Ann Thorac Surg 2009;88:1012-1013. doi:10.1016/j.athoracsur.2009.01.053
© 2009 The Society of Thoracic Surgeons
Case Reports
Massive Bilateral Chylothoraces Complicating Mediastinal Granulomatous Disease
Felix G. Fernandez, MD,
Chadrick E. Denlinger, MD,
G. Alexander Patterson, MD,
Daniel Kreisel, MD, PhD,
Alexander S. Krupnick, MD*
Division of Cardiothoracic Surgery, Washington University School of Medicine, St. Louis, Missouri
Accepted for publication January 19, 2009.
* Address correspondence to Dr Krupnick, Barnes-Jewish Hospital South, Queeny Tower, One Barnes-Jewish Hospital Plaza, 3108, St. Louis, MO 63110 (Email: krupnicka{at}wudosis.wustl.edu).
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Abstract
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Mediastinal granulomatous disease with resulting fibrosis is known to cause several serious complications, including pulmonary artery occlusion, bronchial stenosis, superior vena cava syndrome, and constrictive pericarditis. This process is typically the result of an excessive fibrogenic response to the antigen of the fungus Histoplasma capsulatum. We present a case of a 20-year-old man who had massive bilateral chylothoraxes develop as a complication of mediastinal granulomatous disease. Aggressive surgical therapy was necessary to treat this potentially life-threatening condition with an excellent result.
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Introduction
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Mediastinal fibrosis that develops as a result of mediastinal granulomatous disease may cause several serious complications, such as pulmonary arterial occlusion, bronchial stenosis, superior vena cava syndrome, and constrictive pericarditis [1]. An excessive fibrogenic response to the antigen of the fungus Histoplasma capsulatum is believed to be responsible for this condition. Chylothorax is not a common manifestation of mediastinal fibrosis resulting from granulomatous disease. High output chylous fistulas are potentially life-threatening conditions that generally require aggressive surgical intervention.
A previously healthy 20-year-old man presented with upper respiratory symptoms including cough and mild dyspnea. Despite treatment with antibiotics his dyspnea progressively worsened, and lower extremity edema also developed. His condition deteriorated, and he presented to an emergency room where imaging studies revealed a hemodynamically significant pericardial effusion, as well as large bilateral pleural effusions. Notably, a computed tomographic scan of the chest revealed extensive paratracheal and right hilar calcified lymphadenopathy, suggestive of granulomatous disease (Fig 1). An echocardiographic-guided pericardial drain and bilateral chest tubes were placed. The pericardial fluid was serous with negative cultures and cytology. Copious amounts of pleural fluid, milky white in character, with triglyceride levels exceeding 6,000 mg/dL were drained from both pleural spaces. Oral intake was discontinued, and total parenteral nutrition was initiated. A cervical mediastinoscopy was performed to sample the mediastinal lymph nodes, which revealed hyalinizing granulomas with chronic inflammatory changes. Despite conservative management for 1 week, up to 2 L of chyle continued to drain from each pleural space daily, and the patient was transferred to our institution for further management.
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Fig 1. Computed tomographic scan of the chest reveals extensive paratracheal and right hilar calcified lymphadenopathy suggestive of granulomatous infection.
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The patient was promptly taken to the operating room for a right thoracotomy, ligation of the thoracic duct, and a mechanical pleurodesis. Subsequently, the output from the right chest tube decreased to less than 100 mL of serous fluid per day. However, 1 to 2 L of chyle continued to empty daily from the left pleural cavity for the next 3 days. Therefore, the patient returned to the operating room for a left thoracotomy, mechanical pleurodesis, and ligation of all fatty connective tissue between the esophagus and aorta. This resulted in a decrease of the drainage from the left pleural space to less than 200 mL of serous fluid per day. An oral diet was instituted and slowly progressed to solid food. Chest tubes were removed after it was confirmed that their outputs remained low after this dietary challenge. Parenteral nutrition was discontinued and the patient was discharged home. The patient continues to tolerate a regular diet at 3 months follow-up with a chest roentgenogram demonstrating clear lung fields.
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Comment
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Mediastinal granulomatous disease with resulting mediastinal fibrosis has been reported to cause several serious complications, including pulmonary arterial occlusion, bronchial stenosis, superior vena cava syndrome, and constrictive pericarditis [1]. This process is typically the result of an excessive fibrogenic response to the antigen of the fungus Histoplasma capsulatum, which is prevalent along the Mississippi River. However, isolation of the offending organism is rare in the majority of patients. Mediastinal scarring secondary to granulomatous disease has only rarely been associated with large pleural effusions [2]. Although spontaneous chylothoraxes have been described in patients with thoracic duct obstruction secondary to sarcoidosis, Kaposi's sarcoma, and lymphoma, we believe that the development of bilateral high output chylothoraxes resulting from mediastinal granulomatous disease has not been previously reported [3]. Although serology and cultures were not diagnostic, calcified mediastinal granulomas in a patient living in an endemic area make histoplasmosis the most likely cause in our case. Review of the literature revealed only 1 case describing a right-sided chylothorax associated with mediastinal granulomas from histoplasmosis in a 3-year-old child [4]. Unlike the case in the patient presented here, this child was successfully treated with drainage and a low-fat diet.
Initial management of a chylothorax includes pleural drainage, discontinuation of oral intake, and parenteral nutrition. Patients with low-output chyle leaks (< 500 mL/day) may be given a low-fat diet with only medium chain triglycerides. Administration of octreotide may also be beneficial in decreasing the output in some cases. However, high-output chylothoraxes, such as the case presented herein, can rapidly lead to fluid and electrolyte imbalances, as well as lymphocyte depletion, which can result in nutritional and infectious complications. Conservative management of high-output chylothoraxes has been associated with mortality rates as high as 50%. Therefore, aggressive nutritional support and early surgical intervention with ligation of the thoracic duct and pleurodesis to achieve pleural symphysis is the preferred approach for this condition.
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References
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- Garrett Jr HE, Roper CL. Surgical intervention in histoplasmosis Ann Thorac Surg 1986;42:711-722.[Abstract/Free Full Text]
- Carter AB, Hunninghake GW. Massive pleural effusion in diffuse granulomatous disease Chest 1997;112:284-288.[Abstract/Free Full Text]
- Jarman PR, Whyte MK, Sabroe I, Hughes JM. Sarcoidosis presenting with chylothorax Thorax 1995;50:1324-1325.[Abstract/Free Full Text]
- Tutor JD, Schoumacher RA, Chesney PJ. Chylothorax associated with histoplasmosis in a child Pediatr Infect Dis J 2000;19:262-263.[Medline]
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Abstract
Introduction
