Since the 1950s, aortic graft infections (AGIs) constitute one of the most feared complications after reconstructive vascular surgery. This complication is not frequent, ranging from 1% to 2% in the recently reported series; however, the high rate of death and morbidity after therapeutic attempts justifies its dreadful fame. The majority of cases occur during the first month after surgery. Staphylococcus aureus is the cause of 70% of the early infection cases. Late infections, on the other hand, are even rarer, showing a strong relationship with low virulence microorganisms, where Staphylococcus epidermidis is the main cause. Gram-negative bacteria are also observed in late infections, mainly when an aortic or graft enteric fistula is present. Treatment modalities are plenty, but still debatable. The authors report a case of a woman who was operated on 6 years ago for a reconstructive aortic aneurysm with the implantation of an infrarenal Dacron graft in the aorto bifemoral position. She looked for medical assistance with a 2-month history of weight loss, abdominal/back pain, and fever. Her clinical status rapidly deteriorated. A computed tomography of the abdomen disclosed the diagnosis of an AGI. The patient was promptly treated with antibiotics. Surgery was undertaken to explant the infected graft and another graft was placed into the axillobifemoral position. Culture from the infections site was negative. After surgery the patient quickly developed refractory septic shock and died immediately post-operatively

Known since the 19th century, neuromyelitis optica (NMO), or Devic’s disease, is an idiopathic immune-mediated inflammatory demyelinating disease of the central nervous system selectively affecting the optic nerve and spinal cord. Commonly diagnosed in demyelinating diseases reference centers, we report an 18-year-old female patient who sought medical attention with a 3-month history of weight loss, headache, and vomiting, followed by diplopia, a burning sensation over the lower limbs, and difficulty walking. A few days prior to hospital admission, the muscle strength in her lower limbs became worse and ascended to the upper limbs associated with sensory changes in the trunk and voiding dysfunction. At admission, the neurological examination was consistent with a spinal cord syndrome. After few days of hospitalization, she was tetraplegic with severe signs of brainstem involvement requiring mechanical ventilatory support. Intravenous methylprednisolone and cyclophosphamide were promptly started after ruling out the diagnosis of infectious disease and cord compression. Due to no substantial early improvement, intravenous immunoglobulin was also used. From then on, the neurological status gradually improved. Magnetic resonance imaging showed extensive demyelinating features in the spinal cord, and the serum IgG autoantibody was negative. The patient was referred to a tertiary neurological reference center where she remains under treatment.

Anaplastic large cell lymphoma (ALCL), described less than 30 years ago by Karl Lennert and Herald Stein in Kiel, West Germany, is a T-cell or null non-Hodgkin lymphoma, with distinctive morphology (hallmark cells, prominent sinus and/or perivascular growth pattern), characteristic immunophenotype (CD30+, cytotoxic granules protein+, CD3–/+) and specific genetic features as translocations involving the receptor tyrosine kinase called anaplastic lymphoma kinase (ALK) on 2p23 and variable partners genes, which results in the expression of ALK fusion protein. The absence of ALK expression is also observed and is associated with poorer prognosis that seen with ALK expression. ALK-negative ALCL is more frequent in adults, with both nodal and extra nodal clinical presentation and includes several differential diagnoses with other CD30+ lymphomas. Liver involvement by ALCL is rare and is generally seen as mass formation; the diffuse pattern of infiltration is even more unusual. The authors present a case of a 72-year-old man who presented clinical symptoms of acute hepatic failure. The patient had a long history of alcohol abuse and the diagnosis of alcoholic hepatitis was highly considered, although the serum lactic dehydrogenase (LDH) value was highly elevated. The clinical course was fulminant leading to death on the fourth day of hospitalization. Autopsy demonstrated diffuse neoplastic hepatic infiltration as well as splenic, pulmonary, bone marrow, and minor abdominal lymph nodes involvement by the tumor. Based on morphological, immunophenotypical, and immunohistochemical features, a diagnosis of ALK- negative ALCL was concluded. When there is marked elevation of LDH the possibility of lymphoma, ALCL and other types, should be the principal diagnosis to be considered.

Black esophagus is a rare but underdiagnosed disease. It occurs most frequently in severely ill patients and carries a high mortality rate. Cause of death is usually attributed to the comorbid conditions. Treatment is directed at the underlying cause, acid suppression and keeping the patient nil-per-os. Surgery is needed in complicated cases and stenosis is the most feared longterm sequel. In the present article, two cases are described and literature is reviewed.

Inflammation of the inner layer of the heart, especially the valvular endothelium, chordae tendinae and mural endocardium was first recognized almost 350 years ago. Over the years it has had many names, but is now generally designated infective endocarditis (IE) and has an associated infectious agent. A sterile vegetative process can also affect the valves and is usually referred to as Libman-Sacks endocarditis. The developments of medical science that allowed for our understanding of this entity included refinement of the autopsy, medical microscopy, microbiology, and in recent years, molecular studies. Some observations were misleading but clarification particularly followed the reports of Morgagni, Osler and Libman. As understanding of the pathobiology of infective endocarditis grew so did the effectiveness of therapy. This paper provides a detailed history of the development of the concept of Infective endocarditis citing many key morphological observations and concludes with brief comments about current concepts of pathogenesis as well as a few remarks about therapy.

Congenital intestinal aganglionosis, also called Hirschsprung disease (HD), is defined as the absence of ganglionic cells in the myenteric (Auerbach) and submucosal (Meissner) plexus, due to a failure in the enteric nervous system development. The extent of intestinal involvement may vary according to the age of embryo development in which this failure occurs. It is not unusual for other malformations to be present, as well as chromosomal trisomies, manly trisomy 21. Enterocolitis is a frequent, life threatening, and feared complication of HD. Moreover, oligohydramnios is a well-known condition frequently associated with malformations, including those related to the gastrointestinal tract. The authors report the case of a newborn that presented a delayed meconium passage. On the third day of life, he presented enterocolitis—the outcome of which was favorable with clinical treatment. While the diagnosis of HD was awaiting confirmation, the enterocolitis relapsed and this time he died due to septic shock. The autopsy findings were compatible with a short segment of congenital intestinal aganglionosis. No other malformation was found. The authors call attention for an early diagnosis of HD whenever the meconium passage does not happen for at least 48 hours and for the risk factors of enterocolitis. This case also demonstrates HD associated with oligohydramnios.

The focal calcification or ossification of the ligamentum flavum is a rare cause of thoracic myelopathy and most often occurs among individuals of Japanese descent. It is rare in other ethnic groups and in individuals below the age of 50. It is most often described at the lower thoracic level, being uncommon in the lumbar region and rare in the cervical region. Here, we present the case of a 44-year-old White female patient who sought medical attention with an eightmonth history of paraesthesia of the lower limbs and progressive difficulty in walking. The clinical profile, together with computed tomography and nuclear magnetic resonance imaging of the spine, led to a diagnosis of compressive thoracic myelopathy due to ossification of the ligamentum flavum in the thoracic and lumbar spine. The patient underwent laminectomy and dissection of some of the affected ligamentum flavum, without any intraoperative complications. After three months of clinical follow-up, the patient had progressed favorably, having no sensory complaints and again becoming ambulatory.

Schistosomiasis is one of the most common parasitic diseases, still considered of public health significance. Acute schistosomiasis is of difficult diagnosis and therefore has been overlooked, misdiagnosed, underestimated and underreported in endemic areas. The delay between the exposure to contaminated water and the initial symptoms may explain this challenging diagnosis. Acute schistosomiasis is frequently reported in non-immune individuals while reinfection cases occurring in endemic areas is scarcely documented. The later usually shows a benign course but fatal cases do exist. The authors report a case of a young female patient, in the late puerperium, with a three-month history of weight loss, intermittent fever, cough, thoracic and abdominal pain and increased abdominal girth. Physical examination showed a tachycardia, tachypnea and hypotension. Laboratory tests showed a mild anemia, eosinophilia, and a slightly elevation of liver enzymes. Thorax and abdominal multidetector computed tomography evidenced a diffuse and bilateral pulmonary micronodules and peritoneal and intestinal wall thickening. The patient progressed rapidly to hepatic insufficiency, and death after respiratory insufficiency. An autopsy was performed and the findings were compatible with acute Schistosomiasis in a patient previously exposed to Schistosoma mansoni.

The Editors of Autopsy and Case Reports thank the peer reviewers listed below for the excellent collaborative work, opinions and comments on the papers published in 2011. Their hard work certainly contributed to maintaining the scientific level of this journal.

In emergency services a significant amount of pediatric patients undergo a brain or orbit CT scans for suspicion other than sinusitis. Assuming this premise, this study was held to show the incidental findings of the paranasal sinuses of children with nonspecific symptoms such as fever, headache and vomiting that underwent brain or orbits CT scans, without the initial suspicion of sinusitis. In a retrospective study, we evaluated 70 CT scans of the brain and orbits of children between 0 and 12 years. The incidental findings of the paranasal sinuses occurred in 32 cases (45.7%). Mucosal thickening was the most common incidental finding, being observed in 35% of patients, followed by complete opacification observed in 28% of cases and incomplete opacification observed in 28% of patients. Bilateral involvement occurred in 78% patients. The sinuses most frequently affected were the maxillary sinus followed by ethmoid sinuses. The abnormalities were more severe in children under the age of three years. The prevalence of incidental tomographic abnormalities in patients without an initial diagnosis of sinusitis is high. The predominance of these findings are mild abnormalities.