A case of bilateral amyloidosis localized to extraocular muscles mimicking thyroid eye disease.

BACKGROUND: Amyloidosis is a rare condition characterized by the abnormal deposition of amyloid proteins in various tissues and organs. While systemic amyloidosis has been well-documented, amyloid deposition in extraocular muscles is an exceptionally rare occurrence, with only 35 reported cases. This case report sheds light on the importance of considering amyloidosis in the differential diagnosis of patients presenting with proptosis and diplopia, which are often associated with thyroid eye disease. CASE PRESENTATION: A woman in her twenties sought medical attention due to a complaint of diplopia. Her ocular examination revealed almost normal findings except for exotropia and proptosis. Orbital magnetic resonance imaging displayed fusiform enlargement of nearly all eye muscles, a presentation typically observed in thyroid eye disease. However, despite corticosteroid therapy, her symptoms showed no improvement. Given the unusual lack of response to conventional treatment, and inhomogeneous enhancement of the muscle, an extraocular muscle biopsy was conducted. This biopsy yielded a unique finding-amyloid deposition within the muscle tissue. This discovery was particularly intriguing due to the extreme rarity of amyloidosis affecting extraocular muscles, with fewer than three dozen documented cases worldwide. CONCLUSION: This unique case underscores the critical need for a comprehensive approach to diagnosing patients with proptosis and diplopia. While these symptoms are commonly attributed to thyroid eye disease, it is essential to consider alternative diagnoses such as amyloidosis, especially when standard treatments fail to yield results. The discovery of amyloid deposition in the extraocular muscles, although exceedingly rare, emphasizes the significance of a thorough differential diagnosis. In conclusion, this case report highlights the importance of vigilance in clinical practice, encouraging ophthalmologists to explore less common diagnostic possibilities when faced with challenging cases. Further research and clinical investigation are warranted to better understand the mechanisms and potential treatments for amyloidosis affecting the extraocular muscles.

Characteristics and Prognosis of Binocular Diplopia in Patients With Giant Cell Arteritis.

BACKGROUND: Giant cell arteritis (GCA) is a large vessel vasculitis associated with a risk of permanent ophthalmologic complications. Data about diplopia prognosis in GCA are scarce. This study was designed to better characterize diplopia in newly diagnosed GCA patients. METHODS: All consecutive patients diagnosed with GCA from January 2015 to April 2021 in a French tertiary ophthalmologic center were retrospectively reviewed. GCA diagnosis relied on a positive temporal artery biopsy or high-definition MRI. RESULTS: Among 111 patients diagnosed with GCA, 30 patients (27%) had diplopia. Characteristics of patients with diplopia were similar to other GCA patients. Diplopia resolved spontaneously in 6 patients (20%). Diplopia was attributed to cranial nerve palsy in 21/24 patients (88%), especially third (46%) and sixth cranial nerve (42%). Ocular ischemic lesions occurred in 11 of the 30 patients with diplopia (37%); 2 patients developed vision loss after initiation of corticosteroids. In the remaining 13 patients, diplopia resolved after treatment onset in 12 patients (92%) with a median delay of 10 days. Patients treated intravenously tended to have a quicker improvement than those treated orally, but with a similar resolution rate of diplopia at 1 month. Two patients had relapse of diplopia at 4 and 6 weeks after an initial treatment course of 24 and 18 months, respectively. CONCLUSIONS: Diplopia is a rare feature at GCA diagnosis, but should raise clinician suspicion for GCA when associated with cephalic symptoms and prompt the initiation of corticosteroids to prevent ocular ischemic complications.

Objective Diplopia Outcomes for Patients Treated With Teprotumumab for Thyroid Eye Disease.

BACKGROUND: Thyroid eye disease (TED) is a condition caused by inflammatory damage to the periocular tissue that often leads to double vision. Teprotumumab is an insulin-like growth factor 1 receptor antibody that was FDA approved for the management of TED in 2020, although much is yet to be elucidated regarding its effects on diplopia outcomes among patients with TED. Diplopia is a significant and life-altering effect of TED. Previous studies have reported the effect of teprotumumab on double vision subjectively using the Gorman diplopia score. However, there is a gap in the literature addressing the effect of teprotumumab treatment on objective ocular alignment measures. The purpose of our study was to address this gap. METHODS: We performed a retrospective review of patients who were diagnosed with TED, presented with diplopia, and treated with teprotumumab in a single-center academic ophthalmology practice. The primary outcome was change in ocular alignment in primary gaze position at 6 months (completion of teprotumumab treatment). Secondary outcomes included change in ocular alignment in other gaze positions, proptosis, eyelid position, and clinical activity score (CAS) at 6 months compared with baseline. To determine what factors may predict ocular alignment response to teprotumumab, we analyzed baseline characteristics among 3 groups, divided based on whether ocular alignment was worsened, stable, or improved at 6 months. RESULTS: Seventeen patients met inclusion criteria, 3 (18%) worsened, 10 (59%) were stable, and 4 (24%) improved. CAS ( P = 0.02) was significantly different among the groups and was higher in those who worsened and those who improved compared with those who remained stable. Right gaze horizontal prism deviation ( P = 0.01) and left gaze horizontal prism deviation ( P = 0.03) were significantly different among the groups, with a greater degree of left gaze horizontal prism deviation in the worse group than the stable group ( P = 0.04). CONCLUSIONS: Our study demonstrated that most patients remained stable after teprotumumab treatment regarding ocular alignment in primary gaze and the number of patients who improved was slightly higher than the number of patients who worsened after teprotumumab treatment. There are some baseline measures, such as CAS and right and left gaze horizontal prism deviation that can help better predict how a patient will respond to teprotumumab treatment. Our results can better inform physicians of how to counsel patients with TED when considering teprotumumab therapy.

First Intraoperative Trochlea Reconstruction.

Traumatic detachment of the superior oblique muscle from the trochlea is very rare. The authors present a case of cyclovertical diplopia in downgaze due to traumatic trochlear damage where they performed surgery more than 40 years later. For the first time ever, they describe the reconstruction of the trochlea using a silicone tube, thereby regaining superior oblique muscle function.

Superior Ophthalmic Vein Thrombosis Associated With Asymptomatic COVID-19 Infection.

Superior ophthalmic vein thrombosis is a rare condition scarcely described in clinical literature with potentially severe consequences including permanent vision loss. This report details the case of a 70-year-old woman who presented with acute binocular horizontal diplopia, relative proptosis of the OD by 4 mm, and pain OD. On exam, visual acuity was 20/20 OD and 20/30 OS with full extraocular movements. CT revealed proptosis OD with a thrombosed superior ophthalmic varix. Evaluation for etiology of hypercoagulability was unremarkable, although the patient did have an asymptomatic COVID-19 infection 1 month prior. To the authors' knowledge, this is the first reported case of superior ophthalmic vein thrombosis secondary to an asymptomatic COVID-19 infection.

Cranial Nerve Six Palsy After Vaginal Delivery with Epidural Anesthesia: A Case Report.

BACKGROUND: This case report describes a 34-year-old woman who developed diplopia and strabismus 2 weeks after a vaginal delivery and epidural anesthesia. CASE REPORT: A 34-year-old women presented to the emergency department (ED) with continued headache and new-onset diplopia after having undergone epidural anesthesia for a vaginal delivery 2 weeks prior. During that time, she underwent two blood patches, rested supine, drank additional fluids, and consumed caffeinated products for her spinal headache. When she developed double vision from a cranial nerve VI palsy, she returned to the ED. At that time, she had a third blood patch performed, and she was evaluated by a neurologist. The medical team felt the cranial nerve VI palsy was due to the downward pull of the brain and stretching of the nerve. Magnetic resonance imaging and neurosurgical closure of the dura were considered as the next steps in treatment; however, they were not performed after being declined by the patient. All symptoms were resolved over the next 3 weeks. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: This case illustrates the uncommon complication of a cranial nerve VI palsy from a persistent cerebrospinal fluid leak after a dural puncture. Emergency physicians must be aware that diplopia can be a rare presenting symptom after patients undergo a lumbar puncture. Furthermore, emergency physicians should be aware of the multiple treatment options available. Knowledge of the timeline of resolution of the diplopia is necessary to make shared decisions with our patients about escalating care.

Efficacy of tocilizumab in patients with moderate-to-severe corticosteroid-resistant thyroid eye disease: a prospective study.

PURPOSE: To evaluate the clinical outcomes of intravenous tocilizumab (TCZ) injection in patients with moderate-to-severe active thyroid eye disease (TED). METHODS: Patients with active and moderate-to-severe TED who did not respond to conventional therapies were treated with TCZ from June 2019 to January 2021. The medical records of the patients were evaluated before the treatment. We analyzed patient demographics, including the duration of Graves' disease and TED, and assessed subjective symptoms, such as diplopia and ocular movement, clinical activity score (CAS), modified NOSPECS score, and exophthalmos before treatment and at 4, 8, 12, and 16 weeks after the first drug injection. Blood tests, including TSH Rc Ab and TS Ab, were performed before treatment and 24 weeks after the first injection. And orbital computed tomography (CT) was performed and Barrett's Index was calculated at baseline and after completion of all injections. RESULTS: Nineteen completed the scheduled treatment. There were no significant side effects, other than herpes zoster in one case and headache and dermatitis in another. Clinical symptoms before and 16 weeks after the treatment showed mean CAS decreased by 2.4 points, mean modified NOSPECS score decreased by 3.7 points, and mean exophthalmos decreased by 0.4 mm. Diplopia and extraocular muscle limitation improved in ten and remained stationary in five of the 15 patients, who presented with extraocular motility abnormalities. Six of 11 patients who underwent orbit CT showed improvement in muscle size. The mean TSH Rc Ab decreased by 7.5 IU/L and TS-Ab decreased by 162.9%. CONCLUSION: TCZ can treat active moderate-to-severe TED, showing high drug compliance and reasonable response to inflammation and extraocular motility abnormality.

[Intermittent exotropia complicated with spasm of the near reflex: a case report].

A 21-year-old female patient presented to the Ophthalmology Department of Yunnan University Affiliated Hospital with complaints of "bilateral blurred vision accompanied by diplopia for 3 weeks". The patient's main symptoms included intermittent visual blurring, diplopia, headaches, and ocular discomfort. Ocular examination revealed intermittent exotropia, sometimes accompanied by esotropia or orthotropia, along with signs of pupillary constriction and pseudomyopia. Based on the clinical presentation, a diagnosis of intermittent exotropia complicated by spasm of the near reflex (SNR) was made. The patient underwent bilateral exotropia surgery, which corrected the ocular alignment and resolved the symptoms and signs of SNR postoperatively.

Nonneurologic causes of binocular diplopia for the neurologist.

PURPOSE OF REVIEW: To review several etiologies of nonneurologic diplopia that the neurologist should be familiar with. The pathophysiology, clinical presentation, neuroimaging findings, and treatment options are briefly summarized for each condition. RECENT FINDINGS: Certain etiologies for diplopia can be associated with life-threatening neurologic conditions and should be investigated thoroughly for proper localization and immediate management of the underlying disease. However, nonneurological causes of binocular diplopia are very common and disabling, but are often nonemergent and may not require in-depth investigation or neuroimaging. Some of the etiologies of nonneurologic diplopia mimic cranial nerve palsies, making them confusing to evaluate when the physician is not familiar with these etiologies. Unfamiliarity with these diagnoses can lead to unnecessary neuroimaging, inefficient utilization of medical resources, and undue alarm to the patient. Recent advances in clinical diagnostic tools and neuroimaging have clarified our understanding of the diverse underlying mechanisms of nonneurologic binocular diplopia. SUMMARY: Diplopia can be a difficult symptom for the neurologist to evaluate. A systematic approach to the evaluation of diplopia can help establish the urgency and necessity of an extensive workup.This review will help to familiarize the neurologist with nonneurological causes of binocular diplopia, minimize expensive and unnecessary evaluations, and reassure patients and physicians alike.

Diagnosis and management of Neuro-Behçet disease with isolated intracranial hypertension: a case report and literature review.

BACKGROUND: Neuro-Behçet's disease (NBD), characterized by isolated intracranial hypertension, is a rarely encountered condition, especially in children. In this study, we describe the treatment of a pediatric patient with NBD, who exhibited isolated intracranial pressure elevation as indicated by the initial manifestation of diplopia and physical examination finding of papilledema. CASE PRESENTATION: An 8-year-old boy was admitted to the hospital with a confirmed diagnosis of Behçet's disease (BD) over eight months. The patient also presented with the symptom of diplopia for three days. The evaluation of the patient's nervous system did not reveal any apparent abnormalities. The measurement of cerebrospinal fluid pressure yielded a reading of 470 mm H2O. The examination of the fundus indicated papilledema, and imaging scans revealed evidence of focal demyelination. The symptoms of the child showed alleviation after the administration of mannitol, methylprednisolone, and azathioprine. Furthermore, this study involved a comprehensive analysis of 18 cases of NBD with isolated intracranial hypertension, comprising one case reported herein and 17 cases from the literature review. Three cases were children, and an equal distribution of males and females 9:9 was noted. The average age at the onset of symptoms was 24.7 years (8-38 years). Headache (90%) was the most commonly reported clinical manifestation, followed by blurred vision or diplopia (80%). The ocular manifestations included papilledema (100%), abducent nerve paralysis (20%) and local eye hemorrhages in the retina (30%). Notably, 88.9% of these ocular manifestations were relieved or cured after treatment. CONCLUSION: This study presents the first reported case of NBD with isolated intracranial hypertension in the pediatric population of China. In a child with Bechet's disease presenting with features of raised intracranial pressure, it is important to be aware of neuro Bechet's presenting with intracranial hypertension without other neurological abnormalities. This will help make early diagnosis, institute treatment and prevent sequelae resulting from untreated raised intracranial pressure.

Case Report: Multiple Sclerosis Presenting as Unilateral Gaze-evoked Nystagmus.

SIGNIFICANCE: Unilateral gaze-evoked nystagmus is a rare neurologic finding that is largely diagnosed in connection with ischemic stroke. Gazed-evoked nystagmus is also a rare initial presentation of multiple sclerosis. PURPOSE: This study aimed to report a rare presentation of gaze-evoked nystagmus in a patient with multiple sclerosis and review the mechanism underlying the gaze-evoked nystagmus. CASE REPORT: A 32-year-old man presented with a 1-week history of diplopia. Neurologic examination revealed right-sided gaze-evoked nystagmus and right-sided ataxia. Laboratory test revealed a positive result for oligoclonal bands. Contrast brain MRI revealed multiple hyperintense T2 lesions including a hyperintense patch at the right inferior cerebellar peduncle. A diagnosis of multiple sclerosis was made. The patient received methylprednisolone 500 mg intravenously for 14 days. The diplopia and gaze-evoked nystagmus resolved and remained stable 2 months later. CONCLUSIONS: Our case demonstrates that damage to the inferior cerebellar peduncle may result in ipsilesional gaze-evoked nystagmus and ipsilesional ataxia, in contrast to ipsilesional gaze-evoked nystagmus and contralesional ataxia.

Case Report: Unilateral Cranial Nerve VI Palsy Secondary to Intracranial Hypotension.

SIGNIFICANCE: Intracranial hypotension is a condition that occurs from a cerebrospinal fluid leak. Various visual symptoms have been associated with this condition. Cranial nerve VI (CN VI) palsies are the most common ocular manifestation, as the abducens nerve is prone to injury because of its intracranial anatomical course. PURPOSE: This case report presents a CN VI palsy secondary to intracranial hypotension from ventriculoperitoneal shunt overfiltration. Diagnosis, treatment, and management considerations are discussed. No identifiable health information was included in this case report. CASE REPORT: A 70-year-old White man was referred to the eye clinic for evaluation of binocular horizontal diplopia. The patient had a recent history of a left ventriculoperitoneal shunt for a persistent cerebrospinal fluid leak after complex mastoid surgery. The patient was also symptomatic for positional headaches, which improved in a recumbent position. He was diagnosed with a left CN VI palsy secondary to intracranial hypotension from a ventriculoperitoneal shunt overfiltration. The patient was followed up by neurosurgery for shunt adjustments to resolve the overfiltration. Binocular horizontal diplopia was managed conservatively with Fresnel prism. CONCLUSIONS: Intracranial hypotension should be considered in patients presenting with cranial nerve palsies and positional headaches. Obtaining neuroimaging and comanaging with neurology or neurosurgery are advised to make prompt diagnosis and treatment. Careful clinical monitoring and conservative diplopia therapy are recommended as visual symptoms improve upon resolution of the cerebrospinal fluid leak.

Frequency and Etiologies of Visual Disturbance After Cataract Surgery Identified in Neuro-Ophthalmology Clinics.

BACKGROUND: To identify the frequency and etiologies of visual disturbances after cataract surgery in patients referred to Neuro-ophthalmology. METHODS: This study is a retrospective chart review. Records of patients 18 years and older referred to neuro-ophthalmology clinics for new-onset visual disturbances within 6 months of cataract surgery were reviewed. Those with pre-existing neuro-ophthalmic disorders, combined intraocular procedures with cataract surgery, or inadequate follow-up were excluded. The main outcome measures were frequency and etiologies of visual disturbances after cataract surgery. Secondary analyses of a cohort of patients who had cataract surgery at our institution were performed to determine the frequency and etiology of visual disturbances after uneventful cataract surgery. RESULTS: One hundred seventy-three patients met the inclusion criteria (internal referral: 36/173, from outside surgeons: 137/173). Sixty-one percent (106/173) were newly diagnosed with neuro-ophthalmic etiologies, including 21% (36/173) with afferent and 40% (70/173) with efferent disorders. Thirty-six percent (62/173) of patients had non neuro-ophthalmic causes and 3% (5/173) had systemic conditions responsible for visual disturbances postoperatively. Decompensated strabismus causing diplopia was the most common neuro-ophthalmic diagnosis after cataract surgery (50%, 53/106). Of the 13,715 patients who had cataract surgery performed at our institution over a 9-year period, 20 of 36 patients referred for visual disturbances were identified with neuro-ophthalmic etiologies of which 85% (17/20) had postoperative diplopia. CONCLUSIONS: In our study, decompensated strabismus causing diplopia was the most common neuro-ophthalmic visual disturbance after cataract surgery. Detailed history and ocular alignment should be assessed before cataract surgery to identify patients with the risk.

Temporal Slant Recession of the Inferior Rectus Muscle: A Simple Surgical Treatment for Diplopia Caused by Small Vertical Deviations.

BACKGROUND: To evaluate the therapeutic effect of temporal slant recession of the inferior rectus muscle (TSRIRM) for the treatment of small vertical deviations in patients with vertical diplopia, with consideration of the theoretical additive effects of this procedure on ocular torsion and horizontal incomitance. METHODS: Retrospective review of 11 patients who were treated with TSRIRM. Eight patients with vertical diplopia and small hyperdeviations (up to 6 prism diopters (PDs)) were treated with isolated TSRIRMs. Three patients with vertical diplopia from unilateral superior oblique palsies with large hyperdeviations (>15 PDs) were treated with TSRIRMs in conjunction with contralateral inferior oblique recessions. RESULTS: Six of the 8 patients with small vertical deviations had successful vertical realignment with elimination of symptomatic diplopia after an isolated TSRIRM. Two of the eight patients had residual hypertropia with symptomatic diplopia. Three additional patients with unilateral superior oblique palsy had successful vertical realignment with elimination of symptomatic diplopia after ipsilateral inferior oblique recession and contralateral TSRIRM. In total, 9 of 11 patients had successful surgical results after TSRIRM. CONCLUSIONS: TSRIRM provides an effective and reliable treatment for small angle vertical strabismus. Its ease of surgical access renders it useful for implementation in an outpatient neuro-ophthalmology setting.

Orbital floor fractures-a comparison between CT images and findings at surgery.

PURPOSE: The present study aims to investigate how well CT images correlate to surgical findings in orbital floor fractures and to the presence of diplopia. METHODS: In this cross-sectional study, 27 consecutive patients already selected for surgery due to an orbital floor fracture underwent a routine CT scan (axial, coronal, sagittal). An ophthalmologist established any presence of diplopia. Extent of fracture/injury seen on CT was compared to that discovered during surgery. RESULTS: In the surgeons´ opinions CT-images were in concordance with surgical findings in 71% of the cases. Agreement for pure blow out fractures was high (92%). Tetrapod fractures as a cause of an orbital floor fracture was only identified as such by surgeons in three of 11 cases, all subjected to orbital exploration, not only a closed reduction. Diplopia showed a significant correlation to rounding of the inferior rectus muscle at coronal CT. "Rounding" significantly correlated with the presence of a floor defect, to herniation of soft tissues and to the volume of displaced tissue. CONCLUSIONS: The results imply that the joint professional interaction between neuroradiology and surgery is important and would benefit from the use of an easy and well-defined classification system of orbital floor fractures. In Sweden a national record to collect data on all zygomaticomaxillary complex fractures assessed is to be started aiming at making general statements possible by time.

Superior divisional third nerve palsy due to breast cancer metastasis to the orbital apex.

PURPOSE: To report a case of breast cancer with metastasis to the orbital apex with manifestation of superior divisional third nerve palsy. CASE DESCRIPTION: A 40-year-old female, a known case of breast cancer diagnosed 3 years ago, referred to our neuro-ophthalmology clinic with chief complaint of ptosis and diplopia. In ophthalmic examination, visual acuities of both eyes were 20/20 and there was no significant finding in the anterior and posterior segment ophthalmic examination. There was left eye ptosis with MRD1 of 2 mm. In ocular movement examination, there was limitation of motion in supraduction (-2 to -3) of the left eye. Orbital MRI with contrast was requested for the patient which demonstrated a lesion in the orbital apex of the left eye. At the end, the patient was diagnosed as having superior divisional third nerve palsy. Orbital radiotherapy was recommended for the patient. CONCLUSION: In a patient with breast cancer who presents with diplopia, metastasis to the orbital apex with manifestation of divisional third nerve palsy should be kept in mind, and appropriate orbital imaging should be considered.

Ophthalmologic Findings in Pediatric Erdheim-Chester Disease: A Literature Review With a Novel Case Report.

PURPOSE: The authors aim to describe the ophthalmologic manifestations of pediatric Erdheim-Chester disease (ECD). METHODS: The authors describe a novel case of ECD presenting as isolated bilateral proptosis in a child and provide a comprehensive review of the documented pediatric cases to observe overall trends and ophthalmic manifestations of disease. Twenty pediatric cases were identified in the literature. RESULTS: The mean age at presentation was 9.6 years (1.8-17 years) with a mean time of symptom presentation to diagnosis of 1.6 years (0-6 years). Nine patients (45%) had ophthalmic involvement at diagnosis, 4 who presented with ophthalmic complaints: 3 with observable proptosis and 1 with diplopia. Other ophthalmic abnormalities included eyelid findings of a maculopapular rash with central atrophy on the eyelids and bilateral xanthelasmas, neuro-ophthalmologic findings of a right hemifacial palsy accompanied by bilateral optic atrophy and diplopia, and imaging findings of orbital bone and enhancing chiasmal lesions. No intraocular involvement was described, and visual acuity was not reported in most cases. CONCLUSIONS: Ophthalmic involvement occurs in almost half of documented pediatric cases. Typically presenting with other symptoms, the case highlights that isolated exophthalmos may be the only clinical sign, and ECD should be included in the differential diagnosis of bilateral exophthalmos in children. Ophthalmologists may be the first to evaluate these patients, and a high index of suspicion and an understanding of the varied clinical, radiographic, pathologic, and molecular findings are critical for prompt diagnosis and treatment of this unusual disease.

Intrasellar hemorrhagic chordoma masquerading as pituitary apoplexy: case report and review of the literature.

BACKGROUND AND IMPORTANCE: Chordomas are centrally located, expansile soft tissue neoplasms that arise from the remnants of the embryological notochord. Hemorrhagic presentation is exceedingly rare and can resemble pituitary apoplexy. Moreover, a purely intrasellar location of a chordoma is extremely uncommon. We report a case of a hemorrhagic intrasellar chordoma in an adult male, which presented similarly to pituitary apoplexy and was resolved with surgical resection. CLINICAL PRESENTATION: A 69-year-old male presented with a 4 week history of acute onset headache and concurrent diplopia, with significantly reduced testosterone and slightly reduced cortisol. His left eye demonstrated a sixth cranial nerve palsy. Magnetic resonance imaging of the brain showed a large hemorrhagic mass in the pituitary region with significant compression of the left cavernous sinus and superior displacement of the pituitary gland. The patient underwent an endoscopic endonasal transsphenoidal approach for the resection of the lesion. Near total resection was achieved. Final pathology revealed chordoma with evidence of intratumoral hemorrhage, further confirmed by immunopositive stain for brachyury. Post-operatively, the patient had improved diplopia and was discharged home on low dose hydrocortisone. At 3-month follow-up, his diplopia was resolved and new MRI showed stable small residual disease. CONCLUSIONS: Apoplectic chordomas are uncommon given chordoma's characteristic lack of intralesional vascularity and represent a diagnostic challenge in the sellar region. Our unique case demonstrates that despite our initial impression of pituitary apoplexy, this was ultimately a case of apoplectic chordoma that responded well to endoscopic endonasal surgery.

Pediatric Orbital Fractures: Outcomes in Relation to Time of Surgery.

INTRODUCTION: Orbital trapdoor fractures in children and adolescents can cause persistent problems with vision and appearance. Early surgery is recommended, although, because of the rarity of these fractures, there is a lack of evidence regarding the optimal timing of surgery.The objective of this study was to examine the effect of the time from trauma to surgery on the recovery time and severity of diplopia in children and adolescents with orbital trapdoor fractures. MATERIALS AND METHODS: A retrospective cohort study was performed of all orbital fractures in children and adolescents aged 0 to 20 years, treated at a tertiary referral center in 2005-2017. Data relating to demographics, cause of injury, surgery, time of follow-up, and final outcomes were extracted. The cases of trapdoor fracture were specifically examined with regard to the time from trauma to surgery and diplopia at last follow-up, which was the primary outcome. RESULTS: One hundred thirty-five patients, aged 2.4 to 20 years (mean 17.0), were treated for orbital fractures during the period; 37 (27%) had an isolated orbital floor fracture and 12 (9%) had a trapdoor fracture. All patients with trapdoor fractures underwent surgery; the mean time to surgery was 11.9 days in 2007-2011 and 1.1 days in 2012-2017. Although statistical significance cannot be proven in this small and retrospective study, a shorter time from trauma to surgery seems to lead to fewer problems with diplopia and 2 patient cases that highlight this are presented. CONCLUSIONS: Delayed surgical intervention in pediatric orbital trapdoor fractures increases the risk of delayed recovery and persistent diplopia. Other factors, such as the degree of muscle incarceration and necrosis and the surgeon's experience and skill, may, however, also influence the outcomes.

Reconstruction of Medial Orbital Wall Using Autologous Perpendicular Plate of Ethmoid.

The aim of this study is to investigate the feasibility of perpendicular plate of ethmoid as material for the reconstruction of medial orbital wall. The main outcome measurement was preoperative and postoperative orbital volume. The authors performed a study including 17 patients who have isolated medial orbital wall fracture (blow-out fracture). All the patients were fixed the defect using autologous perpendicular plate of ethmoid under endonasal approach. The authors compared the preoperative and postoperative orbital volume difference (unaffected orbit, affected orbit) of all the patients, and observed the improvement of diplopia or ocular motility disorders after operation. All 17 medial orbital wall reconstruction surgeries were successful with no severe postoperative ophthalmic complications. Statistically significant differences were found between the preoperative and postoperative orbital tissue volumes for the affected orbit. There was no statistically significant difference found between the tissue volume of the contralateral unaffected orbit and the affected orbit after reconstruction. And postoperative computed tomography showed the implant is in place and there is no medial rectus incarceration. Autologous perpendicular plate of ethmoid proved to be safe and effective in the reconstruction of medial orbital wall under endonasal approach with cost-effectivence, low complication rate, high biocompatibility, and minimally invasion.