Congenital Diaphragmatic Hernia - Resuscitation and Stabilisation

Publication: 01/08/2003  --
Last review: 11/03/2020  
Next review: 06/03/2023  
Clinical Guideline
CURRENT 
ID: 223 
Approved By:  
Copyright© Leeds Teaching Hospitals NHS Trust 2020  

 

This Clinical Guideline is intended for use by healthcare professionals within Leeds unless otherwise stated.
For healthcare professionals in other trusts, please ensure that you consult relevant local and national guidance.

Congenital Diaphragmatic - Hernia Resuscitation & Stabilisation

  1. Antenatal
  2. Stabilisation @ Delivery Suite
  3. Stabilisation @ NICU
  4. Surgical Repair
  5. Post-Operative Care

I. Antenatal

  • Consultant neonatologist/ Paediatric surgeon to undertake antenatal counselling. Offer tour of Neonatal unit at this appointment.

                                
    • Document associated major anomalies
        ◦ Arrange fetal ECHO
        ◦ offer genetic testing

    • Consider antenatal steroids < 37 weeks gestation

    • Plan delivery (morning if possible)
        ◦ enables all relevant care providers to be present at birth and to optimise immediate neonatal management

    • Consultant Neonatologist to be informed of planned delivery

 

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II. Stabilisation @ Delivery Suite

Do NOT undertake delayed cord clamping

 Ensure Neonatal consultant aware/present at delivery

    • Immediate intubation by the most senior doctor or ANNP - Do not use face mask ventilation - carefully secure airway with Neofit

        ◦ if unable to intubate consider the use of a short acting muscle relaxant (e.g atracurium) by IM injection and re-attempt intubation.
 
If deteriorating / bradycardic then use an I-Gel to direct air into lungs
•           If I-Gel and intubation are not possible then allow infant to wake up and breathe spontaneously or support breathing with mask ventilation breaths and aspirate air via large bore NGT.

  • Do you need more senior help!

       
        ◦ Gentle inflation pressures <25 cm H2O
               ◦ contralateral pneumothorax is recognised complication of over-vigorous resuscitation.
               ◦ Aim for a raise in heart rate and some chest movement rather than pink baby
     • Attach saturation probe pre-ductally.

         ◦  Follow Resuscitation Council guidance for oxygen delivery during stabilisation. Have a low threshold for increasing to 100% oxygen.

◦ Do not turn pressures up to achieve higher saturations if preductal saturations are >80%  and heart rate is normal. Aim for pre ductal saturations 85-95%
(Higher saturations are acceptable as long as they can be achieved with pressure not exceeding 25 cm H20

    • Insert wide bore NG tube (8Fg/10Fg) and decompress stomach with 50ml syringe

    • maintain normal temperature - check at 10 minutes of age.

Update parents
Transfer to NICU as soon as possible

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III. Stabilisation @ NICU

Commence conventional ventilation as per ventilation section

Consider the use of end tidal/transcutaneous CO2 monitoring

Central venous access – UAC and double lumen UVC

Consider the use of NIRS monitoring

Commence Sedation, consider paralysis

Maintain mean BP in normal range for gestation

Chest X-ray ASAP

• Ventilation
         ◦   Aim for pH >7.25
• start conventional ventilation
         ◦   Nonaggressive gentle ventilation improves outcome
    • PCAC - Do not use Volume guarantee (Vg). Tidal volume will usually be < 4mls/kg

Aim for lowest pressures that allow adequate ventilation:
       
         PIP ≤ 24cm H2O PEEP 5cm H2O
    •Rate - 40-60/min
•O2
    • Pre and Post ductal saturation monitoring

       Aim for pre ductal saturations 85-95%, post ductal saturations >70%

       Reduce pressure or FiO2 if pre-ductal saturations are >95%

If over ventilated the PIP should be reduced in the first instance

Do not routinely give surfactant, unless <34 weeks
         ◦   consider muscle relaxation only if infant not settled with sedation or fighting ventilator

•High Frequency Oscillatory Ventilation (HFOV)
Consider early change to HFOV

    • I.e. when needing PIP > 24 cm H2O
    • Start with "gentle" settings, the Drager babylog VN500 will automatically choose reasonable settings

        ◦ Oxygen as required maintaining target saturations
        ◦ MAP 12-15 cm H2O aim for MAP ≤ 16 cm H2O
        ◦ ΔP 30-40 (ensure adequate chest wall wobble)
        ◦ Frequency 10 Hz
    • do not attempt to normalise blood gas results by using excessive ventilation parameters

Perform chest X-ray an hour post initiation of HFOV to assess for lung expansion.
Continue to utilise chest X-Rays regularly to assess for expansion avoiding over distension

• Adjustments in HFOV
        ◦ Low O2     -   reduce MAP rather than reducing FiO2
          High O2    -      increase oxygen before increasing MAP

        ◦ MAP  -   aim for PaO2 6 - 8 kPa with pre-ductal saturations > 85 %,
                          Increase/decrease in steps of 1cm H2O
                          aim to avoid MAP > 16 cm H2O

        ◦ ΔP     -    allow permissive hypercapnia but keep pH > 7.25
                          Increase/decrease in steps of 2cm H20
                          avoid PaCO2 < 5 kPa

        ◦ Frequency -   reduce Hz to 8 in term infants if no improvement (Consultant decision)

Pulmonary Vascular Management
    • Commence Inhaled Nitric Oxide (iNO):
        ◦ As per unit protocol       
  ◦ If preductal PaO2 < 90% in 100% oxygen

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        ◦ Oxygen Index >20
To calculate oxygen index
Oxygen Index (OI) =Mean airway pressure (cm of water) x FiO2 (%)
                                                      PaO2(kPa)x 7.5
        ◦ start at 20ppm

    • If there is insufficient response to iNO then provide treatment inotropes and volume to push the mean blood pressure to >50mmHg (term) as for PPHN as per PPHN guideline, with aim of reversing the right to left shunting.
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    • Correct:
        ◦ Acidosis: keep pre-ductal pH > 7.25
        ◦ Polycythaemia
        ◦ Hypoglycaemia
        ◦ hypocalcaemia
        ◦ hypothermia

Consider referral to ECMO centre - if not responding to treatment or if OI >40 (or > 30 on nitric oxide and HFOV). A normal cranial ultrasound scan and echo will be required at time of referral. Note oxygenation index should be calculated using preductal blood from right radial artery.

  The Heartlink ECMO Centre at Glenfield Hospital                0300 300 3200
 
Blood and Access
    • Insert UVC + UAC (if UAC unsuccessful: a right radial art (preductal) should be attempted hyperlink-
detail.aspx?ID=5483

    • Bloods
        ◦ FBC, group + save, blood glucose, Blood gas, CRP and blood cultures if risk factors for sepsis
        ◦ Consider genetic testing (if not done antenatal). Discuss with Neonatal consultant, prior to consenting parents.

    • Loading dose of morphine then continue a maintenance dose

    • Benzylpenicillin + Gentamicin if there is suspected sepsis detail.aspx?ID=3688

Fluid Management:
    • NBM
    • Maintenance fluids @ 60 ml/kg/day on Day 1 follow Fluid protocol for on-going management
    • Measure urine output, fluid balance
    • Optimise cardiac output with normal saline bolus (10 ml/kg) if required
        ◦ No more than 20mls/kg bolus in total

After stabilisation:

• Document any dysmorphic features
• Minimal handling - quiet environment, low lighting, minimal handling
• Inform surgical team
• Cardiology
                    ◦ early request for echo to exclude congenital heart disease
                    ◦ many infants have had a full cardiac antenatal echo (detailed in    maternal notes)
                    ◦ Request urgent cardiology opinion if there is deterioration after initial period of stability
                        ◦assess RV function / PA pressure

                        ◦ identify ductus / assess shunting

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IV. Surgical Repair

• Optimal timing
                    ◦ Wait for Reduction in pulmonary vascular resistance documented by   ECHO
                    ◦ Satisfactory conventional ventilation with moderate PIP & FiO2 settings
           
 • Contra-indication
                    ◦ Irreversible pre-ductal hypoxemia and hypercarbia
                     Evidence of supra-systemic pulmonary hypertension

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V. Post-Operative Care

    • Early post-op chest X-ray
        ◦ will always show some residual pneumothorax- - this is just an air pocket and does not need draining
        ◦ Document progressive reduction of air space size on subsequent films

    • FBC, U&Es, Calcium, Phosphate, Blood glucose, blood gas

        ◦ Most babies will have difficulties with feeding due to diaphragmatic splinting (paralytic ileus, intestinal distension, small abdominal cavity).

    • Conventional ventilation
        ◦ Low threshold for initiation/ re-initiation of HFOV
        ◦ avoid hyperinflation of hypoplastic lung

    • BP maintain normal parameters unless otherwise indicated
        ◦ fluid balance - monitor urine output

    • Other
        ◦ Morphine Maintenance 10-30micrograms/kg.hr  see Pain Protocol
        ◦ Benzylpenicillin/Gentamicin as per surgical advice usually 48hrs
        ◦ paralysis for 24 hours only

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Provenance

Record: 223
Objective:
Clinical condition:
Target patient group: Babies
Target professional group(s): Secondary Care Doctors
Secondary Care Nurses
Adapted from:

N/A


Evidence base

  1. Field D, Hyman-Taylor P, Bacon C, Draper ES on behalf of MBRRACE-UK. Perinatal Confidential Enquiry – Congenital Diaphragmatic Hernia Leicester: The Infant Mortality and Morbidity Group. Department of Health Sciences. University of Leicester 2014
  2. Kotecha S, Barbato A, Bush A, Claus F, Davenport M, Delacourt C, Deprest J, Frenckner B, Greenough A, Nicholson AG, Anton-Pacheco JL, Midulla F. (2012) ERS Task force Report Congenital Diaphragmatic Hernia. European Respiratory Journal. 2012 39:820-829
  3. Snoek KG, Reiss IKM, Greenough A, Capolupo I, Urlesberger B,Wessel L, Tibboel D. (2016) Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe: The CDH EURO consortium consensus – 2015 update. Neonatology, 110: 66-74
  4. Putnam LR, Tsao K, Morini F, Lally PA, Miller CC, Lally KP, Harting MT (2016) Evaluation of variability of inhaled nitric oxide use and pulmonary hypertension in patients with congenital diaphragmatic hernia. JAMA Pediatrics 170(12): 1-7
  5. Guidry CA, Hranjec T, Rodgers BM, Kane B, McGahren ED. (2012) Permissive hypercapnia in the management of congenital diaphragmatic hernia: our institutional experience. J Am Coll Surg 214:640-645
  6. Long AM, Bunch KJ, Knight M, Kurinczuk JJ, Losty PD, (2018) Early population based outcomes of infants born with congenital diaphragmatic hernia. Arch Dis Child Fetal Neonatal Ed. Nov;103(6): F517-F522
  7. National Care Principles for the Management of Congenital Diaphragmatic    Hernia (2018) A BAPM Framework for practice. Available from: https://www.bapm.org/resources/22-national-care-principles-for-the-management-of-congenital-diagphragmatic-hernia-2018

Document history

LHP version 2.0

Related information

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