Abstract：
BACKGROUND: Patients with Trisomy 13(T13) and 18(T18) have many comorbidities that may require surgical 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 mortality after surgery. Those who survived to undergo surgery survived longer overall.
METHODS: III.
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 mortality after surgery. Those who survived to undergo surgery survived longer overall.
METHODS: III.
摘要：
背景：13三体（T13）和18三体（T18）患者有许多合并症，可能需要手术干预。然而,手术护理和结果没有很好的描述，使患者选择和家庭咨询变得困难。本文探讨了T13/T18患者的手术史和结果。
方法：对1990年至2020年出生并在三级儿童医院接受护理的T13或T18患者进行回顾性回顾（Riley儿童医院，印第安纳波利斯IN)进行了，排除记录不足的人。感兴趣的主要结果是总体和手术后的死亡率。还评估了可以预测死亡率结果的因素。
结果：纳入了117名患者，有65%的T18和35%的T13。超过一半的患者（65％）有四种或更多种合并症。大多数死亡发生在三个月，中位数为42.0天。经典三体的变体(马赛克，易位,部分重复；p=0.001)，出生体重较高（p=0.002），和较高的胎龄（p=0.01）与较低的总死亡率相关，而心脏病（p=0.002）与较高的死亡率相关。超过一半（n=64）在首次手术时的中位年龄为65天接受了手术。最常见的外科手术是普通外科手术。手术而非手术患者的中位生存时间更长（p<0.001）。变异的三体遗传学（p=0.002）与手术后死亡率较低相关，而普外科合并症(p=0.02)，特别是气管食管瘘/食管闭锁（p=0.02），与手术后死亡率增加有关。
结论：13和18三体患者有巨大的手术需求。变异型三体与术后死亡率较低相关，而普通外科合并症与术后死亡率增加相关。那些幸存下来接受手术的人总体上存活了更长的时间。
方法：III.
方法：对1990年至2020年出生并在三级儿童医院接受护理的T13或T18患者进行回顾性回顾（Riley儿童医院，印第安纳波利斯IN)进行了，排除记录不足的人。感兴趣的主要结果是总体和手术后的死亡率。还评估了可以预测死亡率结果的因素。
结果：纳入了117名患者，有65%的T18和35%的T13。超过一半的患者（65％）有四种或更多种合并症。大多数死亡发生在三个月，中位数为42.0天。经典三体的变体(马赛克，易位,部分重复；p=0.001)，出生体重较高（p=0.002），和较高的胎龄（p=0.01）与较低的总死亡率相关，而心脏病（p=0.002）与较高的死亡率相关。超过一半（n=64）在首次手术时的中位年龄为65天接受了手术。最常见的外科手术是普通外科手术。手术而非手术患者的中位生存时间更长（p<0.001）。变异的三体遗传学（p=0.002）与手术后死亡率较低相关，而普外科合并症(p=0.02)，特别是气管食管瘘/食管闭锁（p=0.02），与手术后死亡率增加有关。
结论：13和18三体患者有巨大的手术需求。变异型三体与术后死亡率较低相关，而普通外科合并症与术后死亡率增加相关。那些幸存下来接受手术的人总体上存活了更长的时间。
方法：III.
