NM-194

A 15-year retrospective review of 30-day post-anesthesia morbidity and mortality in children with sickle cell disease at a tertiary pediatric hospital

1Park B, 1Galvez J, 2Rehman M, 1Simpao A
1Children's Hospital of Philadelphia, Philadelphia, PA, United states; 2Johns Hopkins All Children's Hospital, St. Petersburg, FL, USA

Background
Stroke and acute chest syndrome (ACS) are debilitating complications of sickle cell disease (SCD) that can occur in the perioperative and postoperative periods. The primary goal of this study was to determine the prevalence of ACS, stroke, and death in children with SCD within 30 days of general anesthesia (GA) based on clinical documentation. A secondary goal was to describe the patient and surgical factors that were associated with morbidity and mortality in children with SCD undergoing GA.

Methods
A perioperative database was queried for all children with SCD under 18 years of age who underwent GA at CHOP from Jan 1, 2000 to December 31, 2016. We screened charts for mention of ‘stroke,’ ‘acute chest’ or ‘ACS’ by a clinician within 30 days of the patient undergoing GA. Two reviewers (BP, AS) independently reviewed the patient records that passed the initial screening to confirm the cases that met ACS criteria: evidence of a new segmental lung infiltrate (excluding atelectasis), and one or more of the following symptoms: fever (38.5° C), wheezing, cough, or chest pain.

Results
A total of 748 SCD patients met the study criteria; the study cohort was 58% male, median weight was 26.7 kg (IQR 17-41), and median age was 8 years (IQR 4-13). The most common procedural areas were intra-abdominal/laparoscopic (23.6%, n=177), tonsillectomy and/or adenoidectomy (T&A, 11.9%, n=90), and multiple (10.2%, n=76. The most common procedures performed were multiple (17.4%, n=130), T&A (11.2%, n=89), splenectomy (10.3%, n=77), and cholecystectomy (9.5%, n=71).

There were three cases of stroke within 30 days of GA. The procedural areas were neurosurgical (12.5%, n=2), one of which also led to ACS on the same day, and multiple areas (1.3%, n=1). ACS was recorded in 50 patients during the initial screening and ACS was confirmed in 49 cases. The ACS group was 49% male (n= 24), with a median weight of 29 kg (IQR 20-40), and median age of 10 years (IQR 5-14). The procedural areas with the highest rates of ACS were endoscopic-thoracic (100%, n=1), abdominal-laparoscopic (13%, n=23), abdominal-open (12.5%, n=1), neurosurgical (12.5%, n=2), and endoscopic-GI (10.9%, n=6). The ACS cases involving endoscopic-thoracic and abdominal-open areas occurred in the first decade of the study period. The ACS rates in the three most commonly performed procedures were 4.5% after T&A (n=4) 15.6% after splenectomy (n=12) and 11.3% after cholecystectomy (n=8). No cases of ACS after T&A occurred in the last five years of the study period. There were no deaths.

Discussion:
This is the first study to use clinical reports to describe the patient characteristics and acute chest syndrome in a large perioperative pediatric SCD cohort. ACS rates were highest in abdominal, neurosurgical, and endoscopic procedures, lower in T&A and orthopedic, and nil in vascular access and non-T&A ENT cases. Future research includes identifying the perioperative risk factors to predict and decrease stroke and ACS in children with SCD. Elucidating the perioperative conditions associated with common procedures, such as T&As, as well as the trends in morbidities and mortalities, can further improve outcomes for SCD patients.


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