Congenital diaphragmatic hernia medical therapy
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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
Medical therapy can be subdivided according to antenatal and postnatal management. Antenatal management focuses on close monitoring. Fetal therapy may be an option for eligible patients with resultant fetal tracheal occlusion performed at 26-30 weeks to allow increased surfactant formation. Postnatal management primarily focuses on preventing birth before 37 weeks of gestation, as these neonates have been shown to have worse outcomes comparatively. Other postnatal options include the use of ECMO, the use of a nasogastric tube and intubation where required as well as maintaining adequate ventilation and oxygenation. Medications that may be helpful in reducing pulmonary hypertension include inhaled nitric oxide, sildenafil, bosentan, PGE1, and PGI2.
Medical Therapy
Antenatal Management[1]
- Close monitoring to assess for fetal well-being; fetal therapy may be offered to eligible patients which involves occlusion of the fetal trachea at 26-30 weeks of gestation which allows fluid accumulation in the lung and its stretching. This results in release of surfactant but a potentially increased risk of preterm delivery. Post-natal CDH repair is still required.[2]
Postnatal Management[3]
- Ensure delivery is not before 37 weeks, preferably at 39-40 weeks
- Extracorporeal membrane oxygenation (ECMO) therapy has been used as part of the treatment strategy at some hospitals.[4][5]
Delivery Room Management
- Immediate use of nasogastric tube to decompress the stomach and intestines
- Immediate intubation in case of respiratory distress
| Parameter | Value |
|---|---|
| Pre-ductal saturation | 85%-95% |
| PIP | < 25 cm H2O |
| PEEP | 3-5 cm H2O |
| PCO2 | 45-60 mmHg |
Management of Pulmonary Hypertension
- Adequate ventilation and oxygenation
- Vasopressors
- Inhaled nitric oxide (vasodilator)[6]
- Other drugs: sildenafil, bosentan, PGE1, PGI2
References
- ↑ "StatPearls". ( ). 2022: . PMID 32310536 Check
|pmid=value (help). - ↑ 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.
- ↑ "StatPearls". ( ). 2022: . PMID 32310536 Check
|pmid=value (help). - ↑ 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.
- ↑ 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.
- ↑ Puligandla PS, Grabowski J, Austin M, Hedrick H, Renaud E, Arnold M; et al. (2015). "Management of congenital diaphragmatic hernia: A systematic review from the APSA outcomes and evidence based practice committee". J Pediatr Surg. 50 (11): 1958–70. doi:10.1016/j.jpedsurg.2015.09.010. PMID 26463502.