A Case Report of Lymphangioleiomyomatosis Presenting as Spontaneous Pneumothorax

MILITARY MEDICINE, 177, 4:477, 2012 A Case Report of Lymphangioleiomyomatosis Presenting as Spontaneous Pneumothorax Capt Ramon A. Riojas, USAF MC*; Capt Brady A. Bahr, USAF MC*; Capt David B. Thomas, USAF MC*; John Perciballi, MD†; CDR Lachland Noyes, MC USN‡ ABSTRACT Spontaneous pneumothorax is a commonly encountered problem in the Emergency Department. Patients are often treated without further investigation for an underlying etiology. We present a patient who was unable to completely resolve a pneumothorax and was found to have lymphangioleiomyomatosis (LAM), a rare cystic lung disease. In the past, LAM was difficult to diagnose and had a mortality of 100% after 10 years, but now there is a 71% survival after 10 years. Recent research has led to increased characterization of the pathology and radiographic findings. This article briefly presents the case and discusses the etiology, diagnosis, and treatment of LAM. CASE STUDY A 28-year-old African American nonsmoker female presented to the emergency department (ED) with 3 days of increasing chest pain. The pain worsened with lying flat and improved with leaning forward. She had no additional past medical history. She had a family history significant for her father with lupus. Her vitals were within normal limits. The physical exam demonstrated decreased breath sounds on the right side. An electrocardiogram was normal. A chest roentgenogram revealed a pneumothorax on the right side. A tube thoracostomy with a 28 Fr tube was performed in the ED, and the patient was admitted to the hospital. There was an intermittent air leak, and the pneumothorax failed to completely resolve. A second tube thoracostomy was performed with a larger bore tube. The patient continued to have an air leak, and the pneumothorax did not completely resolve. The patient underwent a video-assisted thoracoscopic surgery (VATS) to perform an apical lung resection and mechanical pleural abrasion. Pathological analysis diagnosed lymphangioleiomyomatosis (LAM). A computed tomography (CT) scan of the chest revealed diffuse, thin cysts within the lung parenchyma bilaterally (Fig. 1). This article will briefly discuss the presentation and management of spontaneous pneumothorax. We will also discuss the diagnosis and treatment of LAM. DISCUSSION Patients with primary spontaneous pneumothorax often present with pleuritic chest pain, increasing dyspnea, and decreased breath sounds on the affected side. Our patient presented with chest pain and decreased breath sounds. A small pneumothorax in stable patients can be managed conser*81st Medical Group, 301 Fisher Street, Keesler AFB, MS 39534. †Veterans Affairs Clinic Pensacola, Joint Ambulatory Care Clinic, 790 Veterans Way, Pensacola, FL 32507. ‡Naval Hospital Pensacola, 6000 W Highway 98, Pensacola, FL 32512. The opinions and/or assertions contained herein are solely those of the authors and should not be construed as reflecting those of the U.S. Air Force, Department of Defense, or U.S. Government. vatively with observation or aspiration, but more severe presentations may require tube thoracostomy or surgery.1,2 Oxygen supplementation enhances pneumothorax resolution 3- to 4-fold and is recommended in all patients. The British Thoracic Society defines a small pneumothorax as less than 2 cm between lung and chest wall, whereas the American College of Chest Physicians (ACCP) defines a small pneumothorax as less than 3 cm of apical interpleural distance.3,4 A retrospective study in two community EDs showed that 79% of patients managed conservatively had resolution of pneumothorax.2 The ACCP recommends observing patients for 3 to 6 hours before repeating chest radiograph and discharging.3,4 Our patient had a large pneumothorax, was given supplemental oxygen, and had a chest tube placed in the ED. Spontaneous pneumothorax is more common in young males, smokers, and tall, thin individuals. Patients with a known history of chronic obstructive pulmonary disease or cystic lung disease may also present with spontaneous pneumothorax, also known as secondary spontaneous pneumothorax. If the patient is unstable or has a large pneumothorax, then they require intervention. Tube thoracostomy is recommended in most cases of secondary spontaneous pneumothorax or large pneumothoraces. Chest tubes should be placed to water seal and may be placed to suction at –10 to –20 cm H20. Chest tubes may be removed if there is radiograph evidence of lung expansion and no air leak without suction.3,4 Our patient was admitted to the hospital after having the tube placed to continuous suction. Patients who fail to resolve a primary spontaneous pneumothorax with a thoracostomy after 4 days should be referred for surgery and further evaluated for an underlying etiology with a CT scan of the chest. The ACCP recommends that patients should undergo surgery if they have a persistent air leak or on the second occurrence. Those at risk of another pneumothorax such as scuba divers, pilots, and submariners should be referred for surgery at first presentation. Our patient had a persistent air leak prompting further intervention. VATS is the preferred approach in many cases of spontaneous pneumothorax. A patient with a persistent air leak may MILITARY MEDICINE, Vol. 177, April 2012 477 Case Report FIGURE 1. (A) A CT with intravenous contrast demonstrates a small residual pneumothorax status post right apical pleurectomy and pleurodesis for a persistent air leak. (A) and (B) There are thin-walled, small, round, diffusely scattered cysts in both lungs consistent with LAM. require surgical wedge resection of the apex of the lung, pleural abrasion, or chemical pleurodesis.3,4 Blebs or bullae can be excised with buttressed linear staples. If no bleb or air leak is observed, blind stapling of the pulmonary apex is an acceptable approach. A single institution retrospective review of 182 patients who underwent VATS bleb excision and pleural abrasion for primary spontaneous pneumothorax had a 3% recurrence rate after a mean follow-up of 93 months. Since this was not less than 1%, the authors did not recommend this approach for certain professions such as aviators, scuba divers, or submariners. In these cases, the authors recommended an apical pleurectomy which they report a 0% recurrence.5 Some of the higher reported rates of recurrence after VATS may be as a result of a learning curve of this procedure.6 Our patient underwent a VATS with an apical wedge resection and mechanical pleurodesis to achieve adequate lung expansion and interpleural adhesion. This led to the pathological diagnosis of a rare cystic lung disease, LAM. LAM is a rare cystic lung disease with an annual incidence of 2.6:1,000,000.7 As with our patient, LAM is more common in women of child-bearing age.8 LAM may worsen during pregnancy, and estrogen has been implicated in causing these exacerbations.9–11 Although our patient was a G1P1, she did not report previous episodes of chest p (...truncated)