Intermittent exophthalmos.

The syndrome of intermittent exophthalmos classically consists of enophthalmos with the head in the upright position, and exophthalmos which develops rapidly in one eye (usually the left) when venous stasis is induced by bending or lowering the head, turning the head forcibly, hyperextension of the neck, coughing, forced expiration with or without compression of the nostrils, or pressure on the jugular veins. The condition is often referred to as orbital varix, varicocele, venous aneurysms, or arterio-venous aneurysm (Walsh and Dandy, i944; Brauston and Norton, I963). Cases of intermittent exophthalmos have been reported by Birch-Hirschfeld (1930), Wissmann and Schulz (1922), Rumjanzewa (I930), Bartok (I93I), de Petri (I935), Marchesini (I935), Hippert (1936), Muirhead (1936), Kraupa and Mendl (1936), Lipowitsch (1936), Petrov (1939), Spektor (1939), Giqu6aux (1942), Poole (1942), Dunphy (1942), Rones (1942), and Walsh and Dandy (I944). Orbital varices constitute about go per cent. of cases of intermittent proptosis (DukeElder, 1952). Recurrent orbital haemorrhages may also cause intermittent proptosis (Meyer, I898; Teillais, I898; Gayet, I899), and highly vascular orbital neoplasms, such as angioma (von Graefe, I866; Hegner, 19I5; Augstein, 19I7; von Hippel, I9I8), lymphangioma (Wintersteiner, I898; Franklin and Cordes, I924; Wolff, 1932), lymphosarcoma (Perzewa, I922), and retrobulbar venous cavernous haemangioma (Zeidler and Zetterstrom, 1959) are other lesions which may give rise to this condition. Intermittent proptosis accompanied by chemosis may be caused by venous congestion; this is sometimes seen during menstruation (Krauss, I9I0; Morelli, I922; Kragh and Holm, I923), during the strain of childbirth (Rumjanzewa, 1930), or during a paroxysm of crying in infants which leads to spasm of the orbicularis, and may even result in dislocation of the globe (Duke-Elder, I952). The condition may also occur if thrombosis of the cavernous sinus reduces the efficiency of the venous return from the orbit. Other rare causes of intermittent proptosis are periodic orbital oedema (particularly of the angioneurotic type), recurrent emphysema (Duke-Elder, I952), intermittent ethmoiditis (Selkin, 1925; Benoit, 1926; Juler, 1932), and intermittent otitis (Eagleton, I 935).