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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 was one of the first intracranial vascular lesions to be recognized. This paper focuses on the historical progression of our understanding of the condition and its symptomatology—from the initial hypothesis of ophthalmic artery aneurysm as the cause of pulsating exophthalmos to the recognition and acceptance of fistulas between the carotid arterial system and cavernous sinus as the true etiology. The authors also discuss the advancements in treatment from Benjamin Travers' early common carotid ligation and wooden compression methods to today's endovascular approaches.

Spontaneous carotid cavernous fistula is usually caused by lesions in the internal carotid cavernous sinus and its branches. We report a young male patient with no history of trauma, in whom the fistula was located at the cavernous sinus of the internal carotid artery. The postoperative angiography found a pseudoaneurysm at the origin of the cavernous sinus of internal carotid artery, which suggested that cavernous sinus fistulas were caused by bleeding from rupture of thin-walled vessels due to the local abnormalities of the vascular wall.

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.

Journal Article; UNLABELLED Carotid-cavernous fistulas are an uncommon diseases characterized by abnormal communications between arteries and veins located in the cavernous sinus. According with Barrow´s classification they could be divided in two groups: direct and indirect. The typical symptoms showed by theses pathologies are: pulsating exophthalmos and orbital blow. The present study describes a case of direct posttraumatic carotid-cavernous fistula in a 26 years old man. Furthermore, we present the images that we used to make the diagnosis. In this light, we decided to treat this case with endovascular approach after considering several therapeutic options. The aim of the present report is twofold. First, we examine the importance of the proper management of the direct posttraumatic carotid-cavernous fistula. Second, we describe this rare syndrome with the goal of proposing suitable treatments. KEY WORDS Carotid cavernous fistulas, pulsating exophthalmos, orbital blow, endovascular approach, Barrow´s classification. Yes

A patient with neurofibromatosis type 1 had pulsating exophthalmos of the right eye with diplopia resulting from severe dysplasia of the sphenoid bone and consecutive herniation of the right temporal lobe. The right orbital tectum was reconstructed with titanium mesh and iliac spongiosa via a lateral orbitotomy using intraoperative navigation. For intraoperative referencing a cortical fixed–reference system and a skin scanning laser device were used. Postoperatively, the diplopia was reduced, but the patient asked for further treatment and the procedure was repeated 6 months later. Intraoperatively, the previously implanted titanium mesh was deformed and most of the transplanted bone was resorbed, probably because of pressure. A more extended mesh was implanted and iliac spongiosa was placed on both sides. Intraoperative navigation was used during both procedures. The adverse effects of diplopia were minimized and follow–up computed tomography after seven months confirmed that the bone graft was in place.

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.

Many cases of disease of the intracranial blood vessels have erroneously been reported as cases of orbital aneurysm or aneurysm of the ophthalmic artery, and several cases have been cited under these titles in the literature with no definite reports to verify the occurrence of the condition. Axenfeld in his textbook1merely mentioned the occurrence of "that extremely infrequent affection" circoid aneurysm of the ophthalmic artery. In de Schweinitz' textbook2it is stated that pulsating exophthalmos was formerly regarded as evidence of true aneurysm of the ophthalmic artery, an assumption probably based on the observation of Guthrie,3who early in the nineteenth century demonstrated at autopsy the presence of an ophthalmic aneurysm which had been associated with pulsating exophthalmos. De Schweinitz and Holloway4in their well known brochure on pulsating exophthalmos summarized 44 cases of this disease which had been reported in the literature

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

THE OCCURRENCE of homolateral pulsating exophthalmos following a carotid-cavernous fistula, either traumatic or spontaneous, is well known. In addition, the same eye often manifests signs of venous congestion, such as dilated veins in the conjunctiva, eyelid, and retina and bulbar chemosis. Not infrequently a similar picture will subsequently develop in the contralateral eye owing to the opening of intercavernous communications. It is not necessary here to go into the anatomical and physiological reasons for this train of events. These, along with the clinical picture and treatment, have already been clearly summarized in the literature.* Cases of carotid-cavernous fistula with absence of classic ocular signs on the homolateral side and the presence of these signs on the contralateral side are rare. Four cases are reported in the literature, and, of these, autopsy was obtained in only one. A fifth case, with autopsy findings, will be presented in this paper. However, I

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

Many cases have been reported in which a pulsating exophthalmos has been dealt with by ligature of the common carotid or successive ligation of both carotids, but as the mortality of this operation is high, it would seem that a method of gradual occlusion of the artery would be preferable. The following report is of a recent operation in which a clamp1 was used to accomplish this occlusion automatically. History and Examination. —A. H., white, a delivery man, aged 39, had no previous disease, except a dysentery while in service in the Philippines. Four years ago last May, during a fight, he was struck a severe blow in the left cheek. He was considerably indisposed after the fight and that night spat blood continuously. On the second day afterward a roaring in his head began which gradually grew worse. At night it was so intense that he could not sleep

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.

Patient. —S. C., a strong, healthy-looking girl, 22 years of age, applied at the eye dispensary of the Jefferson Medical College Hospital July 30, 1903. She complained of pain in the left eye and left side of the head, and of a constant subjective noise. History. —On the evening of July 3 she was struck on the inner angle of the right orbit, between the internal canthus and the nasal bone, by the handle of a heavy butcher knife, thrown from a distance of about 15 feet. Both lids of the right eye were bruised and slightly cut, and the nose bled, but other than these insignificant injuries she suffered no immediate ill effects. On the following day she noticed a noise in the head, which she referred more particularly to the left side. Two weeks later the left eye became prominent. Examination. —At the time of the

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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