PEDBASE.org - The Pediatric Database - Detailed information of EVENTRATION OF THE DIAPHRAGM

EVENTRATION OF THE DIAPHRAGM

DEFINITION:

A congenital anomaly of the diaphragm characterized by respiratory distress and recurrent cough and pneumonia.

EPIDEMIOLOGY:

incidence: ?
age of onset:
- newborn or childhood

PATHOGENESIS:

1. Etiology

1. Congenital

maldevelopment of either the diaphragmatic muscle or phrenic nerve
may be a complete or partial absence of muscular development in the septum transversum

2. Acquired

usually interruption of the phrenic nerve following a traumatic birth or thoracic operation for a congenital heart lesion leading to phrenic nerve paralysis or dysfunction

2. Characteristics

1. Types

total - more frequent on the left
partial or localized - more frequent on the right

2. Associations

may be associated with other congenital abnormalities such as a high renal ectopia or extralobar pulmonary sequestration

3. Pathogenesis

a weakened diaphragmatic muscle results in the upward displacement of abdominal contents into an outpouching of the diaphragm resulting in respiratory compromise

DIFFERENTIAL DIAGNOSIS:

diaphragmatic hernia
phrenic nerve paralysis
tumor, cyst, pleural effusion

CLINICAL FEATURES:

may be asymptomatic even in the presence of a large eventration

1. Respiratory Manifestations

highly variable

1. Severe

respiratory distress with dyspnea, tachypnea, cyanosis
tracheal and cardiac shift
dullness and decreased breath sounds over affected area
asynchronous chest wall movement

2. Mild

recurrent wheeze, chronic cough, pneumonia
exercise intolerance

2. Gastrointestinal Manifestations

nausea and vomiting
flatulence and indigestion
scaphoid upper abdomen

3. Complications

chronic pulmonary suppuration
diaphragmatic rupture
ulcer and volvulus of stomach
death

INVESTIGATIONS:

1. Imaging Studies

1. Chest X-Ray

diaphragmatic elevation
elevation and angulation of the stomach
mediastinal shift
atelectasis

2. Fluoroscopy

diagnostic
mild - minimal synchronous diaphragmatic excursions
severe - paradoxical diaphragmatic excursions

2. Pathology

absence or diminution of the diaphragmatic muscle
replacement of degenerated muscle fibres by fibrous tissue
phrenic nerve is smaller but no evidence of degeneration

MANAGEMENT:

1. Supportive

if only mild respiratory difficulties

2. Surgical

intubate and ventilate
for severe cases with respiratory distress or where ipsilateral lung growth may be impeded
plication to fix and lower the diaphragm
if occurs after surgery then treat conservatively with intubation and ventilation for 2-4 weeks and then trial of extubation, if dyspnea persists then thoracotomy and plication may be needed

Pediatric Database - EVENTRATION OF THE DIAPHRAGM

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