CDH

ECMO

Ventilation

May improve outcomes

Decreased volume of herniated viscera

Used after failure of Conventional ventilation

Long duration, 7-14 days up to 4 weeks

Short term treatment is all about ventilation

Goal Sats% >85

Avoid prolonged mask ventilation

Avoid Barotrauma-permissive hypercapnia

Conventional vs HFOV vs ECMO

HF not usually helpful-High pressure to recruit atelectatic lung not effective

Conventional-RR 30-60, PIP <25

Surfactant, iNO have not shown benefit

Treatment

Congenital Diaphragmatic Hernia

Resources

1. Nelsons

2. Fanaroff

3. Uptodate

Prognosis/Outcomes

Prenatal Factors

LHR

Study <1 no survivors, >1.4 all survived

Liver in thoracic cavity is negative prognostic factor

Post Natal Risk factors

Early symptoms, other major anomalies, severe hypoplasia, ECMO

Long term survival is 67%

Pulmonary problems dominate-

1-2% require oxygen at 1yr

BPD-improves with growth and alveoli development

Delayed growth-catch up by 2yr

Diagnosis and Treatment

Neurocognitive issues

GERD-often requiring surgery

Diagnosis is fairly staright forward, with a couple of interesting details-

Prenatal U/S between 16-24 weeks-50%, MRI

Polyhydramnios, chest mass, mediastinal shift, stomach or liver in chest, hydrops

LHR

Clinically-Respiratory distress within 48 hours

Scaphoid abdomen, increased chest diameter,

decreased BS, bowel sounds

CXR, but not always easy-DDx cystic masses CT

NG tube

Pathogenesis

CDH

Herniation of abominal contents into thoracic cavity via a diaphragmatic defect

Types

Hiatal, paraesophageal, restrosternal, posterolateral

Bochdalek-90%, Morgagni 2-6%

Small hole to complete agenesis of area

What is the Problem?

Delayed presentation-

Associated with GBS sepsis and rare sudden death

As for treatment . . .

Pulmonary hypoplasia

Pulmonary Hypoplasia- yes, but . . .

PPHN, especially early is often the main concern

Compression, but may start before development of defect

Decrease in mass, bronchioles, alveoli

Thickened alveoli and arterioles

Biochemical-decreased surfactant, PC, DNA, and lung protein with increased glycogen

Epidemiology

1/2000-5000

2:1

:

5% bilateral

Mostly sporadic, some familial cases

Many Associations

Case

Associations

30% of cases have associated anomalies

CNS, esophageal atresia, omphalocoele, CV

1

: navicular a

2

: the bone of the thumb side of the carpus that is the largest in the proximal row; also : the navicular bone of the tarsus

— scaphoid adjective

Origin of SCAPHOID

New Latin scaphoides, from Greek skaphoeidēs, from skaphos boat

First Known Use: 1831

Part of:

Trisomies 13, 18, 21

Fryns, Bachmann-de Lange, Pallister-Killian and Turner Syndromes

A newborn develops respiratory distress immediately after birth. His abdomen is scaphoid. No breath sounds are heard on the left side of his chest, but they are audible on the right. Immediate intubation is successful with little or no improvement in clinical status.

Kind of a big topic . . .

Surgery

Surgery is the definitive treatment

Ideal Time is unknown

Usually wait >48hrs

Some wait until off ECMO

Subcostal approach Thorascopic/Laparscopic

Primary repair vs Gore-tex patch Complications-PHTN, chylothorax,

bowel obstruction

All the Rest

In utero repair shows no benefit

Partial Liquid Ventilation (PLV)

Recruits collapsed alveoli-Increasing FRC-improving both V/Q matching and compliance

May also increase surfactant production