An atypical presentation of necrotising fasciitis.

Necrotising fasciitis is a rare infection with a high mortality rate. Clinical manifestation may be influenced by an underlying disease state. We report a case of a 70-year-old man with an anaplastic large-cell lymphoma who presented with inconclusive signs of necrotising fasciitis following a neutropenic phase after chemotherapy. Surgical exploration did not reveal the typical macroscopic features of necrotising fasciitis. Microbiological investigations revealed Escherichia coli. The lack of massive tissue inflammation, probably due to the neutropenia in our patient, has not been described by other authors. Diagnostic uncertainties owing to masked or uncharacteristic signs in immune compromised patients may lead to a delayed surgical debridement, of which clinicians should be aware.

[Proposals for standardization of autopsy reports]. / Vorschläge zur standardisierten Abfassung von Obduktionsberichten.

The decline in the number of autopsies not only in Germany has been attributed to several reasons. Complaints of clinicians about a non-uniform layout of autopsy reports may be one important reason. The lack of initiatives concerning standardization of autopsy reports contributes to a poor comparability of autopsy reports from different institutions and even within one institution. In order to promote harmonization of a protocol for autopsy results proposals for a standardization of autopsy protocols are given. These proposals are based on using modules for different parts of the autopsy protocols, thus giving different institutions the possibility to modify these modules.

[Autopsy as a tool for quality assurance: Leipzig]. / Obduktion als Instrument der Qualitätssicherung--Leipzig.

Autopsy rates have declined throughout the world although autopsy serves as an important tool in quality management. Working together with the Department of Heart Surgery of the Heart Centre in Leipzig clinical and autopsy diagnoses were compared. Between 2000 and 2003 an autopsy was performed in 468 patients. Data from clinical and postmortem examinations were prospectively analysed regarding causes of death, concomitant diseases and postoperative complications. Discrepancies between clinical and postmortem cause of death were identified in 23.1% of these patients. In 99.1% of the patients concomitant diseases (e.g. myocardial hypertrophy, pulmonary embolism, former stroke) could be identified, 57.6% were unknown pre-mortem and 19.2% were considered to be of relevance for surgical therapy. Autopsy is the final and most comprehensive physical examination. Autopsy is also the basis of training for pathologists in interpreting interrelated diagnoses. Therefore the task of pathologists is to enhance the appreciation of autopsy among clinical colleagues by working closely side by side. By communication an autopsy can deliver an effective achievement in terms of being relevant for praxis.

[Lymphomatoid granulomatosis--a short description of an unusual case of the disease]. / Lymphomatoide Granulomatose--eine kurze Darstellung der Erkrankung anhand eines aussergewöhnlichen Falles.

Lymphomatoid granulomatosis (LYG) is a rare disease with an unknown pathogenesis. It is considered as a B-cell disorder with an uncertain malignant potential. The disease is classified as an angiocentric and angiodestructive lymphoproliferative disorder. EBV-association (LMP1) and the detection of the surface antigens CD20 and CD30 are the characteristic pathological findings. The lung, the nervous system, the kidneys and the liver are affected most frequently. In the present report the case of a 79-year-old man is described, who showed a cavity in the upper right lobe which was opened out into the trachea. The initial CXR and CT scan demonstrated a large nodular air space opacity (reversed halo sign) on the upper right area and a smaller one on the left side. In addition to CT and bronchoscopy, a surgical biopsy was performed to confirm the diagnosis of lymphomatoid granulomatosis. Treatment consisted in chemotherapy with rituximab, cyclophosphamide, vincristine and prednisolone (R-CVP). Three months after confirmation of diagnosis the patient died of an abscess-forming pneumonia.

[Evaluation of the clinical utility of autopsy: an investigation at the Institute of Pathology, University Hospital of Leipzig]. / Evaluation des klinischen Nutzens der Obduktion: Eine Untersuchung am Institut für Pathologie des Universitätsklinikums Leipzig.

BACKGROUND: The aim of this study of autopsies performed in our institute was to investigate whether clinical requirements had been met and specific clinical questions answered. METHOD: With each autopsy report, a questionnaire was sent to the clinic where the patient had died. The clinicians evaluated how often additional diagnoses were made and whether these were clinically relevant. RESULTS: Seventy-two percent of all autopsies resulted in further diagnoses. Clinicians estimated that these could have influenced their clinical management in 36% of cases. Weaknesses in interdisciplinary cooperation were uncovered and solved. CONCLUSION: The great benefit of the autopsies conducted in our institute was demonstrated. The results emphasise once again the importance of autopsy for quality assurance in clinical medicine.

[Ultrasound guided transthoracic biopsy - safety and efficaces]. / Ultraschall gestützte Biopsie mit Durchleuchtungskontrolle zur Diagnostik thoraxwandnaher Raumforderungen - sicher und diagnostisch effektiv.

BACKGROUND: The biopsy of pulmonary neoplasms localized in close proximity of the thoracic wall and the histological examination of the specimens represent an important diagonstic tool in the work up of benign and malign pulmonary tumours. The biopsy techniques differ with respect to the guiding imaging method, the devices used and the quantity of specimen. This study investigates efficacy and safety of the ultrasound guided transthoracic biopsy. PATIENTS: 46 consecutive patients (n = 37 male; n = 9 female) with a mean age of 64.4 years were included in this analysis. 13 patients suffered from COPD with a mean residual volume in per cent of total lung capacity of 61.9 +/- 12.6 %. 43 pulmonary or pleural and 3 mediastinal neoplasms were investigated. The neoplasm reached the pleura in 41 cases. The mean tumours sizes was 59.1 +/- 30.6 mm. METHOD: The target structure was localized by ultrasound. The ultrasound probe and in its orientation to the target structure was fluoroscopically visualized. Local anaesthesia and sampling were performed in the same orientation under fluoroscopic control. Using the BioPince device (Medical Device Technologies, Inc.; Florida, USA; length 150 mm; Gauge: 18) two to five biopsy cylinders were taken. Chest X-ray was performed within some hours to rule out pneumothorax. RESULTS: The histological examination provided the diagnosis in 44 of 46 cases (95.6 %): lung cancer n = 29, other malign tumours n = 9; benign tumours n = 6). Complications were observed in three patients: haemoptysis, cutaneous emphysema (without need for intervention), pneumothorax with consecutive chest tube placement (one case each). CONCLUSION: The ultrasound guided transthoracic biopsy may be regarded as a safe diagnostic procedure. Complications, even in patients with structural lung disease are in the range of comparable techniques.

[Granular cell tumor of the orbit]. / Granularzelltumor der Orbita.

Granular cell tumor (GCT, Abrikossoff tumor) is an extremely rare, benign tumor with a neurogenic origin occurring most commonly in the upper aerodigestive tract. GCT of the orbit is a rarity, representing a diagnostic and therapeutic challenge. We report on a 26 year old man suffering from unilateral ocular hypertension and retrobulbar pain. Exophthalmos and disturbance of motility were not noted by the patient. The surgical removal of the GCT, extending into the orbital apex, was performed by an interdisciplinary team using a combination of "midfacial degloving" and LeFort I-osteotomy.

Autopsy findings in patients on postcardiotomy extracorporeal membrane oxygenation (ECMO).

OBJECTIVES: To assess the clinical sensitivity of causes of death, concomitant diseases and postoperative complications including thromboembolic events in ECMO patients. METHODS: Between January 2000 and December 2004 154/202 patients (76.2%) died after postcardiotomy ECMO circulatory support. Autopsy was performed in 78 (50.6%) consecutive patients. Clinical and post-mortem data were prospectively recorded and compared concerning causes of death and postoperative complications including venous and arterial thromboembolisms and significant comorbidities. RESULTS: Mean age was 62.1+/-11.3 years, ejection fraction was 43.4+/-17.3%. 39.7% were emergency operations including acute coronary syndrome in 25.6% and preoperative cardiogenic shock in 28.2%. Successful ECMO weaning rate was 43.6%. Mean postoperative survival was 11.3 days. Premortem unknown concomitant diseases were found in 63 patients (80.8%) with clinical relevance in 9 patients (11.5%). Clinically unrecognized postoperative complications were found in 59 patients (75.6%) including acute cerebral infarction (n=7), acute bowel ischemia (1), intestinal perforation (3), pneumonia (4), venous thrombus formation (25) and systemic thromboembolic events (24). Clinically based causes of death were cardiac in 62.8%, multi-organ failure in 10.3%, cerebral in 5.1%, respiratory in 10.3%, fatal pulmonary embolism in 2.6%, technical in 5.1%, and others in 3.8%. Unexpected causes of death were found by autopsy in 22 patients (28.2%) including myocardial infarction (n=5), acute heart failure (4), fatal pulmonary embolism (2), pneumonia (2), ARDS (1), lung bleeding (1), fatal cerebrovascular event (4) and multiorgan failure (3). CONCLUSIONS: In ECMO patients major discrepancies between clinical and post-mortem examination were found. The true incidence of thromboembolic events is highly underestimated by clinical evaluation.

[Facioscapulohumeral muscle dystrophy and heart disease]. / Fazioskapulohumerale Muskeldystrophie mit Herzbeteiligung.

Cardiac involvement is well known in a number of skeletomuscular diseases but not in facio-scapulohumeral muscular dystrophy (FSHD). We report on a 71 year old woman with progressive cardiac insufficiency in FSHD, which was also confirmed by molecular analysis in one of the two daughters affected by the disease. Autopsy of the deceased patient showed the typical changes in skeletal muscles including focal inflammatory infiltrates in the diaphragm and, in addition, cardiac muscular involvement. The histological changes resembled those seen in primary cardiomyopathy despite the normal muscle mass volume. Both clinically and morphologically, the cardiac disease was the cause of death in this patient with FSHD.