Acute chest syndrome in the postoperative sickle cell patient

Background/Purpose: Acute chest syndrome (ACS), a phenomenon of pulmonary sequestration in sickle cell disease (SCD) patients, is frequently missed in the postoperative SCD child. The constellation of symptoms range from fever and respiratory distress to abdominal discomfort. In its most fulminate state, the syndrome has been reported in some series to carry almost a 25% to 50% mortality rate in the postoperative patient. The incidence in pediatric patients in the era of minimally invasive surgery is unknown. Methods: Since December 1995, 63 episodes of ACS have been documented in the nearly 500 SCD children seen at our institution. Six of 63 episodes occurred within 2 weeks after a surgical procedure under general anesthesia. During this period, 59 operations were performed by the pediatric surgery service on SCD patients with an ACS incidence of 10.2%. Careful review of the preoperative, intraoperative, and postoperative management of these patients was performed. Results: All six received preoperative oxygen saturation monitoring and intravenous fluid (IVF) hydration. One half of these patients required transfusion to achieve a hemoglobin level of greater than 10 mg/dL. Documentation of intraoperatire temperature, hypoxia, volume status, and hypercarbia as well as any atypical perioperative events were monitored and reviewed. All patients received postoperative oxygen supplementation and IVF hydration. Onset 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 for development of postoperative SCD complications, ACS occurred with an incidence much higher than previously reported in the literature (0.4% v 10.2%). Interestingly, five of six cases were after laparoscopic procedures suggesting that the advantages of laparoscopy, such as reduced postoperative pain, do not extrapolate to decreased incidence of ACS.