A fatal combination of duodenal atresia with preduodenal portal vein, malrotation, and hypoplastic left heart syndrome: A case report
Keywords:Duodenal atresia, Preduodenal portal vein, Hypoplastic left heart syndrome, Malrotation
Background: Duodenal atresia (DA) is often associated with anomalies that include annular pancreas, cardiac anomalies, intestinal malrotation, situs inversus, or splenic anomalies. Association of duodenal atresia with complex cardiac anomalies is scarcely reported in the literature.
Case Presentation: A term neonate was diagnosed with duodenal atresia and found to have a preduodenal portal vein and malrotation. A gastro jejunostomy was added to the procedure, due to the pre duodenal portal vein. On post-operative day 4, the patient had sudden desaturation. The respiratory system was normal and there was no evidence of septicaemia. On the post-operative echocardiogram, the diagnosis of hypoplastic left heart was made and the patient eventually succumbed to his complex cardiac disease.
Conclusion: This is a rare combination of multiple anomalies and we report the clinical and anatomical findings of this patient.
Applebaum H, Sydorak R. Duodenal atresia and stenosis- annular pancreas Chapter81. In: Coran AG, Caldamone A, Adzick NS, Krummel TM, Laberge JM, Shamberger R (Eds). Pediatric Surgery.7th ed. Philadelphia, PA: Elsevier Inc;2012. p1051-58.
Kim S, Cho Y, Kim H. Preduodenal portal vein: a 3-case series demonstrating varied presentations in infants. J Korean Surg Soc. 2013;85:195-7.
Choudhry MS, Rahman N, Boyd P, Lakhoo K. Duodenal atresia: associated anomalies, prenatal diagnosis and outcome. Pediatr Surg Int. 2009;25:727-30.
Wabada S, Abubakar AM, Mustapha B, Pius S, Khalil J, Abana AK. Congenital duodenal obstruction due to duodenal atresia with preduodenal portal vein, annular pancreas, and intestinal malrotation associated with situs inversus abdominis: A case report. J Pediatr Surg Case Rep. 2015; 3:545-47.
Mirza B, Sheikh A. Multiple associated anomalies in patients of duodenal atresia: a case series. J Neonatal Surg. 2012; 1:23.
Mboyo A, Khadir SK, Guillaume MP, Massicot R, Flurin V, Lalioui A, et al. An exceptional cause of duodenal obstruction detected antenatally: A compressive preduodenal portal vein. J Pediatr Surg Case Rep.2013; 1:420-24.
Charles DF, Lauren CK. Pediatric Surgery. Chapter 66. In: Townsend C, Beauchamp R, Evers B, Mattox K(Eds). Sabiston textbook of surgery. 20th ed. Philadelphia PA: Elsevier Inc; 2016. p1619-57.
Hickey EJ, Caldarone CA, McCrindle BW. Left ventricular hypoplasia: a spectrum of disease involving the left ventricular outflow tract, aortic valve, and aorta. JACC.2012;59:S43-S54.
Ferreira CR, Aiello VD, Melo AMAGP, Mota LB, de Carvalho ANC, SouzaHSP, et al. Classic form of hypoplastic left heart syndrome diagnosed postnatally: an autopsy report. Autopsy Case Rep. 2014;4:21-30. http://dx.doi.org/10.4322/acr.2014.025.
Wernovsky G, Bove EL. Single ventricle lesions. In: Chang AC, Hanley FL, Wenovsky G, et al, eds. Pediatric Cardiac Intensive Care. Baltimore, Md: Williams & Wilkins; 1998:271–287.
How to Cite
Copyright (c) 2020 Priyanka Mittal, Nitin James Peters, Ram Samujh
This work is licensed under a Creative Commons Attribution 4.0 International License.
You are free to:
- Share — copy and redistribute the material in any medium or format
- Adapt — remix, transform, and build upon the material for any purpose, even commercially.
- Attribution — You must give appropriate credit, provide a link to the license, and indicate if changes were made. You may do so in any reasonable manner, but not in any way that suggests the licensor endorses you or your use.
- No additional restrictions — You may not apply legal terms or technological measures that legally restrict others from doing anything the license permits.