BRAF V600E is associated with higher incidence of second cancers in adults with Langerhans cell histiocytosis.

In this retrospective study, BRAF mutation status did not correlate with disease extent or (event-free) survival in 156 adults with Langerhans cell histiocytosis. BRAFV600E was associated with an increased incidence of second malignancies, often comprising hematological cancers, which may be clonally related.

Treatment evaluation by volumetric segmentation in pediatric optic pathway glioma: evaluation of the effect of bevacizumab on intra-tumor components.

PURPOSE: Progressive pediatric optic pathway gliomas (OPGs) are treated by diverse systemic antitumor modalities. Refined insights on the course of intra-tumoral components are limited. METHODS: We performed an exploratory study on the longitudinal volumetric course of different (intra-)tumor components by manual segmentation of MRI at the start and after 3, 6 and 12 months of bevacizumab (BVZ) treatment. RESULTS: Thirty-one patients were treated with BVZ (median 12 months, range: 2-39 months). During treatment the total tumor volume decreased with median 19.9% (range: - 62.3 to + 29.7%; n = 30) within the first 3 months, decreased 19.0% (range: - 68.8 to + 96.1%; n = 28) between start and 6 months and 27.2% (range: -73.4 to + 36.0%; n = 21) between start and 12 months. Intra-tumoral cysts were present in 12 OPGs, all showed a decrease of volume during treatment. The relative contrast enhanced volume of NF1 associated OPG (n = 11) showed an significant reduction compared to OPG with a KIAA1549-BRAF fusion (p < 0.01). Three OPGs progressed during treatment, but were not preceded by an increase of relative contrast enhancement. CONCLUSION: Treatment with BVZ of progressive pediatric OPGs leads to a decrease of both total tumor volume and cystic volume for the majority of OPGs with emphasis on the first three months. NF1 and KIAA1549-BRAF fusion related OPGs showed a different (early) treatment effect regarding the tumor enhancing component on MRI, which did not correlate with tumor volume changes. Future research is necessary to further evaluate these findings and its relevance to clinical outcome parameters.

Determining Asymmetry Thresholds in Anophthalmia/Microphthalmia Using a Three-dimensional Animated Model.

PURPOSE: Congenital microphthalmia and anophthalmia are rare development disorders with underdevelopment of the orbital region, resulting in asymmetry of the face. No clear guidelines exist to determine when these deviations are acceptable. METHODS: The face of a healthy 6-year-old child was three-dimensionally scanned. On this scan, we modeled various incremental degrees of facial asymmetries using three-dimensional modeling software. We modeled for smaller palpebral fissures, sunken eyes, and downward displacement of the eye. We also tested whether adjusting the vertical palpebral fissure height in relation to the horizontal palpebral fissure width affected perception. A total of 22 videos were created in which the model turned the head horizontally and vertically. We created a questionnaire asking raters how acceptable the face is, on a linear scale from 0 to 10. RESULTS: Results showed a correlation between the degree of asymmetry and the acceptability score of the raters. Enophthalmos of ≥6 mm, palpebral fissure width ≤79% compared with the other eye, and 2 mm or more downward displacement of the eye resulted in a significant different acceptability score. The desire for correction was strongly increased when these thresholds were exceeded. Adjusting the vertical palpebral fissure height to the horizontal palpebral fissure width resulted in a worse acceptability score. CONCLUSION: A unilateral sunken eye (enophthalmos) of 6 mm or more, asymmetric horizontal palpebral fissure length of ≤79%, and a lower position of one eye of more than 2 mm resulted in unacceptable judgment. These data can be used to evaluate treatment outcome in children treated for congenital microphthalmia and anophthalmia.

Dysthyroid Optic Neuropathy.

PURPOSE: Dysthyroid optic neuropathy (DON) is a sight-threatening complication of thyroid eye disease (TED). This review provides an overview of the epidemiology, pathogenesis, diagnosis, and current therapeutic options for DON. METHODS: A literature review. RESULTS: DON occurs in about 5% to 8% of TED patients. Compression of the optic nerve at the apex is the most widely accepted pathogenic mechanism. Excessive stretching of the nerve might play a role in a minority of cases. Increasing age, male gender, smoking, and diabetes mellitus have been identified as risk factors. Diagnosis of DON is based on a combination of ≥2 clinical findings, including decreased visual acuity, decreased color vision, relative afferent pupillary defect, visual field defects, or optic disc edema. Orbital imaging supports the diagnosis by confirming apical crowding or optic nerve stretching. DON should be promptly treated with high-dose intravenous glucocorticoids. Decompression surgery should be performed, but the response is incomplete. Radiotherapy might play a role in the prevention of DON development and may delay or avoid the need for surgery. The advent of new biologic-targeted agents provides an exciting new array of therapeutic options, though more research is needed to clarify the role of these medications in the management of DON. CONCLUSIONS: Even with appropriate management, DON can result in irreversible loss of visual function. Prompt diagnosis and management are pivotal and require a multidisciplinary approach. Methylprednisolone infusions still represent first-line therapy, and surgical decompression is performed in cases of treatment failure. Biologics may play a role in the future.

Perioperative Prophylactic Antibiotics in 1,250 Orbital Surgeries.

PURPOSE: Intravenous antibiotic prophylaxis is used for many clean-contaminated surgeries or clean surgeries with an implant, but its value for clean orbital surgery has not been determined. This study investigated infection risks and adverse effects related to antibiotics in patients undergoing orbital surgery. METHODS: A prospective, nonrandomized comparative case series of all patients undergoing orbital surgery with participating surgeons between October 1, 2013, and March 1, 2015. Types of surgery, antibiotic regimens, corticosteroid use, antibiotic side effects, and surgical site infections (SSIs) were entered into an electronic database and subsequently analyzed. Cases in which patients received postoperative oral antibiotics were analyzed separately. RESULTS: Of 1,250 consecutive orbital surgeries, 1,225 met inclusion criteria. A total of 1208 patients were included in the primary analysis: 603 received no antibiotic prophylaxis (group A), and 605 received a single dose of intravenous antibiotic (group B). Five patients (0.42%) developed an SSI, 3 in group A and 2 in group B. The difference in SSI rates was not statistically significant between the 2 groups (p = 0.66). Antibiotic prophylaxis, alloplastic implants, paranasal sinus entry, and corticosteroid use were not associated with differences in SSI rates. All SSIs resolved on a single course of oral antibiotics; an implant was removed in 1 case. There were no complications associated with a single dose of intravenous prophylaxis. However, 12% of 17 patients (group C) who received 1 week of oral postoperative prophylactic antibiotics developed antibiotic-related complications (diarrhea, renal injury), yielding a number needed to harm of 8.5. CONCLUSIONS: In this large series, antibiotic prophylaxis does not appear to have reduced the already low incidence of SSI following orbital surgery. Given the detriments of systemic antibiotics, the rarity of infections related to orbital surgery, and the efficacy of treating such infections should they occur, patients undergoing orbital surgery should be educated to the early symptoms of postoperative infection and followed closely, but do not routinely require perioperative antibiotics.

Psychosocial well-being of long-term survivors of pediatric head-neck rhabdomyosarcoma.

BACKGROUND: Head and neck rhabdomyosarcoma (HNRMS) survivors are at risk to develop adverse events (AEs). The impact of these AEs on psychosocial well-being is unclear. We aimed to assess psychosocial well-being of HNRMS survivors and examine whether psychosocial outcomes were associated with burden of therapy. PROCEDURE: Sixty-five HNRMS survivors (median follow-up: 11.5 years), treated in the Netherlands and the United Kingdom between 1990 and 2010 and alive ≥2 years after treatment visited the outpatient multidisciplinary follow-up clinic once, in which AEs were scored based on a predefined list according to the Common Terminology Criteria for Adverse Events. Survivors were asked to complete questionnaires on health-related quality of life (HRQoL; PedsQL and YQOL-FD), self-perception (KIDSCREEN), and satisfaction with appearances (SWA). HRQoL and self-perception scores were compared with reference values, and the correlation between physician-assessed AEs and psychosocial well-being was assessed. RESULTS: HNRMS survivors showed significantly lower scores on PedsQL school/work domain (P ≤ 0.01, P = 0.02, respectively), YQOL-FD domains negative self-image and positive consequences (P ≤ 0.01, P = 0.04, respectively) compared with norm data; scores on negative consequences domain were significantly higher (P = 0.03). Over 50% of survivors negatively rated their appearances on three or more items. Burden of AEs was not associated with generic HRQoL and self-perception scores, but was associated with disease-specific QoL (YQOL-FD). CONCLUSION: In general, HRQoL in HNRMS survivors was comparable to reference groups; however, survivors did report disease-specific consequences. We therefore recommend including specific questionnaires related to difficulties with facial appearance in a systematic monitoring program to determine the necessity for tailored care.

Dysthyroid Optic Neuropathy.

PURPOSE: Dysthyroid optic neuropathy (DON) is a serious complication of Graves orbitopathy that can result in irreversible and profound visual loss. Controversy exists regarding the pathogenesis and management of the disease. The authors provide an overview of the current understanding of DON and present a therapeutic guideline. METHODS: A review of the literature. RESULTS: The mechanism of DON appears to be multifactorial: direct compression of the optic nerve by enlarged extraocular muscles, stretching of the optic nerve by proptosis, orbital pressure, vascular insufficiency, and inflammation. Some or all of these factors may be involved in an individual patient. There has only been one controlled trial comparing high-dose intravenous methylprednisolone to bony orbital decompression for DON. Both 2-wall and 3-wall decompression techniques successfully improve visual functions of patients with DON. There are few case reports/case series that suggest biologic agents may improve visual function in DON. CONCLUSIONS: DON is a serious complication of Graves orbitopathy, the diagnosis and management of which is complex and requires a multidisciplinary approach. There is little evidence regarding the optimum management strategy. Based on the current literature, the first line of treatment is intravenous methylprednisolone, with the exact timing and indication of bony orbital decompression still to be determined. In addition, there may be a role for the use of biologic agents that will require a systematic program to determine efficacy.

Surgical Treatment of Diplopia in Graves Orbitopathy Patients.

PURPOSE: To review the authors' current understanding of motility dysfunction in patients with thyroid eye disease and to summarize appropriate evaluation and available management options. METHODS: Data were retrieved from a systematic search of the literature. RESULTS: Diplopia is one of the most disabling symptoms for Graves orbitopathy patients. It occurs in moderate-to-severe Graves orbitopathy stages and does not respond well to available anti-inflammatory treatment. Inflammation of extraocular muscles induces relatively rapid fibrotic and degenerative changes, which lead to scarring and loss of elasticity with preserved contractility. Inferior and medial rectus muscles are most often involved, which results in horizontal or vertical misalignment or both, and sometimes cyclotorsion. Impaired motility can also be caused by entrapment after orbital decompression. The aim of surgical correction is to restore/enlarge the field of binocular single vision and alleviate abnormal head posture. The basis for successful treatment is dependent on the identification of the involved muscles combined with the correct assessment of motility, misalignment, and binocular single vision. CONCLUSIONS: Due to increased muscle tightness, recessions are the first choice of surgical procedures. Dosing is the most difficult part of the surgery. Several techniques are described: deviation-correction with preoperative determination of the dose, duction-correction either by active or passive intraoperative evaluation of ductions, and the application of adjustable sutures. Achievable success rates are comparable among these techniques but are dependent on the surgeon's experience. Cyclotorsion and misalignment after decompression and combined horizontal and vertical misalignments are more challenging to correct. Those types of deviations need special solutions like surgery on the oblique muscles and the usage of implants. The field of binocular single vision is the main outcome criteria for success, and the quantification may differ for research questions, study designs, and clinical purposes.

True abscess formation is rare in bacterial orbital cellulitis; consequences for treatment.

BACKGROUND: Pre- or retroseptal bacterial orbital cellulitis (pOC/rOC) is not an uncommon orbital disease. Treatment consists of antibiotics with or without surgical drainage. Several questions regarding course, complications and outcome of treatment are unanswered and the indication for surgery is not well defined. The aim of this study is to: 1. describe the outcome of orbital cellulitis (OC) in a large cohort, 2. assess the significance of Chandler's classification, 3. assess the incidence of abscess formation in OC, and 4. redefine criteria for surgery. METHODS: Retrospective case series of patients with OC seen between 1-1-2007 and 1-1-2014 in a tertiary referral center. RESULTS: Sixty-eight patients presented with (presumed) bacterial pOC. Two out of these 68 developed rOC. All 68 patients had a full recovery. Forty-eight patients presented with rOC. Four out of 48 (8%) had intracranial extension of the infection at the time of admission. No admitted patient developed distant seeding. Only four (8%) patients with rOC had a true orbital abscess. In the other 92% we found a diffuse orbital inflammation or a subperiosteal empyema. Forty-four (92%) patients with rOC had a full recovery. CONCLUSIONS: 1. The prognosis of both pOC and rOC nowadays is generally favorable. 2. Chandler's classification is of little use. 3. True abscess formation in OC is rare. 4. The indication for surgical intervention must be based on the clinical presentation and the assessment of true orbital abscess formation.

Chronic orbital inflammatory disease and optic neuropathy associated with long-term intranasal cocaine abuse: 2 cases and literature review.

Orbital inflammatory disease and secondary optic neuropathy is a rare but devastating complication of long-term intranasal cocaine abuse. We describe 2 patients with a history of intranasal cocaine consumption who presented with subacute onset of unilateral vision loss from optic neuropathy and limitation of abduction in the affected eye. Magnetic resonance imaging findings included an orbital mass in combination with absent nasal septum and partial destruction of the paranasal sinuses. Biopsies and histopathologic examination of the nasal cavity and the orbital mass revealed chronic inflammation. Both patients were treated with oral corticosteroids, ocular movements completely normalized but no improvement of visual acuity was noted. Intranasal cocaine abuse can cause orbital complications from chronic sinonasal inflammatory disease and these patients are at risk to develop optic neuropathy. Optic neuropathy may be caused by compression, infiltration, or ischaemia.

Orbital Epithelioid Sarcoma: A Case Report.

Epithelioid sarcoma is a rare but often aggressive malignancy of soft tissue that usually occurs in young adults as a superficial lesion in the distal upper limbs. To date, there are only 4 case reports of epithelioid sarcoma primarily occurring in the orbit. Two of these patients were treated with primary exenteration only one of whom was alive 3 years after diagnosis. Radical surgical excision is thus the first treatment of choice for primary orbital epithelioid sarcoma. The authors present a patient with primary orbital epithelioid sarcoma who refused exenteration. Surgical debulking followed by local brachytherapy was performed. The patient remains tumor free 5 years after diagnosis. The literature remains limited regarding treatment options for primary orbital epithelioid sarcoma. However, based on reported cases and this case, the authors conclude that surgical excision combined with local iridium radiation therapy is an acceptable treatment when treating primary orbital epithelioid sarcoma.

Local Resection and Brachytherapy for Primary Orbital Rhabdomyosarcoma: Outcome and Failure Pattern Analysis.

PURPOSE: Survival in patients with orbital rhabdomyosarcoma (RMS) is excellent. Therefore, new local treatment modalities, such as brachytherapy, have been developed to minimize adverse events. Since 1990, patients with orbital RMS and a residual tumor after induction chemotherapy were eligible for resection and brachytherapy. Otherwise patients received external beam radiotherapy. In this study, the authors describe the outcome for 20 patients with primary orbital RMS. The aim was to assess risk factors for treatment failure in this single center cohort. METHODS: In this retrospective cohort study, the authors reviewed imaging studies, surgery reports, histology reports, and radiotherapy plans in a multidisciplinary setting. The authors included 20 consecutive patients with orbital RMS, treated between 1990 and 2007, (median age: 7.4 years, range: 0.7-16.1; median follow up: 11.5 years). RESULTS: After induction chemotherapy, 12 patients were treated with surgery and brachytherapy, 2 with external beam radiotherapy, and in 5 patients who achieved complete remission, local treatment was withheld. In 1 patient, brachytherapy was incorrectly withheld after delayed surgery. Seven patients relapsed (no local treatment, N = 2; surgery and brachytherapy, N = 2; external beam radiotherapy, N = 2; surgery only, N = 1). The authors found no patient, tumor, or treatment characteristics that predisposed for treatment failure. Ten-year-overall survival and event-free survival were 89% and 65%, respectively. CONCLUSIONS: Overall survival in this cohort of orbital RMS patients was good, including surgery and brachytherapy as treatment modality for orbital RMS resulted in an effective local treatment approach with fewer adverse events than external beam radiotherapy. The authors could not identify factors predisposing for treatment failure.

Outcome of inferior and superior rectus recession in Graves' orbitopathy patients.

PURPOSE: To evaluate the surgical effect of unilateral inferior rectus recession (IR-group) with or without a recession of contralateral superior rectus (IR-SR-group) on squint angle and motility restrictions in Graves' Orbitopathy (GO) patients. DESIGN: Retrospective case series. MATERIALS AND METHODS: Primary outcome parameters were the changes of squint angle 3 months and 6-12 months postoperatively. As in a previous study, success was defined as a postoperative vertical squint angle of ≤3° in primary position and on downgaze. Secondary outcome parameters were the influence of surgery on duction range and influence of muscle size on dose-effect response. RESULTS: Fifty-six patients were included in the study; 31 patients in the IR-group and 25 patients in the IR-SR-group. The amount of (fixed suture) recession ranged from 2 mm to 7 mm. Vertical deviations in primary position changed from 8.0° [95% CI 6.6-9.7°] to 1.0° [95% CI -0.4-6.5°] in the IR-group and from 17.0° [95% CI 15.7-20.0°] to 1.5° [95% CI 0.8-2.9°] in the IR-SR-group. The success rate was 74% in the IR-group and 64% in the IR-SR-group. Elevation significantly improved in both groups (IR-group p = 0.007; IR-SR- group p = 0.000). The volume of vertical rectus muscles as assessed on CT-scans did not influence the dose-effect response. CONCLUSIONS: The highest success rate and highest reduction of depression was found in the IR-group. The total duction range remained stable after strabismus surgery (IR-group) or improved (IR-SR-group). Both squint angle and cyclodeviation remained stable during long time follow-up (6-12 months after surgery).

Parallax-free exophthalmometry: a comprehensive review of the literature on clinical exophthalmometry and the introduction of the first parallax-free exophthalmometer.

PURPOSE: To present the first parallax-free exophthalmometer design. BACKGROUND: Exophthalmometry is an important clinical tool. We provide a historic overview of clinical exophthalmometer designs, and we review current problems encountered in exophthalmometry. METHODS: We present a new and parallax-free exophthalmometer design that we have evaluated in 49 patients visiting our orbital clinic. RESULTS: The mean age of the patients was 49.8 years and 72% were female. The Pearson interobserver variation was 0.97, and 94% of the Hertel values measured by the two observers were within the limits (1.6 mm) of agreement. CONCLUSION: This meter appears to be a reliable instrument for exophthalmometry. It is the first instrument that allows for a complete parallax-free measurement.

Ectopic brain tissue in the orbit: 5-year follow-up.

A neonate presented with a dilated pupil of the left eye. Magnetic Resonance Imaging (MRI) showed a mass in the left inferior part of the orbit. Incisional biopsy was performed. Histopathological examination revealed ectopic brain tissue. The patient had a 5-year follow-up without any growth on MRI. Ectopic brain tissue in the orbit is a rare finding in the literature, almost all involved infants or neonates. Pathogenesis is still unknown.

Direct orbital puncture of the cavernous sinus for the treatment of a carotid-cavernous dural AV fistula with a concomitant venous/lymphatic malformation.

A 37- year old male with a long history of a left orbital venous/lympathic malformation presented with ocular injection, increased proptosis and reduced left vision. Angiography demonstrated a carotid cavernous dural AV fistula combined with a concomitant venous/lymphatic malformation. After attempts at transvenous embolization, a direct uncomplicated transorbital puncture of the cavernous sinus via a lateral orbitotomy was performed with complete resolution of ocular symptoms.

Psychological aspects of Graves' ophthalmopathy.

PURPOSE: This review aims to discuss psychological aspects of Graves' ophthalmopathy (GO), estimate the prevalence of depression and anxiety disorders in GO, examine whether these psychiatric disorders are more prevalent in GO than in GD without eye disease, and evaluate the main contributors for depression and anxiety in GO. METHODS: A review of the literature. RESULTS: Both depression and anxiety are associated with GO. The prevalence of depression and anxiety disorders specifically in GO patients was estimated at 18-33% and 26-41%, respectively. The reported prevalence in GD patients ranged from 9-70% for depression and from 18-88% for anxiety disorders. Significantly higher levels of depression and anxiety were found in GD patients compared with patients with non-autoimmune hyperthyroidism. Conflicting results have been reported regarding the association of antithyroid autoantibodies with depression and anxiety disorders. Serum thyroid hormone levels do not correlate with the severity of depression and anxiety. An improvement of psychiatric symptoms is observed in hyperthyroid patients after treatment of thyrotoxicosis. Moreover, depression and anxiety are significantly related to impaired quality of life (QoL) in GO. Exophthalmos and diplopia were not associated with depression nor anxiety, but orbital decompression and strabismus surgery do seem to improve QoL in GO patients. CONCLUSIONS: The results of this review suggest that altered thyroid hormone levels and autoimmunity are prognostic factors for depression and anxiety in GO. With regards to the visual and disfiguring aspects of GO as contributing factors for depression and anxiety, no decisive conclusions can be made.

Long term follow-up of axial length and orbital dimensions in congenital microphthalmia and anophthalmia.

PURPOSE: To evaluate axial length (AL), orbital width (OW) and height (OH) development in congenital microphthalmia and anophthalmia (MICA) using serial ultrasonography measurements. METHODS: A longitudinal prospective cohort (n = 74) of unilaterally and bilaterally affected MICA patients was followed from 2013 to 2022 at the university hospital in Amsterdam, the Netherlands. Clinical entity, age, severity category based on axial length, conformer treatment and intra-orbital cysts were registered. The main outcome measures were the absolute and relative growth of AL, OW and OH. Surgical and intra-orbital cyst cases were described separately. RESULTS: Absolute microphthalmic eye size increased in 27/49 (55%) unilateral MICA eyes, but growth arrest/decrease in the remaining could shift the case to a more severe category over time. A final affected/unaffected orbital symmetry ≥80% was seen in the large majority of unilateral cases (45/46 for OW, 43/46 for OH). Cases with AL < 10.5 mm had orbital symmetry <80% more often. Most orbital symmetry changes were seen in moderate and severe unilateral cases treated with 3D-printed conformer therapy starting at age <1 year, with 6/10 (60%) symmetry increase, 30% unchanged symmetry and 10% symmetry decrease. All cases older than 6.5 years (n = 6) did not show any change anymore, regardless of treatment. For bilateral and unilateral mild cases, orbital dimensions kept the same proportions during follow-up, with or without conformer treatment. CONCLUSIONS: Using severity categories in MICA based on relative AL may aid the decision to start conformer treatment, as most orbital symmetry changes were seen in moderate and severe unilateral cases receiving 3D-printed conformer therapy that started under age 1.

Incidence of A pattern strabismus after inferior rectus recession in patients with Graves' orbitopathy: A retrospective multicentre study.

INTRODUCTION AND PURPOSE: Patients with Graves' orbitopathy (GO) may develop restricted elevation; this can lead to hypotropia, sometimes in combination with an abnormal head posture. Recession of one or both inferior rectus muscles is the first line surgery to restore eye motility in these patients. However, this may result in A pattern strabismus. This study was performed to determine the rate of occurrence of this type of incomitant strabismus and potential predictive factors. METHODS: All patients undergoing surgery on one or two inferior rectus muscles over a 10-year period were screened retrospectively for the A pattern, defined as a ≥5° difference in squint angle between the primary gaze and downgaze. The extraocular muscle thickness in patients with acquired A pattern was determined by computed tomography (CT) and compared with a control group consisting of patients randomly selected from the total cohort. RESULTS: In a total of 590 patients, surgery was performed on the inferior rectus muscle(s) during the study period; the A pattern was identified in 59 patients. Simultaneous surgery was performed on one or both medial rectus muscles in 32% of the patients. This group had significant incyclotorsion (p = 0.000) and less depression (p = 0.000) postoperatively. The mean amount of recession was 4.38 ± 1.53 mm in the A pattern group and 3.91 ± 1.37 mm in the control group (p = 0.032). The amount of depression was 50.2° ± 7.4° in the A pattern group and 57.3° ± 4.4° in the control group (p = 0.045). The inferior rectus muscle was significantly thicker in the A pattern than in the control group (p = 0.027), while there was no significant difference in the thickness of the superior oblique muscle between the two groups (p = 0.870). Of all patients with the A pattern, 47% required further surgery to achieve adequate binocular single vision. CONCLUSION: Increased preoperative inferior rectus muscle thickness and relatively limited depression could be predictors of postoperative A pattern inferior rectus recession in patients with GO. Step-by-step procedures are preferable in this surgically challenging group of patients.

Likelihood of spontaneous closure of cavernous sinus dural arteriovenous fistulas based on clinical and radiological findings.

BACKGROUND: Cavernous sinus dural arteriovenous fistula (CSDAVF) is a rare condition that radiologists would encounter in their careers. We aim to describe the clinical and radiological characteristics of this condition, and to provide a management workflow. METHODS: In our retrospective study, we studied 27 patients with CSDAVF from January 2007 to August 2020. Patients with direct cavernous sinus AVFs and patients with incomplete date were excluded. Clinical and radiological data were collected and analyzed. RESULTS: Fourteen patients were conservatively treated with spontaneous resolution while 13 patients had endovascular intervention performed. In the intervention group, seven patients had intra-cranial reflux seen on radiological imaging and six patients had clinical deterioration, hence requiring intervention. Clinically, among our patients, 21 had proptosis, 20 had conjunctiva hyperaemia, 18 had extraocular movement limitation, 13 had raised intraocular pressure, 11 had chemosis, ten had ocular pain, nine had ocular bruit, eight had headache and six had worsening visual acuity. Radiologically, a concurrence was seen between superior ophthalmic vein thrombosis and spontaneous resolution of the CSDAVF, as compared to those who underwent intervention. A paradoxical increase of ocular symptoms was seen despite a decrease of flow or stagnation of contrast in radiological imaging of CSDAVF. CONCLUSIONS: In our study, 52% of CSDAVF closed spontaneously. As deterioration of ocular symptoms in patients with CSDAVF might also reflect spontaneous progressive occlusion, it warrants dynamic vascular imaging to check the status of venous outflow. Patients with CSDAVF with corticovenous reflux or deterioration of visual acuity need more urgent (endovascular) treatment.