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

doi:10.1016/j.jpedsurg.2015.09.010

Journal of Pediatric Surgery

Volume 50, Issue 11, November 2015, Pages 1958-1970

https://doi.org/10.1016/j.jpedsurg.2015.09.010 Get rights and content

Abstract

Objective

Variable management practices complicate the identification of optimal strategies for infants with congenital diaphragmatic hernia (CDH). This review critically appraises the available evidence to provide recommendations.

Methods

Six questions regarding CDH management were generated. English language articles published between 1980 and 2014 were compiled after searching Medline, Cochrane, Embase and Web of Science. Given the paucity of literature on the subject, all studies irrespective of their rank in the levels of evidence hierarchy were included.

Results

Gentle ventilation with permissive hypercapnia provides the best outcomes. Initial high frequency ventilation may be considered but its overall efficacy is unproven. Routine inhaled nitric oxide (iNO) or other medical adjuncts for acute, severe pulmonary hypertension demonstrate no benefit. Evidence does not support routine administration of pre- or postnatal glucocorticoids. Mode of extracorporeal membrane oxygenation (ECMO) has little bearing on outcomes. While the overall timing of repair does not impact outcomes, early repair on ECMO has benefits. Open repair leads to significantly fewer recurrences. Polytetrafluoroethylene (PTFE) is the most durable patch repair material.

Conclusions

Limited high-level evidence prevents the development of robust management guidelines for CDH. Prospective, multi-institutional studies are needed to identify best practices and optimize outcomes.

Section snippets

Research questions

The American Pediatric Surgical Association (APSA) Outcomes and Evidence Based Practice (OEBP) committee selected the following questions for this systematic review:

1.
What is the optimal ventilator strategy for a newborn with CDH?
2.
What is the best way to manage CDH-associated pulmonary hypertension (PHTN)?
3.
Is there benefit to the use of pre- or postnatal corticosteroids?
4.
Which mode of ECMO is best for CDH, and what is the optimal duration of treatment with ECMO?
5.
What is the optimal technique to

What is the best ventilator strategy for a newborn with CDH?

A total of 978 relevant abstracts were reviewed for this question. There was a significant lack of level 1 or 2 evidence (1 article) addressing ventilation strategies specific to the CDH population. As such, level 3 and 4 evidence (i.e. retrospective case series – 8 articles) formed the basis of this review.

Summary

This systematic review by the APSA Outcomes and Evidence-based Practice Committee attempted to delineate best-evidence recommendations for specific aspects of CDH care. Disappointingly, the quantity of high-level evidence was severely lacking, which precludes the identification of definitive best practices. Moreover, even when fair levels of evidence were available, certain practice patterns continue despite evidence showing no benefit (i.e. use of iNO). Variability in institutional practice

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Early Ventilator Management for Infants With Congenital Diaphragmatic Hernia: Impact of a Standardized Clinical Practice Guideline
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Infants with congenital diaphragmatic hernia (CDH) experience high morbidity and mortality due to pulmonary arterial hypertension and hypoplasia. Mechanical ventilation is a central component of CDH management. Our objective was to evaluate the impact of a standardized clinical practice guideline (implemented in January 2012) on ventilator management for infants with CDH, and associate management changes with short-term outcomes, specifically extracorporeal membrane oxygenation (ECMO) utilization and survival to discharge.
We conducted a retrospective pre-post study of 103 CDH infants admitted from January 2007–July 2021, divided pre- (n = 40) and post-guideline (n = 63). Clinical outcomes, ventilator settings, and blood gas values in the first 7 days of mechanical ventilation were compared between the pre- and post-guideline cohorts.
Post-guideline, ECMO utilization decreased (11% vs 38%, p = 0.001) and survival to discharge improved (92% vs 68%, p = 0.001). More post-guideline patients remained on conventional mechanical ventilation without need for escalation to high-frequency ventilation or ECMO, and had higher pressures and PaCO₂ with lower FiO₂ and PaO₂ (p < 0.05).
Standardized ventilator management optimizing pressures for adequate lung expansion and minimizing oxygen toxicity improves outcomes for infants with CDH.
III.
Evaluation of a biodegradable polyurethane patch for repair of diaphragmatic hernia in a rat model: A pilot study
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Congenital diaphragmatic hernia (CDH) repair is an area of active research. Large defects requiring patches have a hernia recurrence rate of up to 50%. We designed a biodegradable polyurethane (PU)-based elastic patch that matches the mechanical properties of native diaphragm muscle. We compared the PU patch to a non-biodegradable Gore-Tex™ (polytetrafluoroethylene) patch.
The biodegradable polyurethane was synthesized from polycaprolactone, hexadiisocyanate and putrescine, and then processed into fibrous PU patches by electrospinning. Rats underwent 4 mm diaphragmatic hernia (DH) creation via laparotomy followed by immediate repair with Gore-Tex™ (n = 6) or PU (n = 6) patches. Six rats underwent sham laparotomy without DH creation/repair. Diaphragm function was evaluated by fluoroscopy at 1 and 4 weeks. At 4 weeks, animals underwent gross inspection for recurrence and histologic evaluation for inflammatory reaction to the patch materials.
There were no hernia recurrences in either cohort. Gore-Tex™ had limited diaphragm rise compared to sham at 4 weeks (1.3 mm vs 2.9 mm, p = 0.003), but no difference was found between PU and sham (1.7 mm vs 2.9 mm, p = 0.09). There were no differences between PU and Gore-Tex™ at any time point. Both patches formed an inflammatory capsule, with similar thicknesses between cohorts on the abdominal (Gore-Tex™ 0.07 mm vs. PU 0.13 mm, p = 0.39) and thoracic (Gore-Tex™ 0.3 mm vs. PU 0.6 mm, p = 0.09) sides.
The biodegradable PU patch allowed for similar diaphragmatic excursion compared to control animals. There were similar inflammatory responses to both patches. Further work is needed to evaluate long-term functional outcomes and further optimize the properties of the novel PU patch in vitro and in vivo.
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Although rare, neonatal respiratory disease that requires surgery can have devastating consequences. Care for patients with these conditions involves a multidisciplinary team including neonatologists, pediatric surgeons, nurses, and respiratory therapists. A thorough understanding of the surgical neonatal lung diseases and their diagnostic workup and operative management is critical for all members of the team. This chapter will discuss surgical disease of the neonatal respiratory system. These include upper airway stenosis, laryngomalacia and tracheomalacia, congenital lung lesions, and the congenital defects of the diaphragm.
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Thoracoscopic CDH repair is increasingly performed for Type A and small Type B defects that are amenable to primary repair. However, the thoracoscopic approach is controversial for larger defects necessitating a patch due to technical complexity, intraoperative acidosis, and recurrence risk. We aim to compare clinical outcomes between thoracoscopic and open patch repair of Type B/C defects, using a standardized technique.
This is a single-center retrospective review of thoracoscopic and open CDH patch repairs January 2017–December 2021. We excluded primary repairs, Type D hernias, repairs on ECMO, recurrent repairs. Various preoperative, intraoperative, and postoperative variables were compared. Primary outcome was recurrence rate. Secondary outcomes included intraoperative pH and pCO₂, operative time, and complication rates.
Twenty-nine patients met inclusion criteria (open = 13, thoracoscopic = 16). The open cohort had lower o/e total fetal lung volume (29 vs 41.2%, p = 0.042), higher preoperative peak inspiratory pressures (24 vs 20 cm H₂O, p = 0.007), were more frequently Type C defects (92.3 vs 31%, p = 0.002) and had liver “up” in left-sided hernias (46 vs 0%, p < 0.0001). Intraoperatively, median lowest pH and highest pCO₂ did not differ; neither did overall median pH or pCO₂. Operative times were similar (153 vs 194 min, p = 0.113). No difference in recurrence rates was identified, however postoperative complications were higher in the open group. There were no mortalities.
Although we demonstrate higher disease severity of patients undergoing open repair, thoracoscopic patch repair for Type B/C defects is safe and effective in patients with favorable physiologic status, alleviating concerns for intraoperative acidosis, operative length, and risk of recurrence.
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Review ArticleManagement of congenital diaphragmatic hernia: A systematic review from the APSA outcomes and evidence based practice committee

Abstract

Objective

Methods

Results

Conclusions

Section snippets

Research questions

What is the best ventilator strategy for a newborn with CDH?

Summary

Semin Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr

J Pediatr Surg

J Pediatr

J Crit Care

J Pediatr Surg

J Pediatr Surg

J Am Coll Cardiol

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Am Coll Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Surg Res

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

Surgery

J Pediatr Surg

Surgery

J Pediatr Surg

Long-term maturation of congenital diaphragmatic hernia treatment results: toward development of a severity-specific treatment algorithm

Ann Surg

GRADE: an emerging consensus on rating quality of evidence and strength of recommendations

BMJ

The PRISMA statement for reporting systematic reviews and meta-analyses of studies that evaluate health care interventions: explanation and elaboration

Long-term follow-up of newborns with persistent pulmonary hypertension

Crit Care Med

Management of infants with severe respiratory failure and persistence of the fetal circulation, without hyperventilation

Pediatrics

Ventilation with lower tidal volumes as compared with traditional tidal volumes for acute lung injury and the acute respiratory distress syndrome. The Acute Respiratory Distress Syndrome Network

N Engl J Med

Management of acute lung injury and acute respiratory distress syndrome in children

Crit Care Med

Congenital diaphragmatic hernia: a systematic review and summary of best-evidence practice strategies

J Perinatol

Permissive hypercapnia in the management of congenital diaphragmatic hernia: our institutional experience

J Am Coll Surg

Elective high-frequency oscillatory ventilation versus conventional ventilation for acute pulmonary dysfunction in preterm infants

Neonatology

High frequency oscillatory ventilation versus conventional ventilation for infants with severe pulmonary dysfunction born at or near term

Cochrane Database Syst Rev

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