Resolving persistent air leaks associated with autosomal dominant hyper-IgE syndrome using one-way endobronchial valves: report of cases

John Kucera; Munir Buhaya; Nicole Nix Sartain; Kenneth N Olivier; Alexandra F Freeman; Chuong D Hoang

doi:10.21037/acr-23-35

Resolving persistent air leaks associated with autosomal dominant hyper-IgE syndrome using one-way endobronchial valves: report of cases

AME Case Rep. 2024 Mar 20:8:43. doi: 10.21037/acr-23-35. eCollection 2024.

Authors

John Kucera¹, Munir Buhaya², Nicole Nix Sartain³, Kenneth N Olivier⁴, Alexandra F Freeman², Chuong D Hoang⁵

Affiliations

¹ Department of Surgery, Walter Reed National Military Medical Center, Bethesda, MD, USA.
² Laboratory of Clinical Immunology & Microbiology, National Institute of Allergy & Infectious Diseases (NIAID), Bethesda, MD, USA.
³ Clinical Center, National Institute of Allergy & Infectious Diseases (NIAID) National Institutes of Health (NIH), Bethesda, MD, USA.
⁴ Pulmonary Branch, National Heart, Lung and Blood Institute, National Institutes of Health (NIH), Bethesda, MD, USA.
⁵ Thoracic Surgery Branch, National Cancer Institute, National Institutes of Health (NIH), Bethesda, MD, USA.

Abstract

Background: Autosomal dominant hyper-IgE syndrome (AD-HIES) is a rare genetic syndrome that alters typical post-operative wound healing. AD-HIES patients are prone to develop persistent air leaks (PALs) due to bronchopleural fistulas. This report is unique in that it describes a novel approach to managing PALs in this complex population.

Case description: Two patients with AD-HIES were identified in the setting of a PAL. The first patient was a 31-year-old male with recurrent pneumonia, who developed a large hydropneumothorax following re-presentation with fever and cough. A chest tube was inserted, which required continuous suction in the setting of what developed into a PAL. Subsequently, an endobronchial valve (EBV) was deployed to successfully manage the PAL. The second patient was a 25-year-old male, who developed a post-operative large volume air leak following a complicated surgical resection of a giant pneumatocele. Several attempts of placing multiple EBVs were required to finally address the PAL. In both cases, EBVs were successfully employed to manage and eventually resolve symptoms caused by PAL.

Conclusions: Our experience suggests that EBVs are successful in treating PAL in the setting of AD-HIES, which often manifests as highly complex scenarios. Hence, EBVs represent a valuable addition to the therapeutic armamentarium against recalcitrant PAL. EBVs were well-tolerated in patients afflicted by AD-HIES, with no progressive infections noted. Both patients ultimately were able to resolve their PAL following placement of the EBV.

Keywords: Endobronchial valves (EBVs); case report; persistent air leak (PAL).

Publication types

Case Reports