Orbital Abnormalities Research Articles

Magnetic resonance imaging in dissociated strabismus complex demonstrates generalized hypertrophy of rectus extraocular muscles

Dissociated strabismus complex (DSC) is an enigmatic form of strabismus that includes dissociated vertical deviation (DVD) and dissociated horizontal deviation (DHD). We employed magnetic resonance imaging (MRI) to evaluate the extraocular muscles in DSC. We studied 5 patients with DSC and mean age of 25years (range, 12-42years), and 15 age-matched, orthotropic control subjects. All patients had DVD; 4 also had DHD. We employed high-resolution, surface coil MRI with thin, 2mm slices and central target fixation. Volumes of the rectus and superior oblique muscles in the region 12mm posterior to 4mm anterior to the globe-optic nerve junction were measured in quasi-coronal planes in central gaze. Patients with DSC had no structural abnormalities of rectus muscles or rectus pulleys or the superior oblique muscle but exhibited modest, statistically significant increased volume of all rectus muscles ranging from 20% for medial rectus to 9% for lateral rectus (P<0.05). DSC includes various combinations of sursumduction, excycloduction, and abduction not conforming to Hering's law. We have found modest generalized enlargement of all rectus muscles. DSC is associated with generalized rectus extraocular muscle hypertrophy in the absence of other orbital abnormalities.

Role of magnetic resonance imaging in heavy eye syndrome.

PurposeThe aim of this study is to assess the importance of magnetic resonance imaging (MRI) before surgery in highly myopic patients, evaluating the capability of imaging to identify muscles displacement and orbital pulley abnormalities and their role in the pathogenesis of heavy eye syndrome (HES).MethodsWe reviewed the medical records and high-resolution orbital MRIs of highly myopic adults with HES, who underwent surgery for strabismus at the Sant'Orsola-Malpighi University Hospital of Bologna from January 2007 to June 2016. The measure of the angle of dislocation between lateral rectus and superior rectus and lateral rectus-superior rectus band (LR-SR band) were evaluated on coronal MRI imaging.ResultsA total of 54 patients with HES were evaluated by MRI. Mean axial length was 30.5±3 mm. Mean distance esotropia was 49.2±14.4 prism diopters (PD) and the mean hypotropia was 4.3±4.4 PD. All subjects exhibited severe superotemporal globe prolapse that displaced the lateral rectus inferiorly and the superior rectus muscle medially with mean angle of dislocation of 167.5±12.9° on MRI. The LR-SR band was thinned in 56 eyes, ruptured in 8 and not evaluable in 1 case.ConclusionsIn our study, we detected muscles displacement in all subjects and we discovered alteration of the LR-SR band in patients with HES, suggesting a possible role in the pathogenesis of this disease. The evaluation of preoperative orbital MRI imaging in patients with highly myopic strabismus is helpful to detect different anatomical etiology and eventually to drive the choice of appropriate surgery.

CT and MR Imaging in the Diagnosis of Scleritis.

Scleritis is a rare, underdiagnosed vision-threatening condition that can occur isolated or in association with other orbital abnormalities. The etiology of scleritis is mainly inflammatory noninfectious, either idiopathic or in the context of systemic disease. Ultrasonography remains the criterion standard in diagnostic imaging of this condition but might prove insufficient, and studies on the diagnostic value of CT and MR imaging are lacking. We retrospectively analyzed 11 cases of scleritis in which CT and/or MR imaging were performed during the active phase of disease and assessed the diagnostic utility of these techniques. The most important imaging findings of scleritis were scleral enhancement, scleral thickening, and focal periscleral cellulitis. MR imaging is the recommended imaging technique, though posterior scleritis also can be accurately diagnosed on CT. It is important for the radiologist to be acquainted with these findings because being able to diagnose scleritis is of clinical significance and might be vision-saving.

The ophthalmic sequelae of Pfeiffer syndrome and the long-term visual outcomes after craniofacial surgery

Pfeiffer syndrome is a rare, genetic condition characterized by craniosynostosis and midface hypoplasia, with resultant ophthalmic sequelae. The gold standard of treatment is fronto-orbital advancement. We analyzed a large database of Pfeiffer syndrome patients to report the rate of ophthalmic sequelae and the long-term visual outcomes after craniofacial surgery and to compare Pfeiffer syndrome to other craniosynostosis syndromes. The medical records of Pfeiffer syndrome patients examined between 1988 and 2010 were examined retrospectively. Diagnosis was based on clinical and genetic testing. Long-term data were presented as a rate of incidence per person-year to overcome variable follow-up times. A total of 22 patients were included. Proptosis (n=21 [95%]), refractive error (n=13 [59%]), and strabismus (n=12 [55%]) were the most common primary features at presentation. Exposure keratitis (n=9 [41%]) and amblyopia (n=3 [14%]) were the most common secondary features. At presentation, 24 eyes [86%] with documented best-corrected visual acuity were normal; 4 [14%] were impaired; and none were blind. Fronto-orbital advancement reduced the rate of proptosis from 28%/person-year at presentation to 2%/person-year. There were no cases of active exposure disease postoperatively. At last follow-up, there was a 7%/person-year rate of impaired vision secondary to corneal scarring and amblyopia and a 3%/person-year rate of blindness-all from optic atrophy. In this study, the rates of proptosis and exposure keratitis were high in Pfeiffer syndrome, especially compared to Apert and Crouzon syndromes. Fronto-orbital advancement was successful in correcting orbital abnormalities. Long-term ophthalmic follow-up is essential to ensure best visual outcome.

Orbital Complications of The Paranasal Sinuses Disease

Introduction: The close relationship between diseases of the paranasal sinuses system and the orbit is based on their anatomical relationship. About 60% to 80% of the osseous eye socket is made up by sinus walls. Objective: To describe evaluation of orbital complications in patient with paranasal sinuses diseases at Dr. M. Djamil Hospital Padang for 2005-2014. Method: Descriptive study we obtained from medical record of patients during January 2005- December 2014. Data collected including age, sex, diagnosis of paranasal sinuses diseases, involved sinuses, orbital involvement and ocular presentations. Results: There were fifty five patients came to Ophthalmology Department Dr. M. Djamil Hospital Padang during period 2005-2014, either directly came or consulted from ENT Department, with suspicion of orbital involvement resulted from prior paranasal sinuses diseases, 34 ( 61.8%) male and 21 (38.2%) female. The age of patients range between 4 y.o. to 83 y.o., with the most common range of age was between 51 to 60 y.o. Orbital involvelment occurred in 45 patients (81.8%) while 10 patients (18.2%) had no orbital involvement. Orbital infiltration was caused by sinonasal tumor in 35 patients (77.7%), by sinus infection in 8 patients (17.7%), and by mucocele in 2 patients (4.6%). Sinonasal tumor was the most common paranasal sinuses disease which diagnosed in 43 patients (78.2%) and maxillary sinus (58.1%) was the most common involved sinus. Majority of the patients (67.7%) had presented with proptosis followed by ophthalmoplegia (54.8%) and visual acuity reduction (43.5%). Conclusion: The most common affected person were men and more frequently found in range of age between 51 to 60 y.o. Sinonasal tumor was the most etiology of orbital abnormalities in the the present of paranasal sinuses disease with maxillary sinus was the most common involved sinus. Proptosis was the major ocular presentation which found in orbital complications caused by paranasal sinuses diseases.

Hippocampal and orbital inferior frontal gray matter volume abnormalities and cognitive deficit in treatment-naive, first-episode patients with schizophrenia

BackgroundCognitive impairment is a core feature of schizophrenia. Some evidence suggests an association between cognition deficits and gray matter reductions. In this study, we investigated the relationship between cognitive performance and gray matter volumes in patients with treatment-naïve, first-episode schizophrenia. MethodFirst-episode patients with treatment-naïve schizophrenia and healthy controls went through brain imaging scan using high resolution magnetic resonance imaging. A neuropsychological battery including 8 neurocognitive tests was used to assess cognitive function. Voxel-based methods were used for volumetric measure in the brain. ResultsFifty-one patients and 41 healthy controls were included in the analysis. Patients exhibited a poorer performance on all 7 cognitive function tests compared with healthy controls (ps<0.006). There were significant gray matter volume differences between the two groups in bilateral hippocampus gyri, right superior temporal gyrus, left fusiform gyrus and orbital inferior frontal gyri (FDR, ps<0.05). Within the schizophrenia group, multiple regression analysis demonstrated that poorer performance on the working memory, verbal learning and visual learning was associated with smaller hippocampal gray matter volume, and poorer executive function was associated with smaller left orbital inferior frontal gray matter volume after controlling for potential confounding variables (β≥0.420, ps≤0.010). ConclusionsOur findings suggest that cognitive deficits are associated with hippocampal and orbital inferior frontal gray matter volume abnormalities in treatment-naïve, first-episode patients with schizophrenia.

Prevalence and anatomic mechanism of highly myopic strabismus among Japanese with severe myopia

To survey the prevalence of highly myopic strabismus among patients with severe myopia and to elucidate its anatomic mechanism. The subjects (mean age 48.5 ± 17.8 years) were 452 Japanese patients (778 eyes) who presented between May 2011 and April 2012 and were diagnosed as severely myopic (objective refractive error ≥-6.00 D or axial length ≥ 27 mm). For diagnosis, the eye position at 0.33 m, axial length, and diagnostic positions of gaze were determined; magnetic resonance imaging (MRI) was also used. Sixteen eyes of 9 patients fulfilled the disease criteria, and strabismus fixus was observed for 3 eyes of 3 patients. Disease prevalence was 2.65 %. The mean age of the 12 patients was 65.5 ± 17.9 years. For 18 eyes for which the axial length could be measured, mean axial length was 28.9 ± 2.03 mm. For the 16 eyes examined by MRI the mean dislocation angle was 132° ± 14.0°. The axial length was 26 to <30 mm for 13 of the 16 eyes, dislocation angle was 110° to <150° for 14 eyes, and both axial length and dislocation angle were within these ranges for 12 eyes. The prevalence of the disease was 2.65 %. However, this was not a general epidemiological survey and the screening criteria did not adequately cover all disease cases. In addition, even for eyes that are still mildly-moderately myopic, this disease can develop in the presence of orbital pulley abnormalities.

Diagnostic Imaging of Fetal and Pediatric Orbital Abnormalities

The orbit contains structures from which a wide spectrum of disease can arise. This article focuses on orbital anatomy and a simple compartmental approach to evaluating the orbit on diagnostic imaging. The characteristic findings of key fetal structural diseases and a wide spectrum of pediatric orbital disorders, including inflammatory disorders and developmental lesions, and the differential diagnosis of benign versus malignant masses will be discussed. Orbital abnormalities in fetuses may be recognized using ultrasound and MRI. Anophthalmia, hypertelorism, and hypotelorism either may be part of a genetic syndrome or may be related to a developmental abnormality of the fetal skull. In the pediatric population, cross-sectional imaging with CT and MRI offers a means to assess which compartments of the orbit are affected. Aggressive masses have characteristic features and must be evaluated for intracranial extension.

129. Cytomegalovirus antibody elevation: A potential bio-marker of impulsivity in bipolar disorder

Approximately 1/3 of Bipolar Disorder (BD) patients attempt suicide, often in the context of exacerbated impulsivity. In humans and animal models, a buildup of neurotoxic metabolites and contributes to orbital frontal white matter abnormalities. Hypofrontality is associated with impulsivity. However, what factors contribute to mechanisms underlying these cerebral alterations in humans in general and BD in particular remain unclear. Ubiquitous environmental human infections (Cytomegalovirus;CMV), fluctuate between latency and reactivation, potentially triggered by exposure to psychosocial stressors. While CMV reactivation has been shown to induce pro-inflammatory cytokines (e.g. TNF-α) which are themselves associated with depression, suicidality, and neurotoxic metabolite induction, investigations of CMV and its contribution to impulsivity in BD are lacking. We investigated 150 BD type I and 109 healthy control volunteers to determine if CMV IgG antibody concentrations were associated with measures of impulsivity and suicidality in BD-I. Using standard viral assay techniques, we quantified plasma CMV IgG antibodies. CMV antibody concentration was higher in BD-I than in healthy control volunteers (T254 = 2.9, p = 0.004), significantly correlating with measures of neurotic coping style (NEO-PI N5 impulsivity) (p

Comparison of Readout-Segmented Echo-Planar Imaging (EPI) and Single-Shot EPI in Clinical Application of Diffusion-Weighted Imaging of the Pediatric Brain

Readout-segmented echo-planar imaging (EPI) has been suggested as an alternative to single-shot EPI for diffusion-weighted imaging (DWI) with reduced distortion. However, clinical comparisons of readout-segmented EPI and EPI DWI are limited by unmatched imaging parameters and reconstruction procedures. Our goal was to compare the clinical utility of generalized autocalibrating partial parallel acquisition (GRAPPA)-accelerated readout-segmented EPI DWI with GRAPPA-accelerated EPI DWI for visualization of the pediatric brain in regions prone to distortion, such as the orbit, skull base, and posterior fossa. Thirty consecutive patients (mean age, 7.8 years) presenting with orbital, skull base, and posterior fossa neuropathologic abnormalities were scanned at 3 T. Images were obtained using GRAPPA-accelerated readout-segmented EPI and GRAPPA-accelerated EPI with an identical scanning time, acceleration factor, target resolution, and image postprocessing procedure. The two datasets were independently reviewed by two blinded neuroradiologists. Imaging studies were evaluated for resolution, signal-to-noise ratio (SNR), contrast, distortion, lesion conspicuity, and diagnostic confidence and graded using a 7-point Likert scale (1, nondiagnostic; 7, outstanding). There was good reader agreement in the scores (κ = 0.66; 95% CI, 0.54-0.78). The mean scores for EPI and readout-segmented EPI, respectively, were as follows: resolution, 5.0 and 6.0; SNR, 5.5 and 3.0; contrast, 3.7 and 3.2; distortion, 4.8 and 6.0; lesion conspicuity, 4.6 and 5.1; and diagnostic confidence, 4.7 and 5.4. Readout-segmented EPI was superior in resolution, distortion reduction, lesion conspicuity, and diagnostic confidence, whereas EPI scored better in SNR and contrast. Readout-segmented EPI was considered the better sequence overall in 85% of the cases. This study shows the benefits of improved resolution and reduced distortion of readout-segmented EPI in evaluating the orbit, skull base, and posterior fossa, sites of common neuropathologic abnormalities in children.

Crouzon Syndrome: Clinico-Radiological Illustration of a Case

Crouzon syndrome, also called craniofacial dysostosis, is an autosomal dominant disorder with complete penetrance and variable expressivity. Described by a French neurosurgeon in 1912, it is a rare genetic disorder characterized by premature closure of cranial sutures, midfacial hypoplasia, and orbital defects. Here, we report a case of this rare entity. The patient presented with brachycephaly, maxillary hypoplasia, exophthalmos, mandibular prognathism, along with dental and orbital abnormalities.

Magnetic resonance imaging findings of orbital disease in neurofibromatosis type 1 patients

To explore the magnetic resonance imaging (MRI) features of neurofibromatosis type 1 (NF1) with orbital involvement. We retrospectively reviewed the MRI findings of orbital abnormalities in 80 NF1 patients (86 orbits). The diagnosis was confirmed by the updated National Institute of Health (NIH) criteria. All of them underwent MRI scans of orbit or brain while 71 patients had contrast enhancement. The orbital abnormalities were documented in 80 patients. And 79 cases (85 orbits) had plexiform neurofibroma in orbit and/or adjacent regions. The orbital involvements extended to superficial temporal fossa (n = 61), infratemporal fossa (n = 31), cavernous sinus (n = 61) and pterygopalatine fossa (n = 51). There was mild-to-moderate enhancement in 71 orbits on contrast-enhanced MRI examinations. There were localized defects of orbital walls because of sphenoid bone dysplasia of greater wing (n = 79), sphenoid bone dysplasia of lesser wing (n = 72), augmentation (glaucoma) of eyeballs (n = 32) and optic nerve gliomas (n = 5). And 53 cases had hyperintense lesions on T2-weighted brain images. Orbital involvement is common for NF1. And MRI is a useful tool of evaluating the extent of involvements and other abnormalities in the NF1 patients.

MR Imaging of Congenital or Developmental Neuropathic Strabismus: Common and Uncommon Findings

High-resolution MR imaging enables direct imaging of the ocular motor nerves. The aim of this study was to assess the various causes of congenital or developmental neuropathic strabismus by using high-resolution MR imaging. High-resolution MR imaging was performed to evaluate the ocular motor nerves (CNIII, CNIV, CNVI) in 247 consecutive patients with suspected congenital or developmental neuropathic strabismus. These MR images, along with those obtained from conventional MR imaging of the brain and the orbit, were evaluated. MR imaging abnormalities were found in 112 patients: ocular motor nerve abnormalities in 98 patients (88%), orbital abnormalities in 9 patients (8%), and brain abnormalities in 5 patients (4%). Ocular motor nerve abnormalities were CNIV aplasia (63%), CNVI aplasia or hypoplasia (21%), CNIII aplasia or hypoplasia (3%), and combined CNIII aplasia and CNVI hypoplasia (1%). Orbital abnormalities were EOM hypoplasia (7%), EOM hypertrophy (1%), and fibrotic mass (1%). Brain abnormalities were periventricular leukomalacia (4%) and periventricular heterotopia (1%). Various MR imaging abnormalities were associated with congenital and developmental neuropathic strabismus. The most common abnormality was CNIV aplasia.

Optic nerve thickening and infarction as the first evidence of orbital involvement with mucormycosis

We report a 53-year-old female with uncontrolled diabetes mellitus who presented with decreased vision in the right eye for a few hours duration. Orbital computed tomography and magnetic resonance imaging were performed at presentation and showed a thickening and infarction of the right optic nerve with no other orbital abnormalities. A few days later, the patient developed necrosis in the region of the right medial canthus and nasal mucosa. Tissue biopsy confirmed the diagnosis of mucormycosis.

Spontaneous intraorbital hematoma: case report

Spontaneous intraorbital hematoma is an uncommon clinical entity seen in ophthalmology practice. It is poorly represented in the literature. Current evidence attributes it to orbital trauma, neoplasm, vascular malformations, acute sinusitis, and systemic abnormalities. A 65-year-old female presented with spontaneous intraorbital hematoma manifesting as severe ocular pains, eyelid edema, proptosis, and diplopia, without a history of trauma. Computer tomography demonstrated a fairly well defined extraconal lesion with opacification of the paranasal sinuses. The principal differential based on all findings was that of a spreading sinus infection and an extraconal tumor. An unprecedented finding of a spontaneous orbital hematoma was discovered when the patient was taken to theater. We discuss the rarity of this condition and its management.

Post‐traumatic oculomotor disorders

AbstractTrauma is a common ocular motor pathophysiology with a predilection for younger patients. Although afferent visual dysfunction is well known following trauma, the efferent conditions are perhaps less familiar. Trauma has a predilection to produce diplopia, and this may result from muscle, cranial nerve, nuclear, internucelar or supranuclear means. Orbital trauma commonly affects the extraocular muscles with signs to include proptosis and orbital bony abnormalities. CN4 is the most common ocular motor palsy post‐trauma, perhaps due to its long course around the midbrain; CN3 or 6 palsies are well know following trauma but often require more severe trauma than CN4 palsies. Supranuclear ocular motor dysfunction is common after trauma (e.g., skew deviation). Oscillopsia has been reported post‐trauma, most commonly related to vestibular ocular reflex abnormalities or nystagmus.

Prenatal Diagnosis of Craniomaxillofacial Malformations: A Characterization of Phenotypes in Trisomies 13, 18, and 21 by Ultrasound and Pathology

To determine the relationship between trisomies 13, 18, and 21 and craniofacial malformations detected by prenatal sonography. During a 29-year period (1976 through 2004), prenatal sonographic findings of 69 fetuses with trisomy 13; 171 fetuses with trisomy 18; 302 fetuses with trisomy 21; and 17 fetuses with other trisomies were evaluated retrospectively, after fetal karyotype identification. Sonographic findings were compared with autopsy results in 209 patients (trisomy 13, n=39; trisomy 18, n=64; and trisomy 21, n=106). For trisomy 13, cleft deformities were detected prenatally in 65.2%, and of the 39 cases with pathological information, 76.9% were found to have a cleft deformity. Ocular and orbital abnormalities were found in 28%. Malformations of the jaws and abnormal profiles were more frequently diagnosed postnatally than prenatally. For trisomy 18, abnormal profiles (41.5%) and ear abnormalities (5.3%) were the most noticeable ultrasound markers, next to abnormalities of the neurocranium (36.8%) and cranial bone configuration (21.6%). Dysmorphisms of the eye, ear, or nose were detected more frequently in autopsy cases. For trisomy 21, ultrasound showed an aberrant shape of the skull in 14.2% of fetuses. In general, the ocular-orbital and nasal abnormalities in fetuses with trisomy 18 or 21 were more evident in pathological examination than in prenatal ultrasound imaging. Facial anomalies are common in the major trisomies, and their prenatal sonographic identification should be improved. The above-mentioned facial anomalies provide sufficient reason to consider performing cytogenic evaluation.

Nontraumatic Orbital Conditions: Diagnosis with CT and MR Imaging in the Emergent Setting

Imaging provides crucial information regarding emergent orbital abnormalities, and the radiologist fulfills an important role in guiding patient care and contributing to favorable outcomes. Knowledge of the imaging features of nontraumatic orbital conditions commonly seen in the emergent setting-infections, inflammation, vascular abnormalities, and retinal and choroidal detachments-is necessary to achieve a prompt and accurate diagnosis, thereby avoiding permanent vision loss and other potentially devastating consequences. The ability to distinguish these entities from physiologic calcifications, posttherapeutic changes, and orbital devices allows optimal management without unnecessary further diagnostic work-up. For orbital imaging in the acute setting, computed tomography is the first-line modality, with magnetic resonance imaging playing an important secondary role.

Thyroid-Related Orbitopathy: Concepts and Management

Thyroid-related orbitopathy is a disorder related to systemic thyroid dysfunction, which can cause devastating orbital disease affecting nearly all of the orbital tissues. This includes orbital and eyelid abnormalities that may be acute, subacute, or chronic. The disease is complex and difficult to treat due varying activity of the systemic thyroid disease. The resulting morbidity of the disease ranges from mildly abnormal cosmesis and ocular irritation to blindness. A systematic approach to successful management of this disease requires timely medical and surgical interventions.

Orbital Syndromes

The orbit is a complex anatomical structure with unique properties not observed in other regions of the body. Composed of seven bones, the orbit is filled by the eye, optic nerve, lacrimal gland, extraocular muscles, peripheral motor and sensory nerves, fat, arteries, and veins. All these structures are intimately related to one another within an intricate framework of connective tissue. A variety of traumatic, vascular, inflammatory, infectious, and neoplastic processes can affect the orbit and its structures. Aside from the many primary orbital diseases, systemic disorders and pathological processes from neighboring structures (eye, ocular adnexa, oral cavity, paranasal sinuses, and intracranial cavity) can involve the orbit. A careful history and complete physical examination, with special attention to the orbit and ocular adnexa, are required to identify subtle orbital abnormalities that otherwise could be overlooked or mistakenly contributed to a nonorbital process. This article reviews the pertinent orbital anatomy, discusses the clinical evaluation and manifestations of orbital syndromes, and highlights several important orbital syndromes germane to the neurologist, including thyroid-associated orbitopathy, nonspecific orbital inflammation (also known as inflammatory orbital pseudotumor), perineural orbital invasion of cutaneous squamous cell carcinoma, rhino-orbital-cerebral mucormycosis, and carotid-cavernous sinus fistula.