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PubMed1980

Polycythemia vera in childhood: case report and review of the literature.

A patient presented at 5 years of age with polycythemia vera. He subsequently developed splenic infarctions and died at 20 years of age following cerebral hemorrhage and infarctions. Two months before his death, he developed hypertension and had biochemical evidence of primary hyperparathyroidism and possibly pheochromocytoma. Only nine reported childhood cases of polycythemia vera fulfill the criteria of the Polycythemia Vera Study Group. These cases are summarized and the complications discussed. Although none have progressed to myeloid metaplasia or acute leukemia, these patients are at risk of developing thrombo-hemorrhagic complications; available evidence indicates that they should be managed to keep the hematocrit between 40 and 45%.

Bloodletting↗
PubMed1991

Severe falciparum malaria (21 cases).

The incidence of severe falciparum malaria is increasing in the developed countries and mortality remains high despite progress in intensive care management and schizonticide treatment. Many authors emphasize the importance of exchange transfusion (EXT) in the most severe cases. We studied 21 cases (34 +/- 12 years, 6 females; SAPS: 8.4 +/- 3.7) of severe malaria (according to WHO criteria) consecutively admitted to ICU between 1985 and 1990: 3 patients underwent EXT. Twenty were febrile above 39 degrees C, 10 had cerebral malaria, 14 hepatic impairment, 8 acute renal failure, 5 pulmonary oedema. Nine patients required mechanical ventilation, 1 haemodialysis, 1 intracranial pressure monitoring. Mean parasitemia was 13%, 16 patients had thrombocytopenia less than 50 x 10(9)/l, 3 anemia less than 7 g/dl and 3 leucopenia less than 2.8 x 10(9)/l. Nineteen received quinine i.v., 1 mefloquine, 1 chloroquine. Sixteen patients received blood products transfusion, 3 were treated by EXT in addition. Twenty were cured and discharged from hospital without sequelae (mean stay: 14 days); 4 had nosocomial infection, 1 a splenic infarction. One patient (17-years-old; SAPS: 17; parasitemia: 7.8%) died 12 h after admission from non-cardiogenic pulmonary oedema with multi-organ failure. The literature and this study lead us to propose EXT in patients with unfavourable evolution after conventional treatment rather than in all the patients with a parasitemia above 10% at admission. A randomized study to compare conventional treatment in ICU with or without EXT is necessary.

Adolescent↗
PubMed2000

Infective endocarditis affecting both systemic and pulmonary circulations predisposed by a ventricular septal defect.

A 39-year-old woman was admitted to our hospital presenting persisting fever. An echocardiographic examination showed severe aortic and mitral valve regurgitation with moderate tricuspid regurgitation. Small left-to-right shunt through the ventricular septal defect was identified. Vegetation was also detected on the tricuspid, mitral, and aortic valves. At one month after admission, the patient showed sudden onset of headache and abdominal pain. A computed tomographic scan demonstrated cerebral and splenic infarction. A pulmonary perfusion scintigram demonstrated perfusion defects in left-S1 and right-S6 regions. At 4 months after admission, as operation was performed. The aortic valve was replaced with a #23 mm CarboMedics prosthesis and the mitral valve with a #29 mm Carbo Medics prosthesis. Tricuspid valve plasty was performed, with closure of He laceration and perforation of the anterior leaflet combined with a commissuroplasty, according to Kay's method. Ventricular septal defect was closed with a bovine pericardial patch. She was discharged at 19 days after the operation, and is leading a good life. Pervasion of the organism seemed to be initiated from the mitral valve which was conveyed by the blood stream to the aortic valve, and to the tricuspid valve through the ventricula septal defect. Left heart evaluation may be important in cases with infective endocarditis and ventricula septal defect.

Adult↗
PubMed2005

Vascular involvement secondary to tuberculosis of the abdomen.

Although involvement of the abdomen is a common site of extrapulmonary tuberculosis, involvement of abdominal vessels with complications is rare after infections with Mycobacterium tuberculosis (M. tuberculosis). Vascular complications may result from direct involvement by M. tuberculosis of the vascular wall or may occur as a consequence of local spreading from a tuberculous mass. We describe the imaging findings in two patients with proven tuberculosis of the abdomen and significant vascular complications. In one patient, "dry plastic" peritonitis and tuberculous lymphadenopathy at the level of the porta hepatis with subsequent encasement and compression of the portal vein resulted in prestenotic dilatation and varicose formation in the upper abdomen. In the other patient, bulky necrotic tuberculous lymphadenopathy, located at the splenic hilum, resulted in encasement with stenosis of the splenic vessels and subsequent splenic infarction.

Adult↗
PubMed2001

Ineffectiveness of interferon-gamma in the treatment of idiopathic myelofibrosis: a pilot study.

It has been proposed that interferon-gamma (IFN) inhibits collagen synthesis in myeloproliferative disorders through an inhibitory effect on PDGF and TGF-beta. We therefore evaluated the role of IFN-gamma on bone marrow fibrosis in idiopathic myelofibrosis (IMF). After a 3-month observation period, nine patients (five female, four male), median age 64 years (range 43-72 years), received 3 x 3 mU IFN-gamma/week over 6 months and were monitored after withdrawal of IFN-gamma for further 3 months. Three out of nine patients have completed the study according to the protocol. Six patients had to be withdrawn from IFN-gamma due to the following reasons: bacterial infection (three patients), splenic infarction or deterioration of splenomegaly (one patient, each) and refusal to continue IFN-gamma (one patient). Results from seven patients treated for at least 8 weeks were considered measurable. Leukopenia, initially present in one of the evaluated patients, deteriorated during IFN-gamma treatment. This patient died during the observation period shortly after withdrawal of the therapy as a result of septicemia. Transfusion-dependent anemia, initially observed in two of the evaluated patients, deteriorated during the IFN-gamma treatment. Bone marrow fibrosis increased in three patients, whereas it remained unchanged in another and improved in a further patient. Splenomegaly improved in two patients but deteriorated markedly in one. Taking these observations together, four patients had disease progression during IFN-gamma treatment, two had stable disease and one could be qualified as a partial responder. According to these data IFN-gamma cannot be considered as a treatment option for patients with IMF.

Adult↗
PubMed2003

Treatment of six hepatic artery aneurysms.

Hepatic artery aneurysms are rare lesions but of significant clinical importance because rupture is associated with elevated mortality. Although diagnosis using CT scanning and, more importantly, angiography has been well defined, the therapeutic choices are less clear. We retrospectively selected patients from 1985 to 2000 who were treated with either traditional surgical or percutaneous techniques. In total we treated six patients, four males and two females. This represents 17.6% of 34 patients treated by us for splanchnic artery aneurysms. The treatment was surgical in two cases (33.3%) and percutaneous in four cases (66.6%). All lesions were successfully treated with exclusion/ablation of the aneurysm. Mortality was nil; in one of the surgical cases we reported a transient hepatic failure and in the endovascular group, one right pleural effusion, one small splenic infarction, and one pseudoaneurysm of the gastroduodenal artery. The first therapeutic strategy to be taken into consideration is always the percutaneous approach. However, surgery still has a role in those cases where the lesion cannot be repaired percutaneously. Based on our own experience (good results, no mortality, and few complications) and in accordance with the literature, it is clear that an aggressive approach is warranted in those patients.

Aged↗
PubMed1992

Surgical complications in children after liver transplantation.

The frequency of surgical complications after liver transplantation remains high. Sixty transplants were done in 48 patients during 4 years. Eleven patients were retransplanted (re-transplant rate, 20%) for primary nonfunction (6), arterial thrombosis (3), warm ischemia (1), and rejection (2). Right pleural effusions were drained in 13 patients and left ones in 2. Forty-eight re-explorations excluding retransplantation were performed in 20 patients. Twelve laparotomies were for control of postoperative intraabdominal bleeding. The majority of these patients (8/10, 80%) were transplanted with reduced-size grafts. Early postoperative vascular complications were detected in 12 grafts (5 portal vein occlusions, 7 arterial thromboses). All 5 patients with portal vein (PV) occlusions were reexplored, and PV flow was reestablished in all 5. Biliary leaks were diagnosed in 6 patients and were associated with arterial thromboses in 2 cases. Reoperation was required in 4 of 6 patients. Bowel perforation occurred in 4 patients; 2 small bowel, 1 duodenum, and 1 colon. There was 1 postoperative bowel obstruction requiring laparotomy. Two splenectomies were required in 4 patients with splenic infarction. Resection of part of a transplanted liver was done in 1 patient to exclude septic infarcts. Pancreatitis was diagnosed in 4 patients and one required laparotomy for control of pancreatic hemorrhage. Intraabdominal abscesses required open drainage in 2 patients and percutaneous drainage in 4. Seven thoracotomies were done in 6 patients: 5 open lung biopsies, 1 for control of hemorrhage, and 1 for diaphragmatic plication. The current high survival rates following liver transplantation require aggressive surgical management of a myriad of complications and numerous procedures are necessary both as treatment modalities and as diagnostic aids.

Adolescent↗
PubMedDate not supplied

CT manifestations of infectious mononucleosis.

A case of infectious mononucleosis (IM) which, on computed tomography (CT) scan, mimicked the morphologic features of lymphoma is reported. The CT findings in this case include generalized lymphadenopathy, splenomegaly, and focal low-attenuation splenic lesions in a fifty-three year old woman; these findings have not previously been described in patients with IM. This is most likely because IM is usually a clinical diagnosis confirmed by serologic testing. Imaging modalities such as CT scan have not routinely been utilized to support this diagnosis.

Diagnosis, Differential↗
PubMed2006

[Gas embolism during protective ventilation for acute respiratory distress syndrome].

A 34-year-old woman with toxic coma developed inhalation pneumonia complicated by the acute respiratory distress syndrome. Marked parenchymal destruction and recurrent pneumothorax occurred despite protective ventilation. Altered consciousness persisted after sedative withdrawal, and the patient subsequently died. Computed tomography revealed multiple cerebral, renal and splenic infarcts. The only identified cause of systemic embolism was multiple gas embolisms. We discuss the physiopathological mechanisms, and the diagnostic and therapeutic management of such patients.

Adult↗
PubMed2004

Splenic and mesenteric emboli in a patient with protruding aortic arch atheroma.

Protruding atheromas in the thoracic aorta are an important cause of embolic disease. Transesophageal echocardiography (TEE) is the modality of choice for diagnosis of these lesions. We present a patient with splenic infarction in whom TEE revealed a large mobile atheroma in the aortic arch. A few hours following the disappearance of this mass from the aortic arch, the patient developed mesenteric artery embolism requiring subtotal small-bowel resection. We discuss the importance of the aortic arch as a source of peripheral emboli and the treatment modalities in these patients.

Journal Article↗
PubMed2006

Bartonella quintana prosthetic valve endocarditis detected by blood culture incubation beyond 10 days.

We describe the case of a 45-year-old man with mitral and aortic prosthetic valve replacement who presented with symptoms of subacute bacterial endocarditis. Bartonella quintana was grown from blood after prolonged culture. The course of the disease was complicated by splenic infarction, glomerulonephritis resulting in progressive renal insufficiency, and cerebroventricular hemorrhage. Notably, cardiac ultrasonography showed no extensive vegetations but a strand-like lesion. Culture-positive B. quintana prosthetic valve endocarditis in a formerly healthy subject represents a newly observed entity. It should be added to the differential diagnosis of prosthetic valve endocarditis, especially when it presents with features suggesting subacute bacterial endocarditis.

Journal Article↗
PubMed2005

Laparoscopic pocket splenopexy for wandering spleen: a case report.

Wandering spleen is a very uncommon condition. Because of the risk of pedicle torsion and splenic ischemia, severe consequences may occur if not diagnosed and treated in time. Unfortunately, splenectomy is sometimes necessary (ie, when splenic infarction occurs). Once the diagnosis of wandering spleen is made, splenopexy is the treatment of choice. There are numerous techniques designed for splenopexy, either by open surgery or by minimally invasive approaches. We describe here a laparoscopic procedure that allows an excellent fixation of the spleen using the patient's own tissues. After a 2-year follow-up, the organ remains in place with good perfusion.

Abdominal Pain↗
PubMed2005

Wegener's granulomatosis presenting during pregnancy with acute limb ischemia.

We report a rare case of a pregnant woman with Wegener's granulomatosis whose disease involved the lungs, the spleen, and a limb. Wegener's granulomatosis was clinically manifested in the 34th week of pregnancy with pulmonary infiltrates, splenic infarcts, and acute limb ischemia. Successive thrombectomies failed to maintain arterial flow in the distal limb due to the development of active vasculitis. Thrombosis of the tibial arteries and recurrence of thrombosis was a persistent clinical observation. In the meantime, a cesarean section was performed, with a successful delivery of a healthy male newborn. Because of the recent cesarean section, the patient did not undergo thrombolysis. The woman finally underwent amputation of the limb. According to the reviewed literature, this is the sixth reported case of Wegener's granulomatosis presenting with digital ischemia and the first manifested during pregnancy.

Acute Disease↗
PubMed1998

[Gastrointestinal hemorrhage caused by rupture of an aneurysm of visceral arteries. Presentation of 4 cases].

STUDY AIM: Gastrointestinal bleeding by rupture of splanchnic artery aneurysms is very rare. The aim of this study is to report four cases observed between 1990 and 1996. MATERIALS AND METHODS: In the first case, the celiac trunk aneurysm was revealed by hematemesis due to erosion of the posterior wall of the stomach. Excision of the aneurysm associated with splenopancreatectomy was followed by revascularization of the common hepatic artery with a bypass implanted in the aorta. The second case concerned a splenic artery aneurysm revealed by hemosuccus pancreaticus and intestinal bleeding which was treated by excision and splenopancreatectomy. In the third case, the common hepatic artery aneurysm revealed by hemosuccus pancreaticus and intestinal bleeding was treated by obstructive endoaneurysmorrhaphy. The fourth case concerned a superior mesenteric aneurysm revealed by duodenal erosion causing gastric and intestinal bleeding, which was treated by obstructive endoaneurysmorrhaphy and revascularization of the mesenteric artery by a spleno-mesenteric bypass. RESULTS: Surgical treatment was successful in all four patients. In the first case, an acute acalculous cholecystitis required a cholecystectomy after 3 weeks. In the fourth case, a splenic infarction disappeared spontaneously. CONCLUSION: Such observations are rare. The site of the bleeding was located by endoscopy. The aneurysm was recognized by contrast-enhanced computerized tomography (CT) scan and/or celiac and mesenteric arteriography which was performed in all cases and was very useful for the management of such aneurysms. After excision (n = 2) or obliterative endoaneurysmorrhaphy (n = 2), revascularization had to be done in two cases for celiac and mesenteric aneurysms.

Adult↗
PubMed2003

[Laparoscopic pancreatectomy: report of 22 cases].

OBJECTIVE: To evaluate results of laparoscopic pancreatectomy for benign lesions of the pancreas. Peri-operative data, surgical outcomes and techniques are presented. PATIENTS AND METHODS: Eighteen women and four men underwent laparoscopic pancreatectomy and were collected retrospectively from 1999 to 2003. RESULTS: Laparoscopic pancreatectomy was attempted in 22 patients and completed successfully in 18: 10 enucleations, three distal pancreatectomies, four left pancreatectomies and one total pancreatectomy for endocrine and cystic tumors. Left and distal pancreatectomies were performed with preservation of the spleen. Four patients were converted (one enucleation, one whipple procedure and two left pancreatectomy). There was no mortality; the post-operative morbidity included two pancreatic leaks and one case of half splenic infarction. The median length of hospital stay was 12 days. CONCLUSION: Patients appear to benefit from laparoscopic pancreatectomy for pancreatic benign tumors.

Adult↗
PubMed1990

CT features of histiocytic medullary reticulosis.

Five cases of histiocytic medullary reticulosis (HMR) are presented and the computed tomographic (CT) findings described. All patients were young adults in their early twenties with fever, malaise and weight loss as their chief complaints. Survival was less than 5 months in all cases. The CT findings were hepatosplenomegaly (5), abdominal lymphadenopathy (5) and bilateral renal enlargement (2). Patchy low attenuation areas in the liver (2), wedge shaped splenic infarcts (2) and focal low attenuation areas in the kidneys (1) were also noted. Ascites (3) and pleural effusion (3) were associated findings.

Adult↗
PubMed1985

Nocardia asteroides aortitis with perforation of the aorta.

A 53-year-old man died of nocardial aortitis eight months after undergoing aortic valve replacement. Autopsy revealed vegetation in the area of a previous aortotomy incision and a small perforation of the aorta enclosed by pericardium immediately above the prosthetic valve ring. Prosthetic valve endocarditis was not present. Multiple splenic infarcts, microabscesses of both kidneys, and myocarditis were identified. Antemortem blood cultures and postmortem cultures of the aortic vegetation grew Nocardia asteroides.

Aortic Rupture↗