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Congenital diaphragmatic hernia
Classification and external resources
ICD-10 Q79.0
ICD-9 756.6
OMIM 142340 222400
DiseasesDB 32489
MedlinePlus 001135
eMedicine ped/2603
MeSH D006548

Congenital diaphragmatic hernia (CDH) is a congenital malformation (birth defect) of the diaphragm. The most common type of CDH is a Bochdalek hernia; other types include Morgagni's hernia, diaphragm eventration and central tendon defects of the diaphragm. Malformation of the diaphragm allows the abdominal organs to push into the chest thereby impeding proper lung formation.

CDH is a life-threatening pathology in infants, and a major cause of death due to two complications: pulmonary hypoplasia and pulmonary hypertension.[1] Experts disagree on the relative importance of these two conditions, with some focusing on hypoplasia, others on hypertension.[2] Newborns with CDH often have severe respiratory distress which can be life-threatening unless treated appropriately.

Contents

Types of congenital diaphragmatic hernia

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Bochdalek hernia

The Bochdalek hernia, also known as a postero-lateral diaphragmatic hernia, is the most common manifestation of CDH, accounting for more than 95% of cases. [3][4] 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][5]

Morgagni's hernia

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 xiphoid process of the sternum. [3] The majority of hernias occur on the right side of the body and are generally asymptomatic; However newborns may present with respiratory distress at birth similar to Bochdalek hernia. Additionally, recurrent chest infections and gastrointestinal symptoms have been reported in those with previously undiagnosed Morgagni's hernia.[6][7] In asymptomatic individuals laparoscopic surgical repair is still recommended as they are at risk of a strangulated intestine.

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 in the region of eventration the diaphragm is thinner, 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.[8] Minor forms of diaphragm eventration are asymptomatic, however in severe cases infants will present with respiratory distress similar to Bochdalek hernia.[9]

Pathophysiology

It involves three major defects.

Morbidity and mortality

Congenital Diaphragmatic Hernia has a mortality rate of 40-62% [10], outcomes being more favorable in the absence of other congenital abnormalities. Individual rates vary greatly dependent upon multiple factors; size of hernia, organs involved, additional birth defects or genetic problems, amount of lung growth, age and size at birth, type of treatments, timing of treatments, complications such as infections and lack of lung function.

Presentation and diagnosis

This condition can often be diagnosed before birth and fetal intervention can sometimes help, depending on the severity of the condition.[11] Infants born with diaphragmatic hernia experience respiratory failure due to both pulmonary hypertension and pulmonary hypoplasia. The first condition is a restriction of blood flow through the lungs thought to be caused by defects in the lung. Pulmonary hypoplasia or decreased lung volume is directly related to the abdominal organs presence in the chest cavity which causes the lungs to be severely undersized, especially on the side of the hernia.

Survival rates for infants with this condition vary, but have generally been increasing through advances in neonatal medicine. Work has been done to correlate survival rates to ultrasound measurements of the lung volume as compared to the baby's head circumference. This figure known as the lung to head ratio (LHR).

Treatment

First step in management is orogastric tube placement and securing the airway (intubation). ECMO has been used as part of the treatment strategy at some hospitals.[12][13]

Diaphragm eventration is typically repaired thoracoscopically, by a technique called plication of the diaphragm.[14] Plication basically involves a folding of the eventrated diaphragm which is then sutured in order to “take up the slack” of the excess diaphragm tissue.

See also

References

  1. ^ Gaxiola A, Varon J, Valladolid G (April 2009). "Congenital diaphragmatic hernia: an overview of the etiology and current management". Acta Paediatrica (Oslo, Norway : 1992) 98 (4): 621–7. doi:10.1111/j.1651-2227.2008.01212.x. PMID 19154527.  
  2. ^ Migliazza L, Bellan C, Alberti D, Auriemma A, Burgio G, Locatelli G, Colombo A (September 2007). "Retrospective study of 111 cases of congenital diaphragmatic hernia treated with early high-frequency oscillatory ventilation and presurgical stabilization". Journal of Pediatric Surgery 42 (9): 1526–32. doi:10.1016/j.jpedsurg.2007.04.015. PMID 17848243.  
  3. ^ a b c 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. ^ 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.  
  5. ^ 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.  
  6. ^ Thomas GG, Clitherow NR (1977). "Herniation through the foramen of Morgagni in children". The British journal of surgery 64 (3): 215–7. doi:10.1002/bjs.1800640319. PMID 890271.  
  7. ^ Eren S, Ciriş F (2005). "Diaphragmatic hernia: diagnostic approaches with review of the literature". European journal of radiology 54 (3): 448–59. doi:10.1016/j.ejrad.2004.09.008. PMID 15899350.  
  8. ^ Thomas TV (1970). "Congenital eventration of the diaphragm". Ann. Thorac. Surg. 10 (2): 180–92. PMID 4913762.  
  9. ^ Rais-Bahrami K, Gilbert JC, Hartman GE, Chandra RS, Short BL (1996). "Right diaphragmatic eventration simulating a congenital diaphragmatic hernia". American journal of perinatology 13 (4): 241–3. doi:10.1055/s-2007-994372. PMID 8724727.  
  10. ^ http://emedicine.medscape.com/article/978118-overview
  11. ^ "Deadly hernia corrected in womb - Surgeons have developed an operation to repair a potentially fatal abnormality in babies before they are born.". BBC news. 2004-07-26.   - report of new operation, pioneered at London's King's College Hospital which reduced death rates in the most at risk by 50%
  12. ^ Tiruvoipati R, Vinogradova Y, Faulkner G, Sosnowski AW, Firmin RK, Peek GJ (2007). "Predictors of outcome in patients with congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation". J. Pediatr. Surg. 42 (8): 1345–50. doi:10.1016/j.jpedsurg.2007.03.031. PMID 17706494.  
  13. ^ Logan JW, Rice HE, Goldberg RN, Cotten CM (2007). "Congenital diaphragmatic hernia: a systematic review and summary of best-evidence practice strategies". Journal of perinatology : official journal of the California Perinatal Association 27 (9): 535–49. doi:10.1038/sj.jp.7211794. PMID 17637787.  
  14. ^ Becmeur F, Talon I, Schaarschmidt K, et al. (2005). "Thoracoscopic diaphragmatic eventration repair in children: about 10 cases". J. Pediatr. Surg. 40 (11): 1712–5. doi:10.1016/j.jpedsurg.2005.07.008. PMID 16291157.  

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