmologic journals in patients with ARPKD. Funduscopic examination showed bilateral optic nerve head pallor and swelling with associated flame hemorrhages. The fact that this patient already had mildly pale nerves on presentation, along with hemodynamically compensated blood pressure and pulse, suggested chronic as well as acute ischemia. Based on our findings and other reported cases in the literature, ophthalmologic examinations may be indicated in all patients with ARPKD.
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ranking = 0.30720632965795
keywords = blood loss
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4/26. Ectopic intestinal varices as a rare cause of lower gastrointestinal haemorrhage.

    Ectopic intestinal varices are rarely responsible for lower gastrointestinal (GI) haemorrhage. A case of 55 years old male with recurrent melena is being presented, who was found to have scattered varices on small as well as large intestine. Selective review of literature regarding presentation, diagnosis and management of these cases is also part of presentation.
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ranking = 2.5
keywords = haemorrhage
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5/26. Transjugular intrahepatic portosystemic shunt for the treatment of portal hypertension secondary to non-cirrhotic perisinusoidal hepatic fibrosis.

    Non-cirrhotic perisinusoidal hepatic fibrosis is a process of imprecise pathogenesis involving collagenization of the space of Disse. Exposure to chemicals, auto-immunity, thrombophilia and/or infections are suspected primary agents. Here, we present the case of a patient who developed severe portal hypertension with histological features suggesting a non-cirrhotic perisinusoidal hepatic fibrosis. A 52-year-old man was hospitalized for oesophageal variceal haemorrhage. liver cirrhosis or portal vein thrombosis were absent as attested by laboratory tests, duplex sonography, computed tomography scan and histological examination of a liver biopsy specimen. Presinusoidal portal hypertension was suggested by a normal wedge-free hepatic vein gradient. Only electron microscopy examination of a liver biopsy specimen could disclose perisinusoidal fibrosis. This was most probably secondary to a combined chemotherapy received 4 years earlier for non-Hodgkin large-cell lymphoma. As variceal ligation failed to control oesophageal varices while liver function tests were normal, a transjugular intrahepatic portosystemic shunt (TIPS) was performed. This dramatically improved the signs of portal hypertension. This case illustrates the use of TIPS in the treatment of portal hypertension secondary to non-cirrhotic perisinusoidal fibrosis.
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ranking = 0.5
keywords = haemorrhage
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6/26. Pancreatic carcinoma presenting as bleeding from segmental gastric varices: pitfalls in diagnosis.

    splenic vein occlusion leading to gastric variceal haemorrhage should be considered in cases of obscure upper gastrointestinal bleeding. We report an unusual case in which the underlying pathology was a resectable carcinoma of the pancreatic tail.
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ranking = 0.5
keywords = haemorrhage
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7/26. Fatal variceal haemorrhage after paracetamol overdose.

    A patient is described where oesophageal varices developed and bled 13 days after a paracetamol overdose. The bleeding was unresponsive to medical management and proved fatal. There was no evidence that the patient had pre-existing liver disease. At necropsy the liver showed severe acute parenchymal necrosis but chronic lesions were absent. The portal vein and hepatic veins were patent.
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ranking = 2
keywords = haemorrhage
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8/26. Congestive gastroenteropathy--an extension of nonvariceal upper gastrointestinal bleeding in portal hypertension.

    Three cirrhotic patients with acute and chronic gastrointestinal blood loss are described. All had extensive gastric mucosal changes on endoscopy consistent with congestive gastropathy and also had extensive duodenal and jejunal changes consisting of multiple friable punctate areas of erythema. Two patients had esophageal varices from which bleeding could not be documented. The mucosal abnormalities seen in the small intestine of all three patients were similar to those within the stomach and are thought to represent an extension of congestive gastropathy and to be contributing to the blood loss. We propose that the term "congestive gastropathy" be replaced by a more comprehensive term, "congestive gastroenteropathy." The cause of these mucosal abnormalities remains unclear. Attempts at endoscopic therapy of these extensive abnormalities should be avoided until a greater understanding of the underlying pathophysiology is reached.
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ranking = 0.6144126593159
keywords = blood loss
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9/26. Oesophageal varices associated with busulphan-thioguanine combination therapy for chronic myeloid leukaemia.

    5 patients receiving continuous busulphan and 6-thioguanine for chronic myeloid leukaemia (CML) were found to have oesophageal varices associated with abnormal liver function tests. 3 of these cases presented with gastrointestinal haemorrhage and 1 patient died. The 2 other cases had varices discovered at endoscopy. Nodular regenerative hyperplasia (NRH) of the liver was identified as the cause of portal hypertension in the 4 patients on whom liver biopsies were done. The administration of busulphan and thioguanine in combination is likely to be associated with the development of NRH, with portal hypertension and oesophageal varices occurring in a substantial proportion of cases.
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ranking = 0.5
keywords = haemorrhage
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10/26. Repeated shunt surgery in a patient with portal hypertension.

    A 45-year-old male with chronic active hepatitis and portal hypertension had a mesocaval interposition graft performed because of repeated uncontrolled bleeding from oesophageal varices. Acute hepatocellular failure, manifested by Grade IV encephalopathy and severe coagulopathy, developed early in the post-operative course despite the absence of hepatic necrosis or other precipitating factors. Both encephalopathy and coagulopathy resolved rapidly following ligation of the shunt. Variceal bleeding recurred and nine months later an emergency distal lienorenal shunt was performed. Post-operatively the shunt was shown to be patent and there has been no encephalopathy or recurrence of variceal bleeding. It is concluded that (1) the splanchnic haemodynamic effects of a mesocaval interposition graft can result in severe hepatocellular failure and this can be reverted by shunt ligation and (2) the distal lienorenal shunt, while effectively reducing the risk of haemorrhage from varices, may be less likely to result in post-operative encephalopathy than more conventional forms of portal decompressive surgery.
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ranking = 0.5
keywords = haemorrhage
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