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Rationale: Neurofibromatosis type 1 (NF1) is a hereditary disease characterized by café-au-lait spots, peripheral neurofibromas, Lisch nodules, optic nerve glioma, and sphenoid wing dysplasia. Pulsating proptosis is associated with a sphenoid bony defect. Heavy eye syndrome is characterized by acquired esohypotropia in patients with high myopia. This study aimed to describe the presentation of pulsating proptosis and heavy eye syndrome precipitated by NF1 and its management. Patient concerns: A 41-year-old woman presented with progressive pulsating proptosis and hypodeviation of the right eye over the past 2 years. The axial length of the right eye was 36.81 mm. The right eye presented with esohypotropia and hypoglobus. The ocular motility examination showed limitations in all directions, especially in supraduction. Brain computed tomography revealed sphenoid wing dysplasia of the right orbit. The meningocele protruded through the orbital defect, lifting the globe. Brain magnetic resonance imaging demonstrated superior rectus muscle (SR) medial displacement and lateral rectus muscle inferior displacement. Physical examination revealed café-au-lait macules and neurofibromas on the trunk. Diagnosis: NF1 with pulsating proptosis and heavy eye syndrome. Interventions: The patient declined neurosurgery due to risk and economic reasons. To manage her main concern regarding cosmetics, we performed orbital floor decompression, SR resection with advancement, maximal hang-back recession of the inferior rectus muscle, and a partial Jensen's procedure. Outcomes: Proptosis was reduced. The eye position became more symmetrical. The range of eye movements was also increased. Lessons: This case describes a rare synchronous presentation of pulsating proptosis and heavy eye syndrome precipitated by NF1. Adult-onset presentation implied a progressive process in NF1. The case also showed a different etiology from that of typical heavy eye syndrome. It reminds ophthalmologists that orbital imaging should be performed in high myopia patients with strabismus to evaluate the extraocular muscle pathway. Furthermore, the case demonstrated a management that avoided the risk and expensive cost of neurosurgery, which has not been reported.

Carotid cavernous fistula (CCF) is an abnormal connection between the carotid artery and the cavernous sinus. Carotid cavernous fistula of traumatic aetiology occurs more commonly. The characteristic triad of symptoms covers the following: pulsating exophthalmos, a humming sound within the skull, and dilation and tortuosity of conjunctival and episcleral veins. The diagnosis of CCF may constitute a diagnostic problem in the situation when the symptoms occur several weeks after injury, it may overlap with other post-traumatic changes, and the dominant symptom may be cranial nerve palsy. The lack of a correct diagnosis and adequate causative therapy creates the risk of not only loss of vision, but also of life. We present a case of a patient who developed fistula symptoms a few weeks after an injury. The diagnosis of post-traumatic CCF was confirmed by imaging examinations. The application of transluminal embolisation led to the resolution of the majority of symptoms.

The term communicating exophthalmos is suggested for a condition where manual retropulsion of one eye results in proptosis of the other eye. This sign was observed in a patient with bilateral pulsating exophthalmos. The pulsating exophthalmos was ascribed to orbital roof defects in combination with post-traumatic hydrocephalus. It was controlled by a shunting procedure.

Twelve days after an automobile accident, bruit developed, and sixty-three days after the accident a diagnosis of pulsating exophthalmos was established. Ligation of the common carotid at the end of ninety days was immediately followed by complete hemiplegia, which subsided within four days. Bruit returned on the 104th day, the ophthalmic vein was ligated on the 133rd day, and resulting cessation of bruit was followed by neuritis with partial atrophy of the opposite arm and foot. These symptoms improved but the optic nerve atrophied.

An orbital growth, recognized at two years, gradually developed and was removed by repeated chemical cauterization. She was seen when 35, during an attack of influenza. The center of the cornea protruded 17 mm. in advance of the other eye, and was pushed down 10 mm. Ligation of the carotid was followed by lessened distention of the vessels. She returned 5 years later with increased exophthalmos. After electrocoagulation the growth was shelled out with a dull separator. Read before the American Ophthalmological Society, June, 1924.

Pulsating exophthalmos may arise from: (1) arteriovenous aneurysms of the brain, orbit or cavernous sinus; (2) arterial and arteriovenous aneurysms of the orbit and (3) defective roof of the orbit. The treatment for pulsating exophthalmos, therefore, is dependent on and directed to the underlying cause. In the case here reported (fig. 1), the pulsating exophthalmos was due to an orbital meningocele of congenital origin. It would perhaps be more correct to speak of congenital absence of the roof of the orbit, for doubtless this defect was primary, the cerebral meningocele following in its wake as a necessary mechanical sequel. The differential diagnosis of the cause of the pulsating exophthalmos was based on the positive roentgenologic evidence that the roof of the orbit was in large part missing on the affected side (figs. 2 and 3). The explanation of the exophthalmos was that the unopposed pressure of the

✓ A case of pulsating unilateral exophthalmos is presented. It was caused by a traumatic aneurysm of the intraorbital ophthalmic artery, secondary to missile injury. Evolution of the aneurysm was observed clinically, by angiography, and by computerized tomography. The differential diagnosis of pulsating exophthalmos and the pathogenesis of such aneurysms are briefly discussed.

Pulsating exophthalmos is not necessarily a consequence of arterio-venous communication in the cavernous sinus and a carotid-cavernous communication does not necessarily produce pulsating exophthalmos. This quotation of WALSH & HOYT (1969) is the subject of today. Of the 4 main causes of pulsating exophthalmos, the defects in the orbital roof and the orbital vascular tumours will be left out and we will confine ourselves today to the carotid-cavernous communications and the external carotid-orbital venous fistula.

Orbitofacial neurofibromatosis may cause severe soft-tissue deformity along with pulsating exophthalmos. The orbit is enlarged, and the greater wing of the sphenoid is absent. The eye in some cases may be functionally useless, immobile, displaced, and blind. In these, orbital exenteration with soft-tissue excision and orbital reconstruction is recommended. External cover is provided by the thin eyelid skin, and a prosthesis ultimately allows a very satisfactory end result. Four such cases with a minimum of 2 years of follow-up are presented.

A case of dural arteriovenous malformation with giant non-traumatic false aneurysm in the left middle fossa is reported. A 10-year-old female patient was admitted to our hospital with pulsating exophthalmos of the left eye, which was successfully treated by ligation of the main feeder and finally by the radical excision of the dural AVM and false aneurysm. False aneurysm of non-traumatic origin is so rare that only two cases were detected in the literature (Reina et al., Sakaki et al.), and no previous case of false aneurysm complicated by dural AVM seems to have been reported.

IN A RECENT REVIEW of hemicranial aplasia associated with pulsating exophthalmos, an unusual manifestation of Von Recklinghausen's disease, it was pointed out that either this syndrome is not recognized or it is quite rare. As of 1960, there were only 54 case reports of this condition in the world literature. The following case was readily diagnosed and constitutes another example of this syndrome. Report of a Case A 9-year-old white female was admitted to Keesler Air Force Base Hospital in July, 1962. The patient was noted at approximately 1 month of age to have regular pulsations of the left eye in time with the pulse. During her life she had been seen by several physicians, and, though the mother made note of this abnormality, no diagnosis was made. Two months prior to admission the patient was struck on the left zygoma with a baseball bat. X-rays revealed "no fracture" and

Defects of the bony orbit are rare in childhood; therefore, when they are encountered in association with clinical signs and symptoms or as an incidental observation, various diagnostic possibilities arise. This consideration is not given to certain specific entities capable of being suspected on clinical grounds. Experiences with two instances of this rare anomaly have prompted us to report the problems presented and their management. The embryology and anatomy of the orbit are available in standard textbooks * and will not be discussed here. However, it is proper to note that both membranous bone and cartilaginous bone enter into the formation of the orbit, and that several natural apertures provide communication with the cranial cavity. Report of Cases Case1.—A white boy was born on April 5, 1944. He was the first child of young healthy parents. The pregnancy and delivery were not unusual; no disease during pregnancy was recorded. No

For a long time the striking clinical pic­ ture resulting from a carotid-cavernous fistula has been recognized as the manifesta­ tion of a communication between the carotid artery and the cavernous sinus. About 75 percent of the occurrences are attributable to known trauma, as was our case, and the remaining 25 percent appear to have a spontaneous origin secondary to a vascular lesion.' In Locke's cases of pulsating exophthalmos, 77 percent were traumatic in origin, and, of these, 94 percent had a carotid-cavernous fistula. Head injuries, gun­ shot wounds, and so forth, are responsible for the traumatic type;· sclerosis of the arterial wall with resultant weakness causes the spontaneous ones.

TWELVE years ago, at the 93rd Annual Session of the American Medical Association, the subject of pulsating exophthalmos was reviewed. 1 That survey encompassed 812 cases. In practically all those cases an arteriovenous shunt was apparently the basic factor responsible for the condition. It would seem that the term "pulsating exophthalmos" automatically implied the association of an arteriovenous communication in or immediately behind the orbit. No mention was made at that time of a small group of cases of congenital unilateral pulsating exophthalmos in which there is no associated arteriovenous shunt and in which the roentgenologic findings are indicative of defective development of the sphenoid wings and of the orbital plate of the frontal bone. A number of such cases had been placed on the record by 1954, and some authors had found a curious relationship to neurofibromatosis, thus adding further to the mystery of that disease. Also, cases of

Pulsating exophthalmos, or retrobulbar arteriovenous aneurysm, a lesion first reported by Benjamin Travers in 1809, is a relatively rare condition meriting report whenever seen. An average of about five cases is reported each year. Harkness1collected six hundred and twenty-one cases from the literature in his paper published in May, 1930. An exhaustive review of the literature is not attempted here, that angle of the problem having been dealt with ably by de Schweinitz and Holloway,2later by Locke3and still later by Harkness. Diagnosis of the lesion usually may be made from the patient's history and appearance alone. The history is fairly typical: After trauma resulting in a period of unconsciousness, the patient has failure of vision or blindness in one eye, unilateral exophthalmos and a rushing, roaring sound in the head synchronous with the pulse. Palpation of the eye commonly discloses a thrill, and auscultation

Unilateral pulsating exophthalmos was first described by Benjamin Travers in 1809. 1 Since then there have been approximately 812 cases reported in the literature. Any retrobulbar mass may produce an exophthalmos, but usually vascular disease is the basis for the pulsating form. The bruit and pulsations of the orbit usually occur from the synchronous movements of the blood through an arteriovenous fistula. These pathologic processes have been verified by many autopsies. An attempt is being made to present evidence justifying the diagnosis of these conditions and to report a series of cases. A complete summary of all additional reports since Locke's review is submitted. Travers's conception of the etiology was incorrect, although it was realized that compression of the common carotid caused a cessation of the bruit and a decrease in the degree of the exophthalmos. In 1812 Dalrymple reported a second case of this nature. Before 1823 it was

History. —The patient was P. V., aged 32, a Hungarian. His past history was unimportant. Eight months ago, while at work in a foundry, the machine at which he was working upset, striking him on the chin and throwing him violently backwards. He was unconscious for twenty-four hours. A compound, comminuted fracture of the lower jaw was produced. There was considerable hemorrhage from the mouth and nose, but none from the ears. His attending surgeon wired the fragments and the patient made a complete recovery. Three months later a slight exophthalmos developed, which grew steadily worse and was very marked when the patient was admitted in the eye service of St. Francis Hospital. Examination. —Nose and accessory sinuses were found normal. The x-ray examination was negative. The ear examination was also negative. Eye: Marked exophthalmos in both eyes, especially the right. A distinct pulsation, rhythmical with the pulse, could be

SURVEY OF LITERATURE ANEURYSM that it is commonly referred to in any discussion of the differential diagnosis of exophthalmos and that a number of cases, so diagnosed, have been reported in the literature. In a survey of the literature we have found reports of 68 cases in which a diagnosis of intraorbital aneurysm was made. Very few of these reports contain information concerning any pathologic investigation, and in even fewer do the recorded facts indicate that a true intraorbital aneurysm was present. In the great majority of instances the diagnosis rested on clinical observations alone. In the early ophthalmologic literature it is evident that the diagnosis of intraorbital aneurysm was usually based (of course, erroneously) on the finding of a pulsating exophthalmos. In 1823 Guthrie 1 made the first diagnosis of orbital aneurysm, basing his diagnosis on autopsy observations. His was the third recorded case of pulsating exophthalmos, being preceded