Respiratory distress syndrome

Definition

Respiratory distress syndrome (RDS) of the newborn, also known as infant RDS, is an acute lung disease present at birth, which usually affects premature babies. Layers of tissue called hyaline membranes keep the oxygen that is breathed in from passing into the blood. The lungs are said to be airless. Without treatment, the infant will die within a few days after birth, but if oxygen can be provided, and the infant receives modern treatment in a neonatal intensive care unit, complete recovery with no after-effects can be expected.

Description

If a newborn infant is to breathe properly, the small air sacs (alveoli) at the ends of the breathing tubes must remain open so that oxygen in the air can get into the tiny blood vessels that surround the alveoli. Normally, in the last months of pregnancy, cells in the alveoli produce a substance called surfactant, which keep the surface tension inside the alveoli low so that the sacs can expand at the moment of birth, and the infant can breathe normally. Surfactant is produced starting at about 34 weeks of pregnancy and, by the time the fetal lungs mature at 37 weeks, a normal amount is present.

If an infant is born prematurely, enough surfactant might not have formed in the alveoli causing the lungs to collapse and making it very difficult for the baby to get enough air (and the oxygen it contains). Sometimes a layer of fibrous tissue called a hyaline membrane forms in the air sacs, making it even harder for oxygen to get through to the blood vessels. RDS in newborn infants used to be called hyaline membrane disease.

Demographics

According to the National Heart, Lung, and Blood Institute, in 2003, approximately 40,000 infants and 150,000 adults were reported to have RDS. Translated, these figures means RDS affected about one person in 6,800.

Causes and symptoms

RDS nearly always occurs in premature infants, and the more premature the birth, the greater is the chance that RDS will develop. RDS also is seen in some infants whose mothers are diabetic. Paradoxically, RDS is less likely in the presence of certain states or conditions which themselves are harmful: abnormally slow growth of the fetus; high blood pressure, a condition called preeclampsia in the mother; and early rupture of the birth membranes.

Labored breathing (the respiratory distress of RDS) may begin as soon as the infant is born, or within a few hours. Breathing becomes very rapid, the nostrils flare, and the infant grunts with each breath. The ribs, which are very flexible in young infants, move inwards each time a breath is taken. Before long the muscles that move the ribs and diaphragm, so that air is drawn into the lungs, become fatigued. When the oxygen level in the blood drops severely the infant's skin turns bluish in color. Tiny, very premature infants may not even have signs of trouble breathing. Their lungs may be so stiff that they cannot even start breathing when born.

There are two major complications of RDS. One is called pneumothorax, which means "air in the chest." When the infant itself or a breathing machine applies pressure on the lungs in an attempt to expand them, a lung may rupture, causing air to leak into the chest cavity. This air causes the lung to collapse further, making breathing even harder and interfering with blood flow in the lung arteries. The blood pressure can drop suddenly, cutting the blood supply to the brain. The other complication is called intraventricular hemorrhage; this is bleeding into the cavities (ventricles) of the brain, which may be fatal.

When to call the doctor

A doctor attending a birth should recognize respiratory distress and immediately begin appropriate treatment. A doctor should be called if a baby delivered outside of a hospital setting is observed to have any difficulty in breathing or whose skin becomes blue in color (cyanotic).

Diagnosis

When a premature infant has obvious trouble breathing at birth or within a few hours of birth, RDS is an obvious possibility. If premature birth is expected, or there is some condition that calls for delivery as soon as possible, the amount of surfactant in the amniotic fluid will indicate how well the lungs have matured. If little surfactant is found in an amniotic fluid sample taken by placing a needle in the uterus ( amniocentesis ), there is a definite risk of RDS. Often this test is done at regular intervals so that the infant can be delivered as soon as the lungs are mature. If the membranes have ruptured, surfactant can easily be measured in a sample of vaginal fluid.

The other major diagnostic test is a chest x ray. Collapsed lung tissue has a typical appearance, and the more lung tissue is collapsed, the more severe the RDS. An x ray also can demonstrate pneumothorax, if this complication has occurred. The level of oxygen in the blood can be measured by taking a blood sample from an artery, or, more easily, using a device called an oximeter, which is clipped to an earlobe. Pneumothorax may have occurred if the infant suddenly becomes worse while on ventilation; x rays can help make the diagnosis.

Treatment

If only a mild degree of RDS is present at birth, placing the infant in an oxygen hood may be enough. It is important to guard against too much oxygen, as this may damage the retina and cause loss of vision. Using an oximeter to keep track of the blood oxygen level, repeated artery punctures or heel sticks can be avoided. In more severe cases a drug very like natural surfactant (Exosurf Neonatal or Survanta) can be dripped into the lungs through a fine tube (endotracheal tube) placed in the infant's windpipe (trachea). Typically, the infant will be able to breathe more easily within a few days at the most, and complications such as lung rupture are less likely to occur. The drug is continued until the infant starts producing its own surfactant. There is a risk of bleeding into the lungs from surfactant treatment; about 10 percent of the smallest infants are affected.

Infants with severe RDS may require treatment with a ventilator, a machine that takes over the work of the lungs and delivers air under pressure. In tiny infants who do not breathe when born, ventilation through a tracheal tube is an emergency procedure. Assisted ventilation must be closely supervised, as too much pressure can cause further lung damage. A gentler way of assisting breathing, continuous positive airway pressure (CPAP), delivers an oxygen mixture through nasal prongs or a tube placed through the nose rather than an endotracheal tube. CPAP may be tried before resorting to a ventilator or after an infant placed on a ventilator begins to improve. Drugs that stimulate breathing may speed the recovery process.

Pneumothorax is an emergency condition that must be treated right away. Air may be removed from the chest using a needle and syringe. A tube then is inserted into the lung cavity, and suction applied.

Prognosis

If an infant born with RDS is not promptly treated, lack of an adequate oxygen supply will damage the body's organs and eventually cause them to stop functioning altogether. Death is the result. The central nervous system in particular—made up of the brain and spinal cord—is very dependent on a steady oxygen supply and is one of the first organ systems to feel the effects of RDS. By contrast, if the infant's breathing is supported until the lungs mature and make their own surfactant, complete recovery within three to five days is the pattern.

KEY TERMS

Alveoli —The tiny air sacs clustered at the ends of the bronchioles in the lungs in which oxygen-carbon dioxide exchange takes place.

Amniotic fluid —The liquid in the amniotic sac that cushions the fetus and regulates temperature in the placental environment. Amniotic fluid also contains fetal cells.

Endotracheal tube —A hollow tube that is inserted into the trachea (windpipe) through the nose or mouth. It is used to administer anesthesia, to deliver oxygen under pressure, or to deliver medications (e.g. surfactants).

Hyaline membrane —A fibrous layer that settles in the alveoli in respiratory distress syndrome and prevents oxygen from escaping from inhaled air to the bloodstream.

Pneumothorax —A collection of air or gas in the chest or pleural cavity that causes part or all of a lung to collapse.

Preeclampsia —A condition that develops after the twentieth week of pregnancy and results in high blood pressure, fluid retention that doesn't go away, and large amounts of protein in the urine. Without treatment, it can progress to a dangerous condition called eclampsia, in which a woman goes into convulsions.

Steroid —A class of drugs resembling normal body substances that often help control inflammation in the body tissues.

Surfactant —A protective film secreted by the alveoli in the lungs that reduces the surface tension of lung fluids, allowing gas exchange and helping maintain the elasticity of lung tissue. Surfactant is normally produced in the fetal lungs in the last months of pregnancy, which helps the air sacs to open up at the time of birth so that the newborn infant can breathe freely. Premature infants may lack surfactant and are more susceptible to respiratory problems without it.

Ventilator —A mechanical device that can take over the work of breathing for a patient whose lungs are injured or are starting to heal. Sometimes called a respirator.

If an air leak causes pneumothorax, immediate removal of air from the chest allows the lungs to re-expand. Bleeding into the brain is a very serious condition that worsens the outlook for an infant with RDS.

Prevention

The best way of preventing RDS is to delay delivery until the fetal lungs have matured and are producing enough surfactant, generally at about 37 weeks of pregnancy. If delivery cannot be delayed, the mother may be given a steroid hormone, similar to a natural substance produced in the body, which crosses the barrier of the placenta and helps the fetal lungs to produce surfactant. The steroid should be given at least 24 hours before the expected time of delivery. If the infant does develop RDS, the risk of bleeding into the brain will be much less if the mother has been given a dose of steroid.

If a very premature infant is born without symptoms of RDS, it may be wise to deliver surfactant to its lungs. This may prevent RDS or make it less severe if it does develop. An alternative is to wait until the first symptoms of RDS appear and then immediately give surfactant. Pneumothorax may be prevented by frequently checking the blood oxygen content and limiting oxygen treatment under pressure to the minimum needed.

Parental concerns

Parents should monitor their newborn infant's breathing status closely for the first week of life. Premature infants are of particular concern, although many may be hospitalized through the neonatal period. While a newborn is hospitalized, parents should try to maintain as much physical contact with the infant as is allowed by the hospital, and let the infant frequently hear the familiar voices of the parents, especially the mother, when physical contact is not recommended.

Resources

BOOKS

Beamis, John F., et al. Interventional Pulmonary Medicine. New York: Marcel Dekker, 2003.

Frankel, Larry R. "Respiratory Distress and Failure." In Nelson Textbook of Pediatrics , 17th ed. Edited by Richard E. Behrman et al. Philadelphia: Saunders, 2003, pp. 301–2.

Hanley, Michael E., and Carolyn H. Welsh. Current Diagnosis & Treatment in Pulmonary Medicine. New York: McGraw-Hill, 2003.

PERIODICALS

Bandi, V. D., et al. "Acute lung injury and acute respiratory distress syndrome in pregnancy." Critical Care Clinics 20, no. 4 (2004): 577–607.

Dicker, R. A., et al. "Acute respiratory distress syndrome criteria in trauma patients: why the definitions do not work." Journal of Trauma 57, no. 3 (2004): 522–6.

Louis, J. M., et al. "Perinatal intervention and neonatal outcomes near the limit of viability." American Journal of Obstetrics and Gynecology 191, no. 4 (2004): 1398–402.

Verger, J. T., et al. "The pragmatics of feeding the pediatric patient with acute respiratory distress syndrome." Critical Care Nursing Clinics of North America 16, no. 3 (2004): 431–43.

ORGANIZATIONS

American Academy of Emergency Medicine. 611 East Wells St., Milwaukee, WI 53202. Web site: http://www.aaem.org/.

American Academy of Family Physicians. 11400 Tomahawk Creek Parkway, Leawood, KS 66211–2672. Web site: http://www.aafp.org/.

American Academy of Pediatrics. 141 Northwest Point Boulevard, Elk Grove Village, IL 60007–1098. Web site: http://www.aap.org/default.htm.

American College of Emergency Physicians. PO Box 619911, Dallas, TX 75261–9911. Web site: http://www.acep.org/.

American Lung Association. 1740 Broadway, New York, NY 10019. Web site: http://www.lungusa.org/diseases/lungtb.html.

American Thoracic Society. 1740 Broadway, New York, NY 10019. Web site: http://www.thoracic.org/.

Canadian Cystic Fibrosis Foundation. 2221 Yonge St., Suite 601, Toronto, Ontario, M4S 2B4, Canada. Web site: http://www.ccff.ca/home.cfm.

Cystic Fibrosis Foundation. 6931 Arlington Road, Bethesda, MD 0814. Web site: http://www.cff.org/.

WEB SITES

"Infant Respiratory Distress Syndrome." Penn State Children's Hospital. Available online at http://www.hmc.psu.edu/childrens/healthinfo/r/respiratorydistress.htm (accessed December 23, 2004).

"Respiratory distress syndrome (RDS) in infants." National Library of Medicine. Available online at http://www.nlm.nih.gov/medlineplus/ency/article/001563.htm (accessed December 23, 2004).

Rothenhaus, Todd. "Acute Respiratory Distress Syndrome." eMedicine , May 16, 2003. Available online at http://www.emedicine.com/EMERG/topic15.htm (accessed December 23, 2004).

Udobi, Kahdi, and Ed Childs. "Acute Respiratory Distress Syndrome." American College of Family Physicians. Available online at http://www.aafp.org/afp/20030115/315.html (accessed December 23, 2004).

L. Fleming Fallon, Jr., MD, DrPH