[en] Short communication

No abstract available

[en] Sickle cell disease is an important health care issue in the United States and in certain areas in Africa, the Middle East and India. Although a great deal of progress has been made in understanding the disease at the molecular and pathophysiologic level, specific treatment which is safe and accessible for most patients is still elusive. Going into the next millennium, the management of this disease is still largely dependent on early diagnosis and the treatment of complications with supportive care. Thus, diagnosis and evaluation of the complications of the disease are crucial in directing clinical care at the bedside. Modern imaging modalities have greatly improved, and their application in the patient with the sickling disorders has enhanced the decision - making process. The purpose of this article is to review the clinical aspects of common complications of the disease and to discuss imaging approaches which are useful in their evaluation. (orig.)

[en] The incidence of pulmonary embolism in children is not clearly known, but is believed to be low. Risk factors for pulmonary thromboembolism include central venous catheter, malignancy, surgery, infection, trauma, and congenital hypercoagulable disorders. Children with sickle cell disease are prothrombotic and are at an increased risk of thromboembolism. The incidence of this event is unknown because these children are often not thoroughly imaged. We report here a case of a calcified pulmonary thromboembolism in a child with sickle cell disease and emphasize the use of multidetector CT in detection of pulmonary thromboembolism in children with sickle cell disease. (orig.)

[en] Two cases of unilateral and bilateral periorbital hematomas occurred in patients with sickle cell anemia. The cause of periorbital swelling in these cases was found to be orbital and frontal bone infarctions, respectively, diagnosed by technetium Tc 99m medronate bone scintigraphy. To our knowledge, periorbital bone infarction, as a part of the differential diagnosis of periorbital hematoma and as part of the possible ocular manifestations in patients with sickle cell anemia, has not previously been described

[en] Although aneurysmal complications of sickle cell anemia have been described in the intracranial circulation, visceral artery pseudoaneurysms in this disease entity have not previously been reported in the literature. Conventional treatment of visceral pseudoaneurysms has been surgical ligation or resection of the aneurysm. Transcatheter embolization has emerged as an attractive, minimally invasive alternative to surgery in the treatment of these lesions. In certain situations, however, due to the unfavorable angiographic anatomy precluding safe transcatheter embolization, direct percutaneous glue injection of the pseudoaneurysm sac may be considered to achieve successful occlusion of the sac. The procedure may be rendered safer by simultaneous balloon protection of the parent artery. We describe this novel treatment modality in a case of inferior pancreaticoduodenal artery pseudoaneurysm in a patient with sickle cell anemia. Although a complication in the form of glue reflux into the parent vessel occurred that necessitated surgery, this treatment modality may be used in very selected cases (where conventional endovascular embolization techniques are not applicable) after careful selection of the balloon diameter and appropriate concentration of the glue-lipiodol mixture

[en] Our recent results support the suggestions made by Sangkatumvong et al regarding autonomic dysfunction in sickle cell anemia. (letter to the editor)

[en] Skeletal complications of sickle-cell anemia are multiple and can appear on the acute (osseous infarction, acute osteomyelitis) or chronic mode (osteonecrosis, chronic osteomyelitis). The radio-labelled diphosphonate bone scintigraphy remains an important tool in the early diagnosis and in the follow-up of these complications and must form part of the initial assessment of the disease. Through clinical observations, the authors undertake to sum up the bone scintigraphy aspects of these complications. (author)

[en] Pooling of blood in the spleen is a frequent occurrence in children with sickle cell diseases, particularly in the first few years of life, resulting in what is termed ''splenic sequestration crisis.'' The spectrum of severity in this syndrome is wide, ranging from mild splenomegaly to massive enlargement, circulatory collapse, and even death. The diagnosis is usually clinical, based on the enlargement of the spleen with a drop in hemoglobin level by >2 g/dl, and it is rare that imaging studies are ordered. However, in the patient who presents to the emergency department with non-specific findings of an acute abdomen, it is important to recognize the appearance of sequestration on imaging studies. We studied seven patients utilizing contrast-enhanced CT scans and found two distinct patterns - multiple, peripheral, non-enhancing low-density areas or large, diffuse areas of low density in the majority of the splenic tissue. Although radiological imaging is not always necessary to diagnose splenic sequestration, in those situations where this diagnosis is not immediately obvious, it makes an important clarifying contribution. (orig.)

[en] In early life, patients with sickle cell disease (SCD) can have acute, life-threatening emergencies related to splenic hypofunction (overwhelming bacterial sepsis), as well as anemic crises from acute splenic sequestration because of sudden pooling of blood in the spleen. The landmark penicillin prophylaxis study in 1985 showed a remarkable decrease in mortality from sepsis in young children with SCD who were treated with oral penicillin prophylaxis compared to placebo. Since that study, newborns are screened for SCD and placed on oral penicillin prophylaxis in nearly all of the United States, as well as in other countries where the disease is highly prevalent. The previously described permanent, complete and nearly universal ''autosplenectomy'' emerging by late childhood or early adulthood is now challenged by recent findings of reversibility of splenic dysfunction by the antisickling drug hydroxyurea or by successful allogeneic stem cell transplantation, even in older patients. Imaging techniques for hypofunction of the spleen are the most commonly used modalities to guide the clinician in decisions regarding medical or surgical management. (orig.)