Congenital diaphragmatic hernia classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Arooj Naz, M.B.B.S

Overview

There are multiple subtypes of Congenital Diaphragmatic Hernia. These include Bochdalek hernia, Morgagni's hernia and Diaphragm Eventration. Of these, the most common subtype is a Bochdalek hernia in which there is an anatomical defect in the postero-lateral corner of the diaphragm. Morgagni's hernia, which is responsible for approximately 2% of cases, are due to herniation through the foramina of Morgagni.

Classification

Bochdalek Hernia

Bochdalek hernia, also known as a postero-lateral diaphragmatic hernia, is the most common manifestation of CDH, accounting for more than 95% of cases.[1][2] In this instance, the diaphragm abnormality is characterized by a hole in the postero-lateral corner of the diaphragm which allows passage of the abdominal viscera into the chest cavity. The majority of Bochdalek hernias (80-85%) occur on the left side of the diaphragm, a large proportion of the remaining cases occur on the right side, and a small fraction are bilateral i.e., left and right sided defects. [3][4]

Morgagni's Hernia

This rare anterior defect of the diaphragm is variably referred to as Morgagni’s, retrosternal, or parasternal hernia. Accounting for approximately 2% of all CDH cases, it is characterised by herniation through the foramina of Morgagni which are located immediately adjacent to the xyphoid process of the sternum.[5]

Diaphragm Eventration

The diagnosis of congenital diaphragmatic eventration is used when there is abnormal displacement (i.e. elevation) of part or all of an otherwise intact diaphragm into the chest cavity. This rare type of CDH occurs because the diaphragm is thinner in the region of eventration, allowing the abdominal viscera to protrude upwards. This thinning is thought to occur because of incomplete muscularisation of the diaphragm, and can be found unilaterally or bilaterally. [6]

References

  1. Torfs CP, Curry CJ, Bateson TF, Honoré LH (1992). "A population-based study of congenital diaphragmatic hernia". Teratology. 46 (6): 555–65. doi:10.1002/tera.1420460605. PMID 1290156.
  2. Yang W, Carmichael SL, Harris JA, Shaw GM (2006). "Epidemiologic characteristics of congenital diaphragmatic hernia among 2.5 million California births, 1989-1997". Birth Defects Res. Part A Clin. Mol. Teratol. 76 (3): 170–4. doi:10.1002/bdra.20230. PMID 16511883.
  3. Torfs CP, Curry CJ, Bateson TF, Honoré LH (1992). "A population-based study of congenital diaphragmatic hernia". Teratology. 46 (6): 555–65. doi:10.1002/tera.1420460605. PMID 1290156.
  4. Lally KP, Lally PA, Lasky RE; et al. (2007). "Defect size determines survival in infants with congenital diaphragmatic hernia". Pediatrics. 120 (3): e651–7. doi:10.1542/peds.2006-3040. PMID 17766505.
  5. Torfs CP, Curry CJ, Bateson TF, Honoré LH (1992). "A population-based study of congenital diaphragmatic hernia". Teratology. 46 (6): 555–65. doi:10.1002/tera.1420460605. PMID 1290156.
  6. Thomas TV (1970). "Congenital eventration of the diaphragm". Ann. Thorac. Surg. 10 (2): 180–92. PMID 4913762.

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