Published: 2022-09-26

A rare case of an Omphalocele Major with associated Tetralogy of Fallot in a newborn

Dharmendra K. Shah, Shivani R. Chaudhary, Margi A. Shah


Omphalocele major (OM) is a surgically challenging condition which owing to its large defect size and other congenital anomalies, is associated with poor outcomes. Complications while managing it are not uncommon. But here, we report a case of OM in a neonate who was successfully managed in a multidisciplinary approach by a team of neonatologist, surgeons and anesthetist.


Omphalocele, Abdominal wall defect, Neonate

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Anyanwu LC, Ade-Ajayi N, Rolle U. Major abdominal wall defects in the low- and middle-income setting: current status and priorities. Pediatr Surg Int. 2020;36(5):579-90.

Seymour NE, Bell RL. Abdominal Wall, Omentum, Mesentery, and Retroperitoneum In: Brunicardi F, Andersen DK, Billiar TR, Dunn DL, Hunter JG, Matthews JB, Pollock RE, editors. Schwartz's Principles of Surgery. McGraw Hill. 2015.

Bilal R, Rustemov D, Sakuov Z, Ibraimov B and Kozhakhmetov A. Case Report: A Rare Case of a Combination of Omphalocele with Umbilical Teratoma. Front Pediatr. 2021;9:726593.

Nolan HR, Wagner ML, Jenkins T, Lim FY. Outcomes in the Major omphalocele population: A single center comprehensive experience. J Pediatr Surg. 2020;55(9):1866-71.

Bauman B, Stephens D, Gershone H, Bongiorno C, Osterholm E, Acton R, Hess D, Saltzman D, Segura B. Management of Major omphaloceles: A systematic review of methods of staged surgical vs. nonoperative delayed closure. J Pediatr Surg. 2016;51(10):1725-30.

van Eijck FC, Aronson DA, Hoogeveen YL, Wijnen RMH. Past and current surgical treatment of Major omphalocele: outcome of a questionnaire sent to authors. 2011;46(3):488.

Puri B, Sreevastava DK. Exomphalos Defects: A Review of 15 Cases. Med J Armed Forces India. 2008;64(2):115-8.

Vachharajani AJ, Rao R, Keswani S, Mathur AM. Outcomes of exomphalos: an institutional experience. Pediatr Surg Int. 2009;25(2):139-44.

Akinkuotu AC, Sheikh F, Olutoye OO, Lee TC, Fernandes CJ, Welty SE, Ayres NA, Cass DL. Giant omphaloceles: surgical management and perinatal outcomes. J Surg Res. 2015;198(2):388-92.