Acute chest syndrome in the postoperative sickle cell patient

Background/Purpose: Acute chest syndrome (ACS), a phenomenon of pulmonary s equestration in sickle cell disease (SCD) patients, is frequently missed in the postoperative SCD child. The constellation of symptoms range from feve r and respiratory distress to abdominal discomfort. In its most fulminate s tate, the syndrome has been reported in some series to carry almost a 25% t o 50% mortality rate in the postoperative patient. The incidence in pediatr ic patients in the era of minimally invasive surgery is unknown. Methods: Since December 1995, 63 episodes of ACS have been documented in th e nearly 500 SCD children seen at our institution. Six of 63 episodes occur red within 2 weeks after a surgical procedure under general anesthesia. Dur ing this period, 59 operations were performed by the pediatric surgery serv ice an SCD patients with an ACS incidence of 10.2%. Careful review of the p reoperative, intraoperative, and post operative management of these patient s was performed. Results:All six received preoperative oxygen saturation monitoring and intr avenous fluid (IVF) hydration. One half of these patients required transfus ion to achieve a hemoglobin level of greater than 10 mg/dL. Documentation o f intraoperative temperature, hypoxia, volume status, and hypercarbia as we ll as any atypical perioperative events were monitored and reviewed. All pa tients received postoperative oxygen supplementation and IVF hydration. Ons et of ACS ranged from 1 hour to 7 days postoperatively. Only one of six was thought to be of microbial etiology (elevated mycoplasma titers), and all patients received prophylactic antibiotic and aggressive pulmonary therapy. Overall length of hospitalization was increased with an average stay of 6. 1 days. There were no postsurgical ACS deaths. Conclusions: Despite close attention and avoidance of known risk factors fo r development of postoperative SCD complications, ACS occurred with an inci dence much higher than previously reported in the literature (0.4% v 10.2%) . Interestingly, five of six cases were after laparoscopic procedures sugge sting that the advantages of laparoscopy, such as reduced postoperative pai n, do not extrapolate to decreased incidence of ACS. J Pediatr Surg 34:188- 192. Copyright (C) 1999 by W.B. Saunders Company.