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Summary Listeria monocytogenes is a bacterial pathogen classified into distinct serovars (SVs) based on somatic and flagellar antigens. To correlate phenotype with genetic variation, we analyzed the wall teichoic acid (WTA) glycosylation genes of SV 1/2, 3 and 7 strains, which differ in decoration of the ribitol-phosphate backbone with N-acetylglucosamine (GlcNAc) and/or rhamnose. Inactivation of lmo1080 or the dTDP-l-rhamnose biosynthesis genes rmlACBD (lmo1081–1084) resulted in loss of rhamnose, whereas disruption of lmo1079 led to GlcNAc deficiency. We found that all SV 3 and 7 strains actually originate from a SV 1/2 background, as a result of small mutations in WTA rhamnosylation and/or GlcNAcylation genes. Genetic complementation of different SV 3 and 7 isolates using intact alleles fully restored a characteristic SV 1/2 WTA carbohydrate pattern, including antisera reactions and phage adsorption. Intriguingly, phage-resistant L. monocytogenes EGDe (SV 1/2a) isolates featured the same glycosylation gene mutations and were serotyped as SV 3 or 7 respectively. Again, genetic complementation restored both carbohydrate antigens and phage susceptibility. Taken together, our data demonstrate that L. monocytogenes SV 3 and 7 originate from point mutations in glycosylation genes, and we show that phage predation represents a major driving force for serovar diversification and evolution of L. monocytogenes.

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.

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.

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.

Resume Propos Nous rapportons une serie de patients explores en medecine interne pour une inflammation orbitaire et decrivons la forme idiopathique appelee syndrome d’inflammation orbitaire idiopathique (SIOI). Patients et methodes Quarante patients etaient adresses consecutivement par un centre specialise ou une biopsie orbitaire etait realisee en cas de lesion accessible. Onze patients avaient ete exclus en cas de dossiers incomplets ou en raison de lymphome non hodgkinien, de xanthogranulomatose periorbitaire ou de maladie de Basedow. Etaient inclus les patients ayant une maladie systemique ou auto-immune (MS/AI) validant les criteres internationaux, ou un SIOI en l’absence d’etiologie. Les caracteristiques anatomocliniques et immunologiques des patients ayant un SIOI, leur traitement et leurs profils evolutifs sont rapportes selon les formes histologiques decrites par Mombaerts. Resultats Parmi les 29 patients inclus, huit avaient une dacryoadenite revelatrice de MS/AI, principalement une vascularite necrosante chez cinq patients, sept avaient un SIOI presume et 14 un SIOI histologiquement documente. La presentation du SIOI etait dominee par une atteinte diffuse de l’orbite. Le SIOI beneficiait d’une corticotherapie seule ou recevait en plus un immunosuppresseur dans 57 et 24 % des cas, respectivement. L’incidence de la rechute/resistance etait superieure a celle de la remission et en particulier, en cas de SIOI presume ou de sa forme classique. Les patients ayant un stade III-IV de Chisholm etaient tous en rechute ou resistants. Conclusion Une dacryoadenite peut reveler certaines MS/AI. La forme sclerosante du SIOI n’est peut-etre pas un facteur de rechute ou de resistance, contrairement a la sialadenite severe.

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 Introduction La myasthenie est une pathologie de la jonction neuromusculaire pouvant mettre en jeu le pronostic vital et presentant un grand polymorphisme clinique a l’origine d’un diagnostic souvent difficile. Patients et methode Nous presentons une serie de 15 patients, suivis a la Fondation ophtalmologique de Rothschild entre 2002 et 2007 afin d’illustrer la diversite clinique de cette pathologie. Tous les patients ont consulte au depart en ophtalmologie pour des symptomes oculaires ayant secondairement abouti au diagnostic de myasthenie. Resultats Lors du diagnostic de la maladie, 11 patients sur 15 presentaient un ptosis associe a une diplopie, deux patients sur 15 presentaient un ptosis isole et deux patients presentaient une diplopie isolee. Le bilan de myasthenie a permis la decouverte d’un thymome malin chez trois patients, et d’une hyperplasie thymique chez un patient. Une association auto-immune a ete trouvee chez deux patients: le premier presentait une thyroidite de Hashimoto et le second a developpe une neuromyelite optique quelques annees apres sa myasthenie. Deux patients ont secondairement developpe une myasthenie generalisee. Discussion Notre serie de patients presente un faible taux de generalisation compare au donnees de la litteratures qui s’accordent pour dire que deux tiers des patients generalisent leur myasthenie a point de depart oculaire dans les deux a trois ans suivant le diagnostic. Cela pourrait s’expliquer par la prise en charge precoce de ces patients, et la mise en place frequente d’un traitement immunomodulateur, diminuant le risque de generalisation selon certaines etudes. Conclusion Malgre le faible nombre de patients, cette etude met bien en evidence la diversite clinique de cette pathologie et les risques qu’elle peut engendrer. Une collaboration etroite entre ophtalmologistes et neurologues est necessaire pour permettre une prise en charge ideale de ces patients.

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.

Resume Les exophtalmies endocriniennes surviennent dans un contexte de dysthyroidie (hyperthyroidie 80 %, euthyroidie 10 %, hypothyroidie 10 %). Les atteintes sont le plus souvent minimes ou moderees ; il s’agit rarement de formes severes. Les formes moderees et severes evoluent en deux stades : un stade inflammatoire suivi d’un stade sequellaire. L’exophtalmie est souvent consecutive a la mise en route d’un traitement antithyroidien, en particulier l’iode 131. L’evolution se fait par une phase dynamique avec des poussees, des remissions suivies d’une stabilisation des lesions. L’exophtalmie est stable dans 70 % des cas et regresse au maximum dans 30 % des cas. La maladie va evoluer de facon chronique sur 2 a 3 ans avec une amelioration tres lente, sans retour a l’etat anterieur. Toute exophtalmie de type endocrinien necessite un bilan biologique avec dosage de T3, T4 et TSH, un bilan ophtalmologique complet, notamment une etude de la tension oculaire et du nerf optique (vision des couleurs, champ visuel et fond d’œil), et enfin une imagerie (scanner et IRM). Le traitement comporte un retour a l’equilibre thyroidien (Neomercazole®), iode radioactif, thyroidectomie totale. Le traitement de l’exophtalmie proprement dit passe par la corticotherapie en phase inflammatoire, par une eventuelle radiotherapie dans les formes cortico-resistantes et en cas de contre-indication a celle-ci. La chirurgie est indiquee au stade sequellaire, rarement au stade inflammatoire. Les resultats aussi bien fonctionnels qu’esthetiques dependent de nombreux criteres.

Emergencies in childhood orbital tumorals are rare. The absolute emergency involves malignant primary orbital tumors, such as rhabdomyosarcoma or secondary malignant tumors (metastatic neuroblastoma, leukemia), involving a vital prognosis requiring prompt diagnosis. Delayed emergencies are usually vascular lesions. Among these lesions, immature orbital hemangioma, with a good prognosis, must be distinguished from orbital adnexal lymphangiomas, which are less frequent but can lead to dramatic cosmetic and functional disorders. In rare cases, they can be responsible for sudden, painful proptosis, due to orbital hemorrhage, with a risk of optic nerve compression, requiring emergency surgical treatment. Neurogenous lesions, either isolated, such as in gliomas, or associated with a systemic disease, such as Recklinghausen neurofibromatosis, threaten the functional prognosis. Diagnosis of pediatric orbital tumors is based on a good clinical examination, precise imaging investigations, and evaluation of the locoregional extension of the tumor. Biopsy is required in emergency situations, when rhabdomyosarcoma is suspected, in order to start the chemotherapy. However, the biopsy can be superfluous, and even useless or dangerous, when clinical and imaging investigations are sufficient to provide a diagnosis of capillary hemangioma, lymphangioma, or metastatic tumor from an abdominal malignancy. Treatment is closely related to the etiopathogenesis of the tumor. The outcomes are vital, functional and cosmetic. They may require orbital surgery (biopsy, tumoral resection, orbital decompression in case of a compressive hemorrhage), systemic corticotherapy (as in immature adnexal hemangioma), radiation, and chemotherapy (rhabdomyosarcoma, secondary malignant tumor). These diseases require a pediatric ophthalmological medical center specializing in orbital surgery, with close collaboration of multiple specialists such as onco-pediatricians and neurosurgeons.

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.

Introduction Les troubles oculomoteurs sont presents dans 40 % des cas d’orbitopathie dysthyroidienne. A la phase sequellaire, l’hypertrophie fibreuse des muscles oculomoteurs est responsable d’une myopathie restrictive. Nous rapportons notre experience de prise en charge chirurgicale de ces patients. Patients et methodes Nous presentons une etude retrospective portant sur 42 patients operes entre septembre 1998 et avril 2003. Tous les patients ont eu un bilan complet ophtalmologique et orthoptique. La chirurgie a ete proposee dans les cas de diplopie stable depuis au moins 6 mois avec une orbitopathie stable depuis au moins 6 mois. Un recul musculaire a ete realise dans tous les cas, sous anesthesie generale en cas de fibrose clinique, sous anesthesie topique potentialisee avec chirurgie ajustable dans les cas de fibrose moderee. La suppression de la diplopie en position primaire et dans les differents champs du regard a ete evaluee. Resultats Quarante-deux patients, dont 28 femmes et 14 hommes, d’âge moyen 51 ans (39 a 75 ans), ont ete inclus dans cette etude. Le recul moyen est de 48 mois (10 mois a 6 ans). Vingt-six patients presentaient une diplopie verticale, 11 une diplopie horizontale, 5 une diplopie mixte. Ces derniers ont ete operes en deux temps, en commencant par le temps vertical. Sous anesthesie generale, le recul musculaire a ete adapte aux tests de duction forcee et d’elongation musculaire. Nous avons obtenu 94 % de bons resultats dans les formes verticales, 81 % dans les formes horizontales, les resultats incomplets etaient des sous-corrections. Sous anesthesie topique, le recul musculaire a ete ajuste en peroperatoire avec la participation active du patient. Un bon resultat a ete note chez tous les patients. Conclusion Un bon resultat chirurgical depend de l’evaluation precise des muscles responsables de la restriction. Nous insistons sur l’importance des tests de duction forcee et d’elongation musculaire dans les cas restrictifs et sur l’utilite de la chirurgie ajustable dans les formes a fibrose moderee.

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

Les ptosis lies a l’âge s’expliquent par des dehiscences et desinsertions du faisceau aponevrotique du releveur. Ils ont des caracteristiques cliniques bien precises : une ptose mineure ; une bonne fonction du releveur ; un deroulement excessif de la paupiere vers le bas ; un pli haut situe ; une pathologie liee a l’âge de type dermatochalasis, entropion, ectropion ; et aucune anomalie oculomotrice, ce qui les differencie des ptosis myopathiques. Ils repondent parfaitement au traitement chirurgical, qu’il s’agisse d’une chirurgie aponevrotique par voie anterieure associee a une blepharoplastie ou d’une chirurgie mullerienne par voie conjonctivale.

Optic nerve sheath meningiomas are challenging lesions to manage. We report here a case of primary optic nerve sheath meningioma in a pregnant woman with sudden unilateral vision loss. Then we review the current literature on the subject, prognosis factors, and report the results of fractionated radiotherapy and current therapeutic guidelines. Pregnancy may accelerate growth of meningiomas and this diagnosis must be considered in all cases of optic neuropathy in pregnant woman.

Objectif Ce travail a consiste a modeliser la cavite orbitaire et les principaux elements constitutifs des paupieres en utilisant les ressources de la conception tridimensionnelle assistee par ordinateur. Methode Deux logiciels, Amapi Designer 7 et Carrara 3 Studio (Eovia, Mountain View, Etats-Unis), ont ete utilises pour obtenir les modeles. La synthese des images a necessite plusieurs etapes : la modelisation tridimensionnelle de la surface du crâne et la mise en place des principales structures palpebrales en utilisant des surfaces parametriques ou polyedriques, la representation de la vascularisation de l’orbite en nous aidant des reperes osseux et musculaires intra-orbitaires, le plaquage d’une texture sur les differents elements anatomiques (choix arbitraire d’une couleur), l’eclairage, le cadrage de la scene (choix de l’angle de prise de vue pour le rendu), le rendu (creation d’un support numerique). Resultats Nous avons illustre trois techniques chirurgicales. Nous avons modelise deux techniques classiquement decrites dans la chirurgie du ptosis (reinsertion du muscle releveur de la paupiere superieure et resection conjonctivo-mullerienne) et avons detaille la voie d’abord transconjonctivale inferieure (utilisee dans le traitement des lipoptoses palpebrales et dans l’abord du plancher orbitaire). Conclusion La modelisation 3D assistee par ordinateur permet d’obtenir des representations graphiques realistes et didactiques de structures anatomiques complexes comme l’orbite et son contenu. La qualite des images obtenues et la possibilite de representer les etapes importantes d’une technique chirurgicale en font un outil pedagogique interessant et prometteur.

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.

This review covers the techniques of reconstruction of large full-thickness defects of the lower eyelid. The authors describe tree cases of basal cell carcinoma of the lower eyelid, with large excision of the tumor. We excised the tumor and examined the edges with an extemporaneous histological exam. Reconstruction combined grafts and flaps. The advantages and disadvantages of the various techniques of lower-eyelid reconstruction are discussed.

Resume L'appareillage d'une cavite est un acte medical permettant a des patients anophtalmes de reprendre une vie active. Il necessite une collaboration etroite entre le chirurgien et l'oculariste. Une cavite de bonne qualite doit assurer le bon maintien de la prothese oculaire. Sa refection repose sur la restitution d'un bon volume orbitaire, puis sur la prise en charge des pathologies du sac conjonctival. Les anomalies palpebrales sont corrigees en dernier, une fois la prothese en place. Les epitheses osteo-integrees sont utilisees en dernier recours. Ces epitheses permettent d'obtenir un resultat esthetique de tres bonne qualite, tout en simplifiant considerablement le geste chirurgical. Devant toute anophtalmie congenitale, la consultation aupres d'un oculariste est une etape indispensable avant d'envisager tout traitement chirurgical. La chirurgie ne sera reservee qu'aux cas de protheses instables, aux echecs du traitement par conformateur (protheses expansives, expansion orbitaire par osteotomies) et au traitement des malpositions palpebrales apres pose de la prothese definitive.

Blepharoplasty complications are infrequent, most often minor and transitory, rarely major and permanent with functional or esthetic consequences. Treatment is above all preventive: screening at risk patients with a history of ophthalmic problems, but also general illnesses that would contraindicate blepharoplasty. Patients must be informed of possible risks through informative booklets with the most important points underlined. Complications can be purely ophthalmological, the more serious sequelae being partial or complete visual loss due to ischemic optical neuropathy, with very poor prognosis, or more rarely compression of the ocular globe by intraorbital hemorrhage, which has a better prognosis provided the origins are quickly recognized and treated immediately. Other visual complications include oculomotor problems, keratoconjunctivitis sicca, epiphora, and chemosis of lymphatic origin. Eyelid complications are more frequent: ptosis on the upper eyelid or lagophthalmia caused by incorrect resection of the skin, scarring and eyelid fold anomalies. The most serious esthetic complication is the malposition of the lower eyelid, which can manifest as retraction, lagophthalmia, ectropion, deformation of the external canthus, or lower eyelid tissue relaxation. These malpositions are quite often minor, sometimes reversible, but at times major, with psychological, esthetic and functional consequences that are difficult for the patient. Other local complications also arise: enophthalmia with a sunken lid, as well as under- and overcorrection. General complications can include scarring related to pigmentation problems and residual hematomas, and exceptionally infections going as far as the orbital fat tissue. Finally, other complications are related to new laser surgical techniques that are responsible for ectropion of the lower eyelid and even burns or residual redness, or complications related to periocular injections of filling material. A comprehensive review of prevention, diagnosis, and management of complications after blepharoplasty is presented.

Resume L'ophtalmologie est une discipline de plus en plus hautement technologique, beneficiant sans cesse des progres de l'imagerie et des systemes informatises. Cependant, comme dans toute discipline medicale, l'examen du patient doit toujours commencer par un examen clinique rigoureux, systematique, bilateral et comparatif, apres un interrogatoire precis et concis.

In the case of aged patients with chronic facial palsy with severe muscular atrophy, muscle transposition is a good alternative in order to supply some form of movement. The masseter muscle may be transposed through either an intra- or extraoral approach. In the extraoral technique, most of the surgical details have been well documented, except the relationships between the masseter muscle and the parotid duct. Considering the anatomic direction of the parotid duct, a perfect axis of traction is available in order to lift both lips and the buccal commissure. Ten cases of extraoral masseter muscle transposition above the parotid duct were analyzed. Complete facial paralysis resulted from tumor extirpation (seven cases), arteriovenous malformation (two cases) or trauma (one case). The patients’ ages ranged from 47 to 76 years with a median age of 60 years; seven patients were female and three male. The duration of the palsy prior to the muscle transposition, ranged from 8 months to 18 years. The overall results were satisfactory in nine cases. Extraoral masseter muscle transposition is particularly attractive as a primary procedure, because of its efficiency in elderly patients with long-standing facial palsy. As a secondary procedure, masseter transposition may be considered as good an alternative for rehabilitation, when other reconstructive techniques have failed.

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.

La plupart des patients atteints d’une orbitopathie dysthyroidienne presentent une forme peu active qui ne necessite pas de traitement agressif. Des mesures simples sont suffisantes pour pallier l’inconfort visuel : le port de verres teintes, une hygiene parfaite des paupieres et l’utilisation d’agents mouillants ou de gels. Les prismes sont utiles en cas de diplopie. L’arret du tabac est une mesure necessaire. Les formes severes touchent 10 % des patients. La prise en charge est longue, difficile et les resultats parfois decevants. La corticotherapie et la radiotherapie orbitaire sont les deux principaux traitements utilises. La corticotherapie est le plus souvent prolongee plusieurs mois, a fortes doses par voie orale ou intraveineuse. Elle est indiquee dans les formes inflammatoires severes, dans les formes compliquees de neuropathie optique, d’hypertonie oculaire ou d’exposition corneenne. L’association concomitante corticotherapie/radiotherapie permet de reduire la duree des traitements.

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.

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.

To describe a case series of patients investigated in internal medicine for an inflammation of the orbit and to clarify the clinical and pathological features of patients with idiopathic orbital inflammatory syndrome (IOIS).Forty patients were consecutively referred by a specialized center where an orbital biopsy was performed in case of accessible lesion. Eleven patients were excluded because of missing data or diagnosis of lymphomas, periorbital xanthogranulomas, or Graves' disease. Patients with systemic disease (SD) or auto-immune disorder (AID) that validated the international criteria, or those having an IOIS in the absence of local or systemic etiology, were included. The clinicopathologic and immunologic characteristics of IOIS patients, their treatment and their evolutionary profiles are reported according to the histological types described by Mombaerts.Of the 29 patients enrolled, eight had a dacryoadenitis revealing a SD/AID, mainly a necrotizing vasculitis, seven patients had a presumed IOIS and 14 an IOIS histologically documented. The presentation of IOIS was dominated by a diffuse involvement of the orbit. Corticosteroids were administered alone or with an immunosuppressant in 57 and 24% of IOIS patients, respectively. The incidence of relapse/resistance was higher than that of remission, particularly in case of presumed IOIS or in its classical form. All four patients with a stage III-IV of Chisholm were relapsing or resistant.A dacryoadenitis may reveal some types of SD/AID. Unlike the severe sialadenitis, the form of sclerosing IOIS may not be a factor associated with relapse or resistance.

myasthenia gravis is a neuromuscular junction disorder that can jeopardize the patient's life and has a high clinical polymorphism that makes it difficult to diagnose.after reviewing the disease physiology, its clinical symptoms, and the different means to diagnose and treat it, we present a 15-patient series that we cared for at the Rothschild ophthalmologic foundation from 2002 to 2007 for myasthenia gravis that began with isolated ocular symptoms, so as to highlight the clinical diversity of this pathology.when the disease was diagnosed, 11 patients out of 15 had a ptosis with diplopia, two had an isolated ptosis, and two had isolated diplopia. After investigations, we discovered that three patients had a malignant thymoma and one had thymic hyperplasia. An autoimmune disease association was found in two patients: the first one had Hashimoto thyroiditis and the second one developed optical neuromyelitis a few years after his myasthenia gravis. Only three patients secondarily developed a generalized myasthenia gravis.our series of patients has a low disease generalization rate in comparison with the published data in the medical literature, indicating that two-thirds of patients with ocular myasthenia gravis should develop generalized myasthenia gravis within approximately 2 years after the beginning of their illness. This could be explained by the early consultation of these patients and the common prescription of an immunosuppressive therapy, reducing the risk of secondarily generalized myasthenia gravis according to some studies.despite the small number of patients, this study underlines the clinical polymorphism of ocular myasthenia gravis and the risks it may cause. Close collaboration between ophthalmologists and neurologists is needed to ensure good care for these patients.

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.

Dysthyroid orbitopathy is a disease that is edematous and inflammatory, generally chronic, sometimes subacute or acute, and is characterized by exophthalmos in the majority of cases, retraction of the lid, restrictive strabismus with diplopia, corneal ulceration, ocular hypertension, and compressive optic neuropathy. Proptosis is the cardinal sign. Exophthalmos is frequently axial and is bilateral in 85%-90% of cases. Displacement of the globe in the vertical and horizontal axes is not rare, particularly when intense enlargement of an extraocular muscle produces a mass effect. Proptosis stems from a conflict for the space in the orbital cavity. The infiltration of the fat, muscles, and lacrimal gland by lymphocytes, plasma cells, and mucopolysaccharides, which are very hydrophilic, all contribute to the orbitopathy. The majority of patients with minimal exophthalmos do not require special treatment since they tend to improve spontaneously. In severe forms, it is important to evaluate the activity for steroid use to eliminate the inflammation in the soft tissues and to make rehabilitative surgery possible under better conditions. When a good response is not obtained, radiotherapy is evaluated. The surgical treatment by bone orbital decompression and sometimes lipectomy is indicated in inactive forms, disfiguring exophthalmos, certain particular cases such as dysthyroid optic neuropathy, ocular hypertension, corneal exposition.

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.

Emergencies in childhood orbital tumorals are rare. The absolute emergency involves malignant primary orbital tumors, such as rhabdomyosarcoma or secondary malignant tumors (metastatic neuroblastoma, leukemia), involving a vital prognosis requiring prompt diagnosis. Delayed emergencies are usually vascular lesions. Among these lesions, immature orbital hemangioma, with a good prognosis, must be distinguished from orbital adnexal lymphangiomas, which are less frequent but can lead to dramatic cosmetic and functional disorders. In rare cases, they can be responsible for sudden, painful proptosis, due to orbital hemorrhage, with a risk of optic nerve compression, requiring emergency surgical treatment. Neurogenous lesions, either isolated, such as in gliomas, or associated with a systemic disease, such as Recklinghausen neurofibromatosis, threaten the functional prognosis. Diagnosis of pediatric orbital tumors is based on a good clinical examination, precise imaging investigations, and evaluation of the locoregional extension of the tumor. Biopsy is required in emergency situations, when rhabdomyosarcoma is suspected, in order to start the chemotherapy. However, the biopsy can be superfluous, and even useless or dangerous, when clinical and imaging investigations are sufficient to provide a diagnosis of capillary hemangioma, lymphangioma, or metastatic tumor from an abdominal malignancy. Treatment is closely related to the etiopathogenesis of the tumor. The outcomes are vital, functional and cosmetic. They may require orbital surgery (biopsy, tumoral resection, orbital decompression in case of a compressive hemorrhage), systemic corticotherapy (as in immature adnexal hemangioma), radiation, and chemotherapy (rhabdomyosarcoma, secondary malignant tumor). These diseases require a pediatric ophthalmological medical center specializing in orbital surgery, with close collaboration of multiple specialists such as onco-pediatricians and neurosurgeons.

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.

Aponeurotic defects and disinsertion of the levator aponeurosis are responsible for acquired involutional ptosis. The typical clinical findings in aponeurotic defects are high or moderate ptosis, good to excellent levator function, thinning of the eyelid above the tarsus, high to absent lid crease, and normal Müller's muscle function. The purpose of the treatment is to repair the defect or to advance the aponeurosis on the tarsus. This surgery can be done via the anterior approach with aponeurotic surgery or via the posterior approach with Müller's muscle conjunctival resection. In all cases, upper lid blepharoplasty is down.

Optic nerve sheath meningiomas are challenging lesions to manage. We report here a case of primary optic nerve sheath meningioma in a pregnant woman with sudden unilateral vision loss. Then we review the current literature on the subject, prognosis factors, and report the results of fractionated radiotherapy and current therapeutic guidelines. Pregnancy may accelerate growth of meningiomas and this diagnosis must be considered in all cases of optic neuropathy in pregnant woman.

To develop a virtual model of the orbital cavity and the eyelids using a three-dimensional model.Amapi Designer 7 and Carrara 3 Studio (Eovia, Mountain View, CA, USA) software were used to obtain 3D models of the orbital cavity and the eyelids. Three-dimensional creation is divided into two main processes. In the modeling process, a 3D model of the orbit, the arterial supply, and the eyelids are created using polygonal meshes and surgical tools with parametric surfaces. In the rendering process, colors are arbitrarily chosen to make elements in the model easier to read. A virtual camera is adjusted and light sources are created to produce virtual simulations of the orbit and the eyelids.Surgical techniques were illustrated with 3D models. Ptosis surgery (resection of the levator muscle and Müller's muscle conjunctival resection) and the transconjunctival approach to lower eyelid blepharoplasty were described step by step.Three-dimensional models have applications in many medical fields, providing representation of anatomical structures. They can be used to depict the anatomy of the orbital region in a realistic way. Three-dimensional computer-generated images are an aid in teaching residents in orbital anatomy and surgery.

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.

This review covers the techniques of reconstruction of large full-thickness defects of the lower eyelid. The authors describe tree cases of basal cell carcinoma of the lower eyelid, with large excision of the tumor. We excised the tumor and examined the edges with an extemporaneous histological exam. Reconstruction combined grafts and flaps. The advantages and disadvantages of the various techniques of lower-eyelid reconstruction are discussed.

The majority of Graves' patients have mild and nonprogressive ocular involvement that does not require aggressive treatment. Local supportive measures such as wearing tinted lenses, proper eyelid hygiene, and the use of wetting agents or gels are usually sufficient to obtain symptomatic relief until eye disease becomes inactive. Smoking must be stopped and euthyroidism controlled. Management of severe forms (10% of patients) is often difficult and does not provide consistently favorable results. The use of glucocorticoids is a well-established method of treatment, indicated in severe inflammatory forms as well as in complicated forms of optic neuropathy, ocular hypertension, and corneal exposure, and are used orally or intravenously, at high doses and for prolonged periods of time. Prisms are useful in cases of diplopia. Favorable effects are reported in slightly more than 60% of cases. Glucocorticoids can be combined with orbital radiotherapy in patients with severe orbitopathy.

Orbital bony decompression has a definite place in the management of severe manifestations of dysthyroid orbitopathy such as optic neuropathy, exposure keratitis, and exophthalmos. This surgical procedure can be undertaken when medication and radiation therapy fail. Esthetic and functional results are globally satisfying and explain the interest taken in this technique. This article will describe the development of this procedure and will outline the surgical techniques and their complications. It emphasizes the multifactor indications for this procedure.

During upper blepharoplasty, myocutaneous excess and fat pads are treated using an anterior approach. Eyelid malpositions such as involutional ptosis or lid retraction could be associated and should be treated with associated procedures. Aponeurotic surgery on the levator muscle can make use of the same anterior approach, with the major difficulty of dosage. In cases of ptosis with a positive epinephrine test or minor muscular retraction, the Müller muscle-conjunctival surgery via a posterior approach seems to be more reproducible. Double-approach techniques are described.

After reviewing the general principles of eyelid reconstruction, the authors present reconstruction techniques with regard to the location and size of the eyelid defect. When the defect is less than one-quarter of lid length, direct suture is possible. When the defect is larger, reconstruction techniques differ for the upper and lower lid.

Steinert myopathic dystrophy is a generalized, hereditary disease with bone, muscular, heart and ocular involvement.This is a retrospective study of nine patients with significant blepharoptosis due to Steinert disease, who were treated at the Adolphe de Rothschild Ophthalmology Foundation over a period of 5 years. Ptosis was symmetric and major in all cases with poor levator excursion. Severity criteria were an absence of the Bell phenomenon and diminished orbicularis tone. A frontalis suspension was performed in eight cases with intentional undercorrection. The outcome was favorable in all cases, 2 with a slight overcorrection underwent a second operationconclusion: Surgical treatment of ptosis in Steinert disease is difficult because of a risk of lagophthalmic, keratopathy due to the severity of the disease, an absence of the Bell phenomenon and ophthalmoplegia. This surgery must be undertaken with caution, most often using a frontalis suspension. Undercorrection must be systematic, with the single goal of freeing the pupil in the primary position.

To determine the mechanisms and treatment of ocular hypertension in patients with thyroid-associated orbitopathy and to differenciate it from glaucomatous damage.Three case reports.Retrospective review of clinical findings, course, and treatment of the three patients.Elevated intraocular pressure in thyroid-associated orbitopathy observed in the three cases may involve different physiopathological abnormalities such as disturbances of venous circulation, compression by infiltrative muscles, and long corticosteroid use. In the first two cases, defects demonstrated in the perimetry are in consistent with glaucomatous damage. In the third case, visual field abnormalities may be compatible with a glaucomatous disease, but all defects resolved after therapy. Treatement was of the greatest difficulty for the three cases, associating antiglaucomatous medication, steroids, orbital radiotherapy, orbital decompression and extraocular muscle surgery. Intraocular pressure was controlled in all cases.Elevated intraocular pressure in thyroid-associated orbitopathy is distinguished from glaucomatous disease by its physiopathological mechanisms, clinical course, visual field defects, and treatment. The management of this hypertension is closely related to the treatment of dysthyroid orbitopathy.