Tan Tanny, S.P.Comella, A.Hutson, J.M.Omari, T.I.Teague, W.J.King, S.K.2020-04-202020-04-202019Journal of Pediatric Surgery, 2019; 54(12):2473-24780022-34681531-5037http://hdl.handle.net/2440/124250Background: Children born with esophageal atresia (EA) have inherent abnormalities in esophageal motility which may impact upon patient and family Quality of Life (QoL). Currently, paucity of data exists for long-term outcomes of long-gap EA. We aimed to: (1) summarize QoL tools reported in the literature, focusing upon studies involving long-gap EA patients, and (2) compare QoL for long-gap versus non-long-gap EA patients. Method: We performed a systematic review of Cochrane Register of Controlled Trials, PubMed, EMBASE, and Ovid databases (January 1980-May 2018) in accordance with the PRISMA protocol. Result: Six studies were identified (536 patients total), and 419/536 (78%) patients completed QoL assessment. Response rates ranged from 29% to 100%. Median study size was 86 (range 8-159). Esophageal atresia type was described in 477 patients, and 74/477 (16%) were long-gap. Common assessment tools were Gastrointestinal Quality of Life Index and 36-Item Short-Form Health Survey. Compared with healthy individuals, long-gap EA patients suffered more gastrointestinal symptoms. There were no significant differences in QoL outcomes between long-gap and non-long-gap EA patients. Conclusion: Current literature suggests no significant difference in QoL outcomes between long-gap and non-long-gap EA patients. However, due to questionnaire variability and range of response rates, the data should be interpreted with care. Level of Evidence: Level II.enCrown Copyright © 2019 Published by Elsevier Inc. All rights reserved.Esophageal atresia; long-gap; quality of life; systematic reviewJournal article100000512310.1016/j.jpedsurg.2019.08.0400005032279000032-s2.0-85074513811505813Omari, T.I. [0000-0001-5108-7378]Teague, W.J. [0000-0003-4747-6025]