Descending necrotizing mediastinitis complicating hyperimmunoglobulin E syndrome

Area of Science:

• Infectious Diseases
• Immunology
• Thoracic Surgery

Background:

• Descending necrotizing mediastinitis (DNM) is a severe complication of deep neck infections (DNI), often seen in immunocompromised individuals.
• Hyperimmunoglobulin E syndrome (HIES) is a rare primary immunodeficiency characterized by recurrent staphylococcal infections and abscesses.

Observation:

• A 34-year-old woman with HIES presented with cervical lymphadenitis and a retropharyngeal abscess, indicative of DNI.
• Despite initial treatment, the patient progressed to DNM, necessitating urgent mediastinal and neck drainage via thoracotomy.
• Methicillin-resistant Staphylococcus aureus (MRSA) was identified as the causative pathogen.

Findings:

• The patient required a second surgery for a recurrent deep neck abscess.
• Conservative management with long-term anti-MRSA antibiotics led to remission of the mediastinal abscess.
• Successful treatment involved aggressive surgical drainage and comprehensive antibiotic therapy.

Implications:

• This case underscores the potential link between HIES and severe DNI/DNM progression.
• Effective management hinges on prompt surgical intervention, including thoracotomy for mediastinal drainage, and sustained antibiotic treatment.
• Highlights the importance of considering rare immune deficiencies in complex infectious cases.