Abstract
INTRODUCTION: Chronic intestinal pseudo-obstruction is a clinical entity with multiple causes. One of them is intestinal neuronal dysplasia, a rare disease with two subtypes: A and B. OBJECTIVE: To present a clinical case of a patient diagnosed with idiopathic gastroparesis associated with type A intestinal neuronal dysplasia. CLINICAL CASE: A 30-year-old female patient with a diagnosis of idiopathic gastroparesis of a long evolution, with multiple hospitalizations and significant weight loss. She developed a aorto-mesenteric compass syndrome that was treated surgically with partial and temporary improvement. A jejunostomy was performed for feeding when symptoms recurred. During this surgical procedure, gastric and jejunal biopsies were performed and a diagnosis of intestinal neuronal dysplasia type A was made. CONCLUSION: In the presence of symptoms of intestinal pseudo-obstruction or a diagnosis of idiopathic gastroparesis, it is necessary to consider the involvement of the gastrointestinal plexus. Obtaining an adequate tissue sample and evaluating it with appropriate techniques is of paramount importance to diagnosing the neurogenic etiology of the entity.