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The Long-Term Clinical and Endoscopic Outcomes of Cronkhite-Canada Syndrome.

ABSTRACT: INTRODUCTION:The prognosis of Cronkhite-Canada syndrome (CCS) is considered poor. Despite the recent therapeutic improvements, the survival outcomes and prognostic factors have been less studied. This study aimed to investigate the long-term clinical and endoscopic outcomes of CCS. METHODS:Thirty-one patients diagnosed since 1999 and followed up for over 6 months were included. Data regarding survival outcomes, clinical symptoms, endoscopic findings, and treatment were collected and analyzed. R (version 3.6.1) was used to perform the survival analyses. RESULTS:The median (interquartile range) follow-up time was 42.5 (19.5-85.8) months. The 5-year overall survival (OS) was 87.4%. The maximum gastric polyp size over 2 cm was associated with worse OS (Hazard ratio [HR]: 18, 95% confidence interval [CI]: 1.6-210, P = 0.021). The 3-year relapse-free survival (RFS) after corticosteroid treatment was 66.8%. Age older than 60 years (HR: 7.0, 95% CI: 1.5-33.0, P = 0.015) and maximum gastric polyp size over 2 cm (HR: 6.0, 95% CI: 1.6-23.0, P = 0.009) were associated with worse RFS. Twenty-three patients received follow-up endoscopic examinations, with a median (interquartile range) follow-up time of 29.0 (14.0-53.5) months. Eight (34.8%) and 12 (52.2%) patients achieved complete remission under gastroscopy and colonoscopy, respectively. Colonic lesions showed a tendency of earlier responses compared with gastric lesions (25.0 [11.3-39.8] months vs 31.0 [21.0-39.8] months). DISCUSSION:Patients with CCS usually responded well to glucocorticoids with a fairly good 5-year survival rate. Large gastric polyp was associated with worse OS and RFS, whereas age older than 60 years was another predictor for worse RFS. Diffuse gastrointestinal lesions partly or completely resolved after treatment, and colonic lesions showed a better response than gastric lesions.

SUBMITTER: Liu S

PROVIDER: S-EPMC7263663 | biostudies-literature | 2020 Apr

REPOSITORIES: biostudies-literature

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The Long-Term Clinical and Endoscopic Outcomes of Cronkhite-Canada Syndrome.

Liu Shuang S You Yan Y Ruan Gechong G Zhou Liangrui L Chen Dan D Wu Dong D Yan Xuemin X Zhang Shengyu S Zhou Weixun W Li Ji J Qian Jiaming J

Clinical and translational gastroenterology 20200401 4

<h4>Introduction</h4>The prognosis of Cronkhite-Canada syndrome (CCS) is considered poor. Despite the recent therapeutic improvements, the survival outcomes and prognostic factors have been less studied. This study aimed to investigate the long-term clinical and endoscopic outcomes of CCS.<h4>Methods</h4>Thirty-one patients diagnosed since 1999 and followed up for over 6 months were included. Data regarding survival outcomes, clinical symptoms, endoscopic findings, and treatment were collected a ...[more]

PMID: 32352683

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Project description:To assess the clinical outcomes of patients who received direct percutaneous endoscopic jejunostomy (DPEJ) for enteral feeding.This is a 10-year cohort study in a single tertiary center. Main outcome measurements were technical success, and short- and long-term outcomes. DPEJ was attempted in 83 patients (51 men; 55 ± 2 years) for dysphagia (n = 35), gastroparesis with recurrent aspiration (n = 30), and levodopa drug infusion for severe Parkinson's disease (n = 18).DPEJ was successful in 75 (90 %) patients. All technical failures were related to the inability to find adequate trans-illumination, and were not influenced by BMI, age, gender, or indication. Peri-operative (30-day) adverse events occurred in 11 (13 %) patients, including wound infection (3), leakage around the stoma (4), minor bleeding requiring no intervention (2), and aspiration (1). There was one case (1.2 %) of gastric perforation after PEJ insertion for levodopa drug infusion trial. This 60-year-old woman required an emergency laparotomy with nil complications, and levodopa drug infusion recommenced successfully. One case of intestinal perforation (1.2 %) occurred after jejunostomy tube replacement at 6 months of insertion, which was successfully managed with surgery. There were no peri-operative deaths. Adequate delivery of enteral feeding or Duodopa drug was achieved in 66/73 (90 %) patients, with evidence of weight gain or improvement in Parkinson's disease. Seven (8 %) continued to have clinical regurgitation but not aspiration. After a median follow-up of 84 months, 27 (33 %) patients died of their underlying diseases. Seven (8 %) had marked improvement in their underlying disease and had PEJ removed after 5 months (range 1 - 8 months).Single center study.DPEJ is associated with a high technical success rate (90 %), a relatively low rate of peri-operative adverse events (13 %) and an improvement in long-term nutritional support in the majority of patients (90 %). DPEJ should be the procedure of choice to gain enteral access for feeding or drug delivery prior to considering surgery.

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The Long-Term Clinical and Endoscopic Outcomes of Cronkhite-Canada Syndrome.

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The Long-Term Clinical and Endoscopic Outcomes of Cronkhite-Canada Syndrome.

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