Diffuse Esophageal Leiomyomatosis and the Surgical Challenges Encountered

Abstract

Diffuse esophageal leiomyomatosis (DEL) is a rare condition characterized by the growth of multiple benign smooth muscle tumors (leiomyomas) in the esophagus. These tumors can cause obstruction and functional impairment of the esophagus, leading to symptoms such as difficulty swallowing, chest pain, and weight loss. The disease can present with leiomyomas or glomerular nephropathy in other body parts, hearing defects, astigmatism, or myopia (Alport’s syndrome). However, isolated cases are also reported in the literature. Surgical management of DEL presents several challenges due to the location and extent of the tumors. Herein, we describe a girl afflicted with DEL and required multiple surgeries for management, highlighting the unique challenges faced by the surgical team in restoring esophageal function and improving the patient’s quality of life.

Keywords

• colonic interposition
• esophageal leiomyomatosis
• esophageal replacement
• gastric pull-up