Primary spontaneous pneumothorax in children and adolescents: a systematic review

Primary spontaneous pneumothorax (PSP) is common in young population. But despite its incidence, specific guidelines for pediatric patients are not available and an agreement on the optimal management of this pathology has not been reached yet. Current protocols for treatment of PSP are not up to date at genetic and pathologic advances, remaining those of 20th century. If the presence of blebs and bullae in the apex of the lung is still considered the principal determinant for PSP, the process underlying their formation is not completely clarified. Research on connective tissue pathology and on elastogenesis pathway allowed physicians to identify further predisposing factors for the onset of PSP, for that reason the belief that this disease occurs in “otherwise normal lungs” might be reviewed. For pediatric patients, a more accurate risk stratification of PSP and a specific tailored strategy are required. When inherited or acquired predisposing factors are present, children and adolescents have a higher likelihood to develop PSP than adults, and an early surgical treatment can be proposed. When surgery is needed, mini-invasive approaches are the option of choice. After the advent of videothoracoscopy, surgical techniques have been developed up to a single incision thoracoscopic surgery which allows better outcomes and can be more accepted by younger patients. The aim of this review is to summarize the last thirty years of literature on PSP in young patients, to focus the evidences which can improve the management of this disease.