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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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