“Surgical” Abdomen in a Patient with Chronic Lymphocytic Leukemia: A Case of Acquired Angioedema

Introduction  

Acquired angioedema (AAE), an acquired deficiency of C1esterase inhibitor, is a medically treatable condition which can cause severe abdominal pain mimicking an acute surgical abdomen. This disorder is strongly associated with chronic lymphocytic leukemia (CLL) and other indolent lymphoplasmacytic disorders.     

Lemierre’s Syndrome in a Patient with Antiphospholipid Syndrome

Internal jugular vein thrombosis is usually associated with intravenous drug abuse, long-term venous catheterization, local infection, or spontaneous occlusion. The antiphospholipid syndrome is now recognized as one of the commonest causes of acquired arterial or venous thrombosis. Lemierre’s syndrome is an uncommon but potentially lethal complication of internal jugular vein thrombosis after an oropharyngeal infection. A 34-year-old man presented with fever and progressive, painful right neck swelling 6 days after an episode of pharyngitis. Clinical studies including radiological and laboratory tests confirmed the diagnosis of Lemierre’s syndrome associated with antiphospholipid syndrome. Although Lemierre’s syndrome is a well-known cause of internal jugular vein thrombosis, association with antiphospholipid syndrome is very rare.     

Renal Calculi in a Patient with Erdheim–Chester Disease

Erdheim-Chester Disease (ECD) is a non-inherited multifocal lipid storing histiocytosis. It is a rare disease characterised by lipid-laden monocyte infiltration of long bones causing cortical sclerosis and characteristic X-ray appearances. It also involves extraskeletal tissue in up to 50% of cases including retroperitoneal and renal infiltration. We report a patient with long standing ECD with widespread extraskeletal involvement, including significant renal infiltration, presenting with left hydronephrosis secondery obstruction from a proximal ureteric calculas.     

Treatment of a Left Common Iliac Aneurysm with Endovascular Powerlink® Stentgraft in a Patient with an Occlusion of a Left Common Iliac Stent

Isolated iliac aneurysms are uncommon and account for only 2% of all abdominal aneurysms. Typically, patients presenting with this pathology are operated on surgically. In our patient, however, surgery could have been an option but the patient had undergone a laparotomy more than 50 years earlier because of a gun-shot wound, so we preferred an endovascular repair. Furthermore, regular endovascular repair with a straight tube stentgraft was impossible due to the absence of a proximal neck. Consequently, we opted for placing a bifurcated stentgraft to exclude the iliac aneurysm.     

Esophagobronchial Fistula in a Patient with Behçet’s Disease: Report of a Case

Esophageal involvement in Behçet’s disease is generally considered to be very uncommon. So far, six cases of esophageal ulcers associated with perforation, penetration, or fistula in Behçet’s disease have been described in the English literature. This report describes esophagobronchial fistula in a patient with intestinal Behçet’s disease. A 62-year-old man was transferred to our hospital for peritonitis due to a small intestinal perforation after an appendectomy. At the age of 14 years he had had recurrent oral ulcers. Ulcerations of the ileum and epididymitis were found, and a pathological examination revealed nonspecific inflammation. Furthermore, an esophageal ulcer with esophagobronchial fistula was diagnosed. The fistula required not only endoscopic treatment but also surgical intervention. The patient’s clinical features were consistent with the active phase of intestinal Behçet’s disease. The symptoms gradually resolved without any treatment. Four years after remission, however, the symptoms recurred with gastrointestinal hemorrhage and polyarthritis. In the ileocolic region, punch-out ulcerations were noted. The clinical history and features led to a diagnosis of Behçet’s disease associated with recurrent gastrointestinal ulcerations. Steroid therapy (prednisolone, 20 mg daily) was started, and led to a rapid resolution of the symptoms. The patient is now being followed up as an outpatient while receiving prednisolone (10 mg per day), without complaint of any gastrointestinal symptoms.     

Transaortic Mitral Valve Repair Combined with a Modified Bentall Procedure in a Patient with Marfan’s Syndrome: Report of a Case

We report a case of transaortic mitral valve repair combined with aortic root and arch replacement in a patient with Marfans syndrome. Preoperative computed tomography and echocardiography showed acute aortic dissection (DeBakey type 1), severe aortic regurgitation, annuloaortic ectasia, and mild mitral regurgitation (MR). We performed artificial chordae implantation to the anterior mitral leaflet (AML) through the aortic root, followed by insertion of an aortic composite graft and replacement of the aortic arch. The patient is well 55 months after the operation, with minimal MR. We think that the transaortic approach is a good alternative for exposure and correction of the AML and its apparatus in special circumstances.     

Granulomatous Interstitial Nephritis in a Patient with Crohn’s Disease

A case of granulomatous interstitial nephritis (GIN) associated with Crohn’s disease (CD) was reported. GIN is a rare pathological finding in renal biopsy specimens. In a patient affected by CD, granulomas may be found in various tissues and organs such as lymph nodes, mesentery, liver, and lungs and occasionally in bones, joints, and skeletal muscle. Few cases of granuloma have been reported in the kidney, and it is not always possible to relate the presence of granuloma to CD, to other interstitial granulomatosis diseases, or to a drug-induced reaction. The issue has a remarkable clinical effect; indeed, the answer requires a completely different therapeutic approach. The diagnosis analysis on the basis of clinical–pathological evidences and on reports from literature is discussed.     

Coronary Artery Bypass Grafting Under Cardiopulmonary Bypass in a Patient with β-Thalassemia: Report of a Case

We performed successful coronary artery bypass grafting under cardiopulmonary bypass (CPB) in a man with β-thalassemia. Cardiopulmonary bypass for patients with hemoglobin disorders occasionally results in fatal hematologic complications. However, taking careful precautions such as transfusing stored autologous blood and administering haptoglobin may eliminate the need for allotransfusion, and prevent severe or persistent hemolysis and other complications. This case shows that cardiac surgery can be performed safely under CPB in patients with β-thalassemia.     

Primary Hepatic Non-Hodgkin’s Lymphoma in a Patient with Chronic Hepatitis C: Report of a Case

We report a case of primary hepatic non-Hodgkins lymphoma in a 77-year-old man with chronic hepatitis C. Laboratory data revealed slightly elevated liver function parameters and positive antibody for hepatitis C virus (HCV). Abdominal ultrasonography showed a low-echogenic tumor, about 5cm in diameter, in the left lateral segment. Abdominal computed tomography showed that the tumor was marginally enhanced in the early phase, but no enhancement was seen in the late phase. Magnetic resonance imaging showed that the tumor was hypointense in relation to the liver on T1-weighted images, but hyperintense on T2-weighted images. Hepatic angiography showed a homogeneously stained hypervascular tumor. Under the diagnosis of a liver tumor, thought to be a hepatocellular carcinoma, left lateral segmentectomy was performed. Histological examination confirmed a diagnosis of non-Hodgkins diffuse large B-cell lymphoma that was positive for L-26 and CD79, but negative for CD3 and UCHL-1. The surrounding liver tissue showed signs of chronic active hepatitis. Multiple recurrent lesions were found in the liver, spleen, and iliac bones 4 months postoperatively. However, complete remission was achieved after five courses of systemic chemotherapy using pirarubicin, cyclophosphamide, vincristine sulfate, and prednisolone. The patient has been carefully followed up for about 1 year since his operation, and has been doing well. We review the literature on primary non-Hodgkins lymphoma arising in the liver infected by HCV.     

Gallstone Ileus in a Patient with Crohn’s Disease: a Case Report

Gallstone ileus is a rare complication of cholelithiasis. Only 0,3–0,5% of all patients with gallstones will eventually suffer from this condition. It is well known that there is an increased prevalence of gallstones among patients with Crohn’s disease, but gallstone ileus remains even in these patients an unfrequent condition. Because of the rarity of this disease and its presentation as an intestinal (sub)obstruction, mostly without biliary symptoms, diagnosis and surgical treatment are often delayed. We report the case of a 75-year-old woman with a long history of Crohn’s disease presenting with intermittent symptoms of intestinal obstruction since several weeks. Symptoms were thought to be due to recurrence of Crohn’s disease, but the patient did not respond to steroid therapy. Resection of the diseased ileocolic segment was performed and a large impacted stone was detected proximal of the stenotic segment. With this case report we want to emphasize how easily diagnosis of gallstone ileus can be missed, especially in Crohn’s patients and we would like to discuss the different treatment options.     

Progression of Abdominal Aortic Aneurysm After Endovascular Stent-Grafting in a Patient with Behçet's disease: Report of a Case

A 53-year-old man with Behçet's disease was admitted to our hospital for investigation of back and lower abdominal pain. Computed tomography (CT) showed a projecting saccular aneurysm below the right renal artery. We placed a stent-graft just below the right renal artery, successfully excluding the abdominal aortic aneurysm (AAA). His C-reactive protein level and white blood cell count remained elevated after stent-grafting. About 5 months later, he was readmitted with recurrent back and lower abdominal pain and CT showed progression of the AAA. Thus, we performed straight grafting using a woven Dacron graft just below the right renal artery. The patient had an uneventful postoperative course. We discuss the controversial issue of treating AAA in patients with Behçet's disease, focusing on the indications and timing of surgery.     

Computed Tomographic Diagnosis of Incarcerated Meckel’s Diverticulum in a Patient with Bilateral Spigelian Hernia

Introduction  

Spigelian hernia is an uncommon abdominal wall hernia occurring through the linea semilunaris located near the lateral border of the rectus abdominis muscle.     

Evaluation of Cerebral Hypoperfusion by Nuclear Medicine Imaging in a Patient with Takayasu’s Arteritis

In patients with Takayasu’s arteritis presenting with cerebrovascular insufficiency, deciding whether and how to perform surgical intervention is difficult. We report successful extra-anatomical cerebrovascular reconstruction in a patient with Takayasu’s arteritis involving the aortic arch. A 27-year-old woman was diagnosed with Takayasu’s arteritis with occlusion of all three branches of the aortic arch. Single-photon emission computed tomography and positron emission tomography of the brain were useful in assessing the indication and effect of surgical treatment. Right common ilioaxillary artery bypass grafting was performed, and at 18-month follow-up the patient remained symptom-free. Extra-anatomical bypass can be a therapeutic option for surgical management of Takayasu’s arteritis with aortic arch involvement.     

Anterior Spinal Cord Syndrome in a Patient with Behçet's Disease

Although neurological involvement in Beh?et's disease is not so uncommon, isolated spinal cord disease is quite rare and reported to be observed in about 2% of all cases with neurological involvement. Here we report a Beh?et's patient with spinal cord disease presented with anterior spinal cord syndrome. This rare syndrome is caused by hypoperfusion of the anterior spinal artery and to our knowledge has not been previously reported in patients with Beh?et's disease. This report defines the characteristic clinical features of this entity and emphasizes the importance of early immunosuppressive treatment and initiation of rehabilitation.     

Necrotic Vasculitis in a Patient with Abdominal Fibromatosis and Guillain-Barré Syndrome

The case of a 65-year-old woman presenting with Guillain-Barré syndrome is herein reported. Tomographic investigation revealed abdominal and retroperitoneal fibromatosis. During her hospitalization, renal involvement ensued, and subsequent renal biopsy demonstrated findings of crescentic pauci-immune glomerulonephritis negative for ANCA antibodies and with characteristics indicative of necrotic angiitis. The simultaneous existence of the three diseases in the same patient as well as the relation between necrotic vasculitis and G-B syndrome is speculated, and the relevant literature is reviewed.