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Orbital lymphangioma with intracranial extension.

We report a case of biopsy-proven orbital lymphangioma with contiguous spread intracranially in a 14-year-old boy. The patient had a history of congenital orbital lymphangioma and hydrocephalus. Multiple functional and cosmetic periorbital surgeries were done over the years. Severe unresponsive amblyopia developed along with disfiguring anterior orbital and brow invasion of tumor. Since the age of 4 years, the patient had had 12 episodes of periorbital and orbital cellulitis, often after upper respiratory infections. CT and MRI scans have shown progression of the tumor posteriorly beyond the orbital confines. The tumor, now present in the superior and inferior orbital fissure and the paranasal sinuses, extends extradurally into the middle cranial fossa. This case is unique, with documentation of the benign tumor's progressive posterior extension intracranially, along with repeated episodes of severe periorbital cellulitis.

Adolescent↗

Orbital tuberculosis.

Orbital tuberculosis (TB) is an extremely rare condition in North America. The authors present a case of TB involving the orbit, lower lid, and zygoma and review pertinent literature regarding orbital TB. Following excision of the orbital lesion, the patient responded well to anti-TB therapy. With the reemergence of TB associated with AIDS, orbital TB may be a more frequent presentation and should be considered in the differential diagnosis of inflammatory orbital disease.

Adult↗

Relations of the superficial musculoaponeurotic system to the orbit and characterization of the orbitomalar ligament.

The orbital and eyelid relationships of the superficial musculoaponeurotic system (SMAS) were studied in human cadavers. Using gross and microscopic techniques, the SMAS was found to be intimately related to the eyelids and orbicularis oculi muscle, and to have distinct orbital bony attachments. Sub-SMAS fat in the malar region was found to be continuous with the submuscular fat in the eyebrow region. The malar sub-SMAS fat continued superiorly into the lower eyelid above the inferior orbital rim, as a postorbicularis layer. A bony attachment emanating from the inferior orbital rim, the orbitomalar ligament, traveled through the orbicularis oculi muscle in a lamellar fashion prior to inserting into the dermis. The cutaneous insertion of this attachment corresponds to the malar and nasojugal skin folds. With aging, relaxation of the orbitomalar ligament allows inferior migration of orbital fat, in addition to the anterior migration that occurs through an attenuated orbital septum. These findings have implications not only in cosmetic surgery but also in the understanding of facial soft tissue changes that occur with aging.

Adult↗

Secondary orbital tumors.

The authors reviewed their experience with 524 secondary orbital tumor cases diagnosed histopathologically during 1963-1993. Secondary tumors constituted 48.0% of all orbital biopsies during the same period. There were 186 (35.5%) cases with eyelid tumors, 146 (27.9%) cases with intraocular tumors, 137 (26.1%) cases with conjunctival tumors, 26 (5.0%) cases with nasopharyngeal tumors, 22 (4.2%) cases with sinus carcinomas, 3 (0.6%) cases with intracranial meningiomas, 3 (0.6%) cases with esthesioneuroblastomas, and 1 (0.2%) case with chordoma. The three most frequent tumors making secondary orbital invasion were basal cell eyelid carcinoma (129 cases; 24.6%), squamous cell conjunctival carcinoma (125 cases; 23.8%), and retinoblastoma (123 cases; 23.5%). Squamous cell carcinoma, from various sites of origin, was the most frequent histopathologic tumor variant, accounting for 215 (41.0%) cases. Exenteration was performed on 334 (63.7%) of 524 cases with secondary orbital invasion. The ignorance of many lid and conjunctival lesions until the advanced stages, which could otherwise be easily treatable, caused blindness and life-threatening complications under our conditions. Orbital invasion from ocular retinoblastoma and uveal malignant melanoma led to a markedly poor prognosis, again related to the late presentation of many patients. Lack of health consciousness, inadequate primary surgery, and/or insufficient follow-up were the main reasons for secondary orbital invasion in many tumor cases.

Adolescent↗

Solitary fibrous tumor of the orbit.

Solitary fibrous tumor (SFT) of the orbit is a very rare lesion that may be misdiagnosed as fibrous histiocytoma, hemangiopericytoma, or other orbital tumors. We present a 62-year-old man who presented with painless proptosis, 20 years following left eye enucleation for a presumed neurofibroma. On T2-weighted magnetic resonance imaging (MRI), a hypointense tumor almost filled his entire left orbit. There was no intracranial extension. The specimen obtained at orbital exenteration was consistent with the histologic, immunohistochemical, and electron microscopic findings of SFT. The tumor was positive for vimentin and CD34 staining but negative for S-100 protein and epithelial membrane antigen. Only nine other cases of SFT of the orbit have been documented in the literature. Recognition of SFT of the orbit as a distinct pathologic entity and further follow-up of published cases are needed to determine the prognosis of this rare lesion.

Antigens, CD34↗

Interleukin-8 and monocyte chemotactic protein-1 gene expression and protein production by human orbital fibroblasts.

Orbital inflammation is common, but the mechanisms underlying leukocytic infiltration of orbital tissue are poorly understood. We studied resident human orbital fibroblasts (OF) interleukin-8 (IL-8), and monocyte chemotactic protein-1 (MCP-1) mRNA expression and protein secretion in response to lipopolysaccharide (LPS) or recombinant human cytokines that are present during inflammation. Third-passaged cultured human OF were left unstimulated or incubated with varying concentrations of LPS, recombinant interleukin-1-beta (rIL-1 beta), recombinant tumor necrosis factor-alpha (rTNF-alpha), or recombinant interferon-gamma (rIFN-gamma) for 2, 4, 8, or 24 h. Northern blot analysis and ELISA were performed to determine OFIL-8 and MCP-1 mRNA expression and protein secretion, respectively. Experiments were performed in triplicate and repeated four times on different cell lines. OF lacked constitutive IL-8 or MCP-1 gene expression, but produced substantial dose-dependent increases in steady-state IL-8 and MCP-1 mRNA expression by 2 h of LPS or cytokine stimulation (rIL-1 beta > fTNF-alpha > LPS > rIFN-gamma), maintained at 24 h ELISA for IL-8 and MCP-1 proteins showed significant time- and dose-dependent OF secretion after exposure to recombinant cytokine or LPS (rIL-1 beta > rTNF-alpha > LPS), measured after 4 h of exposure (p < 0.01). This increased in the media over the next 20 h. rIFN-gamma was a potent stimulant of OF MCP-1, significant by 2 h (p < 0.05), but only a weak stimulant of IL-8 at 24 h. OF secreted IL-8 and MCP-1 in response to LPS and proinflammatory cytokines, indicating that these resident cells within the orbit have the capacity to actively participate in the initiation and propagation of orbital inflammation. Strategies aimed at modulating local mediators may be helpful in the management of orbital inflammatory disease.

Blotting, Northern↗

Management of relative proptosis with a porous polyethylene orbital rim onlay implant.

PURPOSE: The position of the globe relative to the orbital rim plays a significant role in the relationship between the eyelids and the cornea. A prominent globe (relative proptosis) may cause eyelid retraction and exposure keratopathy. Simple horizontal lower eyelid tightening exacerbates eyelid retraction. Optimal correction with an orbital decompression or advancement of the orbital rim entails considerable risk. A technically simpler alternative, placement of an orbital rim onlay implant, was evaluated. METHODS: Fourteen patients with symptomatic relative proptosis underwent placement of a porous polyethylene orbital rim onlay implant. RESULTS: Lower eyelid position, exposure keratopathy, and ocular discomfort were improved in all patients. Two patients required minor surgical revisions. CONCLUSIONS: Porous polyethylene orbital rim onlay implants are a satisfactory option to treat the sequelae of relative proptosis.

Exophthalmos↗

Low-dose radiotherapy for lymphoid lesions of the orbit and ocular adnexa.

PURPOSE: There is no agreement within the radiation oncology and ophthalmic communities regarding the treatment of lymphoid lesions of the orbit and ocular adnexa. The authors report their experience with the use of low-dose radiation therapy for malignant and benign lymphoid masses of the orbital region in a series of 54 patients treated between 1985 and 1993. METHODS: All patients received 2 Gy per day for a total of 24 Gy, except when the lesion was extensive, in which case the therapy was 1.5 Gy per day for a total of 25.5 Gy. A diagnosis was established by incisional surgical biopsy in 26 patients and aspiration cytology in 28 patients. Those with a malignant or an indeterminate diagnosis were evaluated with a modified Ann Arbor staging system. RESULTS: Low-dose radiation therapy produced a complete response in 100% of the orbital lymphoid lesions. This local control was maintained in 52 patients (96%) for the first year and in 51 patients (95%) for 5 or more years with a mean follow-up of 7 years. One patient died of causes unrelated to the malignant lymphoma after the first year of observation. The mean age of the 54 patients was 67 years, and the range was 37 to 90 years. The mean ages of presentation for each location were: orbit, 67 years; conjunctiva, 68 years; lacrimal gland, 66 years; and eyelids, 72 years. The female-to-male ratio was 1.25:1 (34 women and 20 men). In this series, 9 patients had benign processes, 38 patients had non-Hodgkin's lymphoma, and 7 patients had abnormalities of indeterminate cause. All histologic subtypes of non-Hodgkin's lymphoma involving the orbit responded equally well to therapy. Forty-five patients had clinically staged disease as follows: stage I, 21 patients; stage II, 4 patients; stage III, 2 patients; and stage IV, 18 patients. Benign disease, diagnosed in 9 patients, was not staged. CONCLUSION: Low-dose radiation therapy proved effective in treating lymphoid lesions of the orbital area. No treatment-limiting complications occurred. The only early side effects were mild xerophthalmia and chemosis in 50% of patients, and the only chronic side effect was mild xerophthalmia in 33% of patients. Cataracts, corneal ulcerations, and retinal injury were not observed.

Adult↗

The inferomedial orbital strut: an anatomic and radiographic study.

PURPOSE: To study the anatomic and radiographic features of the inferomedial orbital strut (IOS), with particular emphasis on the region of the posterior IOS. METHODS: Cadaver dissection study of the IOS was performed on 20 orbits of 10 fresh-frozen cadaver specimens. Radiographic volumetric analysis was performed on CT scans of 20 orbits to measure the volume available for decompression in the region of the posterior IOS. RESULTS: The anterior IOS provided bony support to the orbit and served as a site of attachment for globe-supporting suspensory ligaments. The midportion of the IOS demonstrated a contiguous anatomic relation to the maxillary sinus ostium. The posterior IOS consisted of two components: the intraorbital process of the palatine bone and ethmoidal air cells that extended inferior and posterior to the IOS. Radiographic volumetric analysis demonstrated that there was an average of 2.1 mL of volume available for decompression in the region of the posterior IOS (range, 1.2 to 3.3 mL). CONCLUSIONS: The findings of this anatomic and radiographic study of the inferomedial orbital strut support the concept of a posterior inferomedial orbital decompression. The region of the posterior IOS appears to offer significant volume for axial globe mobilization while minimizing the risk of globe dystopia and/or impairment of the maxillary sinus drainage.

Anthropometry↗

Cervical carcinoma metastatic to the orbit.

PURPOSE: To describe a case of orbital metastasis from an undifferentiated pelvic tumor with probable cervical origination. METHODS: Case report. Detailed ophthalmologic examination, orbital CT and MRI, and orbital biopsy with histologic and immunohistochemical analysis. RESULTS: A 29-year-old woman with a history of untreated cervical dysplasia was hospitalized with flank pain, hematuria, and abnormal vaginal bleeding. Biopsy of a large mass, protruding from the cervix as a grape-like cluster, had cellular morphology and immunohistochemical staining patterns consistent with cervical cancer. Diplopia in downgaze developed shortly after hospitalization. Ophthalmic examination was notable for mild right lower eyelid fullness, 1.5 mm right relative proptosis, and a deficit in supraduction and infraduction of the right eye. Orbital MRI and CT revealed a 1.2 x 1.6-cm mass immediately inferior and posterior to the right globe without bone involvement. On biopsy, the mass had histology similar to that of the pelvic tumor. On metastatic workup, lesions were found involving the left femoral head, liver, and spinal column, with adjacent lymphadenopathy. Despite treatment with radiation and chemotherapy, the patient died several months later of causes related to the systemic disease. CONCLUSIONS: Orbital metastasis of cancer originating in the cervix, despite its rarity, should be considered when an orbital mass of unknown cause is present.

Adult↗

Pathways of orbital extension of extraorbital neoplasms.

Orbital involvement was demonstrated by computed tomography in 53 patients with neoplasms of the head and neck. The pathway of extension into the orbit depended on the histology and location of the tumor. Tumors of the paranasal sinuses and face usually extended through the medial wall, floor, anterior orbit, or the inferior orbital fissure. Intracranial lesions most commonly extended through the posterior lateral wall, but the superior orbital fissure and optic canal were also pathways for orbital extension.

Head and Neck Neoplasms↗

Combined frontotemporal-orbitozygomatic approach for tumors of the sphenoid wing and orbit.

An extension of a combined frontotemporal and orbitozygomatic exposure was developed to remove 8 hyperostosing invasive sphenoid wing meningiomas (Group 1) and 11 complicated intraorbital tumors with and without intracranial extension (Group 2). Two separate bone flaps were created: a free frontotemporal-sphenoidal (pterional) bone flap and en bloc removal of the superior and lateral orbital margins with attached zygomatic arch. Cranio-orbital reconstruction was performed using the inner table of the pterional bone flap. Complete tumor removal was achieved in 14 patients and near total removal in 5. There was no mortality and in those patients who did not require orbital exenteration excellent to good cosmetic results were achieved in all but one case. This approach affords a wide exposure of the orbit and anterior and middle skull base, so that large tumors of the orbit and tumors involving the orbital apex, sphenoid wing, and infratemporal and pterygopalatine fossae can be removed.

Adolescent↗

The effect of early fronto-orbital advancement on frontal sinus development and forehead aesthetics.

The frontal sinuses make an important contribution to normal forehead and glabellar contour. This study was designed to test our clinical impression that early fronto-orbital ("frontal bone") advancement could have an adverse effect on frontal sinus development and consequently on forehead aesthetics. A retrospective study was conducted on 11 patients who had undergone fronto-orbital advancement and also had a long period of follow-up at the Institute of Reconstructive Plastic Surgery at New York University. The longitudinal cephalometric data were compared with unoperated controls. With one exception, no patient who underwent bilateral fronto-orbital advancement developed a frontal sinus, and all such patients had a flattened brow contour when compared with unoperated patients, of whom 82 percent developed at least one frontal sinus. Of the three patients who underwent unilateral fronto-orbital advancement for plagiocephaly (flattened forehead), two developed a frontal sinus but only on the unoperated side and one developed bilateral frontal sinuses. The two patients with unilateral frontal sinus development had a particularly obvious deformity resulting from normal glabellar projection on the unoperated side and a flattened contour on the operated side. Fronto-orbital advancement affects forehead aesthetics and should be performed only in infant patients with moderate to severe deformities. patients with plagiocephaly whose deformity is sufficiently severe to warrant surgery should preferably undergo bilateral fronto-orbital advancement (by the technique described) rather than unilateral advancement in order to avoid the brow asymmetry that results from unilateral frontal sinus development.

Adolescent↗

Orbital floor and infraorbital rim reconstruction after total maxillectomy using a vascularized calvarial bone flap.

A number of techniques have been introduced to support the orbital floor after maxillectomy without orbital exenteration. These methods include skin graft or muscular sling, but they have resulted in severe complications, such as enophthalmos, global ptosis, diplopia, and facial deformity. Currently, advanced microvascular reconstruction using bone and soft tissue is performed by many surgeons. This usually results in the filling of the postmaxillectomy defect, but the lack of support for the orbital rim and floor by the bone flap may still cause the complications mentioned above. Vascularized calvarial bone flap was chosen in this study for reconstruction of the orbital floor and infraorbital rim to function as a buttress, to reconstruct recipient sites of poor vascular bed after radiation therapy, and to withstand further postoperative radiation. By providing a solid floor and rim, these complications can be prevented with satisfactory function and aesthetically acceptable results. From September of 1995 to July of 1998, we performed vascularized bone flap for the reconstruction of the orbital floor and infraorbital rim in four cases after total maxillectomy involving the orbital floor. With a follow-up period from 19 to 35 months (mean, 27 months), we obtained significant improvement of functional and aesthetically acceptable results without global ptosis, enophthalmos, diplopia, or severe facial contour deformity.

Aged↗

Endoscopic endonasal reconstruction of blowout fractures of the medial orbital walls.

High-resolution endoscopes and the advent of endoscopic instruments for sinus surgery currently provide surgeons with excellent endonasal visualization and access to the medial orbital walls. The purpose of this study was to demonstrate the reduction of medial orbital wall fractures through an endonasal endoscopic approach that allows the repair of the medial orbital wall fractures without an external incision. This study was a retrospective analysis of 16 patients who underwent surgical repair of medial orbital wall fractures from March of 1997 to May of 1998. The 11 male and five female patients ranged in age from 16 to 54 years (mean, 30.5 years). These patients had undergone primary reduction of medial orbital wall fractures and were observed for at least 12 months after surgery. There were no intraoperative or postoperative complications. Fifteen of 16 patients showed a complete improvement of their symptoms. One patient showed persistent diplopia, which was well managed by prisms. Endoscopic reduction of medial orbital wall fracture using an endonasal approach seems to produce good results and definite cosmetic advantages.

Adolescent↗

Endoscopic transnasal approach for the treatment of medial orbital blow-out fracture: a technique for controlling the fractured wall with a balloon catheter and Merocel.

The authors repaired a medial blow-out fracture by using an endoscopic transnasal technique with a balloon catheter and Merocel packing in 17 subjects. The follow-up periods were from 6 weeks to 2 years, and averaged 6 months. The length of the operation was 50 minutes on average. The enophthalmos was corrected in seven of the eight patients. Supporting material for the fractured medial orbital wall was kept in place for 1 to 3 weeks. The mean volume of balloon inflation was 2 cc. The result was satisfactory. No complications resulted from the transnasal endoscopic technique. This endoscopic transnasal approach allows for a better aesthetic result because it eliminates external scarring and permits a direct approach to the medial orbital wall and has a superior visualization. A balloon catheter was used to support the fractured medial orbital fracture, which was adapted, ballooned, and then visualized using a radiopaque dye (Visipaque) in 11 cases. A postoperative computed tomographic scan revealed that this is a very useful method for controlling the status of the reduced orbital wall and eliminates the possibility of complications resulting from infection. A resected uncinate process was used as a bone graft material to repair the large defect in five cases. This method provides several advantages including a mucoperiosteal attached bone graft, working in the same operative field, and cost-effective surgical time. A transnasal endoscopic technique for medial orbital fracture is also very useful for releasing entrapment of the medial rectus muscle, because it directly pushes against the fractured wall and gives good exposure of the medial orbital wall.

Adult↗

Diagnosis and management of orbital pseudotumor.

Orbital pseudotumor, also known as idiopathic orbital inflammatory syndrome (IOIS), may have protean clinical manifestations. Some presentations of IOIS may mimic common conditions such as orbital cellulitis and optic neuritis. IOIS should be considered a diagnosis of exclusion, with evaluation directed toward eliminating other causes of orbital disease. Orbital magnetic resonance imaging is the single most important diagnostic test, but serologic studies are necessary to exclude a systemic cause. Biopsy is usually not performed at presentation, as the risk of producing damage to vital structures within the orbit outweighs the benefits. Patients with multiple recurrences, or those unresponsive to therapy, should have biopsy samples taken. Corticosteroids are the mainstay of therapy and are administered for several months to ensure remission. Radiotherapy may be used in patients who fail to respond to steroids or who have a rapidly progressive course. For those patients who are refractory to both corticosteroids and radiotherapy, anecdotal reports have suggested the use of chemotherapeutic agents such as cyclophosphamide, methotrexate, and cyclosporine.

Adrenal Cortex Hormones↗

Clinical recommendations for repair of orbital facial fractures.

PURPOSE OF REVIEW: Orbital facial fractures are a common result of facial trauma. Enophthalmos, diplopia resulting from extraocular muscle dysfunction, and infraorbital nerve hypesthesia may occur. The indications and timing for fracture repair are debated. RECENT FINDINGS: The timing and treatment indications for orbital facial fractures are evolving. For orbital floor fractures, nonresolving oculocardiac reflex, the "white-eyed" blowout fracture, and early enophthalmos or hypoglobus are indications for immediate surgical repair. Surgery within 2 weeks is recommended in cases of symptomatic diplopia with positive forced ductions and evidence of orbital soft tissue entrapment on computed tomography examination or large orbital floor fractures, which may cause latent enophthalmos or hypo-ophthalmos. For midfacial, lateral, supraorbital, medial wall, and nasoethmoidal fractures, repair within 2 weeks is indicated to avoid difficult repair from immediate posttraumatic wound healing. SUMMARY: Orbital facial fracture management is germane to ophthalmologists, plastic surgeons, otolaryngologists, and others who treat patients.

Facial Bones↗