Intrathoracic gastric volvulus: an autopsy case report

First described by Berti in 1866, gastric volvulus (GV) is an uncommon and potentially lethal entity. GV occurs when the stomach twists by more than 180º resulting in obstruction of the alimentary tract, visceral ischemia, necrosis, and perforation. It is classified according to the rotation axis in organoaxial, mesenteroaxial or a combination of both. The clinical presentation can be acute, and is usually severe or chronic, which sometimes may be asymptomatic. It predominantly occurs in the fifth decade of life, but children, mainly those under the age of 1 year, may be affected. No ethnicity or gender was observed to show predominance. This entity is related to gastric, diaphragmatic disorders as well as laxity of gastric ligaments. Acute GV may complicate with incarceration and strangulation of the stomach when gastric necrosis ensues. These cases show a mortality rate of 60%. The authors report the fatal case of a surgically treated GV in a 43-year-old female patient who looked for medical care only after 1 month of initial symptoms. Diagnosis was confirmed with a thoracic and abdominal axial computed tomography. Besides the entire stomach being herniated and twisted into the thoracic cavity, the pancreas was pulled up through the hiatal orifice, provoking acute pancreatitis. Because of gastric necrosis and perforation, gastroenteric fluid drained into the mediastinum and left pleural space. The postoperative outcome was unfavorable resulting in the patient’s death. The authors call attention to the severeness of the disease, and therefore the need of precocity of diagnosis and surgical treatment

Aortic aneurysm and diverticulum of Kommerell: a dreadful concomitance

First described in 1936, the diverticulum of Kommerell (DOK) is a dilatation of the proximal segment of an aberrant subclavian artery. Appearing more frequently in the left-sided aortic arch, the aberrant right subclavian artery passes behind the esophagus toward the right arm, causing symptoms in the minority of cases. Diagnosis is generally incidental with this pattern. When symptomatic, dysphagia, respiratory symptoms, hoarseness, chest pain, and upper limb ischemia are the most common complaints. Although debatable, the origin of DOK is accepted as being degenerative or congenital. The degenerative condition is normally associated with atherosclerosis and occurs more frequently after the age of 50 years with no gender predominance. Complications may be life threatening and are more commonly related to the diverticulum aneurysm or when associated with aortic diseases such as aneurysms or dissection. The authors present a case of a 67-year-old male with a history of acute chest pain, neurological disturbances, and hypertensive crisis. The diagnostic workup revealed an aortic arch aneurysm with intramural hematoma and a diverticulum aneurysm of Kommerell. Treatment was conservative at first. The patient presented a satisfactory outcome and was referred to an outpatient clinic for follow up and further therapeutic consolidation

Hepatoid adenocarcinoma of the lung

Lung adenocarcinomas with a mixture of tubular or papillary pattern, sheet-like or trabecular architecture, eosinophilic cytoplasm with centrally located nuclei and alpha-fetoprotein-producing cells have been described as hepatoid adenocarcinomas. Hepatoid adenocarcinomas are mainly found in the stomach but rare cases in other organs have been described. Immunostaining for alpha-fetoprotein (AFP), hepatocyte paraffin 1 (HepPar-1) and thyroid transcription factor-1 (TTF-1) helps in the diagnostic workup. Tumor behavior is still not entirely known but it seems to be associated with early metastases. We report on a 66-year-old, heavy-smoker male patient who had a 10-month history of respiratory complaints and weight loss. At the time he was hospitalized, respiratory failure was already established. The computed tomography corresponded to a collapsed right lung due to a poorly defined expanding mass. The bronchoscopy revealed narrowing of the inferior and medium lobar bronchi. The patient developed irreversible shock and died. At the right lung inferior lobe was extensively replaced by a grayish diffuse neoplasia in a “pneumonia-like” gross pattern. Metastatic disease was found in the right adrenal gland and thoracic and abdominal lymph nodes. Microscopic dissemination through lymphatics, pleura, and airways was detected. Histological examination revealed a poorly differentiated adenocarcinoma with hepatoid features. Immunohistochemmistry stains were positive for keratin 7, polyclonal carcinoembryonic antigen (CEA) in a diffuse pattern, AFP and HepPar-1 antibody. TTF-1 showed a diffuse granular cytoplasmic staining of the neoplastic cells, and only focal nuclear positivity. Multiple bilateral emboli originated from deep venous thrombosis were present in large and medium branches of the pulmonary artery and contributed to the cause of death

Primary aortoesophageal fistula: a rare cause of acute upper gastrointestinal bleeding

Acute upper gastrointestinal bleeding is a potentially life-threateningemergency, especially in the elderly. This condition accounts for approximately1% of all emergency room admissions. Among the causes of such bleedingis aortoesophageal fistula, a dreaded but apparently rare condition, firstrecognized in 1818. The great majority of cases are of primary aortoesophagealfistula, caused by atheromatous aortic aneurysms or, less frequently, bypenetrating aortic ulcer. The clinical presentation of aortoesophageal fistulais typically characterized by the so-called Chiari’s triad, consisting of thoracicpain followed by herald bleeding, a variable, short symptom-free interval,and fatal exsanguinating hemorrhage. The prognosis is poor, the in-hospitalmortality rate being 60%. Conservative treatment does not prolong survival,and the in-hospital mortality rate is 40% for patients submitted to conventionalsurgical treatment. Here, we report the case of a 93-year-old woman whopresented to the emergency room with a history of hematemesis. The patientwas first submitted to upper gastrointestinal endoscopy, the findings of whichwere suggestive of aortoesophageal fistula. The diagnosis was confirmedby multidetector computed tomography of the chest. Surgery was indicated.However, on the way to the operating room, the patient presented with massivebleeding and went into cardiac arrest, which resulted in her death

Calcification of the ligamentum flavum in the thoracolumbar spine: an unusual cause of compressive myelopathy

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 eightmonthhistory 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 thoracicand 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.nul

Segmental omental infarction: an unusual cause of acute abdomen

Omental infarction is a rare cause of acute abdominal pain, and the cause of omental infarction itself remains unknown. The diagnosis of omental infarction is occasionally made intra-operatively, because it is poorly known and presents no specific clinical features. Therefore omental infarction should always be considered in the differential diagnosis of abdominal inflammatory processes, mainly those affecting the right lower quadrant. The increasing use of computed tomography in cases of acute abdomen has facilitated the diagnosis of omental infarction in the pre-operative period. Here, we present the case of a 36-year-old, overweight female patient with clinical symptoms of acute abdomen. Computed tomography of the abdomen revealed indications of omental infarction. The patient was treated conservatively with analgesics and a nonsteroidal anti-inflammatory. The clinical evolution was favorable.nul

Inflammatory myofibroblastic tumor of the bladder in a child: a case report

Tumores miofibroblásticos inflamatórios raramente acometem vias urinárias ou crianças, comumente mimetizando neoplasias malignas nos exames de imagem. Foram descritos apenas 35 casos desses tumores na bexiga de crianças, segundo a literatura recente. Os autores apresentam o caso de uma criança com um tumor miofibroblástico vesical que evoluiu favoravelmente após ressecção cirúrgica completa

Infective endocarditis with left to right intracardiac fistula due to\ud Streptococcus anginosus - a rare complication caused by an even rarer bacterium

Although infective endocarditis (IE) has been described in reports dating\ud from the Renaissance, the diagnosis still challenges and the outcome often\ud surprises. In the course of time, diagnostic criteria have been updated and\ud validated to reduce misdiagnosis. Some risk factors and epidemiology have\ud shown dynamic changes since degenerative valvular disease became more\ud predominant in developed countries, and the mean age of the affected\ud population increased. Despite streptococci have been being well known as\ud etiologic agents, some groups, although rare, have been increasingly reported\ud (e.g., Streptococcus milleri.) Intracardiac complications of IE are common and\ud have a worse prognosis, frequently requiring surgical treatment. We report a\ud case of a middle-aged diabetic man who presented with prolonged fever, weight\ud loss, and ultimately severe dyspnea. IE was diagnosed based on a new valvular\ud regurgitation murmur, a positive blood culture for Streptococcus anginosus, an\ud echocardiographic finding of an aortic valve vegetation, fever, and pulmonary\ud thromboembolism. Despite an appropriate antibiotic regimen, the patient died.\ud Autopsy findings showed vegetation attached to a bicuspid aortic valve with an\ud associated septal abscess and left ventricle and aortic root fistula connecting\ud with the pulmonary artery. A large thrombus was adherent to the pulmonary\ud artery trunk and a pulmonary septic thromboemboli were also identified

Bone marrow necrosis and fat embolism syndrome: a dreadful complication of hemoglobin sickle cell disease

Sickle cell disease encompasses a wide range of genotypic presentation with particular clinical features. The entity affects millions of people, particularly those whose ancestors came from sub-Saharan Africa and other countries in the Western Hemisphere, Saudi Arabia, and India. Currently, the high frequency of S and C genes reflects natural selection through the protection of heterozygotes against severe malaria, the high frequency of consanguineous marriages, improvement of some public health policies and the nutritional standards in the poorer countries where newborns are now living long enough to present for diagnosis and management. Although there is a high burden of the disease, in many countries, the new-born sickle cell screening test is being performed and is rendering an early diagnosis; however, it is still difficult for sickle cell patients to find proper treatment and adequate follow-up. Moreover, in many countries, patients are neither aware of their diagnosis nor the care they should receive to prevent complications; also, they do not receive adequate genetic counseling. Hemoglobin SC (HbSC) disease is the most frequent double sickle cell heterozygosis found in Brazil. The clinical course tends to be more benign with fewer hospitalizations compared with double homozygotic SS patients. However, HbSC patients may present severe complications with a fatal outcome. We report the case of a 36-year-old man who presented to the emergency care facility with symptoms consistent with the diagnosis of sickling crisis. The outcome was unfavorable and death occurred just hours after admission. The autopsy revealed a generalized vaso-occlusive crisis by sickled red cells, bone marrow necrosis, and fat embolism syndrome

Primary diffuse large B-cell lymphoma or lymphomatoid granulomatosis grade 3: \ud a still-puzzling diagnosis in autopsy

Primary lung lymphoma is a rare entity accounting for approximately 0.3% of\ud all primary neoplasia of the lung and includes diffuse large B-cell lymphoma\ud (DLBL) and lymphomatoid granulomatosis (LYG). Considering that clinical\ud features may be similar, whereas epidemiology, morphology, and radiological\ud features are different, the authors report a case of a middle-aged man\ud who presented multiple pulmonary nodules in the lower lobes and groundglass\ud opacities scattered bilaterally on computed tomography. Clinically, he\ud presented a consumptive syndrome with respiratory failure and pleurisy,\ud which progressed until death. The autopsy findings were consistent with\ud lymphomatoid granulomatosis (LYG) grade 3/ diffuse large B-cell lymphoma\ud (DLBL). The authors call attention to the difficulty of establishing an accurate\ud diagnosis, mainly when the demonstration of EBV-infected atypical B-cells\ud fails