Priapism as the first and terminal manifestation of sickle cell disease.
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RATIONALE AND OBJECTIVES: Contrast of trueFISP images depends mainly on the T2/T1 ratio. Consequently, there is a potential gain in signal intensity after administration of paramagnetic contrast medium despite the strong T2 weighting. The purpose of this study was to analyze signal intensities of abdominal organs after applying contrast medium and to determine whether this yields an improved contrast for pathologies compared with precontrast trueFISP. MATERIALS AND METHODS: Fifty patients underwent an abdominal examination, including the trueFISP sequence before and after the administration of contrast medium. All images were obtained with a 1.5 T system. The mean signal-to-noise ratio before and after contrast medium was assessed for abdominal organs, vessels, muscle, and fat. The contrast-to-noise ratio (CNR) of pathologic lesions was calculated. RESULTS: The trueFISP sequence yielded a higher signal-to-noise ratio after application of contrast medium for all organs except for fat and the aorta. CNR of solid lesions (angiomyolipoma, liver adenoma, liver hemangioma, hepatocellular carcinoma) increased whereas contrast of cysts decreased. CONCLUSIONS: TrueFISP imaging after application of contrast medium led to better CNR for many solid lesions while cysts showed a diminished contrast. We advise trueFISP imaging sequences before and after contrast medium application.
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A case of torsion of a wandering spleen in an 8 year old girl is reported. The authors discuss the aetiology and management of this rare condition and review the literature. Wandering spleen is relatively more common in children than in adults. Under the age of 10 the sex distribution is even; over 10, females outnumber males by seven to one. In 66 children under 10 years, 50% of wandering spleens were lost through acute ischaemia. The authors recommend that early splenopexy replace conservative management.
High altitude seemed to be responsible for seven recent cases of sickling crisis. People with sickle-cell trait are at risk if they fly in unpressurized aircraft, which are used for many local air services. Those with sickle-cell haemoglobin C disease should avoid air travel even in pressurized aircraft. Possibly as a result of "autosplenectomy," patients with sickle-cell anaemia seem to be able to fly in pressurized aircraft with little risk. All passengers and aircrew who might have some form of sickle-cell disease should be screened before flight.
The manifestations of the sickling disorders are becoming increasingly familiar to clinicians in Great Britain. One of these disorders, sickle-cell haemoglobin disease, has hitherto received little attention, being regarded as a relatively mild condition. This paper describes some of the distinctive clinical features of the disease as seen in a series of nine cases which have recently presented in London, two of which were fatal. The special hazards of the condition in relation to pregnancy, air travel, and general anaesthesia are discussed.
In 10 patients with a markedly enlarged left lobe of the liver, the posterior (prone) views (99mTc-sulfur colloid) revealed a false-positive splenic defect. In 1 case the defect was also apparent in the anterior view. In all but 1 case this pseudodefect was not present in the posterior image obtained standing up. Use of a spleen-specific or liver-specific radionuclide agent was necessary in that case. The authors caution against diagnosis from an apparently abnormal posterior image unless proved by other measures.
Seventy-five black patients with sickle hemoglobinopathies who are followed by the Colorado Sickle Cell Treatment and Research Center, and 172 of their family members were evaluated by retrospective interview for the occurrence of sickling crises when traveling in the Rocky Mountains or by aircraft. Twenty per cent of 39 patients with sickle cell anemia (Hgb SS) (Hgbs S/C and S/T) have developed crises when traveling in the mountains above 2000m. Vaso-occlusive crises predominated in the SS group and splenic crises occurred primarily in those with Hgbs S/C and S/T. Approximately 20 per cent of those with S/C and S/T, but none with SS, had crises when flying in pressurized aircraft. Among 103 family members with sickle cell trait (Hgb AS), no significant risk of developing crises could be identified with either mountain or pressurized aircraft travel.
The wandering spleen is an unusual entity, which has been described rarely in infants and children. Although patients may be asymptomatic, some may present with torsion of the spleen with associated vovulus and vascular infarcation. Two asymptomatic patients with wandering spleen are described. Radiographic signs and isotopic scan findings of wandering spleen are discussed and the literature is reviewed.
Perceived high operative risk for splenectomy in children with hematologic disorders and hypersplenism has led to attempts at transcatheter splenic embolization (TSE) as a possibly safer alternative. A recent experience with a child who presented with an apparent acute subcapsular hematoma after TSE and who underwent complicated emergency splenectomy prompted review of the risks and complications of TSE. Although theoretically attractive in many respects, TSE is associated with significant morbidity. The resultant diagnostic and therapeutic difficulties of this procedure can potentially complicate patient management, and the often life-threatening sequelae warrant considerable restraint in its use. In addition, TSE often fails to produce the desired, long-term hematologic response. Because of the morbidity and risk associated with TSE, primary operative intervention is advised for most children in whom splenectomy is indicated. Morbidity associated with the direct operative approach may be minimized by judicious use of blood components and by meticulous surgical technique.
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This essay illustrates the appearance of the traumatized spleen on CT scans obtained during the course of conservative treatment. Although the CT appearance of acute rupture of the spleen has been adequately described, little has been reported about the appearance of the spleen as it heals after trauma. Examples of CT studies of splenic injuries illustrate the various changes in appearance over time in the traumatized spleen that is treated nonoperatively.
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