Surgery in patients with cystic fibrosis

A 15-year review of children's hospital patients with cystic fibrosis (CF) who underwent surgery yielded 578 cases in 210 patients (mean 2.7 per patient). The median age was 16 years (range newborn to 43 years). Four hundred procedures were done under general anesthesia and 176 under local. There was one anesthetic complication, respiratory depression in a patient whose MediPort (Cormed, Inc, Medina, NY) was inserted using local anesthesia and sedation. The most frequent procedure was nasal polypectomy, with 165 procedures in 50 patients. The second most common procedures were vascular access procedures: 75 central lines and 29 MediPorts were implanted in 57 patients, complicated by two pneumothoraces. Thoracic procedures included 32 bronchoscopies, 8 lobectomies, 2 pneumonectomies, and 30 pleural strippings. There were three reoperations for bleeding in the pulmonary resection patients. Thirteen newborns underwent a total of 26 procedures for meconium ileus and its complications, with two deaths secondary to respiratory failure and sepsis. These, and one death postlobectomy were the only operative deaths in the entire series of 578 cases (0.5% mortality rate). There were four slings for rectal prolapse; two required removal secondary to infection. Eight patients underwent central splenorenal shunts for portal hypertension, 15 underwent cholecystectomy, 5 underwent Nissen fundoplication, 16 underwent inguinal herniorrhaphy, 2 underwent umbilical herniorrhaphy, 3 underwent orchidopexies, and 4 underwent miscellaneous pediatric surgical procedures. Eleven patients underwent appendectomy for appendicitis; four were ruptured at the time of diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)