Predicting outcome following pulmonary resection in cystic fibrosis patients.

A small subset of cystic fibrosis (CF) patients develop pulmonary disease primarily limited to one lobe or lung segment requiring prolonged recurrent hospitalizations with intensive medical therapy. Although surgery has been advocated for patients who do not respond to medical therapy, very little is known about criteria for selection of patients who might benefit from resection of the involved parenchyma. In an attempt to further define criteria for pulmonary resection in these patients, we retrospectively reviewed our experience at Tulane Medical Center over the past 10 years. Fourteen patients with CF, ranging from 3 to 30 years of age, underwent 17 pulmonary resections. Indications for surgery were persistent lobar or pulmonary atelectasis and bronchiectasis requiring multiple hospitalizations and unresponsive to medical therapy (n = 13), bronchopleural fistula (n = 2), or hemoptysis not responding to medical therapy or selective embolization (n = 2). Thirteen lobectomies and four pneumonectomies were performed. Only two resections were on the left side and 11 right upper lobectomies were performed. Postoperative hospitalization ranged from 5 to 21 days (mean, 8.5 days). Preoperative pulmonary function tests showed widely divergent function in these patients. Forced expiratory volume (FEV1) ranged from 11% to 88% whereas forced vital capacity (FVC) ranged from 20% to 100% of predicted values. Oxygen saturation ranged from 86% to 99%. Although there was no significant difference in preoperative and postoperative FVC or O2 saturation, there was a significant (P less than .003) decrease in the postoperative FEV1. In the 12 surviving patients followed for at least 1 year, there was also a significant reduction (P less than .001) in the number of hospitalizations required due to pulmonary exacerbations from an average 2.2 admissions per year (range, 0.44 to 3.5 admissions per year) to 1.1 admissions per year (range, 0 to 8).(ABSTRACT TRUNCATED AT 250 WORDS)

Surgical management of children and young adults with Marfan syndrome and pectus excavatum.

Significant chest wall deformities occur in two thirds of children with Marfan syndrome (MS). The symptoms, physical findings, and surgical outcome of 11 patients with MS and severe pectus excavatum who required operative repair were reviewed. The diagnosis of MS was made before the pectus repair in six patients, at the time of evaluation of pectus in two patients, and after the repair in three patients. Symptoms included dyspnea upon exertion, tachypnea, and chest pain. Physical findings included aortic root enlargement or valvular disease, mitral valve disease, ligamentous disease, congestive heart failure, and ocular disease. All patients had severe pectus deformities with a narrow anteroposterior diameter in the midline, as well as a broad chest defect that extended bilaterally to the midclavicular line. The heart was shifted into the left side of the chest in all patients. A Ravitch-type pectus repair with a stainless steel substernal strut was used in eight patients, with one patient suffering a late recurrence; in three patients no strut was used, and all three had recurrence. There were no postoperative complications. In the postoperative follow-up of seven patients, symptomatic improvement of cardiopulmonary performance was noted. Four of the patients required subsequent open heart surgery, including replacement of the aortic valve in one patient, and composite grafts of the ascending aorta in three patients. Postoperative cardiac arrest was the only major open heart complication. All four patients recovered and did well after surgery, showing significant cardiac and pulmonary functional improvement.(ABSTRACT TRUNCATED AT 250 WORDS)