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Lege Artis Medicinae

MAY 16, 2007

[ERDHEIM-CHESTER DISEASE: A RARE FORM OF HISTIOCYTOSIS]

NÉMETH István, BOGÁTS Gábor, KAISER László, TISZLAVICZ László

[INTRODUCTION - Erdheim-Chester disease is rare non-Langerhans cell histiocytosis with multisystem involvement. The histological hallmark of the disease is CD1a negative histiocyte proliferation. CASE REPORT - Histiocytosis presented in a 57- year-old Caucasian man with exophtalmus, which was after two years followed by progressive pericardial infiltration and effusion leading to cardiac compression and consequent functional disturbance. It was the histological analysis of a surgical sample removed during pericardial fenestration that revealed Erdheim- Chester disease. The patient died from multisystem involvement one year after the initial diagnosis. Postmortem examination showed long bone, orbital cavity and cranial cavity involvement as well as extensive retroperitoneal and mediastinal xanthogranulomatous infiltration. CONCLUSIONS - The clinical diagnosis of Erdheim-Chester disease is difficult because the symptoms are insidious and non-specific. The histological report is often only descriptive. The prognosis of the disease is poor due to the lack of targeted therapy and to the advanced stage at diagnosis.]

Hungarian Radiology

OCTOBER 20, 2006

[The role of ultrasonography and X-ray examinations in the diagnosis of gallstone ileus in preoperative stage]

FARKAS József, KÁNYA László, LUDVIG Zsuzsanna, BENDE Sándor

[INTRODUCTION - Gallstone ileus develops in elderly patients as a result of complication of cholelithiasis and causes 1-3% of mechanical ileus. Due to its variable presentation and in many cases insidious and intermittent symptoms it is difficult to establish the diagnosis which is often delayed. The mortality rate is high and early diagnosis is essential. The role of X-ray, abdominal ultrasound and recently CT has been emphasized. The role of imaging studies was evaluated in the preoperative diagnosis of gallstone ileus based on their surgically proven cases. PATIENTS AND METHODS - From 1st January 1988 to 30th June 2004 nineteen operations were performed on seventeen patients suffering from gallstone ileus. The average age of the patients was 74.2 years, male/female ratio was 4/13. Ultrasound examination was performed in all cases before the operation. Plane X-ray examination also was carried out except in two cases. CT study was not performed preoperatively. The calculi were removed through an enterotomy whole. In four cases gall bladder were also removed and the biliary fistula was closed. The disease was diagnosed on the basis of symptoms described by Rigler. If two signs from the three was present the diagnosis was established. RESULTS - 17 cases out of 19 mechanical ileus were diagnosed, in one case acute cholecystitis and in one incarcerated abdominal wall hernia was suspected before surgery. Small bowel obstruction was found in all cases (jejunum in six cases, ileum in 13). In five cases multiple calculi were seen in the bowels. Two patients had to undergo surgery twice because of gallstone ileus. It is noted that in eight cases (42.1% of total operations) gallstone ileus was diagnosed before the operation. In seven cases ultrasound played a crucial role in establishing diagnosis. In one case gas was detected in the biliary tract and in six, gallstone was directly seen in the small bowel with ultrasound. CONCLUSIONS - In the preoperative diagnosis of gallstone ileus more and more is expected from appropriate radiological examinations. If diagnosis is primarily based on the physical examination, an illusion of clinical improvement can be created, and the delay of surgical treatment can lead to decompensation of elderly patients with increased mortality rate. A thorough radiological examination can show the stone in the bowel lumen, and observation of the indirect signs together with clinical state can considerably improve the results. The rate of correct diagnosis of gallstone ileus before sugery in our patients can be considered an average and similar to the published data in medical literature.]

Hungarian Radiology

APRIL 07, 2009

[Xanthogranulomatous cholecystitis]

BERÉNYI Zsolt, MORVAY Zita, PALKÓ András

[INTRODUCTION - The xanthogranulomatous cholecystitis is a rare and benign form of lesions associated with diffuse thickening of the gall bladder wall. It is important to recognize it radiologically because it can be mistaken easily for gall bladder carcinoma. The characteristic US, CT and MR findings, however, may be helpful in the differential diagnosis. CASE REPORT - We present the cases of two middleaged female patients suffering from right upper quadrant, radiating abdominal pain for several weeks without occurrence of fever. In both patients, the ultrasound examination revealed marked thickening of the gall bladder wall containing hypoechoic nodules. Further, non-specific sign such as cholecystolithiasis and fine infiltration of the adipose tissue surrounding the gall bladder and dilatation of extrahepatic or intrahepatic bile ducts were visible. On the post contrast CT images, rim enhancement was detectable. MR/MRCP examination showed a sharp delineation of the gall bladder from the liver parenchyma. Both patients underwent cholecystectomy. The pathological examination excluded malignancy and confirmed the diagnosis of xanthogranulomatous cholecystitis. CONCLUSION - The characteristic features of xanthogranulomatous cholecystitis (hypoechoic xanthogranulomas in the markedly thickened gall bladder wall and the presence of calculi) can be detected by ultrasound examination. CT or MRI may play an important role in confirmation of the diagnosis of an inflammatory process and provide useful information in exclusion of gall bladder carcinoma.]