Progressive Dyspnea and Chylothorax in a Young Woman* Marc P. Banner, Maj, USAF, MC
A previously healthy 30-year-old white woman de-
veloped bilateral spontaneous pneumothorax years ago while pregnant. Despite prompt bilateral re-expansion with chest tubes, continuous air leaks prompted sequential bilateral thoracotomies with segmental resection of small blebs and pleural abrasions. A postoperative chest radiograph was interpreted as normal. She remained asymptomatic for three years and then developed progressive exertional dyspnea. Recent episodes of substernal gurgling sounds and production of mucoid sputum prompted admission. The remainder of the history was negative. Positive physical findings were tachypnea, nail bed and mucous membrane cyanosis, and decreased breath sounds at the right lung base. Laboratory studies, including alpha fetoprotein determination and PPD, were negative. Resting arterial blood gases were pH 7.41, Pco2 26 mm Hg, P02 44 mm Hg, and 02 saturation 80 percent. Spirometry disclosed severe obstructive ventilatory impairment with 3~
Deparbnent of Radiology, Wilford Hall United States Air Force Medical Center, Lackland Air Force Base, Texas. The opinions or assertions contained herein are the private views of the author and are not to be construed as official or as reflecting the views of the Deparbnent of the Air Force or the Department of Defense. Reprint requests: Dr. Banner, WU!ord Hall USAF Medical Center, Lackland AFB, Texas 78236 0
CHEST, 71: 6, JUNE, 1977
FlcUBE 1
superimposed restriction of the vital capacity to 44 percent of predicted normal. Diagnostic right thoracocentesis yielded chyle. The current chest radiograph is shown (Fig 1).
ROENTGENOGRAM OF THE MONTH 765
Diagnosis: Pulmonary lymphangiomyomatosis
Posteroanterior chest radiograph (Fig 1) shows diffuse disease, characterized by linear strands and bibasilar honeycombing, a loculated right basal pneumothorax and free fluid in the right pleural space. The left costophrenic angle is blunted. The cardiac silhouette is normal in size and configuration, while the central pulmonary arteries are prominent, suggestive of pulmonary arterial hypertension. Open lung biopsy provided the diagnosis. This unusual disease is characterized pathologically by widespread perilymphatic smooth muscle proliferation throughout the lung. It is regarded as a hamartomatous rather than a neoplastic process. The lungs grossly show diffuse interstitial thickening and multiple cyst-like spaces subpleurally and within the parenchyma. Also found are small mediastinal and retroperitoneal tumors that usually involve the thoracic duct or other large lymphatic channels and show a similar histology. It is postulated that mediastinal lymphatics are involved with and obstructed by a lymphangiomyoma, leading to fistulous communications with the pleural space and chylothorax. This is the 34th reported case of lymphangiomyomatosis. The symptoms and clinical course depend upon the location and extent of the lymphangiomyomatous process and the severity of the pulmonary parenchymal disease. All patients have been women ranging in age from 18 to 70 years; most are in the 30- to 50-year age group. Most patients with pulmonary involvement have dyspnea, the most common cause of which is a chylous pleural effusion. A few also have chylous ascites. No therapeutic measure has proved effective. The most common roentgen finding is chylous
788 MARC P. BANNER
pleural effusion, either unilateral or bilateral. This may occur in the absence of lung involvement or may be associated with interstitial lung disease. Spontaneous pneumothorax is common, presumably related to rupture of a peripheral cyst or bleb formed as a consequence of interstitial involvement by the lymphangiomyomatous tissue. The mediastinal and retroperitoneal masses are relatively small and seldom demonstrated roentgenographically. Lateral ureteral deviation by retroperitoneal tumor has been reported. Lymphangiography, if not contraindicated by severe lung dysfunction, may show lymphatic obstruction at any level, increase in number and caliber of lymphatic channels, and fistulas between abdominal lymphatics and the peritoneal cavity or between the thoracic duct and the pleural space. Pulmonary lymphangiomyomatosis is an uncommon cause of honeycomb lung. More common etiologies include bronchiectasis, histiocytosis X, pneumoconiosis, sarcoidosis, certain collagen vascular diseases (rheumatoid arthritis, scleroderma), and idiopathic interstitial fibrosis. The roentgenographic features of honeycomb lung, pneumothorax, and chylous pleural effusion in a young to middle-aged woman are so characteristic that the correct diagnosis of pulmonary lymphangiomyomatosis can readily be made. REFERENCES
1 Miller WT, Comog JL, Sullivan MA: Lymphangiomyomatosis; clinical-roentgenologic-pathologic syndrome. Am J Roentgenol Rad Ther Nucl Med 111:565-572, 1971 2 Silverstein EF, Ellis K, WolH M, et al: Pulmonary lymphangiomyomatosis. Am J Roentgenol Rad Ther Nucl Med 120:832-850, 1974
CHEST, 71: 6, JUNE, 1977
A previously healthy 30-year-old white woman de-
veloped bilateral spontaneous pneumothorax years ago while pregnant. Despite prompt bilateral re-expansion with chest tubes, continuous air leaks prompted sequential bilateral thoracotomies with segmental resection of small blebs and pleural abrasions. A postoperative chest radiograph was interpreted as normal. She remained asymptomatic for three years and then developed progressive exertional dyspnea. Recent episodes of substernal gurgling sounds and production of mucoid sputum prompted admission. The remainder of the history was negative. Positive physical findings were tachypnea, nail bed and mucous membrane cyanosis, and decreased breath sounds at the right lung base. Laboratory studies, including alpha fetoprotein determination and PPD, were negative. Resting arterial blood gases were pH 7.41, Pco2 26 mm Hg, P02 44 mm Hg, and 02 saturation 80 percent. Spirometry disclosed severe obstructive ventilatory impairment with 3~
Deparbnent of Radiology, Wilford Hall United States Air Force Medical Center, Lackland Air Force Base, Texas. The opinions or assertions contained herein are the private views of the author and are not to be construed as official or as reflecting the views of the Deparbnent of the Air Force or the Department of Defense. Reprint requests: Dr. Banner, WU!ord Hall USAF Medical Center, Lackland AFB, Texas 78236 0
CHEST, 71: 6, JUNE, 1977
FlcUBE 1
superimposed restriction of the vital capacity to 44 percent of predicted normal. Diagnostic right thoracocentesis yielded chyle. The current chest radiograph is shown (Fig 1).
ROENTGENOGRAM OF THE MONTH 765
Diagnosis: Pulmonary lymphangiomyomatosis
Posteroanterior chest radiograph (Fig 1) shows diffuse disease, characterized by linear strands and bibasilar honeycombing, a loculated right basal pneumothorax and free fluid in the right pleural space. The left costophrenic angle is blunted. The cardiac silhouette is normal in size and configuration, while the central pulmonary arteries are prominent, suggestive of pulmonary arterial hypertension. Open lung biopsy provided the diagnosis. This unusual disease is characterized pathologically by widespread perilymphatic smooth muscle proliferation throughout the lung. It is regarded as a hamartomatous rather than a neoplastic process. The lungs grossly show diffuse interstitial thickening and multiple cyst-like spaces subpleurally and within the parenchyma. Also found are small mediastinal and retroperitoneal tumors that usually involve the thoracic duct or other large lymphatic channels and show a similar histology. It is postulated that mediastinal lymphatics are involved with and obstructed by a lymphangiomyoma, leading to fistulous communications with the pleural space and chylothorax. This is the 34th reported case of lymphangiomyomatosis. The symptoms and clinical course depend upon the location and extent of the lymphangiomyomatous process and the severity of the pulmonary parenchymal disease. All patients have been women ranging in age from 18 to 70 years; most are in the 30- to 50-year age group. Most patients with pulmonary involvement have dyspnea, the most common cause of which is a chylous pleural effusion. A few also have chylous ascites. No therapeutic measure has proved effective. The most common roentgen finding is chylous
788 MARC P. BANNER
pleural effusion, either unilateral or bilateral. This may occur in the absence of lung involvement or may be associated with interstitial lung disease. Spontaneous pneumothorax is common, presumably related to rupture of a peripheral cyst or bleb formed as a consequence of interstitial involvement by the lymphangiomyomatous tissue. The mediastinal and retroperitoneal masses are relatively small and seldom demonstrated roentgenographically. Lateral ureteral deviation by retroperitoneal tumor has been reported. Lymphangiography, if not contraindicated by severe lung dysfunction, may show lymphatic obstruction at any level, increase in number and caliber of lymphatic channels, and fistulas between abdominal lymphatics and the peritoneal cavity or between the thoracic duct and the pleural space. Pulmonary lymphangiomyomatosis is an uncommon cause of honeycomb lung. More common etiologies include bronchiectasis, histiocytosis X, pneumoconiosis, sarcoidosis, certain collagen vascular diseases (rheumatoid arthritis, scleroderma), and idiopathic interstitial fibrosis. The roentgenographic features of honeycomb lung, pneumothorax, and chylous pleural effusion in a young to middle-aged woman are so characteristic that the correct diagnosis of pulmonary lymphangiomyomatosis can readily be made. REFERENCES
1 Miller WT, Comog JL, Sullivan MA: Lymphangiomyomatosis; clinical-roentgenologic-pathologic syndrome. Am J Roentgenol Rad Ther Nucl Med 111:565-572, 1971 2 Silverstein EF, Ellis K, WolH M, et al: Pulmonary lymphangiomyomatosis. Am J Roentgenol Rad Ther Nucl Med 120:832-850, 1974
CHEST, 71: 6, JUNE, 1977
