StayCurrentMD · Case-Based Journal Review: Congenital Diaphragmatic Hernia 2023
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Podcast19 min·Published Oct 2023Older

Case-Based Journal Review: Congenital Diaphragmatic Hernia 2023

With Dr. Fernando Bullettin · hosted by Dr. Cecilia Gigena & Dr. Jose Campos & Dr. Todd Ponsky

Chapter 1 of 6 · Evidence & Research

FETO efficacy

Fetal intervention (FETO) for severe CDH

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What the experts said14 expert statements · 14 host summaries
Centers with high CDH volume performing ECMO achieve survival rates of approximately 50% or more in severe CDH patients.
EpidemiologicalFernando Bullettin
In Europe, some maternal-fetal medicine centers perform FETO but then transfer patients to separate centers for delivery and neonatal care, creating a separation between fetal intervention and postnatal ECMO expertise.
ClinicalFernando Bullettin
FETO does not eliminate the need for ECMO; both interventions can be performed in the same patient.
ClinicalTodd Ponsky
One Chilean center's protocol involves ECMO cannulation at 6-8 hours of life followed by CDH repair 8-10 hours after cannulation.
ClinicalFernando Bullettin
For CDH repair on ECMO, anticoagulation management involves decreasing heparin and using Amicar before, during, and after surgery, with neonatologists managing the protocol.
ClinicalTodd Ponsky
At least four published reports confirm the data showing benefit of early repair on ECMO, representing a practice change in the field.
OpinionFernando Bullettin
Prolonged operative time required for thoracoscopic CDH repair in babies requiring inotropic support or special ventilation (with increased CO2 and decreased pH) is detrimental to the patient.
OpinionFernando Bullettin
Possible technical factors contributing to higher recurrence in thoracoscopic CDH repair include: insufficient scar formation from lack of tissue opposition, inadequate unfurling of the diaphragm, taking bites at the same tissue level creating a bundle that can pull away, or insufficient number of sutures.
OpinionTodd Ponsky
A transverse abdominal incision (rather than subcostal) allows intraoperative decision-making between flap and mesh repair based on defect size, making mesh repair slightly more difficult but not impossible.
ClinicalJose Campos
Biological patches are not designed to function as a bridge and should not be used alone for CDH repair; they can be used to reinforce a synthetic patch by placing on top.
OpinionTodd Ponsky
The 80% chest wall deformity rate with synthetic patches may be inflated by inclusion of skeletal deformities inherent to CDH disease rather than caused by the patch itself.
OpinionFernando Bullettin
Type D CDH defects (large defects) are very rare.
EpidemiologicalFernando Bullettin
One surgeon performs fundoplication at initial CDH repair selectively in patients with type D defects who are on ECMO.
ClinicalFernando Bullettin
In one surgeon's experience with selective fundoplication in type D ECMO patients, none have required subsequent fundoplication surgery, representing definitive repair of both CDH and reflux.
ClinicalFernando Bullettin
FETO (fetal endoscopic tracheal occlusion) increases general survival in moderate and severe CDH, with statistically significant benefit specifically in severe CDH cases, based on meta-analysis of 4 randomized controlled trials with 341 patients.
Host summaryCecilia Gigena summarizes what Dr. Fernando Bullettin said · not cited in answers
The apparent benefit of FETO depends on the quality of the comparison center's ECMO results; centers with moderate or poor ECMO outcomes will show larger apparent FETO benefits.
Host summaryJose Campos summarizes what Dr. Fernando Bullettin said · not cited in answers
Boston Children's Hospital retrospective cohort study found that on-ECMO repair demonstrated lower mortality rate, lower hazard ratio, and lower incidence of non-repair compared to post-ECMO repair.
Host summaryCecilia Gigena summarizes what Dr. Fernando Bullettin said · not cited in answers
In the Boston study's second analysis arm, early repair on ECMO was associated with lower mortality, lower hazard ratio, and lower incidence of non-repair compared to late repair on ECMO.
Host summaryCecilia Gigena summarizes what Dr. Fernando Bullettin said · not cited in answers
Meta-analysis from Canada (2014) found that minimally invasive CDH repair has higher recurrence rates (especially when patch is required) and longer operative time compared to open repair.
Host summaryJose Campos summarizes what Dr. Fernando Bullettin said · not cited in answers
The same meta-analysis found open CDH repair was associated with more postoperative ventilator time and higher mortality rates compared to MIS repair.
Host summaryJose Campos summarizes what Dr. Fernando Bullettin said · not cited in answers
University of Colorado study (2019) of 52 patients found that muscle flap repair leads to lower recurrence rates compared to patch repair in CDH cases.
Host summaryCecilia Gigena summarizes what Dr. Fernando Bullettin said · not cited in answers
The lower recurrence rate with flap repair may be due to the flap's ability to grow over time with the child.
Host summaryCecilia Gigena summarizes what Dr. Fernando Bullettin said · not cited in answers
UK systematic review and meta-analysis of 986 CDH patients (226 biological patches, 760 synthetic) found biological patches had a recurrence rate of 30.3% versus 16.7% for synthetic patches.
Host summaryCecilia Gigena summarizes what Dr. Fernando Bullettin said · not cited in answers
In the UK patch study, biological patches had adhesion rates between 7-35% while synthetic patches had adhesion rates between 4-29%.
Host summaryCecilia Gigena summarizes what Dr. Fernando Bullettin said · not cited in answers
Synthetic patches were associated with chest wall deformities in 80% of cases in the UK meta-analysis.
Host summaryCecilia Gigena summarizes what Dr. Fernando Bullettin said · not cited in answers
French prospective cohort study (JPS 2022) of 762 CDH neonates found that 81 underwent fundoplication (either preventive or curative).
Host summaryCecilia Gigena summarizes what Dr. Fernando Bullettin said · not cited in answers
The French study found that preventive fundoplication during patch repair does not decrease the need for future curative fundoplication and is associated with adverse GI outcomes.
Host summaryCecilia Gigena summarizes what Dr. Fernando Bullettin said · not cited in answers
In the French fundoplication study, patients who received fundoplication had failure-to-thrive rates of 81% versus 51% in those who did not receive fundoplication.
Host summaryCecilia Gigena summarizes what Dr. Fernando Bullettin said · not cited in answers