Spontaneous Perforation of the Esophagus as a Manifestation of Dermatomyositis

Abstract

Spontaneous perforation of the esophagus evolving into a retropharyngeal abscess in a child with severe dermatomyositis is reported. Clinical presentation and successful management are discussed. Aggressive surgical debridement with extensive drainage, multiple antibiotic coverage, and careful steroid management are advocated. The histopathology and clinical presentation of dermatomyositis as a type of polymyositis are discussed as pertinent to the otolaryngologic clinician. The literature is reviewed and results of a retrospective analysis of head and neck manifestations are reported in 50 children with dermatomyositis treated at Children's Hospital, Los Angeles, over the past 20 years.