VATS bullectomy and apical pleurectomy for spontaneous pneumothorax in a young patient with Swyer-James-Mc Leod syndrome: case report presentation and literature review focusing on surgically treated cases

Nikolaos Panagopoulos,Gerasimos Papavasileiou,Efstratios Koletsis,Myrto Kastanaki,Nikolaos Anastasiou

doi:10.1186/1749-8090-9-13

Nikolaos Panagopoulos, Gerasimos Papavasileiou + Show 3 more

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https://doi.org/10.1186/1749-8090-9-13

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Abstract

BackgroundSwyer-James-McLeod Syndrome (SJMS) is an uncommon, emphysematous disease characterized by radiologic hyperlucency of pulmonary parenchyma due to loss of the pulmonary vascular structure and to alveolar overdistension.Case reportWe herein describe a 15-year-old Caucasian patient with well-established SJMS since childhood who presented with spontaneous pneumothorax. Video-assisted thoracoscopic bullectomy with apical pleurectomy was performed. Since SJMS is considered an on-going inflammatory process, the patient one year after surgery exhibits excellent quality of life with no pneumothorax recurrence.

Highlights

Swyer-James-McLeod Syndrome (SJMS) is an uncommon, emphysematous disease characterized by radiologic hyperlucency of pulmonary parenchyma due to loss of the pulmonary vascular structure and to alveolar overdistension.Case report: We describe a 15-year-old Caucasian patient with well-established SJMS since childhood who presented with spontaneous pneumothorax
Patients suffering from SJMS may be asymptomatic for many years or suffer from recurrent episodes of pulmonary infections. They usually present with chronic productive cough and dyspnea upon exercise. Do they present with symptoms of spontaneous pneumothorax due to rupture of an emphysematous bulla secondary to the inflammatory process
We present a case of a 15-year old patient with SJMS presented with spontaneous pneumothorax due to emphysematous bulla rupture

Summary

Background

Unilateral pulmonary emphysema or unilateral hyperlucent lung with secondary deficiency of blood supply was first described by Swyer and James [1] in a six year old child. Patients suffering from SJMS may be asymptomatic for many years or suffer from recurrent episodes of pulmonary infections They usually present with chronic productive cough and dyspnea upon exercise. After his exit from the hospital and up to the age of ten years he had repeated episodes of pulmonary infections with bronchitis and dyspnea It was that time were the diagnosis SJMS was made due to bronchiolitis obliterans and resulting emphysema. During the few days the patient continued to exhibit significant amount of air in the underwater seal mainly during coughing and the chest X-rays showed that the pneumothorax still existed; significantly decreased in size. Patient’s underwater seal drain was changed and connected to negative suction in order to remove the remaining air and expand the lung He remained on negative suction for the following seven days with no radiological or clinical improvement; surgical therapy was decided.

Discussion

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