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Pulsatile exophthalmos - Top 30 Publications

Spontaneous Direct Carotid-Cavernous Fistula in an Elderly Patient.

We describe the case of an 83-year-old woman with left-sided ophthalmoplegia. She had no family history of connective tissue disease. The computed tomography study found a dilated left cavernous sinus. The conventional cerebral panangiography confirmed the diagnosis - a direct carotid-cavernous fistula (CCF), with no evidence of ruptured aneurysm. The woman underwent endovascular treatment with coiling of the cavernous sinus in combination with application of the Onyx embolic agent in the fistula. During the first 48 hours after the embolization the local pain, exophthalmos and conjunctival injection of the left eye were significantly ameliorated. The pulsatile tinnitus on the left disappeared and the ptosis of the left eyelid partially recovered. Selective angiography is the best method for the diagnosis and classification of CCF. Currently, treatment is possible with low mortality and morbidity rates. The endovascular intervention is able to completely occlude the fistula and maintain adequate blood fl ow through the carotid artery.

A multidisciplinary approach to sphenoid wing dysplasia presenting with pulsatile proptosis in neurofibromatosis Type 1: A rare case report.

Neurofibromatosis (NF) with sphenoid wing dysplasia is a rare clinical entity. Herewith, we present a case of NF with sphenoid wing dysplasia which presented with pulsatile progressive proptosis. Other ocular symptoms or visual disturbances were absent. Diagnosis of the condition was not easy and the management was a challenging task which needed multidisciplinary approach as there were ocular, neurological, orthopedic, and dermatological manifestations. With neurosurgical intervention, reconstruction of the sphenoid wing was possible. Proptosis was corrected without any disturbance of vision.

Spontaneous bilateral carotid-cavernous fistulas: about a case and review of the literature.

We report a clinical case of spontaneous bilateral carotid-cavernous fistulas. Bilateral pulsatile exophthalmos and symmetric cavernous sinuses detected by CT scan helped to suspect the diagnosis. Arteriography confirmed the diagnosis. The patient underwent embolization with favorable neurologic and ophthalmic evolution. Carotid-cavernous fistula is a rare but severe complication associated with a poor functional (blindness) and vital (meningeal and intracerebral hemorrhage) prognosis. The concomitant use of arteriography and embolization has considerably improved the prognosis.

Altered cerebrospinal fluid dynamics in neurofibromatosis type l: severe arachnoid thickening in patients with neurofibromatosis type 1 may cause abnormal CSF dynamic.

The object of this study is to understand abnormal dynamic of cerebrospinal fluid (CSF) in patients with neurofibromatosis type 1 (NF1), which may cause temporal lobe herniation and bulging of temporal fossa.

Pulsatile exophthalmos revealing spheno-orbital agenesis associated with Von Recklinghausen's disease.

Ophthalmological manifestations of Von Recklinghausen disease are rare. Only a few cases have been reported in the international literature. We here report the case of a patient with pulsatile exophthalmos revealing spheno-orbital agenesis associated with Von Recklinghausen's disease.

Interdisciplinary Management of Minimally Displaced Orbital Roof Fractures: Delayed Pulsatile Exophthalmos and Orbital Encephalocele.

Traumatic orbital roof fractures are rare and are managed nonoperatively in most cases. They are typically associated with severe mechanisms of injury and may be associated with significant neurologic or ophthalmologic compromise including traumatic brain injury and vision loss. Rarely, traumatic encephalocele or pulsatile exophthalmos may be present at the time of injury or develop in delayed fashion, necessitating close observation of these patients. In this article, we describe two patients with minimally displaced blow-in type orbital roof fractures that were later complicated by orbital encephalocele and pulsatile exophthalmos, prompting urgent surgical intervention. We also suggest a management algorithm for adult patients with orbital roof fractures, emphasizing careful observation and interdisciplinary management involving plastic surgery, neurosurgery, and ophthalmology.

Sphenoid Wing Dysplasia with Pulsatile Exophthalmos in Neurofibromatosis Type 1.

Unilateral Direct Carotid Cavernous Fistula Causing Bilateral Ocular Manifestation.

Unilateral carotid cavernous fistula presents with ipsilateral ocular findings. Bilateral presentation is only seen in bilateral fistulas, usually associated with indirect (dural) carotid cavernous fistulas. Direct carotid cavernous fistulas are an abnormal communication between the internal carotid artery and the cavernous sinus. They typically begin with a traumatic disruption in the artery wall into the cavernous sinus, presenting with a classic triad of unilateral pulsatile exophthalmos, cranial bruit and episcleral venous engorgement. We report the case of a 38-year-old male with traumatic right carotid cavernous sinus fistula and bilateral ocular presentation successfully treated by interventional neuroradiology.

Pulsatile enophthalmos, severe esotropia, kinked optic nerve and visual loss in neurofibromatosis type-1.

Neurofibromatosis Type I if associated with aplasia of greater wing of sphenoid may be associated with a pulsatile exophthalmos. However, very rarely it may be associated with a pulsatile enophthalmos. This clinical image describes a rare presentation with pulsatile enophthalmos, esotropia and kinking of the optic nerve due to neurofibomatosis type I.

Video on pulsatile proptosis.

Pulsatile Proptosis.

Surgical care burden in orbito-temporal neurofibromatosis: Multiple procedures and surgical care duration analysis in 47 consecutive adult patients.

Patients with orbito-temporal neurofibromatosis (OTNF) bear a heavy burden of surgical care. We studied 47 consecutive patients with OTNF from the French Neurofibromatosis 1 Referral Center cohort (n > 900), over a 15-year period to determine the clinical features most likely to predict repeat surgery and longer duration of surgical care. Forty-seven patients (5.2% of the NF1 patients' cohort) underwent 79 procedures with a 4.8 years average follow-up. Soft-tissue surgery had a high revision rate (19/45 patients), skeletal surgery did not (2/13 patients). Transosseous wire canthopexy and facial aesthetic unit remodeling were associated with stable outcome. Ptosis repair carried an unfavorable outcome, particularly in the presence of sphenoid dysplasia. Stable skeletal remodeling was achieved with polyethylene implants and/or cementoplasty. Multiple procedures were undertaken in 70% of patients and were predicted by the NF volume, canthopexy, skeletal dysplasia, or a Jackson's classification 2 and/or 3; but not by declining visual acuity. A classification based upon predictive risk of repeated procedures is proposed: Group 1: Isolated soft tissue infiltration not requiring levator palpebrae or canthal surgery; Group 2: Soft tissue involvement requiring ptosis repair or canthopexy, or NF great axis over 4.5 cm; Group 3: Presence of sphenoid dysplasia with pulsatile proptosis, regardless of visual acuity.

Spontaneous Giant Pseudomeningocele in the Middle Cranial Fossa as a Cause of Pulsatile Proptosis.

The authors report a case of a 70-year-old man who presented with a long history of left pulsatile proptosis, irritation, and a pressure sensation behind the eye. Initial computed tomography brain demonstrated the presence of a suspected ethmoid sinus mucocele. Endoscopic sinus surgery was performed to drain the mucocele. On cautious opening of the nasal mucosa, cerebrospinal fluid was encountered requiring subsequent repair with local mucosal flap and fat graft. On further magnetic resonance imaging, the lesion was confirmed to be giant pseudomeningocele arising from the sphenoid sinus, with extensive invasion and erosion of the middle cranial fossa including the left lateral orbital wall. Here, the authors report, to the best of their knowledge, the first case of spontaneous giant pseudomeningocele in the middle cranial fossa causing pulsatile proptosis. In examining this case, the authors will also discuss the pathogenesis, diagnosis, and management of giant pseudomeningoceles, especially in the situation of a misdiagnosis.

Partial thickness autologus calvarial bone orbitocranioplasty for a sphenorbital encephalocele presenting as pulsatile exophthalmos.

Basal encephalocele accounts only 1.5% of all encephaloceles. But Sphenorbital encephalocele is the rarest cause of herniation of brain into orbit leading to pulsatile exphothalmos. Authors presenting a case of sphenorbital encephalocele in a 16 yrs old girl successsfully managed by orbitcranioplasty by partilal thickness autologus calvarial bone graft.

Intraorbital Encephalocele Presenting with Exophthalmos and Orbital Dystopia : CT and MRI Findings.

A 15-year-old female patient with progressive pulsatile exophthalmos caused by intraorbital encephalocele was evaluated with computed tomography (CT) and magnetic resonance imaging (MRI) in our clinic. She had no history of trauma or reconstructive surgery. When she was a little girl, she had undergone surgery for congenital glaucoma on the right eye. On the three-dimensional image of CT, a hypoplasic bone defect was observed in the greater wing of the right sphenoid bone. MRI and CT scan showed herniation through this defect of the arachnoid membrane and protruded cerebral tissue into the right orbita. Intraorbital encephalocele is an important entity that can cause pulsatile exophthalmos and blindness.

Carotid cavernous sinus fistulas without superior ophthalmic vein enlargement.

Diagnosis of carotid cavernous fistula (CCF) relies on clinical findings, such as proptosis, chemosis, and pulsatile tinnitus, plus imaging features including enlargement of the superior ophthalmic vein (SOV). This study reviewed patients with CCF, with a focus on those who were clinically symptomatic but had a normal-appearing SOV on routine scans.

Orbital reconstruction for pulsatile exophthalmos secondary to sphenoid wing dysplasia.

Sphenoid wing dysplasia or absence of the greater sphenoid wing is a rare condition that is considered pathopneumonic for neurofibromatosis type 1 (NF1). It occurs in 4% to 11% of NF1 patients, and its precise cause is unclear. Some cases appear to be congenital, while others have demonstrated it to be a progressive degeneration of the orbital wall. In about half of cases, associated adjacent neurofibromas are described. Consistently, however, the clinical sequelae is herniation of the temporal lobe into the orbit, causing progressive proptosis and pulsatile exophthalmos. Reconstruction of the orbit has traditionally been with bone grafts, but due to problems with bone resorption and recurrence, titanium plates in conjunction with bone grafts have been reported. We present a case of a 6-year-old male patient who was first diagnosed with NF1 and associated absence of the greater sphenoid wing at the age of 2. Four years later, he was referred for reconstruction after the development of pulsatile exophthalmos. Surgical management included dissection of the dura of the temporal lobe off of the periorbita and skull base reconstruction with a combination of radial-shaped titanium mesh and split calvarial bone grafts. Postoperatively, there was near immediate resolution of the pulsatile exophthalmos, and follow-up at 1 year showed no recurrence.

Coexistence of cavernous hemangioma and other vascular malformations of the orbit. A report of three cases.

Coexistence of orbital cavernous hemangioma and other vascular malformations is unusual and few cases have been reported. We describe the clinical and radiological features of three cases of orbital cavernous hemangiomas associated with other vascular malformations, selected reviewing a series of 181 cases of cavernous hemangiomas. All patients were males (age ranging from 43 to 67 years) without vascular systemic disorders and/or a clinical syndrome. They experienced slow progressive exophthalmos. One of them developed acute pulsatile proptosis (case 2), while another experienced slow progressive diplopia (case 3). In one case vascular lesions were bilateral (case 3) and in two patients two different lesions coexisted in the same orbit (cases 1 and 2). All patients underwent surgical excision, which was partial in two cases. Two patients had cavernous hemangiomas in association with a venous malformation (a varix in case 1 and a lymphangioma in case 2), while in the other ones (case 3) cavernous hemangioma was associated with a low-flow arteriovenous malformation. No patient denied visual impairment postoperatively. Few cases of orbital cavernous hemangiomas coexisting with other vascular malformations have been reported in the literature. This entity seems to be an association of different variants of orbital vascular malformations, presenting with a wide spectrum of clinical forms and probably with the same pathogenesis.

Pulsatile exophthalmos: effect of orthostatic pressure difference.

Arachnoid cyst in the middle cranial fossa presenting with pulsatile exophthalmos: case report and literature review.

A 20-year-old woman suffered gradual progression of right pulsatile exophthalmos and slight headache. Computed tomography (CT) demonstrated outward and downward displacement of the right globe and an arachnoid cyst in the right middle cranial fossa associated with thinned and anterior protrusion of a bony orbit. Microscopic cystocisternotomy was performed and the cerebrospinal fluid (CSF) inside of the cyst communicated into the carotid cistern and cistern in the posterior cranial fossa. Pulsatile exophthalmos improved immediately after surgery. Arachnoid cyst in the middle cranial fossa presenting with exophthalmos is rare. Microscopic cystocisternotomy might successfully improve CSF flow and relieve exophthalmos.

Teaching video neuroimages: pulsatile proptosis.

A 44-year-old man with neurofibromatosis type 1 had been aware that his right eye pulsated. His visual acuity was 20/15 in both eyes and his intraocular pressures were normal. He had 4 mm of right exophthalmos and there was pulse-synchronous pulsation of the right eye (video on the Neurology® Web site at No bruit was heard. Lisch nodules were present on both irides. CT showed a large osseous defect of the greater wing of the right sphenoid bone. The differential diagnosis of pulsatile proptosis includes absence of the sphenoid wing in patients with neurofibromatosis 1,(1) carotid-cavernous fistula, orbital roof fractures, and arteriovenous malformations.(2.)

A mysterious post-traumatic pulsatile exophthalmos.

Late intracranial haemorrhage and subsequent carotid-cavernous sinus fistula after fracture of the facial bones.

Carotid-cavernous sinus fistula is an arteriovenous fistula between the internal carotid artery and the cavernous sinus, and is usually caused by a traumatic tear or a ruptured aneurysm of the cavernous segment of the internal carotid artery. We describe a rare case of delayed intracranial haemorrhage and carotid-cavernous sinus fistula that presented 3 weeks after fracture of the facial bones. The patient developed orbital apex syndrome including ptosis of upper eyelid, pulsatile exophthalmos, chemosis, loss of ocular motility, monocular blindness on the right, and numbness of the right infraorbital region. After transcatheter intra-arterial embolisation, the ptosis and chemosis improved.

Orbital schwannoma: clinical, imaging and surgical features.

Schwannoma, or peripheral glioma, is a rare tumor of the orbit (1% of orbital tumors). Its diagnosis remains difficult and can be established only by histological analysis. We discuss through this case report the clinical, radiological and surgical features of this tumor.

Effect of ultrasound radiation force on the choroid.

While visualization of the retina and choroid has made great progress, functional imaging techniques have been lacking. Our aim was to utilize acoustic radiation force impulse (ARFI) response to probe functional properties of these tissues.

Endovascular management of direct carotid-cavernous sinus fistulas.

To evaluate the single-centre experience with endovascular management of direct carotid-cavernous sinus fistulas (DCCF). Between November 2008 and November 2010, a total of 14 patients (11 males) with direct carotid-cavernous sinus fistula underwent 16 transarterial treatment sessions. The patient files and angiograms were analysed retrospectively. Clinical signs and symptoms included exophthalmos [n=12, (85.7%)], pulsatile tinnitus [n=9, (64.3%)], cranial nerve III or VI palsy [n=2, (14.3%)], and impaired vision [n=1, (7.1%)]. Angiography revealed in addition cortical drainage in five patients (35.7%). Transarterial embolization was performed using detachable balloon in nine sessions (56.2%), detachable coils in three sessions (18.8%) and a combination with Onyx in four sessions (25%). Complete interruption of the arteriovenous shunt was achieved in all patients. There was a tendency for ocular pressure-related symptoms to resolve rapidly, while cranial nerve palsy and diplopia improved slowly (7.1%) or did not change (7.1%). The patient with visual impairment recovered within the first two weeks after endovascular treatment. After complete interruption of the arteriovenous shunt, two (12.5%) recurrences were observed in balloon treatment and retreatment was given. Transarterial management is a highly efficient and safe treatment in DCCFs. In the majority of patients a significant and permanent improvement in clinical signs and symptoms can be achieved.

A dural arteriovenous fistula in cavernous sinus developed from viral meningitis.

Although hormonal influences, inflammation, trauma, sinus thrombosis, venous hypertension, and congenital origin have been proposed as sources of dural arteriovenous fistulas (DAVFs) in cavernous and sigmoid sinuses, the etiology of these lesions remains controversial. We present a case with a cavernous sinus DAVF developed from viral meningitis which has not been previously described. A 24-year-old male was admitted to our institute because of periorbital pain, decreased vision, pulsatile tinnitus, chemosis, and exophthalmos on the right side after he had suffered viral meningitis four months before. Cerebral angiography demonstrated a cavernous sinus DAVF, which was successfully obliterated with several platinum coils using a transvenous approach. The viral meningitis most likely caused the inflammation, that may be responsible for the occurrence of the cavernous sinus DAVF. Prompt treatment for inflammation may help to prevent the development of DAVFs.

Intraorbital encephalocele in an adult patient presenting with pulsatile exophthalmos. Case report.

A 25-year-old man presented with an intraorbital encephalocele manifesting as progressive left pulsatile exophthalmos. He had a history of frontal lobe contusion from a motorbike accident 10 years before the onset of the symptom. Computed tomography and magnetic resonance imaging revealed an oval-shaped defect in the left orbital roof with an underlying intracranial cystic lesion, herniated into the orbit. Intraoperative findings included disruption of the dura mater around the bony defect. The loculated arachnoid membrane and protruding brain tissue were excised with primary dural closure and reconstructive cranioplasty with a titanium mesh. The postoperative course was uneventful, and the pulsatile proptosis disappeared immediately after the procedure. Intracranial cyst may be important in the development of progressive pulsatile exophthalmos and intraorbital encephalocele.

Treatment of dural arteriovenous fistulas presenting as pulsatile tinnitus.

To describe the clinical picture and treatment of dural arteriovenous fistulas (DAVFs) presenting as pulsatile subjective tinnitus.

Practical management of proptosis.

The authors report on the management of proptosis. It must first be recognized clinically and confirmed by means of exophthalmometry. The clinical aspects of the proptosis must be described: axial, pulsatile, etc. Radiology and pathology contribute to determining the etiology: Doppler color echography, RX scanner, and MRI can give important details arguing for inflammation (Graves ophthalmopathy or orbital nonspecific inflammation) or orbital tumor. Biopsy or full biopsy in case of suspected tumor provide the diagnosis. The main causes of proptosis are Graves orbitopathy, nonspecific inflammatory orbitopathy, orbital tumor, orbital infection, and orbital injury.