Surgical History and Outcomes in Trisomy 13 and 18: A Thirty-year Review

Background: Patients with Trisomy 13(T13) and 18(T18) have many comorbidities that may require sur-gical intervention. However, surgical care and outcomes are not well described, making patient selection and family counseling difficult. Here the surgical history and outcomes of T13/ T18 patients are explored.Methods: A retrospective review of patients with T13 or T18 born between 1990 and 2020 and cared for at a tertiary children's hospital (Riley Hospital for Children, Indianapolis IN) was conducted, excluding those with insufficient records. Primary outcomes of interest were rates of mortality overall and after surgery. Factors that could predict mortality outcomes were also assessed.Results: One-hundred-seventeen patients were included, with 65% T18 and 35% T13. More than half of patients(65%) had four or more comorbidities. Most deaths occurred by three months at median 42.0 days. Variants of classic trisomies (mosaicism, translocation, partial duplication; p = 0.001), higher birth weight(p = 0.002), and higher gestational age(p = 0.01) were associated with lower overall mortality, while cardiac(p = 0.002) disease was associated with higher mortality. Over half(n = 64) underwent surgery at median age 65 days at time of first procedure. The most common surgical procedures were general surgical. Median survival times were longer in surgical rather than nonsurgical patients(p < 0.001). Variant trisomy genetics(p = 0.002) was associated with lower mortality after surgery, while general surgical comorbidities(p = 0.02), particularly tracheoesophageal fistula/esophageal atresia(p = 0.02), were associated with increased mortality after surgery. Conclusions: Trisomy 13 and 18 patients have vast surgical needs. Variant trisomy was associated with lower mortality after surgery while general surgical comorbidities were associated with increased mor-tality after surgery. Those who survived to undergo surgery survived longer overall.;evel of evidence: III.& COPY; 2022 Elsevier Inc. All rights reserved.

Automated summary

This study retrospectively reviewed the surgical history and outcomes of patients with Trisomy 13 and 18 at Riley Hospital for Children in Indianapolis from 1990 to 2020. The results showed that surgical intervention was necessary for these patients, but the mortality rate after surgery was high. Factors such as genetic variant and surgical comorbidities were associated with postoperative mortality.