Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease commonly complicated by pneumothorax and chylothorax. The Center for LAM and Rare Lung Diseases at New York-Presbyterian and Columbia, which was founded and run by Monica Goldklang, M.D., and Jeanine D’Armiento, M.D., Ph.D., has one of the largest series of LAM patients worldwide. Using this series, Bryan P. Stanifer, M.D., a thoracic surgeon at NewYork-Presbyterian and Columbia, and his colleagues conducted a study aimed to define the optimal management for these LAM-related complications.
Below, Dr. Stanifer discusses the presentation of LAM, ways to manage pleural complications in the condition, and urges clinicians to include LAM in their differential diagnosis for women presenting with pneumothorax or chylothorax.
The standard of care for patients who come in with a spontaneous pneumothorax would be to not operate on them at the initial presentation. However, if this approach is used in patients with LAM, the recurrence rate is approximately 90%.
— Dr. Bryan Payne Stanifer
Research Background
LAM primarily affects women of reproductive age. Most patients with LAM are asymptomatic and tend to be diagnosed when they present with a pneumothorax. The standard of care for patients who come in with a spontaneous pneumothorax would be to not operate on them at the initial presentation. However, if this approach is used in patients with LAM, the recurrence rate is approximately 90%. Due to the high risk of recurrence, our study recommends ipsilateral surgical pleurodesis with indwelling pleural catheter (IPC) placement upon first pneumothorax in LAM patients. Unfortunately, LAM is so rare that it is oftentimes not diagnosed or misdiagnosed, which can lead to missed opportunities for surgical intervention. Complicating matters further, chylous effusions occur infrequently and are recalcitrant in nature, making management of this complication complex and variable across institutions.
Our study was a retrospective chart review of 326 patients with possible LAM seen at the Center for LAM and Rare Lung Diseases at NewYork-Presbyterian and Columbia between January 1, 2000, and March 1, 2023. The purpose of the study was to explore options for the surgical management of pleural disease in LAM.
Key Findings
Of the 326 patients with suspected or confirmed LAM, 16.6% had a history of pneumothorax. Additionally, of these patients:
- 44.4% underwent mechanical pleurodesis
- 18.5% underwent chemical pleurodesis
- 14.8% required pleurectomy
- 7.4% were discharged with tunneled IPC
In patients without initial pleurodesis, 87.1% experienced a recurrent pneumothorax.
We also looked at the incidence of chylous effusions in our cohort. Only 5.6% of patients with confirmed LAM had chylous effusions:
- 25% underwent thoracic duct ligation
- 16.7% underwent thoracic duct embolization
- 33.3% had pleurodesis
Of these patients, 50% underwent pleurodesis with long-term tunneled IPC placement.
If you have a suspected LAM case, we advocate that you should operate the first time, which is a departure from the typical collapsed lung case.
Clinical Implications
In young women with cystic lung disease, clinicians need to have a high index of suspicion of LAM. Clinicians should be aware that there are clinical diagnostic criteria that they can use to diagnose LAM without doing a lung biopsy. If patients do not meet these criteria, clinicians can safely perform a lung biopsy to confirm the diagnosis. If you have a suspected LAM case, we advocate that you should operate the first time, which is a departure from the typical collapsed lung case.
Further, we recommend leaving an IPC for ongoing drainage at home to keep patients out of the hospital. Even if these patients have an early recurrence, we can utilize the tunneled tube, and avoid the painful process of putting in a new chest tube.
Chylous effusions do not need to be managed traditionally, where patients would be put on total parenteral nutrition and kept in the hospital until the chylothorax resolved. LAM patients can maintain a regular diet and go home with an IPC catheter that can be drained at home. The key in these patients is to initiate medical therapy with sirolimus, which is a form of immunosuppression.
Advice for Diagnosing LAM
My first piece of advice for clinicians who encounter women with cystic lung disease is to know about LAM. If you don't have LAM in your differential diagnosis, you'll never make the diagnosis. Be aware of how it tends to present with either pneumothorax or chylothorax. It is possible for men to have LAM, but it is exceedingly rare.
If you encounter a patient who you suspect may have LAM but are not familiar with it, I encourage you to reach out to rare lung disease program, like the Center for LAM and Rare Lung Diseases at NewYork-Presbyterian and Columbia. They will discuss the diagnostic criteria with any patient or provider and help guide you toward the appropriate treatment path.
