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[Orbital varix presenting with enophthalmos. A case report].
Abstract
We report a 23-year-old patient, presenting with enophthalmos in the left eye. The ophthalmologic examination was normal, except a 3-mm enophthalmos measured using the Hertel's exophthalmometer and an intermittent proptosis observed through the Valsalva maneuver. Orbital imaging tests disclosed a retroocular vascular lesion. A carotis arteriography was performed, and it revealed a tumor with vascular elements presumed to be orbital varix. Ultrasound Doppler examination showed a nonechogenic lesion with an increasing volume during Valsalva. Based on these findings, the tumor was diagnosed as orbital varix. We decided to manage the patient conservatively, in the absence of complications such as proptosis with corneal exposure, optic nerve compression, or esthetically unacceptable appearance. Orbital varix is a vascular anomaly, accounting for 2 % of orbital tumors. It generally presents with an intermittent exophthalmos or orbital hemorrhages. The development of enophthalmos associated with varices, such as reported in our case, is rare.
... Unilateral exophthalmos is most often due to an expansive intraorbital process, with more or less insidious development depending on the etiology. The etiologies of unilateral exophthalmos are multiple, rarely represented by an intra-or extra-conical vascular mass and representing about 10% of orbital tumors [1,2]. Orbital varixes are rare, representing 2% of orbital expansive processes and are due to proliferation and dilation of intraorbital venous elements [2,3]. ...
... The etiologies of unilateral exophthalmos are multiple, rarely represented by an intra-or extra-conical vascular mass and representing about 10% of orbital tumors [1,2]. Orbital varixes are rare, representing 2% of orbital expansive processes and are due to proliferation and dilation of intraorbital venous elements [2,3]. They are revealed by an intermittent exophthalmos aggravated by effort or the proclive position. ...
... Vascular tumors and anomalies account for 10% of orbital tumors [1,5]. Among these tumors, hemangiomas, lymphangiomas, and orbital varixes are the most common [2,6]. Orbital varixes are rare, representing 2% of orbital expansive processes. ...
The etiologies of unilateral exophthalmos are multiple, rarely represented by an intra- or extra-conical vascular mass. Orbito-palpebral varixes are rare (2% of orbital masses) and represent a main cause of unilateral intermittent exophthalmos, often of an inflammatory nature.
We report a Case of right orbito-palpebral varix in a 65-year-old adult, with no particular history, evolving for 2 years. The ophthalmological examination showed a right palpebral mass, extended to the right external canthus, with palpebral collateral circulation, moderate right ptosis with exophthalmos, non-axial, non-pulsatile, without thrill, painless, without complication, without visual deficit. A vascular mass was suspected and MRI revealed a right orbito-palpebral varix with temporal extension, confirmed by ANGIO-MRI. The latter also allowed to search for a cerebral venous malformation, an encephalocele or a bone defect, associated and also to eliminate differential diagnoses (tumor, arteriovenous fistula …). Color Doppler ultrasound in the proclive position confirmed the diagnosis of orbital varices. A preventive low-dose anticoagulant treatment was started to avoid thrombosis, with therapeutic abstention in the absence of complications. A rigorous monthly follow-up in consultation is ensured.
Orbito-palpebral varices are characterized by an extensive posterior intra-orbital character, often during their evolution and imposes a strict surveillance. In Case of complication (thrombosis, hemorrhage, pain, compressive signs of the optic nerve), surgical removal or sclerosis of the varix can be envisaged with disappointing results (recurrence, hemorrhage).
L’obésité est considérée depuis quelques années, comme un problème de santé publique majeur et a reçu le
statut «épidémie mondiale» par l’Organisation mondiale de la santé (OMS). L’obésité touche en France 6,9
millions de personnes en 2012. 550 000 personnes (1,2% de la population) souffrent d’une obésité dite
morbide. Selon la Haute Autorité de Santé (HAS), 30 529 patients ont subi une chirurgie bariatrique en
2011. Le nombre d’interventions a ainsi doublé entre 2006 et 2011. Face à une progression importante tant
sur le nombre d’actes que sur les évolutions techniques, l’objectif de ce travail est d’évaluer les enjeux
éthiques et médico-légaux de cette pratique chirurgicale à risque.
Le terme tumeurs orbitaires recouvre un large spectre de pathologies bénignes et malignes, touchant les composants orbitaires ou développées à leur contact. Elles sont découvertes de manière fortuite ou recherchées dans le bilan d’une affection générale ou devant des signes orbitaires (exophtalmie, douleurs…). Le scanner, l’IRM et l’échographie avec Doppler couleur (EDC) jouent un rôle qui varie en fonction de la présentation clinique et de la pathologie recherchée. Cet article, fruit d’une longue expérience dans un centre référent, n’a pas la volonté d’être exhaustif. Nous avons préféré aborder ces tumeurs sous l’angle de leur présentation usuelle, en insistant sur les causes les plus fréquentes et les tableaux radiocliniques qui permettent d’y penser (exophtalmie progressive ou brutale, enfant ou adulte, lésions des glandes lacrymales, lésions périorbitaires, enophtalmie). Nous détaillerons en particulier les atteintes musculaires (maladie de Basedow, tumeurs), les lésions vasculaires (hémangiome caverneux, varices, lymphangiome kystique), les lésions de l’enfant, les hématomes orbitaires. Nous proposons des protocoles d’exploration simples et des diagnostics différentiels utiles. Les références bibliographiques permettront à ceux qui veulent aller plus loin de compléter leurs connaissances dans ce domaine passionnant.
The term orbital tumor covers a wide range of benign and malignant diseases affecting specific component of the orbit or developing in contact with them. They are found incidentally or may be investigated as part of the assessment of a systemic disorder or because of orbital signs (exophthalmos, pain, etc.). Computed tomography, MRI and Color Doppler Ultrasound (CDU), play a varying role depending on the clinical presentation and the disease being investigated. This article reflects long experience in a reference center but does not claim to be exhaustive. We have chosen to consider these tumors from the perspective of their usual presentation, emphasizing the most common causes and suggestive radiological and clinical presentations (progressive or sudden-onset exophthalmos, children or adults, lacrimal gland lesions, periorbital lesions and enophthalmos). We will describe in particular muscle involvement (thyrotoxicosis and tumors), vascular lesions (cavernous sinus hemangioma, orbital varix, cystic lymphangioma), childhood lesions and orbital hematomas. We offer straightforward useful protocols for simple investigation and differential diagnosis. Readers who wish to go further to extend their knowledge in this fascinating area can refer to the references in the bibliography.
In 11 patients with blow out fracture of the orbit, measurement of orbital volume using computed tomography (CT) more than 20 days after injury correlated well with enophthalmos measured from the same scans (r = 0.87, p < 0.001, SEE 0.63 mm), with a 1 cm3 increase in orbital volume causing 0.8 mm of enophthalmos. This confirms the cause of enophthalmos after blow out fracture to be increase in orbital volume rather than fat atrophy or fibrosis. In a further 25 patients scanned within 20 days of injury the degree of enophthalmos was less marked than would be predicted from the orbital volume measurement. This was probably because of the presence of oedema, haemorrhage, or both behind the globe which would prevent immediate development of enophthalmos. CT measurement of orbital volume within 20 days of injury may predict the final degree of enophthalmos and identify those patients at risk of late enophthalmos, allowing appropriate early surgical intervention.
Enophthalmos can be defined as a relative, posterior displacement of a normal-sized globe in relation to the bony orbital margin. Non-traumatic enophthalmos has a wide variety of clinical presentations and may be the first manifestation of a number of local or systemic conditions. It may present with cosmetic problems such as deep superior sulcus, pseudoptosis or eyelid retraction; or functional problems such as diplopia or exposure keratopathy. There are three main pathogenic mechanisms: structural alterations in the bony orbit; orbital fat atrophy; and retraction. Evaluation of enophthalmos patients includes orbital imaging and a thorough ophthalmic and systemic examination. In this review, we discuss the presenting features of non-traumatic enophthalmos and include a brief description of the more important causes. An approach to the clinical evaluation of these patients is also discussed together with a brief overview of the principles of management.
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.
A case of a 65-year-old caucasian woman with progressive hemifacial atrophy (Parry-Romberg syndrome) is reported. The atrophy had begun ten years before. At time of presentation, she was suffering from facial pain and diplopia. She presented with severe enophthalmos associated with eyelid atrophy, loss of cilia and mild blepharoptosis. There was a patch of alopecia and atropic areas of skin, recognised as scleroderma "en coup de sabre". Ophthalmic examination showed miosis. Horner's syndrome and ocular motility disturbance. Biomicroscopic examination of anterior and posterior segments was normal; there was no heterochromia and no disc atrophy. Aesthetic surgical treatment was proposed. The etiology of hemifacial atrophy remains controversial, and its relations with scleroderma and autonomic nervous system disorders are discussed.
A 2-year-old female infant with a presumed diagnosis of infantile hemangioma of the left eyebrow developed acute proptosis of the left eye several days after a mild orbital trauma. Computerized axial tomography (CAT) and carotid angiography confirmed the presence of a vascularized mass in the retrobulbar space, spreading anteriorly to the orbital rim and the frontal area. Due to progression of the proptosis and the development of exposure keratitis, surgical intervention was necessary. A large vascular tumor and a massive hemorrhage occupying the orbit were removed. The pathological examination confirmed the diagnosis of a vascular malformation of the orbit, most probably orbital varices. A relatively good visual acuity was achieved after surgery by occlusion therapy.
Twenty-six cases of enophthalmos were reviewed. The causes in order of frequency were: orbital asymmetry (8); trauma (5); orbital metastasis (4); microphthalmos (2); orbital varix (2); maxillary mucocele (2); localized scleroderma (1); absence of sphenoid wing (neurofibromatosis) (1); post irradiation atrophy (1). Only six of the patients (23%) were referred with the diagnosis of enophthalmos, suggesting the sign maybe subtle and is frequently missed or misdiagnosed. The nature of the causes underscore the need for careful and thorough diagnosis. In particular, the therapeutic implications of diagnosing metastatic disease, maxillary mucocele, and orbital varices is noted. A review of etiology and mechanisms of enophthalmos point to the diversity, importance and conditions causing this sign.
A 34-year-old man with intermittent exophthalmos, found to have a large varix in the right orbit, was treated by endovascular surgery. Percutaneous transfemoral venous catheterisation and embolisation of the orbital varix was performed on two occasions. A Tracker 18 microcatheter was introduced through the right inferior petrosal sinus, cavernous sinus, superior ophthalmic vein and then into the varix, following a guidewire. Superselective venography of the right ophthalmic vein showed the varix. A total of 204 platinum microcoils was used to pack the varix. At the time of discharge, the exophthalmos had largely resolved. As this technique is much less invasive than surgical resection, we recommend an attempt at endovascular embolisation of orbital varices prior to surgical removal.
Orbital venous anomalies and lymphangiomas have been generally classified on morphologic grounds. However, conflicting concepts of these lesions have hampered scientific dialogue and confused clinicians, with occasionally serious consequences for patients. As a step toward uniform nomenclature and better communication, a consensus on the terminology of orbital vascular malformations was sought among the members of the Orbital Society.
A classification of orbital vascular malformations based on their hemodynamic relationships, as revealed by clinical and imaging features, was presented, discussed, and adopted at the 1998 Annual Meeting of the Orbital Society. A consensus statement was drafted and distributed to all members. The statement was then modified in accordance with members' responses, which included minor stylistic recommendations but no substantive dissent.
Orbital vascular malformations may be classified according to their hemodynamic relationships as no flow, venous flow, and arterial flow lesions. Assignment to each group is based on pertinent clinical and imaging criteria. Mixed forms with both venous and no flow components are grouped within the venous flow category to emphasize the clinical importance of that relationship.
A hemodynamic classification of orbital vascular malformations emphasizes features most germane to their management. It should reduce the clinical confusion resulting from purely morphologic differentiation. Widely accepted definitions may allow a better comparison of therapeutic protocols for these troublesome lesions.
To determine the incidence of specific orbital tumors based on patients referred to an ocular oncology center.
Retrospective, observational case series.
A total of 1264 consecutive patients referred to an ocular oncology service because of space-occupying orbital lesions.
A retrospective chart review was carried out for 1264 consecutive patients referred for a suspected orbital mass over a 30-year period. The lesions were grouped into general categories, as shown in "Results." The specific diagnosis in each case was based on clinical findings, computed tomography scan results, magnetic resonance imaging results, and histopathologic analysis results, when available. The number and percentage of benign and malignant tumors per age group also was determined.
The incidence of orbital tumors and pseudotumors.
Among 1264 consecutive patients, the number and percentage of lesions in each general category were as follows: cystic, 70 cases (6%); vasculogenic, 213 cases (17%); peripheral nerve lesions, 23 (2%); optic nerve and meningeal tumors, 105 (8%); fibrocytic lesions, 13 (1%); osseous and fibro-osseous tumors, 21 (2%); cartilaginous lesions, 1 (<1%); lipocytic and myxoid lesions, 64 (5%); myogenic tumors, 36 (3%); lacrimal gland lesions, 114 (9%); primary melanocytic lesions, 11 (<1%); metastatic tumors, 91 (7%); lymphoma and leukemia lesions, 130 (10%); secondary orbital tumors, 142 (11%); histiocytic lesions, 17 (1%); thyroid-related orbitopathy, 67 cases (5%); other inflammatory lesions, 133 cases (11%); and miscellaneous other lesions, 13 (1%). The most common diagnoses were: lymphoid tumor (139 cases;11%), idiopathic orbital inflammation (135 cases; 11%), cavernous hemangioma (77 cases; 6%), lymphangioma (54 cases; 4%), meningioma (53 cases; 4%), optic nerve glioma (48 cases; 4%), metastatic breast cancer (44 cases;4%), orbital extension of uveal melanoma (41 cases; 3%), capillary hemangioma (36 cases;3%), rhabdomyosarcoma (35 cases; 3%), dermolipoma (31 cases; 3%), herniated orbital fat (30 cases; 2%), dermoid cyst (26 cases; 2%), varix (26 cases; 2%), dacryops (19 cases; 2%), and other less common lesions. Of the 1264 lesions, 810 (64%) were benign and 454 (36%) were malignant. The percentage of malignant lesions was 20% in children (age range, 0-18 years), 27% in young adults and middle-aged patients (age range, 19-59 years), and 58% in older patents (age range, 60-92 years). Rhabdomyosarcoma was the most common malignancy in children, representing 3% of all orbital masses, and lymphoma was the most common malignancy in older patients, representing 10% of cases.
A variety of tumors and pseudotumors can involve the orbit. In this series of 1264 lesions, 64% were benign and 36% were malignant. The percentage of malignant tumors increased with age, with malignancies being common in older patients because of the higher incidence of lymphoma and metastasis in the elderly.
To evaluate the different therapeutic solutions for the management of periorbital hemangioma of infancy and childhood.
and methods: A retrospective study conducted between 2000 and 2006, including nine children (five girls and four boys), aged from 1 month to 9 years old. All children underwent a complete clinical evaluation, orbital ultrasonography, computerized tomography, and magnetic resonance imaging scans. The different histopathological forms and the different management options were analyzed: surgical excision, intralesional steroid injection, and oral steroids.
Surgery was performed in three cases and the results are discussed. Intralesional corticosteroid injections are effective and lead to complete reduction of tumoral volume and to the disappearance of the initial symptoms. With oral steroids, regression of the tumor was noted, followed by regrowth of the hemangioma at reduction or termination of therapy; complementary therapy such as surgery or intratumoral corticoid injection was often needed.
It therefore seems necessary to establish therapeutic processes adapted to the age of the child, the tumoral histology, the location of the tumor, as well as the clinical and functional impact, in order to offer the patient the best chances of healing, taking into account that surgery and intralesional corticotherapy remain the methods of choice in the treatment of periorbital hemangioma in infancy and childhood.
Enophthalmos is a relatively frequent and misdiagnosed clinical sign in orbital diseases. The knowledge of the different etiologies of enophthalmos and its adequate management are important, because in some cases, it could be the first sign revealing a life-threatening disease. This article provides a comprehensive review of the pathophysiology, evaluation, and management of enophthalmos. The main etiologies, such as trauma, chronic maxillary atelectasis (silent sinus syndrome), breast cancer metastasis, and orbital varix, will be discussed. Its objective is to enable the reader to recognize, assess, and treat the spectrum of disorders causing enophthalmos.