THE EPONYMS AND VAGARIES OF SARCOIDOSIS

Abstract

Diagnosis: Congenital Bronchogenic Cyst The roentgenogram of the chest (Fig 1) demonstrates a large, oblique, homogeneous shadow with a sharp border in the right lower lobe. No calcifica-tion is seen within the lesion. Thoracotomy revealed a 3x5x2 cm thick-walled cystic mass filled with mucoid material deep within the right lower lobe. It showed no abnormal bronchial communication and had no systemic arterial supply. The mediastinal lymph nodes were not enlarged. Lobectomy was performed. Histologically, the cyst was lined with respiratory epithelial cells, resembling those of bronchial walls. Surrounding the cyst were areas of fibrosis containing cartilage, bone, and nerve tissue. Bronchogenic cysts may be either solitary or multiple. They are thin-walled cavities of varying size and lie within normal pulmonary tissue. Histologi-cally, the cyst wall consists of an epithelial layer of ciliated columnar cells and elements of the bronchial wall. Two types of bronchogenic cyst are known, closed and open.1 The closed cyst has no communication with the bronchial tree, is distended, and contains stagnant fluid. As long as no complication intervenes, these cysts are discovered incidentally. Their rate of growth depends on two factors: the surface dimensions of the included mucosa and the strength of the cyst wall. Atelectasis and infection may develop as a result of compression, and are an indication for surgical intervention.2 An open cyst results from communication with a bronchus and secondary infection of the lumen usually occurs, giving rise to the clinical picture of a chronic abscess. The case under discussion was a closed cyst. Its shape and position suggested intralobar sequestra-tion, a vascular shadow, or benign tumor or cyst. treatment of bronchiogenic cysts of the mediastinum and lung, Hutchinson in 1875 first described this type of skin lesion and called it Mortimer's malady after the name of his patient. The second case of this category was reported by Besnier as lupus pernio in 1889. Ten years later, Boeck referred to the disease as multiple benign sarcoid but renamed it benign miliary lupoid in 1905. Heerfordt is credited with the description (in 1909) of uveoparotid fever as a clinical variety of sarcoidosis. J uengling in 191 1 observed bone changes due to sar-coidosis and classified them as osteitis tuberculosa mul-tiplex cvstica. Schaumann recommended the expressive term benign lymphogranulomatosis for this disease. Of course, no one would call it the