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Splenic syndrome in sickle cell trait: four case presentations and a review of the literature.

Four cases of splenic infarction/sequestration in sickle cell trait (SCT) patients are presented. All four patients were undergoing moderate exercise at elevations ranging from 5,500 to 12,000 feet. The patients include two African-American males, a Hispanic male, and a white female. All four presented with the acute onset of mid epigastric then left upper-quadrant pain, nausea, vomiting, and respiratory splinting. A review of the literature indicates that splenic infarction with SCT is not uncommon; however, not surprisingly, it is often initially misdiagnosed. This is the first report in the literature of a female with SCT incurring a splenic syndrome with exposure to terrestrial altitude. Although SCT is not a contraindication for moderate- or high-altitude activities, military physicians need to consider the diagnosis of splenic infarction early in any patient regardless of race or sex who presents with left upper-quadrant pain at altitudes above 5,000 feet. Prompt evacuation to sea level may hasten recovery and spare further splenic trauma. Although SCT should be considered a relatively benign entity, the literature also suggests a higher than average risk of sudden death in military recruits with SCT from exertional heat illness and rhabdomyolysis.

Adult↗

Splenic artery ligation in distal splenorenal shunts.

Splenic artery ligation, a simple surgical procedure expected to decrease splenic flow and portal pressure in patients with cirrhosis of the liver, was performed concomitantly with a distal splenorenal shunt procedure in six patients and as the main surgical procedure in two patients. Immediate cessation of bleeding was achieved in the four patients in whom the splenic artery was ligated to reduce intraoperative bleeding. However, three of the seven patients with previous gastroesophageal hemorrhage rebled from various postoperatively. Symptoms of splenic infarction were observed in six patients, resulting in thrombosis of the splenic vein and/or of the distal splenorenal shunt in four patients and necessitating splenectomy in one. This incidence of thrombosis of the distal splenorenal shunt is much higher than the overall incidence of 5 per cent observed at our institution. It is thus concluded that the splenic artery should not be ligated in cirrhotic patients with patent distal splenorenal shunts, since splenic arterial collateral vessels have already been reduced by the gastric devascularization, an integral component of the distal splenorenal shunt.

Adolescent↗

Systemic thromboembolism in rheumatic heart disease.

The results of 62 autopsies of patients with rheumatic heart disease have been analyzed. The patients were 14-56 years old (mean 26 years). Thirteen patients had evidence of systemic thromboembolism (STE). All 13 patients had renal infarction, 2 had cerebral infarction, and 1 had a splenic infarction. Only 1 case with cerebral infarct was diagnosed clinically. Seven of 30 patients with atrial fibrillation had STE as did 6 of 32 patients with sinus rhythm. The type of valve involved and previous commissurotomy did not positively correlate with STE. It was observed that in this selected group of chronically ill patients who were autopsied, the elderly male patients with atrial fibrillation demonstrated increased incidence of STE.

Adolescent↗

Splenic abscess diagnosed with the aid of abdominal computerized tomography: report of 2 cases.

Two patients with splenic abscess successfully diagnosed and treated are described. The first case, a diabetic, developed a pyogenic abscess caused by klebsiella, while the other suffered a splenic infarct complicated by splenic abscess. although relatively easy to treat, solitary abscess of the spleen is a potentially lethal condition due to diagnostic difficulties. In both present instances the final diagnosis was easily achieved before surgery by the aid of computerized tomography (CT). This investigation seems to be more accurate than 99Tcm-sulphur colloid scan and ultrasound scan, which failed in both cases to diagnose splenic abscess clearly. The use of this investigation is suggested in every suspected case and will, no doubt, confirm the diagnosis at an early stage of the disease. Splenectomy with antibiotic cover will usually result in cure.

Abscess↗

[Spontaneous splenic rupture after mitral valve replacement for infective endocarditis].

We report a successful treatment of massive bleeding due to spontaneous splenic rupture after mitral valve replacement. A 61-year-old man was admitted to our hospital for intermittent high fever. An echocardiogram demonstrated a large vegetation on the posterior cusp of the mitral valve and mitral regurgitation of moderate degree. Staphylococcus epidermidis was cultured from his arterial blood. He underwent a mitral valve replacement after 3 weeks of antimicrobiological therapy with penicillin G crystalline and minocycline hydrochloeide. The patient fell into hemorrhagic shock on postoperative day 11 after complaining dull pain on his left upper abdomen for 3 days. A computed tomography demonstrated a splenic rupture and massive hematoma in the retroperitoneum. A splenic arterial embolization was done before splenectomy. The blood and clot of 2800 g were sucked from peritoneal and retroperitoneal cavities. There were no mycotic aneurysms nor abscess but the torn capsule on the swelled and partially necrotic spleen. The patient discharged uneventfully on postoperative day 43. Infective endocarditis frequently causes splenic infarction but rarely splenic rupture. Anticoagulation therapy after mitral valve replacement might have emphasized the bleeding in the patient.

Endocarditis, Bacterial↗

Partial splenic embolization for hypersplenism concomitant with or after arterial embolization of hepatocellular carcinoma in 30 patients.

PURPOSE: To study the value of partial splenic embolization (PSE) for the treatment of hypersplenism in patients undergoing embolization of hepatocellular carcinoma (HCC). METHODS: Transcatheter hepatic arterial embolization (THAE) combined with PSE was performed in 30 patients with HCC complicating liver cirrhosis, portal hypertension, and hypersplenism. Gelfoam sponge was used as the embolic material for PSE and limited to 100-150 pieces. RESULTS: More than 50% of splenic parenchyma was infarcted in 27 patients. Leukopenia and thrombocytopenia were corrected by PSE in 25 of 27 patients with hypersplenism. In 26 patients with esophageal varices, including 5 patients with bleeding, no rebleeding occurred during a 6-17 month follow-up. Hypersplenism was not corrected in 2 of 3 patients whose infarcted splenic parenchyma was less than 50%. No splenic abscesses or other severe complications were observed. Of the 30 patients treated, 19 are still alive after 1 year. CONCLUSIONS: THAE combined with PSE is a safe and effective measure for patients with HCC.

Adult↗

Laparoscopic-assisted surgery of the spleen: clinical experience in expanding indications.

Between January 1993 and November 1995 laparoscopic surgery was used in 21 patients with a variety of splenic diseases, namely idiopathic thrombocytopenic purpura, congenital spherocytosis, lymphoma, leukemic infiltrative disease, splenic infarction, trauma, or splenic cyst. Total splenectomy was carried out laparoscopically in 16 patients. Conversion to open splenectomy was necessary in two other patients because of intractable bleeding. Two patients with a splenic cyst underwent laparoscopic unroofing of the cyst. Conservative hemostasis of a spleen injury grade II was carried out in a child after blunt trauma. The total mean duration of the laparoscopic procedures was 158 min and the mean blood loss volume was 350 ml, both conversions being excluded. Postsurgical recovery was excellent and the average hospital stay was 5 days, including the patients with conversion. There were no significant postoperative complications. Our observations indicate that an increasing number of surgical diseases of the spleen can be managed adequately by a less invasive laparoscopic approach. However, the criteria for using this procedure are in an expanding phase and are still mainly dependent on the surgeon's technical experience.

Adolescent↗

Cerebral, myocardial and cutaneous ischemic necrosis associated with calcific emboli from aortic and mitral valve calcification in a patient with end-stage renal disease.

We report the case of a 57-year-old diabetic male with chronic renal failure who developed secondary hyperparathyroidism and calcification of mitral and aortic valves and interatrial septum. Multiple ischemic lesions developed in the skin of hands, feet and penis, and in the brain, and these were presumed to be due to septic emboli from cardiac valvular infective endocarditis. Multiple blood cultures were negative, however, and despite antibiotic therapy the patient expired. Autopsy (limited to trunk) demonstrated multiple calcific emboli in the heart and spleen, apparently derived from the prominent calcific deformities in the aortic and mitral valves. These were associated with acute and organizing myocardial infarcts and acute splenic infarcts, suggesting that the multiple ischemic lesions in the brain were also due to calcific emboli. A possible contributory component of infective endocarditis, however, was indicated by postmortem cultures of aortic and mitral valves positive for Enterococcus faecium. Calcific embolism is a rarely recognized but potentially lethal complication of end-stage renal disease, and the clinical diagnosis and the preventive therapeutic options for the control of the product of calcium and phosphate and/or parathyroidectomy should be considered.

Aortic Valve↗

Splenic artery ligation in selected patients with hepatic cirrhosis and in Sprague-Dawley rats.

The effects of splenic artery ligation were studied in Sprague-Dawley rats and in eight selected symptomatic patients with hepatic cirrhosis. In rats, this maneuver induced splenic infarction, reduced functional splenic mass, transiently raised platelet and reticulocyte counts and was without local complications. In seven selected patients with cirrhosis and prominent splenomegaly, the splenic artery was markedly enlarged, splenic arterial flow was greatly increased and splenic artery ligation partially lowered portal pressure. In three patients with varying cytopenias secondary to hypersplenism splenic artery ligation uniformly improved peripheral blood elements, although varying degrees of hypersplenism later recurred necessitating splenectomy in one. In five other patients, splenic artery ligation in conjunction with coronary vein ligation in four was performed for bleeding esophageal varices. Two patients later required portacaval shunting, and one other in whom operation was undertaken in desperation died of hepatic failure. Celiac-mesenteric arterioportography, operative portography, hemodynamic measurements and examination of peripheral blood elements in these eight patients suggests that splenic artery ligation in conjunction, where appropriate, with coronary vein ligation has several potentially beneficial effects. Hypersplenism may be sufficiently controlled to alleviate clinical symptoms. Arterial inflow into the portal system is reduced tending to lower portal pressure. Transheptic portal flow from the mesenteric bed is preserved. Venous anastomotic channels still functioning around the splenic pedicle and no longer draining a hyperdynamic splenic circuit may be converted into an escape route for mesenteric venous blood entering the portal system under high pressure. Nonetheless, each of these effects and their interrelationships require further study before this operation assumes a larger role in the treatment of complications of portal hypertension.

Animals↗

Enlarged spleen syndrome.

Enlarged spleen without a clear-cut etiology, and believed to be related to malarial infestations, has been referred to as "tropical splenomegaly" and "cryptogenetic splenomegaly." Splenectomy performed in such cases after a failure of antimalarial therapy shows histopathologically, while some of these meet the criteria for tropical splenomegaly, that most were cases of splenic abscess in various stages of formation, and some were lymphosarcoma. In sickle cell disease, one sequence of events involving the spleen is intermittent painful splenic infarction leading to splenic abscess or shrunken, fibrotic, and probably functionless spleen. It is proposed that an elective splenectomy may be indicated in cases of sickle cell disease to forestall this outcome. It is also proposed that an enlarged spleen that fails to respond to antimalarial medication after a certain period should be removed to establish the correct diagnosis, to prevent rupture, and to determine definitive management.

Adolescent↗

The spleen and its vasculature in pancreatitis: CT findings.

The spleen and its vasculature are susceptible to damage from pancreatic inflammatory exudates. Fourteen patients were identified who demonstrated splenic or splenic vascular involvement from pancreatitis on computed tomography. Findings included intra- and perisplenic inflammatory fluid collections (n = 6), acute splenic hematomas (n = 3), splenic infarction (n = 1), splenic artery pseudoaneurysm (n = 1), and splenic vein thrombosis (n = 6). Eight of the 14 patients went on to urgent interventions including percutaneous catheter drainage (n = 2) and transcatheter embolotherapy (n = 6) based on the CT findings.

Aneurysm, False↗

Torsion of the wandering spleen.

A case of torsion of the wandering spleen with splenic infarction is reported. The medical imaging studies performed with typical findings are described. Case reports have been reviewed. Although rare, torsion of the spleen remains an important differential diagnosis in patients presenting with acute abdominal pain. Early intervention is necessary to reduce the risk of splenic infarction and other complications, and an increased awareness of the condition together with the use of appropriate medical imaging can lead to the correct diagnosis more readily.

Abdominal Pain↗

Isolated dissection of the celiac artery--a case report.

Isolated arterial dissection, which occurs with the absence of aortic dissection, has been reported in carotid and renal arteries but rarely in visceral arteries. A case of isolated celiac artery dissection is reported here. A healthy 58-year-old man experienced sudden upper abdominal pain, which continued for several days. A body computed tomogram (CT) showed a multiple low-density wedge-shaped area in the spleen, which was diagnosed as splenic infarction, and an aneurysm with thrombus in the celiac artery. A selective angiogram showed dilatation of the celiac artery with wall irregularity, and proximal occlusion of the hepatic artery. The distal hepatic artery was fed by collateral arteries from the superior mesenteric artery. Splenic infarction was probably due to the embolism from the thrombus in the dissected celiac artery. The absence of other vascular lesions and causes or risks for the arterial dissection would suggest the occurrence of spontaneous dissection. The dissection of visceral arteries should be considered in diagnosing acute abdominal pain.

Abdominal Pain↗

Splenic and perisplenic involvement in acute pancreatitis: determination of prevalence and morphologic helical CT features.

PURPOSE: The purpose of this work was to determine the prevalence and morphologic helical CT features of splenic and perisplenic involvement in patients with acute pancreatic inflammatory disease in correlation with the severity of the pancreatitis. METHOD: One hundred fifty-nine contrast-enhanced helical CT scans of 100 consecutive patients with acute pancreatitis were reviewed retrospectively and independently by three observers. CT scans were scored using the CT severity index (CTSI): Pancreatitis was graded as mild (0-2 points), moderate (3-6 points), and severe (7-10 points). Interobserver agreement for both the CTSI and the presence of splenic and perisplenic involvement was calculated (K statistic). Correlation between the prevalence of complications and the degree of pancreatitis was estimated using the Fisher exact test. RESULTS: The severity of pancreatitis was graded as mild (n = 59 scans), moderate (n = 82 scans), and severe (n = 18 scans). Splenic and perisplenic abnormalities detected included perisplenic inflammatory fluid collections (95 scans, 58 patients), narrowing of the splenic vein (35 scans, 25 patients), splenic vein thrombosis (31 scans, 19 patients), splenic infarction (10 scans, 7 patients), and subcapsular hemorrhage (2 scans, 2 patients). No cases of splenic artery pseudoaneurysm formation, intrasplenic venous thrombosis, intrasplenic pseudocysts, or abscesses were detected. The interobserver agreement range for scoring the degree of pancreatitis and the overall presence of abnormalities was 75.5-79.2 and 71.7-100%, respectively. A statistically significant difference between the presence of abnormalities and the severity of pancreatitis was observed (p < 0.001). CONCLUSION: Splenic vein thrombosis (19%) and splenic infarction (7%) are relatively common CT findings in association with acute pancreatitis. The CTSI proves to be accurate in predicting these complications as there is a statistically significant correlation between the prevalence of these complications and the severity of pancreatitis.

Acute Disease↗

Acquired and transient RBC CD55 deficiency (Inab phenotype) and anti-IFC.

BACKGROUND: Antigens of the Cromer blood group system reside on the glycoprotein CD55 (decay-accelerating factor). The Inab phenotype is the null phenotype of this system. So far, only five propositi have been described who exhibit this phenotype, and single-nucleotide substitutions in the CD55 gene have been found in three of them. This report describes the first example of a patient with an acquired and transient form of the Inab phenotype. CASE REPORT: A 54-year-old black patient was admitted to the hospital because of abdominal pain. Multiple splenic infarctions were visualized in the abdominal computerized tomography scan, and a prophylactic splenectomy was performed. The patient's serum reacted by an IAT with all donor RBCs tested. RESULTS: Serologic analysis showed that the patient had the rare Inab phenotype and that his serum contained anti-IFC. Flow cytometry demonstrated the absence of CD55 on his RBCs, whereas lymphocytes, monocytes, granulocytes, and platelets expressed CD55, albeit at a weaker level than cells of common phenotypes. cDNA revealed no differences from the published sequences. Flow cytometry performed 12 months after splenectomy showed reappearance of the CD55 antigen; serologic tests performed after 17 months revealed that the anti-IFC had almost disappeared and that the RBCs were again agglutinated by various Cromer antibodies. CONCLUSION: A patient with an acquired and transient form of the Inab phenotype is described, in whom the CD55 deficiency is limited to the RBCs and is associated with splenic infarctions.

Abdominal Pain↗

Torsion of a wandering spleen in a one month old, with a confusing ultrasound examination.

Torsion of a wandering spleen is an uncommon diagnosis and rare in children. The entity is commonly misdiagnosed. In our case, a one month old infant presented with an abdominal mass. Ultrasound identified a large heterogeneous mass but also revealed what appeared to be normal splenic tissue below the left hemidiaphragm. The ultrasound findings steered us away from the diagnosis of wandering spleen or splenic infarction. Differential diagnosis of the mass included twisted hemorrhagic ovarian cyst, neoplastic mass, or inflammatory abscess. Postoperative histologic analysis demonstrated splenic infarction.

Diagnosis, Differential↗

The risks of total pancreatectomy and splenic islet autotransplantation.

The intraportal site is the most common site for islet transplantation. Many other sites have been tried experimentally, including the spleen, which has successfully lead to insulin independence in a number of animal models. Nevertheless, there are no detailed reports of total pancreatectomy and splenic islet autotransplantation in humans. Five patients underwent total pancreatectomy and splenic islet autotransplantation for chronic pancreatitis. Four patients had a pylorus-preserving total pancreatectomy and one patient a duodenal-preserving pancreatectomy. In three cases islets were embolized into both the portal vein and spleen. Two patients received splenic islet transplants alone. Islets were transplanted by retrograde venous infusion via the short gastric veins (n = 3), splenic vein stump (n = 1), and the left gastroepiploic vein (n = 1). The total volumes of transplanted pancreatic digest in those receiving combined intraportal and splenic autografts (n = 3) were 15.8, 13.0, and 13.5 ml. The volumes in those receiving a splenic-alone autograft (n = 2) were 12.0 and 5 ml. The mean rise in portal pressure was 18 cm of water. Complications related to the splenic autograft included a wedge splenic infarct, an emergency splenectomy, and a portal vein thrombosis in one patient having a combined intraportal and splenic autograft. Two patients developed insulin independence. two patients were still insulin independent at 1-year follow-up, and all had normal HbA1c levels (mean 5.6, range 5.2-6.3). Splenic islet autotransplantation, after total pancreatectomy, does lead to insulin independence. However, in our experience the combined procedure has a high morbidity because of splenic infarction and venous thrombosis.

Adult↗

Splenic and renal infarction in systemic lupus erythematosus: association with anti-cardiolipin antibodies.

A 37-year-old female, known to have systemic lupus erythematosus (SLE) and markedly raised anti-cardiolipin antibody levels in association with the lupus anticoagulant, presented with a symptomatic segmental splenic infarction. There was a past history of cerebral infarction. Abdominal computed tomography (CT) demonstrated the area of splenic infarction, and an asymptomatic right renal infarct. This patient illustrates the unusual occurrence of multiple visceral infarcts, in association with anti-cardiolipin antibodies, complicating SLE.

Adult↗