title: Descending necrotizing mediastinitis complicating hyperimmunoglobulin E syndrome.
abstract: Descending necrotizing mediastinitis (DNM) is a potentially lethal disease that originates from a deep neck infection (DNI); it is often associated with an immunocompromised state. Hyperimmunoglobulin E syndrome (HIES) is an extremely rare complex immune deficiency characterized by recurrent abscesses of staphylococcal etiology. A rare case of a 34-year-old woman wherein HIES putatively promoted the development of DNI into DNM is described. She presented with cervical purulent lymphadenitis and retropharyngeal abscess. Despite immediate cervical drainage and use of broad-spectrum antibiotics, she developed DNI and subsequently DNM. Mediastinal drainage with thoracotomy and subsequent deep neck drainage were performed. Bacterial culture of the abscess isolated methicillin-resistant Staphylococcus aureus (MRSA). Although a postoperative recurrent deep neck abscess required a second surgery, we succeeded in conservative remission of recurrent mediastinal abscess with long-term use of anti-MRSA drugs. Sufficient drainage under thoracotomy and robust administration of postoperative antibiotics resulted in successful management of HIES-associated DNM.
authors: Department of Thoracic Surgery Fujieda Municipal General Hospital Fujieda Japan.
authors: Yusuke
authors: Y
authors: Takanashi
authors: Department of Thoracic Surgery Fujieda Municipal General Hospital Fujieda Japan.
authors: Takamitsu
authors: T
authors: Hayakawa
authors: Department of Thoracic Surgery Fujieda Municipal General Hospital Fujieda Japan.
authors: Hiroshi
authors: H
authors: Neyatani
authors: Department of Surgery 1 Hamamatsu University School of Medicine Hamamatsu Japan.
authors: Kazuhito
authors: K
authors: Funai
keywords: Descending necrotizing mediastinitis; hyperimmunoglobulin E syndrome; mediastinal drainage; thoracotomy