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| '''For patient information click [[{{PAGENAME}} (patient information)|here]]'''.
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| {{CMG}}
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| __NOTOC__ | | __NOTOC__ |
| '''Associate Editor-In-Chief:''' {{CZ}}
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| {{Editor Help}}
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| ==Overview==
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| {{DiseaseDisorder infobox | | | {{DiseaseDisorder infobox | |
| Name = Congenital diaphragmatic hernia | | | Name = Congenital diaphragmatic hernia | |
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| OMIM_mult = {{OMIM2|222400}} | | | OMIM_mult = {{OMIM2|222400}} | |
| MedlinePlus = 001135 | | | MedlinePlus = 001135 | |
| eMedicineSubj = ped |
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| eMedicineTopic = 2603 |
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| DiseasesDB = 32489 | | | DiseasesDB = 32489 | |
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| {{SI}} | | {{Congenital diaphragmatic hernia}} |
| '''Congenital diaphragmatic hernia''' ([[CDH]]) is a term applied to a variety of [[congenital disorder|congenital birth defects]] that involve abnormal development of the [[diaphragm (anatomy)|diaphragm]]. The term is used most commonly in reference to Bochdalek hernia, however it can also apply to '''[[Morgagni's hernia]]''', '''[[diaphragm eventration]]''' and '''[[central tendon defects]]''' of the diaphragm. A common theme in all scenarios is malformation of the diaphragm; this allows the [[abdomen|abdominal]] contents to protrude into the [[chest]] thereby impeding proper [[lung]] formation. Newborns with CDH often have severe [[respiratory distress]] which can be life-threatening unless treated appropriately.
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| ==Bochdalek Hernia==
| | '''For patient information click [[{{PAGENAME}} (patient information)|here]]'''. |
| 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.<ref>{{cite journal |author=Torfs CP, Curry CJ, Bateson TF, Honoré LH |title=A population-based study of congenital diaphragmatic hernia |journal=Teratology |volume=46 |issue=6 |pages=555–65 |year=1992 |pmid=1290156 |doi=10.1002/tera.1420460605}}</ref><ref>{{cite journal |author=Yang W, Carmichael SL, Harris JA, Shaw GM |title=Epidemiologic characteristics of congenital diaphragmatic hernia among 2.5 million California births, 1989-1997 |journal=Birth Defects Res. Part A Clin. Mol. Teratol. |volume=76 |issue=3 |pages=170–4 |year=2006 |pmid=16511883 |doi=10.1002/bdra.20230}}</ref> 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. <ref>{{cite journal |author=Torfs CP, Curry CJ, Bateson TF, Honoré LH |title=A population-based study of congenital diaphragmatic hernia |journal=Teratology |volume=46 |issue=6 |pages=555–65 |year=1992 |pmid=1290156 |doi=10.1002/tera.1420460605}}</ref><ref>{{cite journal |author=Lally KP, Lally PA, Lasky RE, ''et al'' |title=Defect size determines survival in infants with congenital diaphragmatic hernia |journal=Pediatrics |volume=120 |issue=3 |pages=e651–7 |year=2007 |pmid=17766505 |doi=10.1542/peds.2006-3040}}</ref>
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| ===Pathophysiology===
| | {{CMG}}'''; Associate Editor-In-Chief:''' [[User:AroojNaz|Arooj Naz, M.B.B.S]] |
| It involves three major defects.
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| * A failure of the [[diaphragm (anatomy)|diaphragm]] to completely close during [[Morphogenesis|development]].
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| * [[hernia|Herniation]] of the [[abdomen|abdominal]] contents into the [[chest]]
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| * [[Pulmonary]] [[Hypoplasia]]
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| ===Presentation and Diagnosis=== | | ==[[Congenital diaphragmatic hernia overview|Overview]]== |
| This condition can often be diagnosed before birth and [[fetal intervention]] can sometimes help, depending on the severity of the condition.<!--
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| --><ref>{{cite news | title=Deadly hernia corrected in womb - Surgeons have developed an operation to repair a potentially fatal abnormality in babies before they are born. | date=2004-07-26 | publisher=BBC news | accessdate=2006-07-14}} - report of new operation, pioneered at London's [[King's College Hospital]] which reduced death rates in the most at risk by 50%</ref>
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| 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.
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| 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) may be used to determine if fetal surgery may be used as an option.
| | ==[[Congenital diaphragmatic hernia historical perspective|Historical Perspective]]== |
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| ===Treatment=== | | ==[[Congenital diaphragmatic hernia classification|Classification]]== |
| [[Extracorporeal membrane oxygenation|ECMO]] has been used as part of the treatment strategy at some hospitals.<ref name="pmid17706494">{{cite journal |author=Tiruvoipati R, Vinogradova Y, Faulkner G, Sosnowski AW, Firmin RK, Peek GJ |title=Predictors of outcome in patients with congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation |journal=J. Pediatr. Surg. |volume=42 |issue=8 |pages=1345–50 |year=2007 |pmid=17706494 |doi=10.1016/j.jpedsurg.2007.03.031}}</ref><ref name="pmid17637787">{{cite journal |author=Logan JW, Rice HE, Goldberg RN, Cotten CM |title=Congenital diaphragmatic hernia: a systematic review and summary of best-evidence practice strategies |journal=Journal of perinatology : official journal of the California Perinatal Association |volume=27 |issue=9 |pages=535–49 |year=2007 |pmid=17637787 |doi=10.1038/sj.jp.7211794}}</ref> | |
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| ==Morgagni's Hernia== | | ==[[Congenital diaphragmatic hernia pathophysiology|Pathophysiology]]== |
| 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|xyphoid process]] of the [[sternum]].<ref>{{cite journal |author=Torfs CP, Curry CJ, Bateson TF, Honoré LH |title=A population-based study of congenital diaphragmatic hernia |journal=Teratology |volume=46 |issue=6 |pages=555–65 |year=1992 |pmid=1290156 |doi=10.1002/tera.1420460605}}</ref> 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.<ref>{{cite journal |author=Thomas GG, Clitherow NR |title=Herniation through the foramen of Morgagni in children |journal=The British journal of surgery |volume=64 |issue=3 |pages=215–7 |year=1977 |pmid=890271 |doi=}}</ref><ref>{{cite journal |author=Eren S, Ciriş F |title=Diaphragmatic hernia: diagnostic approaches with review of the literature |journal=European journal of radiology |volume=54 |issue=3 |pages=448–59 |year=2005 |pmid=15899350 |doi=10.1016/j.ejrad.2004.09.008}}</ref> In asymptomatic individuals laparoscopic surgical repair is still recommended as they are at risk of a strangulated intestine.
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| <div align="center">
| | ==[[Congenital diaphragmatic hernia causes|Causes]]== |
| <gallery heights="175" widths="175">
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| Image:Morgagni-hernia-001.jpg|Mass adjacent to heart seen on routine pre-employment chest x-ray
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| Image:Morgagni-hernia-002.jpg|CT Morgagni's Hernia
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| Image:Morgagni-hernia-003.jpg|CT Morgagni's Hernia
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| </gallery>
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| </div>
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| ==Diaphragm Eventration== | | ==[[Congenital diaphragmatic hernia differential diagnosis|Differentiating Congenital diaphragmatic hernia from other Diseases]]== |
| 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. <ref>{{cite journal |author=Thomas TV |title=Congenital eventration of the diaphragm |journal=Ann. Thorac. Surg. |volume=10 |issue=2 |pages=180–92 |year=1970 |pmid=4913762 |doi=}}</ref> Minor forms of diaphragm eventration are asymptomatic, however in severe cases infants will present with respiratory distress similar to Bochdalek hernia.<ref>{{cite journal |author=Rais-Bahrami K, Gilbert JC, Hartman GE, Chandra RS, Short BL |title=Right diaphragmatic eventration simulating a congenital diaphragmatic hernia |journal=American journal of perinatology |volume=13 |issue=4 |pages=241–3 |year=1996 |pmid=8724727 |doi=}}</ref> Diaphragm eventration is typically repaired thoracoscopically, by a technique called plication of the diaphragm. <ref>{{cite journal |author=Becmeur F, Talon I, Schaarschmidt K, ''et al'' |title=Thoracoscopic diaphragmatic eventration repair in children: about 10 cases |journal=J. Pediatr. Surg. |volume=40 |issue=11 |pages=1712–5 |year=2005 |pmid=16291157 |doi=10.1016/j.jpedsurg.2005.07.008}}</ref> 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.
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| ==References== | | ==[[Congenital diaphragmatic hernia epidemiology and demographics|Epidemiology and Demographics]]== |
| {{Reflist|2}}
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| == External links == | | ==[[Congenital diaphragmatic hernia risk factors|Risk factors]]== |
| *[http://www.fetalcarecenter.org/medicine/fetal-surgery/diaphragmatic-hernia.htm Fetal Care Center of Cincinnati: Fetal Surgery for Congenital Diaphrahmatic Hernia / CDH]
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| *[http://www.cherubs-cdh.org/ CHERUBS - The Association of Congenital Diaphragmatic Hernia Research, Advocacy, and Support]
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| *[http://www.breathofhopeinc.com Breath of Hope, Inc.]
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| *[http://realhopeforcdh.com/ Real Hope for CDH: Hope and Support for parents facing CDH]
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| *[http://tinawest.com/ Hope, Patience, Trust: The Journey of having a baby with CDH]
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| {{SIB}}
| | ==[[Congenital diaphragmatic hernia screening|Screening]]== |
| {{Congenital malformations and deformations of musculoskeletal system}}
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| {{Gastroenterology}}
| | ==[[Congenital diaphragmatic hernia natural history, complications and prognosis|Natural History, Complications & Prognosis]]== |
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| | ==Diagnosis== |
| | [[Congenital diaphragmatic hernia history and symptoms| History and Symptoms]] | [[Congenital diaphragmatic hernia physical examination | Physical Examination]] | [[Congenital diaphragmatic hernia laboratory findings|Laboratory Findings]] | [[Congenital diaphragmatic hernia chest x ray|Chest X Ray]] | [[Congenital diaphragmatic hernia CT|CT]] | [[Congenital diaphragmatic hernia MRI|MRI]] | [[Congenital diaphragmatic hernia other imaging findings|Other Imaging Findings]] | [[Congenital diaphragmatic hernia other diagnostic studies|Other Diagnostic Studies]] |
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| | ==Treatment== |
| | [[Congenital diaphragmatic hernia medical therapy|Medical Therapy]] | [[Congenital diaphragmatic hernia surgery|Surgery]] | [[Congenital diaphragmatic hernia primary prevention|Primary Prevention]] | [[Congenital diaphragmatic hernia secondary prevention|Secondary Prevention]] | [[Congenital diaphragmatic hernia cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Congenital diaphragmatic hernia future or investigational therapies|Future or Investigational Therapies]] |
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| | ==Case Studies== |
| | [[Congenital diaphragmatic hernia case study one|Case#1]] |
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| [[Category:Congenital disorders]]
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| [[Category:Diseases involving the fasciae]]
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| [[Category:Developmental biology]]
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| [[Category:Gastroenterology]] | | [[Category:Gastroenterology]] |
| | | [[Category:Disease]] |
| [[de:Zwerchfellhernie]] | |
| [[fr:Hernie diaphragmatique congénitale]]
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| [[zh:先天性橫膈疝氣]]
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