[Management of orbital inflammatory disorders in internal medicine: New findings resulting from a retrospective study of 31 consecutive patients]. / Prise en charge des orbitopathies inflammatoires en médecine interne : nouvelles données issues de l'étude d'une série de 31 patients consécutifs.

PURPOSE: To describe a case series of patients investigated in internal medicine for orbital inflammation (OI) since the individualization of the clinical entity of the IgG4-related orbital disease (IgG4 ROD). PATIENTS AND METHODS: Thirty four patients were consecutively referred by a specialized center where orbital biopsy was performed when the lesion was surgically accessible. Fourteen patients were excluded in case of missing data or lymphoma, periocular xanthogranuloma or Graves' orbitopathy. Patients with systemic or auto-immune disease according to the international criteria, or presenting with idiopathic orbital inflammation syndrome (IOIS), were included. Knowing the histological similarities between IOIS and IgG4 ROD, immunohistochemical assessment of plasma cells for IgG4 positivity was performed for every patient with available biopsy. Clinical and biological characteristics, treatment and response to treatment of included patients are reported. RESULTS: Among 22 included patients, 10 presented with orbital manifestation of a systemic or autoimmune disease including 2 sarcoidosis (9%) and 8 (36%) cases of non specific OI which were reclassified in IgG4 ROD. Finally, IOIS of various clinicopathological presentation was diagnosed for 12 patients including 10 with histological documentation. Whereas relapse and resistance were not found to be related to IgG4 positivity (50% in both IOIS and IgG4 ROD groups), another treatment in addition to corticosteroids was more often necessary in IgG4 ROD patients (50%) than in IOIS patients (25%). CONCLUSION: After ruling out auto-immune orbital diseases, especially IgG4 ROD, IOIS should be discussed. Factors conditioning the corticosteroid response are yet to be determined.

[Management of orbital inflammation in internal medicine. Proposal for a diagnostic work-up]. / Orbitopathies inflammatoires. Que doit savoir l'interniste ?

Inflammatory orbitopathies relate to an inflammatory state originating within the orbit and its adnexes, except the inner ocular globe. Orbital inflammation (OI) may be either localized manifestation of a proven or like autoimmune disease, or local response from immune system against infectious, structural or tumoral antigens. We review the clinical manifestations of OI, which provide helpful clues to the diagnosis and describe the inflammatory, infectious and neoplastic conditions classically associated with OI. Autoimmune diseases are probably the most common causes of OI associated with a bilateral dacryoadenitis (e.g., sarcoidosis, granulomatosis with polyangiitis, IgG4-related disease). We focused on a major part of the IgG4-RD spectrum, the IgG4-related orbital disease which has been recently described and the idiopathic orbital inflammation syndrome that one should consider in patients 40 years of age or older with non specific inflammation OI on biopsy but without underlying local or systemic disease. An algorithm for the diagnostic approach of OI was proposed. If systemic explorations fail to diagnose an underlying disease, histopathologic control is required for distinguishing non-specific OI from other differential diagnosis, especially lymphoma. In the cases of pure myositic locations and posteriorly located tumours where biopsy could damage to the optic nerve, analysis of orbital lesions in T2W IRM sequence may be helpful to distinguish idiopathic OI (IOI) from lymphoma. When the diagnostic work-up fails, a corticosteroid trial could be used, but its beneficial effect has to be cautiously interpretated before definitively diagnosing IOI. Finally, treatments used in main infllammatory orbitopathies were also reviewed.

Usefulness of colour Doppler flow imaging in the management of lacrimal gland lesions.

OBJECTIVE: To assess the role of colour Doppler flow imaging (CDFI) in the diagnosis and management of lacrimal fossa lesions. METHODS: Institutional ethical committee approval was obtained. Fifty-one patients with 62 lacrimal fossa lesions were retrospectively included from 2003-2015. All patients underwent conventional ultrasonography and CDFI, with a qualitative and quantitative analysis of the vascularization. All patients had lacrimal gland surgery. Definitive diagnosis was based on pathological examination. RESULTS: The study included 47 non-epithelial lesions (NEL) and 15 epithelial lesions (EL), with 24 (39 %) malignant lesions and 38 (61 %) benign lesions. NEL were significantly more likely to present with septa (p < 0.001), hypoechogenicity (p < 0.001), high vascular intensity (p < 0.001), both central and peripheral vascularization (p < 0.001), tree-shape vascularization (p < 0.05) and a low resistance index (RI) (p < 0.0001). EL were significantly more likely to present with the presence of cysts (p < 0.001), and a higher RI. Receiver operating characteristic curves identified a RI value of 0.72 as the best cut-off to differentiate NEL from EL, with a sensitivity and specificity of 100 %. CONCLUSION: CDFI is a valuable tool in the differential diagnosis of lacrimal fossa lesions. Resistance index measurement enables substantial distinction between EL and NEL, thus providing crucial data for surgical management. KEY POINTS: • CDFI is a valuable tool in lacrimal fossa lesions. • Resistance Index measurement enables substantial distinction between epithelial and non-epithelial lesions. • Management of patients becomes more appropriate.

Infraorbital nerve involvement on magnetic resonance imaging in European patients with IgG4-related ophthalmic disease: a specific sign.

OBJECTIVES: To measure the frequency of infraorbital nerve enlargement (IONE) on magnetic resonance imaging (MRI) in European patients suffering from an IgG4-related ophthalmic disease (IgG4-ROD) as compared to patients suffering from non-IgG4-related ophthalmic disease (non-IgG4-ROD). METHODS: From January 2006 through April 2015, 132 patients were admitted for non-lymphoma, non-thyroid-related orbital inflammation. Thirty-eight had both pre-therapeutic orbital MRI and histopathological IgG4 immunostaining. Fifteen patients were classified as cases of IgG4-ROD and 23 patients as cases of non-IgG4-ROD. Two readers performed blinded analyses of MRI images. The main criterion was the presence of an IONE, defined as the infraorbital nerve diameter being greater than the optic nerve diameter in the coronal section. RESULTS: IONE was present in 53% (8/15) of IgG4-ROD cases whereas it was never present (0/23) in cases of non-IgG4-ROD (P < 0.0001). IONE was only present in cases where, on MRI, the inflammation of the inferior quadrant was present and in direct contact with the ION canal. CONCLUSIONS: In European patients suffering from orbital inflammation, the presence of IONE on an MRI is a specific sign of IgG4-ROD. Recognition of this pattern may facilitate the accurate diagnosis for clinicians and allow for the adequate management and appropriate care of their patients. KEY POINTS: • IONE on an MRI is a specific sign of IgG4-ROD. • IONE recognition allows for a quicker diagnosis and appropriate management. • IONE appears when inflammation is in direct contact with the ION canal.

Open-angle glaucoma and paraoptic cyst: first description of a series of 11 patients.

We report 11 patients who were referred to our institution for severe open-angle glaucoma who had a paraoptic cyst on MR imaging. All cysts were extraoptic and retrolaminar; most were deforming the adjacent optic nerve. Cysts had a high signal on T2 and FLAIR sequences, and a variable signal on T1 and variable echogenicity, suggesting different proteinaceous content. Arterial vascularization of the optic nerve was normal. Cyst volumes were inversely correlated with the severity of glaucoma on the same eye (P < .01-.05, Spearman correlation coefficient). We hypothesized that such cysts may reflect a valve mechanism, which would allow preservation of the translamina cribrosa pressure and thus could preserve visual function. The rarity of this association, together with the frequent mass effect of the cyst on the optic nerve, stresses the necessity of long-term follow-up in these patients.

Tumor pathology of the orbit.

The term orbital tumor covers a wide range of benign and malignant diseases affecting specific component of the orbit or developing in contact with them. They are found incidentally or may be investigated as part of the assessment of a systemic disorder or because of orbital signs (exophthalmos, pain, etc.). Computed tomography, MRI and Color Doppler Ultrasound (CDU), play a varying role depending on the clinical presentation and the disease being investigated. This article reflects long experience in a reference center but does not claim to be exhaustive. We have chosen to consider these tumors from the perspective of their usual presentation, emphasizing the most common causes and suggestive radiological and clinical presentations (progressive or sudden-onset exophthalmos, children or adults, lacrimal gland lesions, periorbital lesions and enophthalmos). We will describe in particular muscle involvement (thyrotoxicosis and tumors), vascular lesions (cavernous sinus hemangioma, orbital varix, cystic lymphangioma), childhood lesions and orbital hematomas. We offer straightforward useful protocols for simple investigation and differential diagnosis. Readers who wish to go further to extend their knowledge in this fascinating area can refer to the references in the bibliography.

No more fear of the cavernous sinuses!

After a review of the anatomy of the cavernous sinuses (CS), this work presents the clinical picture and imaging protocol of lesions which occur in this area. It outlines extension and imaging features of these lesions. It emphasises MRI appearance, such as T1, T2 and diffusion signal, type of contrast medium uptake. A complementary CT scan is performed if an associated abnormality of the base of the skull is suspected on MRI (lysis, condensation). This paper proposes a straightforward classification system depending on imaging and sets out the principal symptoms of the main aetiologies of CS lesions which are represented by various diseases such as tumours, inflammations, vascular abnormalities. Complementary to imaging, their diagnosis is based on clinical data i.e. known cancer, signs suggesting inflammation. Its rich iconography allows this article to be used as a reference in current clinical practice.

Towards understanding ocular motility: III, IV and VI.

The study of the ocular-motor nerves must be exhaustive from their source (nuclei in the brainstem) down to the effector muscles (orbit). Visual disturbances have to be analysed by differentiating between a decrease in visual acuity and ocular-motor disorders. Imaging tests are dominated by MRI, including fine slices and gadolinium injection. A study of the Circle of Willis vessels is often useful, and essential in the case of type III impairment. A further CT scan is essential for analysis of the foramina, base of the skull and orbital walls. Impairment of CN VI requires a CT scan of the apex of petrous. The study of the cavernous sinuses must be in-depth (T2 and T1 after gadolinium and elimination of fats) and always comparative. Impairment of CN III is often complex, difficult to identify precisely (complete or partial, with or without a pupil impairment, associated with other neurological signs) and requires a reasoned study based on anatomical, semiological and pathological knowledge. Other than tumour diseases, it is necessary to consider less well known malformative, ischemic and inflammatory aetiology.

Trigeminal neuralgia.

Two different clinical entities, essential or secondary neuralgia, are associated with different pathologies. The pathways of CN V comprise the cervical spine, the brainstem, the root of the nerve and the three peripheral branches: V1, V2 and V3. The lesions responsible for neuralgia are neoplastic, vascular, inflammatory, malformative or post-traumatic. The examination protocol should explore the set of CN V pathways. Neurovascular compression is the main cause of essential neuralgia. It is investigated by T2-weighted inframillimetric volume. Two conditions are necessary to diagnose a neurovascular compression: localised on the root entry zone [(REZ), 2-6mm from the emergence of the pons] and perpendicularly. In the absence of neurovascular compression, thin slices and a gadolinium injection are necessary.

A wander through the land of the orbit.

This article shows the pathological and physiological images of the orbit, which each radiologist will commonly see in his or her everyday CT scan practice. It explains the images, following the course of the patient's trajectory through imaging as complications develop or in the post-treatment monitoring of some common disorders (orbital trauma, retinal detachment, postoperative appearances).

[How to deal with orbital syndrome: diagnostic approach]. / Conduite à tenir devant un syndrome orbitaire : approche diagnostique.

Evaluation of a patient with orbital disease needs a multidisciplinary approach. Clinical examination should seek to glean information regarding pathophysiologic effect and location and to evaluate consequences of this disease on cosmetic, vision and ocular motility. Procedures of imaging are based on the implied complementary exams that should be done according to the suspected clinical location and characters help to characterize the underlying process. Imaging can define the location, size, limits and relationship of the disease to other tissues and especially to the vascular system. It may be completed by blood test. The final diagnosis is based on several arguments and can appeal for a diagnostic biopsy in the most difficult cases.

[Free floating thrombus on carotid megabulb or suspended bulb: what kind of dysplasia?]. / Thrombus flottant sur un mégabulbe carotidien ou sur un bulbe suspendu, quel type de dysplasie ?

Floating carotid thrombi are a rare cause of stroke mostly associated with atheromatous plaques, cardiogenic emboli, arterial dissections and systemic diseases related to coagulopathic states or iron deficiency anaemia. We report the cases of two patients with stroke and carotid megabulb or suspended bulb associated with floating thrombus. These findings are rarely described probably related to a form of arterial dysplasia and seem to be responsible of local haemodynamic modifications.

[Imaging of orbital masses]. / Imagerie des masses orbitaires.

A wide spectrum of tumors and pseudotumors may involve the orbit. Their clinical presentation is often nonspecific, consisting of exophthalmos, diplopia, visual loss, orbital pain or signs of orbital inflammation. Imaging plays a major role in the etiological diagnosis, providing a precise analysis of the location of the lesion, its components, and its effects on adjacent and nearby structures. It studies tumor extension and often provides a good evaluation of its composition. Imaging is mainly performed with MRI. CT is needed when the lesion affects the orbital walls. Ultrasound is proposed in case of anterior location of the mass or vascular lesion. This paper presents a brief summary of the technical aspects of imaging and of the normal anatomy of the orbit and its components. It presents the best sequences needed to depict specific pathologies and the aspects of the most common diseases encountered.

Magnetic resonance spectroscopy findings in a case of hyperglycaemic hemianopia.

INTRODUCTION: Patients with nonketotic hyperglycaemia may present with neurological manifestations, including hemianopia. Thus far, the pathophysiology of such neurological events remains unknown, although the findings on magnetic resonance imaging (MRI) may help to elucidate the underlying mechanisms. CASE REPORT: This report describes a patient who had an episode of homonymous hemianopia, which coincided with a state of nonketotic hyperglycaemia. Initial MRI showed hypointense areas on T2-weighted and FLAIR sequences, involving the internal portion of the right occipital cortex and adjacent white matter, with mild hyperintensity on diffusion-weighted imaging. Magnetic resonance spectroscopy revealed significant increases in cerebral metabolites. Dramatic clinical and neuroimaging improvements were progressively observed over 3 weeks, following rehydration and normalization of blood glucose levels with insulin therapy. CONCLUSION: In this patient, magnetic resonance spectroscopy was used in combination with other neuroimaging methods and clinical evidence to suggest that hyperosmolality leading to intracellular dehydration in localized brain regions should be considered a potential underlying mechanism responsible for reversible neurological symptoms in nonketotic hyperglycaemia.

[Post-traumatic carotid cavernous fistula: report of two cases]. / Fistule carotido-caverneuse post-traumatique: à propos de deux cas.

Carotid cavernous fistula (CCF) is an abnormal communication between the cavernous sinus and the carotid arterial system. The authors reported the clinical presentation and therapeutic procedure of two cases. The physician has to be aware of this diagnosis when a patient is referred for a posttraumatic exophthalmia. The medical behaviour is multidisciplinary (ENT, ophthalmologist, radiologist and neurosurgeon). The imaging of choice is the angiography but angio-MRI and angio-CT can help to confirm the diagnosis. The endovascular embolization is the treatment of choice. It presents an acceptable risk of complication and a low risk of failure. In this paper the authors report 2 posttraumatic CCF cases treated with success by endovascular embolization.

[Presentation of a handicap, impairment and disability: the example of cerebral palsy]. / Présentation du handicap, déficit et incapacité: exemple de la paralysie cérébrale (PC).

This article, co-authored by a functional therapist and a neuroradiologist, proposes a definition as well as a general overview of epidemiological, legal and clinical concerns related to the handicap followed by a more in-depth study of subjects with cerebral palsy or other brain motor handicap. This patient population contains an increasing number of adults. The effects of aging and the increased incidence of letal malignancies in this population will be reviewed.The role of imaging in the management of these patients, its key features and related pitfalls are discussed. Finally, the role of MR imaging of the brain in patients with cerebral palsy is discussed with emphasis on the relation between lesion type and impairment, imaging characteristics and injury mechanism.

[Disability and epilepsy]. / Handicap et épilepsie.

The purpose of this article is to familiarize radiologists with the different aspects of severe drug resistant epilepsy. These result in three levels of disability: the disease itself (seizures and their impact, underlying cause), social impact (restrictions, safety and precautions, education, activities of daily life) and issues related to the medical treatment (long term medication intake, side effects, complications). First, clinical and EEG diagnosis will be reviewed to move on to MRI with attention to technical and protocol considerations followed by the imaging features of specific entities causing severe drug resistant epilepsy: migration and gyration disorders, Rasmussen's encephalitis, Sturge Weber disease and post-ischemic sequelae. Finally, current data and the imaging features of hemispherotomy, a radical treatment for epilepsy, will be presented.