Background
Budd-Chiari syndrome (BCS) is a rare disease resulting from obstruction of the hepatic venous outflow tract that typically presents with abdominal pain, jaundice and ascites without frank liver failure. However, BCS may also evolve more rapidly to acute liver failure (ALF-BCS).
Aims
To describe the clinical features, treatment and outcomes of ALF due to BCS and compare our results with those in the published literature.
Methods
Twenty of the 2,300 patients enrolled in the Acute Liver Failure Study Group (ALFSG) registry since 1998, presented with a clinical diagnosis of BCS. An additional 19 cases of ALF-BCS in the English-language literature were reviewed and compared to the ALFSG cases.
Results
Most ALF-BCS patients were white (84%) and female (84%) in their fourth decade. A hypercoagulable state was noted in 63% of cases. BCS was diagnosed by Doppler ultrasonography or abdominal CT in all cases. Liver biopsies (6) all had evidence of severe peri-central necrosis. Treatments used included most commonly anticoagulation (71%), but also transjugular intrahepatic portosystemic shunt (TIPS) (37%) and orthotopic liver transplantation (37%). In-hospital mortality was approximately 60%.
Conclusions
Budd-Chiari syndrome is a rare cause of ALF and mandates prompt diagnosis and management for successful outcomes. Once the diagnosis is confirmed, prompt anticoagulation is recommended in conjunction with evaluation for malignancy or thrombophilic disorder. Mortality may have improved in recent years with use of TIPS and/or OLT compared to prior published reports.
No preventive benefit of aspirin or statins, and no therapeutic benefit of statins, was found for RVO in high-risk patients. High-risk RVO patients suffer substantially worse outcomes than those reported in other studies not limited to such patients.
A n 88-year-old male presented with a gradually enlarging, painless mass on his anterior chest wall for 1 year (Panel A). On exam, there was a 12×10×5 cm, nontender, firm, immobile mass over the body of the sternum. Computed tomography (CT) demonstrated a permeative chest-wall mass (Panel B). Serum prostate-specific antigen (PSA) was 3,019 ng/ml. Histopathology was consistent with prostate adenocarcinoma. The patient had poor functional status and requested hospice.Chest wall tumors account for less than 1 % of all neoplasms and can arise from either a soft tissue or bony structure.1 More than 50 % of chest wall tumors are malignant. Malignant primaries include Ewing's sarcoma, osteosarcoma and chondrosarcoma. Malignant lesions tend to grow faster, are painful, and present as larger masses than benign lesions. Primary malignant tumors of the chest wall should be resected with wide margins and receive adjuvant therapy as appropriate. Metastatic disease accounts for 16 % of malignant chest wall tumors. Chest wall metastases occur more commonly from blood-borne spread than lymphatic or direct spread. The chest wall is an extremely rare site of prostate cancer metastasis. 3 Case reports have described chest-wall metastasis regression with standard hormonal chemotherapy, with median survival of 12-24 months. dashed arrow).1888
Purpose: Posterior segment hemorrhage occurring during or shortly after examination (PSHE) for retinopathy of prematurity (ROP) is a very rare complication. We present a case of and review the literature on PSHE during ROP examination to better characterize this complication. Methods: A case report is presented, followed by a review of similar cases in the literature. Results: An infant undergoing laser photocoagulation for ROP rapidly developed diffuse intraretinal hemorrhages in his right eye during the laser and after a Valsalva event while he was intubated under general anesthesia. The hemorrhages resolved within 1 week. This presentation was similar to those in previously reported cases. Conclusions: PSHE in ROP usually consists of multiple, diffuse, intraretinal hemorrhages that occur within minutes of ROP examination and resolve within a few weeks without any other ocular findings or sequelae. PSHE seems to represent a form of ocular decompression retinopathy.
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