Was your answer D to Fridays CHEST Challenge? You were correct. Read on for complete rationale. A 61-year-old man is referred with a history of several months of exertional dyspnea, abdominal distention, weight loss of approximately 20 lb (9 kg), and generalized malaise and fatigue. Physical examination findings revealed somewhat emaciated upper extremities, painless swelling and heaviness in the legs, and abdominal protrusion with dullness to percussion. Examination of his hands is shown in Figure 71-A. Chest radiographs (Figs 71-B, 71-C) were obtained. A thoracentesis produced 500 mL (0.5 L) of milky fluid that had an elevated albumin level, and flow cytometry revealed immunophenotypically normal T and B lymphocytes with cytology negative for malignancy. Laboratory studies revealed normal CBC count, and creatinine, serum electrolyte, and hepatic transaminase levels. Serum albumin level was 2.9 g/dL (29 g/L). Urinalysis results did not indicate proteinuria. Lower-extremity compression ultrasound results revealed no evidence for thromboembolic disease. Despite initial improvement of dyspnea with the thoracentesis, the effusion reaccumulated several times within 6 weeks. The next step in the investigation of this patient should be: A. Heavy-metal screening using fingernail specimens. B. Pulmonary function testing with methacholine challenge. C. Flexible bronchoscopy with transbronchial biopsies. D. Lymphangiography. RATIONALE: Yellow nail syndrome is a rare condition characterized by primary lymphedema, recurrent pleural effusions, and yellow discoloration of the nails. Approximately 40% of patients have been noted to have bronchiectasis. Although mechanical lymphatic obstruction is assumed to be the underlying pathology, the exact pathophysiologic mechanism is vexing, since it cannot explain the common finding of high albumin concentration commonly found in the pleural space. Increased microvascular permeability has been hypothesized to contribute to the pathogenesis of this syndrome. In patients with recurrent effusions, use of lymphangiography can identify areas of lymphatic channels in the pelvis or extremities that are abnormal and measure lymphatic transit times. In patients for whom attempts at pleurodesis is unsuccessful, if a discrete area of dilation and extravasation of lymphangiographic contrast is identified, surgical intervention using lymphatic or vascular shunts may help minimize recurrence of effusions or ascites (choice D is correct). Images from lymphangiography in this patient are shown from the lower extremity (Figure 71-D) and from lymphatic vessels in the abdomen and pelvis that identify several areas of lymphatic leak (Fig 71-E, arrows). Although fingernail specimens may be used to test a variety of environmental exposures (eg, to heavy metals) or to culture the nails themselves for fungal infections, their use suffers from issues with precision and reliability, and would not be indicated for the patient presented (choice A is incorrect). Pulmonary function testing with a nonspecific airway challenge such as methacholine is useful for assessing airway sensitivity with reversible airflow limitation, but would not play a central role is assessing the exertional dyspnea experienced by this patient (choice B is incorrect). Bronchoscopic evaluation was attempted in 29% of patients reviewed in a retrospective evaluation of individuals diagnosed with yellow nail syndrome, with _ 80% having normal examinations and the remainder noted only to have airway distortion as a result of extrinsic compression from existing pleural effusions. Bronchoscopic lung biopsy specimens were obtained in only one patient; no abnormalities were noted on pathologic study. Therefore, use of bronchoscopy and transbronchial biopsy specimens would not be recommended as a next step without other clinical findings that would suggest a concurrent comorbidity (choice C is incorrect). Arai H, Inui K, Nishii T, et al. A pleuroperitoneal shunt for interactive pleural eff usions with yellow nail syndrome. J Med Cases. 2011;2(3):115-120. Tanaka E, Matsumoto K, Shindo T, Taguchi Y. Implantation of a pleurovenous shunt for massive chylothorax in a patient with yellow nail syndrome. Thorax. 2005;60(3):254-255. Maldonado F, Ryu JH. Yellow nail syndrome. Curr Opin Pulm Med. 2009;15(4):371-375. Maldonado F, Tazelaar HD, Wang CW, Ryu JH. Yellow nail syndrome: analysis of 41 consecutive patients. Chest. 2008;134(2):375-381. D’Alessandro A, Muzi G, Monaco A, Filiberto S, Barboni A, Abbritti G. Yellow nail syndrome: does protein leakage play a role? Eur Respir J. 2001;17(1):149-152.
Posted on: Mon, 04 Aug 2014 13:00:00 +0000
Recently Viewed Topics
© 2015