Diffuse Lung Disease |

Traversing the Milky Ascitic Path to Get to a Diagnosis! FREE TO VIEW

Tanmay Panchabhai, MD; Debabrata Bandyopadhyay, MD; Ruchi Yadav, MD; Valeria Arrossi, MD; Carol Farver, MD; Jihane Faress, MD
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Respiratory Institute, Cleveland Clinic Foundation, Cleveland, OH

Chest. 2014;146(4_MeetingAbstracts):384A. doi:10.1378/chest.1991578
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SESSION TITLE: Interstitial Lung Disease Case Report Posters

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Pulmonary lymphatic diseases typically present with respiratory distress in early infancy. Primary pulmonary lymphatic disorders can pose a diagnostic challenge in adults.

CASE PRESENTATION: We report the case of a 42-year-old female referred to the interstitial lung disease clinic for abnormal chest imaging. A pre-employment chest x-ray 15 years prior was abnormal and she underwent a non-diagnostic bronchoscopy. Because of lack of symptoms, she was followed by yearly chest imaging without further workup until she developed abdominal pain and was found to have recurrent chylous ascites. A chest CT showed extensive septal and peribronchovascular thickening (Figure I) with upper lobe predominant bilateral bronchocentric ground glass opacities with small effusions (Figure II). Few cystic changes were also noted in the upper lobes (Figure II). These imaging findings progressed minimally over the last 2 years. The patient continued to deny breathing problems. Lung exam was significant for decrease breath sounds at the bases. Pulmonary function tests showed reduction of flows with normal diffusion. Connective tissue disease workup was negative. Echocardiography and right heart catheterization ruled out the presence of heart failure, valvular disease and pulmonary hypertension (arterial or venous). A repeat bronchoscopy with transbronchial biopsies failed to give a specific diagnosis. Ultimately a surgical lung biopsy confirmed the diagnosis of lymphangiectasia with the presence of multiple dilated lymphatics. Subsequently a lymphangiogram demonstrated significant opacification of dilated pulmonary lymphatic channels bilaterally, predominantly at the bases consistent with pulmonary lymphangiectasis. The retroperitoneal lymphatics were normal. The patient was placed on a low fat and medium-chain fatty acid enriched diet without further accumulation of chylous ascites.

DISCUSSION: Adult presentations of pulmonary lymphangiectasis has been rarely reported.1,2 Patients generally present for evaluation of interstitial lung disease. Pathologically the dilated lymphatics are positive for CD31 and D2-40 immune markers which help differentiation from other lympho-vascular disorders like LAM and lymphangiomas.

CONCLUSIONS: Our case highlights the fact that isolated pulmonary lymphangiectasia can be seen in adults. It can mimic interstitial lung disease and have a better prognosis than the neonatal forms.

Reference #1: Bolan J, et al. Am J Pathol 2012; 36:1548-54.

Reference #2: Okumura Y, et al. Respiration 2006; 73: 114-116.

DISCLOSURE: The following authors have nothing to disclose: Tanmay Panchabhai, Debabrata Bandyopadhyay, Ruchi Yadav, Valeria Arrossi, Carol Farver, Jihane Faress

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