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Resume Introduction Les demandes de chirurgie esthetiques des paupieres sont frequentes. Le chirurgien dit mener un examen clinique complet afin d’ecarter toutes pathologies orbitaires responsables de poches palpebrales. Methodes Etude de dix patients ayant consulte dans le service de chirurgie oculopalpebrale de la fondation ophtalmologique de Rothschild pour une chirurgie esthetique des poches palpebrales et chez qui a ete diagnostique une pathologie sous-jacente. Resultats L’examen clinique attentif permet de rechercher des signes d’alarmes, domines par les antecedents, de caractere unilateral, des douleurs et de la presence d’une exophtalmie. Ceux-ci doivent conduire a la realisation d’un bilan d’imagerie et a la biopsie chirurgicale si une masse orbitaire a ete identifiee pour l’analyse histologie qui posera le diagnostic. Nous avons identifie quatre cas de lymphome orbitaire, un corps de granulome a corps etranger, une dacryoadenite chronique bilaterale, une atteinte sarcoidosique, un AAPOX syndrom, un hemangiome caverneux et une hernie graisseuse simple. Conclusion Cette serie de cas montre l’importance de ne pas meconnaitre une pathologie orbitaire sous-jacente avant de proposer une chirurgie esthetique pour des poches palpebrales.

BACKGROUND Although several studies have evaluated dynamic contrast-enhanced (DCE) MRI in the orbit, showing its utility when detecting and diagnosing orbital lesions, none have evaluated the pharmacokinetic models. PURPOSE To provide a quality-based pharmacokinetic model selection for characterizing orbital lesions using DCE-MRI at 3.0T. STUDY TYPE Prospective. POPULATION From December 2015 to April 2017, 151 patients with an orbital lesion underwent MRI prior to surgery, including a high temporal resolution DCE sequence, divided into one training and one test dataset with 100 and 51 patients, respectively. FIELD STRENGTH/SEQUENCE 3T/DCE. ASSESSMENT Six different pharmacokinetic models were tested. STATISTICAL TESTS Univariate and multivariate analyses were performed using Wilcoxon-2-sample tests and a logistic regression to compare parameters between malignant and benign tumors for each pharmacokinetic model for the whole cohort. Receiver operating characteristic (ROC) curve analyses were performed on the training dataset to determine area under curve (AUC) and optimal cutoff values for each pharmacokinetic model, then validated on the test dataset to calculate sensitivity, specificity, and accuracy. RESULTS Regardless of the model, tissue blood flow and tissue blood volume values were significantly higher in malignant vs. benign lesions: 103.8-195.1 vs. 65-113.8, P [

Resume Propos Nous rapportons une serie de patients explores en medecine interne pour une inflammation orbitaire (IO) depuis l'individualisation de la maladie a IgG4 (MAG4) en 2009. Patients et methodes Trente quatre patients ont ete adresses consecutivement par un centre specialise ou une biopsie orbitaire a ete realisee en cas de lesion accessible. Quatorze patients ont ete exclus en cas de dossiers incomplets ou en raison de lymphomes, xanthogranulomatoses periorbitaires ou de maladie de Basedow. Ont ete inclus les patients validant les criteres d'une maladie systemique ou auto-immune (MS/AI), ou ayant un syndrome d'inflammation orbitaire idiopathique (SIOI) en l'absence d'etiologie. Du fait de similitudes histologiques avec la MAG4, un immunomarquage a ete realise chez tous les patients avec un SIOI dont les prelevements etaient disponibles. Ont ete analysees les caracteristiques des patients inclus, leurs traitements et leurs profils evolutifs. Resultats Parmi les 22 patients inclus, 10 avaient une MS/AI d'expression orbitaire, 2 cas (9 %) de sarcoidose et 8 cas (36 %) d'IO idiopathique reclasses en MAG4 (MOAG4), et finalement, 12 patients (54,5 %) avaient un SIOI de presentation polymorphe, histologiquement documente pour 10 d'entre eux. Si la remission ne semblait pas dependre de l'immunomarque IgG4, les patients ayant une MOAG4 beneficiaient plus souvent d'un traitement de seconde ligne en complement de la corticotherapie compare aux patients SIOI (50 % vs 25 %). Conclusion Apres avoir elimine les pathologies auto-immunes et en particulier une MOAG4, on pourra evoquer un SIOI dont les facteurs de reponse a la corticotherapie restent a determiner.

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).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.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%).After ruling out auto-immune orbital diseases, especially IgG4 ROD, IOIS should be discussed. Factors conditioning the corticosteroid response are yet to be determined.

Requests for cosmetic eyelid surgery are frequent. The surgeon must carry out a complete clinical examination in order to rule out any orbital pathologies responsible for eyelid pockets.Study of ten patients who have consulted in the oculopalpebral surgery department of La Fondation Ophtalmologique de Rothschild for cosmetic surgery of the eyelid pockets and in whom an underlying pathology was diagnosed.The clinical examination allows to look for signs of alarms, such as medical history, unilateral involvement, pain and the presence of exophthalmos. These should lead to perform orbital imaging and surgical biopsy if an orbital mass has been identified for histological analysis which will make the diagnosis. We identified four cases of orbital lymphoma, one foreign body granuloma, chronic bilateral dacryoadenitis, sarcoidosis involvement, AAPOX syndrom, cavernous hemangioma, and simple fatty hernia.These cases shows the importance of not ignoring an underlying orbital pathology before proposing cosmetic surgery for eyelid pockets.

Objectives To evaluate repeatability of intravoxel incoherent motion (IVIM) diffusion-weighted imaging (DWI) parameters in the orbit. Methods From December 2015 to March 2016, 22 patients were scanned twice using an IVIM sequence with 15b values (0–2,000 s/mm2) at 3.0T. Two readers independently delineated regions of interest in an orbital mass and in different intra-orbital and extra-orbital structures. Short-term test-retest repeatability and inter-observer agreement were assessed using the intra-class correlation coefficient (ICC), the coefficient of variation (CV) and Bland-Altman limits of agreements (BA-LA). Results Test-retest repeatability of IVIM parameters in the orbital mass was satisfactory for ADC and D (mean CV 12% and 14%, ICC 95% and 93%), poor for f and D*(means CV 43% and 110%, ICC 90% and 65%). Inter-observer repeatability agreement was almost perfect in the orbital mass for all the IVIM parameters (ICC = 95%, 93%, 94% and 90% for ADC, D, f and D*, respectively). Conclusions IVIM appeared to be a robust tool to measure D in orbital lesions with good repeatability, but this approach showed a poor repeatability of f and D*. Key Points • IVIM technique is feasible in the orbit. • IVIM has a good–acceptable repeatability of D (CV range 12–25 %). • IVIM interobserver repeatability agreement is excellent (ICC range 90–95 %). • f or D* provide higher test-retest and interobserver variabilities. Electronic supplementary material The online version of this article (doi:10.1007/s00330-017-4933-6) contains supplementary material, which is available to authorized users.

Resume Le kyste anevrysmal osseux est une tumeur benigne rare d’etiologie inconnue, le plus souvent localisee au niveau de la colonne vertebrale et des os longs. Nous rapportons deux cas de kystes anevrysmaux osseux primitifs reveles par un syndrome orbitaire aigu du a une hemorragie sous-periostee. Le premier cas est une femme de 45 ans. L’imagerie montre une lesion frontale kystique de petite taille associee a un hematome sous-perioste. La recuperation visuelle est complete apres drainage de l’hematome, sans recidive postoperatoire. Le deuxieme cas est une femme de 74 ans. L’acuite visuelle est limitee a la perception lumineuse a cause d’un œdeme papillaire. L’imagerie orbitaire montre une large tumeur kystique de l’os frontal et un hematome sous-perioste qui comprime le nerf optique. L’arteriographie preoperatoire montre un blush vasculaire modere. Le traitement consiste en un drainage de l’hematome. Une recurrence locale hemorragique est survenue deux ans apres la chirurgie.

Introduction Carotid-cavernous fistulae are rare and affect visual prognosis. Their clinical presentation is varied and delayed diagnosis is common. They require rapid neuro-radio-ophthalmologic management. Purpose The goal of this study was to evaluate the long-term efficacy and prognostic factors for treatment of carotid-cavernous fistulas by embolization. Materials and methods A total of 60 patients with direct (10/60, 17%) or indirect (50/60, 83%) carotid-cavernous fistulae suspected during ophthalmological examination underwent cerebral angiography from December 2003 to October 2013. Of these, 59 (59/60, 98%) patients were treated with embolization. Treatment response was assessed with a follow-up angiogram confirming the exclusion of the fistula, and clinically by resolution of the initial symptoms. Results The diagnosis was suspected on the basis of proptosis in 45 patients (45/60, 75%), corkscrew episcleral vessels in 38 patients (38/60, 63%), chemosis in 37 patients (37/60, 61%), and diplopia in 30 patients (30/60, 50%). The median delay in diagnosis was 5 ± 5 months [0.5 to 24 months], and mean follow-up was 31 ± 31.5 months [0.5-118 months]. Eighteen patients (18/60, 30%) were lost to follow-up. Clinical response was complete in 24 patients (24/42, 57%) and partial in 14 patients (14/42, 33.5%). Symptoms were stable in one patient (1/42, 2.5%) and worsened in 3 patients (3/42, 7%). Morbidity per procedure was 3.3% and there was no postoperative mortality. Forty patients (40/60, 67%) had radiological follow-up and 39 patients (39/40, 97.5%) had a complete exclusion of the fistula. The presence of diplopia on initial examination was more frequently associated with an incomplete cure (P=0.04). Conclusion The combination of proptosis, corkscrew episcleral vessels and diplopia should rapidly lead to head imaging to search for a carotid-cavernous fistula. Fistula embolization is a safe and effective treatment. The presence of diplopia on initial examination may be associated with a poorer outcome (P=0.044).

Resume Objectif La reconstruction des larges deficits palpebraux superieurs bilamellaires reste un point sensible en oculoplastie. La grande particularite anatomique et fonctionnelle de la paupiere superieure temoigne de toute la difficulte a la reproduire. Nous avons voulu evaluer les resultats qu’apporte la technique du lambeau de Cutler-Beard. Methode Une etude retrospective a ete menee sur l’ensemble des patients ayant beneficie de cette technique dans le service d’oculoplastique de la Fondation Rothschild a Paris, entre janvier 2008 et juin 2013. Elle comprenait 16 patients. Resultats Quinze lambeaux sont restes vitaux tout au long du suivi. L’ouverture, l’occlusion et la position palpebrales etaient tres satisfaisantes dans respectivement 11, 12 et 11 cas. La surface oculaire etait preservee dans 8 cas et ne presentait que des alterations moderees dans 5 cas. Le resultat esthetique moyen etait note a 2/3 pour 11 patients avec photos postoperatoires. Nous n’avons constate aucune recidive en cas d’indication oncologique. Conclusion La reparation palpebrale superieure par la technique du lambeau de Cutler-Beard semble permettre une restauration fonctionnelle et esthetique satisfaisante dans la grande majorite des cas. Dans les autres, un geste complementaire simple sous anesthesie locale et/ou sedation, permet le plus souvent d’apporter une correction suffisante.

Resume L’hemangiome caverneux est la tumeur orbitaire benigne la plus frequente chez l’adulte. L’objectif de cette etude etait d’en etudier les caracteristiques cliniques, d’en definir les indications operatoires et de preciser la place des differentes voies d’abord pratiquees en ophtalmologie : voie transconjonctivale (de plus en plus utilisee), voie anterieure cutanee, voie laterale. Les dossiers de tous les patients pris en charge pour hemangiome caverneux orbitaire (HCO) a la fondation Rothschild depuis 2004 ont ete etudies de facon retrospective. Quarante-trois patients ont ete pris en charge pour HCO. Cinquante-huit pour cent etaient des femmes, l’âge moyen etait de 50,2 ans, 79 % des tumeurs etaient intraconiques. Trente-six patients ont ete operes, 5 patients beneficiaient d’une surveillance reguliere, et 2 patients ont ete perdus de vue. Les principales indications operatoires etaient : compression du nerf optique (26 patients), exophtalmie (24 patients) et diplopie (3 patients). Vingt-huit patients ont beneficie d’une orbitotomie par voie anterieure (16 par voie conjonctivale, 12 par voie cutanee), 4 par voie laterale, et 4 avaient une composante palpebrale facilement accessible en sous-cutane. Une paralysie partielle de la troisieme paire crânienne a ete observee chez 2 patients (l’un avait beneficie d’une voie conjonctivale, l’autre d’une orbitotomie laterale) ; une mydriase, d’une part, et une paralysie de la branche superieure de la 3 e paire crânienne, d’autre part, ont ete observees chez des patients operes par voie anterieure cutanee. L’exerese chirurgicale des HCO est necessaire en cas de retentissement clinique. La voie conjonctivale est une technique sure et efficace lorsque l’indication est bien posee.

Summary Purpose Evisceration can be performed for blind, painful eyes. This surgery can promote the dissemination of tumor cells within the orbit if an ocular tumor has been missed preoperatively. Methods We reviewed the medical records of patients who were eviscerated for blind, painful eyes between 2009 and 2014 and who were referred after the surgery to the Institut Curie or the Rothschild Foundation in Paris. We included the patients with a histological diagnosis of ocular tumor or orbital recurrence. Cytogenetic analysis was performed whenever possible. Results Four patients turned out to have an ocular tumor after evisceration (two choroidal melanomas, a rhabdoid tumor and an adenocarcinoma of the retinal pigment epithelium); two had a history of prior ocular trauma. The tumors were diagnosed either on histological analysis of the intraocular contents (2 patients) or biopsy of orbital recurrence (2 patients). Prior to evisceration, fundus examination was not performed in 3 patients. One had preoperative imaging but no intraocular tumor was suspected. At the time of this study, 3 patients had had an orbital recurrence and died. We also found 2 patients who had an evisceration despite a past history of choroidal melanoma treated with proton beam therapy. Conclusion We showed that evisceration of eyes with unsuspected ocular malignancies was associated with a poor prognosis due to orbital recurrence and metastasis. The evisceration specimen should therefore always be sent for histological analysis in order to perform prompt adjuvant orbital radiotherapy if an ocular tumor is found.

Orbito-palpebral vascular pathology represents 10% of all the diseases of this area. The lesion may be discovered during a brain CT scan or MRI, or because it causes clinical symptoms such as orbital mass, visual or oculomotor alteration, pain, proptosis, or acute bleeding due to a complication of the lesion (hemorrhage, thrombosis). We present these lesions using an anatomical, clinical, imaging and therapeutic approach. We distinguish four different entities. Vascular tumors have common imaging characteristics (hypersignal on T2 sequence, contrast enhancement, abnormal vascularization well depicted with ultrasound and Doppler, and possible bleeding). The main lesions are cavernous hemangiomas, the most frequent lesion of that type during adulthood; infantile hemangiomas, the most frequent vascular tumor in children; and more seldomly, hemangioperitcytomas. True vascular malformations are divided according to their flow. Low flow lesions are venous (orbital varix), capillarovenous or lymphatic (lymphangioma). High flow malformations, more rare, are either arteriovenous or arterial malformations (aneurisms). Complex malformations include both low and high flow elements. Lesions leading to modifications of the orbito-palpebral blood flow are mainly due to cavernous sinus abnormalities, either direct carotid-cavernous fistula affecting young adults after severe head trauma, or dural fistula, more insidious, found in older adults. The last section is devoted to congenital syndromic vascular malformations (Sturge-Weber, Rendu-Olser…). This classification allows for a better understanding of these pathologies and their specific imaging features.

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). 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. 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. 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. • 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.

Resume But Decrire la prise en charge des exenterations orbitaires ainsi que les modalites de reconstruction de la cavite orbitaire. Patients et methodes Etude retrospective portant sur les patients operes d’exenterations orbitaires entre 2000 et 2009. Analyse de l’âge au moment de l’exenteration, des etiologies, des complications ainsi que des methodes de reconstruction de la cavite. Resultats Nous avons opere 56 patients d’exenterations orbitaires entre 2000 et 2009 dont 31 hommes et 25 femmes, d’une moyenne d’âge de 62,5 ans au moment de l’exenteration. Le suivi moyen etait de 23 mois. Les principales etiologies etaient un carcinome basocellulaire palpebral (25 %) et un melanome conjonctival (20 %). Dix-huit patients (32 %) ont beneficie d’une reconstruction par lambeau musculaire et 24 patients (43 %) d’une reconstruction secondaire par implants osteo-integres apres epithelialisation spontanee de la cavite orbitaire. Pres de 50 % des patients ont recu de la radiotherapie postoperatoire. Discussion La reconstruction par lambeau musculaire peut masquer une recidive tumorale. L’epithelialisation avec mise en place secondaire d’implants osteo-integres comme support de l’epithese permet une meilleure surveillance carcinologique de la cavite ainsi qu’une bonne stabilite de l’epithese. La radiotherapie semble etre un facteur de fragilite de ces implants. Conclusion L’exenteration orbitaire est une technique mutilante. Les techniques de rehabilitation se sont ameliorees, en particulier la pose d’implants osteo-integres, permettant l’adaptation d’une epithese avec des resultats esthetiques satisfaisants.

BACKGROUND AND PURPOSE: Palpebral AVMs (pAVMs) are rare vascular lesions for which the treatment is challenging. Our aim was to present the technical aspects of the presurgical treatment by interventional neuroradiology of pAVMs and to report the clinical and angiographic results of combined (interventional neuroradiology/surgery) treatment of these malformations. MATERIALS AND METHODS: Nine patients (5 females, 4 males) with a mean age of 22 years (range, 12–35 years) were treated in our department from December 1992 to April 2007 for superficial pAVMs. Seven patients presented with isolated pAVMs, while 2 had hemifacial AVMs. Ten TAE procedures, by using a liquid embolic agent (glue or Onyx) or microparticles, were performed in 7 patients. Six patients underwent absolute alcohol, glue, or sclerotic agent injection by direct puncture in 8 procedures. Clinical and angiographic follow-up were performed with a mean delay of, respectively, 6.3 and 5 years. RESULTS: Three patients had a single EVT. Iterative procedures were performed in 5 patients. In 1 patient, EVT was not performed because of the risk of occlusion of the central retinal artery. No complication occurred except 1 case of transient palpebral hematoma. No visual acuity loss related to an endovascular procedure was reported. Exclusion of the AVMs at the end of the procedure was >75% in all cases and total in 3/8 cases. All the patients except 2 underwent at least 1 surgical procedure after the embolization. Good clinical regression of the mass was obtained in all patients at long-term follow-up. CONCLUSIONS: Combined endovascular and surgical treatment of pAVMs is an effective and safe technique with good clinical results at long-term follow-up.

Resume Objectif Dans l’orbitopathie dysthyroidienne (OT), l’augmentation volumetrique des muscles oculomoteurs et de la graisse orbitaire aboutit a une exophtalmie et a une augmentation de la pression intraorbitaire. Cette elevation de pression serait incriminee dans l’apparition d’une neuropathie optique compressive (NOC). Afin d’objectiver au mieux cette elevation de pression et ses variations accompagnant la decompression orbitaire, des mesures systematiques ont ete realisees en peroperatoire de toute decompression orbitaire pour OT. Materiel et methode Il s’agit d’une etude prospective portant sur 20 yeux (12 patients) presentant une OT pour laquelle une decompression orbitaire a ete decidee. Pour cinq yeux, l’indication operatoire a ete portee en raison d’une NOC, pour les 15 autres, il s’agissait d’une indication d’ordre esthetique. Sous anesthesie generale, avant toute incision, un capteur de pression (capteur de pression intracrânienne parenchymateuse Codman®) a ete implante par voie transcutanee dans le cadrant orbitaire temporal inferieur. Des mesures ont ete realisees avant incision, apres decompression de chaque paroi et en fin d’intervention. La pression maximale durant l’intervention a ete relevee. Resultats La pression intraorbitaire avant tout geste chirurgical etait de 14,05 mmHg ± 9,19 pour une valeur normale estimee dans la litterature a 4 mmHg ± 1,5 (difference statistiquement significative p Discussion Cette etude montre que la pression intraorbitaire est augmentee dans l’OT et davantage dans les formes graves avec NOC. La decompression orbitaire comme son nom l’indique permet une « decompression » et un retour a une situation pressionnelle proche de la normale. La NOC ne resulte donc pas seulement de la compression directe des muscles oculomoteurs sur le nerf optique mais aussi d’une elevation globale du niveau de pression au sein de la cavite orbitaire. Conclusion La pression intraorbitaire est elevee dans l’OT. Elle participe a l’apparition de la NOC. La decompression orbitaire permet une diminution significative du niveau pressionnel.

Introduction Les orbitopathies inflammatoires (OI) interessent les structures de l’orbite (muscles oculomoteurs, graisse, sclere et nerf optique) et ses annexes (glandes lacrymales et paupieres). Elles peuvent correspondre a l’expression ophtalmologique de maladies systemiques (MS), principalement les dysthyroidies, sarcoidose, granulomatose avec polyangeite (GPA), ou maladie associee aux IgG4 (MAG4). En l’absence de cause retrouvee, on decrit sous le nom de syndrome d’inflammation orbitaire idiopathique (SIOI), une inflammation non specifique purement localisee a l’œil. Les lymphomes orbitaires de bas grade se presentant parfois comme un SIOI, il est important d’apporter une preuve histologique. Cependant, la biopsie n’est pas toujours realisable. L’enquete etiologique doit alors etre la plus exhaustive possible afin d’eliminer toute pathologie sous-jacente. L’absence d’evolution vers une pathologie systemique demeure l’argument le plus robuste en faveur du diagnostic de SIOI [1] . Le TEP-18-FDG est devenu un examen de reference dans l’exploration des lymphomes et MS (sarcoidose, MAG4, GPA). Du fait de sa sensibilite, le TEP-18-FDG pourrait etre capable de detecter les localisations asymptomatiques des maladies sus-citees s’exprimant par l’œil. Nous proposons d’evaluer l’interet du TEP-18-FDG au diagnostic d’OI afin de separer le SIOI d’autres maladies d’expression orbitaire, ce qui a notre connaissance n’a jamais ete realise. Patients et methodes Les dossiers des patients explores consecutivement pour une OI de 2008 a 2017 a l’hopital Avicenne ont ete analyses retrospectivement. Leurs donnees cliniques, biologiques, histologiques et d’imagerie ainsi que leur evolution ont ete recueillies. Les TEP-18-FDG realises au diagnostic ont ete systematiquement re-interpretes en aveugle. Le TEP-18-FDG etait positif s’il mettait en evidence une lesion extra-orbitaire (adenopathies ou autres lesions viscerale) avec hyperfixation significative (Suv > 3). Les malades etaient classes au terme du suivi en SIOI ou pathologies a expression ophtalmologique (LNH histologiquement prouvee ou MS definies selon criteres internationaux). Nous avons evalue la rentabilite du TEP pour le diagnostic etiologique en termes de sensibilite (Se), specificite (Sp), valeur predictive positive (VPP), et valeur predictive negative (VPN). Resultats Parmi les 32 patients selectionnes, 22 ont ete explores par TEP-18-FDG. Ils avaient un âge median de 50 ans (min–max : 20–88 ans) et un sex-ratio F/H de 1,6. L’atteinte ophtalmologique touchait principalement les glandes lacrymales (7/22, 40 %) et etait majoritairement unilaterale (15/22, 68 %). Sur les 22 patients, 11 (50 %) avaient un TEP-18-FDG positif. Une pathologie sous-jacente a l’OI a ete diagnostiquee chez chacun d’eux (vrais-positifs : 100 %, faux-positifs : 0 %). On notait 4 cas de lymphomes, 3 cas de xanthogranulomatoses peri-orbitaires associees a un asthme de revelation tardive, 2 cas de MAG4 a taux serique eleve, 1 cas de GPA et 1 syndrome de Gougerot-Sjogren (SGS). Parmi les 11 patients ayant eu un TEP-18-FDG negatif, 7 patients avaient ete etiquetes SIOI (vrais negatifs : 63 %). Le diagnostic etait histologiquement documente chez 5 d’entre eux et confirme a l’issue du suivi post-TEP (mediane : 18 mois, min–max : 6–96 mois). Enfin, 4 patients sur les 11 ayant un TEP-18-FDG negatif avaient cependant une MS sous-jacente (faux negatifs : 36 %). Il s’agissait dans 3 cas de MAG4 a taux serique normal et d’un cas de SGS. Ainsi, le TEP-18-FDG avait une Se de 73 %, Sp de 100 %, VPP de 100 % et VPN de 63 %. Conclusion Au diagnostic d’une OI, une hyperfixation extra-ophtalmologique au TEP-18-FDG permet d’ecarter l’hypothese d’un SIOI.

Resume Introduction Les corps etrangers vegetaux de l’orbite sont rares et peuvent avoir des presentations cliniques differentes. L’objectif de cet article est de mettre en evidence le danger inherent a ce type de corps etrangers, de presenter les aspects en imagerie et de suggerer une approche diagnostique ainsi qu’une attitude therapeutique quand un corps etranger vegetal est suspecte. Nous rapportons trois cas de corps etrangers vegetaux intra-orbitaires qui se sont presentes par differents tableaux cliniques. Observations Le premier cas est un homme, âge de 43 ans, ayant consulte pour une fistule cutanee chronique de la paupiere inferieure droite depuis 4 mois suite a un traumatisme de l’orbite avec un objet en bois. Deux chirurgies furent necessaires pour l’exerese des corps etrangers vegetaux intra-orbitaires. Dans le deuxieme cas, une femme âgee de 37 ans, examinee pour un ptosis post-traumatique apparu apres une chute de bicyclette, l’imagerie mit en evidence une fracture du toit de l’orbite gauche et un corps etranger extra-conique superieur qui fut enleve par voie neurochirurgicale. Enfin, le troisieme cas est un homme, âge de 69 ans, adresse pour un abces orbitaire droit suite a un traumatisme avec une branche d’arbre. Le patient presentait une inflammation orbitaire droite persistante depuis 4 mois malgre deux chirurgies de drainage et d’exerese d’un corps etranger vegetal de l’orbite. Une troisieme chirurgie fut necessaire pour l’exerese complete des corps etrangers. Discussion/Conclusion La detection d’un corps etranger vegetal intra-orbitaire est parfois difficile, principalement dans les cas ou l’histoire du traumatisme orbitaire n’est pas claire, l’examen ophtalmologique apparemment sans anomalie, ou chez les patients qui consultent plusieurs mois apres le traumatisme. Les corps etrangers vegetaux intra-orbitaires peuvent entrainer des complications orbitaires et intracrâniennes potentiellement graves. La tomodensitometrie et l’echographie orbitaires peuvent etre faussement negatives: en particulier, l’hypodensite du corps etranger en bois a la tomodensitometrie peut etre confondu avec de l’air. Des fragments de corps etranger mobiles ou friables peuvent persister meme apres plusieurs interventions. L’imagerie par resonance magnetique orbitaire est utile dans un contexte de traumatisme quand un corps etranger vegetal est suspecte. La suspicion clinique est cependant primordiale pour poser le diagnostic.

Surgical treatment of diffuse adult orbital lymphangioma: two case studies A. Berthout, P.V. Jacomet, M. Putterman, O. Galatoire, S. Morax. Purpose: Orbital lymphangioma is a rare vascular malformation; it is a benign but severe anomaly because of its infiltrative, diffuse, and hemorrhagic nature, and its high morbidity rate. Surgical resection is a real challenge on account of the intricate architecture of the lesion. The authors report their surgical experience concerning two cases of diffuse orbital lymphangioma whose diagnosis was established in adulthood and whose surgical treatment was successful. Case reports: Two patients presented with adult orbital lymphangioma. Progression was slow during the first decade and then was quickly followed by complications: major exorbitism, compressive optic neuropathy, and corneal exposure. Neuroimaging showed a diffuse and cystic orbital malformation. Surgical resection was performed as completely as possible, in one case with a Kronlein orbitotomy and in the other case only via a conjunctive route. An aspirate drain was put in the orbit for 48 h so as to prevent dead spaces forming after resection, an essential risk factor of hemorrhagic or cystic recurrence. Systemic corticotherapy was administered for the 5 days following surgery. The resection was total in one case and subtotal in the other. The surgical follow-up was uneventful with an excellent aesthetic result and an improvement in visual acuity. After 12 months, no tumoral or hemorrhagic recurrence was noted. Discussion: The surgical treatment of orbital lymphangiomas is challenging because of their infiltrative nature. In diffuse forms, a complete resection is rarely possible because of the risk of sacrificing visual function. In the two cases reported herein, the resection of the extraconal portion was complete, but the intraconal portion was completely removed only in one case. Using the aspirate drain, negative pressure was maintained in the orbital cavity, preventing the formation of chocolate cysts induced by surgery. Although the clinical result was very satisfying, long-term follow-up is necessary to evaluate recurrence. Conclusion: Diffuse orbital lymphangiomas can be treated successfully with a subtotal resection without clinical recurrence in the short term. These results are promising because they demonstrate that many orbital lymphangiomas can benefit from surgical treatment with an excellent esthetic and functional result.

Aneurysmal bone cyst is a rare benign bone neoplasm of unknown cause. The most commonly affected anatomical sites are the vertebral column and long bones. We report two uncommon cases of primary orbital aneurysmal bone cyst presenting as an acute orbital compartment syndrome due to subperiosteal hemorrhage. Case 1 is a 45-year-old woman. Imaging studies revealed a small cystic frontal bone tumour associated with a subperiosteal hematoma. The patient achieved full visual recovery after drainage of the hematoma, with no recurrence after treatment. Case 2 is a 74-year-old woman whose visual acuity was light perception due to severe papilledema. Imaging studies of the orbit revealed a large cystic frontal bone tumor associated with a subperiosteal hematoma causing globe and optic nerve compression. Preoperative arteriography showed a moderate vascular blush. Drainage of the hematoma was performed. A local recurrence with hematoma formation occurred two years after the surgery.

To assess the role of colour Doppler flow imaging (CDFI) in the diagnosis and management of lacrimal fossa lesions. 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. 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

PURPOSE To report a patient with an adenocarcinoma in a pleomorphic adenoma of the lacrimal gland. Adenocarcinoma constitutes a distinct group of epithelial malignancies of the lacrimal gland. METHODS The clinical presentation, workup, surgical approach, and pathological findings were reviewed. RESULTS A 56-year-old man presented with a 3-month history of a lacrimal fossa mass. This patient presented a painless mass in the upper outer eyelid with significant displacement of the globe. The tumor was localized to the lacrimal gland. Radiological investigations showed a round, well-defined lesion in the fossa of the lacrimal gland. We performed a lateral orbitotomy; en bloc resection was accomplished. The final specimen showed an adenocarcinoma in a pleomorphic adenoma. No recurrences were detected during follow-up. CONCLUSION Although adenocarcinoma has rarely been reported in association with a pleomorphic adenoma of the lacrimal gland, this combination can exist. If the malignant tumor is limited in the pleomorphic adenoma, the prognosis appears to be better than in cases of local extension. Adjuvant therapy, radiation or others, are not useful.

Large, full thickness upper eyelid defects are still difficult to deal with in oculoplastic surgery. High specificity in the anatomy and function of the upper eyelid shows how difficult reconstruction can be. We aimed to assess the results provided by the Cutler-Beard flap technique.We conducted a retrospective study of all patients who had undergone Cutler-Beard flap reconstruction in the Oculoplastic service of La Fondation Rothschild in Paris, from January 2008 to June 2013. Sixteen patients were included.Fifteen flaps remained viable during the follow-up examinations. Opening, occlusion and palpebral position were very satisfactory in 11, 12 and 11 cases respectively. The ocular surface was preserved in 8 cases and exhibited only mild alterations in 5 cases. The average aesthetic result was noted 2/3 for 11 patients with postoperative photographs. We did not observe any relapses with oncologic indications.Upper eyelid reconstruction by the Cutler-Beard flap technique appears to provide good functional and aesthetic restoration in the majority of cases. In the others, a simple additional surgery under local anaesthetic and/or sedation provides adequate correction in most instances.

Cavernous hemangioma is the most frequent benign orbital tumor in adults. The purpose of this study was to examine its clinical features, to define surgical indications, and to determine the roles of the various surgical approaches praticed in ophthalmology: transconjunctival (increasingly utilized), anterior transcutaneous, and lateral orbitotomy. The records of all patients treated for orbital cavernous hemangioma (OCH) since 2004 at the Fondation Rothschild (Paris, France) were retrospectively reviewed. Forty-three patients were treated for orbital cavernous hemangioma. Fifty-eight percent were women, mean age 50.2; 79 % of the tumors were intraconal. Among those patients, 36 underwent surgical removal, 5 were followed periodically, and 2 were lost to follow-up. The main surgical indications were: optic nerve compression (26 patients), proptosis (24 patients) and diplopia (3 patients). Transconjunctival, anterior transcutaneous and Kronlein approaches were used in 16, 12 and 4 patients respectively. Four patients had intrapalpebral hemangiomas easily reached transcutaneously. Two patients demonstrated transient partial 3rd nerve palsy (one with the lateral orbitotomy approach and one with the transconjunctival approach), one patient with the lateral orbitotomy approach developed a palsy of the superior branch of the 3rd nerve, and one patient with the transcutaneous anterior approach developed mydriasis. Surgical excision of OCH's is required in the presence of clinical complications. The transconjunctival approach is a safe technique which can lead to complete resection of the tumor in most cases.

Purpose Idiopathic orbital inflammation is a rare clinical entity which has protean clinical manifestations. It’s a diagnosis of exclusion which imposes a biopsy, and which can be made only after a screening to rule out a systemic etiology of inflammation. Methods Retrospective series of 18 histologically-proven orbital inflammation cases seen in our service between 2006 and 2011. Results The study encompassed 7 men and 11 women, with a mean age of 47 years old (4-83). Patients complained of a pain (44%), a diplopia (33%), a decreased visual acuity (17%). A swollen eyelid or a palpable mass were presents in 78% of the cases. Less often, we noted proptosis, or a diminished ocular motility. The radiologic analysis (CT-scan, NMR, doppler ultrasound examination) found an inflammation localized to the orbital fat, to the lacrymal gland, and to one or several oculomotor muscle(s) in respectively 89%, 67%, and 39% of the cases. Excisional biopsy was curative for 33% of the cases. 61% of the patients received a corticotherapy, with a relapse or a recurrence in 60% of the cases, making necessary to have recourse to immunosuppressive agents (methotrexate), with a good control rate on the inflammation (75%). Finally, 76% of the patients obtained a complete resolution of their symptoms at the end of the follow-up (mean : 20 months). Conclusion Treatment lies on surgical exeresis if it is safely. Corticotherapy is frequently not sufficient. Methotrexate seems to be an interesting alternative. Recent works concentrate on Systemic IgG4 Disease, who could be a frequent cause of orbital inflammation, calling the term "idiopathic" into question, and letting us think about targeting treatments.

To describe the management of orbital exenterations and the surgical techniques for the reconstruction of orbital exenteration cavities.This retrospective study includes 56 patients who underwent orbital exenteration between 2000 and 2009. Patients' age at the time of exenteration, diagnoses, complications and reconstructive techniques were studied.Fifty-six patients - 31 male and 25 female patients - who underwent orbital exenteration between 2000 to 2009 were included in the study. The mean age was 62.5 years at the time of exenteration. Average follow-up was 23 months. The principal diagnoses were basal cell carcinoma of the eyelids (25%) and conjunctival melanoma (20%). For orbital reconstruction, 18 patients (32%) had a muscle flap, and 24 patients (43%) underwent secondary reconstruction by bone-anchored implants after spontaneous epithelialisation of the orbit. Approximately 50% of the patients received postoperative radiation therapy.Surgical reconstruction using muscle flaps may mask recurrent tumor. Epithelialisation and bone-anchored implants supporting the prosthesis allow for recurrent cancer surveillance while providing better stabilization of the prosthesis. Radiation therapy seems to render the orbit more fragile and thus less stable for implants.Orbital exenteration is a mutilating technique. Rehabilitation techniques have been improved, in particular the bone-anchored implants which allow adaptation of the prosthesis with satisfactory cosmetic results.

Objectif Le carcinome adenoide kystique de la glande lacrymale est une tumeur rare mais tres agressive par la presence tres precoce d’une invasion perineurale favorisant la rechute locale et metastatique. Tres radioresistante, elle necessite de fortes doses d’irradiation. Nous rapportons dans cette etude l’experience de l’institut Curie d’Orsay dans le traitement du carcinome adenoide kystique de la glande lacrymale. Patients et methodes Entre 2007 et 2012, neuf patients ont ete pris en charge pour un carcinome adenoide kystique de la glande lacrymale. Le traitement comprenait une chirurgie la plus large possible et une radiotherapie postoperatoire. Une seule patiente a beneficie d’une radiotherapie exclusive apres refus de la chirurgie. La radiotherapie a ete delivree exclusivement par protons a la dose de 73,8 Gy (efficacite biologique relative, EBR) (64 Gy - 75,6). Le plan de traitement comportait trois niveaux de doses : 73,8 Gy (EBR) dans le volume tumoral macroscopique (prise de contraste visible a l’imagerie), 64,6 Gy (EBR) au niveau de la glande lacrymale avec l’ensemble de la paroi laterale de l’orbite jusqu’au cone orbitaire, 54 Gy [EBR] au niveau du sinus caverneux homolateral). Resultats Tous les patients sont en vie a ce jour. La tolerance immediate du traitement a ete tres bonne avec seulement deux cas de radioepitheliite de grade 2. A deux ans, la probabilite de de controle local etait de 78 %, celle survie sans metastase de 87,5 % et celle de survie sans maladie de 78 %. Comme toxicite tardive a 2 ans, on notait seulement une radionecrose temporale avec evolution favorable sous corticotherapie. Conclusion La radiotherapie par protons exclusifs permet d’ameliorer les probabilites de survie globale ede le controle local avec une excellente tolerance aigue et tardive.

In Graves' orbitopathy, the volumetric increase of the oculomotor muscles and orbital fat leads to exophthalmia and a rise in orbital pressure. This rise in pressure may be implicated in the appearance of a compressive optic neuropathy. To investigate this increase in pressure and its variations accompanying surgical decompression, systematic measurements were taken before, during, and after every case of orbital decompression in Graves' disease.The intraorbital pressure before the surgical procedure was 14.05 mmHg ± 9.19 for a normal value estimated in the literature at 4 mmHg ± 1.5 (statistically significant difference, P0.0001). In the group presenting a compressive optic neuropathy (NO), the preoperative pressure was 26.8 mmHg ± 7.85 versus 9.8 mmHg ± 4.2 in the group without NO: the pressure was significantly higher in the group with NO (P0.001). After orbital decompression (one to three walls depending on the severity of the exophthalmia), the pressure was measured at 4.3 mmHg ± 2.53 for the entire series: 6.4 mmHg ± 2.07 in the group with NO versus 3.6 mmHg ± 2.32 in the group without NO (significant difference, P0.05). The total decrease in pressure induced by the surgery was 9.75 mmHg ± 7.55 and was significantly greater for the group with NO: reduction of 20.4 mmHg versus 6.2 mmHg for the group without NO (P0.001). The reduction in pressure was greater after collapse of the first wall (floor) than after collapse of following walls for all groups (P0.001). The maximum pressure observed during the intervention (caused by the instruments) was measured at 78.3 mmHg ± 23.47 without pupillary changes.This study shows that the intraorbital pressure is increased in Graves' orbitopathy and more in serious forms with compressive optic neuropathy. Orbital decompression, as its name indicates, provides decompression and a return to a near-normal orbital pressure situation. Compressive optic neuropathy does not result only from the direct compression of the oculomotor muscles on the optic nerve, but also from an overall rise in the pressure level within the orbital cavity.Intraorbital pressure is increased in Graves' orbitopathy, participating in the appearance of compressive optic neuropathy. Orbital decompression provides a significant reduction in intraorbital pressure.

Aging around the eyes plays a considerable role in facial aging. The demand for periocular rejuvenation is increasingly frequent. Until the past few years, eyelid esthetics was synonymous with blepharoplasty: surgery that consisted in resecting the myocutaneous excesses and reducing fatty deposits. The contribution of new medical and surgical treatments combined with new and more rigorous analysis of the esthetic disorders makes it possible to respond more precisely to patient demand and individualize surgical and/or medical treatment that is not highly aggressive and only uses products that are well tolerated, biocompatible, or resorbable.

Nasal sinus mucoceles are an uncommon cause of painful optic neuropathy. We report the case of a patient presenting an acute compressive optic neuropathy related to an anterior clinoid mucocele. The diagnosis was mainly made with CT and MRI, and the patient was treated with endoscopic endonasal transseptal surgery 10 days after the beginning of the disorder. Visual recovery was complete 3 days later. We review the literature on the etiologies of this type of pathology, the work-up, and the possible treatments.

Orbital lymphangioma is a rare vascular malformation; it is a benign but severe anomaly because of its infiltrative, diffuse, and hemorrhagic nature, and its high morbidity rate. Surgical resection is a real challenge on account of the intricate architecture of the lesion. The authors report their surgical experience concerning two cases of diffuse orbital lymphangioma whose diagnosis was established in adulthood and whose surgical treatment was successful.Two patients presented with adult orbital lymphangioma. Progression was slow during the first decade and then was quickly followed by complications: major exorbitism, compressive optic neuropathy, and corneal exposure. Neuroimaging showed a diffuse and cystic orbital malformation. Surgical resection was performed as completely as possible, in one case with a Krönlein orbitotomy and in the other case only via a conjunctive route. An aspirate drain was put in the orbit for 48 h so as to prevent dead spaces forming after resection, an essential risk factor of hemorrhagic or cystic recurrence. Systemic corticotherapy was administered for the 5 days following surgery. The resection was total in one case and subtotal in the other. The surgical follow-up was uneventful with an excellent aesthetic result and an improvement in visual acuity. After 12 months, no tumoral or hemorrhagic recurrence was noted.The surgical treatment of orbital lymphangiomas is challenging because of their infiltrative nature. In diffuse forms, a complete resection is rarely possible because of the risk of sacrificing visual function. In the two cases reported herein, the resection of the extraconal portion was complete, but the intraconal portion was completely removed only in one case. Using the aspirate drain, negative pressure was maintained in the orbital cavity, preventing the formation of chocolate cysts induced by surgery. Although the clinical result was very satisfying, long-term follow-up is necessary to evaluate recurrence.Diffuse orbital lymphangiomas can be treated successfully with a subtotal resection without clinical recurrence in the short term. These results are promising because they demonstrate that many orbital lymphangiomas can benefit from surgical treatment with an excellent esthetic and functional result.

Orbital organic foreign bodies are rare and can present different clinical features. The objective of this report is to show the danger of this type of foreign body, present the imaging data, and suggest a diagnostic approach and therapeutic management. We describe three cases of orbital organic foreign bodies with three different clinical presentations.The first case was a 43-year-old male complaining of a chronic cutaneous fistula of the inferior right eyelid lasting 4 months after an orbital trauma with a wooden object. Two surgeries were necessary to extract the foreign bodies. In the second case, a 37-year-old female with post-traumatic ptosis after a bicycle accident several months before, the imaging exams revealed a fracture of the left orbital ceiling and a superior extraconical foreign body that was removed by a neurosurgery approach. The third case, a 69-year-old male with a right orbit abscess following a trauma with a tree branch had a persistent right orbit inflammation lasting 4 months despite two drainage surgeries and an extraction of an orbital organic foreign body. A third surgery was necessary to completely extract the foreign bodies.Detecting an orbital organic foreign body is sometimes difficult, especially when the clinical history is unclear, the ophthalmologic exam reveals no abnormalities, or if the patient is referred to the hospital several months after the traumatic event. Orbital organic foreign bodies can lead to potentially serious orbital or intracranial complications. Computed tomography and orbital ultrasound sometimes do not yield an evocative pattern: for instance, in computed tomography, the low density of wood can be misdiagnosed as air. On the other hand, these foreign bodies can persist in the orbit after several surgical explorations because they can easily break or migrate. Magnetic resonance imaging is useful when an orbital organic foreign body is suspected, but the analysis is easier when clinical data are suggestive.

To report a patient with an adenocarcinoma in a pleomorphic adenoma of the lacrimal gland. Adenocarcinoma constitutes a distinct group of epithelial malignancies of the lacrimal gland.The clinical presentation, workup, surgical approach, and pathological findings were reviewed.A 56-year-old man presented with a 3-month history of a lacrimal fossa mass. This patient presented a painless mass in the upper outer eyelid with significant displacement of the globe. The tumor was localized to the lacrimal gland. Radiological investigations showed a round, well-defined lesion in the fossa of the lacrimal gland. We performed a lateral orbitotomy; en bloc resection was accomplished. The final specimen showed an adenocarcinoma in a pleomorphic adenoma. No recurrences were detected during follow-up.Although adenocarcinoma has rarely been reported in association with a pleomorphic adenoma of the lacrimal gland, this combination can exist. If the malignant tumor is limited in the pleomorphic adenoma, the prognosis appears to be better than in cases of local extension. Adjuvant therapy, radiation or others, are not useful.

Marginal zone lymphomas (MZL), clearly defined in the WHO classification of lymphoid neoplasms,1 comprise three distinct entities depending on the site of involvement: extranodal MZL or mucosa associated lymphoid tissue (MALT) type, splenic MZL (with or without villous lymphocytes) and nodal MZL. Lacrimal gland and intraorbital sites are the most frequent ophthalmological sites of MALT-type ocular adnexal lymphomas (OAL).2 We report the unusual case of a patient with bilateral intraorbital MALT-type lymphoma with MZL cells in the peripheral blood and ophthalmologic large B cell lymphoma histological transformation at the time of initial diagnosis. A 60-year-old woman with a history …

Nasal sinus mucoceles are an uncommon cause of painful optic neuropathy. We report the case of a patient presenting an acute compressive optic neuropathy related to an anterior clinoid mucocele. The diagnosis was mainly made with CT and MRI, and the patient was treated with endoscopic endonasal transseptal surgery 10 days after the beginning of the disorder. Visual recovery was complete 3 days later. We review the literature on the etiologies of this type of pathology, the work-up, and the possible treatments.

Introduction Tout examen precedant une blepharoplastie esthetique doit etre complet, afin de depister certaines situations a risque : ptosis palpebral, ptose du sourcil, malpositions palpebrales… Dans de rares cas, l’interrogatoire retrouve des antecedents d’œdeme palpebral a repetition pouvant etre un des signes d’appel du syndrome du Morbihan. Devant cet aspect clinique, on eliminera systematiquement un œdeme angioneurotique hereditaire par le dosage des differentes fractions du complement, ainsi qu’une proliferation lymphomateuse par une imagerie et un examen anatomopathologique. Materiels et Methodes Cinq patients, presentant un syndrome du Morbihan, ont ete inclus prospectivement dans le but de definir les criteres diagnostiques de ce syndrome. Les criteres cliniques etudies etaient : l’hypertrophie bilaterale des deux paupieres, l’aspect indure, la presence d’une rosacee, la duree d’evolution superieure a un an, l’intoxication alcoolo-tabagique chronique, l’effet de la corticotherapie, les antecedents allergiques, l’evolution par poussees, la cortico-sensibilite, et les autres localisations faciales associees. Les criteres anatomopathologiques etaient : l’œdeme, la mucosecretion, l’index mitotique et les autres signes de malignite. Discussion Les criteres cliniques retenus chez tous les patients etaient : des œdemes faciaux cortico-resistants, a repetition, evoluant par poussees, depuis plus d’un an, associes a une rosacee, de localisation palpebrale, inter-sourciliere et frontale. Les antecedents d’allergie ou d’intoxication alcoolo tabagique n’ont pas ete retrouves de maniere significative. L’examen anatomopathologique montrait chez l’ensemble des patients un tissu inflammatoire aspecifique sans signe de malignite. Conclusion Le syndrome du Morbihan est un syndrome associant des œdemes palpebraux, cortico-resistants, a repetition, evoluant par poussees, de facon chronique, dans un contexte de rosacee. Le traitement reside en une blepharoplastie apres avoir constate une rarefaction des poussees. L’examen anatomopathologique montre une inflammation aspecifique et elimine surtout une proliferation lymphomateuse. Le patient devra etre prevenu du risque de recidive, restant cependant rare en l’absence de nouvelles poussees.

Introduction L’imagerie medicale est couramment utilisee par les neurochirurgiens pour guider leur geste depuis plus de 10 ans. Les progres constants de la precision de l’imagerie et de la localisation spatiale des instruments, l’apparition de systemes 3D sans reperes osteo-fixes, la fusion des differentes sources d’images (TDM, IRM, echographie per-operatoire) rendent possible l’extension de ces techniques aux lesions orbitaires. Materiels et Methodes Elles sont particulierement prometteuses pour les lesions posterieures, dont la chirurgie classique comporte un risque majore de complications graves : les principales indications sont les lesions proches de l’apex et du toit de l’orbite. Resultats Nous presentons les modalites pratiques de cette nouvelle technique chirurgicale prometteuse pour la biopsie des tumeurs orbitaires, ainsi que les difficultes rencontrees. Discussion Les techniques de neuro-navigation trouvent une extension naturelle en chirurgie orbitaire : l’acces difficile aux lesions posterieures, la proximite de structures nobles fragiles, la mobilite limitee des differents elements anatomiques dans cet espace clos, sont tres similaires aux problemes neurochirurgicaux. Conclusion Nos resultats encouragent a poursuivre cette application, notamment pour developper des outils specifiques surs pour les biopsies orbitaires posterieures.

Introduction Les neuropathies optiques compliquant l’orbitopathie dysthyroidienne sont des complications pouvant mettre en jeu la fonction visuelle. Elles sont rares et peuvent etre meconnues au stade de debut avant la baisse visuelle. Leur traitement est une urgence medico-chirurgicale. Nous presentons ici a partir d’une serie retrospective de 56 yeux les elements de mauvais pronostic visuel. Objectifs et Methodes Nous avons etudie retrospectivement 300 patients pris en charge pour orbitopathie dysthyroidienne entre 1997 et 2007. 10 % d’entre eux (56 yeux) presentaient une neuropathie optique. Parmi eux 30 % ne se sont pas ameliores malgre un traitement precoce medico-chirurgical. Nous analysons ici les differents facteurs pronostiques. Resultats L’âge moyen etait de 56 ans (± 12,1). Le sex ratio a ete de 18 femmes et 11 hommes, 28 % de patients melanodermes et 86 % porteurs de facteurs de risques cardio-vasculaires. 30 % des patients atteints n’ont pas recupere leur vision malgre un traitement combinant les corticoides et la chirurgie de decompression. L’existence d’un deficit pupillaire afferent relatif, d’une amputation altitudinale du champ visuel de depart et/ou une absence de reponse au traitement par bolus de corticoides, sont ici des facteurs de mauvais pronostic. Discussion Les facteurs pronostiques des neuropathies optiques sont d’une part lies au terrain, d’autre part lies au mecanisme de la neuropathie optique. Quatre mecanismes ont ete decrits dans la litterature : compressif, pressionnel, ischemique et inflammatoire. Il semble exister d’autres facteurs correles a une evolution defavorable qui emergent de l’analyse de nos patients : l’existence d’un deficit altitudinal et l’absence d’efficacite des corticoides au moment du diagnostic suggerent qu’une composante non inflammatoire, probablement vasculaire est un facteur de mauvais pronostic. La controverse persiste quant a l’indication et le choix du traitement. Conclusion La prise en charge d’une complication neurologique de l’orbitopathie dysthyroidienne doit etre debutee en urgence. Le pronostic et l’evolution des neuropathies optiques est cependant variable selon le terrain du patient et le mecanisme de cette neuropathie. Une meilleure analyse de ces mecanismes devrait permettre d’orienter la prise en charge therapeutique et d’avoir un avis pronostique plus precoce pour chaque patient.

Introduction Etude retrospective comparant les resultats, a court et moyen terme, du traitement par lipostructure de l’enophtalmie sur cavite anophtalme dans le cadre d’un syndrome de l’œil enuclee (SOE). Materiels et Methodes Quinze patients ont ete traites sur une periode de 2 ans et classes en trois groupes selon l’importance de leur enophtalmie sur les cliches photographiques et scannographiques, moderee, moyenne ou severe. Tous les patients ont beneficie d’une injection intra-orbitaire et sus-tarsale afin de traiter l’ensemble des manifestations du SOE. Plusieurs criteres ont ete analyses : la presence ou non d’une bille intra-cavitaire, le site de prelevement de la graisse et les quantites reinjectees en fonction de l’importance de l’enophtalmie, ainsi que l’evolution post-operatoire des resultats esthetiques et scannographiques. Resultats Trois patients sur 15 presentaient une enophtalmie jugee importante, 6 presentaient une enophtalmie moyenne et 6 une enophtalmie moderee. Nous avons injecte en moyenne 3 cc de graisse en intra-orbitaire et 1,4 cc en sus-tarsal, avec des valeurs pouvant aller jusqu’a 6 cc intra-orbitaire pour des enophtalmies majeures. 5 patients sur 15 ont egalement beneficie d’une lipostructure en paupiere inferieure pour completer le traitement de l’enophtalmie. Discussion Malgre une fonte graisseuse a 6 mois evaluee a 30 %, tous les patients etaient satisfaits du resultat esthetique et aucun d’eux n’a recu de complement therapeutique secondaire. Face a l’emergence d’alternatives therapeutiques (acide hyaluronique fortement reticule, hydroxyapatite liquide), la lipostructure reste un tres bon traitement des enophtalmies sur cavites avec des resultats perenisables dans le temps. Si l’enophtalmie est trop importante, il est preferable de combler la cavite par une greffe dermo-graisseuse associee a une lipostructure sus-tarsale. Conclusion La lipostructure est une technique peu invasive trouvant une tres bonne indication dans le traitement des enophtalmies sur cavites anophtalmes.

Introduction Les hemolymphangiomes orbitaires sont des malformations vasculaires rares, certes benignes mais graves de part leur caractere infiltrant et hemorragique, et leur forte morbidite associee. Leur exerese chirurgicale est particulierement delicate en raison de l’absence de plan de clivage par rapport aux tissus sains. Les auteurs rapportent leur experience chirurgicale concernant 2 cas d’hemolymphangiomes diffus decouverts a l’âge adulte et traites avec succes. Materiels et Methodes Il s’agit d’une analyse retrospective de deux cas d’hemolymphangiomes orbitaires de l’adulte evoluant depuis la premiere decade et compliques d’un exorbitisme majeur avec neuropathie optique compressive et exposition corneenne. La realisation de biopsies a confirme le diagnostic histologique et la neuro-imagerie a mis en evidence une malformation diffuse. Une exerese chirurgicale aussi complete que possible a ete realisee par voie palpebrale anterieure, avec drainage et mise en aspiration des kystes pendant 24 heures. La resection a ete subtotale dans les deux cas. Resultats Les suites operatoires ont ete simples avec un excellent resultat esthetique et fonctionnel. A un an il n’a pas ete note de recidive tumorale. Discussion Le traitement chirurgical des hemolymphangiomes orbitaires diffus de l’adulte est rendu delicat par leur caractere infiltrant. Dans les formes diffuses l’exerese complete est rarement possible au risque de sacrifier la fonction visuelle. Dans nos deux cas la resection de la portion intra-conique de l’hemolymphangiome orbitaire a ete subtotale en revanche la portion extra-conique a pu etre retiree en totalite. Bien que le resultat ait ete tres satisfaisant tant sur le plan clinique que fonctionnel, une evaluation clinique a long terme reste indispensable afin de juger de l’absence de recidive. Conclusion Les hemolymphangiomes orbitaires diffus de l’adulte peuvent etre traites chirurgicalement par resection subtotale avec drainage et aspiration des kystes sans recidive clinique a court terme. Ces resultats sont encourageants puisqu’ils demontrent que des hemolymphangiomes orbitaires diffus gravissimes peuvent etre traites avec succes avec un excellent resultat esthetique et fonctionnel.

Introduction Les lymphangiomes orbitaires representent environ 1 % des tumeurs orbitaires. Il s’agit de malformations vasculaires benignes, potentiellement graves par les complications hemorragiques et compressives qu’elles peuvent entrainer. Leur prise en charge therapeutique est difficile. Objectifs et Methodes Les objectifs de cette etude consistent a presenter les caracteristiques cliniques ainsi que les differents moyens de prise en charge therapeutique des lymphangiomes orbitaires. Il s’agit d’une etude descriptive retrospective d’une serie de 8 enfants presentant un lymphangiome orbitaire. Les aspects clinico-radiologiques des lymphangiomes ainsi que les resultats de leurs traitements ont ete recueillis pour chaque patient. Les differentes etapes d’une procedure de sclerotherapie a l’alcool absolu sont presentees. Resultats Le diagnostic a ete effectue a la naissance pour 5 patients. L’exophtalmie (87 %), l’infiltration palpebrale (75 %) et la diplopie (25 %) ont ete les modes de revelation les plus frequents. Il y avait 6 formes diffuses et 2 formes localisees intraconiques pures. Six patients ont beneficie d’une ou plusieurs procedures de sclerotherapie permettant des ameliorations fonctionnelle et esthetique significatives. Une attitude conservatrice a ete adoptee chez 2 patients. Discussion Nous discutons de la place relative des differents moyens therapeutiques (attitude conservatrice, traitement medical, sclerotherapie, chirurgie) et confrontons nos resultats a ceux de series internationales. Conclusion Les progres realises, notamment dans le domaine de la sclerotherapie radioguidee, permettent d’esperer une diminution des sequelles fonctionnelles graves ainsi qu’un meilleur resultat esthetique dans le cadre des lymphangiomes orbitaires de l’enfant.