Images in Clinical Medicine
Harlequin's Darker Side
N Engl J Med 2007; 357:e22November 15, 2007
- Article
A 39-year-old healthy, female aerobics instructor presented with a 6-week history of flushing on the left side of her face, neck, and upper trunk after 40 minutes of vigorous exercise, with a strict demarcation at the midline (Panel A). After 30 to 40 minutes of rest, her color returned to normal. Magnetic resonance imaging of the chest revealed a large extrapleural mass in the apex of the right thoracic cavity (Panel B, arrow) that was compressing the sympathetic chain (Panel C, arrow). She underwent thoracotomy and excision of the tumor, which was attached to the second sympathetic ganglion. Histopathological examination confirmed the presence of a neurofibroma. Harlequin's syndrome typically is considered to be an idiopathic, benign condition causing localized failure of the upper thoracic sympathetic chain, with sparing of the first (oculomotor) thoracic segment. Although structural lesions are not usually identified, this case emphasizes that unexpected upper mediastinal neoplasia can be responsible. The patient remained well 1 year after surgery.
Martin E. Duddy, M.R.C.P.
Mark R. Baker, M.R.C.P.
Royal Victoria Infirmary, Newcastle upon Tyne NE1 4LP, United Kingdom
martin.duddy@nuth. nhs. uk - Citing Articles (3)
Citing Articles
1
P. V. Pradeep, Ajith K. Benede, Skandha S. Harshita, B. Jayashree. (2011) Harlequin Syndrome in a Case of Toxic Goitre: A Rare Association. Case Reports in Medicine 2011, 1-4
CrossRef2
N. Gavrilova, S. T. Lindau. (2009) Salivary Sex Hormone Measurement in a National, Population-Based Study of Older Adults. The Journals of Gerontology Series B: Psychological Sciences and Social Sciences 64B:Supplement 1, i94-i105
CrossRef3
J. Vent, G. Quante, E. Markert, J.P. Klussmann, D. Beutner. (2009) Zervikales Neurofibrom als Ursache von Globusgefühl. HNO 57:6, 625-628
CrossRef
