Department of Surgery, University of North Carolina, USA.
Department of Surgery, University of North Carolina, USA.
Pancreatology. 2018 Oct;18(7):700-704. doi: 10.1016/j.pan.2018.08.008. Epub 2018 Aug 17.
Patients with cystic fibrosis (CF) and a CFTR gene mutation may present with a variety of pancreatic disorders. The presence of multiple macrocysts (>1 cm) replacing the entire pancreatic parenchyma is termed pancreatic cystosis. Lack of clear data makes clinical decision making challenging and controversial. The aim of this review is to perform a qualitative systematic analysis of the literature with intention to evaluate management plans.
Electronic databases MEDLINE, Embase, and Scopus were searched for relevant studies, and 19 studies describing patients with pancreatic cystosis were included and analyzed for clinical features and therapy offered.
The data of 24 patients were collected from included studies. Eight cases (33%) had a documented CFTR gene mutation and 10 (42%) were symptomatic at presentation. Imaging modalities included ultrasound in 18 (75%), CT in 12 (50%), and MRI in 8 (33%) cases. An average size of the largest cyst was 5.4 cm. 6 (25%) patients were offered therapy that described surgical (3), endoscopic (1), or medical therapy (2). Surgeries offered included total pancreatectomy, partial pancreatic resection of uncertain extent, and complex cyst resection. Endoscopic treatment was cystogastrostomy. Novel medical treatment was utilized with Doxepin, Propantheline, and Clonidine, resulting in reduction in cyst size and overall clinical improvement.
Patients with pancreatic cystosis should not be denied treatment when necessary. This literature review is the most comprehensive thus far of cystic fibrosis and pancreatic cystosis, and it did not provide identification of a definitive treatment plan or demonstrate contraindication to specific therapies.
囊性纤维化（CF）患者和 CFTR 基因突变可能表现出多种胰腺疾病。多个大囊肿（>1cm）替代整个胰腺实质的存在被称为胰腺囊性纤维化。缺乏明确的数据使得临床决策具有挑战性和争议性。本综述的目的是对文献进行定性系统分析，旨在评估治疗方案。
检索 MEDLINE、Embase 和 Scopus 电子数据库，查找相关研究，并纳入和分析了 19 项描述胰腺囊性纤维化患者的临床特征和治疗方法的研究。
从纳入的研究中收集了 24 名患者的数据。8 例（33%）有 CFTR 基因突变的记录，10 例（42%）在就诊时出现症状。影像学检查包括超声 18 例（75%）、CT 12 例（50%）和 MRI 8 例（33%）。最大囊肿的平均大小为 5.4cm。6 例（25%）患者接受了治疗，包括手术（3 例）、内镜（1 例）或药物治疗（2 例）。手术包括全胰切除术、不确定范围的胰腺部分切除术和复杂囊肿切除术。内镜治疗为囊肿胃吻合术。使用 doxepin、propantheline 和 clonidine 进行了新的药物治疗，导致囊肿大小缩小和整体临床改善。
当需要治疗时，不应拒绝胰腺囊性纤维化患者的治疗。这是迄今为止对囊性纤维化和胰腺囊性纤维化最全面的文献综述，它没有确定明确的治疗方案，也没有证明特定治疗方法有禁忌症。
