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Cases from the osler medical service at Johns Hopkins University.

PRESENTING FEATURES: A 29-year-old woman with a history of rheumatic heart disease and one episode of endocarditis as an adolescent was admitted to the hospital after 1 week of headache, fever, and myalgia. Her past medical history was otherwise unremarkable and did not include illicit drug use. On physical examination, she had a previously noted 3/6 holosystolic murmur at the apex, which radiated to her back; a previously noted 1/4 diastolic murmur at the right upper sternal border; diminished strength in her right upper extremity; multiple painful erythematous nodules on her fingers (Figure 1); and red streaks under her nails (Figure 2). Magnetic resonance imaging of the brain demonstrated multiple lesions; the largest was in the right frontal lobe with associated hemorrhage (Figure 3). What is the diagnosis?

Adult↗

Acute pancreatitis with pseudocyst formation in a patient with polyarteritis nodosa.

Polyarteritis nodosa (PAN) is a term that includes patients with necrotizing inflammation of medium sized arteries, and excludes those with microscopic vessel involvement. Its manifestations are protean and include constitutional symptoms such as fever, malaise, weight loss, myalgia, peripheral neuropathy, rash, and gut and renal involvement. Although gastrointestinal manifestations have been noted in up to a third of patients with PAN, clinical presentation with pancreatic involvement has been reported only rarely. We describe a patient with PAN who developed acute pancreatitis with pseudocyst formation as well as infarcts in the spleen and liver.

Acute Disease↗

[Wandering spleen torsion].

The ectopic spleen characterizes for absence of its suspensory ligaments and a long pedicle that are predisposed to complicate it for a torsion with commitment of the venous drainage at first and arterial at a later time himself, producing increase of its volume and infarct. This anomalous situation, it can be had to a congenital malformation of the development of its suspensory elements and fall toward the inferior abdomen or else to an inferior growth of the mesodermic yolk of that this organ originates itself. The suitable treatment is the laparoscopic splenopexy, but when it exists infarction, it is no possible avoid the splenectomy.

Child↗

Splenic rupture complicating periinterventional glycoprotein IIb/IIIa antagonist therapy for myocardial infarction in polycythemia vera.

Polycythemia vera is a myeloproliferative disorder predisposing to thromboembolic and bleeding complications. We report the case of a patient with polyglobuly, leukocytosis, and thrombocytosis, who suffered from acute ST-segment elevation myocardial infarction due to thrombotic high-grade pre-stent stenosis two months after percutaneous coronary intervention for complex coronary one vessel disease. Following re-PTCA and stent implantation in conjunction with periinterventional GP IIb/IIIa antagonist treatment, the patient was initially symptom free for about two hours before rapidly developing signs of a hemorrhagic shock. An abdominal CT scan showed splenic rupture with massive intraabdominal hemorrhage as a consequence of secondary bleeding into multiple pre-existing splenic infarctions. The patient's condition stabilized after emergency splenectomy. Subsequent bone marrow biopsy revealed the presence of polycythemia vera. Post-operatively, the patient was treated with the anti-platelet agents aspirin and clopidogrel to prevent subacute stent thrombosis. Additionally, cyto-reductive therapy with hydroxyurea was initiated because of a further increase in the platelet count. In patients with polycythemia vera, the indication for treatment with GP IIb/IIIa antagonists should be carefully weighed against the potentially serious bleeding complications. Should treatment be established, a risk stratification using abdominal sonography and bleeding time testing is recommended, while during treatment red blood count, platelet count, coagulation tests, and hemodynamic parameters should be closely monitored.

Abciximab↗

Torsion of accessory spleen in an adult patient: imaging findings at CT, MRI and angiography.

A 40-year-old woman presented with acute left upper quadrant pain due to torsion of an accessory spleen around its long vascular pedicle, causing infarction. Torsion of an accessory spleen is extremely rare. As far as we known only 14 cases have previously been reported in the literature, and more than half patients were children. MRI can be helpful in the differential diagnosis of infarction by suggesting haemorrhagic necrosis on the T2-weighted images.

Abdominal Pain↗

[Salmonella enteritidis splenic abscess complicating a Plasmodium falciparum malaria attack].

INTRODUCTION: Salmonella splenic abscesses are rare and usually occur on pre-existing lesions. OBSERVATION: A Moorish 16 year-old woman from Senegal presented with a S. enteritidis abscess without any factor other than an attack of P. falciparum malaria. Treatment associated quinine salts, antibiotherapy and splenectomy. COMMENTS: P. falciparum malaria attacks not only induce humoral and then cellular immunodepression but are also at the origin of infarction or splenic hematoma that may enhance bacterial infection and the development of abscesses. Splenectomy or percutaneous drainage associated with antibiotherapy is the usual treatment for splenic abscesses. Prognosis remains severe (13 to 16% mortality).

Abscess↗

Radiology of the spleen.

The spleen is generally not considered a challenge to the radiologist. Most often it poses a problem by anomalies or an irregular but normal contrast enhancement; however, a variety of inflammatory, infectious and neoplastic diseases may involve the spleen. CT and ultrasonography are screening modalities for the spleen. For problem solving, MR imaging can be helpful, especially due to its free choice of the imaging plane and because of the high resolution in contrast MR imaging. Splenic angiography as a diagnostic tool has generally been replaced by CT, ultrasound, or MR and is now used as an interventional method, e. g., in non-surgical management of patients with chronic idiopathic thrombocytopenia or in patients with splenic trauma. This article reviews the radiology of the spleen, including anatomy, embryology, splenomegaly, splenic injury, infarction, cysts, tumors, abscesses, sarcoidosis, and AIDS. Knowledge about the use of different imaging modalities and underlying gross and microscopic pathologic features leads to a better understanding of the radiologic findings.

Diagnosis, Differential↗

Focal lesions of the spleen: preliminary results with fast MR imaging at 1.5 T.

Sixteen patients with splenic lymphoma and six with nonlymphomatous splenic lesions underwent magnetic resonance (MR) imaging, ultrasound (US), and dynamic CT. All patients were studied at 1.5 T with gradient echo sequences using a repetition time of 80 ms, echo time of 16 ms, and two pulse angles of 30 and 60 degrees. In 14 patients with lymphomatous lesions fast MR showed circumscribed areas of low signal intensity at both pulse angles. The lesion-to-spleen contrast was better on images acquired with a pulse angle of 30 degrees. For fast MR with pulse angles of 30 degrees, the mean lesion-to-spleen contrast was similar to US and contrast-enhanced CT. However, with fast MR the contrast showed a lower variability and was considerably better than with unenhanced CT. In one patient fast MR showed splenic involvement that was missed on both CT and US. The signal characteristics of lymphomatous, leukemic, and sarcoid involvement and of healed infarcts were similar and indistinguishable on fast MR images. Recent splenic infarctions (three cases) were, however, distinctly different, characterized by regions of high signal intensity at both pulse angles. The results of this preliminary study suggest that fast MR imaging is a promising diagnostic tool for the assessment of splenic disorders.

Diagnosis, Differential↗

Torsion of the spleen with incomplete infarction: case report.

Torsion and infarction of a "wandering spleen" is a rare disease which is often confused with other acute abdominal crises. A correct preoperative diagnosis, when made, has usually been determined by arteriographic studies. A child is described in whom changes in the TcSC scan made a correct diagnosis possible by non-invasive methods.

Child, Preschool↗

[Torsion and infarct of the spleen with benign lymphoid hyperplasia of the colon].

A case of an infant with vomiting and a palpable abdominal mass is discussed. His preoperative studies showed a double right collecting system and a benign lymphoid hyperplasia of colon. The laparotomy showed a spleen in an abnormal position with total ischemic necrosis due to torsion of a pedicle. Emphasis is made on the complementary studies to help the diagnosis.

Colon↗

[Development of mycotic aneurysms of the superior mesenteric artery after septic embolism].

Mycotic aneurysms of the aorta and the visceral arteries are life-threatening diseases, due to potential rupture and organ or limb ischemia. They occur in endocarditis, immunodeficiency, bacteremia and fungemia, and have a poor prognosis. We report on a case of a 54-year-old male patient suffering from abdominal angina after mitral valve replacement for septic mycotic endocarditis. In presence of a mycotic-embolic occlusion of the left popliteal artery and multiple septic organ infarctions a mycotic aneurysm of the superior mesenteric artery was found in abdominal spiral-CT. Based on sequential spiral-CT examinations, this case demonstrates the development of a septic aneurysm of the superior mesenteric artery.

Aneurysm, Infected↗

Infarction of accessory spleen.

A rare disease that can mimic acute appendicitis is infarction of accessory spleen. It may occur in children and is difficult to diagnose preoperatively. Greater awareness of this entity may show not only that it is associated with other intraabdominal anomalies but also that it is more common in the tropics than elsewhere. Two cases are reported.

Acute Disease↗

Idiopathic myelofibrosis associated with classic polyarteritis nodosa.

A woman with scleroderma and classic polyarteritis nodosa (PAN) who developed idiopathic myelofibrosis (IM) is reported. The patient presented with a one-year history of weakness, polyarthritis, Raynaud phenomenon, dry cough, and epigastralgia. The diagnosis of scleroderma with visceral involvement was made and treatment with prednisone subsequently started, with good clinical response. Six years later, fever, weight loss, livedo reticularis, and dysesthesias developed. Electromyographic studies were consistent with sensory neuropathy and a sural nerve biopsy yielded the diagnosis of PAN. The patient received cyclophosphamide plus prednisone with a favorable response, but 11 years later she was admitted because of weakness, constitutional symptoms, and abdominal pain due to spleen infarcts. Marked anemia, with aniso-poikilocytosis, tear-drop cells, immature myeloid precursors in the peripheral blood, and an increased serum LDH, was observed and the diagnosis of IM established by bone marrow biopsy. This case represents a new association between IM and an autoimmune disease and supports the hypothesis of an immune basis of IM in some patients.

Abdominal Pain↗

Complications of laparoscopic adrenalectomy: results of 169 consecutive procedures.

Laparoscopic adrenalectomy (LA) has become the gold standard for adrenalectomy. Review of the literature indicates that the rate of intra- and postoperative complications is not negligible. The aim of this study was to evaluate the complications observed in a series of 169 consecutive LAs performed at a same center for a variety of endocrine disorders. Between June 1994 and December 1998 a series of 169 LAs were performed in 159 patients: 149 unilateral LAs and 10 bilateral LAs. There were 98 women and 61 men with a mean age of 49. 7 years (range 22-76 years). There were patients with 61 Conn syndrome, 41 with Cushing syndrome, 1 androgen-producing tumor, 29 pheochromocytomas, and 37 nonfunctioning tumors. Mean tumor size was 32 mm (range 7-110 mm). LA was performed by a transperitoneal flank approach in the lateral decubitus position. Mean operating time was 129 minutes (range 48-300 minutes) for unilateral LA and 228 minutes (range 175-275 minutes) for bilateral LA. There was no mortality. Twelve patients had a significant complication (7.5%): three peritoneal hematomas requiring (in two cases) laparotomy and (in one case) transfusion; one parietal hematoma; three intraoperative bleeding episodes without need for transfusion; one partial infarction of the spleen; one pneumothorax; one capsular effraction of the tumor; and two deep venous thromboses. Eight tumors were malignant at final histology (4.7%), of which four were completely removed laparoscopically. Conversion to open surgery was required in eight cases (5%): for malignancy in four cases, difficulty of dissection in three cases, and pneumothorax in one case. With a mean follow-up of 26.58 months (range 6-60 months) all patients are disease-free. We conclude that LA is a safe procedure. With increasing experience the morbidity becomes minor. To avoid complications LA should be converted to open surgery if local invasion is suspected or if there is difficulty with the dissection.

Adrenal Glands↗