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Malignant rhabdoid tumor of the orbit.

A 36-month-old girl had a 3-week history of proptosis of the right eye. Computed tomography showed an ill-defined homogeneous mass filling the intraconal space. Histopathologic examination and immunohistochemistry findings of an incisional biopsy specimen were consistent with malignant undifferentiated tumor with rhabdoid features. Despite chemotherapy (a combination of vincristine sulfate and dactinomycin) and radiotherapy, massive orbital recurrence occurred 6 months later and orbital exenteration was performed. The recurrent tumor was composed entirely of pleomorphic epithelial cells with prominent nucleoli and many filamentous cytoplasmic inclusions. Immunohistochemical staining showed positive immunoreactivity for vimentin, cytokeratin, and epithelial membrane antigen, and negative immunoreactivity for muscle-specific antigen, melanoma, neural, and histiocytic markers. Electron microscopy excluded myogenic differentiation and showed that the filamentous cytoplasmic inclusions were composed of whorls of intermediate filaments. Aggressive chemotherapy with a combination of vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide phosphate was continued after exenteration. At 17 months' follow-up, orbital debulking surgery with externalization of the maxillary sinus was performed because of massive tumor recurrence in the right orbit and growth into the maxillary sinus. The child died 23 months after initial diagnosis from tumor invasion into the central nervous system. Extrarenal rhabdoid tumor is a rare orbital mass that carries a poor prognosis.

Antineoplastic Combined Chemotherapy Protocols↗

Brown tumor of the orbit associated with primary hyperparathyroidism.

A 70-year-old woman had swelling of her left superior orbital ridge. A tumor was surgically removed, and histologic examination disclosed a tumor containing giant cells. Subsequent tests showed primary hyperparathyroidism caused by a carcinoma of the parathyroid. This rare instance of brown tumor of the orbit led us to review the differential diagnoses of giant-cell lesions of the orbit and to examine published case reports. Laboratory determinations of serum calcium, phosphorus, and alkaline phosphatase levels for all patients with lytic orbital bone lesions can establish whether they have hyperparathyroidism, a diagnosis making orbital surgery unnecessary.

Aged↗

Imaging of orbital lymphoproliferative disorders.

Lymphomas and leukemias account for a large portion of orbital tumors. Orbital lymphoma accounts for 55% of malignant orbital tumors in adults. Idiopathic orbital inflammatory pseudotumors are pathologic entities that often challenge ophthalmologists and radiologists. This article describes the MR and CT features of orbital lymphoma, leukemia, and some other lymphoproliferative disorders.

Adult↗

Orbital tissue differentiation with standardized echography.

The standardized A-scan (Kretztechnik 7200 MA) supplemented by contact B-scan and Doppler ultrasound is a time-tested, highly reliable, and noninvasive tool for the evaluation of patients presenting with signs or symptoms suggesting an orbital lesion. Diseases of the orbital adipose tissue, extraocular muscles, optic nerves and periorbital sinuses are detectable within minutes by the experienced examiner. More than 60 types of orbital lesions can then be differentiated, localized, measured, and schematized. While the value of orbital CT scanning has been widely accepted, standardized echography is now also being recognized as an important modality in the diagnosis and management of orbital and periorbital disease. The use of standardized echography results in more frequent and more accurate tissue differentiation, specifically: (1) standardized tumor patterns closely correlate with histologic diagnoses, (2) vascular flow detection and compression testing (hard or soft), (3) detection and diagnosis of optic nerve sheath distention due to increased subarachnoid fluid (eg, benign intracranial hypertension), and (4) greater sensitivity in detecting mild extraocular muscle thickening (eg, Graves' disease). Additional advantages are the noninvasive nature of the procedure (no radiation) and portability of the equipment.

Arteriovenous Fistula↗

Surgical approaches to diseases of the orbital apex.

The authors report their experience with transcranial approaches to the orbit in situations where adequate exposure would not have been obtained with the usual orbital techniques. In addition to the standard frontal craniotomy, a supraorbital transcranial approach is described that involves removal of the superior rim and roof of the orbit, allowing exposure of all but the floor of the orbit. The main indication for this technique is a mass deep in the orbital apex that would be difficult or impossible to expose with a conventional orbitotomy. Seven representative cases are presented that illustrate conditions falling in that category.

Adult↗

Orbital exenteration for invasive skin tumours.

Orbital exenteration aims at local control of disease invading the orbit that is potentially fatal or relentlessly progressive. Of all exenterations presenting to ophthalmologists, 40-50% are required for tumours in the eyelid or periocular skin. 99% of these are basal cell carcinomas and 4-6% each are squamous cell carcinomas or sebaceous gland carcinomas. Orbital invasion results in progressive fixation of the tumour to bone and reduced ocular motility. Perineural invasion of branches of the trigeminal nerve leads to numbness or pain, and that the facial nerve, to weakness. Biopsy identifies the cell type and the presence of perineural invasion. CT and MRI scanning help in the assessment of tumour spread within the orbit. Management should be in collaboration with an oncologist. Exenteration may be total-the removal of all orbital contents-or lid-sparing if the tumour is placed posteriorly. The socket may be allowed to heal by granulation or lined with a split skin graft or local flap. Complications may be seen following 20-25% of exenterations and include fistulae, tissue necrosis, exposed bone, and infection. Incomplete clearance of tumours occurs in about 38% of total exenterations and 17% of subtotal. The overall 5-year survival is 55-65%, but significantly worse if there was perineural spread. Facial prostheses may be mounted on glasses or secured with tissue glue or osseointegrated implants. Excellent cosmetic results can be achieved but many patients prefer to wear a patch.

Basal Cell Carcinoma↗

[Is orbital exenteration indicated for extensive primary mucosal melanomas of the sinunasal system?].

Primary mucosal melanomas are most frequently localized in the sinunasal system. Orbital involvement must be excluded in the mostly advanced tumor stage. The aim of our study was to find possible indications for orbital exenteration in the T3-4 sinunasal tumor stage. We evaluated 14 treatment courses at our department from 1988 to 2004. The analysis disclosed orbital involvement in 43 % of the patients. The one patient submitted to orbital exenteration had a survival of only 28 months, which was not relevantly longer than in the other 9 patients with a T3-4 tumor stage (median survival of 27 months) or to the two patients with purely palliative treatment (survival of 10 and 21 months). We conclude that, due to the high generalized metastasis rate, radical surgical procedures can only prolong survival in individual cases of advanced-stage sinunasal tumors. Orbital decompression should be considered with reference to the quality of life.

Aged↗

Trapdoor fracture of the orbit in a pediatric population.

Orbital-floor blowout fractures of the trapdoor variety, first described by Soll and Poley, have also appeared in the French and Japanese literature. The trapdoor fracture is described as a pure orbital-floor fracture, linear in form and hinged medially, which allows herniation of orbital contents through the fracture and then entraps these herniated contents. A review of the orbital-floor fracture literature reveals a high incidence of persistent diplopia associated with ocular-muscle entrapment, which may later necessitate corrective surgery of extraocular muscles. Recent publications in the ophthalmologic literature have stressed the importance of early surgical intervention. This article reports a retrospective series of 19 pediatric patients (age range, 5 to 16 years) who presented to two institutions. All patients had radiographic confirmation of a trapdoor fracture. Physical examination demonstrated a high association between these fractures and restricted ocular motility (17 of the 19 patients). In the cases with trapdoor fracture and restricted ocular movement, early intervention was associated with better postoperative function. It is thus recommended that the symptomatic trapdoor orbit fracture be considered an urgent indication for surgical intervention. Practitioners therefore must have a high index of suspicion for these injuries. Prompt diagnosis is critical to maximize clinical outcome.

Adolescent↗

Cavernous sinus/orbital apex syndrome associated with indwelling orbital catheter use.

A 46-year-old man with a history of right orbital fractures and blindness underwent simultaneous fracture repair and enucleation with orbital implantation. During surgery, an orbital catheter was placed for administering local anesthesia to control postoperative pain. After administration of local anesthesia through the catheter on postoperative day 1, the patient had development of a complete ptosis, total ophthalmoplegia, mydriasis, vision loss from 20/20 to NLP, and hypesthesia of the V1 and V2 trigeminal nerve distribution. Intraocular pressures and dilated funduscopic examination were normal. There was no evidence of central nervous system effects or respiratory depression. After 4 hours of observation, the vision, sensation, motility, ptosis, and pupil response all returned to normal. Although rare, contralateral cavernous sinus/orbital apex syndrome may occur with indwelling orbital catheter administration of local anesthetic in an orbit with fractures.

Anesthesia, Local↗

Orbit: initial experience with surface coil spin-echo MR imaging at 1.5 T.

Fifty-nine cases in which surface coil MR imaging of the orbit was performed were reviewed. MR imaging was performed with spin-echo techniques at 1.5 T with both short repetition time/echo time (TR/TE) and long TR/TE sequences in all cases. In all patients short TR/TE images were obtained with small-diameter surface coils; long TR/TE images were usually obtained with a standard head coil. Surface coil MR appears to be an important adjunct in state-of-the-art orbital imaging. Orbital MR imaging may be most useful, providing information not available on computed tomography (CT), in identifying lesions in the orbital apex, superior orbital fissure, and optic canal; differentiating inflammatory pseudotumor from malignancy in clinically similar patients; characterizing lesions containing hemorrhage or other paramagnetic material; defining the posterior extent of optic pathway gliomas; and detecting abnormal flow in intraorbital vascular structures. CT seems to be superior to MR imaging in the evaluation of small perioptic meningiomas, especially those that are calcified.

Eye Neoplasms↗

Fine needle aspiration cytology of orbital masses.

OBJECTIVE: To review our experience with fine needle aspiration of orbital lesions. STUDY DESIGN: Over an eight-year period, 24 orbital fine needle aspiration (FNA) samples were recorded, 22 of them adequate for interpretation. RESULTS: There were two benign tumors. There were 14 malignant tumors, 5 primary, 8 metastatic and 1 malignant and originating in the nasal cavity, invading the orbit. In two cases the original tissue diagnosis was incorrect and was revised by the fine needle aspirate. There were six nonneoplastic cases. In one case an infectious agent was identified, and another showed atypical lymphoid cells. Of the two nondiagnostic cases, one was compatible clinically with an orbital pseudotumor, and the other had no available follow-up. A specific diagnosis was achieved in 18 of 24 instances, or 75% of the cases. CONCLUSION: FNA is useful in the workup of an orbital lesion, leading to prompt diagnosis and treatment.

Adult↗

0.6 T magnetic resonance imaging of the orbit.

Magnetic resonance (MR) imaging of the orbit was performed with a 0.6 T superconducting imaging system in 100 patients with normal orbits who were being evaluated for brain pathology and in 21 additional patients with a variety of orbital lesions to determine the efficacy of MR imaging in displaying orbital abnormalities. Usually, MR studies were performed using a multislice technique with multiple spin-echo pulse sequences and 30, 60, and 90 msec echo times and 500, 1500, and 2000 msec repetition times. Using section thicknesses of about 8 mm, imaging was performed in the transaxial, coronal, and sagittal projections. Pixel size was 0.9 X 1.8 mm, and the examination took about 30 min. The MR findings were compared with computed tomographic (CT) findings in all cases. Either combined axial and coronal studies of 5-mm-thick sections or a thin axial study of 1-mm-thick sections followed by reformatting techniques to obtain multiplanar images was used. Contrast enhancement was used in the CT studies. Both MR and CT clearly demonstrated the soft-tissue abnormality in all cases except two, in which MR failed to detect the abnormality. In one, MR failed to detect a small retrobulbar hemorrhage that occurred after a surgical procedure for retinal detachment. In the second case, rather extensive calcification in the posterior choroidal layers and lens was not detected by MR imaging. In several other cases, MR provided information beyond that obtained with CT. MR has the advantage of providing exquisite anatomic detail in multiplanar images, and it appears to be more sensitive than CT in detecting small, subacute and chronic hemorrhage within soft-tissue masses in the orbit and in detecting ischemia of the globe. CT is superior to MR imaging in portraying fine bone detail.

Adult↗

CT of the inferomedial orbit and the lacrimal drainage apparatus: normal and pathologic anatomy.

The normal and pathologic CT anatomy of the lacrimal drainage apparatus was examined during study of the orbits, nasal cavity, and paranasal sinuses in 100 patients with a variety of clinical complaints related to the inferomedial orbit. The bony lacrimal fossa, the nasolacrimal canal, and the fluid- or air-filled lacrimal sac and nasolacrimal duct were readily recognized in all cases. The lacrimal fossa and sac are found at the inferomedial orbit and are preseptal structures. Cystic expansion of the lacrimal sac (dacryocystitis) may mimic orbital abscess clinically; however, the radiographic recognition of a cystic, peripherally enhancing mass centered at the lacrimal fossa should exclude postseptal abscess and permit more conservative therapy. Obstruction or invasion of the drainage apparatus by tumor, infection, or posttraumatic scarring is readily depicted by CT. Sagittal/coronal images reformatted from thin transverse axial sections are often useful in defining the origin of an inferomedial orbital mass and its relation to the lacrimal sac when clinical studies and axial CT findings are equivocal.

Adult↗

Differential connections of the perirhinal and parahippocampal cortex with the orbital and medial prefrontal networks in macaque monkeys.

Previous anatomical studies indicate that the orbital and medial prefrontal cortex (OMPFC) of monkeys is organized into an "orbital" network, which appears to be related to feeding and reward, and a "medial" network, related to visceral control and emotion. In this study, we examined the connections of the orbital and medial prefrontal networks with the perirhinal (areas 35 and 36) and parahippocampal (areas TF and TH) cortex with anterograde and retrograde axonal tracers. The perirhinal cortex is reciprocally connected with orbital network areas Iapm, Iam, Ial, 13m, 13l, 12r, and 11l. In contrast, the parahippocampal cortex is reciprocally connected with the medial network, especially areas around the corpus callosum (areas 24a/b, caudal 32, and 25), and with area 11m. Projections from the parahippocampal cortex also extend to areas 10m, 10o, Iai, and rostral area 32, as well as to dorsolateral areas 9 and 46. In addition, both the perirhinal and parahippocampal cortex are reciprocally connected with areas that are intermediate between the orbital and medial networks (areas 13a, 13b, and 14c) and with the supracallosal area 24a'/b'. Outside the frontal cortex, the perirhinal cortex and the orbital prefrontal network are both interconnected with the ventral part of the temporal pole (TG), area TE and the ventral bank and fundus of the superior temporal sulcus (STS), and the dysgranular insula. In contrast, the parahippocampal cortex and the medial prefrontal network are connected with the dorsal TG, the rostral superior temporal gyrus (STG) and dorsal bank of STS, and the retrosplenial cortex.

Animals↗

Retrograde labeling of neurons in the spinal cord that project directly to the amygdala or the orbital cortex in the rat.

The amygdala and orbital cortex are thought to play an important role in the regulation of autonomic functions, hormonal secretion, and behavioral expression in response to sensory stimulation. The responsiveness of neurons in these regions to stimulation of cutaneous and visceral organs indicates that sensory information reaches the amygdala and orbital cortex. In the past, a large number of studies have thoroughly documented multiple neural pathways by which sensory information can reach these regions via relay nuclei in the brainstem and diencephalon. Recent studies reported that the amygdala and orbital cortex also receive direct input from the spinal cord. The aim of this study was to determine the magnitude and the origin of these projections in the rat. Injections of the retrograde tracer Fluoro-Gold (FG), restricted to the amygdala, labeled several hundred neurons bilaterally (60% contralateral) throughout the length of the spinal cord. More than 60% of labeled neurons were found in the lateral reticulated area of the deep dorsal horn and the gray matter surrounding the central canal. Many neurons were also found in the lateral spinal nucleus. Labeled neurons were concentrated in upper lumbar and upper cervical segments. Injections of Fluoro-Gold that were centered in the orbital cortex labeled only a small number of neurons (73% contralateral) within the spinal cord. Most labeled neurons were found in the lateral reticulated area. Neurons located in the intermediate zone and the gray matter surrounding the central canal were found mainly in upper lumbar and upper cervical segments. These findings, together with the anterograde tracing observations, provide evidence for direct projections of spinal cord neurons to the amygdala and orbital cortex. Their laminar distribution in the spinal cord and the involvement of the amygdala and orbital cortex in limbic functions suggest that these pathways may play a role in neuronal circuits that enable somatosensory information, including pain, to affect autonomic, endocrine and behavioral functions.

Amygdala↗

Early social environment interferes with the development of NADPH-diaphorase-reactive neurons in the rodent orbital prefrontal cortex.

The influence of early parental deprivation on the development of NADPH-diaphorase-(NO-synthase) reactive neuron numbers in subregions of the orbital prefrontal cortex (ventrolateral orbital, lateral orbital, and agranular insular cortex) was quantitatively investigated in the precocious lagomorph Octodon degus. Forty-five-day-old degus from three groups were compared: (1) repeated parental separation: degus that were repeatedly separated from their parents during the first three postnatal weeks and thereafter raised in undisturbed social conditions; (2) chronic isolation: degus that were raised under undisturbed social conditions until postnatal day 21, and then were reared in chronic social isolation; and (3) control: degus that were reared undisturbed in their families. Compared to the control animals the ventrolateral orbital prefrontal cortex and agranular insular cortex of the two deprived groups displayed significantly decreased density of NADPH-diaphorase-reactive neurons (down to 62% in the ventrolateral orbital prefrontal cortex of males, 70% in the agranular insular cortex, and in the lateral orbital prefrontal cortex 80% in both genders). These results confirm that early changes of social environment interferes with the development of limbic circuits, which might determine normal or pathological behaviors in later life.

Animals↗

Increased sensitivity through use of overlapping 180 degrees orbits in clinical myocardial perfusion imaging.

Clinical cardiac imaging is hindered by noise due to limited activity and imaging time. Use of 90 degrees dual-detector systems with 90 degrees gantry rotation may provide the best sensitivity/resolution for cardiac imaging, but this option is not readily available to those using a triple-detector system with detectors at 120 degrees intervals. This study utilizes a cardiac/chest phantom to compare several triple-detector orbits, with assessment of sensitivity and resolution. A 180 degrees rotation with reconstruction of two of the three heads was evaluated, resulting in overlapping 180 degrees orbits; use of a starting angle of 165 degrees for the first head placed the overlapping portion of the orbits over the LAO myocardial region, where camera-cardiac distance is most favorable. Use of this overlapping orbit yielded resolution equivalent to a conventional (single-head) 180 degrees rotation. Sensitivity was 87%-90% of that of a 90 degrees dual-detector system, and 16%-20% better than the common practice of using a 120 degrees orbit with reconstruction of 1 1/2 heads to achieve 180 degrees of data. Use of 360 degrees acquisition with reconstruction of all three heads provided the greatest sensitivity, though at the expense of some loss in image quality. Thus, for those centers performing cardiac imaging using a triple-detector system, use of overlapping 180 degrees orbits is the preferred acquisition choice.

Gamma Cameras↗

Orbital septorhaphy for the correction of baggy upper and lower eyelids.

Eyelid bags are the result of relaxation of lid structures like the skin, the orbicularis muscle, and mainly the septum, with subsequent protrusion or pseudo herniation of intraorbital fat contents. The logical treatment of baggy upper and lower eyelids should therefore include repositioning the herniated fat into the orbit and strengthening the attenuated septum in the form of a septorhaphy as a hernia repair. The preservation of orbital fat results in a more youthful appearance. The operative technique of the orbital septorhaphy is demonstrated for the upper and lower eyelid. A prospective series of 60 patients (50 upper and 90 lower blepharoplasties) with a maximum follow-up of 17 months were analyzed. Pleasing results were achieved in 56 patients. A partial recurrence was noted in 3 patients and widening of the palpebral fissure in 1 patient. Orbital septorhaphy for baggy eyelids is a rational, reliable procedure to correct the herniation of orbital fat in the upper and lower eyelids. Tightening of the orbicularis muscle and skin may be added as usual. The procedure is technically simple and without trauma to the orbital contents. The morbidity is minimal, the rate of complications is low, and the results are pleasing and reliable.

Adipose Tissue↗