RESUMO
A 68-year-old woman being treated with hemodialysis for autosomal dominant polycystic kidney disease was admitted for progressive dyspnea over 6 months. On chest radiography, her cardiothoracic ratio had increased from 52.2% 6 months prior, to 71%, and echocardiography revealed diffuse pericardial effusion and right ventricular diastolic insufficiency. A resultant pericardial tamponade was thought to be the cause of the patient's dyspnea, and therefore a pericardiocentesis was performed, with a total of 2,000mL of fluid removed. However, 21 days later the same amount of pericardial fluid had reaccumulated. The second pericardiocentesis was performed, followed by transcatheter renal artery embolization (TAE). The kidneys, which were hard on palpation before TAE, softened immediately after TAE. After resolution of the pericardial effusion was confirmed, the patient was discharged after 24 days in hospital. Twelve months later, the patient was asymptomatic, the cardiothoracic ratio decreased to 48% on chest radiography and computed tomography revealed no reaccumulation of pericardial effusion. This case illustrates a potential relationship between enlarged kidneys in autosomal dominant polycystic kidney disease and pericardial effusion.
Assuntos
Derrame Pericárdico , Rim Policístico Autossômico Dominante , Feminino , Humanos , Idoso , Rim Policístico Autossômico Dominante/complicações , Rim Policístico Autossômico Dominante/terapia , Artéria Renal , Derrame Pericárdico/diagnóstico por imagem , Derrame Pericárdico/etiologia , Derrame Pericárdico/terapia , Rim , Dispneia/complicaçõesRESUMO
Blue rubber bleb nevus syndrome (BRBNS) is a rare syndrome characterized by venous malformations in the skin and gastrointestinal tract, especially in the small intestine. Patients with BRBNS have increased risks of gastrointestinal hemorrhage and anemia. This is the first report in the English literature on BRBNS with hemangiomas in the small intestine that were treated successfully by endoscopic clipping using double-balloon endoscopy. A 25-year-old Japanese man presented to a local clinic with dyspnea, fatigue, and a hemoglobin level < 5 g/dL. The diagnosis was iron deficiency anemia. Since childhood, he had had a hemangioma in the shoulder joint and hemangiomas in the skin on the left arm. However, neither upper nor lower gastrointestinal endoscopy showed any lesions and he was referred to us for further evaluation and treatment of the anemia. Small bowel capsule endoscopy (SBCE) revealed hemangiomas in the small intestine, one of which was bleeding. Transanal DBE revealed a 10-mm bluish-purple hemangioma with erosion on the surface, which became smaller after application of five clips. Follow-up SBCE on day 50 showed that the hemangioma had completely disappeared. Clipping may be a safe and effective treatment for small bowel hemangioma in BRBNS.