Abstract:
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Since 1982, the British Columbia's Children's Hospital Home Ventilation Program has cared for 326 patients with only four cases of pneumothorax, three occurring spontaneously off ventilation, and one occurring while the patient was on daytime noninvasive ventilation (NIPPV) during an acute viral illness. Patient #1 is a 20 year old young man with a mitochondrial myopathy, receiving nighttime NIPPV, who underwent pleurectomy at 17 years of age after recurrence of a right sided pneumothorax. Patient #2 is a 10 year old girl with Nemaline Myopathy, dependent on nighttime NIPPV, who at 9 years of age, was treated conservatively with only chest drainage with a persistent right pneumothorax, and was only observed, after a left pneumothorax which occurred 3 weeks later. She did not have “blebs” on computerized tomography (CT) of the chest. Patient #3 is an 18 year old young man with Duchenne Muscular Dystrophy, dependent on nighttime NIPPV, who was first treated conservatively, without drainage, after a spontaneous left pneumothorax. The pneumothorax recurred on five occasions. After the 5th recurrence, chest drainage under Radiological guidance failed to completely decompress the pneumothorax as the pleural space had become septated, and the pneumothorax was loculated. He underwent pleurectomy complicated by profuse hemorrhage and hemothorax, intraoperative hypotension, and postoperative arrythmias. Twomonths later he presented with a left pneumothorax, and after chest drainage, underwent an uneventful left pleurectomy. Standard guidelines for the treatment of spontaneous pneumothorax suggest treating the initial pneumothorax with underwater seal chest drainage, and if the pneumothorax does not resolve with drainage, or if the patient has a recurrence on the same side, or a new pneumothorax on the ipsilateral side, to consider pleurectomy. With chest CT, it has been appreciated that perhaps 50% of patients have multiple small cystic lesions (“blebs”), usually at the apices of the lung, but occasionally elsewhere, which may represent potential “air leaks”. Modified guidelines suggest pleurectomy if “blebs” are present on chest CT regardless of whether the patient has had only one pneumothorax, and bilateral pleurectomy if “blebs” are present on the unaffected side. More recently however, physicians and surgeons have become less aggressive in surgically treating spontaneous pneumothorax, and adherence to standard guidelines has relaxed. Patients with a non-tension pneumothorax, without distress may simply be observed rather than have a chest drain placed, and patients may be allowed a second pneumothorax without pleurectomy, provided that the recurrence has occurred on the same side as the original pneumothorax, and there are no blebs” apparent on chest CT scan. When a spontaneous pneumothorax occurs in a patient with who is dependent on NIPPV, the presence of the underlying disease process often leads to less aggressive interventions. Ideally patients with an undrained pneumothorax should not receive mechanical ventilation. Our first patientwas able to forgoNIPPV for several nightswhile his initial pneumothorax resolved. The second and third patients were nighttime NIPPV dependent and unable to go without nighttime assisted ventilation, however theywere able to bemaintained comfortably with reduced IPAP and EPAP. We are presenting these three patients to stimulate discussion. We violated “the guidelines” twice, once with success, once with a near disastrous result. How would you approach this problem?
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