scholarly journals Clinical and Endoscopic Characteristics of Chinese Cronkhite-Canada Syndrome Patients: A Retrospective Study of 103 cases

2021 ◽  
Author(s):  
Yuanyuan Lu ◽  
Fengxing Huang ◽  
Youwei Wang ◽  
Juan Zhou ◽  
Qiu Zhao ◽  
...  

Introduction: Cronkhite-Canada syndrome (CCS) is a rare non-inherited disease characterized by extensive gastrointestinal (GI) polyposis and ectodermal dysplasia. So far, most of CCS related literatures are published as single case report or reviewed with limited case numbers. Our study was to update the clinical and endoscopic characteristics of Chinese CCS patients. Methods: This retrospective study was conducted in 103 Chinese CCS patients (102 cases from literatures and 1 case from our department). Their clinical and endoscopic data were collected, and statistical analyses were performed. Results: 1) In Chinese population, people aged 50-70 years (62.62%) had a high incidence of CCS, and the ratio of male-to-female was 2.68:1. 2) The diverse range of GI manifestations were observed in all the patients, and almost all the patients had at least one symptom of ectodermal dysplasias. 3) All CCS patients presented multiple polyps in the GI tract except esophagus, and the size and appearance of polyps were diverse. Congestion, edema and erosion were very common on the surface of polyps (96.83%) and the surrounding mucosa (85.71%) . 4) The common pathological features of polyps were hyperplastic polyps (49.25%) and tubular adenomatous polyps (44.78%). There is 5.97% cancer reported. Conclusions: middle-aged and elderly people are the high-risk group; various GI symptoms are observed in Chinese patients; the typical endoscopic finding is multiple small sessile polyps; these GI polyps has a chance of malignant potential. Long-term endoscopic surveillance and follow-up are recommended for the Chinese CCS patients. Key words: Cronkhite-Canada syndrome, Clinical characteristics, Endoscopy

2020 ◽  
Author(s):  
Yuanyuan Lu ◽  
Fengxing Huang ◽  
Youwei Wang ◽  
Juan Zhou ◽  
Qiu Zhao ◽  
...  

Abstract Background: Cronkhite-Canada syndrome (CCS) is a rare non-inherited disease characterized by extensive gastrointestinal polyposis and ectodermal dysplasia. Its etiology still remains indefinite to us. Currently, the diagnosis is mainly based on typical clinical manifestations and endoscopic features. However, there is a lack of these information from Chinese CCS patients. Our study was to investigate the clinical and endoscopic characteristics of Chinese CCS patients. Methods: This retrospective study was conducted in 103 Chinese CCS patients (102 cases from literature and 1 case from our department). Their clinical and endoscopic data were collected, and statistical analyses were performed. Results: 1) In Chinese population, people aged 50-70 years (62.62%) had a high incidence of CCS, and the ratio of male-to-female was 2.68:1. 2) The diverse range of gastrointestinal (GI) manifestations were observed in all the patients, and the most common symptoms were weight loss (98.80%) and loss of appetite (93.94%). Almost all the patients had at least one symptom of ectodermal dysplasias. 3) All CCS patients presented multiple polyps in the GI tract except esophagus. The size of polyps varied from 0.5 cm (88.64%) to 0.6-1.0 cm (81.82%) . The polyps were various in shapes, the most of them were nodular polyps (58.14%) ,what’s more,the sessile polyps were the most common(90.24%). Congestion, edema and erosion were very common on the surface of polyps (96.83%) and the surrounding mucosa (85.71%) . 4) The common pathological features of polyps were hyperplastic polyps (49.25%) and tubular adenomatous polyps (44.78%). There is only 5.97% cancer reported. Conclusions: middle-aged and elderly people are the high-risk group; various gastrointestinal symptoms are observed in Chinese patients; the typical endoscopic finding is multiple small sessile polyps; these gastrointestinal polyps has a chance of malignant potential. Long-term endoscopic surveillance and follow-up are recommended for the CCS patients in China.


Medicina ◽  
2020 ◽  
Vol 56 (4) ◽  
pp. 185 ◽  
Author(s):  
Cornel Savu ◽  
Alexandru Melinte ◽  
Radu Posea ◽  
Niculae Galie ◽  
Irina Balescu ◽  
...  

Introduction: The purpose of this paper is to study the type, the clinical presentation, and the best diagnostic methods for pleural solitary fibrous tumors (PSFTs), as well as to evaluate which is the most appropriate treatment, especially as PSFTs represent a rare occurrence in the thoracic pathology. Material and Method: A retrospective study was conducted on a group of 45 patients submitted to surgery between January 2015 and December 2019. In most cases, the diagnosis was established through imaging studies—thoracic computed tomography (CT) scan with or without contrast—but also using magnetic resonance imaging (MRI) or positron emission tomography (PET) scans when data from CT scans were scarce. All patients were submitted to surgery with curative intent. Results: Most patients included in this study were asymptomatic, with this pathology being more common in patients over 60 years of age, and more common in women. The occurrence of malignant PSFT in our study was 17.77% (8 cases). All cases were submitted to surgery with curative intent, with a single case developing further recurrence. In order to achieve complete resection en bloc resection of the tumor with the chest wall, resection was performed in two cases, while lower lobectomy, pneumectomy, and hemidiaphragm resection, respectively, were needed in each case. Postoperative mortality was null. Conclusion: Thoracic CT scan remains the most important imagistic investigation in diagnosing. MRI is superior to thoracic CT, especially in cases that involved the larger blood vessels within the thorax, spinal column, or diaphragm. Complete surgical resection is the gold standard in treatment of PSFT, and the prognosis in benign cases is very good.


2020 ◽  
Vol 35 (8) ◽  
pp. 1811-1819
Author(s):  
Dong Li ◽  
Bin Chen ◽  
Hao Zhang ◽  
Yuan Yuan ◽  
Wenxin Fan ◽  
...  

Author(s):  
Imen Ksiaa ◽  
Safa Ben Aoun ◽  
Sourour Zina ◽  
Dhouha Nefzi ◽  
Sana Khochtali ◽  
...  

Abstract Objective To describe a case of Behçet disease (BD) uveitis manifesting with sequential bilateral neuroretinitis associated with prepapillary inflammatory vitreous exudate (PIVE). Material and methods A single case report documented with multimodal imaging. Results A 37-year-old man developed neuroretinitis with associated PIVE in the left eye. He was diagnosed with ocular toxoplasmosis and treated accordingly based on positive serologic testing and negative work-up for other entities, including BD. The disease course was favorable, but 1 year later a similar neuroretinitis developed in the right eye. Extraocular features of BD became evident only at the time of the second eye involvement, and the patient received corticosteroid and immunosuppressive therapy. Swept source (SS) OCT showed at the acute phase in both eyes a typical “mushroom-shaped” prepapillary hyperreflectivity of the PIVE. SS OCT angiography (OCTA) demonstrated a corresponding prepapillary hypointense area due to shadowing effect, decreasing in size while scanning deeper layers. It also detected peripapillary retinal hypervascularity in both eyes and a sectoral area of flow signal loss in the first involved left eye. Visual acuity improved following the resolution of the PIVE and associated acute inflammatory changes in both eyes. The left eye showed residual optic disc pallor and retinal nerve fiber layer defects. Conclusion Sequential bilateral neuroretinitis associated with PIVE may occur before other clinical features of BD become evident. SS OCT and OCTA can provide useful information for the diagnosis and management of this rare, but typical, ocular manifestation of BD uveitis.


Cortex ◽  
1988 ◽  
Vol 24 (2) ◽  
pp. 347-355 ◽  
Author(s):  
A. Charnallet ◽  
S. Carbonnel ◽  
J. Pellat

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