Management of congenital diaphragmatic hernia: A systematic review from the APSA outcomes and evidence based practice committee

Puligandla, Pramod S.; Grabowski, Julia; Austin, Mary T.; Hedrick, Holly L.; Renaud, Elizabeth; Arnold, Meghan A.; Williams, Regan F.; Graziano, Kathleen; Dasgupta, Roshni; McKee, Milissa; López, Mónica E.; Jancelewicz, Timothy; Goldin, Adam B.; Downard, Cynthia D.; Islam, Saleem

doi:10.1016/j.jpedsurg.2015.09.010

Cited by 146 publications

(84 citation statements)

References 94 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The recurrence rate of CDH notruedefect within 1 year was higher than in previous statements, where no recurrences in CDE were reported [7,[19][20][21][22]. Then again, the total number of recurrences found in the Bochdalek type CDH in this cohort (5%) is relatively low in comparison to other studies, and the sample sizes of both eventration and hernia sac patients are relatively small [8,24]. Previous studies suggested that the severity or defect size of CDH would be a prognostic value for recurrence rates [24][25][26][27], but these cohorts were not adjusted for CDE and CDH with hernia sacs.…”

Section: Discussioncontrasting

confidence: 65%

See 1 more Smart Citation

Congenital diaphragmatic eventration and hernia sac compared to CDH with true defects: a retrospective cohort study

et al. 2020

View full text Add to dashboard Cite

Congenital diaphragmatic eventration (CDE) and congenital diaphragmatic hernia (CDH) with or without hernia sac are three different types of congenital diaphragmatic malformations, which this study evaluates. All surgically treated patients with CDE or Bochdalek type CDH between 2000 and 2016 were included in this retrospective analysis. Demographics, CDH-characteristics, treatment, and clinical outcome were evaluated. In total, 200 patients were included. Patients with an eventration or hernia sac had no significant differences and were compared as patients without a true defect to patients with a true defect. The 1-year survival of patients with a true defect was significantly lower than patients with no true defect (76% versus 97%, p = 0.001). CDH with no true defect had significantly better short-term outcomes than CDH with true defect requiring patch repair. However, at 30 days, they more often required oxygen supplementation (46% versus 26%, p = 0.03) and had a higher recurrence rate (8% versus 0%, p = 0.006) (three eventration and two hernia sac patients). Conclusion: Patients without a true defect seem to have a more similar clinical outcome than CDH patients with a true defect, with a better survival. However, the recurrence rate and duration of oxygen supplementation at 30 days are higher than CDH patients with a true defect. What is Known:• Congenital diaphragmatic hernia with or without hernia sac and congenital diaphragmatic eventration (incomplete muscularization) are often treated similarly. • Patients with hernia sac and eventration are thought to have a relatively good outcome, but exact numbers are not described. What is New: • Congenital diaphragmatic eventration and patients with hernia sac seem to have a more similar clinical outcome than Bochdalek type CDH with a true defect. • Patients without a true defect (eventration or hernia sac) have a high recurrence rate.

show abstract

Section: Discussioncontrasting

confidence: 65%

“…Clinical manifestations are diverse, varying from asymptomatic to life-threatening respiratory distress. Overall, it is thought to have a relatively good outcome, but exact numbers are not described [7], because most studies do not specify this subtype as a specific entity [2,[8][9][10][11][12].…”

Section: Introductionmentioning

confidence: 99%

Congenital diaphragmatic eventration and hernia sac compared to CDH with true defects: a retrospective cohort study

et al. 2020

View full text Add to dashboard Cite

show abstract

“…Although a systematic review39 suggested that there was no overall advantage with either the VV or VA technique, there is a difference in preferred mode of cannulation between centres. VA ECMO seem the more popular of the two modes according to the published paper and ECMO registries; presumably as VA ECMO may give the additional benefit in the presence of severe cardiac dysfunction.…”

Section: Technique and Applicationmentioning

confidence: 99%

Role of ECMO in congenital diaphragmatic hernia

McHoney

Hammond

2017

Arch Dis Child Fetal Neonatal Ed

View full text Add to dashboard Cite

Congenital diaphragmatic hernia (CDH) is typified morphologically by failure of diaphragmatic development with accompanying lung hypoplasia and persistent pulmonary hypertension of the newborn (PPHN). Patients who have labile physiology and low preductal saturations despite optimal ventilatory and inotropic support may be considered for extracorporeal membrane oxygenation (ECMO). Systematic reviews into the benefits of ECMO in CDH concluded that any benefit is unclear. Few randomised trials exist to demonstrate clear benefit and guide management. However, ECMO may have its uses in those that have reversibility of their respiratory disease. A few centres and networks have demonstrated an increase in survival rate by post hoc analysis (based on a difference in referral patterns with the availability of ECMO) in their series. One issue may be that of careful patient selection with regard to reversibility of pathophysiology. At present, there is no single test or prognostication that predicts reversibility of PPHN and criteria for referral for ECMO is undergoing continued refinement. Overall survival is similar between cannulation modes. There is no consensus on the time limit for ECMO runs. The optimal timing of surgery for patients on ECMO is difficult to definitively establish, but it seems that repair at an early stage (with careful perioperative management) is becoming less of a taboo, and may improve outcome and help with either coming off ECMO or decisions on withdrawal later. The provision of ECMO will continue to be evaluated, and prospective randomised trial are needed to help answer question of patient selection and management.

show abstract

“…Its clinical management highly impacts the outcome of vulnerable patients and still remains challenging for the involved medical specialists [1][2][3]. In CDH, an incomplete diaphragm fails to separate the thoracic and abdominal cavities during development.…”

Section: Introductionmentioning

confidence: 99%

STATs in Lung Development: Distinct Early and Late Expression, Growth Modulation and Signaling Dysregulation in Congenital Diaphragmatic Hernia

Piairo

Moura

Baptista

et al. 2017

Cell Physiol Biochem

View full text Add to dashboard Cite

Key Words Signal transducer and activator of transcription (STAT) • Suppressor of cytokine signaling (SOCS) • Congenital diaphragmatic hernia (CDH) • Lung development • NitrofenAbstract Background: Congenital diaphragmatic hernia (CDH) is a life-threatening developmental anomaly, intrinsically combining severe pulmonary hypoplasia and hypertension. During development, signal transducers and activators of transcription (STAT) are utilized to elicit cell growth, differentiation, and survival. Methods: We used the nitrofen-induced CDH rat model. At selected gestational time points, lungs were divided into two experimental groups, i.e., control or CDH. We performed immunohistochemistry and western blotting analysis to investigate the developmental expression profile of the complete family of STATs (STAT1-6), plus specific STATs activation (p-STAT3, p-STAT6) and regulation by SOCS (SOCS3) in normal lungs against those of diseased lungs. The normal fetal lung explants were treated with piceatannol (STAT3 inhibitor) in vitro followed by morphometrical analysis. Results: Molecular profiling of STATs during the lung development revealed distinct early and late expression signatures. Experimental CDH altered the STATs expression, activation, and regulation in the fetal lungs. In particular, STAT3 and STAT6 were persistently over-expressed and early over-activated. Piceatannol treatment dose-dependently stimulated the fetal lung growth. Conclusion: These findings suggest that STATs play an important role during normal fetal lung development and CDH pathogenesis. Moreover, functionally targeting STAT signaling modulates fetal lung growth, which highlights that STAT3 and STAT6 signaling might be promising therapeutic targets in reducing or preventing pulmonary hypoplasia in CDH.

show abstract

Management of congenital diaphragmatic hernia: A systematic review from the APSA outcomes and evidence based practice committee

Cited by 146 publications

References 94 publications

Congenital diaphragmatic eventration and hernia sac compared to CDH with true defects: a retrospective cohort study

Congenital diaphragmatic eventration and hernia sac compared to CDH with true defects: a retrospective cohort study

Role of ECMO in congenital diaphragmatic hernia

STATs in Lung Development: Distinct Early and Late Expression, Growth Modulation and Signaling Dysregulation in Congenital Diaphragmatic Hernia

Contact Info

Product

Resources

About