바로가기메뉴

본문 바로가기 주메뉴 바로가기

Tuberculosis & Respiratory Diseases

Article Contents

Prev Next
e-Submission

Vol.70 No.5
Citation Share

A Case of Pulmonary Lymphangiomatosis

Tuberculosis & Respiratory Diseases / Tuberculosis & Respiratory Diseases,
2011, v.70 no.5, pp.428-432









& (2011). A Case of Pulmonary Lymphangiomatosis. Tuberculosis & Respiratory Diseases, 70(5), 428-432.
  • Downloaded
  • Viewed
PDF Download

Abstract

Pulmonary lymphangiomatosis is a rare disorder involving the entire intrathoracic lymphatic system from the mediastinum to the pleura. Pulmonary lymphangiomatosis mostly occurs in children and young adults without gender predilection. Although it is pathologically benign, it shows a progressive and fatal course with variable initial presentation. We now report a case of pulmonary lymphangiomatosis in a 35-year-old man. He presented with hemoptysis 6 months previously. Chest x-ray and a chest computed tomography scan showed diffuse interstitial thickening with left pleural effusion. Chylothorax was confirmed by thoracentesis. Lymphangiography showed dilated and tortuous lymphatic channels. Surgical lung biopsy revealed proliferation of complex anastomosing lymphatic channels. He was diagnosed with pulmonary lymophangiomatosis. Closed thoracostomy and chemical pleurodesis were done and the dyspnea was reduced.

keywords
Lymphangiomatosis, pulmonary, Chylothorax, Lung diseases, Interstitial, Lymphangiomatosis, pulmonary, Chylothorax, Lung diseases, Interstitial

Reference

1.

1. Faul JL, Berry GJ, Colby TV, Ruoss SJ, Walter MB, Rosen GD, et al. Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med 2000;161: 1037-46.

2.

2. Hilliard RI, McKendry JB, Phillips MJ. Congenital abnormalities of the lymphatic system: a new clinical classification. Pediatrics 1990;86:988-94.

3.

3. Tazelaar HD, Kerr D, Yousem SA, Saldana MJ, Langston C, Colby TV. Diffuse pulmonary lymphangiomatosis. Hum Pathol 1993;24:1313-22.

4.

4. Raman SP, Pipavath SN, Raghu G, Schmidt RA, Godwin JD. Imaging of thoracic lymphatic diseases. AJR Am J Roentgenol 2009;193:1504-13.

5.

5. Swensen SJ, Hartman TE, Mayo JR, Colby TV, Tazelaar HD, Müller NL. Diffuse pulmonary lymphangiomatosis: CT findings. J Comput Assist Tomogr 1995;19:348-52.

6.

6. Yekeler E, Dursun M, Yildirim A, Tunaci M. Diffuse pulmonary lymphangiomatosis: imaging findings. Diagn Interv Radiol 2005;11:31-4.

7.

7. Jang HJ, Lee KS, Han J. Intravascular lymphomatosis of the lung: radiologic findings. J Comput Assist Tomogr 1998;22:427-9.

8.

8. Molitch HI, Unger EC, Witte CL, vanSonnenberg E. Percutaneous sclerotherapy of lymphangiomas. Radiology 1995;194:343-7.

9.

9. Rostom AY. Treatment of thoracic lymphangiomatosis. Arch Dis Child 2000;83:138-9.

10.

10. Reinhardt MA, Nelson SC, Sencer SF, Bostrom BC, Kurachek SC, Nesbit ME. Treatment of childhood lymphangiomas with interferon-alpha. J Pediatr Hematol Oncol 1997;19:232-6.

11.

11. Kandil A, Rostom AY, Mourad WA, Khafaga Y, Gershuny AR, el-Hosseiny G. Successful control of extensive thoracic lymphangiomatosis by irradiation. Clin Oncol (R Coll Radiol) 1997;9:407-11.

12.

12. Valentine VG, Raffin TA. The management of chylothorax. Chest 1992;102:586-91.

13.

13. Calabrese PR, Frank HD, Taubin HL. Lymphangiomyomatosis with chylous ascites: treatment with dietary fat restriction and medium chain triglycerides. Cancer 1977;40:895-7.

14.

14. Kransdorf MJ. Benign soft-tissue tumors in a large referral population: distribution of specific diagnoses by age, sex, and location. AJR Am J Roentgenol 1995; 164:395-402.

15.

15. Cho YS, Yoo JH, Son SY, Cho HL, Kim SJ, Han MS, et al. Pulmonary lymphangiomatosis. Tuberc Respir Dis 1999;47:533-7.

Tuberculosis & Respiratory Diseases

e-Submission OA Policy