101. Pancreatic cystosis in patients with cystic fibrosis: A qualitative systematic review
- Author
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K. Khan, Jennifer S. Vonderau, Todd H. Baron, Rebecca McCall, and Chirag S. Desai
- Subjects
medicine.medical_specialty ,Cystic Fibrosis ,Endocrinology, Diabetes and Metabolism ,medicine.medical_treatment ,MEDLINE ,Cystic fibrosis ,030218 nuclear medicine & medical imaging ,03 medical and health sciences ,0302 clinical medicine ,Cystogastrostomy ,Humans ,Medicine ,Genetic Predisposition to Disease ,In patient ,Cyst ,Contraindication ,Hepatology ,business.industry ,Gastroenterology ,medicine.disease ,medicine.anatomical_structure ,030220 oncology & carcinogenesis ,Pancreatectomy ,Radiology ,Pancreatic Cyst ,business ,Pancreas - Abstract
Background 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. Methods 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. Results 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. Conclusion 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.
- Published
- 2018
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