Swyer-James-MacLeod syndrome presenting as spontaneous pneumothorax in an adult: Case report and review of literature

Abstract

Swyer-James-MacLeod Syndrome (SJMS) is an uncommon, emphysematous disease characterized by obliteration of the small bronchioles, hypoplasia/or absence of pulmonary artery and peripheral vascular bed. It is most commonly diagnosed in childhood. Patients are often asymptomatic or they could suffer from symptoms of recurrent pulmonary infections. Spontaneous pneumothorax is a rare presentation of this syndrome. We report a case of a 42-year old female patient presented at our Emergency Department with complaints of dyspnea and pleuritic chest pain with a diagnosis of spontaneous pneumothorax in emphysematous disease. The diagnosis of SJMS is usually based on imaging and clinical findings rather than on the results of pathologic examination; indeed, asymptomatic adult patients with SJMS are often diagnosed after a chest radiograph obtained for another reason. High-resolution computed tomography (HRCT) seems to be the most appropriate technique for the diagnosis. SJMS can be associated with spontaneous pneumothorax which represent an emergency condition due to the underlying pathological disease. The surgical treatment of the affected lung should be considered when conservative approach is ineffective.

Swyer-James Syndrome in a Patient Admitted for Investigation of Pulmonary Thromboembolism

“…Although it classically involves an entire lung, the disorder can be lobar or segmental. (CONTI et al, 2021) Swyer-James-MacLeod syndrome is considered a post-infectious type of bronchiolitis obliterans. Pulmonary infection with adenovirus, respiratory syncytial virus, influenza A, measles, tuberculosis, mycoplasma pneumoniae and Bordetella pertussis has been reported as possibly responsible for the development of the syndrome.…”

Síndrome de Swyer James MacLeod. Reporte de caso de pulmón hiperlúcido

“…1 Se trata de una enfermedad enfisematosa infrecuente que se caracteriza por la obliteración de los bronquiolos pequeños, hipoplasia o ausencia de arteria pulmonar y lecho vascular periférico. 3 La anormalidad funcional de este síndrome es indudablemente obstrucción crónica espiratoria difusa con atrapamiento distal de aire. 4 La característica más distintiva en comparación con el enfisema es la ausencia de patología obstructiva crónica en la vía aérea pequeña, 3 que coincide con los datos de la FEF 25-75% 85.39 (88%) 72.89, obtenida de la paciente.…”

“…Se diagnostica con mayor frecuencia en la infancia, típicamente ocurre en niños menores de ocho años, antes que el pulmón haya completado el desarrollo y la maduración pulmonar. 5 Puede cursar de forma asintomática hasta la edad adulta, presentarse con neumotórax espontáneo generalmente asintomático, 2 o como condición poco frecuente de emergencia, 3 como bula calcificada 6 y con transfiguración placentaria del parénquima pulmonar. 7 La radiografía y tomografía de tórax de la paciente, mostraron pulmón segmentario hiperlúcido con alteración de la vascularidad, lo que normalmente describen otros autores como hiperclaridad pulmonar unilateral o lobar, asociada a atrapamiento aéreo del pulmón hiperclaro durante la espiración.…”