IS Case 422: Congenital Morgagni hernia

Jeremy Duda, MD

University of Rochester

Imaging Sciences URMC 2010
Publication Date: 2010-08-27

History

Patient is a 10-month-old male with trisomy 21 presenting for pre-operative evaluation for repair of a Tetrology of Fallot malformation.

Findings

PA and lateral chest radiographs demonstrate bilateral peribronchial haziness consistent with known cardiac anomaly, including a right-sided aortic arch and uplifted cardiac apex. Air filled-loops of bowel are seen protruding through an defect in the diaphragm anterior to the mediastinum.

Discussion

Morgagni hernia comprises 3-5% of congenital diaphragmatic hernias. The entity is named in reference to the defect through which abdominal contents protrude. The foramen of Morgagni is a potential space formed by fibers of the diaphragm that originate on the sternum and xiphoid process anteriorly and insert into the central diaphragmatic tendon, through which the superior epigastric vessels pass. Congenital herniation results when the fibrotendinous portions of the pars sternalis and the costochondral arches do not properly fuse. Herniation is right-sided 90% of the time owing to the presence of pericardial attachments to the left side of the thorax.

Symptoms are often nonspecific, but are usually from gastrointestinal problems or recurrent respiratory infections, and may be delayed. Presentation in adulthood can be caused by any mechanism which increases intra-abdominal pressure such as trauma, pregnancy or obesity.

Although rare, congenital Morgagni hernia is often associated with congenital abnormalities, reaching as high as 70% prevalence in one study. Trisomy 21, cardiac abnormalities and malrotation are the three most common associated conditions.

Initial imaging may be directed toward the patient's symptoms, including chest or abdominal x-ray or echocardiography. Bowel loops, fat or other abdominal contents may be identified above the diaphragm on lateral films anterior to the mediastinum. Ancillary modalities such as barium studies, CT scan may also be conducted to define the anatomic location of herniation, continuity of the bowel, and signs of strangulation.

Once identified, surgical treatment of congenital morgagni hernia may be pursued to avoid complications such as ischemia and perforation.

References

  1. Minneci PC, Deans KJ, Kim P, Mathisen DJ. Foramen of Morgagni hernia: changes in diagnosis and treatment. Ann Thorac Surg. 2004 Jun;77(6):1956-9. PMID: 15172245
  2. Al-Salem AH. Congenital hernia of Morgagni in infants and children. J Pediatr Surg. 2007 Sep;42(9):1539-43. PMID: 17848245
  3. Honoré LH, Torfs CP, Curry CJ. Possible association between the hernia of Morgagni and trisomy 21. Am J Med Genet. 1993 Aug 15;47(2):255-6. PMID: 8213915

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