APICAL BLEBS LEADING TO PNEUMOTHORAX IN MARFAN SYNDROME A CASE REPORT
Yuli Prihastuti,Dwikisworo Setyowireni
Department of Child Health Faculty of Medicine Public Health and Nursing Universitas Gadjah Mada Yogyakarta Indonesia Department of Child, Health Faculty of Medicine, Public Health, and Nursing, Universitas Gadjah Mada, Yogyakarta, Indonesia
Background : Marfan syndrome (MFS) is a common inherited connective tissue disorder with several respiratory manifestations. We present a boy with right-sided pneumothorax diagnosed as MFS. This report emphasizes the apical blebs following to pneumothorax in a MFS.
Case Presentation Summary : We reported a 14-year-old boy was suddenly suffering from tightness in his chest and shortness of breath after sprinting accompanied by tachypnea and tachycardia. We found decreased breath sound in the right chest and right pneumothorax was confirmed. A water sealed drainage tube was inserted but no improvement. A thoracic high resolution computed tomography (HRCT) was showing multiple apical blebs in the right lung (Fig.1). We conducted bullectomy (Fig.2) resulting in complete re-expansion of right lung. The manifestation of MFS such as aortic root dilatation Z ? 2, mitral valve prolapse, arm span/height ratio was > 1.05 (1.06), a positive thumb sign and positive wrist sign. Clinical diagnosis of MFS was based on revised Ghent nosology for the MFS, with score was 7.
Learning Points/Discussion : Pneumothorax is very rare in MFS. Definitive surgical treatment should be performed at the first occurrence of pneumothorax because of the high rate of recurrence after treatment with chest tube. Evaluation based on revised Ghent criteria should be done annually.
Keywords: blebs pneumothorax Marfan syndrome
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