Dural venous sinus thrombosis in a patient with sickle cell disease: case report and literature review

We report a case of dural venous sinus thrombosis (DVST) in a patient who developed seizures following exchange transfusion for treatment of acute chest syndrome associated with sickle cell disease. Evaluation with magnetic resonance imaging and magnetic resonance venography of the brain indicated left sigmoid sinus thrombosis. The history and laboratory evaluation did not reveal any other inherited or acquired hypercoagulable states. This is the fourth case of dural venous sinus thrombosis associated with sickle cell disease reported in literature. The patient had a favorable outcome with early treatment of unfractionated heparin.     

Subendocardial arteriovenous malformation in a patient with unstable angina

A variety of arteriovenous abnormalities of the heart have been previously described. Arteriovenous malformations are uncommon developmental abnormalities documented in almost every organ of the body. A subendocardial arteriovenous malformation in a patient with unstable angina is described which we believe to be the first reported case.     

烟雾病合并动静脉畸形一例

患者男,46岁。因“发作性右侧肢体麻木、无力11个月”于2009年2月4日在军事医学科学院附属医院神经外科就诊。患者于2008年3月突发右侧肢体麻木、无力,发作时不能站立和持物,持续约10min后症状缓解。在当地医院给予改善微循环治疗后,上述症状未再发作。     

Pancreaticobiliary arteriovenous malformation with common bile duct dilation in a patient with hemobilia.

Arteriovenous malformation of the pancreas is a very rare entity. We describe a case involving the head of the pancreas associated with progressive hemobilia bleeding from the lower part of the common bile duct. The patient was a 50-year-old man with acute epigastralgia. Endoscopic retrograde cholangiopancreatography revealed hemobilia and cystic dilation of the common bile duct. Angiography demonstrated increased blood volume in the head of the pancreas and early filling of the superior mesenteric and portal veins. Abdominal pain and progressive anemia caused by hemobilia required surgical treatment. Histologic examination of the resected specimen revealed marked proliferation of the blood vessels in the pericholedochal area and the exact point of bleeding from the pancreaticobiliary arteriovenous malformation.     

Posterior mediastinal arteriovenous malformation: report of a case

Vascular malformations of the mediastinum are extremely rare. We report a histologically proven case of a posterior mediastinal arteriovenous malformation in a 54-year-old woman simulating a mediastinal tumor and we describe the findings in conventional radiography, computed tomography, and digital subtraction angiography.     

Progression of a dural arteriovenous malformation resulting in an intracerebral hematoma. A case report.

A progressing, essentially extracranial arteriovenous malformation with purely external carotid feeders can involve the intracranial vasculature by enlargement of its drainage area. Thus it can cause cerebral dysfunction by an intracranial bleed or raised intracranial pressure due to venous engorgement. A case is described to support this possibility.     

Fatal pulmonary hypertension in a patient with mixed connective tissue disease: report of an autopsy case.

A 34-yr-old woman with mixed connective tissue disease (MCTD) who died of heart failure due to pulmonary hypertension (PH) is reported. She showed various symptoms and signs over the previous 8 yr, including lymphadenopathy, Raynaud's phenomenon, polyarthralgia, sclerodactylia, esophageal disturbance, PH, Sj?gren's syndrome and renal tubular acidosis. Autopsy findings revealed severe intimal proliferation with marked luminal narrowing of the small arteries in the lung. Echocardiographic and mechanocardiographic methods were very useful for assessment of the degree of right ventricular dysfunction associated with PH on the early stage of MCTD.     

Pulmonary varix mimicking pulmonary arteriovenous malformation in a patient with Turner syndrome

A 36-year-old asymptomatic female with Turner syndrome was referred for a 3-cm opacity of the left lung detected by routine chest X-ray. A computed tomography scan of the chest suggested a vascular lesion such as pulmonary arteriovenous malformation, and transcatheter embolotherapy was considered. The lack of a right-to-left shunt on contrast echocardiography led to suspect an alternate diagnosis. Magnetic resonance imaging and pulmonary angiography eventually demonstrated a pulmonary varix associated with a partial anomalous pulmonary venous return. Contrast echocardiography may help to distinguish between pulmonary varix and arteriovenous malformation.     

Acquired arteriovenous malformation induced by pressure: a case report

Arteriovenous malformations may be congenital or acquired. In the latter case, usually a traumatic injury to the arteries precedes the arteriovenous anastomoses. Two elderly patients presented with large, purple-colored verrucous tumors on the buttocks. Both patients were obese and immobile, and reported repeated bleeding from the lesions after minor trauma. The tumors were soft and could be emptied by applying pressure. Doppler examination revealed arterial pulsations over the lesions. Both cases were diagnosed as pressure-induced arteriovenous malformations. The lesions are assumed to have been caused by tissue damage in the deep subcutis induced by decubitus.     

Leopard syndrome with hypertrophic obstructive cardiomyopathy and cerebral arteriovenous malformation. Report of a case

We report a case of a 27-year-old woman with Leopard syndrome in which we observed the association of hypertrophic obstructive cardiomyopathy with arteriovenous cerebral shunt. This association has not been reported previously in the literature.     

Disappearance of an intracerebral arteriovenous malformation in an HIV-infected patient after initiation of HAART

HIV infection or complications of HIV-induced immunodeficiency may affect the central nervous system (CNS). However, vascular cerebral pathologies are very rare, in particular intracerebral arteriovenous malformations (AVM). We report the case of an HIV-infected patient who had a cerebral AVM leading to symptoms such as recurring focal seizures. Only after initiation of potent antiretroviral combination therapy, but not antiretroviral monotherapy or bitherapy, could the viral load be suppressed and immunodeficiency resolved. Two years after the start of highly active antiretroviral therapy (HAART) total occlusion of the AVM could be demonstrated. Taken together, this case report may demonstrate the potent angiogenic activity of HIV for AVM. Also, this case report might show that inhibition of such a cofactor may lead to resolution of an AVM. Received: June 15, 2001 · Revision accepted: December 3, 2001     

Gross hematuria due to renal arteriovenous malformation successfully treated with embolization in an elderly patient

Renal arteriovenous malformation is a rare anomaly of the urinary system. We report an elderly patient aged 60 years who presented with gross hematuria due to left renal arteriovenous malformation. This case highlights the importance of careful diagnostic work-up in the evaluation of gross hematuria and emergency treatment with transcatheter arteriographically directed embolization.     

Renal cortical necrosis at presentation in a patient with systemic lupus erythematosus: an autopsy case report

Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disorder. Renal involvement has the worst prognosis. However, renal cortical necrosis is extremely unusual in SLE. In this case report, we describe the autopsy findings in a young female patient with SLE presenting with renal failure. At autopsy, there was Libmann–Sacks endocarditis with multiorgan infarcts and renal cortical necrosis. Secondary antiphospholipid antibodies contribute to the cardiac and renal manifestations in SLE. We discuss the incidence and pathogenesis of endocarditis with differential diagnosis for cortical necrosis in a patient of SLE.     

Intrahepatic cholangiocarcinoma with sarcomatous transformation: an autopsy case.

We report an adult case of intrahepatic cholangiocarcinoma with remarkable sarcomatous changes. At autopsy a yellowish-white tumor (8 x 6 cm) was found in the left hepatic lobe, and there were several daughter nodules in both hepatic lobes. Histologically, most of the main tumor, and all of the daughter nodules examined, showed sarcomatous changes (spindle- or fusiform-shaped and pleomorphic cells). Histologic examination of a whole slice of the main tumor disclosed a focus of adenosquamous carcinoma (cholangiocarcinoma) within the tumor. Frequent transitions between adenosquamous carcinoma areas and sarcomatous areas suggested that sarcomatous transformation occurred in cholangiocarcinoma and then grew to spread rapidly. Immunohistochemically, squamous carcinoma and, to a lesser degree, adenocarcinoma elements were strongly positive for keratin and epithelial membrane antigen, both being also weakly positive in sarcomatous cells, supporting that possibility. Vimentin was positive only in sarcomatous elements. Cholangiocarcinoma should be included in the list of hepatic tumors showing sarcomatous change.     

Report of an autopsy case of colon cancer with amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a degenerative disease involving both upper and lower motor neurons and the pathogenesis of this disorder is still unknown. To date, few reports have suggested that motor neuron diseases may have a paraneoplastic origin. However, it is still under discussion whether ALS occurring in cancer patients is paraneoplastic. A 60-year-old man with rectal cancer (Stage IV) having multiple lung, liver and para-aortic lymph node metastases underwent anterior resection of the rectum as palliative surgery. He was referred to our hospital for adjuvant chemotherapy. Lung and lymph node metastases decreased after 2 courses of chemotherapy using CPT-11 and 5-FU/LV but liver metastases were enlarged, following up increase in CEA. Thereafter, he suffered from muscle weakness in hands, arms, and legs and results of neurophysiologic studies were compatible with primary lateral sclerosis (ALS). For second line chemotherapy, he was treated with low-dose CDDP/5-FU over 6 courses. As a result, the size the of metastatic lesions markedly reduced and CEA was decreased to the normal level. Although significant tumor reduction was observed, his neurological symptoms rapidly progressed. He died of aspiration pneumonia 8 months after onset of the disease. Autopsy revealed that his neuropathological findings were compatible with ALS, and it was thought to be the primary cause of death in the because of absence of cancer progression. In this case the neurological syndrome was not affected by cancer therapy. Thus our case does not support the hypothesis that ALS in associated with cancer and the relationship between both disorders remains uncertain.     

肺、肝多发性血管畸形1例并文献复习

肺多发性血管畸形很少见,肺多发性血管畸形合并肝血管畸形更罕见[1-2]。现将本院收治的1例肺动静脉畸形（pulmonary arteriovenous malformations,PAVMs）合并肝血管畸形报道如下,并结合文献进行复习。     

Pancreatic arteriovenous malformation with pancreatitis involving a pancreatico-venous fistula.

An arteriovenous malformation of the pancreas is a very rare disease, but its presentation is distinct and unique. In this report, we describe a patient who presented with this malformation which was localized in the tail of pancreas and demonstrated by abnormal angiography findings. The patient was a 60-year-old male with severe left hypochondralgia. Angiography revealed an increased blood volume in the tail of the pancreas with arteriovenous shunting. Secondary pancreatitis caused by the arteriovenous malformation was suspected by abnormal laboratory data, and confirmed by histology from the resected tail of the pancreas. This is a very rare report in which pancreatic arteriovenous malformation involving a pancreatico-venous fistula was confirmed by endoscopic retrograde cholangiopancreatography (ERCP).