Huge ruptured and infected pseudoaneurysm of the ascending aorta and aortic arch with erosion of sternum after previous cardiac surgery

A 77-year-old woman underwent aortic valve replacement and coronary bypass grafting in 2007 in the Emirates. Evolution was uneventful until December 2011. After repeated episodes of unspecific infections, a computed tomographic scan showed a large pseudoaneurysm of the distal ascending aorta. The site of aortic rupture was closed with a Gore-Tex patch and a Staphylococcus aureus infection treated appropriately. Two months later, a small cutaneous lesion on the cranial part of the sternotomy started bleeding. Computed tomographic scan demonstrated recurrence of a false aneurysm with erosion of the sternum and a large subcutaneous hematoma caused by the fistula. The patient was transferred to our institution. The challenges of this case included safe surgical approach (sternotomy, cannulation, perfusion, cerebral protection) as well as complete removal and extensive debridement of the infected material and reconstruction of the aortic arch. Using fully biological material, reconstruction of the ascending aorta and proximal arch was successfully performed.

Acute partial Budd-Chiari syndrome and portal vein thrombosis in cytomegalovirus primary infection: a case report

BACKGROUND: Splanchnic vein thrombosis may complicate inherited thrombotic disorders. Acute cytomegalovirus infection is a rare cause of acquired venous thrombosis in the portal or mesenteric territory, but has never been described extending into a main hepatic vein. CASE PRESENTATION: A 36-year-old immunocompetent woman presented with acute primary cytomegalovirus infection in association with extensive thrombosis in the portal and splenic vein. In addition, a fresh thrombus was evident in the right hepatic vein. A thorough evaluation for a hypercoagulable state was negative. The clinical course, biological evolution, radiological and histological findings were consistent with cytomegalovirus hepatitis complicated by a partial acute Budd-Chiari syndrome and portal thrombosis. Therapeutic anticoagulation was associated with a slow clinical improvement and partial vascular recanalization. CONCLUSION: We described in details a new association between cytomegalovirus infection and acute venous thrombosis both in the portal vein and in the right hepatic vein, realizing a partial Budd-Chiari syndrome. One should be aware that this rare thrombotic event may be complicated by partial venous outflow block.

Disseminated Mycobacterium marinum infection with extensive cutaneous eruption and bacteremia in an immunocompromised patient

Mycobacterium marinum can cause fish tank granuloma (or swimming pool or aquarium granuloma) in immunocompetent patients. Dissemination of Mycobacterium marinum-infection is a rare condition which occurs mainly in immunocompromised patients and can be life-threatening. We report the case of an 87-year-old woman who was treated with oral corticosteroids for polymyalgia rheumatica for many years and developed erythema nodosum-like lesions on the right forearm and arthritis of the right wrist. By increasing the steroid dosage and adding methotrexate only short-term remission was achieved. Seven months later painful erythematous nodules occurred on all extremities which became necrotic, ulcerative and suppurative. Ziehl-Neelsen staining revealed acid-fast bacilli and Mycobacterium marinum was cultured from skin biopsies, blood, and urine. The critically ill patient was treated with clarithromycin and ethambutol resulting in a dramatic improvement of the general condition. After four months, doxycycline had to be added because of new skin lesions. This case illustrates the impact of Mycobacterium marinum infection in immunocompromised patients.

Fractured and embolized coronary stent mimicking significant internal carotid stenosis

We describe the case of 77-year-old woman who underwent five coronary angiographies in 9 months due to in-stent re-stenosis of Cypher-Stents, and who finally required coronary artery bypass grafting surgery. Preoperative investigations revealed a significant internal carotid stenosis, which was due to a fractured and embolized Cypher-Stent.

Uveitic glaucoma and Rosai-Dorfman disease (sinus histiocytosis)

PURPOSE: To report a novel association of uveitic glaucoma with Rosai-Dorfman disease. METHODS: Case report. RESULTS: A 67-year-old Caucasian woman presented with a chronic bilateral granulomatous uveitis which did not respond to conventional topical steroid therapy. She also had raised intraocular pressures, glaucomatous optic disc changes and diffuse nodular fibrous skin lesions. Subsequent skin biopsy immuno-cytochemistry showed S-100 staining, consistent with Rosai-Dorfman disease. The uveitis and glaucoma were highly resistant to standard medical treatments, but completely resolved together with the systemic features of the disease after six months. CONCLUSIONS: Rosai-Dorfman disease has not previously been reported to cause uveitic glaucoma and should be considered in non-responsive cases presenting with a rash. The disease is entirely self-limiting and early diagnosis may therefore avoid unnecessary trabeculectomy and/or systemic immune suppression.

Novel mutation in the ceruloplasmin gene causing a cognitive and movement disorder with diabetes mellitus

In a Chinese woman who had diabetes mellitus, undetectable ceruloplasmin, hand tremor, neck dystonia, and cognitive disturbances, genetic analyses revealed a novel homozygous mutation (848G > C or W283S) in exon 5 in the ceruloplasmin gene. Another member with a milder phenotype was also affected by this mutation. The healthy sister was heterozygous at the same position. Aceruloplasminemia has not yet been reported in China. This case suggests that increased awareness should be paid to this disorder in the presence of the typical symptoms.

Contrast enhanced ultrasound eases interpretation of an unclear renal tumor in addition to CT, MRI and histological findings--a case report in a young patient

Renal cancer represents accounts for approximately 3% of all adult malignancies with a rising incidence. Incidental diagnosis is mostly based upon ultrasound (US). US and Computed tomography (CT) are the standard imaging modalities for detecting renal cell cancer (RCC). Differentiation between malignant and benign renal tumors is of utmost importance. Contrast enhanced ultrasound (CUS) seems to be a promising new diagnostic option for diagnosis and preoperative treatment planning for patients with renal cancer. It is an additional examination to baseline ultrasound and CT. We report a case of a 37-year-old woman with a papillary renal cell cancer in which CUS helped to differentiate dignity of the tumor. CUS is an additional examination to baseline ultrasound and CT. It is a less invasive technique than contrast enhanced CT and shows even slight tumor blood flow. In addition it may allow a more rapid diagnosis, because of its bedside availability.

Multiple spinal extradural meningeal cysts presenting as acute paraplegia. Case report and review of the literature

Multiple spinal extradural meningeal cysts are rare. To the authors' knowledge, there have been only four reported cases in the world literature. The authors report a case of multiple spinal extradural meningeal cysts in a 31-year-old woman presenting with acute paraplegia. Magnetic resonance imaging of the thoracolumbar spine revealed multiple extradural cystic lesions extending from T-7 to T-8 and from T-12 to L-3. Intraoperative findings demonstrated a white, fibrous, and tense cyst filled with cerebrospinal fluid-like colorless fluid. Excision of the posterior wall of the symptomatic cyst was followed by immediate neurological improvement. The examination of the pathological specimen showed a thick duralike layer of collagen and an inner membrane of arachnoid that is often not found in these lesions. The final diagnosis was based on combined imaging, intraoperative, and histopathological findings. The authors review the literature and discuss the etiological, diagnostic, and therapeutic aspects of this lesion.

Low awareness of transient ischemic attacks and risk factors of stroke in a Swiss urban community

BACKGROUND AND PURPOSE: Time delays from stroke onset to arrival at the hospital are the main obstacles for widespread use of thrombolysis. In order to decrease the delays, educational campaigns try to inform the general public how to act optimally in case of stroke. To determine the content of such a campaign, we assessed the stroke knowledge in our population. METHODS: The stroke knowledge was studied by means of a closed-ended questionnaire. 422 randomly chosen inhabitants of Bern, Switzerland, were interviewed. RESULTS: The knowledge of stroke warning signs (WS) was classified as good in 64.7%. A good knowledge of stroke risk factors (RF) was noted in 6.4%. 4.2% knew both the WS and the RF of stroke indicating a very good global knowledge of stroke. Only 8.3% recognized TIA as symptoms of stroke resolving within 24 hours, and only 2.8% identified TIA as a disease requiring immediate medical help. In multivariate analysis being a woman, advancing age, and having an afflicted relative were associated with a good knowledge of WS (p = 0.048, p < 0.001 and p = 0.043). Good knowledge of RF was related to university education (p < 0.001). The good knowledge of TIA did not depend on age, sex, level of education or having an afflicted relative. CONCLUSIONS: The study brings to light relevant deficits of stroke knowledge in our population. A small number of participants could recognize TIA as stroke related symptoms resolving completely within 24 hours. Only a third of the surveyed persons would seek immediate medical help in case of TIA. The information obtained will be used in the development of future educational campaigns.

Is right coronary artery hypoplasia and sudden death an underdiagnosed association?

Determining whether hypoplasia of a coronary artery has caused or contributed to death is often complicated by an absence of histologic evidence of myocardial ischemia in the area of the heart supplied by the affected artery and also by the lack of data for assessing coronary artery size at autopsy. A 45-year-old woman is reported who collapsed and died and who was found at autopsy to have a dominant, small-caliber, right coronary artery, with acute and chronic ischemic changes in the posterior interventricular septum supplied by the diminutive vessel. This case provides evidence that small-caliber coronary arteries may be associated with a lethal outcome. Given the difficulties that may occur in determining whether there is a causal link between small coronary artery caliber and death, it is possible that this may be an underdiagnosed cause of sudden cardiac death, rather than a coincidental finding of minimal significance.

Mechanisms of unexpected death and autopsy findings in Leigh syndrome (subacute necrotising encephalomyelopathy)

A 21-year-old previously-well woman who was undergoing medical investigations for problems with balance and suspected multiple sclerosis, developed a headache and breathing difficulties, and died suddenly and unexpected at home. The autopsy was unremarkable except for pulmonary and cerebral oedema. However, subsequent microscopy of the brain revealed characteristic features of Leigh syndrome with multifocal areas of astrogliosis, capillary proliferation, and parenchymal vacuolation. While Leigh syndrome is more commonly diagnosed in infancy, manifestations may occur throughout early life into adulthood. Sudden and unexpected death is a rare presentation that may be associated with cerebral necrosis and oedema. An awareness of the variable manifestations of Leigh syndrome is necessary in forensic practice as not all cases will present in a typical manner and sudden death may occur before a diagnosis has been established. The heritable nature of this condition makes accuracy of diagnosis essential.

[Bisexuality and AIDS. Implications for heterosexual HIV infections in women]

One of the possible pathways into heterosexual population is the transfer of HIV-virus from bisexual men to their female partners. Therefore sexual behaviour of HIV-positive and -negative bisexual men (n = 31) before and after Aids-disease was analysed. Prior to the Aids-epidemic promiscuous behavior towards male partners (about one female partner per year versus 10 male partners per year). Relatively common contacts to woman occurred within steady relationships. After contact with HIV-test and personal counselling sexual practice became significantly different. The number of female and male contacts was markedly reduced, especially female chance acquaintances were avoided; safer sex was preferred and readiness to inform female partners about bisexuality was increased. This study suggests that reduction of risk for HIV-infection of female partners by homosexual men can be achieved by means of a HIV-test and personal counselling, a possibility that should be considered in preventive concepts.

Acute pancreatitis and thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP) has multiple clinical manifestations and risk factors, but the events that actually trigger acute episodes of TTP are often unclear. We describe the case of a 56-year-old woman who presented with clinical signs and symptoms of TTP and acute pancreatitis. We discuss whether pancreatitis was due to ischemic pancreatic damage caused by microvascular platelet clumping in the frame of TTP, or whether acute pancreatitis, a disorder that results in an intense systemic inflammatory response, may be a triggering event for acute episodes of TTP.

A rare anomaly of the anterior communicating artery complex hidden by a large broad-neck aneurysm and disclosed by three-dimensional rotational angiography

Double fenestration of the anterior communicating artery (ACoA) complex associated with an aneurysm is a very rare finding and is usually caused by ACoA duplication and the presence of a median artery of the corpus callosum (MACC). We present a patient in whom double fenestration was not associated with ACoA duplication or even with MACC, representing therefore, a previously unreported anatomic variation. A 43 year old woman experienced sudden headache and the CT scans showed subarachnoid haemorrhage (SAH). On admission, her clinical condition was consistent with Hunt and Hess grade II. Conventional digital subtraction angiography (DSA) was performed and revealed multiple intracranial aneurysms arising from both middle cerebral arteries (MCA) and from the ACoA. Three-dimensional rotational angiography (3D-RA) disclosed a double fenestration of the ACoA complex which was missed by DSA. The patient underwent a classic pterional approach in order to achieve occlusion of both left MCA and ACoA aneurysms by surgical clipping. The post-operative period was uneventful. A rare anatomical variation characterised by a double fenestration not associated with ACoA duplication or MACC is described. The DSA images missed the double fenestration which was disclosed by 3D-RA, indicating the importance of 3D-RA in the diagnosis and surgical planning of intracranial aneurysms.