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Category: Volume 09 Issue 11 November 2021
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Title: Gigant Morgagni Congenital Hernia Associated with Intestinal Malrotation and Meckel Diverticulum in Child with Down Syndrome- Clinical Case Presentation and Literature Review

Authors: Babuci S.,  Negru I., Eremia V., Malanco S.

 DOI: https://dx.doi.org/10.18535/jmscr/v9i11.22

Abstract

Introduction

Morgagni congenital hernia is an intrathoracic protrusion of the abdominal contents by a congenital retrosternal diaphragmatic defect, caused by fusion disorders of the diaphragmatic transverse septum and chondrocostal arches (Larrey slot), triangular spaces located anteriorly between the diaphragmatic bundles [3,5,11].

There is some confusion about the name of this diaphragmatic hernia, with some authors using the term "Morgagni hernia" for the right hernia and "Larrey hernia" for the left hernia[22]. In the literature this type of congenital diaphragmatic defect can also be found under the name of: Morgagni-Larrey hernias, retro-costo-xiphoid, parasternal, retrochondrosteranal, retrosternal, substernal, etc.[5,22].

Morgagni's hernia is relatively rare, accounting for approximately 3-5% of all types of congenital diaphragmatic hernia. About 90% of this type of hernia occurs on the right, 2% on the left, and in 8% of cases bilateral forms are found. The herniated sac is present in over 95% of cases [7, 10, 25].

The incidence of abnormalities associated with Morgani hernia is about 64%, in 30-40% of patients with Down syndrome, and in 23-44% being found congenital heart malformations[4,7]. Other malformations associated with this type of diaphragmatic defect are: Cantrell's pentalogy, Noonan syndrome, Pradere Willi syndrome, Turner syndrome, intestinal malrotation, anorectal malformations, etc.[4,9,10].

References

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Corresponding Author

Babuci S.

IMSP Mother and Child Institute “Natalia Gheorghiu” National Scientific-Practical Center for Pediatric Surgery