Sudden, Unexpected Death Due to Pseudo-Meigs Syndrome: A Case Report and Review of the Literature.

Meigs syndrome is the triad of ascites, hydrothorax, and benign ovarian tumor (mostly fibroids). It is a diagnosis of exclusion, and the characteristic symptoms disappear after resection of the tumor. Instead, in Pseudo-Meigs syndrome, the triad includes a nonfibroma ovarian tumor. The latter may consist of benign tumors (ie, of fallopian tube or uterus, struma ovarii, and ovarian leiomyomas) but can also comprise ovarian or metastatic gastrointestinal malignancies.The authors describe a case of sudden death in a 43-year-old woman, with no noteworthy reported history of present illness or medical history and in apparently good health before death.The autopsy showed a picture of bilateral hydrothorax with lung collapse, ascites, and a large left-sided ovarian mass, approximately 15 cm in diameter. Histopathological examinations revealed an ovarian epithelial malignancy (cystadenocarcinoma). There was also lung atelectasis with accompanying thrombosis of small and medium blood vessels. The combination of autopsy and histological findings allowed us to establish the diagnosis of Pseudo-Meigs syndrome, undiagnosed antemortem, resulting in death due to pulmonary and thrombotic complications. Our subsequent review of the literature found no case reports of undiagnosed Pseudo-Meigs syndrome presenting as sudden death, highlighting the uniqueness of the case presented herein.

Intraductal aspiration: a promising new tissue-sampling technique for the diagnosis of suspected malignant biliary strictures.

BACKGROUND: Brushing is the most commonly used technique for biliary sampling at ERCP, despite its limited sensitivity. OBJECTIVE: To evaluate intraductal aspiration (IDA) as a new combined endoscopic technique for cytodiagnosis, its cellular adequacy, diagnostic accuracy for cancer detection, feasibility, and safety. DESIGN: Prospective, observational study. SETTING: Single tertiary referral center. MAIN OUTCOME MEASUREMENTS: IDA cellular adequacy, diagnostic accuracy for cancer detection, feasibility, and safety. PATIENTS AND METHODS: From April 2009 to September 2010, 42 consecutive patients with suspected malignant biliary stricture underwent ERCP, with tissue sampling obtained with IDA. IDA included performance of standard brushing in all patients. After standard brushing, to perform IDA, we removed the brush from its catheter and used the tip of the catheter as a scraping device. The tip was scraped back and forth across the stricture at least 10 times. The catheter and a suction line were connected to a specimen trap to obtain intraductal aspiration of fluids and samplings. RESULTS: Our cytopathologists found adequate cellular yield in 39 of the 42 IDA samples (92.8%) versus 15 of the 42 brushing samples (35.7%) (P < .001). IDA showed a significantly higher sensitivity than brushing (89% vs 78% for adequate samples and 89% vs 37% for all samples) and provided significantly superior cellular adequacy (92.8% vs 35.7%). LIMITATIONS: Observational study, small number of patients. CONCLUSIONS: IDA significantly improves brushing cellular adequacy and has high sensitivity for cancer detection. It was also safe, simple, rapid, and applicable during routine diagnostic ERCP, with no additional costs.

Sudden death in Leigh syndrome: an autopsy case.

The present report describes the sudden death of a 3-year-old female child who had been clinically diagnosed with Leigh syndrome.Leigh syndrome is a heterogeneous progressive neurodegenerative disorder, which is characterized by focal or bilateral lesions in the thalamus, basal ganglia, brainstem, cerebellum, and spinal cord. Affected patients exhibit a variable clinical picture that frequently includes psychomotor retardation or regression, recurrent episodes of vomiting, failure to thrive, and signs of brainstem and basal ganglia dysfunction.The child was found dead in bed. Autopsy described the presence of symmetrical, necrotizing lesions scattered within the basal ganglia, thalamus, diencephalon, brainstem, and spinal-cord gray matter and revealed the presence of gastric contents in the upper and lower airways. We report the results of genetic investigations and describe the histological and immunohistochemical features that confirmed the diagnosis. These findings suggest that Leigh syndrome should be regarded as predisposing children to sudden death, especially by asphyxia secondary to the neurological disorder.

Potential nerve damage following contact with a piezoelectric device.

OBJECTIVES: The aim of the study was to assess the extent of the potential nerve damage following prolonged contact with a piezoelectric device. METHODS: The study was conducted with 30 patients; all of the patients had cervical metastatic lymph nodes at levels II, III, and IV (N2b) and a negative evaluation for metastatic disease (MO). The patients underwent radical neck dissection. After its skeletonization, the spinal nerve was exposed directly to ultrasonic activation with a piezoelectric device for various times (5, 10, and 20 seconds) and with different inserts (OP3 insert and OT7 insert). The axonal damage was graded from 0 to 3 as follows: 0, no damage; 1, minor axonal damage; 2, severe axonal damage but not covering the entirety of the nerve fascicles; 3, severe axonal damage covering the entirety of the nerve fascicles. RESULTS: Histologic examination showed no evidence of damage to the perineurium and axons after 5 and 10 seconds of exposure to ultrasonic activation with each insert. CONCLUSIONS: Our histologic data highlight the selective action of the piezoelectric device, which reduces the risk of accidental nerve damage in otolaryngological bone surgery.

Treatment of recurrent hepatitis C (genotype 1) with pegylated interferon alfa-2b and ribavirin combination and maintenance therapy.

BACKGROUND/AIMS: This study aims at evaluating the efficacy of treatment with pegylated interferon (PEG-IFN) alfa-2b and ribavirin in patients with recurrent hepatitis C (genotype 1) after orthotopic liver transplantation (OLT) and the impact of this therapy on hepatic fibrosis at the end of conventional therapy and at the end of a period of maintenance treatment in non-responder patients. METHODOLOGY: Thirty-two consecutive patients diagnosed with recurrent HCV were considered candidates for antiviral therapy. RESULTS: Ten patients (31.2%) interrupted therapy due to side effects; sustained virological response (SVR) was observed in 27.2%, sustained biochemical response (SBR) in 31.8% and NR in 40.9% of cases. Eighteen patients underwent a biopsy at the end of conventional treatment: improved fibrosis score in all patients with SVR, improved score in 1 patient with SBR and stable score in 6 patients with SBR, worse score in 1 NR patient and stable in 6 NR patients. Six NR patients with stable score submitted to a maintenance therapy: improved score in 1 patient and stable score in 5 patients. CONCLUSIONS: In recurrent hepatitis C, in spite of the type of response, treatment slows down hepatic fibrotic evolution.

Kidney graft loss associated with JC polyomavirus nephropathy.

This is the first case of documented treatment failure of JCV nephritis, despite a reduction of immunosuppressive agents and the use of antiviral therapy. We consistently detected high levels of JCV viremia, which correlated with the progressive deterioration of the graft and caused the final loss of the kidney, suggesting that JCV plays an etiological role in the onset of severe nephropathy in kidney transplant patients.

Influence of body mass index, cholesterol, triglycerides and steatosis on pegylated interferon alfa-2a and ribavirin treatment for recurrent hepatitis C in patients transplanted for HCV and alcoholic cirrhosis.

Up to today no work has evaluated yet the importance of parameters such Body Mass Index (BMI), cholesterol, triglycerides (TGC) and hepatic percentage of steatosis in the response to therapy with Pegylated Interferon Alfa-2a and Ribavirin in patients with recurrent hepatitis C (genotype 1). 30 consecutive prospectively followed patients diagnosed with recurrent HCV were considered candidates for antiviral therapy. Qualitative and quantitative detection of HCV-RNA was performed with the Cobas Amplicor Hepatitis C Virus Test, version 2.0 and the Cobas Amplicor HCV Monitor, version 2.0 (Roche Diagnostics, Branchburgh, NJ, U.S.A.). HCV genotyping was performed by sequencing of the 5 untraslated region (5' UTR) (Visible Genetics TruGene Hepatitis Assay, Toronto, Canada). The observed distribution of BMI, cholesterol, TGC and steatosis were confirmed to be normally distributed by the one-sample Kolmogorov-Smirnov Goodness of fit test procedure. Comparison of BMI, cholesterol, TGC and steatosis between non responders (NR), sustained virological responders (SVR) and sustained biochemical responders (SBR) groups were analyzed by ANOVA with a post hoc Bonferroni test and correlation between variables was tested by Pearson test. The multivariate analysis was performed to estimate the chance of response on basis of the above mentioned variables. In patients with abnormal results in at least two out of four considered variables the chance of no-response was 40 times higher than that of SBR and 96 times than that of SVR. We can conclude how the management of dismetabolism, diet and exercise therapy can improve BMI, liver histology and, therefore, the response to PEG-IFN Alfa-2a and Ribavirin.

Laparoscopic lymph node biopsy in intra-abdominal lymphoma: high diagnostic accuracy achieved with a minimally invasive procedure.

Ultrasound or computed tomography-guided percutaneous lymph nodes biopsy often do not supply sufficient tissue for the histopathologic diagnosis of a lymphoma. Laparoscopic lymph node biopsy (LLB) has the advantage of obtaining the entire lymph node and avoiding the invasivity and all the possible complications of a laparotomy. The aim of the present study is to assess the safety and diagnostic accuracy of the LLB in intra-abdominal lymphoma. Between April 1999 and October 2005, 36 LLB were performed in 35 patients to rule out or to follow the progression of a lymphoma. The clinical outcome and the pathology reports were analyzed retrospectively. A conversion to laparotomy was necessary in 2 cases due to intraoperative difficulties (5.8%). No major postoperative complications or mortality occurred. Mean hospital stay was 2.1 days. In 9 patients, LLB was performed to follow a possible progression of the lymphoma, whereas in 26 patients it was used to establish a diagnosis. Two repeated LLB were necessary to achieve a correct diagnosis in 1 patient. Fourteen patients had non-Hodgkin lymphoma, 6 patients had Hodgkin lymphoma, 9 patients presented an infiltration by primitive or metastatic tumors, and 7 patients had benign lymphadenopathy. In 97% of the cases, LLB supplied the necessary information for the correct diagnosis, classification, and subsequent therapeutic decisions. In conclusion, LLB is a safe and effective procedure. Its diagnostic accuracy is superior to percutaneous techniques. LLB can be proposed as the procedure of choice to sample deep lymphatic tissues in patients with intra-abdominal lymphadenopathy at a very low morbidity rate and as an outpatient procedure in selected cases.

Unexpected and sudden death due to undiagnosed medulloblastoma in twin pregnancy: A case report.

The authors describe an unusual case of sudden and unexpected death caused by a medulloblastoma in a woman aged 28, native of South America, at the 33rd week of twin pregnancy, with neurological signs appeared a month before death. The initial symptoms were attributed to epiphenomena of pregnancy. Two weeks after hospitalization, the woman showed an acute frontal headache that prevented movement and caused a rapid lowering of arterial oxygen saturation. The patient died around 3h later, despite resuscitation. Immediately after, a caesarean section was performed but it was not enough to prevent the death of the two foetuses. The autopsy revealed the presence of a tumour between the left lobe of the cerebellum and the vermis. Histological examination enabled to identify a medulloblastoma. Death was attributed to acute cardio-respiratory insufficiency caused by compression of the brain stem. Foetuses showed no malformation and their death was due to an acute hypoxia resulting from the mother cardiovascular arrest.

Sudden death due to primary atrial neoplasms: report of two cases and review of literature.

Primary cardiac tumours are very uncommon lesions with an estimated incidence of less than 0.03% during autoptic examination. Among these, about 75% are benign, mainly myxomas. The intracardiac tumours occur over a wide range of ages and can progress silently or have many clinical presentations, such as valvular dysfunction, cardiac compression and embolic accidents, mostly in case of atrial localization. Primary atrial tumours often progress asymptomatically. Sudden death could represent the "first symptom" of these pathological findings. Indeed, cardiac neoplasms may cause disorders of atrioventricular or intraventricular conduction, which are manifested by fatal arrhythmias. Two cases of sudden death due to atrial tumours are reported. A complete autoptic examination, with histologic and immunohistochemical study of cardiac lesions, confirmed that these neoplasms were primary. Very few cases of sudden death due to right atrial tumours have been described in forensic literature, a fact which emphasizes their rarity.

Sudden death due to isolated right ventricular infarction: a case report.

INTRODUCTION: Isolated right ventricular infarction (RVI) is a rare phenomenon associated with atherosclerotic disease of the acute marginal vessels or of a non-dominant right coronary artery. It may happen in the absence of coronary disease when substantial right ventricular hypertrophy is present. The prognosis is usually good, although sudden collapse can occur due to ventricular fibrillation, rupture of the right ventricular free wall, or a massive pulmonary embolism. In this report, a case of sudden death in a patient with an isolated RVI due to an acute thrombosis of a non-dominant right coronary artery is presented. METHODS: A 46-year-old man without previous cardiopulmonary disease died suddenly at home. A medicolegal autopsy was performed within 72 h in order to clarify the circumstances that suddenly led the man to an unexpected death. Samples were collected for histological, immunohistochemical, and toxicological examination. RESULTS: The postmortem investigation revealed central cyanosis, polyvisceral stasis, and pulmonary oedema. The macroscopic examination of the heart showed left and right ventricular hypertrophy. A fresh thrombus located in the right coronary artery accompanied by a haemorrhagic infiltration of the posterolateral right ventricular wall was found. Microscopic findings confirmed the observations from the autopsy and showed miliary necrosis of the right ventricular wall. Toxicology was negative for drugs and alcohol. CONCLUSIONS: On the basis of morphologic and microscopic data, the cause of death was determined to be an isolated RVI. The autopsy findings of both right and left ventricular hypertrophy associated with a nondominant right coronary artery thrombosis were observed. In cases like this, the authors would like to underline the importance of a complete postmortem examination and a full pathological approach.

Early effects of portal flow modulation after extended liver resection in rat.

INTRODUCTION: The incidence of small-for-size-liver-syndrome after liver transplantation and extended liver resection may be reduced by portal flow modulation. However, many aspects of the small-for-size-liver-syndrome pathogenesis are still unclear. In this experimental study we evaluated the early effects of portal flow modulation after 80% hepatic resection in rats. MATERIALS AND METHODS: Rats were randomised in: sham operation (G1), conventional hepatic resection (G2), splenectomy and hepatic resection (G3), splenic transposition followed by hepatic resection after three weeks (G4). Six hours after operation, oxygen saturation of hepatic vein blood, glutathione, and standard liver markers were measured from hepatic venous blood. Glutathione measurement and histopatological examination were performed in the remnant liver. RESULTS: Total bilirubin and liver glutathione did not show differences between groups. Aspartate aminotransferase and alanine aminotransferase significantly increased in G2-G4 groups. Blood glutathione and oxygen saturation of hepatic vein blood were lower in G2 than in other groups. A gradient of micro-vesicular degeneration was more severe in G2 compared with G3 and G4. Apoptosis, hemorrhagic necrosis, mitochondrial damage and leucocyte adhesion were evident in G2. CONCLUSION: The portal flow modulation induced by splenectomy or splenic transposition was effective in limiting early damage after extended liver resection.

Two fatal cases of hidden pneumonia in young people.

Acute respiratory distress syndrome (ARDS) is a severe lung disease characterized by inflammation of the lung parenchyma leading to impaired gas exchange. This condition is often lethal, usually requiring mechanical ventilation and admission to an intensive care unit. We present two fatal cases of hidden pneumonia in young people and discuss the pathophysiological mechanism of ARDS with reference to the histological pattern. A complete forensic approach by means of autopsy and histological, immunohistochemical, and microbiological, examination was carried out. In both cases the cause of death was cardio-respiratory failure following an acute bilateral pneumonia with diffuse alveolar damage and ARDS associated with sepsis and disseminated intravascular coagulation. Our cases suggest on one side the importance of an early diagnosis to avoid unexpected death while on the other that the diagnosis of ARDS has to be confirmed on the basis of a careful postmortem examination and a complete microscopy and microbiological study.

Death caused by toxic epidermal necrolysis (Lyell syndrome).

Toxic epidermal necrolysis (TEN) is characterized by fever, scalded appearance of the skin, and epidermolysis associated to blister formation and exfoliation, and it is caused by hypersensitivity reaction to a drug. The authors report two cases of death as a result of TEN; both referred to old aged women treated with a polytherapy including allopurinol. Both patients displayed erythematous skin lesions similar to scald burns and epidermolysis at the face, chest, and abdomen and died shortly after hospitalization. Autopsy findings and histological examinations revealed epidermal necrolysis and confirmed the clinical diagnosis. A strict time-correlation between allopurinol administration and symptoms was evidenced. Because of its iatrogenic origin, TEN often arises suspicions of medical liability; however, because of its unpredictable nature, the occurrence of this syndrome cannot be ascribed to the medical staff whose main task is the rapid diagnosis and the correct management.

Primitive malignant melanoma of the parotid gland.

Melanoma of the parotid gland is a very rare event: it can occur as a primary disease or as spread of the intraglandular nodes. The authors report a case and review the few cases of primary melanoma of the parotid gland reported in the literature.