Esophageal atresia: predicting outcomes and decreasing mortality

Abstract

This literature review is devoted to the problem of predicting in-hospital mortality in newborns with esophageal atresia (EA). According to epidemiological study data, in developed countries, the mortality rate in newborns with EA ranges from 9% to 11% over the past 20 years. Three classifications were developed, Waterston 1962, Montreal 1993, and Spitz 1994, to assess the prognostic significance of risk factors. They considered birth weight, the presence of concomitant congenital malformations and pneumonia, and the need for mechanical ventilation. The choice of a model for predicting outcomes depends on the level of health care and other factors, such as prematurity, low birth weight, late diagnosis, and infectious complications. These factors have a greater impact on patient survival in developing countries than in developed ones, where insurmountable risk factors come out on top: combined congenital malformations and very low birth weight. Also, the magnitude of diastasis between segments of the esophagus creates difficulties in choosing surgical tactics and managing such patients in the postoperative period. In addition, the management of such patients in the intensive care unit, both preoperatively and postoperatively, has a significant impact on the outcome. The literature review underlined "pain points" in the treatment of newborns with EA in regions with different levels of medical care, the consideration of which will allow the achievement of better results.

Keywords

esophageal atresia / neonatal surgery / risk factors / mortality

References

[1] Holcomb GW III, Murphy JD, Shawn D St P. Holcomb and Ashcraft’s Pediatric Surgery. 7th ed. Elsevier, 2020. p 437.

[2] Караваева С.А., Котин А.Н., Немилова Т.К., и др. История хирургии атрезии пищевода // Российский вестник детской хирургии, анестезиологии и реаниматологии. — 2011. — № 1. — С. 9–12. [Karavaeva SA, Kotin AN, Nemilova TK, et al. History of surgery for oesophageal atresia. Russian Journal of Pediatric Surgery, Anesthesia and Intensive Care. 2011;(1):9–12. (In Russ.)]

[3] Pedersen RN, Calzolari E, Husby S, et al. Oesophageal atresia: prevalence, prenatal diagnosis and associated anomalies in 23 European regions. Arch Dis Child. 2012;97(3):227–232. https://doi.org/10.1136/archdischild-2011-300597

[4] Sfeir R, Bonnard A, Khen-Dunlop N, et al. Esophageal atresia: data from a national cohort. Jurnal of pediatric surgery. 2013;48(8):1664–1669. https://doi.org/10.1016/j.jpedsurg.2013.03.075

[5] Losty PD. Oesophageal Atresia. In: Rickham’s Neonatal Surgery. Losty PD, Flake AW, Rintala RJ, editors. London: Springer Publishers, 2018. 541–562 p. https://doi.org/10.1007/978-1-4471-4721-3_23

[6] Birketvedt K, Mikkelsen A, Klingen LL, et al. Nutritional Status in Adolescents with Esophageal Atresia. J Pediatr. 2020;218:130–137. https://doi.org/10.1016/j.jpeds.2019.11.034

[7] Rayyan M, Embrechts M, Veer VH, et al. Neonatal factors predictive for respiratory and gastro-intestinal morbidity after esophageal atresia repair. Pediatrics & Neonatology. 2019;60(3):261–269. https://doi.org/10.1016/j.pedneo.2018.07.003

[8] Rozensztrauch A, Śmigiel R, Patkowski D. Congenital Esophageal Atresia-Surgical Treatment Results in the Context of Quality of Life. Eur J Pediatr Surg. 2019;29(03):266–270. https://doi.org/10.1055/s-0038-1641597

[9] Ritz LA, Widenmann-Grolig A, Jechalke S, et al. Outcome of Patients With Esophageal Atresia and Very Low Birth Weight (≤1.500 g). Front Pediatr. 2020;8:587285. https://doi.org/10.3389/fped.2020.587285

[10] Svoboda E. A patient led, international study of long term outcomes of esophageal atresia: EAT 1. J Pediatr Surg. 2018;583:8–9. https://doi.org/10.1016/j.jpedsurg.2017.05.033

[11] Prato AP, Carlucci M, Bagolan P, et al. A cross-sectional nationwide survey on esophageal atresia and tracheoesophageal fistula. J Pediatr Surg. 2015;50:1441–1456. https://doi.org/10.1016/ j.jpedsurg.2015.01.004

[12] Sparey C, Jawaheer G, Barrett AM, et al. Esophageal atresia in the Northern Region Congenital Anomaly Survey, 1985–1997: prenatal diagnosis and outcome. Am J Obstet Gynecol. 2000;182:427–431. https://doi.org/10.1055/s-0038-1641597

[13] Zimmer J, Eaton S, Murchison LE, et al. State of play: eight decades of surgery for esophageal atresia. Eur J Pediatr Sur. 2019;29(01):39–48. https://doi.org/10.1055/s-0038-1668150

[14] Lal D, Miyano G, Juang D, et al. Current patterns of practice and technique in the repair of esophageal atresia and tracheoesophageal fistua: an IPEG survey. J Laparoendosc Adv Surg Tech A. 2013;23(7):635–638. https://doi.org/10.1089/lap.2013.0210

[15] Zani A, Eaton S, Hoellwarth ME, et al. Agostino PierroInternational survey on the management of esophageal atresia. Eur J Pediatr Surg. 2014;24(01):3–8. https://doi.org/10.1055/s-0033-1350058

[16] Waterston DJ, Carter RB, Aberdeen E. Oesophageal atresia: tracheo-oesophageal fistula: a study of survival in 218 infants. Lancet. 1962;1(7234):819–822. https://doi.org/10.1016/S0140-6736(62)91837-8

[17] Poenaru D, Laberge JM, Neilson IR, et al. A new prognostic classification for esophageal atresia. Surg. 1993;113(4):426–432. https://doi.org/10.1016/0022-3468(91)91022-Q

[18] Spitz L, Kiely EM, Morecroft JA, et al. Oesophageal atresia: at-risk groups for the 1990s. J Pediatr Surg. 1994;29(6):723–725. https://doi.org/10.1016/0022-3468(94)90354-9

[19] Peters RT, Ragab H, Columb MO, et al. Mortality and morbidity in oesophageal atresia. Pediatr Surg Int. 2017;33(9):989–994. https://doi.org/10.1007/s00383-017-4124-1

[20] Malakounides G, Lyon P, Cross K, et al. Esophageal atresia: improved outcome in high-risk groups revisited. Eur J Pediatr Surg. 2016;26(03):227–231. https://doi.org/10.1055/s-0035-1551567

[21] Sulkowski JP, Cooper JN, Lopez JJ, et al. Morbidity and mortality in patients with esophageal atresia. Surg. 2014;156(2):483–491. https://doi.org/10.1016/j.surg.2014.03.016

[22] Wang B, Tashiro J, Allan BJ, et al. A nationwide analysis of clinical outcomes among newborns with esophageal atresia and tracheoesophageal fistulas in the United States. J Surg Res. 2014;190(2):604–612. https://doi.org/10.1016/j.jss.2014.04.033

[23] Yamoto M, Nomura A, Fukumoto K, et al. New prognostic classification and managements in infants with esophageal atresia. Pediatr Surg Int. 2018;34(10):1019–1026. https://doi.org/10.1007/s00383-018-4322-5

[24] Hartley MJ, Smith NP, Jaffray B. Statistical modelling of survival for babies with oesophageal atresia. J Pediatr Surg. 2016;51(7):1110–1114. https://doi.org/10.1016/j.jpedsurg.2015.11.016

[25] Schmidt A, Obermayr F, Lieber J, et al. Outcome of primary repair in extremely and very low-birth-weight infants with esophageal atresia/distal tracheoesophageal fistula. J Pediatr Sur. 2017; 52(10):1567–1570. https://doi.org/10.1016/j.jpedsurg.2017.05.011

[26] Hannon EJ, Billington J, Kiely EM, et al. Oesophageal atresia is correctable and survivable in infants less than 1 kg. Pediatr Surg Int. 2016;32(6):571–576. https://doi.org/10.1007/s00383-015-3851-4

[27] Folaranmi SE, Jawaid WB, Gavin L, et al. Influence of birth weight on primary surgical management of newborns with esophageal atresia. J Pediatr Surg. 2020;55(11):2251. https://doi.org/10.1016/j.jpedsurg.2020.11.023

[28] Quiroz HJ, Turpin A, Willobee BA, et al. Nationwide analysis of mortality and hospital readmissions in esophageal atresia. J Pediatr Surg. 2020;55(5):824–829. https://doi.org/10.1016/j.jpedsurg.2020.01.025

[29] Tanny SPT, Fearon E, Hawley A, et al. Predictors of Mortality after Primary Discharge from Hospital in Patients with Esophageal Atresia. J Pediatr. 2020;219:70–75. https://doi.org/10.1016/j.jpeds.2019.12.031

[30] Davari HA, Hosseinpour M, Nasiri GM, et al. Mortality in esophageal atresia: assessment of probable risk factors (10 years’ experience). J Res Med Sci. 2012;17(6):540–542.

[31] Vukadin M, Savic D, Malikovic A, et al. Analysis of prognostic factors and mortality in children with esophageal atresia. Ind J Pediatr. 2015;82(7):586–590. https://doi.org/10.1007/s12098-015-1730-6

[32] Ammar S, Sellami S, Sellami I, et al. Management of esophageal atresia and early predictive factors of mortality and morbidity in a developing country. Dis Esophagus. 2019;32(6):doy135. https://doi.org/10.1093/dote/doy135

[33] Akhter N, Javeria J, Jan IA, et al. Factors affecting survival in patients with oesophageal atresia and tracheo-oesophageal fistula. J Ayub Med Coll Abbottabad. 2009;21(4):129–133.

[34] Nagdeve N, Sukhdeve M, Thakre T, et al. Esophageal atresia with tracheo-esophageal fistula presenting beyond 7 days. J Neonatal Surg. 2017;6(3):57. https://doi.org/10.21699/jns.v6i3.577

[35] Li XW, Jiang YJ, Wang XQ, et al. A scoring system to predict mortality in infants with esophageal atresia: A case-control study. Medicine (Baltimore). 2017;96(32):e7755. https://doi.org/10.1097/MD.0000000000007755

[36] Brown AK, Tam PK. Measurement of gap length in esophageal atresia: a simple predictor of outcome. J Am Coll Surg. 1996;182(1):41–45.

[37] Hands LJ, Dudley NE. A comparison between gap-length and Waterston classification as guides to mortality and morbidity after surgery for esophageal atresia. J Pediatr Surg. 1986;21(5):404–406. https://doi.org/10.1016/S0022-3468(86)80508-5

[38] Upadhyaya VD, Gangopadhyaya AN, Gupta DK, et al. Prognosis of congenital tracheoesophageal fistula with esophageal atresia on the basis of gap length. Pediatr Surg Int. 2007;23(8):767–771. https://doi.org/10.1007/s00383-007-1964-0

[39] Tandon RK, Sharma S, Sinha SK, et al. Esophageal atresia: Factors influencing survival-Experience at an Indian tertiary centre. J Ind Ass Pediatr Surg. 2008;13(1):2–6. https://doi.org/10.4103/0971-9261.42564

[40] Новожилов В.А., Валеев В.В., Козлов Ю.А., и др. Прогностические факторы летальности у новорожденных с атрезией пищевода // Acta Biomedica Scientifica. — 2006. — № 6. — С. 82–86. [Novozhilov VA, Valeyev VV, Kozlov JA, et al. The prognostic factors of mortality in newborns with esophageal atresia. Acta Biomedica Scientifica. 2006;(6):82–86. (In Russ.)]

RIGHTS & PERMISSIONS

Mukhametshin R.F., Toropov N.V., Kabdrakhmanova O.T.