Cystic lung disease encompasses a group of acquired and congenital disorders with distinct features but plenty of overlap among the disease characteristics. The term "cystic" refers to small lesions in the lung containing air or fluid, called cysts. Since the first successful treatment for cystic lung disease was developed in 1933, advances in medicine have resulted in a high success rate for treating cystic lung disease and its complications.
Classification
Cystic lung disease encompasses four categories of disease: bronchogenic cysts, cystic adenomatoid malformation, lobar emphysema, and pulmonary sequestration. Bronchogenic cysts arise from abnormal folding of tissue during fetal development. These cysts form in the midline chest area, such as the trachea and esophagus, which compromises nearby airways, according to an article in the September 2007 issue of "Thorax." Cystic adenomatoid malformations are non-cancerous, or benign, masses of abnormal lung tissue that generally occur on one side of the lung. Lobar emphysema occurs when an obstruction in one lung lobe traps airflow. This trapped air stretches and expands the lung tissue, leading to cyst development. Pulmonary sequestration is a mass of lung tissue that is not connected to any airways and thus performs no function. The sequestration can be either inside or outside the lungs.
Causes
Cystic lung disease develops from either a developmental abnormality (congenital) or is acquired from an infection, injury, or as a complication of another disease. Congenital cystic lung disease can arise from a variety of developmental anomalies, such as congenital pulmonary airway malformation or trisomy 21-associated peripheral pulmonary cysts, according to an article published in the August 2009 issue of "Fetal and Pediatric Pathology." The type of cystic lung disease that develops is often contingent upon the timing of the insult during fetal development. Acquired cystic lung disease can arise from trauma to the lungs, as a complication from infection, or from degenerative diseases such as interstitial pulmonary emphysema.
Symptoms
The signs and symptoms of cystic lung disease resemble many other lung diseases and conditions, so it is important to consult a physician to receive an evaluation and correct diagnosis. Common symptoms include labored and painful breathing, shortness of breath, wheezing and recurrent pneumonia. Complications may arise from cystic lung disease, and some of these can be severe, such as acute respiratory distress, circulatory collapse, increased risk of infection, and heart failure, according to an article published in the February 2008 issue of "Seminars in Pediatric Surgery."
Diagnosis
The majority of cystic lung diseases are congenital and a result of abnormal fetal development. Therefore, prenatal ultrasound commonly identifies the condition before the child is born, especially cystic adenomatoid malformations and bronchogenic cysts. After birth, babies with cystic lung disease may show signs of respiratory distress and have the condition diagnosed using a CT scan of the lungs or a chest x-ray, according to a February 2008 article in "Seminars in Pediatric Surgery." Confirmation tests are needed if the chest radiography is negative but symptoms are still present. Confirmation tests may include a CT scan, ultrasonography, or contrast esophagography.
Treatment
Treatment options for cystic lung disease exist and are dependent on many factors such as age, overall health, and extent of the disease. Congenital cystic adenomatoid malformations are often treated by surgical removal of the solid mass and surgical aspiration, a procedure in which fluid is suctioned from the cysts. Pulmonary and lobar emphysema generally require a lobectomy in which the affected lobe is removed. A lobectomy has a high success rate and generally results in little or no permanent breathing impairments, according to Children's Hospital Boston. Bronchogenic cysts may be treated with minimally invasive thoracoscopic surgery.
