Characteristics and Outcomes of Pediatric Trisomy 13 and 18 Inpatient Admissions in Texas.

INTRODUCTION

Trisomy 13 (T13, Patau syndrome) and trisomy 18 (T18, Edwards syndrome) are the second and third most common aneuploidies with significant morbidity and mortality. Survival beyond infancy is becoming increasingly recognized. However, surgical needs of these populations are not well described. We aim to investigate the characteristics and outcomes of the surgical care of pediatric patients with T13 and T18 in Texas.

METHODS

The Texas Hospital Inpatient Discharge Public Use Data File (2017-2023) was queried to identify all pediatric admissions (<18 y) with T13 and T18 using International Classification of Diseases, Tenth Revision codes. Therefore, transfers to outside hospital were excluded to avoid systematic double counting. Descriptive statistics were performed (median, [interquartile range]).

RESULTS

A total of 582 admissions were identified (181 T13, 401 T18). The majority of admissions occurred in the first year of life. Overall inpatient mortality rates were high in both (T13 27.1%, T18 23.7%) with highest mortality in neonates (≤28 d; T13 65.6%, T18 56.4%). Notably, inpatient mortality in older admissions dramatically decreased. Across all age groups, length of stay and median charges were highest for infants (29-365 d) (T13 length of stay 17 d [3-31], T18 16 d [5-43]) with frequent operative interventions, which persisted throughout childhood. While cardiovascular anomalies were prevalent in the younger cohort, supportive operative interventions including airway, vascular, and feeding access were the most common procedures.

CONCLUSIONS

While T13 and T18 both have high mortality, particularly in infancy, there is survival with persistent health-care utilization and operative needs throughout childhood. This highlights significant long-term health-care needs and evolving surgical indications. Longitudinal care coordination of multidisciplinary surgical care is needed to optimize outcomes of affected patients.