Villalpando-Canchola R, Reyes-Garcia G, Morales-Juvera E, Hernandez-Gavilan KH, Laguna-Baca B

Language: Spanish
References: 5
Page: 34-37
PDF size: 268.78 Kb.

ABSTRACT

Introduction: Marfan syndrome is an autosomal dominant disorder with an incidence of 1 in 10 000 births. This syndrome is based on clinical findings, pathogenesis is related to abnormal biosynthesis of fibrin. There are no research studies that offer a therapeutic strategy correct management of spontaneous pneumothorax in patients with Marfan syndrome.
Case report: Male, 14 years old. Were diagnosed with Marfan syndrome. Go to the Thoracic Surgery Service where toracospia undergoes bull by resection and closure of bronchial fistula left with satisfactory postoperative outcome, graduates at 7 days. Readmitted with right pneumothorax which affects 70% of the hemithorax, is managed initially with chest tube placement is performed the next day thoracoscopy with resection of the right pulmonary bulla, torpid with the presence of hemothorax, responds to hemodynamic management without requiring surgical intervention is graduated with no complications.
Discussion: There are few cases of spontaneous pneumothorax in patients with Marfan syndrome have been reported in medical literature and optimal treatment is not yet known. Some suggest early surgical resection of the Bull and others are more conservative under the morbidity of open surgery. It has been observed in patients with Marfan syndrome presenting spontaneous neumatórax have a high risk of recurrence that makes recommended surgical resection of the Bulls early. Today thoracoscopic surgery is recommended resection of the bullae and pleurodesis because it is considered a safe and effective procedure for treatment of spontaneous pneumothorax in children without the morbidity of open surgery and should therefore be the treatment of choice.

REFERENCES

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2. 2.Kouerinis IA, Zografos GC: Spontaneous rupture of the colon sigmoid following spontaneous recurrent pneumothorax in a patient with marfan syndrome. medscimonit,2006; 12 (3):CS21-23.

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5. 5.Grewal H, Jackson J. Video-Assisted Thoracic Surgery Is Safe and Effective in the Treatment of Spontaneous Pneumothorax in –Children. Pediactric Endosurgery & Innovative Techniques, 2001;5,4:365-369.