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ARTICLE |

SCHÜLLER-CHRISTIAN DISEASE (XANTHOMATOSIS):  REPORT OF A CASE WITH POSTMORTEM OBSERVATIONS

JOHN M. WHEELER, M.D.
Arch Ophthalmol. 1934;11(1):214-224. doi:10.1001/archopht.1934.00830080222023.
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ABSTRACT

In June, 1930, I brought to the attention of the American Ophthalmological Society the syndrome of "exophthalmos associated with diabetes inspidus and large defects in the bones of the skull," and described the case of a patient who had come under my care. By surgical exploration of the orbit I was able to establish definitely the cause of the exophthalmos by finding xanthomatous nodules in the orbit. It was possible also to explain the impairment of sight by the presence of a firm mass that completely buried the optic nerve. Previously Schüller, Christian and others had assumed that the exophthalmos in their reported cases was caused by large defects in the roof of the orbits. In other words, they thought that the pathologic process had performed decompression, with encroachment of the contents of the cranial cavity on the contents of the orbital cavity. At the time of

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