To the Editor: A 26-year-old Yemenite male presented with complaints of chronic shortness of breath, dyspnea, pleural effusions, and recurrent respiratory infections. At age 5, he developed lymphedema and was diagnosed with yellow nail syndrome (YNS). He underwent pericardial window at age 10 for pericardial effusion and myringotomy at age 19 to drain an effusion from the middle ear. He has several cousins with chronic lymphedema. In the 2 years before the current admission, he had undergone right-sided thoracentesis twice for recurrent pleural effusions. Pleural fluid analysis on both occasions revealed a serosanguinous, lymphocyte-predominant exudate. Cultures for bacteria, mycobacteria, and fungi were negative. Cytologic examination was negative for malignant cells. On the current admission, CT scan (Fig. 1) showed a large, loculated right and a moderate left pleural effusion. The patient underwent VATS and the right parietal and visceral pleura were found to be fibrotic with areas of calcification. A large amount of debris and approximately 100 mL of fluid were removed. The parietal pleura, which was 3 to 4 mm thick in some areas, was removed entirely down to the chest wall. The lung remained trapped by a dense visceral pleural peel. This was excised permitting almost complete reexpansion. Pathologic examination of the intrapleural debris showed organizing blood clots, granulation tissue, and fibrohyaline/collagenous tissue. The pleural showed fibrosis and chronic inflammatory changes. Pleural fluid cultures were negative for bacteria, fungi, and acid-fast bacilli. His postoperative course was complicated by an episode of hospital-acquired pneumonia. Exertional dyspnea improved progressively after discharge.FIGURE 1: Preoperative computed tomography scan showing loculated right pleural effusion and marked pleural thickening. Intrapleural air from recent thoracentesis.YNS is a rare lymphatic disorder of unknown etiology. It is characterized by the triad of deformed yellow nails, lymphedema, and respiratory manifestations. These most commonly include pleural effusions, bronchiectasis, sinusitis, and recurrent pneumonia.1 Women are affected twice as often as men.2 The characteristic nail findings are present in 89% of patients2 but are not necessary for diagnosis. The complete triad is seen in only about 30% of cases3 and the manifestations may become apparent over a considerable span of years.2 The pleural effusions are bilateral in 50% of cases. The fluid is most often a yellow exudate with lymphocytic predominance, and normal glucose level.2 The pathophysiology seems to result from hypoplasia or functional blockage of the lymphatic system.4 Previous literature on YNS demonstrates resolution of pleural effusions through thoracentesis,4 pleuroperitoneal shunting,5 pleurovenous shunting,6 and mechanical or talc slurry pleurodesis as a last resort.1 In our patient, extensive parietal and visceral pleural thickening resulted in a trapped lung. To the best of our knowledge, this has been previously reported only once before in YNS. In that case, the patient was managed with open thoracotomy and decortication of the parietal pleura.7 In our patient, parietal and visceral pleural decortication was accomplished exclusively through VATS. The patient’s clinical presentation was also atypical. It is rare for YNS to present in childhood.4 In one of the largest series of patients with YNS reported, the median age at diagnosis was 61 years.1 Our patient presented at the age of 5 with peripheral edema, pericardial effusion, and otitis media. In addition, although familial predisposition has not been reported,3 our patient had an extensive family history of lymphedema suggesting a possible hereditary component in YNS. Videoscopic surgical decortication seems to have benefit in this systemic disease. 13th INTERNATIONAL CONGRESS ON PEDIATRIC PULMONOLOGY The 13th International Congress on Pediatric Pulmonology (CIPP XIII) will take place in Bruges, Belgium, from June 26 through 29, 2014. As the only global congress devoted entirely to pediatric pulmonology, CIPP XIII offers a unique opportunity for state-of-the-art reviews of advances in this field, debates on controversial issues, unique clinical sessions and cutting-edge clinical science. For more information about the congress, please visit the Web site: www.cipp-meeting.org. Jessie Rosenberg, MD* Steven D. Herman, MD† Peter R. Smith, MD† Verena Liu, MD† Joshua Rosenberg, MD‡ *University of Maryland Medical Center, College Park, MD †Divisions of Thoracic Surgery and Pulmonary Medicine, SUNY-Downstate University Hospital of Brooklyn at Long Island College Hospital, and SUNY-Downstate Medical Center, Brooklyn ‡The Brooklyn Hospital Center, New York, NY
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Dec 28, 2017
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