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Criteria for nonsurgical management of subperiosteal abscess of the orbit: analysis of outcomes 1988-1998.

PURPOSE: On the basis of bacteriologic studies, we have recommended expectant observation with intravenous antibiotics for subperiosteal abscess (SPA) of the orbit in patients less than 9 years of age, given the absence of eight other specific surgical criteria. We attempted to test these recommendations with a prospective study. STUDY DESIGN: Prospective noncomparative case series. PARTICIPANTS: Forty patients younger than 9 years of age treated for orbital SPAs at the Childrens Hospital of Wisconsin from 1988 to 1998. METHODS: Specific management criteria were applied to a cohort of 37 orbital SPA patients. Three other orbital SPA patients under the age of 9, either ineligible for medical therapy or treated outside our guidelines, were also studied. Clinical data for all patients were reviewed and analyzed. MAIN OUTCOME MEASURES: Clinical resolution of the abscess, as evidenced by normal visual acuity, pupillary examination, motility, and globe position on the affected side. RESULTS: Eight patients met criteria for surgical treatment and underwent prompt drainage. Of the 29 patients for whom initial nonsurgical management was recommended, 27 (93.1%) SPAs cleared with expectant observation on intravenous antibiotics, and 2 patients defaulted to surgical intervention. All cases had successful clinical outcomes. CONCLUSIONS: In patients less than 9 years of age, orbital SPAs are likely to resolve without surgery, provided certain surgical criteria are absent.

Abscess↗

Orbital inflammatory pseudotumor and ischemic vasculitis in Churg-Strauss syndrome: report of two cases and review of the literature.

OBJECTIVE: To clarify the characteristics of ocular manifestations in Churg-Strauss syndrome (allergic granulomatosis and angiitis). DESIGN: Two interventional case reports and literature review. PARTICIPANTS: Two patients with Churg-Strauss syndrome with ocular manifestations are described; 15 previously reported cases and the present 2 cases of Churg-Strauss syndrome with ocular manifestations are reviewed. INTERVENTION: Ocular manifestations were divided into two groups: orbital inflammatory pseudotumor and ischemic vasculitis. MAIN OUTCOME MEASURES: The onset, conjunctival involvement, orbital imaging, antineutrophil cytoplasmic antibodies (ANCA), and visual prognosis were evaluated. RESULTS: The characteristics of the orbital inflammatory pseudotumor type (eight cases) are chronic onset, positive conjunctival involvement, abnormalities in orbital imaging studies, negative ANCA, and good visual prognosis. The ischemic type (nine cases) is characterized by sudden onset, no conjunctival involvement or abnormalities in imaging studies, positive ANCA, and occasional poor visual prognosis. CONCLUSIONS: Orbital inflammatory pseudotumor and ischemic vasculitis may represent two essential characteristics of Churg-Strauss syndrome, granulomatosis and angiitis, respectively. The clinical features of the two types are so distinct that differentiation may be meaningful for diagnosis and treatment of Churg-Strauss syndrome with ocular manifestations.

Adult↗

Angle-closure glaucoma in association with orbital pseudotumor.

PURPOSE: To describe the pathophysiology of angle-closure glaucoma secondary to idiopathic inflammatory orbital pseudotumor. DESIGN: Retrospective, small noncomparative case series. PARTICIPANTS: Three patients with angle-closure glaucoma and orbital pseudotumor. METHODS: The pathophysiology of this entity was investigated using magnetic resonant imaging (MRI) and ultrasound biomicroscopy (UBM). MAIN OUTCOMES MEASURES: Clinical features, anterior chamber angle configuration, and intraocular pressure. RESULTS: Angle closure from anterior rotation of the ciliary body caused by choroidal effusions secondary to pseudotumor was demonstrated using MRI and UBM. Two of the three cases resolved after treatment for orbital pseudotumor. CONCLUSIONS: Idiopathic orbital pseudotumor is a cause of secondary angle-closure glaucoma. The mechanism of angle closure is anterior rotation of the ciliary body secondary to choroidal effusions resulting from the orbital inflammation.

Adult↗

Orbital involvement in cherubism.

PURPOSE: To demonstrate the clinical, radiologic, and histopathologic features of a patient with orbital involvement in cherubism that prompted surgical treatment. DESIGN: Single interventional case report. INTERVENTION: Findings of the ophthalmic evaluation, computed tomography (CT) scans, intraoperative examination, and light microscopy of the specimens were analyzed. MAIN OUTCOME MEASURES: Globe displacement, orbital bony lesions detected on CT scans, histopathology, and postoperative results were assessed. RESULTS: A 27-year-old female was seen with a slowly progressive superonasal globe displacement and a temporal orbital mass bilaterally of 6 years' duration. She had a history of cherubism, but her cheeks and jaws had a normal appearance instead of the bilateral fullness of the lower half of the face typical of the disease. CT scans demonstrated multicystic bony lesions arising from the orbital floors bilaterally. The masses were excised using an anterior transcutaneous transseptal orbitotomy. Histopathology demonstrated numerous giant cells in a fibrovascular stroma, confirming the clinical diagnosis of cherubism. Postoperative recovery was complete. CONCLUSIONS: Orbital involvement in cherubism may develop beyond puberty, after stabilization or regression of the lesions in the jaws. Patients with cherubism should be routinely evaluated by an ophthalmologist.

Adult↗

Retained orbital wooden foreign body: a surgical technique and rationale.

OBJECTIVE: To emphasize the potential complications of a retained orbital wooden foreign body (WFB) and the rationale of a surgical technique. DESIGN: Two interventional case reports. PARTICIPANTS: Two patients sustained an orbital WFB injury. Both patients had ocular complications despite repeated attempts to remove the suspected residues. INTERVENTION: Computed tomography and magnetic resonance imaging of both patients at different intervals revealed evidence of orbital foreign body migration toward the cranium. A surgical technique combing transcranial and orbital approaches was used to remove the residues. MAIN OUTCOME MEASURES: Preoperative and postoperative vision, proptosis, ocular motility, and various ocular symptoms and signs. RESULTS: In both patients, no postoperative complications were seen, and all preoperative symptoms and signs were resolved at 9- and 19-month follow-ups, respectively. CONCLUSIONS: A retained orbital WFB can cause early or late complications and is known to have the potential to migrate intracranially. In selected patients, a team approach may be the best technique to ensure complete removal.

Adult↗

Histiocytoid variant of eccrine sweat gland carcinoma of the eyelid and orbit: report of five cases.

OBJECTIVE: To study the clinicopathologic features of the histiocytoid variant of adenocarcinoma of the eccrine sweat gland of the eye and orbit. DESIGN: Retrospective case series. PARTICIPANTS: Five patients undergoing orbital and eyelid biopsy as a diagnostic procedure. METHODS: The authors examined the clinical histories and pathologic findings of five patients with eccrine adenocarcinoma of the eyelid with orbital invasion. MAIN OUTCOME MEASURES: Clinical and histopathologic examinations, including routine histopathology, immunohistochemistry, and electron microscopy studies. RESULTS: The tumors presented as insidious, diffusely infiltrative, firm cutaneous masses in the periocular area that later infiltrated the orbit. Histopathologic examination revealed that the tumors infiltrated the dermis and were composed of cells with a histiocytic to signet ring appearance. Tumor cells exhibited intracellular mucin production. Immunohistochemical stains were positive in tumor cells for low and high molecular weight cytokeratins, carcinoembryonic antigen, and epithelial membrane antigen. Electron microscopic examination showed lumen formation and intracytoplasmic mucin in tumor cells. CONCLUSIONS: The histiocytoid variant of adenocarcinoma of the eccrine sweat gland of the eyelid may present as an insidious tumor and diffusely invade the orbit. These cases may be confused with metastatic adenocarcinoma.

Adenocarcinoma↗

Downgaze "hang-up" of the upper eyelid in patients with adult-onset ptosis: an important sign of possible orbital malignancy.

PURPOSE: To highlight, in patients with adult-onset ptosis, the importance of upper eyelid hang-up during downgaze as a clinical sign of serious orbital disease. DESIGN: Retrospective, observational case series. PATIENTS AND METHODS: Retrospective review of the presenting clinical symptoms and signs for a series of seven patients, some referred for treatment of adult-onset ptosis, with hang-up of the upper eyelid on down-gaze. RESULTS: All patients had symptoms of upper eyelid ptosis at presentation, starting at ages ranging from 29 to 81 years (mean, 64 years; median, 70 years). All patients displayed marked hang-up of the affected upper lid on downgaze, and the levator excursion was reduced (range, 2-13 mm; mean, 9 mm) compared with the asymptomatic side. All patients with downgaze hang-up had associated orbital malignancy, but they otherwise had only minimal or no underlying orbital disease. Exophthalmometry varied between subtle (1 mm) relative enophthalmos in two cases, to mild relative proptosis (3/7 cases; range, 2-3 mm). Ipsilateral up gaze was reduced in 4 of 7 patients (57%). CONCLUSION: In contrast to patients with adult-onset blepharoptosis (resulting from dehiscence of the levator muscle aponeurosis), adults presenting with ptosis resulting from malignant infiltration of the upper orbital tissues may display hang-up of the upper eyelid in downgaze. This important sign should alert the clinician to the possibility of serious underlying orbital disease.

Adenocarcinoma↗

Primary T-cell immunoblastic lymphoma of the orbit in a pediatric patient.

BACKGROUND: The authors report a case of an 8-year-old pediatric patient with a 2-week history of painless periorbital swelling unresponsive to antibiotic treatment. METHODS: Computed tomography (CT) showed a large, lateral, anterior left orbital soft tissue mass with bony erosion into the anterior cranial fossa through the roof of the orbit laterally. Surgical exploration showed a hard white mass that had eroded through the roof of the left orbit and into the anterior cranial fossa, with herniation of the brain and associated dura through the defect. Results of a complete evaluation of the child for systemic lymphoma, including a lumbar puncture, chest x-ray, bone scan, bone marrow aspirate, and chest/abdomen CT, were negative. RESULTS: Results of histopathologic and immuno-histochemical evaluation showed a primary orbital T-cell immunoblastic lymphoma. The patient was treated with intrathecal ara-C (Cytosar-U) and methotrexate, 16.2 Gy of whole brain irradiation, and a chemotherapeutic protocol consisting of cyclophosphamide (Cytoxin), vincristine (Oncovin), methotrexate, daunomycin, and prednisone. The patient remains free of lymphoma 33 months after diagnosis, with 20/20 visual acuity in both eyes. CONCLUSION: The authors believe that this is the youngest documented case of a primary T-cell immunoblastic lymphoma of the orbit.

Antineoplastic Combined Chemotherapy Protocols↗

Capillary hemangioma of the lids and orbit: an analysis of the clinical features and therapeutic results in 101 cases.

The clinical records of 101 patients (biopsies done on 48) with capillary hemangiomas of the orbit, orbit and lids, and lids were analyzed. Lesions predominated in females and typically appeared at birth or within the first two months of life. The superior orbit and lids were definite sites of predilection. Telltale periocular cutaneous involvement occurred in one third of the cases. Pure proptosis caused by a deep orbital tumor without an anterior component was found in seven cases. Proptosis in general was mild, but in two cases was severe enough to cause corneal exposure. Findings suprortive of a diagnosis of an orbital or periorbital hemangioma include (1) change in tumor size on crying (46%); (2) a dark vascular coloration to the subcutaneous tissue (80%); and (3) the existence of nonperiocular strawberry marks (25). Diagnostic tests of most value were plain radiography, ultrasonography, computed axial tomography, and arteriography. The complication rate in the 50 patients followed up over five years was 80% (60% with amblyopia). Present treatment modalities do not appear to be achieving the desired goals of a good cosmetic and functional outcome. Radiotherapy and corticosteroids are both effective in individual cases, but many lesions are resistant to all forms of present treatment. Amblyopia therapy is difficult and often unsuccessful in these patients.

Age Factors↗

Management of limited rhino-orbital mucormycosis without exenteration.

Eight cases of rhino-orbital mucormycosis managed successfully without exenteration were reviewed. The favorable outcome was attributable to early diagnosis and management of focal areas of fungus infection. Treatment included: correction of diabetic ketoacidosis or other concomitant metabolic derangement; wide local excision and debridement of all involved and devitalized oral, nasal, sinus, and orbital tissue, while establishing adequate sinus and orbital drainage; daily irrigation and packing of the involved orbital and paranasal areas with amphotericin B; and intravenous amphotericin B. This represents the largest reported series of rhino-orbital mucormycosis survivors without mutilating surgery and with unaltered visual acuity.

Adult↗

Congenital neurocutaneous melanosis with metastatic orbital malignant melanoma.

Proptosis and ptosis, caused by a large orbital mass that was excised and determined to be malignant melanoma, developed in a 4-year-old girl with congenital neurocutaneous melanosis (multiple large or giant cutaneous nevi associated with abnormal leptomeningeal pigmentation). Shortly thereafter, the patient had evidence suggestive of systemic metastases and died. The orbital tumor was likely metastatic from a primary meningeal melanoma. Other possible sources of metastatic tumor are discussed. It is unlikely that this was a primary orbital melanoma because the patient had no clinical or pathologic manifestations of pre-existing oculocutaneous melanosis, orbital melanosis, or orbital blue nevus.

Biopsy↗

Growth of the conjunctival fornix and orbital margin in term and premature infants.

Knowledge of the growth of the neonatal conjunctival fornix and anterior orbit will aid development of neonatal prosthetic conformers and conjunctival sensors for oxygen determinations, as well as help define orbital malformation syndromes. These data are not available in any prospective series in the literature. The authors studied 55 premature and term neonates and determined horizontal and vertical dimensions of the conjunctival fornix and orbital margin and palpebral fissure width. Each of the ocular parameters had statistically significant correlation coefficients in relation to weight and gestational age (P less than 0.05). Linear regression analysis showed significant relationships which predict conjunctival fornix, orbital margin, and palpebral fissure dimensions from body weight (P less than 0.05). Statistically significant correlation coefficients and linear regression relationships were found for horizontal, but not vertical, diameter measurements of the conjunctival fornix in relation to orbital margin and palpebral fissure dimensions (P less than 0.01).

Birth Weight↗

Extracellular deposition of eosinophil major basic protein in orbital histiocytosis X.

Although eosinophils are prominent in orbital lesions of patients with histiocytosis X (Langerhans' cell histiocytosis), little is known of their pathogenic significance in the disease. To determine whether eosinophils degranulate and deposit toxic proteins in orbital histiocytosis X, the authors examined lesions by indirect immunofluorescence for localization of the core granule protein (major basic protein) outside of eosinophils. Four patients with histiocytosis X were studied: three with eosinophilic granuloma and one with Hand-Schüller-Christian disease. Tissue eosinophilia was prominent in all specimens; striking extracellular deposition of eosinophil major basic protein was noted in three patients, and focal deposition was present in the fourth patient. Orbital specimens obtained at autopsy from patients without orbital disease were studied as control specimens; no tissue eosinophilia or deposition of eosinophil major basic protein was observed. These findings indicate that eosinophils likely degranulate in lesions of orbital histiocytosis X and may participate in the pathogenesis of the disease.

Adolescent↗

Epithelioid hemangioendothelioma of the orbital bones.

PURPOSE: The authors report a case of an epithelioid hemangioendothelioma arising in the orbital bones. A review of the literature related to this rare orbital neoplasm identified eight well-documented cases, one of which occurred in a patient younger than that reported here, but none of which originated in bone. METHODS: A 3 1/2-month-old boy had a left inferior orbital mass that had grown rapidly over a 3-day period. An emergency computed tomography scan showed a large neoplasm with significant bone destruction of the zygoma and maxilla. Initial examination suggested a rhabdomyosarcoma, and a transconjunctival biopsy was performed, which was complicated by significant blood loss. The final pathologic diagnosis was an epithelioid hemangioendothelioma, or grade 2 hemangioendothelioma, of bone origin. No other sites of disease were found on metastatic survey. Subsequent treatment consisted of an en bloc tumor resection sparing the orbital soft tissues and globe. RESULTS: The patient is free of disease and has normal visual fixation and ocular motility 20 months after surgery. CONCLUSION: Epithelioid hemangioendothelioma, a vascular malignancy of endothelial cell origin, very rarely involves the orbit. This case is notable for its early development, rapid growth, bony origin, and epithelioid histology.

Biopsy↗

Complications associated with alloplastic implants used in orbital fracture repair.

BACKGROUND: The treatment of orbital wall fractures involves observation and/or surgical reduction with repositioning of herniated orbital tissues. To prevent reherniation of tissue and development of enophthalmos, the orbital floor or wall defect is commonly covered with an alloplastic implant. Complications associated with these implants are infrequent and generally appear as isolated case reports. METHODS: The authors reviewed the files of four consultative oculoplastic surgeons and searched for individuals with complications secondary to their alloplastic implants used during orbital fracture repair. FINDINGS: Seventeen patients were identified with a variety of complications related to their alloplastic implant. CONCLUSION: Although these implants are relatively inert and develop a fibrous capsule walling them off from the surrounding orbit, they remain foreign bodies and are thus subject to possible complications at any time. The authors review the spectrum of complications occurring with various alloplastic implants.

Adult↗

Orbital polymyositis and giant cell myocarditis.

BACKGROUND: Orbital polymyositis associated with giant cell myocarditis rarely has been reported in the literature. The authors report the clinical, neuroradiographic, and histopathologic features of the only patient to survive this usually fatal syndrome after cardiac transplantation. FINDINGS: This 22-year-old white woman presented in 1991 with periorbital redness, swelling, and pain in both eyes that was unresponsive to antibiotic therapy. Results of her examination were significant for limited extraocular movements, ptosis, erythema, edema, chemosis, and exophthalmos. Electrocardiogram and chest x-ray were normal. Orbital computed tomographic scan showed swelling of the extraocular muscles up to and including their insertions. The patient was given the diagnosis of orbital polymyositis and her condition improved clinically and radiographically while taking parenteral steroids. One month after discharge, the patient was in cardiogenic shock. Endomyocardial biopsy showed giant cell myocarditis, and the patient underwent emergent cardiac transplantation. Despite a complicated postoperative course, the patient has done remarkably well. CONCLUSION: Although this disorder is rare, this case suggests the need for a high index of suspicion for giant cell myocarditis in patients with inflammatory orbital polymyositis. In non-Graves orbital polymyositis the patient should be questioned and instructed concerning the signs and symptoms of congestive heart failure. Chest x-ray, Holter monitoring, and electrocardiogram also should be performed and be repeated with an echocardiogram if there are any cardiac symptoms. In addition, early endomyocardial biopsy should be considered in the proper clinical setting, allowing timely diagnosis and expeditious cardiac transplantation.

Adult↗

Color Doppler imaging of normal orbital vasculature.

BACKGROUND: Color Doppler imaging has become a useful adjunctive tool in diagnosing ophthalmic vascular disease. However, a paucity of information exists regarding normal values. This investigation was conducted to generate a database of normal retrobulbar circulatory parameters. METHODS: The authors prospectively studied the retrobulbar hemodynamic parameters of 53 normal eyes from 32 healthy individuals of various ages, all without risk factors for the development of systemic vascular disease. Preliminary Doppler spectral analysis was used to demonstrate normal carotid arterial, carotid siphon, and ophthalmic vascular anatomy. RESULTS: Mean hemodynamic values were recorded for each retrobulbar vessel. Diastolic blood pressure significantly correlated with ophthalmic arterial end-diastolic velocity (P < 0.05) and pulsatility index (P < 0.05). Although end-diastolic velocity significantly decreased (P < 0.05) and vascular resistance significantly increased (P < 0.05) as a function of age in the posterior ciliary and central retinal arterial circulation, no such correlation was identified in the ophthalmic artery which demonstrated significantly more hemodynamic variability among individuals (P < 0.00001). No significant differences in blood flow or vascular resistance were noted between sexes, fellow orbits, or the nasal and temporal branches of the posterior ciliary artery within individual orbits. CONCLUSION: Orbital hemodynamic parameters do not appear to be sex dependent or variable between fellow orbits. These data suggest that age and diastolic blood pressure may affect normal orbital blood flow and vascular resistance patterns. Significant interindividual variability is limited only to ophthalmic arterial blood flow.

Adult↗

Orbital infarction syndrome after surgery for intracranial aneurysms.

BACKGROUND: Global orbital infarction results from ischemia of the intraocular and intraorbital structures due to hypoperfusion of the ophthalmic artery and its branches. PATIENTS: The authors describe six patients in whom acute proptosis, ophthalmoplegia, and blindness developed immediately after surgery for intracranial aneurysms. RESULTS: All patients underwent standard frontotemporal craniotomies to clip their aneurysms. In all patients, proptosis, ophthalmoplegia, and blindness developed in the immediate postoperative period; fundus abnormalities included retinal edema, retinal arteriolar narrowing and other vascular abnormalities, and pale optic disc swelling. Some patients had facial and corneal anesthesia. Ophthalmoplegia and facial anesthesia improved in most patients, but none regained any vision in the affected eye. CONCLUSION: Orbital infarction syndrome is a rare complication of neurosurgical procedures. Increased orbital pressure probably reduced ophthalmic artery and collateral arterial perfusion, resulting in ischemia of the intraocular and intraorbital structures. There may be multiple factors that compound the risk for orbital infarction, and patients with subarachnoid hemorrhage, increased intracranial pressure, anomalous arterial or venous circulation, or impaired orbital venous outflow seem particularly vulnerable.

Adult↗