Diaphragmatic hernia: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{DiseaseDisorder infobox | | {{DiseaseDisorder infobox | | ||
Name = {{PAGENAME}} | Name = {{PAGENAME}}| | ||
Image = Peritoneopericardial diaphragmatic hernia.JPG | | Image = Peritoneopericardial diaphragmatic hernia.JPG | | ||
Caption = | Caption = By Joel Mills - Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=946353 | | ||
OMIM = | | OMIM = | | ||
}} | }} | ||
{{ | |||
{{Diaphragmatic hernia}} | |||
{{CMG}};{{AE}} {{AY}} {{MAD}} | |||
{{SK}} Congenital diaphragm hernia, Diaphragm hernia, Hernia through the diaphragm. | |||
==Overview== | ==Overview== | ||
Diaphragmatic hernia is a congenital anomaly that occurs due to failure of the diaphragm to close leading to herniation of the abdominal content into the thoracic cavity leading to pulmonary hypoplasia. It most commonly presents in the neonates as respiratory distress. It is classified according to the site of herniation into anterior, posterolateral, and hiatal diaphragmatic hernia. It can cause complications in both the GIT and the cardiopulmonary circulation. It can be diagnosed antenatally in the week 24 and after birth, it is apparent on chest x-ray. The mainstay of treatment is surgical correction which must be done when the infant is completely stabilized. | Diaphragmatic hernia is a congenital anomaly that occurs due to failure of the [[diaphragm]] to close leading to herniation of the abdominal content into the [[thoracic cavity]] leading to pulmonary hypoplasia. It most commonly presents in the neonates as [[respiratory distress]]. It is classified according to the site of herniation into [[Morgagni's hernia|anterior]], [[Bochdalek hernia|posterolateral]], and [[Hiatus hernia|hiatal diaphragmatic hernia]]. It can cause complications in both the GIT and the cardiopulmonary circulation. It can be diagnosed antenatally in the week 24 and after birth, it is apparent on chest x-ray. The mainstay of treatment is surgical correction which must be done when the infant is completely stabilized. | ||
==Historical Perspective== | ==Historical Perspective== | ||
*In 1679, the first case of congenital diaphragmatic hernia was reported by Riverius. | *In 1679, the first case of congenital diaphragmatic hernia was reported by Riverius.<ref name="pmid8982561">{{cite journal| author=Irish MS, Holm BA, Glick PL| title=Congenital diaphragmatic hernia. A historical review. | journal=Clin Perinatol | year= 1996 | volume= 23 | issue= 4 | pages= 625-53 | pmid=8982561 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8982561 }}</ref> | ||
*In 1888, the first procedure to repair congenital diaphragmatic hernia in an adult was done and in 1889, the first procedure on an infant was done. | *In 1888, the first procedure to repair [[congenital]] diaphragmatic hernia in an adult was done and in 1889, the first procedure on an infant was done. | ||
*In 1905, the first successful operation was done but the success rate remained below 60% until 1925. | *In 1905, the first successful operation was done but the success rate remained below 60% until 1925.<ref name="pmid89825612">{{cite journal| author=Irish MS, Holm BA, Glick PL| title=Congenital diaphragmatic hernia. A historical review. | journal=Clin Perinatol | year= 1996 | volume= 23 | issue= 4 | pages= 625-53 | pmid=8982561 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8982561 }}</ref> | ||
*In 1940, A set of diagnostic criteria for diaphragmatic hernia were suggested. | *In 1940, A set of diagnostic criteria for diaphragmatic hernia were suggested. | ||
*In 1950, the transthoracic approach was suggested instead of the transabdominal approach. | *In 1950, the transthoracic approach was suggested instead of the transabdominal approach. | ||
==Classification== | ==Classification== | ||
Congenital diaphragmatic hernia can be classified into three types according to the site of herniation. | Congenital diaphragmatic hernia can be classified into three types according to the site of [[herniation]].<ref name="pmid24580764">{{cite journal |vauthors=Badillo A, Gingalewski C |title=Congenital diaphragmatic hernia: treatment and outcomes |journal=Semin. Perinatol. |volume=38 |issue=2 |pages=92–6 |year=2014 |pmid=24580764 |doi=10.1053/j.semperi.2013.11.005 |url=}}</ref><ref name="pmid20112629">{{cite journal |vauthors=Schumacher L, Gilbert S |title=Congenital diaphragmatic hernia in the adult |journal=Thorac Surg Clin |volume=19 |issue=4 |pages=469–72 |year=2009 |pmid=20112629 |doi=10.1016/j.thorsurg.2009.08.004 |url=}}</ref> | ||
===Posterolateral (Bochdalek) diaphragmatic hernia=== | ===Posterolateral (Bochdalek) diaphragmatic hernia=== | ||
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===Anterior (Morgagni) diaphragmatic hernia=== | ===Anterior (Morgagni) diaphragmatic hernia=== | ||
*In anterior diaphragmatic hernia, the intestine bulges into the thorax through the anterior midline. | *In anterior diaphragmatic hernia, the intestine bulges into the [[thorax]] through the anterior midline. | ||
*Most of the cases of anterior diaphragmatic hernia occur on the right side. | *Most of the cases of anterior diaphragmatic hernia occur on the right side. | ||
===Hiatal hernia=== | ===Hiatal hernia=== | ||
*The intestine finds its way to the thorax through the esophageal hiatus. | *The [[intestine]] finds its way to the thorax through the [[esophageal hiatus]]. | ||
*More common in the adults than in | *More common in the adults than in [[neonates]]. | ||
==Pathophysiology== | ==Pathophysiology and embryogenesis== | ||
*The herniated viscera decrease the area needed by the lung to develop appropriately. | * Hernia starts during the [[lung]] development, from the third week to the 16<sup>th</sup> week of [[gestation]]. Interference with lung development leads to pulmonary [[hypoplasia]] and hypoplasia of [[cardiac]] structures.<ref name="pmid7472945">{{cite journal| author=DiFiore JW, Fauza DO, Slavin R, Wilson JM| title=Experimental fetal tracheal ligation and congenital diaphragmatic hernia: a pulmonary vascular morphometric analysis. | journal=J Pediatr Surg | year= 1995 | volume= 30 | issue= 7 | pages= 917-23; discussion 923-4 | pmid=7472945 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7472945 }}</ref> | ||
* It is due to failure of normal closure of the [[Pleuroperitoneal|pleuroperitoneal folds]] during the 4<sup>th</sup> to 10<sup>th</sup> weeks.<ref name="pmid17558598">{{cite journal| author=Bielinska M, Jay PY, Erlich JM, Mannisto S, Urban Z, Heikinheimo M et al.| title=Molecular genetics of congenital diaphragmatic defects. | journal=Ann Med | year= 2007 | volume= 39 | issue= 4 | pages= 261-74 | pmid=17558598 | doi=10.1080/07853890701326883 | pmc=2174621 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17558598 }}</ref> | |||
* The vast majority of congenital diaphragmatic hernias occurs sporadically. However, familial cases involving [[autosomal recessive]], [[autosomal dominant]], and [[X-linked]] inheritance patterns have been reported.<ref name="pmid3701555">{{cite journal| author=Mishalany H, Gordo J| title=Congenital diaphragmatic hernia in monozygotic twins. | journal=J Pediatr Surg | year= 1986 | volume= 21 | issue= 4 | pages= 372-4 | pmid=3701555 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3701555 }}</ref> | |||
* Many different [[genetic defects]] (eg, [[aneuploidies]], [[Deletion (genetics)|deletions]], duplications, [[translocations]]) have been identified among sporadic cases.<ref name="pmid12725592">{{cite journal| author=Lurie IW| title=Where to look for the genes related to diaphragmatic hernia? | journal=Genet Couns | year= 2003 | volume= 14 | issue= 1 | pages= 75-93 | pmid=12725592 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12725592 }}</ref> | |||
*The herniated [[viscera]] decrease the area needed by the lung to develop appropriately.<ref name="pmid8982563">{{cite journal |vauthors=Langham MR, Kays DW, Ledbetter DJ, Frentzen B, Sanford LL, Richards DS |title=Congenital diaphragmatic hernia. Epidemiology and outcome |journal=Clin Perinatol |volume=23 |issue=4 |pages=671–88 |year=1996 |pmid=8982563 |doi= |url=}}</ref> | |||
*According to the degree of herniation, the degree of pulmonary hypoplasia is determined. | *According to the degree of herniation, the degree of pulmonary hypoplasia is determined. | ||
*The herniated viscera impairs the development of the bronchial tree. | *The herniated [[viscera]] impairs the development of the [[bronchial tree]]. | ||
*As a consequence, the alveoli development is also slowed. | *As a consequence, the [[alveoli]] development is also slowed.<ref name="pmid20610190">{{cite journal |vauthors=Keijzer R, Puri P |title=Congenital diaphragmatic hernia |journal=Semin. Pediatr. Surg. |volume=19 |issue=3 |pages=180–5 |year=2010 |pmid=20610190 |doi=10.1053/j.sempedsurg.2010.03.001 |url=}}</ref> | ||
*The development of the pulmonary artery tree is halted too resulting in excessive masculinization of the arteries. | *The development of the pulmonary artery tree is halted too resulting in excessive [[masculinization]] of the arteries. | ||
*The increased pulmonary pressure leads to increased pressure on the right ventricle leading to the development of a right to left shunt | *The increased pulmonary pressure leads to increased pressure on the [[right ventricle]] leading to the development of a [[right to left shunt]]. | ||
*As most of the cases of diaphragmatic hernias are unilateral, the pulmonary hypoplasia is also usually unilateral, but it can be bilateral if the [[mediastinum]] is pushed by a massive unilateral hernia. | |||
*As most of the cases of diaphragmatic | *The abnormal alveolar development can lead to [[hypoxemia]] leading to pulmonary [[vasoconstriction]] which aggravates the condition. | ||
*The abnormal alveolar development can lead to hypoxemia leading to pulmonary vasoconstriction which aggravates the condition. | |||
==Risk factors== | ==Risk factors== | ||
*Male gender | Risk factors of diaphragmatic hernia include:<ref name="pmid3904042">{{cite journal |vauthors=Cullen ML, Klein MD, Philippart AI |title=Congenital diaphragmatic hernia |journal=Surg. Clin. North Am. |volume=65 |issue=5 |pages=1115–38 |year=1985 |pmid=3904042 |doi= |url=}}</ref> | ||
*Premature birth | *[[Male]] gender | ||
*Macrosomia | *[[Premature]] birth | ||
*Low birth weight | *[[Macrosomia]] | ||
*[[Low birth weight]] | |||
*[[Vitamin A deficiency]]<ref name="pmid18321479">{{cite journal| author=See AW, Kaiser ME, White JC, Clagett-Dame M| title=A nutritional model of late embryonic vitamin A deficiency produces defects in organogenesis at a high penetrance and reveals new roles for the vitamin in skeletal development. | journal=Dev Biol | year= 2008 | volume= 316 | issue= 2 | pages= 171-90 | pmid=18321479 | doi=10.1016/j.ydbio.2007.10.018 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18321479 }}</ref> | |||
*Exposure to thalidomide | |||
*Exposure to [[anticonvulsants]] and [[quinine]]<ref name="pmid9788565">{{cite journal| author=Enns GM, Cox VA, Goldstein RB, Gibbs DL, Harrison MR, Golabi M| title=Congenital diaphragmatic defects and associated syndromes, malformations, and chromosome anomalies: a retrospective study of 60 patients and literature review. | journal=Am J Med Genet | year= 1998 | volume= 79 | issue= 3 | pages= 215-25 | pmid=9788565 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9788565 }}</ref> | |||
==Differentiating Diaphragmatic hernia from other Diseases== | ==Differentiating Diaphragmatic hernia from other Diseases== | ||
Congenital diaphragmatic hernia may be differentiated from other thoracic lesions:<ref name="pmid127255922">{{cite journal| author=Lurie IW| title=Where to look for the genes related to diaphragmatic hernia? | journal=Genet Couns | year= 2003 | volume= 14 | issue= 1 | pages= 75-93 | pmid=12725592 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12725592 }}</ref> | |||
* [[Congenital cystic adenomatoid malformation]] | |||
* [[Pulmonary sequestration|Bronchopulmonary sequestration]] | |||
* Bronchopulmonary foregut malformation | |||
* [[Bronchogenic cyst|Bronchogenic cysts]] | |||
* [[Bronchial atresia]] | |||
* Enteric cysts | |||
* [[Teratoma|Teratomas]] | |||
Congenital diaphragmatic hernia may be differentiated from other causes of [[pulmonary hypertension]]: | |||
* Neonatal [[pneumonia]] | |||
* [[Transient tachypnea of the newborn]] | |||
* [[Respiratory distress syndrome]] | |||
* [[Sepsis]] | |||
==Epidemiology== | ==Epidemiology== | ||
===Incidence=== | ===Incidence=== | ||
*The incidence of congenital diaphragmatic hernia ranges from 50-150 per 100.000 live births.<ref name="pmid19524735">{{cite journal |vauthors=Fisher JC, Haley MJ, Ruiz-Elizalde A, Stolar CJ, Arkovitz MS |title=Multivariate model for predicting recurrence in congenital diaphragmatic hernia |journal=J. Pediatr. Surg. |volume=44 |issue=6 |pages=1173–9; discussion 1179–80 |year=2009 |pmid=19524735 |pmc=3072822 |doi=10.1016/j.jpedsurg.2009.02.043 |url=}}</ref> | *The [[incidence]] of congenital diaphragmatic hernia ranges from 50-150 per 100.000 live births.<ref name="pmid19524735">{{cite journal |vauthors=Fisher JC, Haley MJ, Ruiz-Elizalde A, Stolar CJ, Arkovitz MS |title=Multivariate model for predicting recurrence in congenital diaphragmatic hernia |journal=J. Pediatr. Surg. |volume=44 |issue=6 |pages=1173–9; discussion 1179–80 |year=2009 |pmid=19524735 |pmc=3072822 |doi=10.1016/j.jpedsurg.2009.02.043 |url=}}</ref> | ||
===Prevalence=== | ===Prevalence=== | ||
*The prevalence of congenital diaphragmatic hernia ranges from 10 to 40 per live births.<ref name="pmid25411443">{{cite journal |vauthors=McGivern MR, Best KE, Rankin J, Wellesley D, Greenlees R, Addor MC, Arriola L, de Walle H, Barisic I, Beres J, Bianchi F, Calzolari E, Doray B, Draper ES, Garne E, Gatt M, Haeusler M, Khoshnood B, Klungsoyr K, Latos-Bielenska A, O'Mahony M, Braz P, McDonnell B, Mullaney C, Nelen V, Queisser-Luft A, Randrianaivo H, Rissmann A, Rounding C, Sipek A, Thompson R, Tucker D, Wertelecki W, Martos C |title=Epidemiology of congenital diaphragmatic hernia in Europe: a register-based study |journal=Arch. Dis. Child. Fetal Neonatal Ed. |volume=100 |issue=2 |pages=F137–44 |year=2015 |pmid=25411443 |doi=10.1136/archdischild-2014-306174 |url=}}</ref> | *The [[prevalence]] of congenital diaphragmatic hernia ranges from 10 to 40 per live births.<ref name="pmid25411443">{{cite journal |vauthors=McGivern MR, Best KE, Rankin J, Wellesley D, Greenlees R, Addor MC, Arriola L, de Walle H, Barisic I, Beres J, Bianchi F, Calzolari E, Doray B, Draper ES, Garne E, Gatt M, Haeusler M, Khoshnood B, Klungsoyr K, Latos-Bielenska A, O'Mahony M, Braz P, McDonnell B, Mullaney C, Nelen V, Queisser-Luft A, Randrianaivo H, Rissmann A, Rounding C, Sipek A, Thompson R, Tucker D, Wertelecki W, Martos C |title=Epidemiology of congenital diaphragmatic hernia in Europe: a register-based study |journal=Arch. Dis. Child. Fetal Neonatal Ed. |volume=100 |issue=2 |pages=F137–44 |year=2015 |pmid=25411443 |doi=10.1136/archdischild-2014-306174 |url=}}</ref> | ||
===Sex=== | ===Sex=== | ||
*Congenital diaphragmatic hernia has no sex predilection.<ref name="pmid25411443">{{cite journal |vauthors=McGivern MR, Best KE, Rankin J, Wellesley D, Greenlees R, Addor MC, Arriola L, de Walle H, Barisic I, Beres J, Bianchi F, Calzolari E, Doray B, Draper ES, Garne E, Gatt M, Haeusler M, Khoshnood B, Klungsoyr K, Latos-Bielenska A, O'Mahony M, Braz P, McDonnell B, Mullaney C, Nelen V, Queisser-Luft A, Randrianaivo H, Rissmann A, Rounding C, Sipek A, Thompson R, Tucker D, Wertelecki W, Martos C |title=Epidemiology of congenital diaphragmatic hernia in Europe: a register-based study |journal=Arch. Dis. Child. Fetal Neonatal Ed. |volume=100 |issue=2 |pages=F137–44 |year=2015 |pmid=25411443 |doi=10.1136/archdischild-2014-306174 |url=}}</ref> | *Congenital diaphragmatic hernia has no sex predilection.<ref name="pmid25411443">{{cite journal |vauthors=McGivern MR, Best KE, Rankin J, Wellesley D, Greenlees R, Addor MC, Arriola L, de Walle H, Barisic I, Beres J, Bianchi F, Calzolari E, Doray B, Draper ES, Garne E, Gatt M, Haeusler M, Khoshnood B, Klungsoyr K, Latos-Bielenska A, O'Mahony M, Braz P, McDonnell B, Mullaney C, Nelen V, Queisser-Luft A, Randrianaivo H, Rissmann A, Rounding C, Sipek A, Thompson R, Tucker D, Wertelecki W, Martos C |title=Epidemiology of congenital diaphragmatic hernia in Europe: a register-based study |journal=Arch. Dis. Child. Fetal Neonatal Ed. |volume=100 |issue=2 |pages=F137–44 |year=2015 |pmid=25411443 |doi=10.1136/archdischild-2014-306174 |url=}}</ref> | ||
===Age=== | ===Age=== | ||
*90% of the congenital diaphragmatic hernias present in the neonatal period. | *90% of the congenital diaphragmatic hernias present in the neonatal period. | ||
==Screening== | ==Screening== | ||
* According to [[USPSTF]], there is no screening recommended for diaphragmatic hernia. The recurrence risk for multiple congenital anomalies of unknown etiology is considered to be <5 percent. [41] | |||
* Referral to a genetic counselor can be useful for thorough genetic evaluation and counseling. | |||
* [[Microarray]]-based comparative [[Hybridization|genomic hybridization]] is a powerful tool for detection of submicroscopic [[Chromosome abnormality|chromosomal abnormalities]]. | |||
==Natural history, complications and prognosis== | ==Natural history, complications and prognosis== | ||
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===Natural history=== | ===Natural history=== | ||
*90% of the cases of congenital diaphragmatic hernia presents in the neonatal period. | *90% of the cases of congenital diaphragmatic hernia presents in the neonatal period. | ||
*If left untreated, congenital diaphragmatic hernia can be fatal due to pulmonary hypoplasia and severe pulmonary hypertension. | *If left untreated, congenital diaphragmatic hernia can be fatal due to pulmonary hypoplasia and severe [[pulmonary hypertension]]. | ||
===Complications=== | ===Complications=== | ||
====GI complications==== | ====GI complications<ref name="pmid22214468">{{cite journal |vauthors=Tovar JA |title=Congenital diaphragmatic hernia |journal=Orphanet J Rare Dis |volume=7 |issue= |pages=1 |year=2012 |pmid=22214468 |pmc=3261088 |doi=10.1186/1750-1172-7-1 |url=}}</ref>==== | ||
*Abnormal developmental rotation of the midgut | *Abnormal developmental [[Midgut|rotation of the midgut]] | ||
*Midgut volvulus | *[[Midgut volvulus]] | ||
*Gastric volvulus | *[[Gastric volvulus]] | ||
====Cardiopulmonary complications==== | ====Cardiopulmonary complications<ref name="pmid22214468">{{cite journal |vauthors=Tovar JA |title=Congenital diaphragmatic hernia |journal=Orphanet J Rare Dis |volume=7 |issue= |pages=1 |year=2012 |pmid=22214468 |pmc=3261088 |doi=10.1186/1750-1172-7-1 |url=}}</ref>==== | ||
*Pulmonary hypoplasia | *Pulmonary hypoplasia | ||
*Pulmonary artery hypertension | *[[Pulmonary hypertension|Pulmonary artery hypertension]] | ||
*In severe cases, ventricular hypoplasia | *In severe cases, ventricular hypoplasia | ||
===Prognosis=== | ===Prognosis=== | ||
*The prognosis is mainly dependent on the size of the defect and the degree of the herniation. | *The prognosis is mainly dependent on the size of the defect and the degree of the herniation.<ref name="pmid28331629">{{cite journal |vauthors=Chandrasekharan PK, Rawat M, Madappa R, Rothstein DH, Lakshminrusimha S |title=Congenital Diaphragmatic hernia - a review |journal=Matern Health Neonatol Perinatol |volume=3 |issue= |pages=6 |year=2017 |pmid=28331629 |pmc=5356475 |doi=10.1186/s40748-017-0045-1 |url=}}</ref> | ||
*The presence or absence of liver herniation on fetal MRI is the most reliable prediction of the postnatal survival. | *The presence or absence of liver herniation on [[MRI|fetal MRI]] is the most reliable prediction of the postnatal survival. | ||
*The estimation of fetal lung volume and lung area to head circumference is a useful indicator of the prognosis of the disease in the absence of liver herniation. | *The estimation of fetal [[lung volume]] and lung area to head circumference is a useful indicator of the prognosis of the disease in the absence of liver herniation. | ||
*The mortality ranges from 25-60% of the cases. | *The [[mortality]] ranges from 25-60% of the cases. | ||
==History and Symptoms== | ==History and Symptoms== | ||
CDH most often presents in the neonatal period as: | CDH most often presents in the neonatal period as:<ref name="pmid22034651">{{cite journal |vauthors=Kotecha S, Barbato A, Bush A, Claus F, Davenport M, Delacourt C, Deprest J, Eber E, Frenckner B, Greenough A, Nicholson AG, Antón-Pacheco JL, Midulla F |title=Congenital diaphragmatic hernia |journal=Eur. Respir. J. |volume=39 |issue=4 |pages=820–9 |year=2012 |pmid=22034651 |doi=10.1183/09031936.00066511 |url=}}</ref> | ||
*[[Cyanosis]] | *[[Cyanosis]] | ||
* [[Tachypnea|Rapid breathing (tachypnea)]] | * [[Tachypnea|Rapid breathing (tachypnea)]] | ||
* [[Dyspnea|Shortness of breath (dyspnea)]] | * [[Dyspnea|Shortness of breath (dyspnea)]] | ||
* [[Labored breathing|Increased work of breathing (labored breathing)]] | * [[Labored breathing|Increased work of breathing (labored breathing)]]<ref name="pmid20512059">{{cite journal |vauthors=Peetsold MG, Kneepkens CM, Heij HA, IJsselstijn H, Tibboel D, Gemke RJ |title=Congenital diaphragmatic hernia: long-term risk of gastroesophageal reflux disease |journal=J. Pediatr. Gastroenterol. Nutr. |volume=51 |issue=4 |pages=448–53 |year=2010 |pmid=20512059 |doi=10.1097/MPG.0b013e3181d1b149 |url=}}</ref> | ||
*[[Lethargy]] | *[[Lethargy]] | ||
*Intolerance to feeding due to inadequate oxygenation. | *Intolerance to feeding due to inadequate oxygenation. | ||
*[[Adrenal insufficiency]]: it is a common association with congenital diaphragmatic hernia. | *[[Adrenal insufficiency]]: it is a common association with congenital diaphragmatic hernia. | ||
{{#ev:youtube|jPpGHVkB54A}} | |||
==Physical Examination== | ==Physical Examination== | ||
===General appearance=== | ===General appearance=== | ||
*A neonate with CDH usually appears cyanotic and in acute distress. | *A neonate with CDH usually appears [[cyanotic]] and in acute distress.<ref name="pmid23378478">{{cite journal |vauthors=Haroon J, Chamberlain RS |title=An evidence-based review of the current treatment of congenital diaphragmatic hernia |journal=Clin Pediatr (Phila) |volume=52 |issue=2 |pages=115–24 |year=2013 |pmid=23378478 |doi=10.1177/0009922812472249 |url=}}</ref> | ||
===Vital signs=== | ===Vital signs=== | ||
*Heart rate: tachycardia | *Heart rate: [[tachycardia]] | ||
*Respiratory rate: tachypnea | *Respiratory rate: [[tachypnea]] | ||
*Blood pressure: The infant may have hypotension due to compression of the ventricles | *Blood pressure: The infant may have [[hypotension]] due to compression of the ventricles | ||
===Lungs=== | ===Lungs=== | ||
*The chest may have a barrel shape. | *The [[chest]] may have a barrel shape. | ||
*Absent breath sounds at the side of the hernia | *Absent [[breath sounds]] at the side of the hernia<ref name="pmid8176602">{{cite journal |vauthors=Lund DP, Mitchell J, Kharasch V, Quigley S, Kuehn M, Wilson JM |title=Congenital diaphragmatic hernia: the hidden morbidity |journal=J. Pediatr. Surg. |volume=29 |issue=2 |pages=258–62; discussion 262–4 |year=1994 |pmid=8176602 |doi= |url=}}</ref> | ||
*Peristaltic sounds in the chest | *Peristaltic sounds in the chest | ||
===Heart=== | ===Heart=== | ||
*The heart sounds may be displaced to the other side of the chest. | *The [[heart sounds]] may be displaced to the other side of the chest. | ||
*The heart sounds may reveal the murmurs of associated cardiac anomalies. | *The heart sounds may reveal the [[murmurs]] of associated cardiac anomalies. | ||
===Abdomen=== | ===Abdomen=== | ||
*The abdomen is scaphoid due to herniation of the abdominal content into the thoracic cavity. | *The abdomen is [[scaphoid]] due to herniation of the abdominal content into the thoracic cavity. | ||
==Laboratory Findings== | ==Laboratory Findings== | ||
*Arterial blood gas: ABG may show hypoxemia, metabolic acidosis, and hypercapnia. | ===Antenatal tests=== | ||
*The infant should be investigated for the presence of associated chromosomal abnormalities. | *[[Amniocentesis]]: [[Karyotype|karyotyping]] should be obtained to rule out associated [[chromosomal abnormalities]]. | ||
*[[Alpha-fetoprotein|Maternal alpha-fetoprotein]] is decreased in CDH<ref name="pmid26403792">{{cite journal |vauthors=McHoney M |title=Congenital diaphragmatic hernia, management in the newborn |journal=Pediatr. Surg. Int. |volume=31 |issue=11 |pages=1005–13 |year=2015 |pmid=26403792 |doi=10.1007/s00383-015-3794-9 |url=}}</ref> | |||
===Postnatal tests=== | |||
*[[Arterial blood gas]]: ABG may show [[hypoxemia]], [[metabolic acidosis]], and [[hypercapnia]]. | |||
*The infant should be investigated for the presence of associated [[chromosomal abnormalities]].<ref name="pmid1444648">{{cite journal |vauthors=Lally KP, Paranka MS, Roden J, Georgeson KE, Wilson JM, Lillehei CW, Breaux CW, Poon M, Clark RH, Atkinson JB |title=Congenital diaphragmatic hernia. Stabilization and repair on ECMO |journal=Ann. Surg. |volume=216 |issue=5 |pages=569–73 |year=1992 |pmid=1444648 |pmc=1242675 |doi= |url=}}</ref> | |||
==Radiological tests== | ==Radiological tests== | ||
===Antenatal ultrasound=== | ===Antenatal ultrasound=== | ||
*CDH can be diagnosed in the 24th week. | *CDH can be diagnosed in the 24th week.<ref name="pmid19019293">{{cite journal |vauthors=Waag KL, Loff S, Zahn K, Ali M, Hien S, Kratz M, Neff W, Schaffelder R, Schaible T |title=Congenital diaphragmatic hernia: a modern day approach |journal=Semin. Pediatr. Surg. |volume=17 |issue=4 |pages=244–54 |year=2008 |pmid=19019293 |doi=10.1053/j.sempedsurg.2008.07.009 |url=}}</ref> | ||
*In addition to the hernia, ultrasound can show polyhydramnios and absence of the gastric air bubble | *In addition to the hernia, ultrasound can show [[polyhydramnios]] and absence of the gastric air bubble | ||
*Left sided CDH is far more common than right sided CDH, while bilateral CDH is rare. | *Left sided CDH is far more common than right sided CDH, while bilateral CDH is rare. | ||
*The presence of liver herniation is associated with worse prognosis and survival. | *The presence of liver herniation is associated with worse prognosis and survival. | ||
*The estimation of lung area to head circumference ratio can give an idea about the prognosis (especially if there is no liver herniation). The lower the ratio, the worse the prognosis. | *The estimation of lung area to head circumference ratio can give an idea about the prognosis (especially if there is no liver herniation). The lower the ratio, the worse the prognosis. | ||
[[Image:Congenital-diaphragmatic-hernia-25.jpg|thumb|300px|center|Coronal ultrasound showing herniation of the abdominal content into the thorax and displacement of the herat to the right - Case courtesy of Dr Laughlin Dawes , Radiopaedia.org, rID: 36006]] | |||
===Chest x-ray=== | ===Chest x-ray=== | ||
*Bowel loops in the thoracic cavity (can be further demonstrated through placement of a feeding tube) | *Bowel loops in the thoracic cavity (can be further demonstrated through placement of a feeding tube) | ||
*Shifting of the mediastinum to the contralateral side | *Shifting of the [[mediastinum]] to the contralateral side | ||
[[Image:Congenital-diaphragmatic-hernia-2.jpg|thumb|center|Anteroposterior x-ray showing herniation of the intestine into the thorax and shifting of the mediastinum to the right - Case courtesy of Dr Mohd Imran, Radiopaedia.org, rID: 7392]] | |||
===Echocardiography=== | ===Echocardiography=== | ||
*Echocardiography must be done to exclude associated cardiac anomalies and to exclude the presence of an associated ventricular strain from the pressure. | *[[Echocardiography]] must be done to exclude associated cardiac anomalies and to exclude the presence of an associated ventricular strain from the pressure. | ||
==Medical treatment== | ==Medical treatment== | ||
'''Criteria for mechanical ventilation:''' | |||
* Pressure-limited ventilation at rates of 30 to 70 breaths per minute | |||
* Positive end-expiratory pressure (PEEP) should be maintained at physiologic levels (3 to 5 cm H<sub>2</sub>O) whenever possible. | |||
* High-frequency ventilation (HFV) is reserved for neonates who continue to have hypoxia and hypercarbia (PaCO<sub>2</sub> >65 mmHg) refractory to CMV. | |||
===Antenatal glucocorticoids=== | ===Antenatal glucocorticoids=== | ||
Antenatal glucocorticoids improve the survival by increasing the secretion of surfactant and thus stimulating lung maturation and preventing severe pulmonary hypoplasia. | [[Glucocorticoids|Antenatal glucocorticoids]] improve the survival by increasing the secretion of [[surfactant]] and thus stimulating lung maturation and preventing severe pulmonary hypoplasia.<ref name="pmid24580764">{{cite journal |vauthors=Badillo A, Gingalewski C |title=Congenital diaphragmatic hernia: treatment and outcomes |journal=Semin. Perinatol. |volume=38 |issue=2 |pages=92–6 |year=2014 |pmid=24580764 |doi=10.1053/j.semperi.2013.11.005 |url=}}</ref> | ||
===Preoperative stabilization=== | |||
*Preoperative stabilization of the patient is important as performing the operation in an unstable infant is associated with higher [[morbidity]] and [[mortality]] rates. | |||
Preoperative stabilization measures include:<ref name="pmid22229104">{{cite journal |vauthors=Kesieme EB, Kesieme CN |title=Congenital diaphragmatic hernia: review of current concept in surgical management |journal=ISRN Surg |volume=2011 |issue= |pages=974041 |year=2011 |pmid=22229104 |pmc=3251163 |doi=10.5402/2011/974041 |url=}}</ref> | |||
*[[Mechanical ventilation]]: The aim is to ensure proper oxygenation without causing any [[barotrauma]] to the lung. Peak inspiratory pressure should be always less than 30 cm H2O | |||
*[[Nasogastric tube|Nasogastric tube insertion]]: The aim is to decompress the [[bowel]] and to give more space for the lung | |||
*[[Blood pressure]] support: using [[vasopressors]] or isotonic fluid: The aim is to keep the [[Mean arterial pressure|mean blood pressure]] above 50 mmHg.<ref name="pmid22229104">{{cite journal |vauthors=Kesieme EB, Kesieme CN |title=Congenital diaphragmatic hernia: review of current concept in surgical management |journal=ISRN Surg |volume=2011 |issue= |pages=974041 |year=2011 |pmid=22229104 |pmc=3251163 |doi=10.5402/2011/974041 |url=}}</ref> | |||
*High-frequency oscillatory ventilation: It is useful in improving the oxygenation without increasing the pressure in the airways or the alveoli. | |||
*[[Nitric oxide|Inhaled nitric oxide]]: The [[hypoxemia]] resulting from lung compression can cause hypoxemia that may result in pulmonary vasoconstriction and hypertension. | |||
*[[Vasodilator]] agents such as [[sildenafil]] and [[bosentan]] are used for severe and persistent pulmonary hypertension in the management of CDH | |||
[[Extracorporeal membrane oxygenation]] ([[Extracorporeal membrane oxygenation|ECMO]]): It is used as the last resort in patients resistant to all the conventional methods of ventilation. | |||
Indications for ECMO: | |||
* Inability to maintain preductal PaO<sub>2 </sub>saturations >85 percent or postductal PaO<sub>2</sub> >30 mmHg | |||
* PIP >28 cm H<sub>2</sub>O or mean airway pressure (MAP) >15 cm H<sub>2</sub>O | |||
* Hypotension that is resistant to fluid and inotropic support | |||
* Inadequate oxygen delivery with persistent metabolic acidosis | |||
Inclusion criteria for ECMO, including in our center, also include all of the following:<ref name="pmid8936654">{{cite journal| author=Lally KP| title=Extracorporeal membrane oxygenation in patients with congenital diaphragmatic hernia. | journal=Semin Pediatr Surg | year= 1996 | volume= 5 | issue= 4 | pages= 249-55 | pmid=8936654 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8936654 }}</ref> | |||
* Birth weight (BW) >2 kg | |||
* Gestational age (GA) >34 weeks | |||
* Absence of intracranial hemorrhage greater than grade I | |||
* Absence of chromosomal anomalies | |||
==Surgical treatment== | ==Surgical treatment== | ||
* | *The procedure must not be done unless the infant is stable.<ref name="pmid9694085">{{cite journal |vauthors=Clark RH, Hardin WD, Hirschl RB, Jaksic T, Lally KP, Langham MR, Wilson JM |title=Current surgical management of congenital diaphragmatic hernia: a report from the Congenital Diaphragmatic Hernia Study Group |journal=J. Pediatr. Surg. |volume=33 |issue=7 |pages=1004–9 |year=1998 |pmid=9694085 |doi= |url=}}</ref> | ||
=== | |||
*Most of the operations are done through a subcostal incision. | *Most of the operations are done through a subcostal incision. | ||
*The repair can be done in one of two ways depending on its size: | *The repair can be done in one of two ways depending on its size:<ref name="pmid28331629">{{cite journal |vauthors=Chandrasekharan PK, Rawat M, Madappa R, Rothstein DH, Lakshminrusimha S |title=Congenital Diaphragmatic hernia - a review |journal=Matern Health Neonatol Perinatol |volume=3 |issue= |pages=6 |year=2017 |pmid=28331629 |pmc=5356475 |doi=10.1186/s40748-017-0045-1 |url=}}</ref> | ||
**Primary closure: it involves repairing the defect using non-absorbable sutures. It is used if the defect is relatively small. | **Primary closure: it involves repairing the defect using non-absorbable sutures. It is used if the defect is relatively small. | ||
**Patch closure: using a prosthetic or fascial patch to close the defect. It is useful especially with large defects but increases the risk of infection. | **Patch closure: using a [[prosthetic]] or fascial patch to close the defect. It is useful especially with large defects but increases the risk of infection. | ||
*Endoscopic procedures through the thorax or the abdomen have been reported to have lower mortality rates but have higher recurrence rates. | *[[Endoscopic|Endoscopic procedures]] through the [[thorax]] or the [[abdomen]] have been reported to have lower mortality rates but have higher recurrence rates.<ref name="pmid23378478">{{cite journal |vauthors=Haroon J, Chamberlain RS |title=An evidence-based review of the current treatment of congenital diaphragmatic hernia |journal=Clin Pediatr (Phila) |volume=52 |issue=2 |pages=115–24 |year=2013 |pmid=23378478 |doi=10.1177/0009922812472249 |url=}}</ref> | ||
*If CDH repair is associated with an abdominal wall defect, silo closure may be tried temporarily until the CDH is stabilized. | *If CDH repair is associated with an [[abdominal wall defect]], silo closure may be tried temporarily until the CDH is stabilized.<ref name="pmid22682380">{{cite journal |vauthors=Shue EH, Miniati D, Lee H |title=Advances in prenatal diagnosis and treatment of congenital diaphragmatic hernia |journal=Clin Perinatol |volume=39 |issue=2 |pages=289–300 |year=2012 |pmid=22682380 |doi=10.1016/j.clp.2012.04.005 |url=}}</ref> | ||
{{#ev:youtube|aA-fwcH0eoA}} | |||
==Refrences== | ==Refrences== | ||
{{reflist|2}} | {{reflist|2}} |
Latest revision as of 15:57, 7 February 2018
Template:DiseaseDisorder infobox
Diaphragmatic hernia Main page |
Diagnosis |
---|
Treatment |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Ahmed Younes M.B.B.CH [2] Mohammed Abdelwahed M.D[3]
Synonyms and keywords: Congenital diaphragm hernia, Diaphragm hernia, Hernia through the diaphragm.
Overview
Diaphragmatic hernia is a congenital anomaly that occurs due to failure of the diaphragm to close leading to herniation of the abdominal content into the thoracic cavity leading to pulmonary hypoplasia. It most commonly presents in the neonates as respiratory distress. It is classified according to the site of herniation into anterior, posterolateral, and hiatal diaphragmatic hernia. It can cause complications in both the GIT and the cardiopulmonary circulation. It can be diagnosed antenatally in the week 24 and after birth, it is apparent on chest x-ray. The mainstay of treatment is surgical correction which must be done when the infant is completely stabilized.
Historical Perspective
- In 1679, the first case of congenital diaphragmatic hernia was reported by Riverius.[1]
- In 1888, the first procedure to repair congenital diaphragmatic hernia in an adult was done and in 1889, the first procedure on an infant was done.
- In 1905, the first successful operation was done but the success rate remained below 60% until 1925.[2]
- In 1940, A set of diagnostic criteria for diaphragmatic hernia were suggested.
- In 1950, the transthoracic approach was suggested instead of the transabdominal approach.
Classification
Congenital diaphragmatic hernia can be classified into three types according to the site of herniation.[3][4]
Posterolateral (Bochdalek) diaphragmatic hernia
- It is the most common subtype.
- Most commonly occurs on the left side and rarely occurs bilaterally.
Anterior (Morgagni) diaphragmatic hernia
- In anterior diaphragmatic hernia, the intestine bulges into the thorax through the anterior midline.
- Most of the cases of anterior diaphragmatic hernia occur on the right side.
Hiatal hernia
- The intestine finds its way to the thorax through the esophageal hiatus.
- More common in the adults than in neonates.
Pathophysiology and embryogenesis
- Hernia starts during the lung development, from the third week to the 16th week of gestation. Interference with lung development leads to pulmonary hypoplasia and hypoplasia of cardiac structures.[5]
- It is due to failure of normal closure of the pleuroperitoneal folds during the 4th to 10th weeks.[6]
- The vast majority of congenital diaphragmatic hernias occurs sporadically. However, familial cases involving autosomal recessive, autosomal dominant, and X-linked inheritance patterns have been reported.[7]
- Many different genetic defects (eg, aneuploidies, deletions, duplications, translocations) have been identified among sporadic cases.[8]
- The herniated viscera decrease the area needed by the lung to develop appropriately.[9]
- According to the degree of herniation, the degree of pulmonary hypoplasia is determined.
- The herniated viscera impairs the development of the bronchial tree.
- As a consequence, the alveoli development is also slowed.[10]
- The development of the pulmonary artery tree is halted too resulting in excessive masculinization of the arteries.
- The increased pulmonary pressure leads to increased pressure on the right ventricle leading to the development of a right to left shunt.
- As most of the cases of diaphragmatic hernias are unilateral, the pulmonary hypoplasia is also usually unilateral, but it can be bilateral if the mediastinum is pushed by a massive unilateral hernia.
- The abnormal alveolar development can lead to hypoxemia leading to pulmonary vasoconstriction which aggravates the condition.
Risk factors
Risk factors of diaphragmatic hernia include:[11]
- Male gender
- Premature birth
- Macrosomia
- Low birth weight
- Vitamin A deficiency[12]
- Exposure to thalidomide
- Exposure to anticonvulsants and quinine[13]
Differentiating Diaphragmatic hernia from other Diseases
Congenital diaphragmatic hernia may be differentiated from other thoracic lesions:[14]
- Congenital cystic adenomatoid malformation
- Bronchopulmonary sequestration
- Bronchopulmonary foregut malformation
- Bronchogenic cysts
- Bronchial atresia
- Enteric cysts
- Teratomas
Congenital diaphragmatic hernia may be differentiated from other causes of pulmonary hypertension:
Epidemiology
Incidence
Prevalence
- The prevalence of congenital diaphragmatic hernia ranges from 10 to 40 per live births.[16]
Sex
- Congenital diaphragmatic hernia has no sex predilection.[16]
Age
- 90% of the congenital diaphragmatic hernias present in the neonatal period.
Screening
- According to USPSTF, there is no screening recommended for diaphragmatic hernia. The recurrence risk for multiple congenital anomalies of unknown etiology is considered to be <5 percent. [41]
- Referral to a genetic counselor can be useful for thorough genetic evaluation and counseling.
- Microarray-based comparative genomic hybridization is a powerful tool for detection of submicroscopic chromosomal abnormalities.
Natural history, complications and prognosis
Natural history
- 90% of the cases of congenital diaphragmatic hernia presents in the neonatal period.
- If left untreated, congenital diaphragmatic hernia can be fatal due to pulmonary hypoplasia and severe pulmonary hypertension.
Complications
GI complications[17]
- Abnormal developmental rotation of the midgut
- Midgut volvulus
- Gastric volvulus
Cardiopulmonary complications[17]
- Pulmonary hypoplasia
- Pulmonary artery hypertension
- In severe cases, ventricular hypoplasia
Prognosis
- The prognosis is mainly dependent on the size of the defect and the degree of the herniation.[18]
- The presence or absence of liver herniation on fetal MRI is the most reliable prediction of the postnatal survival.
- The estimation of fetal lung volume and lung area to head circumference is a useful indicator of the prognosis of the disease in the absence of liver herniation.
- The mortality ranges from 25-60% of the cases.
History and Symptoms
CDH most often presents in the neonatal period as:[19]
- Cyanosis
- Rapid breathing (tachypnea)
- Shortness of breath (dyspnea)
- Increased work of breathing (labored breathing)[20]
- Lethargy
- Intolerance to feeding due to inadequate oxygenation.
- Adrenal insufficiency: it is a common association with congenital diaphragmatic hernia.
{{#ev:youtube|jPpGHVkB54A}}
Physical Examination
General appearance
Vital signs
- Heart rate: tachycardia
- Respiratory rate: tachypnea
- Blood pressure: The infant may have hypotension due to compression of the ventricles
Lungs
- The chest may have a barrel shape.
- Absent breath sounds at the side of the hernia[22]
- Peristaltic sounds in the chest
Heart
- The heart sounds may be displaced to the other side of the chest.
- The heart sounds may reveal the murmurs of associated cardiac anomalies.
Abdomen
- The abdomen is scaphoid due to herniation of the abdominal content into the thoracic cavity.
Laboratory Findings
Antenatal tests
- Amniocentesis: karyotyping should be obtained to rule out associated chromosomal abnormalities.
- Maternal alpha-fetoprotein is decreased in CDH[23]
Postnatal tests
- Arterial blood gas: ABG may show hypoxemia, metabolic acidosis, and hypercapnia.
- The infant should be investigated for the presence of associated chromosomal abnormalities.[24]
Radiological tests
Antenatal ultrasound
- CDH can be diagnosed in the 24th week.[25]
- In addition to the hernia, ultrasound can show polyhydramnios and absence of the gastric air bubble
- Left sided CDH is far more common than right sided CDH, while bilateral CDH is rare.
- The presence of liver herniation is associated with worse prognosis and survival.
- The estimation of lung area to head circumference ratio can give an idea about the prognosis (especially if there is no liver herniation). The lower the ratio, the worse the prognosis.
![](/images/8/8c/Congenital-diaphragmatic-hernia-25.jpg)
Chest x-ray
- Bowel loops in the thoracic cavity (can be further demonstrated through placement of a feeding tube)
- Shifting of the mediastinum to the contralateral side
![](/images/7/7a/Congenital-diaphragmatic-hernia-2.jpg)
Echocardiography
- Echocardiography must be done to exclude associated cardiac anomalies and to exclude the presence of an associated ventricular strain from the pressure.
Medical treatment
Criteria for mechanical ventilation:
- Pressure-limited ventilation at rates of 30 to 70 breaths per minute
- Positive end-expiratory pressure (PEEP) should be maintained at physiologic levels (3 to 5 cm H2O) whenever possible.
- High-frequency ventilation (HFV) is reserved for neonates who continue to have hypoxia and hypercarbia (PaCO2 >65 mmHg) refractory to CMV.
Antenatal glucocorticoids
Antenatal glucocorticoids improve the survival by increasing the secretion of surfactant and thus stimulating lung maturation and preventing severe pulmonary hypoplasia.[3]
Preoperative stabilization
- Preoperative stabilization of the patient is important as performing the operation in an unstable infant is associated with higher morbidity and mortality rates.
Preoperative stabilization measures include:[26]
- Mechanical ventilation: The aim is to ensure proper oxygenation without causing any barotrauma to the lung. Peak inspiratory pressure should be always less than 30 cm H2O
- Nasogastric tube insertion: The aim is to decompress the bowel and to give more space for the lung
- Blood pressure support: using vasopressors or isotonic fluid: The aim is to keep the mean blood pressure above 50 mmHg.[26]
- High-frequency oscillatory ventilation: It is useful in improving the oxygenation without increasing the pressure in the airways or the alveoli.
- Inhaled nitric oxide: The hypoxemia resulting from lung compression can cause hypoxemia that may result in pulmonary vasoconstriction and hypertension.
- Vasodilator agents such as sildenafil and bosentan are used for severe and persistent pulmonary hypertension in the management of CDH
Extracorporeal membrane oxygenation (ECMO): It is used as the last resort in patients resistant to all the conventional methods of ventilation.
Indications for ECMO:
- Inability to maintain preductal PaO2 saturations >85 percent or postductal PaO2 >30 mmHg
- PIP >28 cm H2O or mean airway pressure (MAP) >15 cm H2O
- Hypotension that is resistant to fluid and inotropic support
- Inadequate oxygen delivery with persistent metabolic acidosis
Inclusion criteria for ECMO, including in our center, also include all of the following:[27]
- Birth weight (BW) >2 kg
- Gestational age (GA) >34 weeks
- Absence of intracranial hemorrhage greater than grade I
- Absence of chromosomal anomalies
Surgical treatment
- The procedure must not be done unless the infant is stable.[28]
- Most of the operations are done through a subcostal incision.
- The repair can be done in one of two ways depending on its size:[18]
- Primary closure: it involves repairing the defect using non-absorbable sutures. It is used if the defect is relatively small.
- Patch closure: using a prosthetic or fascial patch to close the defect. It is useful especially with large defects but increases the risk of infection.
- Endoscopic procedures through the thorax or the abdomen have been reported to have lower mortality rates but have higher recurrence rates.[21]
- If CDH repair is associated with an abdominal wall defect, silo closure may be tried temporarily until the CDH is stabilized.[29]
{{#ev:youtube|aA-fwcH0eoA}}
Refrences
- ↑ Irish MS, Holm BA, Glick PL (1996). "Congenital diaphragmatic hernia. A historical review". Clin Perinatol. 23 (4): 625–53. PMID 8982561.
- ↑ Irish MS, Holm BA, Glick PL (1996). "Congenital diaphragmatic hernia. A historical review". Clin Perinatol. 23 (4): 625–53. PMID 8982561.
- ↑ 3.0 3.1 Badillo A, Gingalewski C (2014). "Congenital diaphragmatic hernia: treatment and outcomes". Semin. Perinatol. 38 (2): 92–6. doi:10.1053/j.semperi.2013.11.005. PMID 24580764.
- ↑ Schumacher L, Gilbert S (2009). "Congenital diaphragmatic hernia in the adult". Thorac Surg Clin. 19 (4): 469–72. doi:10.1016/j.thorsurg.2009.08.004. PMID 20112629.
- ↑ DiFiore JW, Fauza DO, Slavin R, Wilson JM (1995). "Experimental fetal tracheal ligation and congenital diaphragmatic hernia: a pulmonary vascular morphometric analysis". J Pediatr Surg. 30 (7): 917–23, discussion 923-4. PMID 7472945.
- ↑ Bielinska M, Jay PY, Erlich JM, Mannisto S, Urban Z, Heikinheimo M; et al. (2007). "Molecular genetics of congenital diaphragmatic defects". Ann Med. 39 (4): 261–74. doi:10.1080/07853890701326883. PMC 2174621. PMID 17558598.
- ↑ Mishalany H, Gordo J (1986). "Congenital diaphragmatic hernia in monozygotic twins". J Pediatr Surg. 21 (4): 372–4. PMID 3701555.
- ↑ Lurie IW (2003). "Where to look for the genes related to diaphragmatic hernia?". Genet Couns. 14 (1): 75–93. PMID 12725592.
- ↑ Langham MR, Kays DW, Ledbetter DJ, Frentzen B, Sanford LL, Richards DS (1996). "Congenital diaphragmatic hernia. Epidemiology and outcome". Clin Perinatol. 23 (4): 671–88. PMID 8982563.
- ↑ Keijzer R, Puri P (2010). "Congenital diaphragmatic hernia". Semin. Pediatr. Surg. 19 (3): 180–5. doi:10.1053/j.sempedsurg.2010.03.001. PMID 20610190.
- ↑ Cullen ML, Klein MD, Philippart AI (1985). "Congenital diaphragmatic hernia". Surg. Clin. North Am. 65 (5): 1115–38. PMID 3904042.
- ↑ See AW, Kaiser ME, White JC, Clagett-Dame M (2008). "A nutritional model of late embryonic vitamin A deficiency produces defects in organogenesis at a high penetrance and reveals new roles for the vitamin in skeletal development". Dev Biol. 316 (2): 171–90. doi:10.1016/j.ydbio.2007.10.018. PMID 18321479.
- ↑ Enns GM, Cox VA, Goldstein RB, Gibbs DL, Harrison MR, Golabi M (1998). "Congenital diaphragmatic defects and associated syndromes, malformations, and chromosome anomalies: a retrospective study of 60 patients and literature review". Am J Med Genet. 79 (3): 215–25. PMID 9788565.
- ↑ Lurie IW (2003). "Where to look for the genes related to diaphragmatic hernia?". Genet Couns. 14 (1): 75–93. PMID 12725592.
- ↑ Fisher JC, Haley MJ, Ruiz-Elizalde A, Stolar CJ, Arkovitz MS (2009). "Multivariate model for predicting recurrence in congenital diaphragmatic hernia". J. Pediatr. Surg. 44 (6): 1173–9, discussion 1179–80. doi:10.1016/j.jpedsurg.2009.02.043. PMC 3072822. PMID 19524735.
- ↑ 16.0 16.1 McGivern MR, Best KE, Rankin J, Wellesley D, Greenlees R, Addor MC, Arriola L, de Walle H, Barisic I, Beres J, Bianchi F, Calzolari E, Doray B, Draper ES, Garne E, Gatt M, Haeusler M, Khoshnood B, Klungsoyr K, Latos-Bielenska A, O'Mahony M, Braz P, McDonnell B, Mullaney C, Nelen V, Queisser-Luft A, Randrianaivo H, Rissmann A, Rounding C, Sipek A, Thompson R, Tucker D, Wertelecki W, Martos C (2015). "Epidemiology of congenital diaphragmatic hernia in Europe: a register-based study". Arch. Dis. Child. Fetal Neonatal Ed. 100 (2): F137–44. doi:10.1136/archdischild-2014-306174. PMID 25411443.
- ↑ 17.0 17.1 Tovar JA (2012). "Congenital diaphragmatic hernia". Orphanet J Rare Dis. 7: 1. doi:10.1186/1750-1172-7-1. PMC 3261088. PMID 22214468.
- ↑ 18.0 18.1 Chandrasekharan PK, Rawat M, Madappa R, Rothstein DH, Lakshminrusimha S (2017). "Congenital Diaphragmatic hernia - a review". Matern Health Neonatol Perinatol. 3: 6. doi:10.1186/s40748-017-0045-1. PMC 5356475. PMID 28331629.
- ↑ Kotecha S, Barbato A, Bush A, Claus F, Davenport M, Delacourt C, Deprest J, Eber E, Frenckner B, Greenough A, Nicholson AG, Antón-Pacheco JL, Midulla F (2012). "Congenital diaphragmatic hernia". Eur. Respir. J. 39 (4): 820–9. doi:10.1183/09031936.00066511. PMID 22034651.
- ↑ Peetsold MG, Kneepkens CM, Heij HA, IJsselstijn H, Tibboel D, Gemke RJ (2010). "Congenital diaphragmatic hernia: long-term risk of gastroesophageal reflux disease". J. Pediatr. Gastroenterol. Nutr. 51 (4): 448–53. doi:10.1097/MPG.0b013e3181d1b149. PMID 20512059.
- ↑ 21.0 21.1 Haroon J, Chamberlain RS (2013). "An evidence-based review of the current treatment of congenital diaphragmatic hernia". Clin Pediatr (Phila). 52 (2): 115–24. doi:10.1177/0009922812472249. PMID 23378478.
- ↑ Lund DP, Mitchell J, Kharasch V, Quigley S, Kuehn M, Wilson JM (1994). "Congenital diaphragmatic hernia: the hidden morbidity". J. Pediatr. Surg. 29 (2): 258–62, discussion 262–4. PMID 8176602.
- ↑ McHoney M (2015). "Congenital diaphragmatic hernia, management in the newborn". Pediatr. Surg. Int. 31 (11): 1005–13. doi:10.1007/s00383-015-3794-9. PMID 26403792.
- ↑ Lally KP, Paranka MS, Roden J, Georgeson KE, Wilson JM, Lillehei CW, Breaux CW, Poon M, Clark RH, Atkinson JB (1992). "Congenital diaphragmatic hernia. Stabilization and repair on ECMO". Ann. Surg. 216 (5): 569–73. PMC 1242675. PMID 1444648.
- ↑ Waag KL, Loff S, Zahn K, Ali M, Hien S, Kratz M, Neff W, Schaffelder R, Schaible T (2008). "Congenital diaphragmatic hernia: a modern day approach". Semin. Pediatr. Surg. 17 (4): 244–54. doi:10.1053/j.sempedsurg.2008.07.009. PMID 19019293.
- ↑ 26.0 26.1 Kesieme EB, Kesieme CN (2011). "Congenital diaphragmatic hernia: review of current concept in surgical management". ISRN Surg. 2011: 974041. doi:10.5402/2011/974041. PMC 3251163. PMID 22229104.
- ↑ Lally KP (1996). "Extracorporeal membrane oxygenation in patients with congenital diaphragmatic hernia". Semin Pediatr Surg. 5 (4): 249–55. PMID 8936654.
- ↑ Clark RH, Hardin WD, Hirschl RB, Jaksic T, Lally KP, Langham MR, Wilson JM (1998). "Current surgical management of congenital diaphragmatic hernia: a report from the Congenital Diaphragmatic Hernia Study Group". J. Pediatr. Surg. 33 (7): 1004–9. PMID 9694085.
- ↑ Shue EH, Miniati D, Lee H (2012). "Advances in prenatal diagnosis and treatment of congenital diaphragmatic hernia". Clin Perinatol. 39 (2): 289–300. doi:10.1016/j.clp.2012.04.005. PMID 22682380.