RESUMO
Wounds and perforations of the upper gastrointestinal tract are serious and life-threatening. The hypopharynx and cervical esophagus, by their respective anatomical positions, are exposed to traumatic wounds, most often during diagnostic tests, but management such wounds remains a subject of discussion. The present article analyzes the current state of knowledge on epidemiology, etiologies, risk factors, diagnostic management, prognostic factors and available treatments.
Assuntos
Perfuração Esofágica , Esôfago/lesões , Hipofaringe/lesões , Ferimentos Penetrantes , Queimaduras Químicas/etiologia , Queimaduras Químicas/terapia , Perfuração Esofágica/diagnóstico , Perfuração Esofágica/etiologia , Perfuração Esofágica/terapia , Esôfago/diagnóstico por imagem , Corpos Estranhos/complicações , Corpos Estranhos/terapia , Humanos , Hipofaringe/diagnóstico por imagem , Doença Iatrogênica , Prognóstico , Ferimentos Penetrantes/diagnóstico , Ferimentos Penetrantes/etiologia , Ferimentos Penetrantes/terapiaRESUMO
INTRODUCTION: Epidermolysis bullosa (EB) is a congenital disease characterized by fragility of epithelial structures. The skin is the organ primarily affected, resulting in the formation of skin blisters. Some forms of EB may also present mucosal lesions. CASE REPORT: We report the case of a girl with epidermolysis bullosa simplex (EBS) associated with muscular dystrophy secondary to congenital plectin deficiency. She presented severe respiratory tract lesions extending from the oral cavity to the larynx. In particular, we describe our medical and surgical management of the laryngeal lesions, responsible for several episodes of respiratory distress and feeding difficulties. DISCUSSION: Epidermolysis bullosa simplex associated with muscular dystrophy is a rare hereditary form of EB, as fewer than 50 cases have been reported in the literature. This form is characterized by mucosal lesions involving the upper aerodigestive tract, with consequences for feeding, phonation and breathing. Special care must be taken when performing diagnostic and therapeutic procedures to avoid worsening the lesions of this very fragile mucosa. Tracheotomy is a harmful procedure in these patients and should only be considered as a last resort.