Address of Corresponding Author

Respiration 2006;73:114-116 (DOI: 10.1159/000088003)

 Outline

• Key Words
• Abstract
• Introduction
• Case Report
• Discussion
• References

• Author Contacts
• Article Information
• Publication Details
• Drug Dosage / Copyright

  Key Words

• Lymphangiectasis
• Pulmonary lymphatic abnormalities
• Adult pulmonary lymphangiectasis

  Abstract

Pulmonary lymphangiectasis is a rare anomaly in infancy that is characterized by dilatation of pulmonary lymphatic vessels resulting in fetal respiratory distress. Pulmonary lymphangiectasis is considered to occur exclusively in young children and neonates, and very few survive beyond an early age. We herein present an asymptomatic adult case of localized pulmonary lymphangiectasis with multiple nodules. A 27-year-old asymptomatic female presented with multiple nodules on chest computed tomogram images. An exploratory video-assisted thoracoscopy revealed multiple yellowish cysts on the visceral pleura, which were histologically diagnosed as lymphangiectasis. In the present study, the pathogenesis and clinical characteristics are discussed.

Copyright © 2006 S. Karger AG, Basel

 Introduction

Pulmonary lymphangiectasis is a rare and poorly documented disease entity that was first described by Virchow [1] over 100 years ago, which can be classified as primary, secondary or part of a generalized lymphatic abnormality. The primary or congenital form of lymphangiectasis appears more often than the secondary type [2, 3] and is associated with extrapulmonary manifestations due to the lymphatic dilatation of multiple organs [4]. Microscopically, the disease is characterized by abnormal pulmonary lymphatics in the subpleural and interlobar connective tissue such as dilatation of the septal, perivascular and subpleural lymphatic vessels [2]. Pulmonary lymphangiectasis occurs exclusively in young children and neonates, and patients rarely survive beyond infancy due to the dismal respiratory problems. We describe an asymptomatic adult patient who presented multiple nodules on chest computed tomogram (CT) and was diagnosed with pulmonary lymphangiectasis.

 Case Report

A 27-year-old female patient was first admitted to the Toneyama National Hospital for evaluation of multiple pulmonary masses. Her past history was essentially not contributory, and she had been asymptomatic without any respiratory illness episodes. Her mother was healthy, while her father had undergone a hepatic resection for hepatoma and was doing well at that time. A physical examination revealed a healthy young female of moderate stature without cyanosis. On auscultation for breath and heart sounds, there were no rales in the lung field or murmur along the heart border. Chest X-ray images showed a well-circumscribed rounded mass 1 cm to 3 mm in size in the right lower lobe (fig. 1). Chest CT images revealed multiple known masses with a cystic nature (fig. 2) predominantly in the bilateral lower lobes, particularly along the subpleural regions. Laboratory data showed essentially normal findings without elevation of serum tumor markers, and a pulmonary function test revealed no restrictive, obstructive or gas diffusion impairments. A gynecological survey was also performed; however, no abnormal findings were revealed. Under a diagnosis of suspected metastatic lung tumors with an undetermined primary site, exploratory video-assisted thoracic surgery was performed. During surgery, multiple yellowish fluid-containing cysts were found predominantly on the surfaces of the right lower lobes (fig. 3), which were resected thoracoscopically. A lung biopsy specimen taken by wedge resection demonstrated cystic spaces in the subpleural space, however, not in the intralobar septum. The cysts were lined with a thin layer of flattened cells (fig. 4). Based on the macroscopic appearance and histological findings, we reached a final diagnosis of localized limited pulmonary lymphangiectasis. The patient is currently doing well without any respiratory symptoms 2 years after the surgical biopsy.

Fig. 1. Chest X-ray image showing a well-circumscribed round mass 1 cm to 3 mm in size in the right lower lobe.

Fig. 2. Chest CT image showing multiple known masses in the bilateral lower lobes.

Fig. 3. At surgery, multiple yellowish cysts with fluid were found predominantly on the surfaces of the right lower lobes.

Fig. 4. Lung biopsy specimen taken by video-assisted thoracic surgery wedge resection showing cystic spaces in the subpleural layers. The cysts were lined with a thin layer of flattened cells.

 Discussion

Pulmonary lymphatic abnormalities are generally categorized as lymphangioma, lymphangioleiomyomatosis, lymphatic dysplasia or lymphangiectasis, and are thought to result from developmental abnormalities in the lymphatic system, the latter probably resulting from a failure of the pulmonary interstitial connective tissues to regress [5]. Pulmonary lymphangiectasis is histologically characterized by intercommunicating thin-walled, endothelium-lined fluid cysts of variable size [6], as seen in the present case. In addition to its rarity, pulmonary lymphangiectasis can occur sporadically and is associated with syndromes such as Noonan [2] and Down [4]. In asymptomatic adult patients, lymphangiectasis is rarely considered as a differential diagnosis of pulmonary nodules, because of its generally aggressive clinical features. The rarity of lymphangiectasis and the wide variety of its clinical presentation provides a challenge to establishing ideal therapy as well as making an accurate diagnosis.

Usually, the radiographic appearances of the lungs of patients with lymphangiectasis are abnormal and show a generalized congestive pattern of increased pulmonary vascular marking [6] or a reticulonodular pattern due to cystic pulmonary lymphatics in infants [6,7,8] and adults [9]. In some cases, early stage of lymphangiectasis were diagnosed by lymphangiography [7]. In accordance with the anatomical abnormality, a thickening of the interstitium may be seen on chest CT images, and Baltaxe et al. [8] stressed that patchy subpleural and perihilar ground-glass opacity fixed in location may be useful and characteristic findings of this disease. Noonan et al. [2] characterized this disease entity and subclassified it into the following: (1) generalized form of pulmonary lymphangiectasis, (2) secondary to pulmonary venous hypertension or obstruction and (3) primary developmental defect of the lung. The congenital form is associated with a high mortality rate, and few children with the disease survive beyond infancy [8].

In a case of infantile lymphangiectasis with systemic involvement recently encountered [Dr. Hirano, pers. commun.], the patient showed severe respiratory distress and died of respiratory failure 3 days after birth. An autopsy revealed systemic lymphangiectasis without other cardiopulmonary anomalies. Grossly, the lung showed massive cystic changes, which were not due to alveolar spaces but rather lymphatic dilatation. Microscopically, diffuse lymphatic dilatation was seen in both the subpleural and intralobar septum of the lung. Compared to this typical case of infantile pulmonary lymphangiectasis, the cystic lesions of the present patient were scarce and patchy, and histological changes were limited to the subpleural space; however, microscopic features are quite similar to the infantile type of lymphangiectasis. Based on these findings, other congenital lesions including lymphangiomatosis, lymphatic dysplasia, interstitial emphysema and congenital pulmonary cyst were excluded, and systemic survey revealed no involvement in the other organs. White et al. [9] recently reported a more benign case of lymphangiectasis in a 25-year-old male. Dietary modifications, such as low fat intake to reduce lymphatic flow, have been reported to be effective in controlling symptoms in benign adult cases [10]. Collectively, pulmonary lymphangiectasis is pathologically defined regardless of the severity of the lesion. We speculated that the present case should be categorized as type 3, with a presumably congenital origin. When these lesions are limited to the pleural surface, the patient can survive without physical deficit; however, he/she will show typical pathologic features. This report may support the heterogeneity of the pathology as well as the clinical presentation of this disease.

In summary, we described an asymptomatic case of pulmonary lymphangiectasis, which may be considered in adults as a differential diagnosis of pulmonary nodules.

  References

1.

Virchow R: Gesammelte Abhandlungen zur wissenschaftlichen Medizin. Frankfurt, Meidinger, Sohn und Co, 1896.

2.

Noonan JA, Walters LA, Reeves JT: Congenital pulmonary lymphangiectasis. Am J Dis Child 1970;120:314-319.

3.

Wagenaar SS, Swierenga J, Wagenvoort CA: Late presentation of primary pulmonary lymphangiectasis. Thorax 1978;33:791-795.

4.

Bouchard S, Lorenzo MD, Youssef S, Simard P, Lapierre JG: Pulmonary lymphangiectasia revisited. J Pediatr Surg 2000;35:m796-m800.

5.

Faul JL, Berry GJ, Colby TV, Ruoss SJ, Walter MB, Rosen GD, Raffin TA: Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med 2000;161:1037-1046.

6.

Shannon MP, Grantmyre EB, Reid WD, Wotherspoon AS: Congenital pulmonary lymphangiectasis. Report of two cases. Pediatr Radiol 1974;2:235-240.

7.

Chung CJ, Fordham LA, Barker P, Cooper LL: Children with congenital pulmonary lymphangiectasia: After infancy. AJR Am J Roentgenol 1999;173:1583-1588.

8.

Baltaxe HA, Lee JG, Ehlers KH, Engle MA: Pulmonary lymphangiectasia demonstrated by lymphangiography in 2 patients with Noonan syndrome. Radiology 1975;115:149-153.

9.

White JES, Veale D, Fishwick D, Mitchell L, Corris PA: Generalized lymphangiectasia: Pulmonary presentation in an adult. Thorax 1996;51:767-768.

10.

MacLean JE, Cohen E, Weinstein M: Primary intestinal and thoracic lymphangiectasia: A response to antiplasmin therapy. Pediatrics 2002;109:1177-1180.

  Author Contacts

Shin-ichi Takeda, MD
Toneyama National Hospital
Toneyama 5-1-1 Toyonaka
Osaka 560-8552 (Japan)
Tel. +81 6 6853 2001, Fax +81 6 6850 1750, E-Mail stakeda@toneyama.hosp.go.jp

  Article Information

Received: December 17, 2003
Accepted after revision: May 26, 2004
Published online: August 30, 2005
Number of Print Pages : 3
Number of Figures : 4, Number of Tables : 0, Number of References : 10

  Publication Details

Respiration (International Journal of Thoracic Medicine)

Vol. 73, No. 1, Year 2006 (Cover Date: February 2006)

Journal Editor: Bolliger, C.T. (Cape Town)
ISSN: 0025-7931 (print), 1423-0356 (Online)

For additional information: http://www.karger.com/RES

  Drug Dosage / Copyright

Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.