Cases reported "Hypersplenism"

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1/12. Complications of partial splenic embolization in cirrhotic patients.

    In recent years, partial splenic embolization (PSE) has been widely used in patients with cirrhosis and hypersplenism caused by portal hypertension. We investigated the complications associated with PSE cases seen in our hospital. Seventeen cases of liver cirrhosis that had undergone PSE were examined to investigate the complications associated with it. Mean infarcted area of the spleen was 66.2%. Leukocyte and platelet counts in 16 of 17 patients were seen to improve after PSE and persisted for at least one year. The most frequent side effects were abdominal pain (82.4%) and fever (94.1%). Severe side effects were seen in two of those 17 patients. One patient died from acute on chronic liver failure. The other patients contracted bacterial peritonitis and splenic abscess and needed drainage of splenic abscess before recovery. These two cases were in child-Pugh class B. In conclusions, PSE is a useful treatment for patients with cirrhosis and hypersplenism caused by portal hypertension. However, the possibility of severe complications, especially in patients with noncompensated cirrhosis, should be kept in mind.
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2/12. Recurrent portal hypertension after composite liver/small bowel transplantation.

    Late technical complications of composite liver/small bowel transplantation procedures are often complex and have not been well defined. Here we describe the unusual presentation and management of two cases of recurrent thrombocytopenia due to hypersplenism resulting from portacaval shunt stenosis. Both patients presented with portal hypertension late after composite liver/small bowel transplantation. One patient presented with recurrent bouts of upper gastrointestinal hemorrhage and was ultimately found to have a stenosis of her native portacaval shunt. After unsuccessful balloon dilatation of the anastomosis, a successful side-to-side distal splenorenal shunt was performed. The second patient presented with severe thrombocytopenia, the etiology of which was determined to be a short segment occlusion of the inferior vena cava between the native portacaval shunt and the piggyback outflow anastomosis of the liver graft. Total caval occlusion prevented balloon dilatation; the patient was relisted for transplantation but died of chronic rejection four months later. Recurrent portal hypertension is challenging in patients who have had combined liver/small bowel transplantation. Surgeons performing intestinal transplantation need to be increasingly aware of these possible late complications.
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3/12. turner syndrome associated with acquired von Willebrand disease, primary biliary cirrhosis, and inflammatory bowel disease.

    We report a unique case of turner syndrome associated with acquired von Willebrand disease (AvWD), primary biliary cirrhosis (PBC), and inflammatory bowel disease (IBD). During 7 years of close follow-up, the patient presented with multiple major episodes of upper and lower gastrointestinal bleeding caused by different pathogenic mechanisms, such as IBD, AvWD, gastric varices, and thrombocytopenia. AvWD mimicking familial vWD type III on laboratory testing was most probably triggered by autoimmune mechanism associated with PBC. Therapy of PBC with ursodeoxycholic acid (UDCA) resulted in significant decrease of liver enzymes followed by normalization of vWF and FVIII levels. Portosystemic shunt placement with ligation of gastric varices improved hypersplenism and severe thrombocytopenia and led to clinical stability for more than 24 months. The clinicopathological features of these disorders and of the recurrent bleeding episodes are discussed in the text along with a review of the literature.
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4/12. portal vein thrombosis after laparoscopy-assisted splenectomy and cholecystectomy.

    A 12-year-old girl underwent laparoscopy-assisted splenectomy and cholecystectomy with removal of her spleen through a small Pfannenstiel incision. She had an unremarkable postoperative course but returned 16 days later because of increasing right-sided abdominal pain. The pain was constant, sharp, and stabbing without radiation. Abdominal examination showed diffuse right upper quadrant and epigastric tenderness without peritoneal irritation. Laboratory test results included white blood cell count, 14.4 x 10(9)/mm3; hemoglobin, 8.5 g/dL; platelets, 1,483,000; and normal values for lipase, amylase, aspartate transaminase, and alanine transaminase. Evaluation with ultrasonography and vessel Doppler studies showed an occlusive thrombus throughout the portal and splenic veins. The patient underwent intravenous heparin anticoagulation therapy. Her symptoms resolved completely over the next 2 days. The patient is currently receiving warfarin and anagrelide as an outpatient (international normalized ratio, 2). There were no long-term complications caused by portal vein thrombosis. This is the first reported case of portal vein thrombosis after laparoscopic splenectomy in the pediatric population.
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keywords = abdominal pain, upper
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5/12. Hematological malignancy manifesting as ascites.

    BACKGROUND: A 63-year-old Caucasian woman presented with intermittent, left-sided abdominal discomfort without other symptoms. physical examination revealed massive splenomegaly and complete blood counts showed thrombocytopenia. splenectomy was recommended but the patient declined. She was lost to follow-up twice but returned with tense ascites about 2 years after the initial presentation. Despite aggressive medical management, the ascites did not improve. INVESTIGATIONS: Bone-marrow and liver biopsies, abdominal ultrasound, esophagogastroduodenoscopy, abdominal CT scan and peripheral blood smear. diagnosis: Primary splenic lymphoma with hepatic infiltration causing portal hypertension and ascites. MANAGEMENT: paracentesis, dietary sodium restriction and diuretics, splenectomy, splenorenal shunt and chemotherapy (cyclophosphamide, doxorubicin, vincristine and prednisone).
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keywords = discomfort
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6/12. Effectiveness of Rex shunt in the treatment of portal hypertension.

    BACKGROUND: Children with portal venous thrombosis often have severe symptoms secondary to portal hypertension including recurrent upper gastrointestinal (UGI) bleeds and hypersplenism. We report results of the use of the mesenterico-left portal bypass (Rex shunt) in 5 consecutive patients. methods: A retrospective chart review of all patients with portal venous thrombosis who underwent Rex shunt procedure was performed. Children were evaluated preoperatively with magnetic resonance angiogram, Doppler ultrasound, and mesenteric angiogram. Postoperative ultrasounds were performed in follow-up. RESULTS: All patients had evidence of portal hypertension and hypersplenism. The average age of the patients was 13.2 /- 4.9 (7-19) years. The patient had an average of 2.6 /- 1.7 UGI bleeds requiring banding and 3.4 /- 4.2 U of blood transfused before undergoing the shunt. The mean operative time was 383 /- 46 minutes, and length of stay was 10.4 /- 7.1 days. In follow-up of 18.8 /- 5.2 months (11-24 months), all but 1 patient had a patent shunt. One narrowed shunt was successfully dilated by percutaneous angioplasty. thrombocytopenia improved significantly in patients with functioning shunts but did not correlate with a significant decrease in splenic size. CONCLUSIONS: The Rex shunt reestablishes normal hepatopedal portal flow, and in patients with functioning shunts, no recurrent UGI bleeds or transfusions were required or evidence of encephalopathy were noted.
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7/12. Partial splenectomy for massive splenomegaly secondary to Gaucher's disease.

    A 16 year old girl with Type 1 Gaucher's disease presented with massive splenomegaly, hypersplenism and abdominal discomfort. Traditionally hypersplenism has been treated with splenectomy, but this results in a high incidence of overwhelming sepsis and accelerated sphingolipid deposition in both liver and bone. A 90% partial splenectomy was therefore performed leaving a fully vascularized inferior segment of the spleen and resecting 5.8 kg of splenic tissue. The patient made an uneventful recovery with a marked improvement in her haematological parameters and general condition.
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keywords = discomfort
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8/12. Transcatheter partial splenic arterial embolization in patients with hypersplenism: a clinical evaluation as supporting therapy for hepatocellular carcinoma and liver cirrhosis.

    Eleven cases with hypersplenism, one with liver cirrhosis and ten with hepatocellular carcinoma (HCC) associated with liver cirrhosis, underwent transcatheter partial splenic arterial embolization. In four of ten HCC cases, the spleen was accidentally infarcted during the procedure of transcatheter hepatic arterial embolization (TAE). The mean infarcted area of the spleen was 55.7%. An increase in the peripheral platelet count was particularly remarkable and continued over one year after the embolization. High fever and abdominal pain were observed in all cases. The fever was seen for 18.0 days and pain was noted for an average of 12.8 days after the embolization. Other adverse effects such as pleural effusion and ascites were transitorily observed. Splenic embolization is an effective supporting therapy for hypersplenism in patients with cirrhosis or HCC.
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9/12. gaucher disease: treatment of hypersplenism with splenic embolization.

    hypersplenism is a frequent complication of gaucher disease requiring splenectomy. A patient with gaucher disease and severe hypersplenism was treated with partial splenic embolization to avoid the increased risk of serious infectious complications and deterioration of the disease associated with splenectomy. A first embolization (25% ablation) was performed at 4 years. Because of persisting abdominal discomfort, failure to thrive and signs of hypersplenism a second embolization (40-50% ablation) was performed 18 months later. Subsequently, the patient's health improved remarkably and 4 years later he achieved normal growth, maintains normal haematologic parameters, is free of symptoms and has no skeletal abnormalities. No serious infections have occurred. The size of the liver and the spleen has not changed appreciably. It appears that partial splenic embolization may be preferable to splenectomy in patients with gaucher disease, especially in those of young age.
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ranking = 3.5609107528934
keywords = discomfort
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10/12. thrombocytopenia in association with a wandering spleen.

    An ectopic, so-called wandering spleen is an uncommon occurrence. We present the case of a young woman who presented with abdominal pain and was found to have an enlarged spleen, located in the lower abdomen and pelvis. The possibility of lymphoma was entertained because of concomitant findings of thrombocytopenia and a possible mesenteric mass. The mass was subsequently found at laparotomy to be the tail of the malpositioned pancreas, and the thrombocytopenia resolved with splenectomy. review of the literature indicates that lymphoma is an uncommon finding in wandering spleens, that wandering spleens are enlarged in most cases, and that thrombocytopenia, while uncommon, can be seen, in particular when associated with torsion of an elongated splenic pedicle.
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