To operate or not to operate? Assessing NSQIP surgical outcomes in trisomy 18 patients

Trisomy 18 is associated with a wide range of potentially fatal congenital conditions. Historically, clinical attitudes on treatment have been ambiguous, with palliative care as the standard of care. The aim of our study was to provide a descriptive analysis of surgical outcomes in patients with trisomy 18.

We identified patients with trisomy 18 aged 0-18 years using the NSQIP-Pediatric database from 2012 to 2017 and analyzed demographics, surgery types, and perioperative characteristics of patients with trisomy 18 patients undergoing surgical intervention. Additionally, a case-match analysis was performed to assess surgical outcome differences.

A total of 310 patients with trisomy 18 were identified. Thirty-one percent were >5 years of age and 73% were female. The most common surgical types were general surgery procedures (57.4%), followed by orthopedics (18.1%) and ENT (10.3%). Operations performed increased from 8% (2012) to 26% (2017), and only 23% of patients had previous cardiac surgery. Majority of patients had no prior history of malignancy (95%) and 5% had a tracheostomy placed. Discharge to home was achieved in 74% of patients, with a median total hospital length of stay of 5 days (IQR 17). Furthermore, 90% survived over 30 days from the operation. Thirty-two patients had readmissions and the most common reasons were dehydration, gastrostomy infection or malfunction. Surgical site infections occurred in <3% of patients. No differences in complications, length of stay, reoperations, and readmissions were identified by case-match analysis.

In this data set, patients with trisomy 18 undergoing noncardiac surgical procedures experience excellent surgical outcomes with minimal morbidity and low mortality. Most patients more than a year of age will experience similar outcomes to patients without trisomy 18.

Treatment study (retrospective comparative study) LEVEL OF EVIDENCE: Level III.