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.

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.

[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.

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.

[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.

[CT and MR brain imaging following hemispherotomy]. / Imagerie scanner et IRM de l'encéphale après hémisphérotomie.

PURPOSE: The aim of the study was to define the usual and pathological modifications arising in the brain following hemispherotomy for intractable epilepsy in children. METHODS: Preoperative MRI and postoperative imaging scans (CT in the first week, MRI at 3 months and 1 year after surgery) were reviewed in a series of 52 patients, average age 8 years and 7 months, with intractable epilepsy due to dysplasia, Rasmussen's encephalitis, ischemic lesions and/or Sturge-Weber disease. The posterior fossa, brain parenchyma, ventricles and subdural space were also analyzed. RESULTS: Hemispheric scarring was a typical finding on CT and MRI as a consequence of the surgical procedure. Also frequently seen were small subdural effusions, bleeding along the surgical scar on early CT, and chronic subdural effusions with no mass effect on mid-term and late MRI scans. Other features - such as large subdural effusions that required external shunts and hydrocephalus - were rare, but severe, and considered to be postoperative complications. In contrast to the complications associated with other surgical techniques such as hemispherectomy, infection, extensive edema or hemosiderosis were never found in our series. CONCLUSION: Hemispherotomy is a surgical technique performed to treat intractable epilepsy. Our findings will help to identify the typical morphology of postsurgical scars, and to differentiate the usual features and complications seen in the postoperative period on CT and MRI brain scans.

[Neurenteric cyst of the posterior fossa. Case report and review of the literature]. / Kyste neurentérique de la fosse postérieure. A propos d'un cas, revue de la littérature.

Intracranial neurenteric cysts are rare entities. The term is currently used to describe epithelial cysts that are lined with a presumed endodermal-derived epithelium and are mostly located in the posterior fossa. Preoperative diagnosis is often difficult because of their clinical presentation, which may resemble a subarachnoid hemorrhage, and the radiological aspect, which can mimic vascular pathologies. We describe a posterior fossa neurenteric cyst in a 27-year-old woman, who presented with sudden headache as the only symptom and who was addressed to our hospital for subarachnoid hemorrhage. Diagnostic angiography was negative and MRI revealed a prepontine cystic lesion. The patient underwent a posterolateral approach on the right side, with subtotal resection of the lesion. We discuss the embryologic, diagnostic and therapeutic aspects of these cysts and review the literature.

[Bilateral papilledema: prospective study of fifty patients]. / OEdèmes papillaires bilatéraux : étude prospective de 50 patients.

In this prospective study, we report fifty consecutive cases of bilateral papilledema without neurosurgical or obvious ophthalmologic etiology, referred to our institution between January 2005 and March 2007. Lumbar puncture with opening CSF pressure measurement distinguished two groups of patients: Group 1 (n=39) with and Group 2 (n=11) without intracranial hypertension. In Group 1, 9/39 patients presented secondary intracranial hypertension mainly due to cerebral venous thrombosis. In 30 patients, after complete investigations, a diagnosis of idiopathic intracranial hypertension was made: as commonly reported, patients were predominantly overweight (96.7% with body mass index>25kg/m2) young (mean age=27.6 years) and women (96.7%). Eleven patients with intracranial hypertension had no headaches. In Group 2, the most common diagnosis was bilateral non-arteritic anterior ischemic optic neuropathy, but rare causes have been identified.

[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.

[MRI of the temporal bone]. / IRM du temporal.

Lesions arising within the temporal bone, where audition and balance receptors are located, are multiple. Beginning with a short clinical and technical overview, this presentation aims to review the most common temporal bone lesions, according to their location. Tumors and malignant otitis externa are the most common lesions of the external auditory canal. MRI features of cholesteatoma redux, transtegmental masses, and paragangliomas are described in the middle ear MRI study. The diagnosis of petrous apex abnormalities is emphasized: cholesterol granuloma, malignant tumor, epidermoid cyst, cholesteatoma, and petrositis. The diagnostic value of CT scan associated with MRI is stressed. This study also includes the main aspects of facial nerve lesions and vascular abnormalities of the area on MRI. The conclusion summarizes the main indications of temporal bone MRI.

[Vascular tumours and malformation of the orbit]. / Les lésions vasculaires de l'orbite.

Vascular diseases are an important part of orbital pathology. We describe vascular tumours of the orbit and vascular diseases with repercussion on the orbit, from intra or extra orbital origin. The classification of these abnormalities is difficult and several terms are used to describe the same histological entity. The objective of this work is, using the current classification, to illustrate the different imaging aspects of the most frequent vascular diseases of the orbit.

[Imaging of postoperative failures and complications in stapes surgery for otosclerosis]. / Imagerie des échecs et complications post-opératoires de la chirurgie de l'otospongiose.

Otosclerosis (OS) is a dysplasia of the otic capsule located in most cases on the anterior margin of the oval window or fissula ante fenestrum. Progressive conductive hearing loss is the major clinical symptom, due to stapedovestibular ankylosis. Stapes surgery is the only effective treatment of OS, with excellent functional results in more than 90% of cases. However, failures and complications of the surgery may be observed. In theses cases, the etiologic work-up includes imaging evaluation (CT and MRI). Imaging findings are extremely useful in the therapeutic decision. Surgical failure represents 80% of the causes for surgical revision. The main causes of failure are: displacement of the prosthesis, fibrosis of the oval window, erosion of the long process of the incus, incudo-mallear dislocation, obliterative otosclerosis. CT is essential for diagnosis. MR imaging is rarely indicated in the work-up of surgical failures. Labyrinthine complications account for less than 20% of surgical revisions. Etiologies of labyrinthine complications are: intravestibular penetration of the prosthesis, perilymphatic fistula, intra-vestibular granuloma, labyrinthitis and intravestibular bleeding. CT and MRI are complementary for the work up of these complications.

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.

Study of isotonic contrast media in intravenous digital subtraction angiography.

Arterial enhancement obtained with isotonic contrast media in intravenous digital subtraction angiography was studied. Ten dogs were injected with ioxaglate, iopamidol, and ioxitalamate at equal iodine concentration and at concentrations corresponding to plasma osmolality. Three variables were studied: osmolality, injection rate, and iodine dose. Provided their iodine concentration is sufficient, isotonic contrast media appear as efficient as the corresponding hypertonic formulation, at equal iodine dose. Moreover, the use of isotonic ioxaglate allows a lower dose of iodine to be administered without significant reduction in peak arterial value.

Contrasted diffusion and the use of CT scanner equipment in France.

During the last 4 years, there has been a rapid catching-up in the distribution level of CT-scanners in France. In a survey of CT-scanner utilization, data from all machines operating in the PACA Region in 1986, showed that the mean activity was 6,080 examinations per machine. The co-existence of both private and public medicine led to different use of materiel (5,500 to 5,800 exams per machine in the public versus 8,600 in the private sector and a higher productivity in the profit-making establishments. This difference was not explained by procedural variations in carrying out the technique, which appeared to be similar and standardized everywhere, but probably reflected patient selection (80% of ambulatory patients in the private sector, 25 to 50% in PTH).

Pseudotumoral neuro-Behçet's disease.

Pseudotumoral presentation of Neuro-Behçet's disease has been described before. The diagnosis may be difficult obtain in patients without mucosal, cutaneous, and ocular signs. We report the observation of a young patient suffering from a right hemiparesis with computed-tomographic (CT) features suggestive of a thalamocapsular expanding lesion. Histologic study of brain biopsy tissue ruled out a tumor but did not show any specific diagnosis. The patient improved with steroid therapy.