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A 42-Year-Old Woman With Abnormal Chest CT Scan and Chylous Ascites FREE TO VIEW

Tanmay S. Panchabhai, MD, FCCP; Debabrata Bandyopadhyay, MD; Ruchi Yadav, MD; Andrea V. Arrossi, MD; Atul C. Mehta, MD, FCCP; Jihane A. Faress, MD
Author and Funding Information

CORRESPONDENCE TO: Jihane A. Faress, MD, Department of Pulmonary and Critical Care Medicine, Case Western Reserve University Louis Stokes Cleveland VA Medical Center, 10701 E Blvd, Cleveland, OH 44106


Copyright 2016, . All Rights Reserved.


Chest. 2016;149(1):e25-e28. doi:10.1016/j.chest.2015.10.032
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Published online

A 42-year-old white woman presented to the pulmonary clinic for evaluation of abnormal chest imaging. Twenty years prior to presentation, she was noted to have an abnormal chest radiograph during a routine preemployment evaluation. A subsequent bronchoscopy was nondiagnostic. She was followed up with annual imaging, which demonstrated little or no progression of her disease. She remained symptom free throughout this period. A year before her visit to the pulmonary clinic, she developed abdominal discomfort and was found to have ascites. Subsequently, she underwent three paracenteses with analysis revealing chylous fluid. She was a nonsmoker without a history of exposures or travel.

Figures in this Article

On examination, the patient appeared well nourished, without any distress. She was afebrile (36.7°C) with a heart rate of 72/min, BP of 130/70 mm Hg, and respiratory rate of 18/min. Her oxygen saturation was 100% at room air. There was no peripheral lymphadenopathy. Chest auscultation demonstrated decreased air entry at both lung bases but no adventitious sounds. Her abdomen was soft, nontender, and mildly distended because of ascites but without organomegaly. No clubbing or pedal edema was noted.

The laboratory studies showed normal blood count, basic metabolic panel, and liver enzymes. Her collagen vascular disease workup was negative. A CT scan of the chest (Figs 1, 2) revealed extensive septal and peribronchovascular thickening, upper-lobe predominant bilateral bronchocentric ground-glass opacities, cystic changes in the upper lobes, and small pleural effusions. These imaging findings showed minimal change when compared with studies performed during the 2 previous years. An echocardiogram and right-sided heart catheterization ruled out the presence of any cardiac dysfunction, valvular heart disease, or pulmonary hypertension. Her spirometry suggested restriction but with normal diffusion capacity. A bronchoscopy was performed, and the BAL cell differential was 12% lymphocytes, 71% macrophages, and 12% neutrophils. Transbronchial biopsies were nondiagnostic. Subsequently, the patient underwent surgical lung biopsy with histology as shown in Figures 3 and 4.

Figure 1
Figure Jump LinkFigure 1 CT scan showing bilateral interlobular septal thickening.Grahic Jump Location
Figure 2
Figure Jump LinkFigure 2 A, B, CT scans showing peri-bronchovascular ground-glass opacities and cystic changes.Grahic Jump Location
Figure 3
Figure Jump LinkFigure 3 A, B, Low-power (hematoxylin and eosin, original magnification × 2) (A)) and intermediate power (hematoxylin and eosin, original magnification × 4) (B) Views of the lung biopsy specimen showing dilated lymphatic channels in the thickened pleura (wide arrows) and interlobular septa (thin arrows).Grahic Jump Location
Figure 4
Figure Jump LinkFigure 4 Hematoxylin and eosin, 10 × magnification, showing dilated lymphatics (L) around a bronchovascular bundle with artery (A) and bronchiole (B). The lymphatics are lined by flat endothelial cells (arrows).Grahic Jump Location

What is the diagnosis?

Diagnosis: Pulmonary lymphangiectasia with the presence of multiple dilated lymphatics

Pulmonary lymphangiectasia is a rare disease characterized by the presence of dilated lymphatics that drain the subpleural and interstitial parts of lung. Pulmonary lymphangiectasia can be classified as

  • Primary: This is a developmental abnormality in the lymphatic system caused by failure of the normal regression of the lymphatic channels. This abnormality may be limited to the lungs or may involve the lungs as a part of generalized lymphangiectasia. Lung involvement may be mild in patients with a generalized form, and edema is the predominant feature.

  • Secondary: This occurs as a result of lymphatic or venous outflow obstruction in congenital heart disease, thoracic duct hypoplasia, perinatal infections, or radiation.

Primary pulmonary lymphangiectasia is usually seen in children. It is associated with a high incidence of stillbirth and neonatal mortality; few affected patients survive beyond infancy. In the perinatal period, this condition is associated with hydrops fetalis accompanied by respiratory distress, tachypnea, and cyanosis, ultimately culminating in respiratory failure and the need for mechanical ventilation. The failure of normal regression of the pulmonary lymphatics around 20 weeks’ gestation and failure of these lymphatics to drain lymph from the pulmonary interstitium lead to chylous pleural, pericardial effusions, and ascites, which contribute to high mortality in the perinatal period. Children often develop cough caused by recurrent bronchitis and bronchiectasis from repeated pulmonary infections. Growth delay and gastroesophageal reflux are common in those who survive to adult life. Rare cases presenting in adult life have been reported, most commonly presenting with cough and diffuse pulmonary opacities simulating interstitial lung disease. However, the spectrum varies from asymptomatic lung nodules to hypoxemic respiratory failure. These patients predominantly had isolated involvement of the pulmonary lymphatics. Pulmonary function testing shows a varying combination of obstructive and/or restrictive impairment.

A CT scan typically demonstrates interstitial opacities with reticulation, thickened septal lines, ground-glass opacities, extensive pleural thickening and effusion, and rarely, cysts. In adult patients, the differential diagnoses to consider in those presenting with these radiographic features include left-sided heart failure, lymphangitic carcinomatosis, lymphoma, lymphangiomatosis, pulmonary venoocclusive disorders, Kaposi sarcoma, and lymphocytic interstitial pneumonitis. Definitive diagnosis of lymphangiectasia requires lung biopsy. MRI is another useful tool for detecting dysplasia of the lymphatic vessels in the presence of lymphatic dilatation in lymphangiectasia when a lymphangiogram is not readily available.

The treatment of lymphangiectasia is mostly supportive. Nutritional therapy involving either medium-chain triglycerides or total parental nutrition can be considered when chylous effusions or ascites are present.

Clinical Course

A lymphangiogram was performed, which demonstrated significant opacification of dilated pulmonary lymphatic channels bilaterally, predominantly at the bases and consistent with pulmonary lymphangiectasia. The retroperitoneal lymphatics were normal.

The patient was placed on a low-fat and medium-chain fatty acid-enriched diet and did not have any further accumulation of chylous ascites. Her lung function continues to be stable and she remains asymptomatic from a respiratory standpoint.

  • 1.

    Pulmonary lymphangiectasia usually presents shortly after birth and is associated with high neonatal mortality. Rarely, however, patients can present with respiratory symptoms later in life. A few patients may present with only imaging abnormalities such as in the presented case without respiratory symptoms.

  • 2.

    Diffuse pulmonary lymphangiectasia is a generalized condition, more common in infancy. Localized pulmonary lymphangiectasia is a localized defect of the pulmonary lymphatics. The localized form of pulmonary lymphangiectasia seems more likely to mimic interstitial lung disease.

  • 3.

    Dietary fat restriction is beneficial in the management of lymphangiectasia, especially when associated with chylous effusions or ascites.

  • 4.

    A high index of suspicion should be harbored in patients presenting with imaging abnormalities such as interstitial opacities with reticulation, thickened septal lines, ground-glass opacities, extensive pleural thickening and effusion, and rarely, cysts. When accompanied by chylous effusions (pleural or ascites), this could be a strong indicator of pulmonary lymphangiectasia.

Financial/Nonfinancial disclosures: None declared.

Other contributions:CHEST worked with the authors to ensure that the Journal policies on patient consent to report information were met.


Figures

Figure Jump LinkFigure 1 CT scan showing bilateral interlobular septal thickening.Grahic Jump Location
Figure Jump LinkFigure 2 A, B, CT scans showing peri-bronchovascular ground-glass opacities and cystic changes.Grahic Jump Location
Figure Jump LinkFigure 3 A, B, Low-power (hematoxylin and eosin, original magnification × 2) (A)) and intermediate power (hematoxylin and eosin, original magnification × 4) (B) Views of the lung biopsy specimen showing dilated lymphatic channels in the thickened pleura (wide arrows) and interlobular septa (thin arrows).Grahic Jump Location
Figure Jump LinkFigure 4 Hematoxylin and eosin, 10 × magnification, showing dilated lymphatics (L) around a bronchovascular bundle with artery (A) and bronchiole (B). The lymphatics are lined by flat endothelial cells (arrows).Grahic Jump Location

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