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Acute chest syndrome in the postoperative sickle cell patient
S J Delatte1, A Hebra, E P Tagge
1Department of Surgery, Medical University of South Carolina, Charleston 29425, USA.
Insights
Acute chest syndrome (ACS) in pediatric sickle cell disease (SCD) patients is a serious postoperative risk, occurring more frequently after minimally invasive surgery than previously thought. Vigilant monitoring and management are crucial to prevent mortality in these vulnerable children.
Area of Science:
- Pediatric Surgery
- Hematology
- Pulmonary Medicine
Background:
- Acute Chest Syndrome (ACS) is a severe complication in pediatric sickle cell disease (SCD) patients.
- ACS is often overlooked in the postoperative setting and can have high mortality rates.
- The incidence of ACS in pediatric SCD patients undergoing minimally invasive surgery is not well-established.
Purpose of the Study:
- To determine the incidence of ACS in pediatric SCD patients after surgery.
- To identify risk factors and patterns of ACS in the postoperative period.
- To evaluate the effectiveness of current management strategies for postoperative ACS.
Main Methods:
- A retrospective review of nearly 500 pediatric SCD patients seen since December 1995.
- Analysis of 59 surgical procedures performed by the pediatric surgery service.
- Detailed examination of preoperative, intraoperative, and postoperative management for patients who developed ACS.
Main Results:
- Six of 63 ACS episodes (10.2% incidence) occurred within 2 weeks post-surgery.
- All patients received preoperative oxygen, IV fluids, and some required transfusions.
- Postoperative care included oxygen supplementation and IV fluids; onset ranged from 1 hour to 7 days.
- No deaths occurred, but hospitalization increased by an average of 6.1 days.
Conclusions:
- Postoperative ACS incidence in pediatric SCD patients is significantly higher (10.2%) than previously reported (0.4%).
- Laparoscopic procedures were associated with a higher incidence of ACS, challenging assumptions about their safety.
- Close monitoring and management are essential, as known risk factors do not fully prevent ACS.
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 intraoperative 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.