Intracoronary IgG4-related disease as an unusual cause of sudden cardiac arrest: a case series.

BACKGROUND: IgG4-related disease (IgG4-RD) is a fibro-inflammatory condition classically causing retroperitoneal fibrosis, aortitis, thyroiditis, or pancreatitis. Diagnosis includes the presence of lymphoplasmacytic infiltrate (with >40% ratio IgG4+:IgG plasma cells) and fibrosis. Cardiac involvement may include aortic, pericardial, or coronary disease. Coronary manifestations encompass obstructive intra-luminal lesions, external encasing pseudo-tumour on imaging, or lymphoplasmacytic arteritis. CASE SUMMARY: Case 1: A fit and healthy 50-year-old man was found deceased. His only known medical condition was treated Hashimoto's thyroiditis. Post-mortem examination demonstrated an isolated severe stenosis of the left anterior descending (LAD) coronary artery without histopathological evidence of acute myocardial infarction. Coronary plaque histopathology showed florid IgG4-positive plasma cell infiltrate throughout all layers of the artery with dense fibrous tissue connective tissue stroma, all features consistent with coronary artery IgG4-RD. Case 2: A 48-year-old man collapsed at work. Computed tomography scan 1 week prior reported an ill-defined para-aortic retroperitoneal soft tissue density. No cardiac symptoms were reported in life. Post-mortem examination showed coronary arteritis and peri-arteritis with sclerosing peri-aortitis in the LAD. There was myocardial fibrosis of the anterior left ventricle and focal myocarditis of the right ventricle. DISCUSSION: IgG4-related disease presenting as sudden cardiac death without any preceding symptoms is very rare (six prior cases identified on literature review). Reported targeted successful interventions for intracoronary IgG4-RD diagnosed in life have included steroid therapy and B cell depleting therapy (i.e. rituximab). If cardiac symptoms are present in a patient with known IgG4-RD, cardiac investigations should be promptly arranged.

Superior mesenteric vein tumour thrombus in a patient with caecal adenocarcinoma: a rare and important finding.

Venous tumour thrombosis refers to the invasion of tumour into the venous system. Extramural venous invasion is routinely searched for and reported in rectal carcinoma due to its prognostic significance and influence on staging, prognosis and treatment approach. We describe a case of extramural venous invasion occurring as superior mesenteric vein tumour thrombus in the setting of a caecal carcinoma.

The Ebola epidemic in Liberia and managing the dead-A future role for Humanitarian Forensic Action?

With some of their economies, communities and health systems weakened by decades of war and poor governance, it was no accident that an epidemic of Ebola virus disease broke out in west Africa. Being spread in part by contact with body fluids of those who had died from the disease, funerary rites and the way dead bodies were managed were important modes of transmission. The Liberian Red Cross, supported by the International Federation of Red Cross and Red Crescent Societies and the International Committee of the Red Cross, undertook the challenging task of managing the dead bodies in Monrovia during the epidemic. The work was undertaken by volunteers, not health care workers, who were trained and equipped for this task. The authors observed their work and were impressed. Valuable lessons were learned for mortuaries generally, and for Humanitarian Forensic Action involving the management of highly infectious human remains.

Electric fences and accidental death.

Deaths which occur in association with agricultural electric fences are very rare. In fact, electric fences have undoubtedly saved numerous human and animal lives by safely and reliably keeping livestock confined to their fields and enclosures and thus preventing motor vehicle incidents when livestock get onto roads and highways. Accidental and intentional human contact with electric fences occurs regularly and causes little more than transient discomfort, however, on exceptional occasions, contact with electric fences appears to be directly related to the death of the individual. The precise pathophysiological cause of these deaths is unclear. We present two cases of deaths associated with electric fences, discuss the possible pathophysiological mechanisms in these cases, and suggest a universal approach to the medico-legal investigation and documentation of these deaths.

Lethal hemorrhage from a ureteric-arterial-enteric fistula diagnosed by postmortem CT angiography.

Fistula formation following pelvic surgery and radiotherapy, including ureteric-arterial fistulas (UAF), is well documented, however, ureteric-arterial-enteric fistula is extremely rare. Conventional autopsy is usually required for the definitive diagnosis of pelvic vascular fistulas although an accurate diagnosis can still be complicated and challenging. The role of post-mortem computed tomography (PMCT) as an adjunct to conventional autopsy is well documented in the literature. One of the limitations of PMCT is the diagnosis of vascular conditions. Post-mortem computed tomography angiography (PMCTA) is a recently introduced technique that can assist in detecting such pathology. We present a case of post-radiotherapy ureteric-arterial-enteric fistula presenting as massive rectal and vaginal bleeding diagnosed prior to autopsy on PMCTA. The role of PMCTA in the diagnosis of such a UAF has not previously been reported in the literature.

Ancient schwannoma of the vulva.

BACKGROUND: Ancient schwannoma, a variant of schwannoma (previously termed neurilemoma), arises from the Schwann cells of the nerve sheath. It presents with swelling, pain, or paresthesia. CASE: A 53-year-old woman presented with a history of a left vulvar swelling, which had been present for many years. The tumor was slowly increasing in size and affected activities such as sitting and walking. Occasionally, she experienced a burning sensation at the site of the swelling. Surgical excision was undertaken, and histology demonstrated an ancient schwannoma. CONCLUSION: The characteristic histological appearance seen in ancient schwannoma should prevent the erroneous diagnosis of a malignant tumor. Malignant change is exceedingly rare. Treatment is complete surgical excision.