Fibrocystic Pulmonary Dysplasia

Genetically transmitted respiratory disease characterized by rapidly progressive dyspnea and cyanosis, digital clubbing, polycythemia, pulmonary hypertension, and diffuse pulmonary fibrosis (fatal within months of onset).

Corrigan Cirrhosis; Cryptogenetic Fibrosing Alveolitis; Familial Interstitial Lung Disease; Hamman Rich Disease; Idiopathic Pulmonary Fibrosis; Liebow Pneumonia; Usual Interstitial Pneumonia of Liebow.

First described by Sir Dominic John Corrigan in 1838. Respiratory disorder characterized by chronic nonproductive cough and dyspnea with changes in the diffusion capacity of the lung. The disease evolves toward respiratory failure and death.

Prevalence: 1.34:1,000,000 population in the United Kingdom. Occurs more frequently in males than in females.

Autosomal dominant.

Could result from an inherited anomaly in the immune system, but it is yet to be confirmed.

Based on clinical, radiologic, and respiratory function tests and lung biopsy if necessary. Radiologic changes show a nodular ground-glass appearance, predominantly in the bases. Respiratory function tests indicate a diffusion defect that is associated with a decrease in arterial oxygen saturation made worse by exercise. Usually, there is no restrictive pulmonary disorder until very late in the disease. Biopsy demonstrates the presence of interstitial fibrosis and cysts.

Affected patients present with a nonproductive cough that is associated with dyspnea, chest pain, and cyanosis. The disease evolves over many years toward hypoxemia, respiratory failure, and right heart failure. It can be exacerbated by spontaneous pneumothorax and recurrent infections. Clubbing and pulmonary osteoarthropathy are usually present. Patients become very disabled in the third or fourth decade, and death usually occurs in their 50s or earlier if they are victims of complications.

Before going to the operating room, the patient should be evaluated by a pneumologist for respiratory function testing, arterial blood gas, recurrent infections, and exacerbation of symptoms. Also obtain a chest radiograph and an ECG. If right heart failure is suspected, an echocardiogram is recommended. An anesthesiology consultation is highly recommended for elective surgery.

Because patients are “respiratory cripples,” tracheal intubation should be avoided as much as possible to prevent serious postoperative complications, including prolonged mechanical ventilation. Regional anesthesia may be preferable.

Be careful when using opioids alone or in combination with benzodiazepines because patients may be sensitive to the respiratory depressant effect of the medication.