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Management and Treatment of Congenital Diaphragmatic Hernia - Neonatal - Inpatient

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1
Management and Treatment of
Congenital Diaphragmatic Hernia -
Neonatal - Inpatient
Clinical Practice Guideline
Note: Active Table of Contents – Click to follow link
Table of Contents
EXECUTIVE SUMMARY ........................................................................................................... 3
SCOPE ................................................................................................................................... 5
METHODOLOGY .................................................................................................................... 5
INTRODUCTION ..................................................................................................................... 7
RECOMMENDATIONS ............................................................................................................ 7
Delivery Room Management and Transfer to AFCH ............................................................ 7
Preoperative Management .............................................................................................. 10
Nursing Care and Room Setup ........................................................................................................................... 10
Ventilator Management ..................................................................................................................................... 12
Acute Pulmonary Hypertension (PHTN) and Cardiac Management .................................................................. 14
Pharmacotherapy ............................................................................................................................................... 16
Extracorporeal membrane oxygenation (ECMO) ............................................................................................... 17
Surgery ............................................................................................................................ 17
Postoperative Care .......................................................................................................... 18
Special Considerations Specific to the Post-operative Period ........................................................................... 19
Weaning Phase ................................................................................................................................................... 19
Pulmonary Hypertension ................................................................................................................................... 21
Discharge Planning .......................................................................................................... 22
UW HEALTH IMPLEMENTATION ........................................................................................... 23
APPENDIX A. HIGH RISK PERINATAL CLINIC CARE PLAN ....................................................... 24
REFERENCES ........................................................................................................................ 30
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2
CPG Contact for Content:
Name: Charles Leys, MD- Surgery- Pediatric Surgery
Phone Number: (608) 263-9419
Email Address: leys@surgery.wisc.edu

CPG Contact for Changes:
Name: Lindsey Spencer, MS- Center for Clinical Knowledge Management (CCKM)
Phone Number: (608) 890-6403
Email Address: lspencer2@uwhealth.org

Coordinating Team Members:
Michael Wilhelm, MD- Pediatrics- ICU
David McCulley, MD- Pediatrics- Neonatology
C. Lydia Wraight, MD- Pediatrics- Neonatology
Jamie Limjoco, MD- Pediatrics- Neonatology
Hau Le, MD- Surgery- Pediatric Surgery
Ben Walker, MD- Anesthesiology- General
Adam Bauer, MD- Pediatrics
Jane Harney-Ralphe, NP- Pediatrics- Neonatology
Laura Konkol, MSN, RN- Nursing- Neonatal ICU
Angela Baker, MS, RNC-NIC- Nursing- Neonatal ICU
Megan Berge, BSN, RNC-NIC- Nursing- Neonatal ICU
Deb Soetenga, MS, RN, CNNS- Pediatric Intensive Care
Susan Quamme, RN- Pediatric Intensive Care
Kelly Pfeil, RN- Pediatric Intensive Care
Rhonda Yngsdal-Krenz, RT- Respiratory Therapy- AFCH
Laura Bodine, MS, RD, CNSC, CD- Clinical Nutrition- AFCH
Amy Hood, MPH, RD, CNSC, CD- Clinical Nutrition- AFCH
Anne Rose, PharmD- Pharmacy- Inpatient Services
Josh Vanderloo, PharmD- Drug Policy Program
Suzanne Hoffman, MT, ASCP- Clinical Labs- Hem/Special Coagulation
Jennifer Grice, PharmD, BCPS- Center for Clinical Knowledge Management (CCKM)

Review Individuals/Bodies:
Kara Gill, MD- Radiology- General
Petros Anagnostopoulos, MD- Surgery- Cardiothoracic
Luke Lamers, MD- Pediatrics- Cardiology
Peter Nichol, MD- Surgery- Pediatric Surgery
Sushant Srinivasan, MD- Pediatrics- ICU

Committee Approvals/Dates:
Clinical Knowledge Management (CKM) Council (Last Periodic Review: 06/23/2016)

Release Date: June 2016 | Next Review Date: June 2018

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3
Executive Summary
Guideline Overview
This guideline outlines evidence-based recommendations related to the management of
neonatal patients with congenital diaphragmatic hernia transferred to the UW Health American
Family Children’s Hospital. It is based primarily on the systematic review conducted by the
American Pediatric Surgical Association (APSA), as well as local discussions of the published
literature and past clinical experiences.1

Key Practice Recommendations
1. Transfer to AFCH should occur ideally between 6-8 hours, but no later than 12 hours after
delivery. (UW Health Very low quality evidence, weak/conditional recommendation)
2. Minimal stimulation should be maintained. (UW Health Very low quality evidence, strong
recommendation) Trainee exams (other than fellow) should only be performed when
accompanied by a bedside RN or attending exam. Minimal repositions to relieve pressure
points should be performed, while holding and bathing are not allowed until the patient is
stable post-operatively. Bedding changes should also be delayed until the patient is stable.
3. All babies should be well sedated from birth (minimally responsive to noxious stimuli, i.e.,
State Behavioral Scale (SBS) score -2 or Neonatal Pain Agitation and Sedation Scale (N-
PASS) score of -2 to -5). (UW Health Low quality evidence, strong recommendation)
4. The recommended general philosophy or approach to ventilator management focuses on
minimizing lung injury related to high pressure and oxygen toxicity, while tolerating mild
hypoxemia and moderate hypoventilation during the pre-operative period.2,3 Careful
monitoring is recommended to detect and respond to early evidence of worsening
respiratory status. The primary goal is to avoid the need for dramatic increases or decreases
in support. Current evidence does not support one ventilator mode over another, however
due to overall clinical experience, conventional ventilation is recommended as the initial
mode of ventilation.1,4 (UW Health High quality evidence, weak/conditional recommendation)
a. Permissive hypercarbia should be tolerated as long as the patient is comfortable.
The pH/PCO2 target range is pH ≥ 7.25 and PCO2 45-65 mmHg. Higher PCO2 levels
should be tolerated during the initial transition period and intermittently during the
pre-operative period. (UW Health Low quality evidence, weak/conditional recommendation)
b. If CO2 is elevated and patient is not critically desaturated (SpO2 < 75-80%), do not
change CO2 rapidly. (UW Health Very low quality evidence, strong recommendation) Rapid
PCO2 changes may result in acute changes in cerebral blood flow, particularly with
disrupted cerebral autoregulation present in a sick newborn.
c. Pre-ductal saturations should be maintained between 90 and 95%. (UW Health Low
quality evidence, strong recommendation) Post-ductal saturations as low as 70 may be
acceptable (and even lower if urine output remains reasonable and lactate low). (UW
Health Low quality evidence, weak/conditional recommendation)
d. Prior to increasing respiratory support it is critical to note changes in respiratory
pattern from baseline (UW Health Low quality evidence, strong recommendation):
i. Paradoxical chest wall movement
ii. Significant increase in retractions
iii. Preductal SaO2 < 80%
iv. pCO2 > 65 mmHg (pH < 7.25)
v. Sustained preductal to postductal SaO2 difference >10% indicate PHTN and
should prompt investigation (see pulmonary hypertension section).
e. Use of high frequency oscillatory ventilation (HFOV) may be considered as an initial
mode of ventilation for any of the following: failure to improve over 30 minutes on
conventional ventilation (PIP > 25 cm H2O and Rate > 60) while maximizing
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sedation, positioning, and pulmonary toilette; failure to oxygenate despite use of
conventional ventilation in conjunction with iNO; or for pneumothorax.1,5 (UW Health
Very low quality evidence, weak/conditional recommendation)
5. Inhaled nitric oxide (iNO) should be used as first line treatment for acute pulmonary
hypertension with a suggested starting dose of 20 ppm.6-11 (UW Health High quality evidence,
weak/conditional recommendation) Reassessment should occur within 30-60 of iNO
initiation. If no improvement, patient should be weaned off iNO quickly. It is
recommended to wean iNO when pulmonary hypertension and RV function have improved,
or after FiO2 has been weaned to 0.4-0.55. (UW Health Very low quality evidence, strong
recommendation)
6. There are no specific criteria for ECMO in CDH patients; however the following indications
and contraindications may be used to determine eligibility. Patients who are unable to
maintain adequate oxygen delivery with reasonable ventilator settings should be considered
for ECMO.1,12 (UW Health Low quality evidence, weak/conditional recommendation)
7. The timing of surgery should be based upon each individual clinical scenario, risks and
benefits of the procedure, and professional clinical judgement of the surgical staff. (UW
Health Low quality evidence, weak/conditional recommendation) Although the level of evidence is
low, repair of CDH within 72 hours on ECMO can be considered, as it may improve outcome
and decrease time on ECMO.1,13 (UW Health Low quality evidence, weak/conditional
recommendation)
8. Ventilator support may need to be increased in the immediate post-op period in order to
regain stability; however, the main objective remains gentle ventilation (just as in
preoperative period).
9. Care management by a multidisciplinary team for follow-up after surgery is recommended.14
(UW Health Low quality evidence, strong recommendation) The following imaging studies are
recommended no later than 2 weeks prior to discharge: echocardiogram, chest x-ray, and
brain MRI.14

Companion Documents
1. High Risk Perinatal Clinic Care Plan
2. Congenital Diaphragmatic Hernia Bedside Reference Card

Collateral UW Health Guidelines
1. Neonatal Analgesia – Neonatal – Inpatient/Ambulatory Guideline
2. Enteral Nutrition – Neonatal – Inpatient Guideline
3. Parenteral Nutrition – Pediatric/Neonatal – Inpatient/Ambulatory Guideline
4. Management of Unfractionated Heparin in ECMO – Pediatric/Neonatal – Inpatient Guideline
5. Epoprostenol Inhaled – Adult/Pediatric/Neonatal – Inpatient Guideline
6. Prevention of Ventilator Associated Pneumonia – Pediatric/Neonatal – Inpatient Guideline


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Scope
Disease/Condition(s): Congenital diaphragmatic hernia (CDH)

Clinical Specialty: General Surgery, Neonatology, Critical Care, Pharmacy, Nursing

Intended Users: Physicians, Advanced Practice Providers, Nursing, Pharmacists, Respiratory
Therapists

Objective(s): To outline evidence-based recommendations and reduce practice variation in the
management of patients with congenital diaphragmatic hernia transferred to the American
Family Children’s Hospital.

Target Population: Neonatal patients with congenital diaphragmatic hernia.

Interventions and Practices Considered:
ξ Surgical repair
ξ Extracorporeal membrane oxygenation (ECMO)

Major Outcomes Considered:
ξ Mortality rates
ξ ECMO requirements

Methodology
Methods Used to Collect/Select the Evidence:
Electronic database searches (e.g., PUBMED) were conducted by the guideline author(s) and
workgroup members to collect evidence for review. Expert opinion and clinical experience were
also considered during discussions of the evidence.

Methods Used to Formulate the Recommendations:
The workgroup members agreed to adopt recommendations developed by external
organizations and/or arrived at a consensus through discussion of the literature and expert
experience. All recommendations endorsed or developed by the guideline workgroup were
reviewed and approved by other stakeholders or committees (as appropriate).

Methods Used to Assess the Quality of the Evidence/Strength of the Recommendations:
Recommendations developed by external organizations maintained the evidence grade
assigned within the original source document and were adopted for use at UW Health.

Internally developed recommendations, or those adopted from external sources without an
assigned evidence grade, were evaluated by the guideline workgroup using an algorithm
adapted from the Grading of Recommendations Assessment, Development and Evaluation
(GRADE) methodology (see Figure 1).

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6
Figure 1. GRADE Methodology adapted by UW Health

Rating Scheme for the Strength of the Evidence/Recommendations:

GRADE Ranking of Evidence
High We are confident that the effect in the study reflects the actual effect.
Moderate We are quite confident that the effect in the study is close to the true effect, but it
is also possible it is substantially different.
Low The true effect may differ significantly from the estimate.
Very Low The true effect is likely to be substantially different from the estimated effect.

GRADE Ratings for Recommendations For or Against Practice
Strong The net benefit of the treatment is clear, patient values and circumstances are
unlikely to affect the decision.
Weak/conditional Recommendation may be conditional upon patient values and preferences, the
resources available, or the setting in which the intervention will be implemented.

Recognition of Potential Health Care Disparities: Hospital survival for congenital
diaphragmatic hernia was related to sex, birth weight, race and socioeconomic status in a
retrospective analysis of 2,774 hospitalizations using the Kids’ Inpatient Database.15 Higher
survival rates were seen in males, patients with a birth weight > 3 kg, white patients, patients
with private insurance, and patients in the highest median household income quartile. Black
race and other non-Hispanic minorities were identified as independent predictors of mortality.
Clinical staff and providers should remain aware of these statistics, and work to provide the best
appropriate care to all patients, regardless of gender, race, or socioeconomic status.





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Introduction
Congenital diaphragmatic hernia (CDH) is a congenital condition that affects 1 in every 2,000-
3,000 infants each year.16 Patients are born with a hole in their diaphragm that allows the
abdominal organs to move into the chest, causing pulmonary hypoplasia. This condition
requires surgical repair of the diaphragm and the detailed coordination of care between
neonatology, intensive care, and surgery.
Recommendations
DELIVERY ROOM MANAGEMENT AND TRANSFER TO AFCH
General Philosophy
Delivery room and early newborn care should focus on establishing stability on the ventilator,
providing medication for sedation and pain control, decompressing the bowel, and placement of
venous and arterial access.

Resuscitation Plan
1. Delivery room and early newborn management plan (for template see Appendix A) should
be placed in maternal chart following prenatal consult visit.

2. Orotracheal intubation should occur immediately after birth, and should be performed by an
experienced provider (e.g., senior fellow, attending).17,18 (UW Health Very low quality evidence,
strong recommendation) Bag-mask ventilation should be avoided as it may cause
distension of the stomach which limits lung expansion.17,18 (UW Health Very low quality
evidence, strong recommendation) If bag-mask is required, cricoid pressure should be used.

3. Gastric decompression should be performed to allow for lung expansion using 10F replogle
and 30 ml syringe in the delivery room. (UW Health Very low quality evidence, weak/conditional
recommendation)

4. Patient should be kept warm during transport from delivery room to NICU using warm
blankets. (UW Health Very low quality evidence, strong recommendation)

Neonatology Care Plan
1. It is recommended that an experienced provider (e.g., senior fellow, attending) perform all
procedures/interventions. (UW Health Very low quality evidence, strong recommendation)

2. Emotional support should be provided to the parents and family. (UW Health Moderate quality
evidence, strong recommendation) Consider a request for spiritual care/baptism as able prior to
transport.

3. Patients should be placed on servo control of 36.5°C on radiant warmer. A skin temperature
probe is recommended to periodically assess temperature. (UW Health Very low quality
evidence, strong recommendation)

4. To minimize persistent newborn pulmonary hypertension (PPHN) and stabilize for transport,
clinical staff should:
a. Minimize handling and patient stimulation from noises and light. (UW Health Very low
quality evidence, strong recommendation)
b. Maintain mean arterial pressures 40-50 mmHg and consider dopamine (suggested
starting dose 5 mcg/kg/min) if lower.17
c. Consider administration of inhaled nitric oxide (iNO) as needed for oxygen index (OI)
> 25. (UW Health High quality evidence, weak/conditional recommendation)The suggested
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8
starting dose is 20 ppm.6-11 Time of initiation, indication for use, and patient
response should be clearly documented.

5. The following labs are recommended (UW Health Very low quality evidence, strong
recommendation):
a. Arterial blood gas (Blood gas parameters: pH > 7.20, PCO2 < 65 mmHg)
b. Lactate (include lactate levels with all blood gas draws)
c. Glucose
d. Blood Culture
e. Complete Blood Count (CBC) with manual differential

Ventilator Management
1. Position the patient’s head toward the defect to optimize inflation of “good lung” (i.e. position
with affected side slightly down and head turned toward side of diaphragmatic defect). (UW
Health Very low quality evidence, strong recommendation)

2. Pre and post ductal saturations should be monitored. (UW Health Very low quality evidence,
strong recommendation) Probe placement should be on right upper extremity and either lower
extremity.

3. Gentle ventilation is recommended with tolerance for hypercapnea (PCO2 < 65 mmHg) and
hypoxemia (pre-ductal saturation > 85%).17,18 (UW Health Very low quality evidence, strong
recommendation) In the first 1 – 2 hours saturations of 75 – 85% are acceptable.18 The goal
is to inflate the lungs, but minimize exposure to high pressure with preference for high rate
over high pressure. The following ventilator settings can be used17,18:
a. PIP 15 – 25 cm H2O
b. PEEP 3 – 5 cm H2O
c. Rate 40 – 60 breaths/minute
d. iT 0.3 – 0.5
e. FiO2 – 1.00. Wean FiO2 slowly for pre-ductal saturation > 95%
4. Move to high frequency oscillatory ventilation (HFOV) if PIP > 25 cm H2O.1 Initial settings
are described in further detail in the preoperative management section. (UW Health Very low
quality, weak recommendation)
5. Routine use of surfactant is not recommended, but can be considered in patients with
RDS/prematurity (< 36 weeks gestational age).2,17-19 (UW Health Low quality evidence,
weak/conditional recommendation)
Lines/Drains/Tubes
1. Orotracheal intubation should be maintained on arrival to the NICU.17 (UW Health Very low
quality evidence, strong recommendation)

2. The OG/NG tube should be placed on low intermittent suction immediately.17 (UW Health
Very low quality evidence, strong recommendation)

3. Umbilical catheter access should be placed on arrival to the NICU. (UW Health Very low
quality evidence, strong recommendation)
a. Umbilical artery catheter (UAC) should be single-Lumen (3.5F for preterm, 5F for
term) with a depth: 3x + 9, x= birth weight in kg. The goal position is T6-8; however,
a less desirable alternative is a low-lying line at L3-4. (UW Health Very low quality
evidence, weak/conditional recommendation)
i. Start Amino acids with heparin 0.5 units/mL at 1mL/hr
b. Umbilical venous catheter (UVC) should be double-lumen (3.5F for patients < 1.2 kg, 5F
for patients > 1.2 kg) with a depth: 1.5x + 6, x= birth weight in kg.
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9
i. If position cannot be adequately verified, consider use as low-lying line for
administration of maintenance fluids and emergent medications until PICC line
can be placed. (UW Health Very low quality evidence, weak/conditional
recommendation)
ii. Start D10 TrophAmine® with heparin 0.5 units/mL at 80ml/kg/d (subtract out UAC
fluids).

4. Placement of the UAC and UVC should be confirmed by x-ray, however a UVC may also be
confirmed using an ultrasound or echocardiogram, as x-ray can be challenging to interpret
due to malposition of the liver.18

Pharmacotherapy
1. Sedation is required for all intubated infants with CDH18 (minimally responsive to noxious
stimuli, i.e., State Behavioral Scale (SBS) score -2 or Neonatal Pain Agitation and Sedation
Scale (N-PASS) score of -2 to -5). (UW Health Very low quality evidence, strong
recommendation) Morphine 0.1 mg/kg IV should be administered as soon as possible after
birth followed by a continuous infusion at 0.01 mg/kg/hr with 0.05-0.1 mg/kg bolus doses
every two hours as needed.

2. Paralysis should be avoided, unless patient requires maximal respiratory support with
persistent hypoxemia.18 (UW Health Low quality evidence, weak/conditional recommendation)
Rocuronium 0.45-0.6 mg/kg every 30 minutes as needed may be considered for initial
stabilization and transport.

3. Inhaled nitric oxide (iNO) may be needed for patients with severe pulmonary hypertension.
A trial of iNO should be made in patients with an oxygenation index (OI) > 25 and should be
started at 20 ppm. Physician should document time and reason for initiating iNO as well as
response. If there is not improvement within 30-60 minutes of starting iNO the patient should
be weaned off.

4. Consider normal saline (10 mL/kg aliquots) for low blood pressure, poor perfusion, or
metabolic acidosis.17 Dopamine (suggested starting dose 5mcg/kg/min) may be considered
for persistently low blood pressure and poor perfusion.17 (UW Health Very low quality evidence,
weak/conditional recommendation)

5. Consider antibiotics for routine indications (prolonged rupture of membranes or
chorioamnionitis) or in infants who are severely symptomatic (low blood pressure, persistent
metabolic acidosis, maximal respiratory support). (UW Health Very low quality evidence,
weak/conditional recommendation) Due to the concern for ototoxicity with gentamicin, ampicillin
and cefotaxime are recommended.

Imaging
1. Obtain babygram after admission to confirm position of ETT, vascular access, and gastric
suction catheter. (UW Health Very low quality evidence, strong recommendation)

2. An echocardiogram should be considered if saturations remain low (< 80% despite 100%
FiO2) or for low blood pressure and poor perfusion, but is not required after line placement
and stabilization. (UW Health Very low quality evidence, weak/conditional recommendation)
a. Limited ECHO for PPHN to limit stimulation if extensive cardiac work-up was done
prenatally.

3. Head ultrasound should be considered if ECMO is likely but is not required in all patients.
(UW Health Very low quality evidence, weak/conditional recommendation)
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a. Discuss timing of echocardiogram and head ultrasound with PICU attending
(especially if need for ECMO is likely soon after transfer to PICU).

Transport to AFCH
1. Transfer to AFCH should occur ideally between 6-8 hours, but no later than 12 hours after
delivery. (UW Health Very low quality evidence, weak/conditional recommendation)

PREOPERATIVE MANAGEMENT
General Philosophy and Goals of Therapy3,16,17
1. Maintain adequate systemic/cerebral oxygen delivery.
2. Avoid lung injury and promote lung growth.
3. Optimize right heart function.
4. Lower pulmonary vascular resistance (PVR) if necessary. Patients who demonstrate
reactivity and have good right heart function may not need additional intervention.
5. Minimize stimulation and agitation to reduce risk for pulmonary hypertensive crises.
6. ECMO should only be considered if there is evidence of a reversible process that is
impairing lung or cardiovascular function. Avoid extracorporeal membrane oxygenation
(ECMO) when possible.
Nursing Care and Room Setup
1. When possible, patients should be admitted to a large room, with the ventilator positioned
on the patient’s left side. It is recommended to use ECMO positioning (i.e., head of the
patient at the foot of the bed). (UW Health Very low quality evidence, strong recommendation)

2. Minimal stimulation should be maintained. (UW Health Very low quality evidence, strong
recommendation)
a. In order to minimize light stimulation keep the room dimly lit, protect the patient’s
eyes from light during assessments or when direct light is used, and make efforts to
avoid prolonged direct light exposure. Use of a phototherapy mask may be
considered if indirect light must be on for prolonged periods of time. If used, the
mask should be removed once direct light is turned off. Stimulation from noise can
be reduced by placing the patient in a low traffic area, holding conversations away
from the patient bedside, and speaking in low, soft tones.
b. It is important to coordinate hands on assessments and cluster care to minimize
disruptions to the infant. Trainee exams (other than fellow) should only be performed
when accompanied by a bedside RN or attending exam. Minimal repositions to
relieve pressure points should be performed, while holding and bathing is not
allowed until the patient is stable post-operatively. Bedding changes should also be
delayed until the patient is stable.

3. Routine suctioning should be avoided unless clinically indicated (e.g., desaturation,
increased agitation, hypercapnia). (UW Health Low quality evidence, strong recommendation) To
minimize physiologic decompensation and risk of infection, suction the endotracheal tube
using a closed suction kit.20 For additional recommendations refer to the UW Health
Prevention of Ventilator Associated Pneumonia – Neonatal/Pediatric – Inpatient Guideline.
If unable to resolve clinical decompensation following closed suctioning, consider open
suctioning via bag and suction catheter and avoid routine installation of normal saline.
Routine open suctioning may be considered for patients with thick secretions that have
required frequent open suctioning to resolve respiratory decompensation. (UW Health Very
low quality evidence, weak/conditional recommendation)
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11
4. Complete physiologic assessments should be performed every 4-6 hours, with focused
assessments as needed, maintaining vigilant nursing care. (UW Health Very low quality
evidence, weak/conditional recommendation) Cardiorespiratory monitoring, pre- and post-ductal
oxygen saturation monitoring, and renal and cerebral NIRS monitoring should occur.17 (UW
Health Very low quality evidence, strong recommendation) aEEG/EEG may be considered for
patients on continuous muscle relaxants. (UW Health Low quality evidence, weak/conditional
recommendation) All vital signs (and other continuously monitored parameters on the
cardiorespiratory monitor or other devices) should be documented hourly.
a. Routine pupil checks should be avoided and completed only on admission or with
acute changes. (UW Health Very low quality evidence, strong recommendation)
b. Weight should be measured on admission and then should not be routinely
performed, but can be obtained when volume status is uncertain. (UW Health Very low
quality evidence, strong recommendation) Birth weight is used for the first 14 days for
dosing.
c. Temperature measurements should NOT be obtained rectally, but instead it is
recommended to perform axillary measurements with full assessments. (UW Health
Very low quality evidence, strong recommendation) Continuous skin temperature
monitoring in servo control on radiant warmer should also be maintained. Patients
should maintain normothermic temperature (36.5-37.5°C) measured axillary. (UW
Health Very low quality evidence, strong recommendation)
d. Continuous arterial blood pressure monitoring is preferred. (UW Health Very low quality
evidence, strong recommendation) Avoid cuff blood pressures, unless arterial line is not
present. It is important to check non-invasive blood pressure measurements once
per shift to assess accuracy of invasive pressures.
e. Deviations of > 20% from baseline renal or cerebral NIRS should be placed in the
context of other measures.
i. Renal NIRS should be within 10-15 of lower extremity sat. If greater
difference, investigate impaired cardiac output.
ii. Cerebral NIRS should be within 20-25 of systemic saturation. Note: Will vary
directly with PaCO2.

Lines/Drains/Tubes (UW Health Very low quality evidence, strong recommendation)
1. Orotracheal intubation should be maintained and confirmed by daily x-ray.17

2. Replogle needs to be functioning and in good position continuously. A daily x-ray should be
used to confirm position and adequate gastric decompression.18
a. Use 10 French catheter in patients > 36 weeks gestational age
b. No manipulation of nasogastric/orogastric (NG/OG) tubes unless necessary
c. Flush/aspirate NG/OG every 4-6 hours with 3-4 mL sterile water
d. Low-intermittent suction
3. Umbilical artery catheter (UAC) and umbilical venous catheter (UVC) should be well-
positioned.18 The UAC and UVC should be removed by day of life (DOL) #7-10.

5. Consider peripherally inserted central catheter (PICC) or femoral central venous line (CVL) if
additional access is needed, or if higher concentration fluids/high dose medications are
required. If a low lying UVC was previously placed, it should be replaced by a PICC line
within 24-48 hours. Patients scheduled for an open repair should have a PICC line (double
lumen preferred) placed prior to surgery, as the UAC and UVC lines are often removed to
prep and access the abdomen.
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12
Ventilator Management
1. The recommended general philosophy or approach to ventilator management focuses on
minimizing lung injury related to high pressure and oxygen toxicity, while tolerating mild
hypoxemia and moderate hypoventilation during the pre-operative period.2,3 Careful
monitoring is recommended to detect and respond to early evidence of worsening
respiratory status. The primary goal is to avoid the need for dramatic increases or decreases
in support. Current evidence does not support one ventilator mode over another, however
due to overall clinical experience, conventional ventilation is recommended as the initial
mode of ventilation.1,4,17 (UW Health High quality evidence, weak/conditional recommendation)

2. There is NO GOAL TIDAL VOLUME for patients with CDH; therefore, pressure control
modes are generally used. Recruitment is generally not needed. Therefore, lower PEEP
(i.e., 3 or 4 cm H2O) may be used to augment tidal volume while not increasing peak
inspiratory pressures.

Initial Ventilator Settings1,5,18
1. The general approach includes using a higher respiratory rate to manage ventilation and
minimize ventilator-induced injury. A lower tidal volume (2-5 mL/kg) should be well tolerated
in CDH patients.

2. The initial SIMV settings initiated after delivery should be continued17 (UW Health Low quality
evidence, weak/conditional recommendation):
a. PIP 15 – 25 cm H2O
b. PEEP 3 – 5 cm H2O
c. RR 40 – 60 breaths/minute
d. iT 0.3 – 0.5
e. FiO2 – titrate to keep pre-ductal saturation > 95%

Monitoring and Target Ranges5,18
1. Ventilation: Permissive hypercarbia should be tolerated as long as the patient is
comfortable.17 The pH/PCO2 target range is pH ≥ 7.25 and PCO2 45-65 mmHg.17 Higher
PCO2 levels should be tolerated during the initial transition period and intermittently during
the pre-operative period. (UW Health Low quality evidence, weak/conditional recommendation) It
is important to correlate the transcutaneous or end-tidal CO2 monitor with initial blood gas
measurements and use to follow trend and minimize need for frequent blood draws.
a. If CO2 is elevated and patient is not critically desaturated (SpO2 < 75-80%), do not
change CO2 rapidly. (UW Health Very low quality evidence, strong recommendation) Rapid
PCO2 changes may result in acute changes in cerebral blood flow, particularly with
disrupted cerebral autoregulation present in a sick newborn.

2. Oxygenation: If perfusion and hemoglobin are adequate, patients will tolerate lower
saturations during initial transition and pre-operative period.
a. Pre-ductal saturations should be maintained between 90 and 95%. (UW Health Low
quality evidence, strong recommendation) Lower saturations (> 80-85%) may be
tolerated when balancing O2 toxicity vs. benefits of higher FiO2, particularly if patient
is in acute transitional period (several hours after birth) or if unable to wean FiO2 by
48-72 hours of age. (UW Health Low quality evidence, weak/conditional recommendation)
Wean FiO2 slowly for pre-ductal saturation > 95% (by no more than 2-5% every 30-
60 minutes) to minimize reactive pulmonary hypertension. (UW Health Low quality
evidence, strong recommendation)
b. Post-ductal saturations are indicative of right-to-left shunt and not a measure of
cerebral or myocardial oxygen delivery. Post-ductal saturations as low as 70 may be
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13
acceptable (and even lower if urine output remains reasonable and lactate low). (UW
Health Low quality evidence, weak/conditional recommendation) Post-ductal monitoring is
primarily helpful to understand and monitor lability of PVR rather than an indicator of
O2 content.
c. PaO2 measurements should not be the sole indicator for increasing support. (UW
Health Low quality evidence, strong recommendation) The trend in saturation and
evidence of end organ function are more important indicators of overall adequacy of
oxygenation. Right radial arterial monitoring may be useful if there is a large right-to-
left ductal shunt (to help avoid cerebral and retinal hyperoxygenation) but is not
necessary for acute monitoring.
d. Cerebral and renal NIRS, lactate, urine output, and venous saturations can all be
helpful additional indicators of systemic oxygen delivery and can be considered. (UW
Health Moderate quality evidence, weak/conditional recommendation)
e. In general the inability to achieve oxygen saturations greater than 85% for at least 1
hour at some point using this low pressure, lung protective approach and/or pre-
operative pneumothorax suggests the possibility of inadequate lung parenchyma for
survival.

Indications for Increasing Support and Approach
1. Prior to ventilator changes, evaluate adequacy of sedation, position of patient, position of
ETT, need for suctioning, and adequacy of bowel decompression. (UW Health Very low quality
evidence, strong recommendation) Most CDH patients have increased work of breathing at
baseline with tachypnea and mild retractions being common. Prior to increasing respiratory
support it is critical to note changes in respiratory pattern from baseline (UW Health Low
quality evidence, strong recommendation). The following are reasons to consider ventilator
changes:
a. Paradoxical chest wall movement
b. Significant increase in retractions
c. Preductal SaO2 < 80%
d. pCO2 > 65 mmHg (pH < 7.25)
e. Sustained preductal to postductal SaO2 difference >10% indicate PHTN and should
prompt investigation (see pulmonary hypertension section).

2. SIMV-PC changes should be made for hypoxemia or hypercarbia. (UW Health Low quality
evidence, weak/conditional recommendation)
i. Focus on changes that minimize exposure to high pressure (i.e., preference
for high rate over high pressure).
ii. IMV rate as high as 60 for respiratory distress
1. Decrease PEEP to avoid air trapping
2. Decrease iT 0.3 – 0.4 while monitoring I:E ratio
iii. If tidal volume is low (< 2 – 5 mL/kg) evaluate compliance and pressure-
volume loops before increasing PIP. Consider decreasing PEEP to increase
∋P while minimizing exposure to high pressure.
iv. If PIP higher than 25 cm H2O or rate higher than 60, can consider “high-
frequency positive pressure ventilation”. If used, the following settings are
recommended (UW Health Low quality evidence, weak/conditional
recommendation):
3. PIP 20 cm H2O
4. PEEP 0 cm H2O
5. Rate 100
6. iT 0.3
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7. Flow 10-12 L

3. Use of high frequency oscillatory ventilation (HFOV) may be considered as an alternative
mode of ventilation if there is: 1) failure to improve over 30 minutes on conventional
ventilation (PIP > 25 cm H2O and Rate > 60) while maximizing sedation, positioning, and
pulmonary toilette; 2) failure to oxygenate despite use of conventional ventilation in
conjunction with iNO; or 3) for pneumothorax.1,5,17 (UW Health Very low quality evidence,
weak/conditional recommendation)
a. The following initial settings are recommended:
i. Mean airway pressures (MAP) 14 – 16 cm H2O
ii. Hz 10 – 12. This range is higher than normal for term infant but CDH patients
have pulmonary hypoplasia and therefore higher Hz is preferred for lung
protection and is generally adequate.
iii. Amplitude 30 – 40 cm H2O
iv. Goal rib expansion: 8 – 10 ribs on contralateral side. To minimize lung injury,
avoid flattening of the diaphragm. It is recommended to closely monitor for
signs of hypotension due to decreased venous return.

Indications for Weaning and Approach
1. Weaning in the pre-operative period should be very gradual to avoid the need to
dramatically increase support during periods of decompensation.

2. Prioritize weaning pressure over FiO2 over rate. (UW Health Very low quality evidence, strong
recommendation)
a. FiO2 should be weaned to maintain preductal SaO2 90-95%.
b. Wean FiO2 no faster than 2-5% every 30-60 min.
c. Goal FiO2 0.6 by 12 hours; FiO2 0.4 by 48 hours.
Acute Pulmonary Hypertension (PHTN) and Cardiac Management
Initiation of Treatment
1. Some PHTN is expected, right (systolic) and left (diastolic) heart function are the primary
concerns. In the setting of PHTN with good right ventricular (RV) function and pre-ductal
saturation, there is likely no need to treat. Furthermore, the systemic circulation may be
dependent on ductal dependent perfusion from the RV given in utero underdevelopment and
diastolic dysfunction of the LV. Treatment for acute PHTN is recommended in patients with
one or more of the following indications5,6,11 (UW Health High quality evidence, strong
recommendation):
a. Pre-ductal SpO2 < 90% despite optimizing other ventilatory maneuvers
b. Oxygenation index (OI) > 25
c. Post-ductal saturations < 70% and/or evidence of end organ dysfunction (e.g., rising
lactate, acidosis or oliguria)
d. Echocardiogram demonstrates systemic or near-systemic right-sided pressures and
depressed RV function or if evidence of supra-systemic to near-systemic right-sided
pressures with borderline oxygenation.

2. DO NOT HYPERVENTILATE. Lung protection is the primary goal.

Treatment Options
1. Inhaled nitric oxide (iNO) should be used as first line treatment with a suggested starting
dose of 20 ppm.6-11,17 (UW Health High quality evidence, weak/conditional recommendation)
Reassessment should occur within 30-60 of iNO initiation. If no improvement, patient
should be weaned off iNO quickly (i.e. over 60 minutes). In patients receiving iNO more
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15
than 4 hours, weaning needs to occur more gradually – see iNO algorithm attached to Policy
3.53. It is recommended that iNO be weaned once pulmonary hypertension and RV function
have improved, or after FiO2 has been weaned to 0.4-0.55. (UW Health Very low quality
evidence, strong recommendation)

2. It is recommended to optimize hemodynamic parameters, including pH, systemic vascular
resistance (SVR), myocardial function and oxygen carrying capacity. (UW Health Very low
quality evidence, strong recommendation) Adjunctive therapy may be helpful.
a. Consider metabolic means of improving pH (sodium bicarbonate, acetate, or THAM)
(UW Health Very low quality evidence, weak/conditional recommendation)
b. It is recommended to correct ionized hypocalcemia. (UW Health Very low quality
evidence, strong recommendation)
c. Consider red blood transfusion if Hct < 35%. (UW Health Very low quality evidence,
weak/conditional recommendation) Hemoglobin labs should be drawn every 12 hours to
assess oxygen carrying capacity.
d. For RV dysfunction, milrinone (suggested starting dose 0.5 mcg/kg/min; 0.25-0.75
mcg/kg/min) is the preferred first line agent to improve RV function in the setting of
adequate systemic blood pressure.21,22 (UW Health Very low quality evidence,
weak/conditional recommendation) A combination of dopamine and milrinone is
recommended in patients with mild hypotension and RV dysfunction. (UW Health Very
low quality evidence, weak/conditional recommendation) Dobutamine (suggested starting
dose 3 mcg/kg/min; 3-25 mcg/kg/min) may be used as a second line agent or used in
combination with milrinone. (UW Health Very low quality evidence, weak/conditional
recommendation)
e. For hypotension, use of the following medications is encouraged to increase SVR
and raise systemic blood pressure in the setting of high PVR and good RV function
in an effort to decrease right-to-left ductal shunting.17,18 (UW Health Very low quality
evidence, weak/conditional recommendation)
i. Dopamine 3-25 mcg/kg/min (suggested starting dose 5 mcg/kg/min)
ii. Epinephrine if dopamine > 20 mcg/kg/min (suggested starting dose 0.03
mcg/kg/min; maximum dose 2.6 mcg/kg/min).23-25
iii. Vasopressin should be used only in refractory cases (suggested starting
dose 0.5 milliunits/kg/min; maximum dose 2 milliunits/kg/min).
f. Prostaglandins may be useful in the treatment of severe PHTN.8,9,17,26 (UW Health
Very low quality evidence, weak/conditional recommendation)
i. PGE1 IV has the advantage of opening the duct in the setting of severe
PHTN and RV failure allowing a “pop-off” for the RV.
ii. Inhaled prostacyclin has the advantage of possibly having improved
ventilation-perfusion matching. For dosing recommendations related to
inhaled prostacyclin (epoprostenol), see UW Health Epoprostenol Inhaled –
Neonatal/Pediatric/Adult – Inpatient Guideline.

Monitoring Response to PHTN Treatment
1. An initial echocardiogram should be obtained within 0-12 hours of age to ensure no
structural anomalies, document whether PH is present, and evaluate right and left heart
function.17 (UW Health Low quality evidence, strong recommendation)

2. Additional echocardiograms are indicated if considering starting or stopping iNO, if
additional information regarding cardiac function is desired, or if there is concern that
systemic oxygen delivery is compromised because of a restrictive PDA with PH.9 (UW Health
Low quality evidence, weak/conditional recommendation)
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16
Pharmacotherapy
1. Use birth weight as dosing weight for first 2 weeks of life.

2. All babies should continue to be well sedated through the perioperative period (minimally
responsive to noxious stimuli, i.e., State Behavioral Scale (SBS) score -2 or Neonatal Pain
Agitation and Sedation Scale (N-PASS) score of -2 to -5).17 (UW Health Low quality evidence,
strong recommendation)
a. Morphine (continuous infusion start at 10 mcg/kg/hr (0.01 mg/kg/hr), bolus 0.05-0.1
mg/kg every 2 hours as needed) or fentanyl (continuous infusion start at 1-2
mcg/kg/hr, bolus 1 mcg/kg every 2 hours as needed) may be used to achieve
adequate sedation. It is recommended to titrate dose to effect.
i. Due to greater development of tolerance, if patient is receiving fentanyl for
more than 5 days, or rapidly escalating dose, consider transition to morphine.
b. Additional sedation with midazolam (bolus starting dose 0.1 mg/kg every 2 hours as
needed), lorazepam (starting dose 0.1 mg/kg every 6 hours as needed), or
dexmedetomidine (starting dose 0.5 mcg/kg/hr infusion) is frequently necessary if
opioids do not provide adequate sedation. (UW Health Very low quality evidence,
weak/conditional recommendation)

3. Paralysis should be avoided unless significant respiratory asynchrony or for procedures.18
(UW Health Very low quality evidence, strong recommendation)
a. Patients on ECMO do not necessarily require continuous paralysis after placement of
the cannula.
b. Concurrent sedation is mandatory whenever a paralytic is administered. (UW Health
Moderate quality evidence, strong recommendation)

4. Due to risk of hearing impairment in patients with CDH, attempt to minimize exposure to
ototoxic medications (e.g., loop diuretics, aminoglycosides) when possible. (UW Health Very
low quality evidence, strong recommendation)

5. Routine antibiotic treatment and presurgical prophylaxis are not required, unless clinically
indicated for other reasons in the newborn period. (UW Health Very low quality evidence, strong
recommendation)

Nutrition
1. Establish total fluid goal (80 mL/kg/day within the first day of life) and re-visit daily based on
patient’s clinical status. (UW Health Very low quality evidence, weak/conditional recommendation)

2. Initiate “starter” parenteral nutrition (PN) upon admission and transition to a custom PN
when electrolytes are stable per recommendations within the UW Health Parenteral Nutrition
– Pediatric – Inpatient/Ambulatory Clinical Practice Guideline.17

3. Use human milk for oral cares, even for those patients that are NPO, unless otherwise
specified or patient does not tolerate. (UW Health Very low quality evidence, strong
recommendation)

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17
Extracorporeal membrane oxygenation (ECMO)
1. There are no specific criteria for ECMO in CDH patients; however the following indications
and contraindications may be used to determine eligibility. Patients who are unable to
maintain adequate oxygen delivery with reasonable ventilator settings should be considered
for ECMO.1,12 (UW Health Low quality evidence, weak/conditional recommendation)

Table 1. Indications and Contraindications for ECMO
ECMO Indications5,16,27,28 Absolute ECMO Contraindications
Relative ECMO
Contraindications
ξ Preductal SaO2 < 85%
despite iNO and inotropic
support
ξ Severe lability
ξ OI > 20
ξ PIP/AMP > 30
ξ Sustained lactate > 4 mmol/L
ξ Consider early ECMO for
LHR < 1.4, liver in chest
ξ Gestational age < 34 weeks
ξ Birth weight < 1.6 kg
ξ Lethal comorbidity
(Pentalogy of Cantrell,
Frynn's syndrome, Bilateral
defects)
ξ IVH > grade 3
ξ Gestational age 34-36 weeks
ξ Birth weight < 2 kg
ξ IVH > grade 2
ξ Cardiac lesion (coarctation)*
ξ Failure to achieve SaO2 > 85%
(and sustain for 1 hour) within
the first 2-4 hours of life despite
maximal ventilatory maneuvers
* ECMO for patients with CDH and congenital heart disease will be considered on a case-by-case basis depending
on the type of cardiac lesion and severity of the CDH.

2. Veno-venous (VV) ECMO should be considered if cardiac function is adequate, even in the
setting of pulmonary hypertension, as the oxygenation of venous blood can mitigate the
effects of right-to-left shunt.1 (UW Health Low quality evidence, weak/conditional recommendation)

3. As with all patients on ECMO, strict attention must be paid to fluid administration and fluid
balance, as a delayed diuresis will prolong the patient’s ECMO course.

4. Anticoagulation recommendations are outlined within the UW Health Unfractionated Heparin
in ECMO – Neonatal/Pediatric – Inpatient Guideline.

5. Lung inflation should be maintained with “sigh” breaths on the ventilator and the team must
pay strict attention to pulmonary toilet and patency of the ETT. The RV is under chronic
strain in severe CDH and atelectatic lungs will increase PVR and afterload significantly.
a. Rest settings (conventional ventilator): PEEP 10, PC 10, R10, i-time 1s, <50%
ξ No prolonged clamping of ETT without ventilation
ξ All patients bagged during transport to avoid dense atelectasis
ξ Recruitment maneuvers are not generally indicated on ECMO
b. Wean pressures if evidence of overdistension on chest x-ray (9-10 rib expansion on
unaffected lung)

SURGERY
General Philosophy and Timing
1. The timing of surgery should be based upon each individual clinical scenario, risks and
benefits of the procedure, and professional clinical judgement of the surgical staff.17 (UW
Health Low quality evidence, weak/conditional recommendation) Current evidence suggests that
timing of CDH repair does not affect survival.1,28

2. However, with current gentle ventilation strategy, it is recommended that surgical repair
should NOT be performed while there is uncontrolled pulmonary hypertension. (UW Health
Very low quality evidence, strong recommendation)

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18
Surgery on ECMO
Although the level of evidence is low, repair of CDH within 72 hours on ECMO should be
considered, as it may improve outcome and decrease time on ECMO.1,13,17 (UW Health Low
quality evidence, weak/conditional recommendation)

Type of Surgical Repair
1. Thoracoscopic repair may be done for primary repair.29 (UW Health Low quality evidence,
weak/conditional recommendation) For thoracoscopic repair, intraoperative PaCO2 and pH
should be checked frequently. If the case is going to be long and there is concern about
blood gas, it should be converted to open. If patch repair is required, it is recommended that
the surgery be converted to an open approach due to increased risk of recurrence in
patients who underwent laparoscopic patch closure of the diaphragm.

2. Use of oversized/domed patches might reduce recurrence rates associated with patch
repair, however available evidence showed no clear difference in recurrence rate.1,30 For
patch repair, the type of patch should be based on the surgeon’s preference and
consideration for the individual clinical scenario. (UW Health Very low quality evidence,
weak/conditional recommendation)

Ventilation During Surgery
The ICU attending, anesthesia attending, and respiratory therapist should discuss ventilation
during surgery and should specifically discuss using the ICU ventilator during the case.
Humidification during surgery is suggested to reduce postoperative mucous plugging. (UW
Health Very low quality evidence, strong recommendation)

POSTOPERATIVE CARE
Transition from Operating Room (Handoff)
1. Staff should provide clear communication and follow the recommendations below to
effectively transition care from the operating room to the intensive care unit. (UW Health Very
low quality evidence, strong recommendation)
a. OR staff should contact the ICU with an update, brief report, and estimated time of
arrival. A second call should occur before the patient leaves the OR to assure that
ICU staff is available for admission.
b. An interdisciplinary handoff should occur at the bedside in the ICU with no more than
five staff (e.g., two ICU nurses, one anesthesia representative until the handoff is
complete, one respiratory therapist to help anesthesia transition to the ICU ventilator,
and ICU representative who are helping with the transition), unless there is a
significant event.
i. The handoff should not begin until the Primary Nurse is ready.
ii. All staff talking should be away from the bed, with the total number of staff
minimized to reduce disruption to the patient.
iii. The patient’s PDM should be connected to the bedside monitor. The Primary
Nurse should complete a quick ABC assessment.
iv. The Surgeon and Anesthesiologist will give orders until the handoff is
complete. After the handoff orders will be given by the ICU Intensivist or
Surgeon.

2. No routine postoperative labs are recommended unless the patient is on ECMO. (UW Health
Very low quality evidence, weak/conditional recommendation)

3. Indications for ECMO do not change in the postoperative period (see ECMO section).

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19
4. Lines/tubes/drains should be positioned and their placement confirmed as they were
preoperatively. (UW Health Very low quality evidence, strong recommendation)
a. If a chest tube is placed intra-operatively or post-operatively for fluid removal it
should be placed on water seal and not to suction. (UW Health Low quality
evidence, strong recommendation)

5. Heated/humidified ventilation should be maintained. (UW Health Very low quality evidence,
strong recommendation)

Special Considerations Specific to the Post-operative Period

Ventilator Management and Respiratory Considerations
1. Pulmonary toilette should begin upon readmission with suctioning. Open bag-suctioning may
be required, particularly in the immediate post-operative period. (UW Health Very low quality
evidence, weak/conditional recommendation)
a. If open bag suctioning, consider an alternative technique to improve mobilization of
secretions (e.g., one trial of nebulized saline). (UW Health Low quality evidence,
weak/conditional recommendation) Patient decompensation is expected and has been
demonstrated in published literature.

2. Ventilator support may need to be increased in the immediate post-op period in order to
regain stability, however, the objective remains gentle ventilation. The following strategies
are recommended to regain stability (UW Health Very low quality evidence, weak/conditional
recommendation):
a. Higher FiO2 (0.5-0.55) may be needed to permit lower pressures while keeping SaO2
over 92-93%.
b. Higher rates are preferred to higher peak pressures to maintain pH > 7.3. If peak
pressures > 25 to generate goal tidal volumes (from pre-op), consider trial of
decreased PEEP (as low as 3 cm H2O) or transition to HFOV.

3. Pleural effusion on the effective side is an expected consequence of repair of CDH.
However, if there is evidence of mass effect (i.e., shift of the mediastinum to contralateral
side, or decreased respiratory compliance) consider drainage and investigate etiology (e.g.,
chylothorax).

Pharmacotherapy
Medications for pain and sedation may need to be increased 10-20% during the immediate
post-op period (up to 48 hours post-CDH repair) to account for postoperative pain. (UW Health
Low quality evidence, weak/conditional recommendation) Refer to the Pain and Agitation Continuous
Infusion Titration – Pediatric – Inpatient Practice Protocol.
Weaning Phase
Beginning 1-3 days following surgery and depending on the condition of the individual patient
attempts to wean the ventilator, wean the sedation, and establish enteral nutrition should begin
and be advanced on a regular basis. (UW Health Very low quality evidence, weak/conditional
recommendation)

Sedation Weaning
1. 24 – 48 hours following surgery, regularly scheduled opioids or continuous infusions should
begin to be weaned by 10-20% of the maximal dose. (UW Health Low quality evidence,
weak/conditional recommendation) Weans should be considered every 12 hours as tolerated.
Begin withdrawal scoring at initiation of weaning and avoid weaning by more than 20% of
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20
maximal dose every 24hrs. Bolus doses should be given as needed and should be used as
an indicator if the patient is tolerating the wean.

2. Long-term sedation may be required for patients that do not wean easily from respiratory
support. (UW Health Low quality evidence, weak/conditional recommendation) If long-term
sedation is required beyond 7 days, and the patient has not tolerated initial attempts to wean
as above, an extended weaning regimen should be employed to address and minimize
withdrawal symptoms.

Ventilator Weaning
1. Patients with CDH will often have lower tidal volumes and higher respiratory rates. The goal
is to achieve near-normal ventilation and oxygenation while minimizing ventilator-induced
lung injury and not producing excessive respiratory work.
a. Increased pressure support may be useful to allow weaning of rate and enhanced
patient synchrony. (UW Health Very low quality evidence, weak/conditional
recommendation) May need to increase pressure support level to achieve
“reasonable” tidal volumes (i.e., 3-5 cc/kg).
b. Inspiratory pressures should be weaned (if possible) if chest excursion good and
reasonable tidal volumes. (UW Health Very low quality evidence, weak/conditional
recommendation)
c. Once peak pressures in low 20s weaning rate is the next priority. Weaning by 5-10%
of current rate once or twice a day is reasonable in patients who do not have
deterioration in gas exchange or notable retractions or asynchrony. (UW Health Very
low quality evidence, weak/conditional recommendation)

2. Because of the increased need for sedation and risk of PHTN, patients generally are not
weaned for 48 – 72 hours post-operatively unless over-ventilated (CO2 < 45), over
oxygenated (saturation > 98%), or receiving large tidal volumes (more than 6 mL/kg or more
than pre-operatively). Indications to start weaning ventilatory support include: no increased
support in the prior 12 hours, stable or weaning FiO2 requirement, and stable or falling
PaCO2. (UW Health Very low quality evidence, weak/conditional recommendation)

3. When possible, follow the main goal for weaning and wean pressure over FiO2 over rate.
Once peak pressures < 22 and FiO2 < 0.45, begin weaning rate. (UW Health Very low quality
evidence, weak/conditional recommendation)

4. To assist with weaning it is recommended to use respiratory modes that improve patient
synchrony on the ventilator. (UW Health Very low quality evidence, strong recommendation)
a. Pressure Control: Patients can be weaned using the same mode of ventilation they
have been on since the OR. (UW Health Very low quality evidence, weak/conditional
recommendation)
b. Neurally adjusted ventilator assist (NAVA) is a mode of ventilation in which the
electrical activity of the diaphragm (Edi) is used to synchronize mechanical breaths
with the patient’s respiratory drive while providing both synchronous and
proportionate breaths. NAVA also allows extubation to NAVA-driven non-invasive
ventilation.
i. Establishing Settings:
1. Understanding of peak pressures and tidal volumes should be known
prior to initiation of NAVA as this will help guide initial NAVA level.
2. NAVA catheter should be placed per vendor recommendations.
Appropriate depth is verified when both middle positioning EKG lines
are BLUE on NAVA screen as well as by x-ray confirmation.
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21
ii. Initial Settings (UW Health Very low quality evidence, weak/conditional
recommendation):
1. NAVA level of 1 (adjust by increments of 0.1-0.2 to obtain appropriate
peak pressures and tidal volumes)
2. PEEP of 5 cm H2O (range 3-7)
3. Back up setting should be set (i.e., PEEP 5 cm H2O, PIP 20 cm H2O,
Rate 40)
iii. Indications for weaning (UW Health Very low quality evidence, weak/conditional
recommendation):
1. NAVA level is maintained and tidal volumes improve with
decreasing/stable Edi values and peak pressures
2. NAVA level is weaned by 0.1-0.2 and patient is able to maintain
appropriate tidal volumes without significant increase in work of
breathing
iv. Indications for NOT weaning vs. changing to conventional ventilator (UW
Health Very low quality evidence, weak/conditional recommendation):
1. Minimal to no Edi signal
2. Wide fluctuations in Edi
3. Tidal volumes show significant decrease
4. Increase in FiO2 requirements (i.e. >10-15% above baseline)
5. Increase in CO2 values (i.e. >10 above baseline)
6. Increase work of breathing
d. Extubation criteria will vary for each patient
i. Maximum settings to consider extubation: PIP 20 cm H2O, PEEP 5 – 6, Rate 20
– 30,Pressure support 14
ii. Post-extubation respiratory support with NIPPV via Ram cannula can be
considered: PIP 20 – 24 cm H2O, PEEP 5 – 6 cm H2O, Rate 40, I-time 0.5
1. NAVA NIPPV can also be used
Establish Nutrition
1. Infants with severe CDH frequently have higher caloric requirements for growth.31 Ultimately,
this usually requires fortification of human milk or infant formula. It is recommended to start
enteral nutrition (EN) as soon as GI function returns post-operatively. (UW Health Low quality
evidence, weak/conditional recommendation) When clinically able to initiate EN, the patient
should be on trophic feeds (20 mL/kg/day) for 24 to 72 hours, and then advance EN
volumes as appropriate for gestational age per recommendations within the UW Health
Enteral Nutrition – Neonatal – Inpatient Clinical Practice Guideline.

2. Consider NAVA catheter placement when initiating feeds. (UW Health Very low quality
evidence, weak/conditional recommendation)

3. Use human milk for oral cares, even for those patients that are NPO, unless otherwise
specified. (UW Health Low quality evidence, weak/conditional recommendation)

4. If reflux symptoms are present, it is recommended to initiate treatment in order to preserve
lung function.17 (UW Health Very low quality evidence, weak/conditional recommendation)
a. First line therapy for reflux symptoms should be a proton-pump inhibitor, followed by
a prokinetic agent if symptoms persist. (UW Health Low quality evidence,
weak/conditional recommendation)
Pulmonary Hypertension
1. Patients who do not have significant pulmonary hypertension prior to surgical repair should
be monitored for pulmonary hypertension during the post-operative period with weekly
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22
echocardiograms (for the first 2-3 weeks) while weaning from respiratory support. (UW Health
Very low quality evidence, strong recommendation)
a. Recurrent desaturation episode during ventilator weaning should prompt evaluation
of pulmonary hypertension. (UW Health Very low quality evidence, weak/conditional
recommendation)
b. An echocardiogram should be repeated prior to discharge and follow-up with
cardiology should be scheduled for all patients. (UW Health Very low quality evidence,
weak/conditional recommendation)

2. Patients who do have significant pulmonary hypertension prior to surgery who require
treatment with iNO should be monitored by echocardiogram to document changes in
pulmonary vascular resistance and right heart function while weaning from ventilator
support. (UW Health Very low quality evidence, weak/conditional recommendation)
a. Weaning of inhaled nitric oxide should occur coincidently with ventilator weaning. It is
recommended to begin weaning iNO once FiO2 is < 0.4. (UW Health Very low quality
evidence, strong recommendation)
b. A repeat echocardiogram should be obtained once the patient is off iNO and then
followed weekly while weaning respiratory support. (UW Health Very low quality
evidence, weak/conditional recommendation)
c. An echocardiogram should be repeated prior to discharge and follow-up with
cardiology should be scheduled for all patients. (UW Health Very low quality evidence,
weak/conditional recommendation)

3. Patients who cannot be effectively weaned from iNO should be evaluated by interventional
cardiology to consider direct measurement of the pulmonary vascular pressures and
responsiveness to pulmonary vasodilating medications. Long-term pulmonary vasodilator
treatment might include sildenafil, prostacyclin (IV or inhaled) or endothelin receptor
antagonists.17 Caution must be used as all these (except inhaled prostacyclin) can worsen
V/Q mismatch in CDH patients who are hypoxic. (UW Health Low quality evidence,
weak/conditional recommendation)

DISCHARGE PLANNING
Care management by a multidisciplinary team for follow-up after surgery is recommended.14
(UW Health Low quality evidence, strong recommendation) If not performed in the last two weeks, the
following imaging studies are recommended prior to discharge:14
ξ Echocardiogram
ξ Chest x-ray
ξ A brain MRI should be obtained prior to discharge in any newborn with a severe course
or preterm delivery, ideally at 1-2 months of age. (UW Health Very low quality evidence,
strong recommendation) All patients with fetal surgery and those who are status post
ECMO require MRI. (UW Health Low quality evidence, strong recommendation) However, we
have seen brain injury on MRI and other abnormalities in infants with CDH who have not
had either of these procedures.32




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Contact: Lee Vermeulen, CCKM@uwhealth.org Last Revised: 02/2017CCKM@uwhealth.org




23
UW Health Implementation
Potential Benefits:
ξ Improved patient mortality
ξ Reduction of practice variation

Potential Harms:
ξ Potential ototoxicity
ξ Chronic lung disease and long-term ventilation
ξ Neonatal drug dependence and withdrawal

Pertinent UW Health Policies & Procedures
1. Nursing Patient Care Policy 1.48-P- Care of Umbilical Catheters (Arterial and Venous)
2. Nursing Patient Care Policy 1.49-P- Use of Near-Infrared Spectroscopy (Cerebral and Somatic
Oximetry) in the Pediatric and Neonatal ICU
3. Nursing Patient Care Policy 2.20- Care and Maintenance of Enteral Tubes
4. Nursing Patient Care Policy 7.11-P- Care of the Intubated Patient
5. Nursing Patient Care Policy 7.12AP- Closed Chest Tube Drainage System
6. Respiratory Care Services Policy 1.53 (AFCH Initial Ventilator Management and Weaning Algorithms)
7. Respiratory Care Services Policy 2.02- Mechanical Ventilation Adult and Pediatric
8. Respiratory Care Services Policy 2.03- High Frequency Oscillatory Ventilation (HFOV)
9. Respiratory Care Services policy 3.42- Suctioning
10. Respiratory Care Services policy 3.53- Nitric Oxide
11. UW Health Clinical Policy 8.14- Guidelines for Administration of Continuous Invasive and Non-
Invasive Respiratory Support

Patient Resources
1. Kids Health- Genetic Counseling
2. Kids Health- Birth Defects

Guideline Metrics:
1. Mortality rate
2. Average timing of repair
3. Percentage of CDH patients requiring ECMO
4. Percentage of CDH patients with imaging studies completed prior to discharge

Implementation Plan/Tools
1. Guideline will be posted on uConnect in a dedicated location for Clinical Practice Guidelines.
2. Release of the guideline will be advertised in the Physician/APP Briefing newsletter.
3. Content and hyperlinks within clinical tools, documents, or Health Link related to the guideline
recommendations will be reviewed for consistency and modified as appropriate.

Practice Protocols
Pain and Agitation Continuous Infusion Titration – Pediatric – Inpatient [5]

Disclaimer
Clinical practice guidelines assist clinicians by providing a framework for the evaluation and treatment of patients.
This guideline outlines the preferred approach for most patients. It is not intended to replace a clinician’s judgment or
to establish a protocol for all patients. It is understood that some patients will not fit the clinical condition contemplated
by a guideline and that a guideline will rarely establish the only appropriate approach to a problem.
Copyright © 2017 Univ ersity of Wisconsin Hospitals and Clinics Authority
Contact: Lee Vermeulen, CCKM@uwhealth.org Last Revised: 02/2017CCKM@uwhealth.org




24
Appendix A. High Risk Perinatal Clinic Care Plan

Delivery room and early newborn management plan template – to be placed in maternal
chart following prenatal consult visit.

High Risk Perinatal Clinic Care Plan

Diagnosis: Fetal (side and type) Diaphragmatic Hernia

Background: Maternal history, pregnancy history, summary of antenatal testing

Impression from fetal ultrasound: Ultrasound conducted at *** weeks gestation
identified a ***-sided *** type diaphragmatic hernia. The lung-to-head ratio at that time
was measured to be *** with an observed to expected ratio of ***.

Impression from Fetal MRI: MRI conducted at *** weeks gestation identified a ***-
sided *** type diaphragmatic hernia containing ***. Heart is shifted to the ***. Estimated
total lung volume is *** mL with observed to expected lung volume ration of X. ***
additional anomalies were noted.

Summary: Incorporate data from ultrasound and MRI to give estimate of risk of
mortality and need for ECMO. Include references for data.

ANTEPARTUM PLAN

Estimated date of delivery:

Prenatal Care provider:
Fetal Concerns Care Coordinator:

Fetal surveillance schedule
Growth US:
Fetal echocardiogram:
Fetal MRI:

Prenatal Consults
Maternal Fetal Medicine:
Neonatology:
Pediatric Surgery:
Meriter Tour:
AFCH Tour:

Delivery Plan
Provider to manage delivery:
Reason for IOL:
Timing and Mode:

Contraception
Choice:
If tubal, papers signed:
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25
Notifications to be made upon admission of mother to Labor and Delivery (page
via U-Connect or call directly)
1. Admitting OB team notifies Meriter Neonatology team (mid-level, fellow, or
attending)
2. Neonatology team notifies attending pediatric surgeon of pending delivery
3. Neonatology team notifies AFCH NICU/PICU of pending delivery and eventual
need for transfer
a. A separate call will need to be made through the UW Access Center to
arrange transfer once the infant has been delivered

LABOR AND DELIVERY PLAN

Discuss location of resuscitation with labor and delivery team
Consider conducting resuscitation of CDH patients in the resuscitation area outside the
c-section OR if possible for improved access to resuscitation equipment and ease of
transport to the NICU.

Page for delivery: "C3 - Neonatologist needed, room number, reason for page"

Genetic testing from cord blood: (indicate which of the following is needed)
To be sent from Meriter
Chromosomes from Cord Blood - ORDER IN BABY CHART
1. Chromosomes - 5ml dark green sodium heparin tube
a. Order # LAB3252 - goes to State Lab
2. If NICU admission, send specimen with baby and NICU team will order.
3. Order under Pediatric provider, NNP or Neonatologist (NOT Resident)

Microarray from Cord Blood - ORDER IN BABY CHART
1. Microarray – 4ml dark green sodium heparin tube
a. Order "Lab Not Found - ", EAPLAB 2607
b. Write in comments: "Microarray to go to State Lab of Hygiene" DO NOT
ORDER "MICROARRAY" (instead do the "Lab not found") IN EPIC - if you
do, the sample will go to Mayo labs instead and we want it to go to State
Lab.)
2. If NICU admission, send specimen with baby and NICU team will order.
3. Order under Pediatric provider, NNP or Neonatologist (NOT Resident)

Resuscitation Plan
Red crash cart inside room

Consider use of shuttle for resuscitation
To minimize handling and movement after resuscitation consider using the shuttle and
warmer for the resuscitation. Discuss with charge nurse.




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26
Assign tasks to team members
Make sure team members are aware of their specific tasks during the resuscitation
(intubation, heart rate detection, pulse ox. placement, cord milking, emergency line
placement, medications, event recording . . .)

Orotracheal Intubation
Immediately after birth – to be performed by experienced provider (e.g., senior fellow,
experienced nurse practitioner, or attending). Bag-mask ventilation should be
avoided as it may cause gastric distention and impair lung inflation.
1. Be prepared with the following:
a. FiO2 at 1.00
b. 3.0 and 3.5 ET tube with stylet
c. 0 and 1 laryngoscope blades with laryngoscope handle
d. CO2 detector
e. Pulse oximeter probe
f. Suction
g. Panda warmer with T-piece resuscitator tubing connected and appropriate
size mask.
h. Panda warmer to have both oxygen (green) and air (yellow) tank for
transport of infant to NICU
i. Warm blankets for transport from DR to NICU

Cord Milking
Cord milking should be performed to improve oxygen carrying capacity of CDH infants
and can be done during intubation

Gastric Decompression
NICU charge nurse to bring 10F Replogle and 30ml syringe, place in DR

NEONATOLOGY CARE PLAN

General Philosophy
Delivery room and early newborn care should focus on establishing stability on the ventilator,
providing medication for sedation and pain control, decompressing the bowel, and placement of
venous and arterial access.

NICU room set-up
1. Infant to remain on Panda warmer from DR
2. Pre and post ductal saturation monitors
3. Umbilical line set-up: double-lumen UVC (3.5F for babies <1.2kg, 5F for babies
>1.2kg), single-lumen UAC (3.5F for preterm, 5F for term).
a. Have trays set-up and ready before the patient enters the unit
b. Have extra flush solutions (normal saline and heparin syringes) on trays
with extra stopcocks and extra red syringe-transfer adapters
4. 3 suction canisters: 1 for OG/NG to low continuous suction, 1 for ETT, 1 for oral
5. IV pole with multiple pumps at bedside
6. IV tubing and arterial transducer
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7. Have D10 TrophAmine® with heparin 0.5 units/mL for UVC and TrophAmine®
with heparin 0.5 units/mL for UAC ready at bedside.
8. Morphine for sedation ready at bedside. (bolus dose of 0.05-0.1 mg/kg followed
by an infusion to start at 10 mcg/kg/hour)

Admission Care: Nursing and Respiratory
1. Requires one-to-one nursing care plus charge RN to assist with admission
2. Place OG/NG to low intermittent suction
3. Place patient on servo control of 36.5 on RW, attach skin temperature probe
4. Place pre and post ductal sat probes (RUE, and either lower extremity)
5. RT present in NICU room at time of admission (NICU HUC to notify RT when
team is in DR)
6. RT places patient on ordered ventilator settings and is ready to add HFOV if
needed

Ventilator Management
Gentle ventilation: tolerance for hypercapnea (PCO2 <65) and hypoxemia (pre-ductal
sat > 85%).
1. Goal is to inflate lungs but minimize exposure to high pressure.
2. In first 1 – 2 hours saturations of 75 – 85% are acceptable while maximizing
ventilation, sedation, positioning, and giving 100% FiO2
3. Prefer ventilating with high rate over high pressure.

4. Initial Settings:
a. PIP 15 – 25
b. Rate 40 – 60
c. PEEP 3 – 5
d. iT 0.3 – 0.5
e. FiO2 – 1.00. Wean FiO2 slowly for pre-ductal Sat > 95%.

5. Consider HFOV if PIP >25

6. iNO PRN – MD must clearly document time of initiation, indication for use, and
patient response
a. Initiate for OI > 25 start at 20PPM
b. OI (oxygenation index) = [MAP x FiO2 x 100 ]/PaO2

7. Positioning: turn patient’s head toward the defect to optimize inflation of “good
lung” (i.e. position with affected side slightly down and head turned toward side of
diaphragmatic defect)

8. Surfactant – consider for RDS/prematurity only (< 36 weeks gestational age)

Vascular Access
Umbilical catheter access placed on arrival to NICU.
1. UAC
a. Single-Lumen: 3.5F for preterm, 5F for term
b. Depth: 3x + 9, x= birth weight in kg
c. Confirm position by x-ray- goal position is T6-8. A less desirable
alternative is a low-lying line at L3-4.
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d. Start Amino acids with heparin 0.5 units/mL at 1mL/hr

2. UVC
a. Double-lumen: 3.5F for babies<1.2kg, 5F for babies>1.2kg
b. Depth: 1.5x + 6, x= birth weight in kg
c. May confirm UVC using ultrasound or echocardiogram as x-ray can be
challenging to interpret due to malposition of the liver.
d. If position cannot be adequately verified consider pulling back to low-lying
position to provide access for maintenance fluids and sedation until PICC
line can be inserted.
e. Start D10 TrophAmine® with heparin 0.5 units/mL at 80 ml/kg/d (subtract
out UAC fluids)

Sedation
Sedation is required for all intubated infants with CDH - have morphine infusion ready
at patient bedside

1. Morphine 0.1 mg/kg IV as soon as possible after birth
a. Morphine infusion to start at 10 mcg/kg/hr (0.01 mg/kg/hr) with 0.05-0.1
mg/kg bolus every 2 hours PRN
2. Avoid paralysis unless requiring maximal respiratory support with persistent
hypoxemia
a. Consider rocuronium 0.45-0.6 mg/kg every 30 minutes PRN for initial
stabilization and transport only

Additional Measures
1. Consider normal saline (10 mL/kg) for low blood pressure, poor perfusion, or
metabolic acidosis

2. Consider dopamine for persistently low blood pressure and poor perfusion. Start
at 5 mcg/kg/min at 5 mcg/kg/min
a. Use echocardiogram to assist with volume resuscitation versus ionotropic
support for low blood pressure, acidosis, or poor perfusion

3. Consider antibiotics for routine indications (prolonged rupture of membranes or
chorioamnionitis) or in infants who are severely symptomatic (low blood
pressure, persistent metabolic acidosis, maximal respiratory support).
a. Due to increased risk of hearing impairment in infants with CDH consider
using ampicillin and cefotaxime

Imaging
1. Babygram after admission to confirm position of ETT, vascular access, and
gastric suction catheter

2. An echocardiogram should be considered if saturations remain low (< 80%
despite 100% FiO2) or for low blood pressure and poor perfusion, but is not
required in all patients.
a. Limited ECHO for PPHN to limit stimulation if extensive cardiac work-up
was done prenatally

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29
3. Head ultrasound should be considered if ECMO is likely but is not required in all
patients

Laboratory studies
1. ABG, Lactate, Glucose, Blood Culture, Complete Blood Count (CBC) with
manual differential
2. Blood gas parameters: pH > 7.20, PCO2 < 65, include lactate levels with all
blood gas draws

Continued Care – goal is to minimize PPHN and stabilize for transport
1. Minimal handling (decrease agitation to minimize effect of PPHN) and minimize
noise and light
2. Maintain mean arterial pressures 40-50 and consider dopamine if lower
3. Supportive positioning (position with affected side slightly down and head turned
toward side of diaphragm defect)
4. Discuss timing of echocardiogram and head ultrasound with PICU attending
(especially if need for ECMO is likely soon after transfer to PICU).
5. Emotional support to parents: request spiritual care/baptism as able prior to
transport

Transport to AFCH
1. Shortly after admission to Meriter NICU, attending Neonatologist will call AFCH
PICU through UW Access Center (608-263-3260) or AFCH NICU through Mother
Baby Care Access Center (866-678-7636) to arrange transport
2. Ideally transport will occur by 6 – 8 hours, but no longer than 12 hours after birth
3. Transport will be done by CHETA team if available, but consider sending
experienced neonatal provider (e.g. senior fellow, experienced NP or hospitalist,
or Neonatologist) for patients who require frequent active management


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30
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Copyright © 2017 Univ ersity of Wisconsin Hospitals and Clinics Authority
Contact: Lee Vermeulen, CCKM@uwhealth.org Last Revised: 02/2017CCKM@uwhealth.org