Xanthogranulomatous cholecystitis: report of a case and literature review on clinical differentiating factors from gallbladder carcinoma

Xanthogranulomatous cholecystitis (XGC) is a rare, benign, destructive inflammatory disease of the gallbladder that is assumed to be a variant of chronic cholecystitis. We herein present a rare case of XGC, which simulates gallbladder carcinoma with extensive involvement of the liver, omentum and the biliary trees. At surgery, total cholecystectomy with partial hepatectomy of the gallbladder bed and excision of adjacent xanthogranulomatous tissue was performed, but bilio‐enteric anastomosis for biliary decompression, which was the procedure preoperatively planned, was impossible to indicate because the common bile duct could not be clearly exposed by its infiltration showing mass formation. Therefore, retrograde transhepatic biliary drainage was eventually indicated for subsequent endoscopic therapy using stent placement to deal with the biliary structure caused by XGC. The patient has been leading a normal life after stent placement in the biliary tract for 6 months duration without any symptoms suggesting biliary stricture. In conclusion, XGC can simulate gallbladder cancer in its clinical presentation, radiological findings and even gross operative features. It is important to make preoperative ultrasound‐guided fine‐needle aspiration cytological diagnosis or intraoperative pathological diagnosis in order to avoid misdiagnosis and unnecessary therapy. Cholecystectomy, excision of adjacent xanthogranulomatous tissue, which often includes partial hepatic resection, are still the best management of XGC.     

Xanthogranulomatous cholecystitis mimicking gallbladder carcinoma: An analysis of 42 cases

AIM: To review and evaluate the diagnostic dilemma of xanthogranulomatous cholecystitis (XGC) clinically.METHODS: From July 2008 to June 2014, a total of 142 cases of pathologically diagnosed XGC were reviewed at our hospital, among which 42 were misdiagnosed as gallbladder carcinoma (GBC) based on preoperative radiographs and/or intra-operative findings. The clinical characteristics, preoperative imaging, intra-operative findings, frozen section (FS) analysis and surgical procedure data of these patients were collected and analyzed.RESULTS: The most common clinical syndrome in these 42 patients was chronic cholecystitis, followed by acute cholecystitis. Seven (17%) cases presented with mild jaundice without choledocholithiasis. Thirty-five (83%) cases presented with heterogeneous enhancement within thickened gallbladder walls on imaging, and 29 (69%) cases presented with abnormal enhancement in hepatic parenchyma neighboring the gallbladder, which indicated hepatic infiltration. Intra-operatively, adhesions to adjacent organs were observed in 40 (95.2%) cases, including the duodenum, colon and stomach. Thirty cases underwent FS analysis and the remainder did not. The accuracy rate of FS was 93%, and that of surgeon’s macroscopic diagnosis was 50%. Six cases were misidentified as GBC by surgeon’s macroscopic examination and underwent aggressive surgical treatment. No statistical difference was encountered in the incidence of postoperative complications between total cholecystectomy and subtotal cholecystectomy groups (21% vs 20%, P > 0.05).CONCLUSION: Neither clinical manifestations and laboratory tests nor radiological methods provide a practical and effective standard in the differential diagnosis between XGC and GBC.     

Clinical course of esophageal stricture managed by bougienage

To define the clinical course of patients with benign esophageal stricture treated with bougienage, we reviewed the records of 76 patients undergoing dilatation. Patients with the diagnosis of scleroderma and those with previous hiatal hernia repair or gastric surgery were excluded. Initial evaluation included contrast study, esophagoscopy, and biopsy. Patients underwent a series of dilatations until a 44-Fr or larger bougie was passed. Patients were then instructed to return for recurrence of dysphagia. In this group, mean duration of follow-up from the first dilatation was 21.1 months. A total of 569 dilatations were performed with one major complication and no mortality. Benign esophageal stricture recurred in 65% of patients. After two or more recurrences, the likelihood of requiring an additional dilatation was 86–94% after each recurrence. The interval between required dilatations was variable; however, after 8 dilatations, it approximated once monthly. The shorter mean follow-up time of patients requiring a single dilatation (9.7 months) compared to those requiring multiple dilatations (28.8 months) suggests that the recurrence rates noted in this study are underestimations. In spite of high recurrence rates and short recurrence intervals, the low morbidity and absent mortality over long-term follow-up suggests that repeated bougienage is, an effective modality for the management of benign esophageal stricture.     

Xanthogranulomatous cholecystitis: the use of preoperative CT findings to differentiate it from gallbladder carcinoma

Background and aim

A retrospective analysis was performed on 32 patients with histologically confirmed xanthogranulomatous cholecystitis (XGC) and 21 patients with gallbladder carcinoma who underwent surgical treatment between 1998 and 2007.

Methods

All patients underwent preoperative CT scanning. The CT features analyzed were: the presence of intramural hypoattenuated nodules or bands, mucosal line, the patterns of wall thickening and enhancement, and the presence of stones in the gallbladder. The variables of the CT findings with XGC were analyzed using multivariate logistic regression analysis.

Results

Intramural hypoattenuated nodules were observed in 21 patients (65%) with XGC, but in only six patients (29%) with gallbladder carcinoma (P < 0.01). The mucosal line was observed in 27 patients (84%) with XGC and in only four patients (19%) with gallbladder carcinoma (P < 0.0001). Gallstones were noted in 24 patients (75%) with XGC and five patients (24%) with gallbladder carcinoma (P < 0.001). There was no significant difference in the pattern of gallbladder wall thickening (diffuse or focal) and the presence of changes outside the gallbladder. Multivariate logistic regression analysis revealed from the CT features that the enhanced continuous mucosal line (P = 0.0013) and the presence of gallstones (P = 0.0072) were independently correlated with XGC.

Conclusion

CT features of the enhanced continuous mucosal line in a thickened gallbladder wall, together with gallstones in a patient with chronic gallbladder disease, are highly suggestive of XGC. Accurate diagnosis of XGC may therefore indicate the need to select a less aggressive surgical approach.     

Xanthogranulomatous cholecystitis: case series in a rural area, clinicopathological study and review of literature

Xanthogranulomatous cholecystitis is a destructive inflammatory disease of the gallbladder, rarely involving adjacent organs and mimicking an advanced gallbladder carcinoma. The diagnosis is usually possible only after pathological examination. We are reporting two of such rare cases in female patients attending our institute. In both patients xanthogranulomatous cholecystitis was diagnosed on histopathology.     

Opsoclonus-myoclonus syndrome with severe clinical course and beneficial outcome: A case report

Rationale:Opsoclonus-myoclonus syndrome (OMS) is a rare immune-mediated movement disorder, mostly of paraneoplastic or idiopathic origin. The disease usually has an acute onset, serious course and leads rapidly to disability in adult patients. To the best of our knowledge, this is the fourth presented case of OMS with a severe course and complete reversibility of neurological symptoms in a pregnant woman. This report includes videos and a literature review.Patient concerns:A 30-year-old woman in the 12th week of pregnancy developed severe nausea and vomiting, after several days balance and gait disorders appeared. On admission to hospital, neurological examination revealed opsoclonus, dysarthria, myoclonic jerks with ataxia of the trunk and limbs with inability to sit, stand or walk.Diagnosis:Well-known causes of OMS were excluded. Although in our patient the idiopathic origin of the disorder was taken under consideration, diagnosis of opsoclonus-myoclonus related to the pregnancy was highly likely.Interventions:After administration of steroids and benzodiazepines the patient improved.Outcomes:In the 6th month of pregnancy, after termination of immunotherapy, she recovered completely and was able to sit, stand and walk independently. In the 39th week of pregnancy, she delivered a healthy child.Lessons:We confirm that understanding of clinical symptoms and rare causes of OMS contributes to early diagnosis and therapy, which ensures an optimal outcome. One probable cause of OMS could be a physiological change to immune system regulation during pregnancy. The relationship between OMS and pregnancy remains uncertain and needs further investigation.     

Role of inflammation markers in the evaluation of the clinical course of myocardial infarct

The authors examined the time course of changes in the levels of chlorine soluble mucoprotein and glycosaminoglycans as markers of an acute period of myocardial infarction. A significant correlation was established between the mass of the infarcted myocardium and the severity of its acute reaction as evaluated from the integral volume of various inflammation markers. A mathematical model of this relationship was proposed for patients with complicated and uncomplicated myocardial infarction. It was shown that it was possible to apply these parameters to the assessment of the severity and features of a myocardial infarction course.     

Predictive factors for a severe clinical course in ulcerative colitis: Results from population-based studies

Ulcerative colitis(UC)is characterized by chronic inflammation of the large bowel in genetically suscep-tible individuals exposed to environmental risk factors.The disease course can be difficult to predict,with symptoms ranging from mild to severe.There is no generally accepted definition of severe UC,and no single outcome is sufficient to classify a disease course as severe.There are several outcomes indicating a severe disease course,including progression of the disease’s extension,a high relapse rate,the development of acute severe colitis,colectomy,the occurrence of colorectal cancer and UC-related mortality.When evaluating a patient’s prognosis,it is helpful to do so in relation to these outcomes.Using these outcomes also makes it easier to isolate factors predictive of severe disease.The aims of this article are to evaluate different disease outcomes and to present predictive factors for these outcomes.     

Heart failure caused by severe systolic ventricular dysfunction of hypertensive origin. Long-term clinical and functional course

AIMS: The aims of our study were to evaluate survival and evolution of functional class and ventricular function in patients with severe congestive heart failure due to dilated cardiomyopathy. METHODS: Inclusion criteria were: class III or IV heart failure, dilated left ventricle with ejection fraction < 40%, history of poor controlled hypertension, and exclusion of other etiologies for heart failure. We studied 17 patients with these features; mean age was 64 +/- 7 years and 70% were male. They were followed up during a mean period of 3.3 +/- 1 years (2 to 6; median 3 years). RESULTS: Baseline left ventricular ejection fraction was 30 +/- 5% (20 to 40); 35% of patients were in functional class III and 65% in class IV; 100% of patients received ACE inhibitors and diuretics, 53% betablockers, and 35% calcium-antagonists. Survival was 100%. Left ventricular ejection fraction increased from 30 +/- 5% to 44 +/- 11% at one year, to 50 +/- 11% at 3 years and 51 +/- 10% at the end of follow-up (p < 0.001). This improvement was mainly due to a reduction in systolic left ventricular diameter (from 51 +/- 4 mm to 42 +/- 11 mm, p < 0.01), since diastolic diameter did not significantly changed (63 +/- 4 to 59 +/- 11 mm). CONCLUSIONS: The evolution of severe systolic left ventricular dysfunction due to arterial hypertension is favourable at long-term, with null mortality and clinical ejection fraction and functional improvement. Nevertheless, the persistence of left ventricular dilatation suggests that myocardial damage caused by chronic pressure overload does not disappear.     

Differences in ways of development of myocardial infarct with severe and mild clinical course

In 43 dead and 83 survived patients with myocardial infarction, the severity of the disease was compared with hourly changes in indications of a precordial cardiogram made in the first 2 days. The signs were defined, which made it possible to predict a relapse of myocardial infarction 10-15 hours before its onset. The rate of transformation of myocardial lesion foci to necrosis was found to affect the lifespan of patients. The end mass of myocardial infarction was shown to be formed via confluence of individual necrotic foci which appear in the myocardium at various intervals. The early signs were described, which enabled severe cases of myocardial infarction, resulting in death to be differentiated from those leading to a patient's recovery.     

Long-term clinical course of a patient with anti PL-12 antibody accompanied by interstitial pneumonia and severe pulmonary hypertension

We report a case of a patient with anti PL-12 antibody accompanied by interstitial pneumonia and severe pulmonary hypertension. At first presentation, hyperkeratotic skin lesions were found, although the diagnosis of CVD was not conclusive. Lung histology showed diffuse fibrosing interstitial pneumonia predominantly in the subpleural regions. During the seven-year follow-up period, severe pulmonary hypertension developed, although the progression of lung fibrosis was relatively limited. Anti-PL12 antibody was detected, and therefore the patient was diagnosed as having antisynthetase syndrome. Lung histology and pulmonary arteriogram suggested that vascular involvement of the disease contributed to the development of severe pulmonary hypertension.     

The aetiology and course of isolated severe aortic regurgitation: a clinical, pathological, and echocardiographic study

Seventy two consecutive patients with severe isolated aortic regurgitation were evaluated by preoperative echocardiographic and angiographic assessment of the aortic root. Biopsy specimens of the aortic wall were taken at operation. Two major groups of patients were found: those with cusp derangement but normal aortic roots and those with normal cusps but dilated aortic roots. Of the 42 cases of abnormal cusps, 20 were rheumatic, 15 were infective, and six were bicuspid. One patient had a tear in an otherwise normal cusp. Of the 30 cases of abnormal roots but normal cusps, six had inflammatory changes (syphilis, Reiter's disease, giant cell aortitis) and 24 had root dilatation caused by non-inflammatory destruction of elastic laminae. Echocardiographic measurement of the aorta at the level of the top of the commissures predicted the findings at pathology. In 37 of 39 patients with cusp disease the measurement was less than 37 mm. In 27 of 33 patients with root disease the measurement was greater than or equal to 37 mm. This difference was statistically significant. There was no difference in the sizes of the prosthesis used in each group, suggesting that it was the diameter of the junction of the aorta with the sinuses rather than the junction of the sinuses with the ventricle that was important in aortic regurgitation. Clinical progression in patients with non-inflammatory aortic root disease is slower than in patients with infective disease but faster than in those with rheumatic cusp disease.     

Magnetic resonance imaging assessment of spinal inflammation in ankylosing spondylitis: Standard clinical protocols may omit inflammatory lesions in thoracic vertebrae

Objective

Radiologic assessment of spinal inflammation in patients with ankylosing spondylitis (AS) relies primarily on magnetic resonance imaging (MRI), although little is known about the distribution of inflammatory lesions within the structures of the spine. Our objective was to compare the distribution of inflammatory lesions centrally and laterally within the thoracic and lumbar spine vertebral bodies.

Methods

We studied 49 patients with AS who were scanned with STIR and T1‐weighted spin‐echo MRI of the whole spine. Scans were read by 2 musculoskeletal radiologists, with a third reader as the arbitrator. Controls included 6 age‐matched individuals. We recorded bone marrow edema on STIR images from each vertebral body, separately identifying central and lateral slices. The latter were defined as images that included or were lateral to the pedicle. Interreader reproducibility was assessed by kappa statistics.

Results

Inflammation was present in 263 (45%) of 588 thoracic and 86 (35%) of 245 lumbar vertebrae; the mean number of affected thoracic and lumbar vertebrae per patient were 5.4 and 1.8, respectively. Inflammation was present in the lateral aspect of 219 (37%) of 588 thoracic vertebrae and 45 (18%) of 245 lumbar vertebrae (P < 0.001). Lesions were more common laterally than centrally for all thoracic vertebrae except for T7. Involvement of only the lateral slices was observed in as many as 19.6% of thoracic vertebrae.

Conclusion

Evaluation of spinal inflammation by MRI may omit lesions in up to 20% of inflamed thoracic vertebrae if both scanning and image assessment do not include sagittal slices that extend to the lateral edges of all vertebrae.     

Outpatient treatment of deep venous thrombosis: a clinical care pathway managed by the emergency department

STUDY OBJECTIVE: We evaluate the effectiveness and safety of an outpatient clinical care pathway for the initial treatment of acute proximal lower-extremity deep venous thrombosis (DVT) with low molecular weight heparin (LMWH) managed by the emergency department of 2 affiliated community hospitals. METHODS: This observational, retrospectively defined, population-based study with 39(1/2) months of preintervention analysis and 32(1/2) months of postintervention analysis was conducted in 2 suburban EDs of a large group model health maintenance organization. Our outpatient DVT clinical care pathway used careful patient selection and multidisciplinary follow-up. Ninety-six patients before the intervention and 178 patients after the intervention met eligibility criteria for the pathway. Adverse events during the first 2 weeks of treatment included symptomatic pulmonary embolism (PE), progressive DVT, minor and major bleeding, and death. RESULTS: Demographic and baseline clinical characteristics of the 2 groups were similar. Five (5.2%) of 96 preintervention subjects (95% confidence interval [CI] 2.4 to 8.1) developed adverse events compared with 5 (2.8%) of 178 postintervention subjects (95% CI 1.5 to 4.1; difference between groups 2.4%; P =.50). In each group, 1 (1.0% versus 0.6%) subject developed a PE, 2 (2.1% versus 1.1%) developed progressive symptoms of progressive DVT, and 2 (2.1% versus 1.1%) developed minor bleeding. Major bleeding occurred in 1 (1.0%) preintervention subject and no postintervention subjects. No patient in either cohort died. CONCLUSION: Managed by the ED, an outpatient DVT clinical care pathway using careful patient selection and an integrated multidisciplinary approach can provide a similar degree of effectiveness and safety as customary inpatient therapy.     

The aetiology and course of isolated severe aortic regurgitation: a clinical, pathological, and echocardiographic study.

Seventy two consecutive patients with severe isolated aortic regurgitation were evaluated by preoperative echocardiographic and angiographic assessment of the aortic root. Biopsy specimens of the aortic wall were taken at operation. Two major groups of patients were found: those with cusp derangement but normal aortic roots and those with normal cusps but dilated aortic roots. Of the 42 cases of abnormal cusps, 20 were rheumatic, 15 were infective, and six were bicuspid. One patient had a tear in an otherwise normal cusp. Of the 30 cases of abnormal roots but normal cusps, six had inflammatory changes (syphilis, Reiter's disease, giant cell aortitis) and 24 had root dilatation caused by non-inflammatory destruction of elastic laminae. Echocardiographic measurement of the aorta at the level of the top of the commissures predicted the findings at pathology. In 37 of 39 patients with cusp disease the measurement was less than 37 mm. In 27 of 33 patients with root disease the measurement was greater than or equal to 37 mm. This difference was statistically significant. There was no difference in the sizes of the prosthesis used in each group, suggesting that it was the diameter of the junction of the aorta with the sinuses rather than the junction of the sinuses with the ventricle that was important in aortic regurgitation. Clinical progression in patients with non-inflammatory aortic root disease is slower than in patients with infective disease but faster than in those with rheumatic cusp disease.