| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
Ann Thorac Surg 2001;71:694-696
© 2001 The Society of Thoracic Surgeons
a Section of Cardiothoracic Surgery, Mayo Medical Center, Jacksonville, Jacksonville, Florida, USA
b Department of General Surgery, Mayo Medical Center, Jacksonville, Jacksonville, Florida, USA
Accepted for publication May 1, 2000.
Address reprint requests to Dr Odell, Section of Cardiovascular and Thoracic Surgery, Mayo Medical Center, Jacksonville, 4500 San Pablo Rd, Jacksonville, FL 32224
e-mail: odell.john{at}mayo.edu
 |
Abstract |
|---|
 Top Abstract IntroductionCommentReferences |
|---|
|
Introduction |
|---|
|
TopAbstractIntroductionCommentReferences |
|---|
In August 1996 a healthy 59-year-old white woman experienced sudden severe chest pain. A chest radiograph revealed a mediastinal abnormality (Fig 1). A computed tomographic scan of the chest and upper abdomen demonstrated a large cystic mediastinal mass that evaginated the great vessels in the mediastinum (Fig 2). Multiple lesions of variable density and size were detected in the liver, spleen, and several vertebral bodies (Fig 3). No retroperitoneal or mediastinal lymphadenopathy could be visualized. A metastatic carcinoma was suspected and she was referred for management.
|
|
|
Fine needle aspiration biopsies of a liver lesion did not reveal malignancy. Further observation was advised because of the biopsy findings and the improvement in her symptoms. In June 1997 it was noted that the left neck mass had markedly increased in size, disturbing the patient both cosmetically and functionally; she could not turn her head. She also noted a sensation of tightness in her neck, dyspnea, and coughing, which tended to be worse at night. On physical examination a fluctuant mass (8 x 6 cm) free from underlying tissues was detected in the left supraclavicular region. Surgical excision of the neck and mediastinal cysts were planned because of her symptoms.
The mediastinal lesion was approached by a median sternotomy, and the cervical cyst by a left horizontal supraclavicular incision. The mediastinal cyst was located posterior to the left innominate vein and extended between the superior vena cava and the ascending aorta overlying the trachea. Both pleural spaces were opened to assess the extent of the lesions and to visualize the phrenic nerves. Multiloculated lymphatic cavities were seen in the apical region of the left pleural cavity; these could not be safely excised. During resection of the mediastinal lesion, there was drainage of milky colored fluid from the cystic cavities. Initially the patient could not be weaned from the ventilator. A chest radiograph demonstrated an increasing left pleural opacity and atelectasis. A left lateral thoracotomy was performed 5 days later because of a strong suspicion that the effusion represented loculated chyle and because residual cysts were still present. The pericardium was distended and 400 mL of serous fluid was evacuated; a pericardial window was created. The cystic mass in the left pleural cavity was excised and the thoracic duct was ligated. Pathologic examination of the obtained specimens confirmed the diagnosis of lymphangioma with atypical lymphoid hyperplasia consisting of polyclonal B-lymphocytes and cells with normal T-cell antigen expression. Immunohistochemical staining of factor VIII was positive.
The postoperative course progressed slowly with the ability to extubate and discharge 13 days after the second procedure. A follow-up computed tomographic scan of the chest after 3 months demonstrated almost complete absence of cystic cavities in the chest. Her dyspnea had improved.
|
Comment |
|---|
|
TopAbstractIntroductionCommentReferences |
|---|
When the pathologic process involves multiple organ systems, as in the case described, the term lymphangiomatosis is applied. The origin of this pathologic entity is controversial. Some authors discuss a hamartomatous or neoplastic origin [4]; still others believe that the cysts represent congenital anomalies of lymphojugular sacs in the developing embryo [5]; and in some instances it is associated with malformation of the venous system, (eg, left sided superior vena cava) [6].
The incidence of the syndrome of lymphangiomatosis is not known. It is recognized that these lymphatic abnormalities may affect every organ system, namely the musculoskeletal system (with the exception of the small bones of the hands and feet), the lungs and pleura, pericardium, abdominal viscera, and testes [7]. The only organ system not reported to be involved is the central nervous system, presumably due to its lack of a lymphatic system. In addition, this syndrome shows no predilection based on age, sex, race, or heredity.
Most patients with multiorgan lymphangiomatosis present with an asymptomatic, nonpulsating fluid filled mass, not adherent to underlying tissues. Other modes of presentation, mainly compressive in nature, are dyspnea, easy fatigability, chylopericardium, chylothorax, electrolyte and protein depletion, weight loss, intermittent chest pain, and dysphagia. In rare instances, as the one reported by Daniel and colleagues [8], major veins of the superior mediastinum can be compressed with resultant swelling of an upper extremity or superior vena cava syndrome. Our patient presented with atypical chest pain and progressively worsening dyspnea. Dietz and Stuart [9] described a patient with extensive hygromatous change of the spleen leading to sequestration thrombocytopenia and consumptive coagulopathy.
Different diagnostic modalities can provide clues to the possible diagnosis. Many patients, including ours, present with nonspecific symptoms, and a chest radiograph incidentally reveals a mediastinal mass or widened mediastinum. The density of the lesions on computed tomographic scan may be suspicious for cystic masses. Lymphangiographic studies provide anatomical details of the lymphatic system and distinguish these cysts from other masses. A delayed computed tomographic scan taken 48 hours after injection of a lymphangiographic contrast medium allows the computed tomography to detect accumulation of contrast in cystic cavities in the skeleton and sometimes the viscera that otherwise could be easily overlooked [10]. Ultrasound as a diagnostic modality has not been helpful because characteristic echography features are lacking. In patients with chylothorax, thoracentesis and analysis of the aspirated pleural effusion may arouse suspicion.
There is no effective treatment besides surgical excision that should be done for cosmetic effects or compressive symptoms. Total excision and cure is rare. At surgery the pericardium should be inspected for signs of pericardial effusion. The occurrence of pericardial effusion preoperatively or as a postoperative complication arouses questions about the need of performing prophylactic pericardial window drainage. The most common postoperative complications are chylothorax and recurrence of the disease because of incomplete excision of the lesion. Other complications are injuries to the phrenic nerve, the vagus, the lungs, and the great vessels.
In conclusion, with proper timing and treatment these patients have a reasonable chance of symptomatic relief of their disease.
|
References |
|---|
|
TopAbstractIntroductionCommentReferences |
|---|
This article has been cited by other articles:
|
|
 |
|
  M. G. Benninghoff, W. U. Todd, and R. Bascom Incidental Pleural-Based Pulmonary Lymphangioma J Am Osteopath Assoc, September 1, 2008; 108(9): 525 - 528. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 B. V. Duwe, D. H. Sterman, and A. I. Musani Tumors of the Mediastinum Chest, October 1, 2005; 128(4): 2893 - 2909. [Abstract] [Full Text] [PDF]
|
|
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| ANN THORAC SURG | ASIAN CARDIOVASC THORAC ANN | EUR J CARDIOTHORAC SURG |
| J THORAC CARDIOVASC SURG | ICVTS | ALL CTSNet JOURNALS |
