Appearances of von meyenburg complex on cross sectional imaging.

The von Meyenburg complex (VMC) is an uncommon congenital malformation and is characterized by benign bile duct hamartomas. These are usually discovered incidentally and may represent a diagnostic dilemma when liver metastases are suspected. MRI of VMC shows distinct imaging characteristics, but reporting of lesional contrast enhancement has been inconsistent, whilst microbubble contrast enhanced ultrasound provides 'real-time' evaluation of soft tissue vascularity. Given the diagnostic uncertainty over imaging in VMC, biopsy is often recommended as the definitive diagnosis. We report a biopsy proven case of VMC on a background of primary colonic malignancy investigated with ultrasound, contrast enhanced ultrasound, computed tomography CT, and magnetic resonance imaging MRI, and review the key imaging features.

Polyarteritis nodosa of the breast: presentation and management.

Polyarteritis nodosa (PAN) of the breast is a rare condition where literature review identified eleven patients so far. The clinical presentation ranged from localized disease involving the breast parenchyma and skin only to breast manifestations as part of systemic PAN. The diagnosis of PAN could be challenging as it can mimic breast cancer, inflammatory carcinomatosis or breast infection including mastitis and necrotizing fasciitis. The key importance is accurate diagnosis to avoid unnecessary other treatment modalities and the timely recognition of PAN in cases of localized forms. The authors present three new cases which represent the full range of the clinical spectrum and their management.

Fatal granulomatous amoebic meningoencephalitis due to Balamuthia mandrillaris.

Amoebic infections of the central nervous system (CNS) are very rare and usually fatal. A 33-year-old Bolivian male injured his elbow 18 months ago in an accident and, months later, developed multiple skin lesions. He was admitted with confusion, and brain images showed large multifocal lesions with mass effect. Review of the skin biopsy revealed the presence of amoebic trophozoites within a granulomatous inflammation. Despite treatment, he continued to deteriorate and died 17 days after admission. The brain was swollen with prominent uncal herniation, and the leptomeninges showed patchy exudate. Coronal sections revealed widespread hemorrhagic and necrotic lesions. Histology confirmed granulomatous amoebic meningoencephalitis (GAM) with the presence of amoebic trophozoites and occasional cysts. Post-mortem culture confirmed Balamuthia mandrillaris. GAM due to B. mandrillaris may occur in any age group, may or may not be associated with immunosuppression, and is present worldwide. It usually has a subacute and fatal course from hematogenous dissemination of chronic skin or lung lesions.

Sickle liver disease--an unusual presentation in a compound heterozygote for HbS and a novel beta-thalassemia mutation.

A 38-year-old Ghanaian man presented with a 6-month history of worsening pruritus, jaundice, and ascites. He was previously fit and well and rarely drank alcohol. Screening tests for chronic liver disease including viral, autoimmune, and other metabolic causes including iron overload were unremarkable. A liver biopsy performed at the referring hospital demonstrated intralobular cholestasis and cirrhosis. He was listed for liver transplantation but subsequently developed sepsis with multiple organ failure and died. The sickle solubility test was positive. Blood smear showed cells consistent with liver failure and no sickle cells. Hemoglobin electrophoresis revealed HbA2 2.8%, HbF 0.5%, and HbS greater than HbA (49.6% vs. 41.3%) in the absence of blood transfusion. Sequence analysis of the beta-globin genes showed he was a compound heterozygote for the Hbs mutation at codon 6 (CAG --> GTG) and a novel mutation at position 844 of intron 2 (betaIVS2-844 C --> A). A diagnosis of sickle hepatopathy causing decompensated cirrhosis was made. This case is unusual insomuch as this patient was asymptomatic for over 35 years and represents a novel presentation of sickle cell disease. Sickle cell disease should be considered in appropriate patients when unusual presentations of liver disease arise.

Aspergillus aortitis: a cause for aortic perforation in a patient following combined aortic valve surgery and liver transplantation.

We report a case of aspergillus fumigatus aortitis in a patient following combined aortic valve replacement and orthotopic liver transplantation for significant aortic stenosis and alcoholic liver cirrhosis. At operation, a suspicious excavating lesion was found on the native aortic valve and specimen sent for culture. The ascending aorta and aortic sinuses were found to be normal. Routine immunosuppression was commenced post transplant. Urgent valve microscopy was highly suspicious of fungal growth, and antifungal therapy was instituted. Day 18 post-surgery the patient unexpectedly arrested. Post mortem findings showed ascending aortic perforation with multiple fungal lesions noted. Microscopy demonstrated aspergillus fumigatus invading the aortic wall. This is the first case report illustrating a dormant phase of aspergillus fumigatus endocarditis that was activated following surgery and immunosuppression leading to aortitis and subsequent perforation.

Telepathology in a routine clinical environment: implementation and accuracy of diagnosis by robotic microscopy in a one-stop breast clinic.

The concept of using telepathology as a way of increasing the efficiency of pathologists is not new. There have been many studies attempting to evaluate the role of telepathology diagnosis, predominantly using transmission of still digital images. This study evaluates the potential value of remote diagnosis using robotic microscopy in the setting of a one-stop breast clinic. A Zeiss Axiopath telepathology system was used. The aim was to identify deficiencies in software and the minimum specifications for the computer hardware and network capability needed for reproducible pathological diagnosis with a view to developing a system that can preclude the need for an on-site pathologist. Forty-seven fine needle aspiration smears were diagnosed simultaneously by a pathologist in the breast clinic and by a different pathologist operating a robotic microscope situated in the clinic in a different wing of the hospital from the pathology department. The diagnoses, the time taken for clinic and remote diagnosis, and difficulties in using the system were recorded. Absolute correlation was achieved in 80.9% of cases. There was one false-positive diagnosis of cancer and no false negatives. The mean time taken for diagnosis per case was 2.39 min in clinic and 10.81 min by remote control robotic microscopy. However, as the pathologist did not have to leave the department, remote diagnosis was overall more economical of pathologists' time. Image quality was generally found to be good and not an obstacle to diagnosis. There were operational and technical problems that made remote diagnosis tedious and lengthy. Although at present the system is not capable of replacing an on-site pathologist, the results were encouraging and suggest that remote control remote diagnosis has the potential to increase the efficiency of pathologists.