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A 45-year-old man presented with sharp, left-sided chest pain of one-hour duration that radiated to the right chest and back, and started two hours after snorting cocaine. He was an active smoker and his medical history included pulmonary embolism on anticoagulation, heart failure with reduced ejection fraction of 9% with an implantable cardioverter-defibrillator, and cocaine use disorder. Vital signs were normal, except for a respiratory rate of 20 breaths per minute. Laboratory examinations were unremarkable, and an electrocardiogram was unchanged when compared to previous ones. A chest x-ray showed the absence of pulmonary vascular markings in the right lung apex (Fig. 1A). A chest computerized tomography angiogram was obtained to evaluate for aortic dissection. Aortic dissection was ruled out, but the computerized tomography showed the right lung findings in Fig. 1B. What is the diagnosis for Fig. 1B?
Fig. 1A) Chest x-ray on admission showing absence of pulmonary vascular markings in the apex of the right lung. B) Right giant bullae on presentation, occupying more than one-third of the right hemithorax. C) Right apical bullae 12 months before presentation. D) Right apical bullae 4 months before presentation.
The right lung findings were consistent with vanishing lung syndrome, which is defined as the presence of giant bullae in one or both lungs, occupying at least one third of the hemithorax and compressing surrounding normal lung parenchyma [
]. The condition is progressive, with continuous enlargement of the bullae that results in further compression of the adjacent lung parenchyma and worsening dyspnea. Its management includes optimizing of the medical conditions, and surgical interventions such as bullectomy or endobronchial valve insertion. Indications for bullectomy include severe dyspnea, pneumothorax, pain, infection, and/or hemoptysis [
]. Pulmonary function testing is required for pre-operative evaluation. Although bullectomy is the treatment of choice, indications for surgery should be assessed individually. Severe comorbidities such as pulmonary hypertension or end-stage heart failure may preclude any surgical interventions.
This patient was admitted to the hospital for evaluation for acute coronary syndrome, which was negative, and his chest pain resolved spontaneously. A review of the patient's previous imaging findings revealed progressive expansion of the right apical bullae over one year (Figs. 1C and 1D). The patient did not undergo surgical evaluation for bullectomy due to his end-stage heart failure and decision to continue smoking.
Notation of prior abstract publication/presentation
The contents of this manuscript have not been presented in any meeting.
Ethics committee approval
Not applicable. The patient provided signed informed consent for the publication of this manuscript.
Funding information
No funding was provided for the preparation of this manuscript.
Declaration of Competing Interest
The authors declare they have no conflict of interest.
Acknowledgements
None. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
References
Stern E.J.
Webb W.R.
Weinacker A.
Muller N.L
Idiopathic Giant Bullous Emphysema (Vanishing Lung Syndrome): imaging Findings in Nine Patients.
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