RECURRENT NECK SWELLING LEADS TO MULTIPLE SPONTANEOUS CHYLOTHORACES

Abstract

SESSION TITLE: Disorders of the Pleura 1 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 pm - 02:15 pm INTRODUCTION: Spontaneous Chylothorax is a rare clinical entity and often with an obscure presentation. Chylothorax is defined as the presence of chyle in the pleural space. The etiology of this condition is often traumatic however, non-traumatic etiologies including malignancies such as lymphoma and other autoimmune conditions can occur. CASE PRESENTATION: 69 year old female with no significant past medical history presents to the ED with painless swelling on the left side of her neck. While driving back to Scottsdale Arizona from Montana she noticed an acute swelling on the left side of her neck. The patient experienced some generalized chest tightness and shortness of breath with the neck swelling. She denied recent history of fever, chills, weight loss, malaise, rash, or arthralgias. On exam, her vitals, joint, lymphatic and abdominal exams were all normal except for non-tender swelling on the left neck. A neck CT demonstrated either edema, hematoma, or other infiltrating process involving the root of the left neck, the left chest wall and axilla, the mediastinum, and the upper retroperitoneum without abscess, obvious mass, abnormal enhancement, or vascular abnormality. There were small pleural effusions. A thoracentesis was performed and cloudy white fluid was extracted from the left pleural space. Pleural fluid analysis was significant only for the findings of chylomicrons. Further imaging with a nuclear lymphatic scan showed an intact thoracic duct without evidence of lymphatic leak. The patient had a normal CBC, CMP, ESR, CRP, SPEP, lymphocyte panel, fungal screening and cancer markers. Patient’s ANA returned positive with a speckled pattern and at titer of 1:160, however, additional markers for lupus were done and all returned negative. At that time the patient’s symptoms resolved and she was discharged. Patient presented two months later with a second episode. The supraclavicular swelling was now painful and associated with pleuritic chest pain and tightness. Further workup for lymphoma and auto-immune causes included a bone marrow biopsy that did not show any evidence of lymphoma, a negative flow cytometry study, a repeat nuclear lymphoscintigraphy of the head was negative for lymphatic leak. She experienced 14 more episodes of neck swelling over the course of 6-8 months and went to Mayo for a second opinion and a PET scan showed no evidence of malignancy or obstruction. DISCUSSION: Based on testing done to date, no evidence of a lymphoproliferative disorder, autoimmune disease or malignancy has been detected. Chylothorax accounts for approximately 3% of pleural effusions in adults. Primary etiology is malignancy with 75% of cases being lymphoma. Other rare conditions include lymphangiomyomatosis, sarcoidosis, tuberculosis, venous thrombosis, congenital lymphatic malformations and trauma. CONCLUSIONS: With no clear etiology the diagnosis falls into the category of spontaneous chylothorax. Reference #1: Etiology of Chylothorax in 203 PatientsClinton H. Doerr, Mark S. Allen, Francis C. Nichols III, Jay H. RyuMayo Clinic Proceedings, Vol. 80, Issue 7, p867–870, July 2005 Reference #2: Varona Porres D, Persiva O, Pallisa E, Sansano I. Diagnostic imaging in spontaneous rupture of a thoracic duct cyst in the mediastinum. Radiologia. 2016;58(6):491-495 Reference #3: Lyon S, Mott N, Koukounaras J, Shoobridge J, Hudson PV. Role of interventional radiology in the management of chylothorax: A review of the current management of high output chylothorax. Cardiovasc Intervent Radiol. 2013;36(3):599-607 DISCLOSURES: No relevant relationships by Alexis MacDonald, source=Web Response No relevant relationships by Kimberly Parker, source=Web Response