congenital lobar emphysema

Congenital Lobar Emphysema 

Definition

Congenital lobar emphysema is a chronic disease that causes respiratory distress in infants.

Description

Congenital lobar emphysema, also called infantile lobar emphysema, is a respiratory disease that occurs in infants when air enters the lungs but cannot leave easily. The lungs become over-inflated, causing respiratory function to decrease and air to leak out into the space around the lungs.

Half of the cases of congenital lobar emphysema occur in the first four weeks of life, and three-quarters occur in infants less than six months old. Congenital lobar emphysema is more common in boys than in girls.

Each person has two lungs, right and left. The right lung is divided into three sections, called lobes, and the left lung into two lobes. Congenital lobar emphysema usually affects only one lobe, and this is usually an upper lobe. It occurs most frequently in the left upper lobe, followed by the right middle lobe.

Causes and symptoms

The cause of congenital lobar emphysema often cannot be identified. The airway may be obstructed or the infant's lungs may not have developed properly. Congenital lobar emphysema is almost never of genetic origin.

Symptoms of congenital lobar emphysema include:

• shortness of breath
• wheezing
• lips and fingernail beds that have a bluish tinge

Diagnosis

Congenital lobar emphysema is usually identified within the first two weeks of the infant's life. It is diagnosed by respiratory symptoms and a chest x ray, which shows the over-inflation of the affected lobe and may show a blocked air passage.

Treatment

For infants with no, mild, or intermittent symptoms, no treatment is necessary. For more serious cases of congenital lobar emphysema, surgery is necessary, usually a lobectomy to remove the affected lung lobe.

Alternative treatment

Alternative treatments that may be helpful for congenital lobar emphysema are aimed at supporting and strengthening the patient's respiratory function. Vitamin and mineral supplementation may be recommended as may herbal remedies such as lobelia (Lobelia inflata) that strengthen the lungs and enhance their elasticity. Homeopathic constitutional care may also be beneficial for this condition.

Key terms

Congenital — A disease or condition that is present at birth.

Emphysema — A condition in which the air sacs in the lungs become overinflated, causing a decrease in respiratory function.

Lobar — Relating to a lobe, a rounded projecting part of the lungs.

Prognosis

Surgery for congenital lobar emphysema has excellent results.

Prevention

Congenital lobar emphysema cannot be prevented.

Resources

Organizations

American Lung Association. 1740 Broadway, New York, NY 10019. (800) 586-4872. http://www.lungusa.org.

National Heart, Lung and Blood Institute. P.O. Box 30105, Bethesda, MD 20824-0105. (301) 251-1222. http://www.nhlbi.nih.gov.

National Jewish Center for Immunology and Respiratory Medicine. 1400 Jackson St., Denver, CO 80206. (800) 222-5864. 〈http://www.nationaljewish.org/main.html〉.

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emphysema /em·phy·se·ma/ (em″fĭ-se´mah)

1. a pathologic accumulation of air in tissues or organs.

2. pulmonary e.emphysem´atous

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atrophic emphysema  senile e.

bullous emphysema  single or multiple large cystic alveolar dilatations of lung tissue.

centriacinar emphysema , centrilobular emphysema focal dilatations of respiratory bronchioles rather than alveoli, throughout the lung among normal lung tissue.

congenital lobar emphysema  overinflation of a lung, usually in early life in one of the upper lobes, with respiratory distress.

hypoplastic emphysema  pulmonary emphysema due to a developmental anomaly, with fewer and abnormally large alveoli.

infantile lobar emphysema  congenital lobar e.

interlobular emphysema  air in the septa between lung lobules.

interstitial emphysema  air in the peribronchial and interstitial tissues of the lungs.

intestinal emphysema  pneumatosis cystoides intestinalis.

mediastinal emphysema  pneumomediastinum.

obstructive emphysema  that associated with partial bronchial obstruction that interferes with exhalation.

panacinar emphysema , panlobular emphysema a type characterized by enlargement of air spaces throughout the acini.

pulmonary emphysema  abnormal increase in size of lung air spaces distal to the terminal bronchioles.

pulmonary interstitial emphysema  (PIE) a condition seen mostly in premature infants, in which air leaks from lung alveoli into interstitial spaces, often because of underlying lung disease or use of mechanical ventilation.

senile emphysema  overdistention and stretching of lung tissues due to atrophic changes.

subcutaneous emphysema  air or gas in subcutaneous tissues, usually caused by intrathoracic injury.

surgical emphysema  subcutaneous emphysema following surgery.

vesicular emphysema  panacinar e.

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congenital lobar emphysema

Etymology: L, congenitus, born with; Gk, lobos, lobe; Gk, en, in + physema, a blowing

a condition characterized by overinflation, commonly affecting one of the upper lobes and causing respiratory distress in early life. Also called congenital lobar overinflation.

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lobar

pertaining to a lobe.

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congenital lobar emphysema

emphysema of one or more lung lobes usually the result of bronchial dysplasia or agenesis in the neonate.

lobar pneumonia

pneumonia affecting one or more lobes of the lungs. See also lobar pneumonia.