A man with a Hippocratic syndrome?

Abstract

A 30-year-old man came, in January, 1994, to the Orbital Center, Amsterdam, Netherlands, with a painful swelling of the left lacrimal gland. The swelling had begun 3 months earlier, accompanied by inflammation and ptosis of the upper eyelid. He was treated with amoxycillin for bacterial dacryoadenitis. 12 years earlier, he recalled an episode of similar swelling of both upper eyelids with blurring of his sight, which resolved spontaneously after several weeks. On examination, a well-demarcated lump in the left temporal upper eyelid was noted. Orbital computed tomography showed a mass confined to the left lacrimal gland without bone destruction. Fine-needle aspiration biopsy of the mass showed reactive inflammatory cells and normal lacrimal-gland acinic cells. A transpalpebral anterior orbitotomy was done to debulk the mass. Histological examination showed chronic inflammatory mixed cellular infiltration with fibrosis and lacrimal-gland destruction of non-specific aetiology. These findings were suggestive of lacrimal-gland pseudotumour, for which surgical debulking is the treatment of choice. 1 Mombaerts I Schlingemann RO Goldschmeding R Noorduyn LA Koornneef L The surgical management of lacrimal gland pseudotumors. Ophthalmology. 1996; 103: 1619-1627 Summary Full Text PDF PubMed Scopus (35) Google Scholar The postoperative period was uneventful. However, 1 year later, he developed a similar swelling of his right upper eyelid (figure). Orbital computed tomography confirmed a right lacrimal-gland mass. The lacrimal-gland mass was surgically debulked. Histopathology showed chronic inflammatory mixed-cellular infiltration, and vasculitis of small arteries and veins. Screening for systemic diseases including Wegener's granulomatosis, lupus erythematosus, and sarcoidosis was negative. His symptoms resolved and he was discharged. 7 months later, he suddenly developed oedema and painful paresis of his legs. Abdominal computed tomography with intravenous contrast revealed thrombosis of his inferior vena cava. Laboratory studies for hypercoagulability were negative.