%0 Journal Article
%T Impact of definitive surgery for esophageal atresia on long-term outcomes in patients with trisomy 18.
%A Tamaki S
%A Iwatani S
%A Ikuta T
%A Takeoka E
%A Matsui S
%A Mimura H
%A Yokoi A
%A Hatakeyama T
%A Yoshimoto S
%A Nakao H
%J Am J Med Genet A
%V 0
%N 0
%D 2024 Jun 17
%M 38884184
%F 2.578
%R 10.1002/ajmg.a.63792
%X This study investigates the long-term outcomes of palliative and definitive surgeries for esophageal atresia (EA) in patients with trisomy 18 syndrome. A retrospective study included 25 cases undergoing EA surgery at our center between 2008 and 2022. The Palliative group (n = 16) comprised 13 cases with esophageal banding and 3 with tracheoesophageal fistula (TEF) division. The Definitive group (n = 9) included 5 cases with primary repair and 4 with staged repair following TEF division. The patient characteristics exhibited no significant differences between the groups. In the Definitive group, 56% (5/9) were successfully weaned off mechanical ventilation, compared with none in the Palliative group (p = 0.002). Survival-to-discharge rates were 31% (5/16) in the Palliative group and 67% (6/9) in the Definitive group. Home ventilator management was required for all 5 cases that required ventilation in the Palliative group, whereas only 17% (1/6) in the Definitive group needed it. The Palliative group also required continuous oral suction for persistent saliva removal, with two cases undergoing laryngotracheal separation. Overall, definitive surgery for EA in patients with trisomy 18 syndrome may provide enhanced respiratory stability, thereby improving the survival-to-discharge rate and overall quality of life for patients and their families.