Congenital diaphragmatic hernia natural history, complications and prognosis: Difference between revisions
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In many neonates, there is an initial period of 24-48 hours of stability after which [[Acute respiratory distress syndrome|acute respiratory distress]] develops. [[Pulmonary hypoplasia]] is a common complication amongst almost all affected children.<ref name="pmid20301533">{{cite journal| author=Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Gripp KW | display-authors=etal| title=GeneReviews® | journal= | year= 1993 | volume= | issue= | pages= | pmid=20301533 | doi= | pmc= | url= }}</ref> Major complications include: | In many neonates, there is an initial period of 24-48 hours of stability after which [[Acute respiratory distress syndrome|acute respiratory distress]] develops. [[Pulmonary hypoplasia]] is a common complication amongst almost all affected children.<ref name="pmid20301533">{{cite journal| author=Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Gripp KW | display-authors=etal| title=GeneReviews® | journal= | year= 1993 | volume= | issue= | pages= | pmid=20301533 | doi= | pmc= | url= }}</ref> Major complications include: | ||
* [[Tracheobronchomalacia]] | *[[Tracheobronchomalacia]] | ||
* [[Pneumothorax]] | *[[Pneumothorax]] | ||
* [[Pulmonary hypertension|Pulmonary Hypertension]] | *[[Pulmonary hypertension|Pulmonary Hypertension]] | ||
* [[Pulmonary infection]]; especially [[viral pneumonia]] and [[aspiration pneumonia]] | *[[Pulmonary infection]]; especially [[viral pneumonia]] and [[aspiration pneumonia]] | ||
* [[Respiratory failure]] | *[[Respiratory failure]] | ||
* Reduced exercise tolerance | *Reduced exercise tolerance | ||
* [[Stunted growth]] due to aversion to oral feeding | *[[Stunted growth]] due to aversion to oral feeding | ||
* [[Gastroesophageal reflux disease|Gastroesophageal reflux]] <ref name="pmid32310536">{{cite journal| author=| title=StatPearls | journal= | year= 2022 | volume= | issue= | pages= | pmid=32310536 | doi= | pmc= | url= }}</ref> | *[[Gastroesophageal reflux disease|Gastroesophageal reflux]] <ref name="pmid32310536">{{cite journal| author=| title=StatPearls | journal= | year= 2022 | volume= | issue= | pages= | pmid=32310536 | doi= | pmc= | url= }}</ref> | ||
* Chest asymmetry, [[pectus excavatum]] and [[scoliosis]] following surgical repair of the chest wall | *Chest asymmetry, [[pectus excavatum]] and [[scoliosis]] following surgical repair of the chest wall | ||
* [[Sensorineural hearing loss]] <ref name="pmid323105362">{{cite journal| author=| title=StatPearls | journal= | year= 2022 | volume= | issue= | pages= | pmid=32310536 | doi= | pmc= | url= }}</ref> | *[[Sensorineural hearing loss]] <ref name="pmid323105362">{{cite journal| author=| title=StatPearls | journal= | year= 2022 | volume= | issue= | pages= | pmid=32310536 | doi= | pmc= | url= }}</ref> | ||
==Prognosis== | ==Prognosis== | ||
A diaphragmatic hernia is a very serious disorder. The outcome of surgery depends on how well | A diaphragmatic hernia is a very serious disorder. The outcome of surgery depends on how well the baby's lungs have developed. Usually, the outlook is very good for infants who have enough lung tissue. Affected infants often require mechanical ventilation and oxygenation, followed by the use of diuretics after surgical correction. The use of oxygen and diuretics can be discontinued, often within the first two years of life.<ref name="pmid20301533" /> For children presenting with intermittent [[wheezing]], [[bronchodilators]] are required. Some children require continued nutritional supplements. With advances in neonatal and surgical care, survival is now greater than 60%-80%. Ventilation strategies have also contributed to this increased survival rate. | ||
Some important factors contributing to the prognosis include: <ref name="pmid323105363">{{cite journal| author=| title=StatPearls | journal= | year= 2022 | volume= | issue= | pages= | pmid=32310536 | doi= | pmc= | url= }}</ref> <ref name="pmid203015332">{{cite journal| author=Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Gripp KW | display-authors=etal| title=GeneReviews® | journal= | year= 1993 | volume= | issue= | pages= | pmid=20301533 | doi= | pmc= | url= }}</ref> | |||
* defect size | |||
* degree of [[pulmonary hypoplasia]] | |||
* development of [[pulmonary hypertension]]; this is often unresponsive to medical therapy | |||
* isolated or complex defect; complex defects are associated with higher mortality rates | |||
* presence of [[CVS malformations]] | |||
Proposed indicative methods include prenatal estimates of [[lung-to-head ratio]], [[observed/expected-total fetal lung volum]]<nowiki/>e, as well as [[liver herniation percentage]]. [[Liver herniation]] is associated with a poor prognosis. | |||
==References== | ==References== | ||
Revision as of 00:49, 23 April 2022
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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
Natural History
Complications
In many neonates, there is an initial period of 24-48 hours of stability after which acute respiratory distress develops. Pulmonary hypoplasia is a common complication amongst almost all affected children.[1] Major complications include:
- Tracheobronchomalacia
- Pneumothorax
- Pulmonary Hypertension
- Pulmonary infection; especially viral pneumonia and aspiration pneumonia
- Respiratory failure
- Reduced exercise tolerance
- Stunted growth due to aversion to oral feeding
- Gastroesophageal reflux [2]
- Chest asymmetry, pectus excavatum and scoliosis following surgical repair of the chest wall
- Sensorineural hearing loss [3]
Prognosis
A diaphragmatic hernia is a very serious disorder. The outcome of surgery depends on how well the baby's lungs have developed. Usually, the outlook is very good for infants who have enough lung tissue. Affected infants often require mechanical ventilation and oxygenation, followed by the use of diuretics after surgical correction. The use of oxygen and diuretics can be discontinued, often within the first two years of life.[1] For children presenting with intermittent wheezing, bronchodilators are required. Some children require continued nutritional supplements. With advances in neonatal and surgical care, survival is now greater than 60%-80%. Ventilation strategies have also contributed to this increased survival rate.
Some important factors contributing to the prognosis include: [4] [5]
- defect size
- degree of pulmonary hypoplasia
- development of pulmonary hypertension; this is often unresponsive to medical therapy
- isolated or complex defect; complex defects are associated with higher mortality rates
- presence of CVS malformations
Proposed indicative methods include prenatal estimates of lung-to-head ratio, observed/expected-total fetal lung volume, as well as liver herniation percentage. Liver herniation is associated with a poor prognosis.
References
- ↑ 1.0 1.1 Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Gripp KW; et al. (1993). "GeneReviews®". ( ): . PMID 20301533.
- ↑ "StatPearls". ( ). 2022: . PMID 32310536 Check
|pmid=value (help). - ↑ "StatPearls". ( ). 2022: . PMID 32310536 Check
|pmid=value (help). - ↑ "StatPearls". ( ). 2022: . PMID 32310536 Check
|pmid=value (help). - ↑ Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Gripp KW; et al. (1993). "GeneReviews®". ( ): . PMID 20301533.