Morbidity in children after fetoscopic endoluminal tracheal occlusion for severe congenital diaphragmatic hernia: Results from a multidisciplinary clinic

Sferra, Shelby; Nies, Melanie; Miller, Jena L.; Garcia, Alejandro V.; Hodgman, Erica I.; Penikis, Annalise B; Engwall-Gill, Abigail J.; Burton, Vera Joanna; Rice, Jessica L.; Mogayzel, Peter J.; Baschat, Ahmet; Kunisaki, Shaun M.

doi:10.1016/j.jpedsurg.2022.09.042

Cited by 8 publications

(8 citation statements)

References 24 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A recent meta-analysis found that the survival benefit of FETO is most apparent in care settings where the fetal and neonatal therapy programs operate in an integrated institutional care setting ( 9 ). Given the higher background survival rates for CDH achieved at pediatric high volume centers the benefit of FETO may extend beyond a measurable impact on survival but also decrease respiratory ( 79 ), gastrointestinal as well as neurodevelopmental morbidity ( 80 ). Based on the current level I evidence FETO should be strongly considered for isolated severe left sided CDH, recognizing that the anticipated survival benefit may vary based on care setting and integration with the pediatric management expertise.…”

Section: Fetal Therapy For Cdh By Fetoscopic Tracheal Occlusionmentioning

confidence: 99%

Management advances for congenital diaphragmatic hernia: integrating prenatal and postnatal perspectives

Baschat,

Desiraju,

Bernier

et al. 2024

Transl Pediatr

View full text Add to dashboard Cite

In congenital diaphragmatic hernia (CDH), abdominal organs are displaced into the chest, compress the lungs, and cause mediastinal shift. This contributes to development of pulmonary hypoplasia and hypertension, which is the primary determinant of morbidity and mortality for affected newborns. The severity is determined using prenatal imaging as early as the first trimester and is related to the laterality of the defect, extent of lung compression, and degree of liver herniation. Comprehensive evaluation of fetal CDH includes imaging-based severity assessment, severity assessment, and evaluation for structural or genetic abnormalities to differentiate isolated from complex cases. Prenatal management involves multispecialty counseling, consideration for fetal therapy with fetoscopic endoluminal tracheal occlusion (FETO) for severe cases, monitoring and intervention for associated polyhydramnios or signs of preterm labor if indicated, administration of antenatal corticosteroids in the appropriate setting, and planned delivery to optimize the fetal condition at birth. Integrated programs that provide a smooth transition from prenatal to postnatal care produce better outcomes. Neonatal care involves gentle ventilation to avoid hyperinflation and must account for transitional physiology to avoid exacerbating cardiac dysfunction and decompensation. Infants who have undergone and responded to FETO have greater pulmonary capacity than expected, but cardiac dysfunction seems unaffected. In about 25–30% of CDH neonates extracorporeal life support is utilized, and this provides a survival benefit for patients with the highest predicted mortality, including those who underwent FETO. Surgical repair after initial medical management for the first 24–48 hours of life is preferred since later repair is associated with delayed oral feeding, increased need for tube feeds, and increased post-repair ventilation requirement and supplemental oxygen at discharge. With overall survival rates >70%, contemporary care involves management of chronic morbidities in the context of a multidisciplinary clinic setting.

show abstract

Section: Fetal Therapy For Cdh By Fetoscopic Tracheal Occlusionmentioning

confidence: 99%

Management advances for congenital diaphragmatic hernia: integrating prenatal and postnatal perspectives

Baschat,

Desiraju,

Bernier

et al. 2024

Transl Pediatr

View full text Add to dashboard Cite

show abstract

“…Infants with CDH from all racial and ethnic categories were admitted to the ICU for a comparable number of days ( [SD] 14 [28] days vs Black: 18 [37] days; difference, −5 days; 95% CI for difference −9 to −.2; P = .04).…”

Section: Hospital Coursementioning

confidence: 99%

“…Failed embryonic closure of the diaphragm in affected fetuses results in intrathoracic abdominal viscera herniation and varying degrees of pulmonary hypoplasia, pulmonary hypertension, and cardiac dysfunction at birth . Prenatally diagnosed cases require increased surveillance and, in the most severe cases, fetal endoscopic tracheal occlusion has been shown to improve survival with acceptable long-term morbidity . In the majority of patients, survival is improved with delivery at high-volume, designated CDH centers that have a multidisciplinary care approach and access to adjuvant therapies, including extracorporeal life support (ECLS) .…”

Section: Introductionmentioning

confidence: 99%

“… 24 , 25 , 26 Prenatally diagnosed cases require increased surveillance and, in the most severe cases, fetal endoscopic tracheal occlusion has been shown to improve survival with acceptable long-term morbidity. 27 , 28 In the majority of patients, survival is improved with delivery at high-volume, designated CDH centers that have a multidisciplinary care approach and access to adjuvant therapies, including extracorporeal life support (ECLS). 29 , 30 Despite such intensive care, the overall neonatal mortality rate in CDH approaches 25% to 30%.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Racial and Ethnic Disparities in Outcomes Among Newborns with Congenital Diaphragmatic Hernia

et al. 2023

Self Cite

View full text Add to dashboard Cite

ImportanceThere is some data to suggest that racial and ethnic minority infants with congenital diaphragmatic hernia (CDH) have poorer clinical outcomes.ObjectiveTo determine what patient- and institutional-level factors are associated with racial and ethnic differences in CDH mortality.Design, Setting, and ParticipantsMulticenter cohort study of 49 US children’s hospitals using the Pediatric Health Information System database from January 1, 2015, to December 31, 2020. Participants were patients with CDH admitted on day of life 0 who underwent surgical repair. Patient race and ethnicity were guardian-reported vs hospital assigned as Black, Hispanic (White or Black), or White. Data were analyzed from August 2021 to March 2022.ExposuresPatient race and ethnicity: (1) White vs Black and (2) White vs Hispanic; and institutional-level diversity (as defined by the percentage of Black and Hispanic patients with CDH at each hospital): (1) 30% or less, (2) 31% to 40%, and (3) more than 40%.Main Outcomes and MeasuresThe primary outcomes were in-hospital and 60-day mortality. The study hypothesized that hospitals managing a more racially and ethnically diverse population of patients with CDH would be associated with lower mortality among Black and Hispanic infants.ResultsAmong 1565 infants, 188 (12%), 306 (20%), and 1071 (68%) were Black, Hispanic, and White, respectively. Compared with White infants, Black infants had significantly lower gestational ages (mean [SD], White: 37.6 [2] weeks vs Black: 36.6 [3] weeks; difference, 1 week; 95% CI for difference, 0.6-1.4; P &lt; .001), lower birthweights (White: 3.0 [1.0] kg vs Black: 2.7 [1.0] kg; difference, 0.3 kg; 95% CI for difference, 0.2-0.4; P &lt; .001), and higher extracorporeal life support use (White: 316 patients [30%] vs Black: 69 patients [37%]; χ21 = 3.9; P = .05). Black infants had higher 60-day (White: 99 patients [9%] vs Black: 29 patients [15%]; χ21 = 6.7; P = .01) and in-hospital (White: 133 patients [12%] vs Black: 40 patients [21%]; χ21 = 10.6; P = .001) mortality . There were no mortality differences in Hispanic patients compared with White patients. On regression analyses, institutional diversity of 31% to 40% in Black patients (hazard ratio [HR], 0.17; 95% CI, 0.04-0.78; P = .02) and diversity greater than 40% in Hispanic patients (HR, 0.37; 95% CI, 0.15-0.89; P = .03) were associated with lower mortality without altering outcomes in White patients.Conclusions and RelevanceIn this cohort study of 1565 who underwent surgical repair patients with CDH, Black infants had higher 60-day and in-hospital mortality after adjusting for disease severity. Hospitals treating a more racially and ethnically diverse patient population were associated with lower mortality in Black and Hispanic patients.

show abstract

“…The technique of FETO involves the insertion of an inflatable balloon into the fetal trachea4 to achieve temporary tracheal occlusion. Two RCTs7 8 and many observational studies and systematic reviews9–12 have reported the benefits of FETO, although some experts have cautioned against the widespread implementation of FETO in Extracoporeal membrane oxygenation (ECMO) centres because aggressive postnatal management with early ECMO can achieve very good outcomes even in severe CDH 13…”

Section: Introductionmentioning

confidence: 99%

Prevalence of symptomatic tracheal morbidities after fetoscopic endoluminal tracheal occlusion: a systematic review and meta-analysis

Tho,

Rath,

Tan

et al. 2023

Arch Dis Child Fetal Neonatal Ed

View full text Add to dashboard Cite

BackgroundFetoscopic endoluminal tracheal occlusion (FETO) has been shown to improve survival of infants with congenital diaphragmatic hernia (CDH). However, there are concerns that FETO may lead to tracheomegaly, tracheomalacia and related complications.MethodsA systematic review was conducted to estimate the prevalence of symptomatic tracheal complications in infants who underwent FETO for CDH. Presence of one or more of the following was considered as tracheal complication: tracheomalacia, stenosis, laceration or tracheomegaly with symptoms such as stridor, effort-induced barking cough, recurrent chest infections or the need for tracheostomy, tracheal suturing, or stenting. Isolated tracheomegaly on imaging or routine bronchoscopy without clinical symptoms was not considered as tracheal morbidity. Statistical analysis was performed using the metaprop command on Stata V.16.0.ResultsA total of 10 studies (449 infants) were included (6 retrospective cohort, 2 prospective cohort and 2 randomised controlled trials). There were 228 infants who survived to discharge. Prevalence rates of tracheal complications in infants born alive were 6% (95% CI 2% to 12%) and 12% (95% CI 4% to 22%) in those who survived to discharge. The spectrum of severity ranged from relatively mild symptoms such as effort-induced barking cough to the need for tracheostomy/tracheal stenting.ConclusionA significant proportion of FETO survivors have symptomatic tracheal morbidities of varying severity. Units that are planning to adopt FETO for managing CDH should consider ongoing surveillance of survivors to enable early identification of upper airway issues. Inventing FETO devices that minimise tracheal injury is needed.

show abstract

Morbidity in children after fetoscopic endoluminal tracheal occlusion for severe congenital diaphragmatic hernia: Results from a multidisciplinary clinic

Cited by 8 publications

References 24 publications

Management advances for congenital diaphragmatic hernia: integrating prenatal and postnatal perspectives

Management advances for congenital diaphragmatic hernia: integrating prenatal and postnatal perspectives

Racial and Ethnic Disparities in Outcomes Among Newborns with Congenital Diaphragmatic Hernia

Prevalence of symptomatic tracheal morbidities after fetoscopic endoluminal tracheal occlusion: a systematic review and meta-analysis

Contact Info

Product

Resources

About