Abstract
Congenital gastric ectopic pylorus (CGEP), also known as gastric ectopic pyloric opening, is a rare congenital gastric abnormality. It was first reported and termed by Yu and Zhao from China in 1983, and in 2007, Uraz et al published the first report of CGEP in the English literature. We conducted a systemic review of the literature of CGEP published in English or Chinese, and found that CGEP occurred more frequently in the 6(th) and 7(th) decade of life, with a male predominance (89.7%). The majority of reported cases (89.2%) were from East Asia, and the underlying mechanism remains unknown. Most patients with CGEP presented non-specific symptoms, including abdominal pain, bloating, regurgitation, and belching. A few patients (30%) experienced upper gastrointestinal bleeding. Given the non-specific symptoms, misdiagnosis or underdiagnosis often occurs. Gastroscopy and upper gastrointestinal radiography are the main examinations used to confirm the diagnosis. Currently, conservative medication is the mainstay treatment. In this systematic review, we describe the history, etiology, diagnosis and treatment of CGEP in detail, aiming to provide a comprehensive understanding of this rare disease and avoid misdiagnosis.