EndoScoops
In a first, an Ormond Beach, Fla, cardiothoracic surgeon used thoracoscopy to obtain biopsy material to diagnose a case of lymphangiomyomatosis, a rare, potentially lethal lung disease affecting young women.
When he performed thoracoscopy on a 32-year-old woman suffering from recurrent spontaneous pneumothorax, William H. Johnson, MD, assumed he was treating pneumothorax caused by congenital blebs or some other usual cause. He resected some apical blebs and performed a standard pleurodesis; the patient subsequently did well.
But upon reviewing the resected material, the pathologist found that she had lymphangiomyomatosis. She was referred to a physician at the National Institutes of Health (NIH) who runs an ongoing treatment program.
"We were able to diagnose this woman's very rare problem serendipitously by doing a bleb resection through thoracoscopic means," Dr. Johnson said. "She can now get treatment that may extend her life."
Dr. Johnson, who is chief of surgery at Ormond Memorial Hospital, said that because the woman underwent a thoracoscopic lung resection, she recovered from the operation more quickly and was ready to start the NIH treatment protocol for her disease sooner than if she had undergone a thoracotomy.
Research has shown that therapy should be initiated as soon as the diagnosis of lymphangiomyomatosis is established because of the progressive nature of the disease. The currently recommended therapy in woman's is oophorectomy and daily progesterone. According to Dr. Johnson, lymphangiomyomatosis, though not a malignant disease, is characterized by smooth muscle proliferation throughout the bronchi, blood vessels and lymphatic vessels of the lung. Most patients die from progressive pulmonary insufficiency within 10 years.
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