O0351 Oct.3: 5:31pm - Trisomy 18 trends over the last 20 years

Background: Patients born with trisomy 18 are known to have multiple congenital anomalies potentially requiring intervention. Population based data on hospital admissions for patients with trisomy 18 are not widely available. We sought to describe the trends in hospitalizations for children coded for trisomy 18 over time and to determine the rate of performance of invasive interventions on these children.

Methods: A retrospective analysis using the Healthcare Cost and Utilization Project (HCUP) KID data from 1997- 2016 was performed using ICD-9 /10 codes for trisomy 18. We evaluated survival to discharge as well as the presence of pulmonary, skeletal, neurologic, GI, renal, hematologic/bleeding problems. We also sought the following interventions, if performed: gastrostomy tube placement, tracheostomy or cardiac intervention.

Results: Over this period 10,151 admissions occurred in children with a diagnosis of trisomy 18. Between 1997 and 2016 the number of children admitted with Trisomy 18 increased 42% from 1,036 to 1,798. The average age at admission was 19.3 months. The average admission age increased throughout the study period, from 13.0 months in 1997 to 31.1 months in 2016. The proportion of patients born prematurely (<38 weeks gestation) remained stable throughout the study ranging from 14-16% born prematurely. 69 of 1,036 patients in 1997 cohort had an intervention performed, consisting of 6.5% of cases. In 2016, 756 out of 1798 patients had procedures done, consisting of 42% of patients. Gastrostomy tube was placed in 47 of 1,036 patients in 1997 and 568 of 1,798 patients in 2016, an increase from 4.5% to 31.5% respectively. Tracheostomy was performed in 14 of 1,036 pts in 1997 and 148 of 1,798 in 2016, which represented an increase from 1.3% to 8.2%. Cardiac intervention was performed in 8 of 1,036 patients in 1997, while 40 of 1,798 received a cardiac intervention in 2016 (Figure 1). The overall mortality rate decreased in those with trisomy 18 from 32% in 1997 to 21% in 2016. This trend was identified throughout different regions of the country, but was more pronounced in the northeast region than in other areas (Figure 2).

Conclusions: We highlight here a decreased mortality rate between 1997 and 2016 for children diagnosed with trisomy 18. Throughout this time period the number of children receiving interventions such as gastrostomy tube and tracheostomy increased, as did the number of children undergoing cardiac intervention. There has been a marked increase in gastrostomy tube placements as of late. While the number of procedures has increased with the mortality rate decreasing, it is unclear at present if the two are related. Further evaluation and planning for children with Trisomy 18 is necessary as more children are surviving.