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Treatment
Early
versus late repair:
–
Rapid
reduction of the herniated abdominal viscera from the thorax would acutely
relieve progressive hypoxia and acidosis
–
Delay
the repair until adequate gas exchange with minimal intracardiac shunt
is achieved (deterioration in lung compliance after repair)
Ventilatory
support:
Conventional or HFOV
–
Permissive
hypercapnia as long as pH can be buffered with bicarbonate
Goal:
Preductal O2 Sat greater than 90% in the absence of metabolic acidosis
–
ECMO
when previous treatment failed
Pulmonary Morbidity in 100 survivors of CDH Monitored in a Multidisciplinary Clinic
Use of ECMO and patch repair are independent predictors of delay in extubation and delay in discharge
ECMO
survivor:
40% probability of the need for O2 at discharge
50% chance of having a patch repair
79% chance of requiring diuretics at discharge
72% probability of needing Bronchodilators and
55% inhaled Steroids at discharge or in the 1st year of life
Oxygen
at discharge:
16 patients.
Mean duration 14.5 months
Beta
adrenergic medications, corticosteroids:
Majority patients
Diuretics:
Majority of ECMO patients
Chest
x-rays:
80% abnormal
Arterial
blood gases:
< 10% patients with mild CO2 retention in the first year of life.
V/Q
scan were done every 6 to 12 months in patients < 5 years old
ECMO
is associated with left to right shunt and V/Q mismatch.
Ventilation
improved with time but perfusion remained unchanged
Spirometry
> 5 y:
27% Obstructive Airway Disease (23% if they had a prior V/Q mismatch)
RSV prophylaxis < 2 y
Associated
morbidity:
–
Developmental
delay
–
Poor
growth
–
Gastroesophageal reflux
–
Hearing
loss
–
Musculoskeletal abnormalities
–
Prosthetic patch reherniation
Muratore
et al, Boston. Jour Ped Surg Jan, 2001
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