Abstract Introduction Hyper-IgE Syndrome (HIES) is a rare primary immunodeficiency that is characterized by abnormally high levels of serum IgE. These patients can develop pneumatoceles following lung infections, with Aspergillus fumigatus being a common cause.1 However, management of them is complex, often resulting in the need for chest tube and subsequent development of persistent air leak (PAL). Here we describe a patient with HIES for which endobronchial valves (EBVs) were used to treat PAL after removal of a pneumatocele secondary to A. fumigatus infection. Case Presentation A 15-year-old female with HIES, recurrent necrotizing pneumonia secondary to A. fumigatus, and resulting right lower lobe pneumatocele was referred to our hospital for possible bone marrow transplant. With concern that the pneumatocele may act as a nidus for infection post-transplant, she underwent right lower lobectomy. After lobectomy and chest tube placement, she developed a PAL, and on POD 7 underwent EBV placement in her right upper lobe and right middle lobe (5 valves) with resolution of PAL. Her chest tube was removed on POD 5 after EBV placement and chest x-ray showed stable right pneumothorax ex vacuo. She then developed tension physiology requiring new chest tube. On bronchoscopy, she was found to have small dehiscence of the bronchial stump of the superior segment of the right lower lobe. Due to the bronchial stump being too short to accommodate an EBV, this was addressed with an autologous blood patch, fibrin sealant, and another layer of autologous blood patch. This resulted in improvement of pneumothorax and chest tube was removed. Four days after blood patching, she redeveloped a tension pneumothorax requiring replacement of chest tube. Xenon MRI showed residual ventilation of right upper lobe suggesting new air leak. On repeat bronchoscopy, the EBV in the right upper lobe apical segment was found to have migrated. It was replaced, and the patient’s pneumothorax improved. She was discharged home with a Heimlich valve three days later. Her chest tube was removed two weeks after discharge without worsening of her pneumothorax. Discussion With PAL being one of the most common complications after lobectomy, occurring in 30% to 50% of patients 2, prolonged need for chest tubes can become a source of morbidity and prolonged hospitalization in pediatric patients. This case exemplifies how - in the hands of a skilled, multidisciplinary team - a medically complex patient who develops PAL in the post-op period can be managed effectively with EBVs. This abstract is funded by: None
Marburger et al. (Fri,) studied this question.