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Orbital presentation of posttransplantation lymphoproliferative disorder: a small case series.

OBJECTIVE: To describe a small series of patients with orbital presentation of posttransplantation lymphoproliferative disorder (PTLD). DESIGN: Retrospective, interventional case series. PARTICIPANTS: Three patients with orbital presentation of histologically diagnosed PTLD. METHODS: Review of medical records. MAIN OUTCOME MEASURES: Measured parameters included vision, proptosis, and tumor extent. RESULTS: Three cases of orbital PTLD are described. In two of the cases, the tumor initially demonstrated orbital signs and symptoms, whereas in the third case, orbital and systemic signs were synchronous. Two of three patients had disseminated disease discovered at the time of presentation. One adult patient had synchronous presentation of PTLD in the orbit and prostate. One pediatric patient had tumor dissemination to the liver at the time of presentation. The PTLD tumors were classified histologically as diffuse large cell lymphoma of monomorphic or immunoblastic type in all three cases. Treatment included local irradiation, decreased immunosuppression, and antilymphocyte monoclonal antibodies. CONCLUSIONS: Orbital presentation is a rare manifestation of PTLD. However, ophthalmologists must consider this diagnosis carefully in organ transplant recipients with subtle orbital signs and symptoms at presentation. Early detection may alter prognosis. In each case presented, the diagnosis was established via lesion biopsy and subsequent histologic or flow cytometric evaluation, or both.

Antineoplastic Combined Chemotherapy Protocols↗

Osteogenic sarcoma after orbital radiation rhabdomyosarcoma.

PURPOSE: We describe the occurrence of maxillary and orbital osteogenic sarcoma in a child after treatment of contralateral orbital rhabdomyosarcoma with external beam radiation and chemotherapy. DESIGN: Interventional case report. INTERVENTION: Treatment of a maxillary and orbital rhabdomyosarcoma with surgical resection, systemic chemotherapy, and local radiation. MAIN OUTCOME MEASURES: Occurrence and histology of secondary malignancy after orbital radiation. RESULTS: An eleven year-old male presented for evaluation of left facial swelling, occurring ten years after successful treatment of a right orbital embryonal rhabdomyosarcoma with chemotherapy and 5040 cGY of external beam radiation. Computerized tomography demonstrated a mass involving the left maxillary sinus and orbital floor. Biopsy demonstrated osteogenic sarcoma. Despite attempted total excision with radical maxillectomy, resection margins were found to have microscopic extension of the tumor. Postoperatively he was treated with systemic chemotherapy and local radiation. Eight months postoperatively he remains alive despite local progression. CONCLUSIONS: Osteogenic sarcoma can occur as a secondary malignancy years after the successful treatment of orbital rhabdomyosarcoma with external beam radiation and chemotherapy. After orbital radiation, subjects should undergo routine lifelong examinations.

Child↗

Primary and secondary implants in the anophthalmic orbit. Preoperative and postoperative computed tomographic appearance.

Sagittal computed tomographic (CT) scans of 10 anophthalmic orbits with a primary implant, an acrylic ball covered with donor sclera, and 10 anophthalmic orbits before and after insertion of a secondary implant were studied. For each patient, the scans of the anophthalmic and contralateral normal orbit were compared. In the anophthalmic orbits, the anatomy was optimally restored in those with a primary implant, and suboptimal results were achieved in the orbits with a secondary implant. The greatest differences were noticed in the orbits without an implant. Differences were noticed for the position and length of the superior muscle complex and the inferior rectus muscle, the position of the upper eyelid, the depth of the superior sulcus, and the backward tilt of the prosthesis. The authors conclude that the rotatory displacement of the orbital contents after enucleation, which explains the sequelae of the anophthalmic orbit, is grossly circumvented by the insertion of an implant. In achieving this effect, primary implants are better than secondary ones.

Adolescent↗

Hydraulic orbital injection injuries.

BACKGROUND: A high-pressure stream from mechanical equipment may inject gas or liquids deep into the orbit with few initial clinical signs. Aggressive surgical debridement as used in the extremities for the treatment of injection injuries is not possible in the orbit. METHODS: Four patients with orbital injection injuries from farm or industrial equipment are presented. Previously reported cases of high-pressure injection injury are reviewed. RESULTS: Two patients suffered localized anterior orbital inflammation partially responsive to steroidal and nonsteroidal anti-inflammatory agents. Late debridement was required in one patient for a persistent lipogranuloma. Two patients suffered more dramatic and diffuse injections of hydrocarbon mixtures, requiring emergent early surgical debridement and decompression for compressive orbital signs. All patients attained an adequate functional outcome, with one patient's vision limited by a coexisting ocular injury. CONCLUSIONS: High-pressure orbital injection injuries manifest a spectrum of signs ranging from acute inflammation with tissue necrosis and compressive visual loss to late chronic inflammation with a pseudotumor-like course. The authors recommend the initial treatment of orbital injection injuries with systemic antibiotics followed by prompt neuroradiologic imaging. Systemic corticosteroids should be added for confirmed injection injuries with surgical debridement of discrete masses and orbital decompression when indicated. Continued therapy with anti-inflammatory medication may be required to suppress chronic inflammation with selective late surgical debridement of lipogranulomas.

Adult↗

Orbital fat removal. Decompression for Graves orbitopathy.

BACKGROUND: Orbital decompression has been used to describe surgical procedures that remove some portion of the orbital walls to reduce pressure on the orbital contents. Substantial morbidity associated with these procedures includes infraorbital anesthesia, worsened extraocular motility, globe displacement, and blindness. The authors believe that orbital contents also may be decompressed by removing orbital fat. METHODS: Eighty-one patients with nonactive Graves orbitopathy were selected for orbital fat decompression based on the presence of proptosis and associated signs and symptoms to avoid bone removal. Soft-tissue analysis by computed tomography (CT) scan showed distended pockets of fat extending into the intraconal space, which were removed through medial-upper and lateral-lower anterior orbitotomies. Decompression with bone removal was reserved for those few patients with compressive optic neuropathy unresponsive to medical treatment and those patients with residual deforming exophthalmos after fat removal. RESULTS: One hundred fifty-eight fat decompressions were performed on 81 patients over 9 years. The authors measured an average reduction in proptosis of 1.8 mm (range, 0-6.0 mm). The greatest average reduction in proptosis (3.3 mm) was produced in patients with preoperative Hertel measurements of greater than 25.0 mm. Morbidity was limited to temporary motility impairment of the inferior oblique in two patients. CONCLUSION: The concept of orbital decompression can include removal of orbital fat to reduce proptosis, eliminate symptoms, and improve appearance with far less morbidity than when bone decompression is used as the primary decompressive procedure.

Adipose Tissue↗

Combined venous lymphatic malformations of the orbit (so-called lymphangiomas). Association with noncontiguous intracranial vascular anomalies.

OBJECTIVE: The authors present seven cases of orbital combined venous lymphatic vascular malformations (CVLVM) (lymphangioma) with evidence of noncontiguous intracranial vascular anomalies. DESIGN: The study design was a review. MAIN OUTCOME MEASURES: Radiologic studies of 25 patients with combined venous lymphatic vascular malformations were evaluated for noncontiguous intracranial vascular anomalies. Features of the intracranial anomalies and orbital lesions, their clinical presentation, and prognosis are described. RESULTS: Seven patients (28%) had associated noncontiguous intracranial vascular anomalies. Intracranial hemorrhage occurred in one of these patients. The intracranial anomalies had radiologic characteristics of developmental venous anomalies (DVAs). Diffuse orbital lesions with superficial and deep components (7/7), orbital bony expansion (7/7), and intraconal and extraconal components (4/7) were most common. They involved the inferior orbital fissure and extended into the pterygopalatine fossa in five patients. Involvement of the superior orbital fissure was noted in all seven patients with extension into the middle cranial fossa in three patients. At birth, these patients generally had a visible superficial component and then had episodes of sudden proptosis associated with deep orbital hemorrhages. Visual outcome was poor (20/200 or less) in four (57%) of seven cases. Anterior extension into soft tissues of the face and forehead and other associated vascular lesions, such as palatal involvement, were relatively common. In contrast, CVLVMs (lymphangiomas) without noncontiguous intracranial vascular anomalies were more anterior, less diffuse, less likely to extend into the soft tissues of the face, have associated vascular lesions, or have a poor visual outcome. CONCLUSIONS: Orbital CVLVMs (lymphangiomas) may be associated with noncontiguous intracranial vascular anomalies that may bleed. This association with intracranial DVAs has not been reported previously. The intracranial vasculature should be evaluated prospectively in these lesions, especially if they are diffuse.

Adolescent↗

Distensible venous malformations of the orbit: clinical and hemodynamic features and a new technique of management.

OBJECTIVE: To investigate distensible venous malformations of the orbit (DVMO) as part of a spectrum of orbital vascular malformations, including some that involved periorbital skin, extraorbital sites (central nervous system or nasal sinuses), or combinations of these. The authors also investigated the effectiveness of a new technique of management for selected cases. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Thirty patients had distensible venous anomalies, of which four were combined distensible venous-lymphatic vascular malformations. Distensible lesions were defined as those showing clinical or radiographic expansion with Valsalva maneuver or when the head was placed in a dependent position. These lesions were then classified as superficial (anterior to the equator of the globe), deep (posterior to the globe's equator), combined (deep and superficial), or complex (with intracranial or major extraorbital involvement). INTERVENTION: Surgery was performed on 15 patients (50%), mainly for pain or for cosmetic indications. Six patients underwent this new technique, which involved intraoperative direct venography with control of outflow via pressure at the superior or inferior orbital fissure. The venous malformation was then embolized (by use of cyanoacrylate glue) and excised. RESULTS: The mean age at presentation was 28.2 years (range, 8 months to 75 years). Sixty-six percent of cases involved the left orbit. Superior and medial orbital involvement was most common. Three cases (10%) were classified as superficial, and 13 (43%) as deep. Six patients (20%) had combined superficial and deep components. Eight (27%) had major extraorbital involvement (4 intracranial, 2 facial, and 2 paranasal sinus). Direct venography demonstrated complex multichannel anomalies draining to various sites, including the face and pterygopalatine fossa, without necessarily having a direct connection to the major orbital venous circulation. CONCLUSIONS: Distensible venous malformations of the orbit are part of a spectrum of developmental venous malformations that may be localized to the orbit or involve it as part of a more extensive lesion. The authors describe their clinical and radiologic features and report a new technique of management for selected cases. This method of vascular isolation and embolization of lesions may greatly facilitate excision.

Adolescent↗

Orbital helical computed tomography in the diagnosis and management of eye trauma.

OBJECTIVE: To prospectively determine the accuracy of helical computed tomography (CT) and multiplanar reconstruction and its value in surgical planning for the management of ocular trauma with suspected intraocular and orbital foreign bodies using surgical and clinical follow-up findings as the gold standard. DESIGN: Prospective, observational case series. PARTICIPANTS: Thirty-six patients with ocular trauma and suspected foreign bodies were studied. INTERVENTION: All patients were examined using a standardized scanning protocol with helical CT direct scanning in the axial plane and multiplanar reconstruction of coronal and sagittal planes. MAIN OUTCOME MEASURES: The images were analyzed for the presence and number of intraocular and orbital foreign bodies, anatomic location, and foreign body size. The surgical and clinical follow-up findings (contact lens examination, gonioscopy, indirect ophthalmoscopy and scleral depression, perimetry, color testing, measurement of size) were used as the gold standard to which the image results were compared. RESULTS: Helical CT showed a single intraocular foreign body in 14 patients, a single orbital foreign body in 9 patients, and multiple orbital foreign bodies in 2 patients. Intraocular or orbital foreign bodies were excluded in 11 patients. Twenty foreign bodies were correlated with surgical results. Surgical and clinical follow-up findings were in agreement with helical CT results regarding the detection and determination of the number of presumed foreign bodies. Localization to intraocular versus orbital compartment and proximity to the optic nerve was accurate in all patients. Determination of size of the foreign bodies on the helical CT images was reliable and repeatable. CONCLUSIONS: Helical CT axial scanning with multiplanar reconstruction is accurate at detecting and localizing intraocular and orbital metallic, glass, and stone foreign bodies. This imaging method aids the surgeon in choosing the surgical approach to retained intraocular and orbital foreign bodies.

Adolescent↗

Use of intraoperative neuromonitoring to prevent orbital complications in ethmoid sinus surgery.

Orbital injury is one of the most serious complications in sinus surgery. Although these injuries are generally infrequent, there is a considerable increased risk for orbital complications during procedures in which anatomic landmarks are distorted because of the severity of disease or prior surgery. Currently there are no methods to detect early defects in the lamina papyracea or to prevent continued resection until orbital fat or periorbita is seen. Thirteen New Zealand white rabbits were studied by use of a nerve monitor to identify the periorbita and orbital fat in surgically created lamina defects. Evoked potentials were measured at the medial orbit when the lamina papyracea, periorbita, and orbital fat were stimulated at current intensities ranging from 0.25 to 1.00 mA. Stimulation of the lamina at 0.8 and 1.0 mA resulted in response amplitudes that were significantly lower compared with those of periorbita stimulation (means at 1 mA, 38.26 microV vs. 117.85 microV; p < 0.01). Stimulation of the orbital fat also resulted in higher potentials (mean, 59.47 microV) than those of the lamina papyracea (mean, 38.26 microV) but did not reach statistical significance. The statistically significant difference in response amplitudes between the lamina papyracea and the periorbita indicate that intraoperative monitoring may be useful in identifying early lamina defects, which could in turn reduce the risk of orbital injury during sinus surgery.

Animals↗

Management of the trochlea of the superior oblique muscle in the repair of orbital roof trauma.

PURPOSE: This study evaluated whether reattachment of the trochlea is necessary to assure normal postoperative extraocular muscle movements after orbital roof exploration and trochlea detachment. PATIENTS AND METHODS: This was a retrospective review of 889 operative records of one surgeon practicing at a level I trauma center between January 1, 1992 and December 31, 1998. Three hundred twenty-four of these cases were trauma related, 97 of which involved the upper facial third or upper midface. From this group, 15 patients required orbital roof exploration with trochlea detachment. Age, gender, cause of injury, form of craniofacial injury, method of repair, materials used, date of first notation of intact extraocular movement, and date of last follow-up were recorded and analyzed. RESULTS: From among the 15 patients requiring orbital roof exploration and repair, 14 (93%) were male, with a mean age of 34.7 +/- 15.2 years. Eight (53%) were involved in motor vehicle accidents, 3 (20%) in motorcycle accidents, and 4 (27%) in high-energy impacts. Most (95%) had injuries that included the frontal sinus, the naso-orbital-ethmoid region (60%), and the orbital rims (60%). After bitemporal flap reflection, careful subperiosteal dissection, supraorbital and supratrochlear nerve repositioning, and trochlea detachment, simple reapproximation of the orbital soft tissues to the reconstructed orbit resulted in satisfactory extraocular muscle movements in all cases. CONCLUSIONS: When careful subperiosteal dissection is used, simple reapproximation of the soft tissues adjacent to the reconstructed orbital roof, without reattachment of the trochlea, is all that was necessary to assure satisfactory extraocular muscle movements postoperatively.

Adult↗

Repair of traumatic orbital wall defects with nasal septal cartilage: report of five cases.

PURPOSE: This article reports the use of nasal septal cartilage for the repair of traumatic orbital wall defects. PATIENTS AND METHODS: Five patients with disruption of the orbital wall after facial trauma were included in this retrospective review. All of the patients underwent open reduction with internal fixation of the fractures as well as repair of the orbital wall defect with autogenous septal cartilage. RESULTS: Nasal septal cartilage was used in four cases of orbital floor defect and one case of orbital roof defect. All of the cases were successfully treated by restoration of the orbital wall continuity. CONCLUSION: Nasal septal cartilage is a readily accessible autogenous material that can be easily harvested with minimal donor site morbidity, and it should be considered when an autogenous orbital implant is needed for the repair of a traumatic orbital wall defect.

Adolescent↗

Orbital myositis following streptococcal pharyngitis in a pediatric patient.

BACKGROUND: Orbital myositis is a relatively rare ocular inflammatory disease. It is currently classified as an idiopathic orbital inflammatory disease, but has been associated with ocular and systemic disorders, including scleritis, rheumatoid arthritis, Crohn's disease, and systemic lupus erythematosis. Orbital myositis has also been associated with infectious disease such as streptococcal pharyngitis, viral upper respiratory infection, and Borrelia burgdorferi infection. CASE REPORT AND DISCUSSION: This report documents treatment and imaging of a 13-year-old boy who had an episode of right-sided orbital myositis following streptococcal pharyngitis with anti-streptolysin-o (ASO) titer elevation to 1,188 IU/ml. Clinically, this patient demonstrated acute onset of marked restriction and mild under-action of the lateral rectus muscle of the right eye. Thickening of the lateral and medial recti was demonstrated on computed tomography. The mild orbital myositis was treated with oral nonsteroidal anti-inflammatory drug therapy. The nonconcomitant strabismus resolved approximately 7 weeks after initial presentation. General orbital inflammatory disease management and the association of upper respiratory tract infection with orbital myositis are discussed. CONCLUSION: Orbital myositis is a distinct clinical entity that can occur after streptococcal upper respiratory infection, consisting of pain on ocular movement, localized swelling, restrictive strabismus, and inflammatory signs of the involved muscle(s).

Adolescent↗

Preseptal and orbital cellulitis: a 10-year review of hospitalized patients.

BACKGROUND: Preseptal and orbital cellulitis range in severity from minor to potentially severe complications. The purpose of this study is to describe the clinical features of patients with preseptal or orbital cellulitis in one medical center in Taiwan, and to assess the effectiveness of treatments and the complications. METHODS: Patients admitted between 1996 and 2005 to Taipei Veterans General Hospital under the diagnosis of preseptal or orbital cellulitis were retrospectively reviewed. The demographics, administrative history, past history, clinical presentations, treatments, and complications were analyzed. RESULTS: In total, 94 patients fulfilling the diagnostic criteria for preseptal or orbital cellulitis were identified (67 had preseptal cellulitis, 27 had orbital cellulitis). While paranasal sinus disease was the most common predisposing cause in orbital cases, skin lesions in children and dacryocystitis in adults were the most common in preseptal cases. Microbiologic investigations showed variable results, but the most common pathogen isolated was Staphylococcus aureus. Cultures from eye swabs and local abscesses gave the highest positive yield. Blood cultures were taken in some patients, but the positive rate was extremely low. Treatments included intravenous antibiotics alone, or intravenous antibiotics combined with surgical drainage. Only one case had permanent ocular motility impairment after removal of the orbital foreign body. CONCLUSION: Despite the past history of potential morbidity and even mortality from orbital cellulitis, early diagnosis and prompt treatment with proper antibiotics and/or surgical intervention can achieve a good prognosis.

Adolescent↗

Orbital space-occupying lesions in Denmark 1974-1997.

PURPOSE: To determine the frequency, distribution according to diagnosis and the recurrence frequency of orbital space-occupying lesions in Denmark, to determine sex and age of the patients and to establish a national orbital data register. METHODS: All biopsied/surgically removed orbital lesions collected by Danish pathological departments during the period 1974-1997 were identified by SNOMED codes. In addition, in each case gender and age of the patients and number of recurrences were registered. RESULTS: 965 orbital lesions from 841 patients were identified during the 24-year period. The incidence of orbital lesions increased significantly over the study period and at present about 80 cases/year are registered. The ratio benign/malignant lesion per year remained constant during the observation period. Lesions in children constituted a total of 152 (16%), the percentage of lesions in children being constant during the period. Malignant tumors comprised a total of 364 (45%) in adults and 34 (22%) in children. Malignant tumors were in adults distributed with 43% primary, 48% secondary invading and 9% metastatic tumors, and in children with 65% primary, 29% secondary invading and 6% metastatic tumors. Normal tissue was only found in 6% of the cases recorded. CONCLUSION: A registration of all histologically evaluated orbital space-occupying lesions in Denmark during a period of 24 years was performed and a national orbital database established. Orbital lesions are rare and primarily benign.

Adolescent↗

Orbital vascular anatomy.

The anatomy of the orbital vascular bed is highly complex, with tremendous interindividual variations. The main source of blood supply to the orbit is by the ophthalmic artery, the first branch of the internal carotid artery. The origin, course, and branches of the ophthalmic artery, and the genesis of the variations in origin, course, and branching pattern of the ophthalmic artery are discussed. The external carotid artery normally contributes only to a small extent to the orbital blood supply via the infraorbital artery and orbital branch of the middle meningeal artery.The complex, highly variable and confusing orbital venous system can be divided into: (i) main orbital veins (superior and inferior ophthalmic veins), (ii) inconstant orbital veins (middle and medial ophthalmic veins and four collateral veins), (iii) orbital venous networks, and (iv) various venous tributaries. All these are described briefly.

Carotid Artery, External↗

Orbital and adnexal cysticercosis.

Orbital and adnexal cysticercosis is emerging as a far commoner disease than previously considered, both in endemic and nonendemic areas of cysticercosis. A review of the literature on orbital and adnexal cysticercosis found that it has a predilection for children and young adults with no definite sex predilection. The extraocular muscle form is the commonest type of orbital and adnexal cysticercosis. Lodgement of cysts in the subconjunctival space is another common site, followed by the eyelid, optic nerve, retro-orbital space and lacrimal gland. Association of orbital cysticercosis with systemic cysticercosis is quite rare. The clinical manifestations of orbital or adnexal cysticercosis are entirely different and depend on the location, size, relation to adjacent structures and stage of evolution of the cyst. Diagnosis of cysticercosis is based mainly on orbital imaging because of its highly specific appearance. Tissue diagnosis is not essential for initiating treatment. Medical therapy is the recommended treatment for the extraocular muscle form and retro-orbital cysticer-cosis. Surgical removal is advocated for subconjunctival and eyelid cysticercosis. Because of the limited number of cases of optic nerve and lacrimal gland cysticercosis, their treatment is controversial.

Adult↗

Repair of traumatic inferior orbital wall defects with nasoseptal cartilage.

PURPOSE: This study evaluated the effectiveness of nasoseptal cartilage for repairing traumatic orbital floor defects. PATIENTS AND METHODS: Autogenous septal cartilage was used in 20 patients. They were evaluated for the presence or absence of diplopia, enophthalmus, infraorbital nerve paresthesia, and ocular motility disorders. Surgical indications for orbital exploration included entrapment of orbital tissues, large orbital defect (greater than 50% of the orbital floor or more than 8 mm), or orbital floor defects with involvement of other zygomaticofrontal complex fractures. RESULTS: All patients were successfully treated by restoration of the orbital wall continuity. Follow-up at 1 week to 6 months showed 1 patient with postoperative enophthalmos and 1 patient with lower lid edema. There were no donor site and graft infections or graft extrusion. CONCLUSIONS: Nasal septal cartilage is a readily accessible autogenous tissue that should be considered when an autogenous graft is needed for orbital floor defect reconstruction.

Adolescent↗

A computer-based method for calculation of orbital floor fractures from coronal computed tomography scans.

PURPOSE: A computer program recently developed for the calculation of the orbital floor and fracture areas from coronal computed tomography (CT) scans was used in a study to evaluate the accuracy and ability of this new method. MATERIAL AND METHODS: The size of orbital floors and fabricated fractures in 14 dried, anatomic specimens were measured in coronal CT scans by 3 independent observers. Based on this data set, the orbital floor and fracture regions were calculated with the newly developed computer program. These calculated regions were then compared with a direct measurement of the specimens that had been obtained by digital photography. The accuracy of the computer-based calculations was assessed using Lin's concordance correlation coefficient. RESULTS: The size of the orbital floor (mean +/- SD) was found to be 5.21 +/- 0.39 cm(2) by direct measurement of the specimens and 5.30 +/- 0.52 cm(2) by calculation with the computer program. The region of the fracture (mean +/- SD) was 1.05 +/- 0.64 cm(2) by direct measurement and 1.01 +/- 0.62 cm(2) by computer calculation. The between-method mean difference (direct measurement minus computer based calculation) was -0.09 cm(2) (or 1.7% of mean orbital floor region) for orbital floor region and 0.04 cm(2) (or 3.8% of mean fracture region) for fracture region. CONCLUSIONS: This accurate and time-saving method is practicable for determining the size and location of orbital floor fractures. This calculation program can be advantageously applied in the clinical management of blowout fractures of the orbit.

Algorithms↗