Cases reported "Splenic Diseases"

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1/7. Generalized lymphangiomatosis and chylothorax in the pediatric age group.

    Four patients with generalized lymphangiomatosis presenting with chylothoraces are described. All four had bone involvement, two had involvement of the spleen, and one of the pericardium. The diagnosis was confirmed by typical radiology, histology, and in three patients by immunohistochemistry. Treatment was mainly palliative. Three patients died within 1/2 to three years of presentation.
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ranking = 1
keywords = angiomatosis
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2/7. Cystic angiomatosis with splenic involvement: unusual MRI findings.

    Cystic angiomatosis is a rare disorder with a poor prognosis. We describe a case of a 33-year-old woman who presented with long-standing bone pain, hemolytic anemia, and an enlarged spleen. Radiologically, multiple osseous lesions with a mixed pattern of lytic and sclerotic areas were seen within the shoulders, spine, and pelvis. On CT and MRI of the abdomen, the spleen was markedly enlarged, with internal hyperdense foci on non-contrast CT scan, corresponding to low signal intensity areas on all MR pulse sequences. After administration of contrast, a mottled enhancement pattern throughout the entire spleen was seen both on CT and MRI. Cystic angiomatosis was proven by histological analysis of a biopsy specimen of an involved vertebra and histopathological examination of the spleen after subsequent splenectomy. This is the first report of a patient with disseminated cystic angiomatosis with splenic involvement in which the MRI features differ from the previous reports. Instead of the usual pattern consisting of multiple well-defined cystic lesions, a diffuse involvement replacing the entire spleen, with heterogeneous signal intensities on T2-weighted images and heterogeneous enhancement pattern, was seen in our patient.
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ranking = 1.4
keywords = angiomatosis
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3/7. Systemic cystic angiomatosis in pregnancy: a case presentation and review of the literature.

    Systemic cystic angiomatosis is the involvement of multiple organ systems with a congenital vascular malformation. A combination of vascular anomalies, namely lymphangioma and hemangioma, can coexist. The liver, spleen, kidney, and colon are the most commonly affected organs. The clinical presentation varies and generally reflects the involved organ system. A case of systemic cystic angiomatosis involving the spleen, liver, and kidney is presented. The diagnosis and management during pregnancy is discussed.
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ranking = 1.2
keywords = angiomatosis
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4/7. Splenic cysts: aspiration, sclerosis, or resection.

    Percutaneous aspiration and tetracycline sclerosis is a safe but temporary therapy of large splenic cysts in children. Between 1985 and 1987, three girls with splenic cysts were seen. Their ages ranged from 5 to 14 years, and the cysts were from 8 to 16 cm in diameter. Despite their large size, all were asymptomatic and were discovered upon physical examination or ultrasound for unrelated conditions. All cysts were avascular by scan and had irregular crenated or smooth walls by ultrasound. Further investigation excluded infectious or parasitic causes. Each cyst was aspirated for diagnosis, and a pigtail catheter was inserted for drainage and sclerotherapy. All needle aspirations resulted in cyst collapse, but in one patient the pigtail catheter insertion was unsuccessful, and in the other two cases, multiple attempts of tetracycline sclerosis failed to obliterate the cysts. There were no other complications. Surgery for the recurrent splenic cysts was performed 3 months to 2 years following the percutaneous procedures. The two patients operated on with 3 months of aspiration underwent successful partial splenectomy and have normal splenic function by ultrasound scan, and absence of RBCs. The third patient had progression of the cystic disease throughout the spleen, and required splenectomy. pathology confirmed multiseptate congenital mesothelial cysts in the first two patients and massive lymphangiomatosis in the third. In all three cases, percutaneous therapy was safe but did not result in long-term control. In one patient, the cystic disease progressed following sclerotherapy and may have influenced the need for complete splenectomy. Prior manipulation did not adversely affect the dissection and mobilization of the spleens.(ABSTRACT TRUNCATED AT 250 WORDS)
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ranking = 0.2
keywords = angiomatosis
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5/7. Bacillary angiomatosis of the spleen.

    Bacillary angiomatosis is a recently described vasoproliferative lesion associated with infection by a newly characterized rickettsial organism, Rochalimaea henselae. Most previous reports have described skin lesions in immunocompromised patients infected with human immunodeficiency virus. This is the first case report detailing the features of bacillary angiomatosis of the spleen occurring in a patient undergoing cytotoxic chemotherapy for disseminated ovarian carcinoma.
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ranking = 1.2
keywords = angiomatosis
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6/7. Cystic angiomatosis: case report and review of the literature.

    The objective of this article was to offer a better characterization of the typical clinical presentation, radiologic findings, histology, treatment approaches, and differential diagnosis of cystic angiomatosis, a rare condition of which previous reports have been confusing because of unclear diagnostic criteria, different classifications, and variations in terminology. A case report using the improved imaging techniques of computed tomography scanning is presented in addition to an analysis and review of the previous literature, which relied heavily on plain film radiography, biopsy, and necropsy for diagnosis. A case report of a 26-year-old man initially symptomatic at age 12 is presented. Although a rare condition, cystic angiomatosis must be considered in pediatric and young adult patients presenting with diffuse, multifocal, cystic skeletal lesions, with or without visceral involvement.
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ranking = 1.2
keywords = angiomatosis
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7/7. Granulomatous hepatitis and necrotizing splenitis due to bartonella henselae in a patient with cancer: case report and review of hepatosplenic manifestations of bartonella infection.

    Bacillary angiomatosis and the related disorders of bacillary peliosis hepatis and bacillary splenitis are manifestations of infection with bartonella henselae and bartonella quintana in immunocompromised persons. B. henselae infection, but not B. quintana infection, is linked to contact with cats and is presumed to cause visceral cat-scratch disease. We reports a case of visceral infection by B. henselae in an adult patient with cancer who was receiving chemotherapy and had had no contact with a cat or dog. The patient--whose illness was eventually diagnosed on the basis of findings of histologic, polymerase chain reaction, and serological studies--was treated with doxycycline and rifampin, and the infection resolved. In addition, 41 cases of documented or suspected bartonella infection of the liver, spleen, or both in immunocompetent or immunocompromised hosts are reviewed.
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ranking = 0.2
keywords = angiomatosis
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