Ann Thorac Surg 2002;74:575-578
© 2002 The Society of Thoracic Surgeons
Case report
Chylous reflux into localized pulmonary lymphangiectasis
Françoise Le Pimpec-Barthes, MDa,
Alain Badia, MDa,
Michel Febvre, MDb,
Philippe Legman, MDc,
Marc Riquet, MD, PhD*a
a Service de Chirurgie Thoracique, Hôpital Européen Georges Pompidou, Paris, France
b Service de Pneumologie, Hôpital Saint Antoine, Paris, France
c Service de Radiologie, Hôpital Cochin, Paris, France
Accepted for publication February 2, 2002.
* Address reprint requests to Dr Riquet, Service de Chirurgie Thoracique, Hôpital Européen Georges Pompidou, 20 rue Leblanc, 75015 Paris, France
e-mail: marc.riquet{at}hop.egp.paris.ap-hop-paris.fr
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Abstract
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Spontaneous chylothorax is uncommon and may originate from different etiologies either pleural, pulmonary, or mediastinal. Chyloptysis is a still rarer clinical problem and always of pulmonary origin. We report 2 cases: the first, a 63-year-old woman presenting with a chylothorax, and the second, a 28-year-old man with chyloptysis. Both were successfully treated with a medium chain triglyceride diet. Lymphangiograms demonstrated an identical origin for the 2 cases: reflux from the thoracic duct into right lower lobe lung lymphangiectasis. In our experience, chylous reflux into pulmonary lymphangiectasis is not as rare as believed and many cases probably remain undiagnosed.
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Introduction
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Lymphangiectasis is defined as a congenital or acquired dilation of the lymphatic vessels [1]. Acquired or secondary thoracic lymphangiectasis present as localized or diffuse pulmonary interstitial infiltrates, or cystic lesions on chest radiography [2]. We report 2 cases of localized pulmonary lymphangiectasis complicated by spontaneous chylothorax and chyloptysis.
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Case reports
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Patient 1
A 63-year-old woman, with an insignificant past medical history, was seen 6 months after the onset of a persisting cough. Chest roentgenogram demonstrated left upper lobe infiltrates and a small ipsilateral pleural effusion. Bronchofibroscopy was positive for Klebsiella pneumoniae. The patient was placed on appropriate antibiotic therapy and improved over the following weeks. One month later, she complained of chest pain, dyspnea, and cough. Chest roentgenogram demonstrated right-sided Kerley B lines and a small right-sided pleural effusion. The left hemithorax appeared normal; bronchoscopy was performed and was normal. Pulmonary embolism was ruled out. Six months following this episode, she again complained of dyspnea and persistent cough. Chest roentgenogram demonstrated a right-sided pleural effusion. Thoracocentesis yielded a milky appearing fluid which proved to be a chylothorax (triglycerides 24.92 mMol/l, cholesterol 3.05 mMol/l, and lymphocyte cell count of 90%). Medical work up included thoracic and abdominal computed tomodensitometry, and phlebocavography which were normal. Because the chylothorax reappeared and was important (six thoracocentesis of about 1 liter each), the patient was admitted to the thoracic surgery department. Placement of a thoracostomy tube yielded 2.1 liters of chyle. The patient was immediately placed on a medium chain triglyceride diet. Bilateral pedal lymphography demonstrated opacification of a normal thoracic duct with chylous reflux into the subcarinal nodes and into lymphangiectasis within the right lower lobe (Fig 1).
The chylous effusion progressively decreased and the chest tube was removed 12 days after insertion. The medium chain triglycerides diet was discontinued 3 months later. The patient was recently seen at surgical clinic after 5 years and is doing well.
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Fig 1. (Patient 1) Lymphangiogram: chylous reflux from the thoracic duct into right lower lobe telangiectasis.
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Patient 2
A 28-year-old man, with no significant past medical history, complained of a permanent cough and yellowish expectoration without fever. First symptoms appeared 2 months earlier, following transient chest pain occurring while performing physical activity. Physical examination revealed crepitations in both lung fields. Chest roentgenogram demonstrated localized interstitial infiltrates in the right lung, and computed tomography showed reticular shadowing in the middle and right lower lobes (Fig 2).
Fiberoptic bronchoscopy demonstrated an inflammatory mucosa and brownish secretions issuing from the middle lobar bronchus. Submucosal lymphangiectasis were suspected. Bronchoalveolar lavage revealed an elevated lymphocyte cell count (76%), triglyceride concentration of 4.73 mMol/l, and cholesterol concentration of 1.33 mMol/l. Bipedal lymphography was performed and the thoracic duct was opacified throughout its length. Lymphangiectasies extending into peribronchial lymphatics and into the middle lobe bronchus were also demonstrated (Fig 3).
The patient was placed on a medium chain triglyceride diet and both cough and expectorations disappeared soon thereafter. The medium chain triglyceride diet was discontinued 6 months after its start and the patient is still doing well 3 years later.
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Fig 2. (Patient 2) Tomodensitometry: reticular infiltrates in the middle and right lower lobes due to chylous reflux into the interstitial and peribronchial lymph vessels.
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Fig 3. (Patient 2) Lymphangiogram: chylous reflux from the thoracic duct into middle and right lower lobe lymphangiectasis.
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Comment
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Chyle is the product of lipid digestion [3]. It is drained via the lymphatic vessels (LVS) of the intestines to the roots of the thoracic duct (TD). The chylous lymph is then transported via the TD to the circulation at the level of the left jugular supraclavicular venous junction. The LVS and the TD are valved and continent, not allowing chylous reflux. When the lymphatic system is normal, only injury of the intestinal LVS or of the TD may led to a chylous effusion.
Dilation of the LVS is termed lymphangiectasis. In case of lymphangiectasis, the LVS valves become incontinent. Lymphangiectasis may be congenital (primary) or acquired (secondary). Secondary or acquired lymphangiectasis occur if surgery, radiation, trauma, or other various causes disturb effective lymphatic drainage [2]. Subsequent valve incontinence leads to lymph reflux or lymph stasis until the lymph opens new collateral pathways permitting it to flow further in an attempt to connect into the circulatory system.
Chylous reflux occurs when the TD is not patent (eg, agenesis, tumor, or ligation). When the TD is occluded at its origin, chyle is transported to the neck via opening of parietal or mediastinal collateral lymphatic pathways, their eventual rupture into the pleural cavity leading to chylothorax [4]. When the TD is patent, chylous reflux may occur into incontinent LVS either directly connected with the TD within the mediastinum or indirectly by way of other incontinent LVS.
LVS of the lungs and bronchi may directly drain into the TD in the mediastinum [5]. Reflux at this level explains the lymphography imaging observed in both case reports. In both cases, reflux concerned localized lymphangiectasis and chyle backflow within one lung lobe. In the first case, the disease was diagnosed because of chylothorax (due to a ruptured lung subpleural lymphangiectasis), and in the second case, because of chyloptysis (ruptured submucosal endobronchial lymphangiectasis).
Pulmonary lymphangiectasis complications such as chylothorax and chyloptysis are rare [6]. However, they probably do not account for the frequency of latent intrapulmonary lymphangiectasis that coexists with a patent TD and will never rupture, as was demonstrated by Maier [7]. Chylous reflux due to undiagnosed telangiectasis probably also explains many of the so-called spontaneous idiopathic chylothorax. In fact, Bernier and colleagues [8] reported that the TD was always found to be patent on lymphographies performed in such cases. Thus, in patients with a chylothorax, we recommend lymphography to identify the cause and the location of the lymphatic abnormality [6]. This also applies to chyloptysis. However, the reason why localized lymphangiectasis occur remains difficult to assess, and to our knowledge remains unexplained. In our first case report, the role of a possible previous infectious disease is possible, but uncertain.
Most patients suffering from chylothorax and chyloptysis could recover with conservative medical treatment provided the TD is patent and continent. This is another reason for performing lymphography, the only means of demonstrating its patency. In case of medical treatment failure, TD ligation below the chylous leak is effective. Interruption of the chylous reflux can also be resolved by suturing [4] or by resecting the ectatic and incontinent tributaries provided they are ligated at the level they join the TD.
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References
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- Perelman M.E., Sedova T.N. Chylopericardium, bronchochylorrhea, and chylothorax in pulmonary lymphagiectasis. In: Deslauriers J., Lacquet L.K., eds. . International trends in general thoracic surgery.Thoracic surgery: surgical management of pleural diseases. St. Louis, MO: CV Mosby, 1990:383-391.
- Faul J.L., Berry G.J., Colby T.V., et al. Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med 2000;161:1037-1046.[Free Full Text]
- Olszelwski W.L. Lymph stasis: pathophysiology, diagnosis and treatment. Boca Raton, FL: CRC Press, 1991:633.
- Servelle M., Nogues C. The chyliferous vessels. Paris: Expansion Scientifique Française, 1981:262.
- Riquet M., Hidden G., Debesse B. The lymphatic drainage of the lungs into the TD in the mediastinum. Surg Radiol Anat 1988;10:165-166.[Medline]
- Browse N.L., Allen D.R., Wilson N.M. Management of chylothorax. Br J Surg 1997;84:1711-1716.[Medline]
- Maier H.C. Chylous reflux in the lungs and pleurae. Thorax 1968;23:281-296.[Abstract/Free Full Text]
- Bernier C., Haouzi S., Bouvard E., Massiani M.A., Meyniel D. Spontaneous idiopathic chylothorax. Case report and review of the literature. Rev Pneumol Clin 2000;56:369-373.[Medline]
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Abstract
Introduction
