Direct Transorbital Approach for Treatment of Carotid Cavernous Fistula: An Illustrative Case Series.

BACKGROUND AND OBJECTIVES: Endovascular techniques have become the first-line treatment for carotid-cavernous fistulas (CCFs). Direct transorbital venous access may be used if anatomic constraints limit standard transarterial or transvenous access. We describe our institutional experience with the transorbital approach for Barrow Type A-D CCFs. METHODS: Patients with CCFs undergoing transorbital endovascular treatment at our institution between 2017 and 2019 were retrospectively reviewed. Demographic, treatment, and outcome data were collected. RESULTS: Eight patients met inclusion criteria, 4 female and 4 male patients. The mean age was 43 years, with 6 right-sided CCF and 2 left-sided CCFs. Symptoms were present for an average of 1.5 months before treatment. All patients presented with eye pain and subjective visual changes. Seven (87.5%) patients presented with proptosis, 6 (75%) patients had elevated intraocular pressure (IOP), and 3 (37.5%) patients had ophthalmoplegia. Six CCFs (75%) were spontaneous, and 2 CCFs (25%) were traumatic. Barrow types were A (n = 1), B (n = 1), C (n = 1), and D (n = 5). All patients underwent direct percutaneous transorbital embolization with coils followed by Onyx. Three patients had undergone prior transarterial and/or transvenous treatment. A radiographic cure was obtained in all patients after direct transorbital embolization. After CCF cure, cranial nerve palsies resolved in 66.7% of patients, visual acuity in the affected eye was improved or stable in 75% of patients, and IOP had normalized in 85.7% of patients. Proptosis improved in all patients, with complete resolution in 75%. CONCLUSION: Direct transorbital embolization is a safe and potentially curative treatment for all 4 Barrow types of CCFs.

Transfacial venous embolisation of a spontaneous indirect paediatric caroticocavernous fistula.

We present a rare case of a spontaneous indirect caroticocavernous fistula (CCF) in an early adolescent, who exhibited a 1-month history of redness, decrease in vision and proptosis of the right eye without a history of trauma or connective tissue disorder. An MRI of the brain and orbit and a digital subtraction angiography confirmed the diagnosis of Barrow type D CCF. The same was then treated with coiling and a liquid embolising agent using the transfacial venous route, resulting in complete obliteration. Reduction in conjunctival congestion with mild improvement in visual acuity was noted postprocedure. Complete recovery was noted at the 6-month follow-up visit. CCF, though rare in the paediatric population, should be highly considered in the differential diagnosis when the above-mentioned complaints are present. Early diagnosis and intervention have favourable outcomes.

The Possibility of Internal Carotid-Cavernous Fistula After Maxillary Fracture.

The authors first reported a 42-year-old female patient with carotid-cavernous fistula (CCF) that occurred after maxillary fractures. She developed unilateral exophthalmos, bulbar conjunctival hyperemia, and complained of tinnitus after the operation, and was diagnosed as internal CCF through computerized tomography and Digital Substraction Angiography. After the diagnosis, the internal carotid artery was embolized with a coil under general anesthesia. The treatment effect was satisfactory but the vision of the affected side was eventually lost due to the long course of congestion. Although patients with maxillary fractures can safely undergo surgery, some unexpected complications may occur in trauma, such as internal CCF. Therefore, each patient must be subjected to accurate posttraumatic examinations and inquiries to discover possible clinical complications. Patients with exophthalmos, tinnitus, and conjunctival hyperemia may be accompanied by internal CCF. Once diagnosed, appropriate treatment such as embolization should be carried out in time to eliminate further serious sequelae.

"Pulsating proptosis and heavy eye syndrome precipitated by neurofibromatosis type 1: A case report".

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.

Transvenous Onyx Embolization of a Type D Carotid-Cavernous-Fistula: Operative Video.

Carotid-cavernous fistulas (CCFs) are acquired pathologic shunts between the carotid circulation and the cavernous sinus that result in venous congestion.1 They often present with ocular symptoms, such as chemosis, proptosis, and blurry vision. Cranial nerve deficits and increased intraocular pressure are often seen on the neuro-ophthalmologic examination.2 If left untreated, they can lead to cortical venous reflux and intracranial hemorrhage. A cerebral angiogram is the gold standard to diagnose these lesions. The hallmark of dural CCF is opacification of venous structures in the arterial phase of the angiogram. Dependent on carotid branches contributing to the fistula, 4 types are classically defined by Barrow et al.3 When the fistula is indirect (types B-D), the goal of treatment is obliteration via the transvenous route.4 We present the case of a patient who had chemosis and proptosis of the left eye with imaging findings concerning for dural CCF (Video 1). An informed consent was obtained and the patient underwent a cerebral angiogram and treatment of the CCF. In the operative video, we showcase the treatment of a type D CCF using transvenous embolization with Onyx (Covidien, Irvine, CA) and achieve angiographic cure of the fistula. We were able to use Onyx for embolization since the superselective injection did not show cortical venous drainage. This is important as obliteration of cortical veins with liquid embolisate could cause venous infarcts. To our knowledge, this is the first video article that illustrates the endovascular embolization of a CCF and highlights the angiographic findings pre- and post-embolization.

Bilateral Traumatic Carotid-Cavernous Fistula in a Child: Corkscrew Eyelid Vessels as an Indicator of Severe Congestion.

An 11-year-old girl presented with bilateral traumatic caroticocavernous fistula associated with corkscrew eyelid vessels, which were considered indicators of severe congestive disease in this case. Coil embolization was performed; similar to other congestive findings such as proptosis, orbital bruit, increased intraocular pressure, congested scleral and retinal vessels, engorged eyelid vessels resolved immediately after coil embolization. This pediatric case is unique given the caroticocavernous fistula was bilateral and was associated with prominent dilatation of the eyelid vessels, named for the first time as corkscrew eyelid vessels.

Mucormycosis with orbital compartment syndrome in a patient with COVID-19.

During the current pandemic of COVID-19, a myriad of manifestations and complications has emerged and are being reported on. We are discovering patients with COVID-19 are at increased risk of acute cardiac injury, arrythmias, thromboembolic complications (pulmonary embolism and acute stroke), and secondary infection to name a few. I describe a novel case of COVID-19 in a previously healthy 33-year-old female who presented for altered mental status and proptosis. She was ultimately diagnosed with mucormycosis and orbital compartment syndrome, in addition to COVID-19. Early identification of these high morbidity conditions is key to allow for optimal treatment and improved outcomes.

[Cavernous hemangioma of the orbit: diagnosis and management]. / Hémangiome caverneux de l'orbite: diagnostic et prise en charge.

The cavernous hemangioma (or angioma) of the orbit (HCO) is a benign, encapsulated venous malformation and the most common primary lesion of the orbit in adults. It occurs more often in women. These lesions can cause a unilateral proptosis (exophthalmos), optic nerve damage and other signs of orbital pathology, with varying degrees of visual impairment.When an HCO is suspected, ultrasound, scanner or magnetic resonance imaging (MRI) are a valuable aid to its definitive diagnosis. When HCO is symptomatic, multidisciplinary surgical management by a trained specialist in orbital surgery should be considered.This article aims to present the surgical management of HCO. Topographic classifications and surgical approaches are also discussed.

L'hémangiome caverneux (ou angiome) de l'orbite (HCO) est une malformation veineuse encapsulée bénigne et la lésion primaire la plus courante de l'orbite chez l'adulte. Elle survient plus souvent chez les femmes. Ces lésions peuvent provoquer une proptose unilatérale (exophtalmie), des lésions du nerf optique et d'autres signes de pathologie orbitaire, avec une déficience visuelle.Lorsqu'un HCO est suspecté, l'échographie, le scanner ou l'IRM sont une aide précieuse pour son diagnostic définitif. Lorsque l'HCO est symptomatique, une prise en charge chirurgicale multidisciplinaire par un spécialiste de la chirurgie orbitaire doit être envisagée.Cet article vise à présenter la prise en charge chirurgicale du HCO. Les classifications topographiques et les approches chirurgicales sont également discutées.

Autoimmune hyperthyroidism in children & adolescents in Sudan: a 13 years' experience of a Paediatric Endocrinology Clinic.

Background Little has been published about hyperthyroidism in children from Sudan or Africa. In limited resource countries, lack of facilities and sociocultural factors might make international diagnosis and management guidelines difficult to follow. We aimed to determine the magnitude of autoimmune hyperthyroidism, clinical presentation, diagnosis, management and its outcome in Sudan. Method Records of all patients diagnosed as Graves' disease (GD) or Hashitoxicosis (HTx) were reviewed and missing data filled by interviewing patients and/or their families. Data including age, sex, clinical presentation, investigations, management and outcome were obtained. Results Eighty-eight patients, 80 with GD (F:M = 4.7:1) and 8 with HTx (F:M = 7:1), were diagnosed at 11.8 ± 3.05 and 11.23 ± 2.78 years, respectively (p = 0.52). GD patients were diagnosed based on presence of exophthalmos (66.25%), positive thyroid receptor autoantibodies (12.5%), prolonged illness duration (8.75%) or remission failure to only B blocker (16.25%). All GD patients were started on carbimazole and cumulative remission rate was 11.8%, 32.4% and 41.2% by end of the second, third and fourth year respectively, however it plateaus after that. While 12 GD patients underwent surgery, only three opted for radioiodine ablation. Conclusion Hyperthyroidism is not an uncommon problem. In absence of laboratory facilities, differentiation between GD and HTx can be made based on clinical grounds. Continuation of medical treatment for 4 years can increase the remission rate to 41.2%. In Sudan, surgery is the preferred method of definitive therapy.

Association of carotido-cavernous fistula and controlateral optic neuropathy secondary to cranial trauma.

PURPOSE: to report a case of a woman who had a severe cranial trauma complicated by two ophalmologic potentially cecitating complications: right carotido-cavernous fistula and left traumatic optic neuropathy. OBSERVATION: A 56 years-old woman, without any medical history, referred, one month after fall from a height of 3 meters, for right exophthalmos. Ophthalmic examination of the right eye completed by retinal angiography suspected carotid-cavernous fistula which was confirmed by angio-MRI. In the left eye, the visual acuity was decreased to no light perception and fundus examination showed optic nerve head palor secondary to traumatic optic neuropathy. Arterial embolization was performed and allowed closing of the fistula. CONCLUSION: Optic neuropathy and carotido-cavernous fistula are two severe complications that can occur simultaneously in the same patient. The prognosis of the optic neuropathy may be compromised, and the treatment of carotido-cavernous fistula benefited from progress in interventional neuro-radiology.

[Acupoint selection rules of hyperthyroidism and related exophthalmos treated with acupuncture].

To explore the clinical acupoint selection rules for hyperthyroidism and related exophthalmos treated with acupuncture. By taking "hyperthyroidism" "acupuncture and moxibustion" as keywords，literature regarding acupuncture for hyperthyroidism and related exophthalmos published was collected in the Chinese Journal Full-text Database (CNKI), VIP Database (VIP) and WANFANG database. The literature was organized, the database of acupuncture prescription was established and the characteristics and rules of acupoint selection were analyzed. A total of 46 papers were included, involving 89 acupoints, the frequency of acupoint application was 449 times. The most commonly used 6 acupoints for hyperthyroidism treated with acupuncture were Sanyinjiao (SP 6), Neiguan (PC 6), Zusanli (ST 36), Shuitu (ST 10), Hegu (LI 4), Taichong (LR 3). And the most meridians of acupoints were the stomach meridian and the pericardium meridian. The most commonly used 6 acupoints for hyperthyroidism related exophthalmos treated with acupuncture were Fengchi (GB 20), Shangtianzhu (Extra), Hegu (LI 4), Sanyinjiao (SP 6), Cuanzhu (BL 2), Yangbai (GB 14). And the most meridian of acupoints was the gallbladder meridian. The most commonly used specific acupoints for hyperthyroidism treated with acupuncture were crossing points, yuan-source points and five-shu points. The most commonly used specific acupoints for hyperthyroidism related exophthalmos treated with acupuncture were crossing points, yuan-source points and five-shu points. Acupuncture masters in modern times have achieved significant therapeutic effect in the treatment of hyperthyroidism，which has showed the principles of searching for the primary cause of disease in treatment and giving consideration to both the root cause and symptoms. But there is a lack of simple and effective treatment methods that can be rapidly promoted in clinical practice.

Hydatidosis of infratemporal fossa with proptosis - an unusual presentation: a case report and review of the literature.

BACKGROUND: Hydatid disease is one of the common zoonotic diseases caused by the larval stage of Echinococcus granulosus. It is endemic in sheep-raising and cattle-raising areas worldwide and humans are an accidental intermediate host following the ingestion of the larvae. Head and neck involvement of echinococcosis is a rare entity and involvement of the infratemporal region is extremely rare even in endemic areas. Only a few cases of hydatid cysts located in the infratemporal fossa have been reported in the literature. Moreover, extension of the hydatid cyst into the intraorbital region and infiltrating into the surrounding orbital bone is even rarer. CASE PRESENTATION: We present a case of a 65-year-old Gurung Nepalese woman with painless proptosis of her left eyeball of 2 months' duration with recent progressive diminution of vision for 15 days. Radiological findings showed a cystic mass in the left infratemporal fossa extending into the left orbit and involving the surrounding orbital bone. Surgical removal was carried out. On histopathological evaluation, it was reported as hydatid cyst infiltrating into the bone. She was prescribed albendazole and discharged after surgery. However, she was lost to follow up and returned after 15 months with recurrence and proptosis of the same eye. Repeat excision of the lesion was carried out and postoperatively she was administered tablet albendazole. She was found to be disease free after 6 months of follow up. CONCLUSIONS: Clinical and radiological findings are important but may not be sufficient in the preoperative diagnosis of hydatid disease especially if rare sites are involved. Proptosis may be seen in several conditions and orbital or infratemporal hydatidosis, although rare, should be considered a differential diagnosis.

Orbital and nasal meningoencephaloceles secondary to chronic hydrocephalus: A rare cause of bilateral proptosis.

Introduction Orbital meningoencephalocele formation is primarily a result of congenital defects in the pediatric population and trauma of the anterior cranial fossa in adults. We present a unique case of nontraumatic nasal and orbital meningoencephaloceles presenting as bilateral proptosis with exotropia secondary to chronic hydrocephalus. Clinical presentation A 20-year-old male with a history of tuberous sclerosis, X-linked intellectual disability, and epilepsy presented to the emergency department with two days of nausea, emesis, seizures, and two months of progressive proptosis. Initial radiographs of the skull showed a "copper beaten" appearance, indicating chronically elevated intracranial pressure. Computed tomography imaging of the head demonstrated bilateral defects in the cribriform plate and anterior cranial fossa. Magnetic resonance imaging of the brain revealed triventricular hydrocephalus with meningoencephalocele extension into the nasal cavity and frontal horn herniation into the extraconal space of the orbits. The hydrocephalus was managed with ventriculoperitoneal shunt placement with rapid and complete resolution of the proptosis. Conclusion No reports have described bilateral proptosis as the presenting finding of orbital and nasal meningoencephaloceles in the absence of trauma or congenital defect. This case study demonstrates the management of meningoencephalocele formation secondary to chronic hydrocephalus.