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PubMed1997

Orbital venous anomalies demonstrated by spiral computed tomography.

OBJECTIVE: To describe the radiographic appearance of acute hemorrhage in orbital venous malformations and how spiral computed tomography (CT) can aid in the diagnosis of these lesions in patients with atypical presentations. DESIGN: Case series from the Eye Plastics and Orbital Service of Massachusetts Eye and Ear Infirmary. PARTICIPANTS/INTERVENTION/MAIN OUTCOME MEASURES: Three patients who initially presented with signs and symptoms of orbital hemorrhage are presented. Their initial clinical and radiologic imaging, follow-up examination, and results of the spiral CT are summarized. RESULTS: The initial CT in each case showed a well-localized homogeneous mass in the posterior/inferior orbit. In the two cases without antecedent trauma, it was difficult to distinguish these localized hemorrhages from possible intraorbital neoplasm. On resolution of the hemorrhage, these three patients had different presentations. The first patient had intermittent proptosis that was documented by increase in exophthalmometry measurement before and after Valsalva maneuver (symptomatic and with clinical signs). The second patient had a subjective orbital pressure sensation, but no visible change by examination (symptomatic but without clinical signs). The third patient was not symptomatic and had no significant clinical findings (asymptomatic and without clinical signs). Spiral CT showed the presence of an enlarging inferior orbital mass during Valsalva maneuver, which was not apparent pre-Valsalva in all these patients. CONCLUSIONS: Localized hemorrhages easily may be mistaken for solid intraorbital masses: therefore, accurate determination can avoid unnecessary surgical intervention. Patients with orbital venous malformation may or may not have symptoms and clinical signs of intermittent proptosis. After the resolution of the initial hemorrhage, spiral CT during Valsalva maneuver using a single breath hold technique is useful in showing the presence of this venous anomaly when suspicious of this entity, even in patients who are asymptomatic.

Acute Disease↗
PubMed1997

The clinical and pathologic constellation of Wegener granulomatosis of the orbit.

PURPOSE: Wegener granulomatosis (WG) may present as an orbital mass without obvious upper respiratory or systemic features. The authors examined the clinical and pathologic features of a series of cases of orbital WG to define the features of presentation and progression of this disorder. METHODS: Thirteen subjects with orbital presentations of WG were identified from the University of British Columbia Orbit Clinic index of diseases. Clinical features were correlated with the results of computed tomography in 12 cases and orbital biopsy in 11 cases. Antineutrophil cytoplasmic antibody (c-ANCA) testing was performed in five cases. RESULTS: The main ocular symptoms were decreased vision, redness, and ocular and facial pain, whereas the main signs were proptosis, scleritis, and lid inflammation. Progression was marked by an increased incidence of bilaterality and systemic features. Ear, nose, and throat features were discovered at presentation in 11 cases and became universal during the follow-up period. Initial antineutrophil cytoplasmic antibody test results were negative in five patients but became positive later in three patients. Orbital biopsy specimens typically had features of mixed inflammation, fat disruption, and small areas of necrosis. The combination of cyclophosphamide and oral steroids was highly effective in terminating disease episodes. CONCLUSIONS: Orbital WG can be recognized by a constellation of clinical and radiologic findings with evidence of an often erosive, infiltrating, and restrictive fibrotic, inflammatory mass. Concurrent ear, nose, and throat or specific ocular findings such as scleritis with typical limbal infiltrate can occur. Biopsy results show mixed inflammation with evidence of necrosis that must not be regarded as a nonspecific finding.

Adolescent↗
PubMed1997

Current status in the treatment of orbital myositis.

PURPOSE: Orbital myositis is described as exquisitely sensitive to therapy with systemic corticosteroids. However, it may turn into a recurrent or chronic disease, requiring repeated courses or maintenance of corticotherapy that often is complicated with serious side effects. The authors conducted this study to evaluate their experience and the current status in the literature as to treatment of orbital myositis with corticosteroids and corticosteroid-sparing alternatives. METHODS: The clinical records and computed tomography scans of patients with orbital myositis who presented at the Orbital Center Amsterdam between 1977 and 1991 were studied for the clinical course and therapeutic outcome. Recurrences were defined as a new attack of the same or another extraocular muscle as the primary affected, in the same or the other orbit. RESULTS: The study group comprised 16 patients. All patients responded to initial treatment with oral corticosteroids (14/16) or nonsteroidal anti-inflammatory drugs (NSAID) (2/16). Of these 16 patients, 9 (56%) had 1 or more recurrences. Recurrences responded to repeated courses of corticosteroids, NSAID, or 20 Gy irradiation. Despite radiotherapy in six patients, recurrences continued to occur within a mean period of 2.7 years (range, 0.5 month-8.8 years). The mean follow-up after initial therapy in general was 9.7 years (range, 2.6-16.9 years), and after radiotherapy, in particular, was 7.4 years (range, 4.1-10.5 years). CONCLUSIONS: Orbital myositis responds well to oral corticosteroids, but recurs in 50% of the cases. Based on a long-term follow-up of six patients who received irradiation of 20 Gy, radiotherapy appears ineffective in stopping recurrent orbital myositis.

Administration, Oral↗
PubMed1998

Malignant tumors of the orbit. Analysis of the Florida Cancer Registry.

PURPOSE: The purpose of the study is to determine the relative frequency and incidence of primary malignant tumors of the orbit occurring in Florida. METHODS: Cases of primary orbital malignancy were identified in the Florida Cancer Data System registry from 1981 through 1993. Analysis was restricted to persons who reside within the state. RESULTS: Three hundred fourteen primary orbital malignancies were reported; 55% of malignant orbital tumors were lymphoma. The average annual incidence for all malignancies was 2 cases per million. There was a steady increase in the annual incidence of orbital malignancy during the 13 years of the study. Lymphoma showed the greatest rise in annual incidence. The average annual incidence of lymphoma increased 166% the last 6 years of the study compared to the first 6 years. CONCLUSIONS: A large increase in orbital lymphoma was documented in the Florida cancer registry from 1981 through 1993. The increase in incidence cannot be explained by improved compliance in reporting or by the small increase in incidence of systemic lymphoma during the survey period. Additional studies are needed to determine whether the observed increase in incidence in orbital lymphoma is genuine or spurious.

Adolescent↗
PubMed2002

Post-traumatic orbital floor reconstruction with nasoseptal cartilage in children.

OBJECTIVE: Repair of orbital floor fractures may require the placement of a graft or implant. Both autogenous and alloplastic materials have been used for this purpose. This article reports the use of nasal septal cartilage for the repair of orbital floor defect secondary to blunt facial trauma in children. METHODS: Three children with disruption of the orbital floor after facial trauma were included in this prospective review. All children underwent open reduction with rigid fixation of the facial fractures and reconstruction of the orbital floor with nasoseptal cartilage. RESULTS: All of the cases were successfully treated by restoration of the orbital floor continuity. On follow-up clinical examination, one patient had persistent mild enophthalmus. CONCLUSIONS: Nasal septal cartilage is a readily accessible autogenous material with minimal donor site morbidity, and should be considered when an autogenous orbital implant is needed for the repair of a traumatic orbital floor defect.

Cartilage↗
PubMed2004

[Spontaneous orbital hematoma in an adult. A case report].

Spontaneous orbital hematoma is uncommon, especially in adults. It produces proptosis which progress rapidly and is often associated with ocular movement disturbance. The Authors report a case of spontaneous orbital hematoma in a 45-year-old woman, who had had no previous disease and had experienced sudden onset diplopia associated with moderate proptosis and pain in her right orbit. The diagnosis of orbital hematoma was made by computed tomographic scan. Further investigations revealed arterial hypertension. After a needle aspiration of this hematoma, proptosis resolved, ocular movement began to improve as well as visual acuity. Orbital hematoma revealing arterial hypertension is exceptional. It could be associated with ocular and orbital complications that lead the patient to consult an ophthalmologist. The diagnosis is based essentially on radiology. The other causes of orbital process, inflammation, tumor or vascular malformation, should be considered in the differential diagnosis. Therapeutic management could require surgical evacuation when visual function is compromised (compressive optic neuropathy).

Female↗
PubMed2005

[Langerhans-cell histiocytosis of the orbit. A case study].

PURPOSE: Langerhans' cell histiocytosis is a rare disease representing less than 1% of orbital tumors. METHODS: We report a case of Langerhans cell histiocytosis with orbital involvement in a 9-year-old boy. He presented with an inflammatory swelling if the left lateral orbital wall. The computed tomographic scan revealed an orbital cellular mass with lytic bone lesion within the orbital roof and intracranial enlargement. RESULTS: The cytological study after a biopsy showed infiltrates of histiocytes derived from Langerhans cells. Diagnosis was confirmed by immunohistochemistry, which identified positive staining with anti-S100 and anti-CD1a antibodies. The rapidly expanding orbital tumor, posing a threat of ocular compression as well as intracranial spreading, was treated by chemotherapy (Vinblastine) combined with a steroid. CONCLUSION: A 2-year follow-up showed no evidence of recurrence or systemic involvement. According to this observation, the authors describe the clinicopathological and histological features of orbital involvement in Langerhans cell histiocytosis.

Child↗
PubMed2001

[Idiopathic orbital myositis].

INTRODUCTION: Orbital myositis is a subtype of nonspecific orbital inflammation confined to one or more of the extraocular muscles. EXEGESIS: Three female patients presented presumed orbital myositis, in each of whom the diagnosis was suspected clinically and confirmed by the computed tomography (CT) scan appearances. All presented with orbital pain, diplopia and periorbital edema. All patients responded to systemic corticosteroid with prompt resolution of symptoms and signs, but one patient had recurrent episodes of myositis, leading to the prescription of azathioprine. The clinical and radiological constellation of findings allowing a definitive diagnosis of the nonspecific orbital inflammatory syndrome in most cases is discussed. CONCLUSION: The nonspecific orbital inflammatory syndromes are a heterogenous group of conditions and the CT scan is a valuable tool in differentiating these conditions. Most cases of idiopathic orbital myositis require corticosteroids, but therapeutic alternatives to corticosteroids in refractory diseases are warranted.

Adult↗
PubMed1996

Poly(L-lactide) implants for repair of human orbital floor defects: clinical and magnetic resonance imaging evaluation of long-term results.

PURPOSE: The purpose of this study was to evaluate the long-term outcome of repair of orbital floor defects in patients with resorbable as-polymerized poly(L-lactide) (PLLA) implants and to determine whether these patients showed symptoms that could be indicative of the presence of a late tissue response. PATIENTS AND METHODS: Six patients (four women, two men; mean age, 39 years; range, 18 to 67 years) treated with PLLA implants for orbital floor fractures were recalled for follow-up examination after a period ranging from 3 1/2 to 6 1/2 years. The examination consisted of an interview and a physical examination, including an ophthalmologic and orthoptic consultation. For evaluation of the orbital tissues, coronal spin echo T1- and T2-weighted magnetic resonance images (MRIs) were made through both orbits. RESULTS: None of the patients reported any problems in the years preceding the follow-up examination that might have indicated complications. Clinical examination of the operative sites revealed no abnormalities. At ophthalmologic and orthoptic consultation, normal eye function, without diplopia or restriction of motility, was found in all patients. The MRIs showed no indication of an abnormal or increased soft tissue reaction in the orbital region. CONCLUSIONS: Based on the results of this study, it can be concluded that PLLA orbital floor implants have the potential for successful use in repair of human orbital floor defects.

Adolescent↗
PubMed2003

Simultaneous orbital expansion and intraoral distraction osteogenesis of upper and lower jaws in a patient with hemifacial microsomia.

BACKGROUND: Correction of a micro-orbit, caused by clinical anophthalmia is a very challenging task. In hemifacial microsomia a micro-orbit may be combined with hypoplasia of the malar and the ascending mandibular ramus. MATERIAL: A 5-year-old patient with hemifacial microsomia is described. Hypoplasia of the malar bone and the tilted occlusal plane were corrected by means of intraoral distraction osteogenesis in the upper jaw following precise simulation surgery on a 3D-model. At the same time, the ascending mandibular ramus was lengthened with a second distraction device and a spherical tissue expander was inserted into the hypoplastic orbit. RESULTS: The malar as well as upper and lower jaws were lengthened and positioned symmetrically. The orbital cavity was expanded to 79% of that of the healthy side. Following removal of the distraction devices, the expander was exchanged for a larger one and orbital expansion was continued until overcorrection of the orbit up to 118 percent was achieved. CONCLUSION: Orbital expansion is a minimally invasive method of enlarging the volume of the eye socket three-dimensionally. Intraorally activated buried distraction devices enable "growth" of the jaws making bone transplants avoidable in many cases. By combining both methods, complex malformations can be corrected simultaneously in children.

Child, Preschool↗
PubMed1999

Radiation therapy for benign disease of the orbit.

Benign diseases of the orbit can cause significant impairment of visual function through direct effects on the eye or supporting orbital tissues. Persistent moderate-to-severe inflammatory symptoms, diplopia, and visual loss may prompt therapeutic intervention. Low doses of external-beam irradiation (20 Gy in 2-Gy fractions) have an efficacy equivalent to corticosteroid medications in the treatment of both Graves' ophthalmopathy and orbital pseudotumor, with response rates of 50% to 80%. Appropriate patient selection, coordination with other medical subspecialties, and careful treatment planning are important in maximizing benefit from radiation therapy. In the case of Graves' ophthalmopathy, quantitative assessment of orbital disease severity and thyroid status as well as attainment of cross-sectional imaging should be performed before treatment. Patients whose thyroid disease is controlled but who have moderate-to-severe active orbital involvement can be offered orbital radiation therapy with a high likelihood of response. Stability of disease is generally obtained within 6 months; surgical correction of residual abnormalities may be required. For orbital pseudotumor, attainment of histological material before treatment is important in excluding benign or malignant systemic diseases, including malignant lymphoma. Genotypic abnormalities may exist in patients with reactive lymphoid hyperplasia, some of whom ultimately develop systemic lymphoma. Within the dose range recommended for treatment of Graves' ophthalmopathy and pseudotumor, late radiation effects on the retina or optic nerve should be nonexistent. Adequate lens shielding is required, however, to prevent cataract formation. This may require individualized treatment planning with computed tomography at the time of simulation. Pterygia recurrence after bare sclera excision occurs in at least 30% of cases, usually within 6 months of surgery. Radiation of the surgical bed using a strontium-90 source, beginning within 24 hours postoperatively, reduces the risk of recurrence substantially. Late scleral complications have been associated with large single-fraction treatments. To achieve maximal efficacy with minimal complications, fractionated treatment should be given by radiation oncologists experienced with the technique using sources calibrated by the National Bureau of Standards.

Humans↗
PubMed1998

Medical treatment of pediatric subperiosteal orbital abscess secondary to sinusitis.

BACKGROUND: Subperiosteal abscess may accompany orbital cellulitis secondary to sinusitis. Common surgical principles include incision and drainage of all abscesses. Previous evidence suggests that some orbital abscesses may be treatable with intravenous antibiotics, especially in young children. Children's hospital records were reviewed to determine which abscesses may be treated medically. PATIENTS AND METHODS: Records of patients admitted for orbital cellulitis from 1993 to 1996 were reviewed. Patients with subperiosteal abscess on CT scan were included. Clinical outcomes for initial surgical versus medical management of medial abscesses were compared. Differences in age, hospital stay, and intracranial involvement were analyzed for medial versus nonmedial abscesses. RESULTS: All patients had abscesses adjacent to infected sinuses. Eighteen young children had medial abscesses. Twelve of 13 were cured by initial medical treatment; 4 of 5 underwent successful initial drainage. Outcomes were not statistically different (P > .490). Seven children with nonmedial abscesses were older (P < .001) and had more complicated courses than those with medial abscess. Three of 6 children with superior orbital abscess also had intracranial abscess. Intracranial complication was more likely with superior versus medial orbital abscess (P < .01). CONCLUSIONS: Medial subperiosteal orbital abscesses secondary to sinusitis in children 6 years of age and younger are highly amenable to treatment with intravenous antibiotics. Older children and children with nonmedial abscesses may have more complicated infections. Children with superior orbital abscesses are at higher risk for intracranial abscess.

Abscess↗
PubMed2001

Orbital involvement in sickle cell disease: a report of five cases and review literature.

PURPOSE: To present five cases of orbital infarction in sickle cell disease and review relevant literature. METHOD: We reviewed the hospital records of 5 patients with sickle cell disease who developed a periorbital swelling during a vaso-occlusive crisis and were managed at our hospital between April 1992 and June 2000. RESULTS: The 5 patients (4 with homozygous sickle cell disease and 1 with sickle cell-beta-thalassaemia disease) were aged 6-15 years with a history of multiple admissions for vaso-occlusive crises. The periorbital swelling spread to the orbit in 4 cases and resulted in proptosis (2 cases), restriction of ocular motility and visual impairment. In all 4 cases, computed tomography and/or magnetic resonance imaging of the orbits showed a mass adjacent to the orbital wall. In 2 cases the mass was identified as a haematoma. Orbital wall infarction was demonstrated in 3 cases by bone/bone marrow scintigraphy. Epidural haematomas were detected by computed tomography in one case. All patients received intravenous fluids, analgesics, broad spectrum antibiotics and steroids, as well as simple or exchange transfusion, and responded well to medical management. CONCLUSIONS: Infarction of orbital bones during vaso-occlusive crises in sickle cell disease presents acutely with a rapidly progressive periorbital swelling. Haematomas frequently complicate the condition and, along with the inflammatory swelling, may lead to orbital compression syndrome. The condition is therefore sight-threatening, and necessitates prompt diagnosis and appropriate management for resolution without adverse sequelae. Imaging techniques are invaluable in the evaluation of patients. The majority of cases resolve with conservative treatment that includes steps to combat the vaso-occlusive crisis and use of systemic steroids under antibiotic cover.

Adolescent↗
PubMed2004

Nasopharyngeal carcinoma with orbital invasion.

PURPOSE: To review the clinical profiles of patients having nasopharyngeal carcinoma (NPC) with orbital invasion, which occurs more frequently in patients with Chinese and south-east Asian racial backgrounds. Although this disease frequently spreads superiorly to the skull base and intracranium, it rarely invades the orbit and we wish to examine some cases in which it has. METHODS: In this observational case series, we examined 406 patients, who were diagnosed with orbital tumour at Taipei Veterans General Hospital between January 1974 and December 2001. In all, 13 of the patients (11 males, two females) were found to have NPC with orbital invasion. Neuroimaging studies, pathologic reports, and clinical courses of these 13 patients were then reviewed retrospectively and survival analysis was carried out using a Kaplan-Meier estimate. RESULTS: One patient presented with eyelid tumour and three other patients presented with proptosis as the initial manifestation of NPC. The other nine cases, already diagnosed to have NPC, visited our department with a variety of ocular symptoms and signs, such as proptosis, diplopia, visual impairment, and orbital pain. Palliative treatment with radiotherapy alone or in combination with chemotherapy was given in 12 cases. During the follow-up period ranging from 4 to 64 months, six patients died, one was lost during follow-up, and six were still alive. CONCLUSIONS: Although orbital invasion in NPC is rare, we found a 5-year survival rate of 28% in our patients, indicating that orbital invasion with NPC confers a particularly poor prognosis.

Adult↗
PubMed2006

Endoscopic transnasal orbital decompression for visual failure due to sphenoid wing meningioma.

AIM: To review a group of patients with sphenoid wing meningioma and visual impairment. The technique and results for endoscopic transnasal decompression of the orbital apex is presented. PATIENTS AND METHODS: Patients presenting between 1993 and 2004 with visual impairment due to sphenoid wing meningioma were identified. All patients had full ophthalmic assessment, automated visual field testing, and appropriate orbital imaging. Patients with clinical evidence of significant progression in visual impairment (loss of acuity, reduced colour discrimination, or field deterioration) underwent endoscopic transnasal orbital decompression. OUTCOME MEASURES: Assessment of visual function (Snellen acuity, Ishihara colour perception, and visual field testing), together with other measures of orbital structure and function (ocular balance, ductions, and exophthalmometry). RESULTS: Twelve patients (all women) presented to the Orbital Clinic between the ages of 38 and 71 years (mean 42.6; median 48.5 years) and with symptoms for an average of 32 months (3-102 months; median 22). The most common symptom was swelling and proptosis, in 11 (91%) patients, and subjective deterioration of vision had been noted in six cases (50%). Eight right orbits (67%) were affected and the preoperative acuity varied between Snellen 6/5 and counting fingers, with visual field impairment in all cases. There was an average of 5.0 mm of axial proptosis (range 5-9 mm; median 5). Endoscopic decompression was without complication in all cases. With a follow-up interval of 33.9 months (range 5-80; median 26 months), there was a subjective and objective improvement in visual functions-with Snellen acuity improving between 1 and 4 lines in seven patients, reduction in relative afferent pupillary defect in 10/12 patients, and improved visual field testing. There was a 2.3 mm reduction in proptosis (1-4 mm; median 3 mm) with less lid swelling, improved ocular balance and motility in four patients, and slightly worse diplopia in one patient. Three patients required further procedures: one had ipsilateral middle meatal antrostomy for retained secretions at 18 months and two had strabismus surgery. Three patients underwent fractionated radiotherapy for large tumours, or for late tumour growth and recurrent visual impairment. CONCLUSION: Orbital decompression by transnasal endoscopic ethmoidectomy appears to alleviate optic nerve compression due to sphenoid wing meningioma, with a reasonable relief of the condition for some years. Some patients will require later radiotherapy for progressive tumour growth or visual failure.

Adult↗
PubMed1999

Current treatment and outcome in orbital cellulitis.

BACKGROUND: Bacterial orbital cellulitis is an uncommon condition previously associated with severe complications. The purpose of this paper is to describe current investigations and treatment in orbital cellulitis, establish their effectiveness and to describe the incidence of complications and surgical intervention. METHODS: A chart review of all patients admitted to the Royal Victorian Eye and Ear Hospital and the Royal Children's Hospital, Melbourne with a diagnosis of orbital cellulitis for the period July 1993 to July 1997. RESULTS: A total of 52 patients fulfilling the diagnostic criteria for orbital cellulitis were identified. Paranasal sinus disease was the commonest predisposing cause, especially in the paediatric age group. Diagnosis was made clinically with radiological confirmation in all cases. Microbiological investigation and results varied. The commonest species isolated were Staphylococci and Streptococci. Three cases of mixed anaerobes and one of Clostridium were seen. Cultures from abscess cavities and infected sinuses gave the highest positive yield (50-100%). Blood cultures were taken in 26% of adults and in 56% of children; none was positive. Treatment was either by intravenous broad-spectrum antibiotics alone or with surgery to drain orbital abscesses, usually n conjunction with sinus surgery. No patient suffered permanent visual impairment other than one case of enucleation for endophthalmitis that had caused orbital cellulitis. There was one case of permanent ocular motility impairment, and one of meningitis. CONCLUSIONS: Despite its past history of severe morbidity and even mortality, adequately treated orbital cellulitis rarely has significant morbidity today. Paranasal sinus disease remains the commonest cause. Culture of infected paranasal sinuses or pus from abscesses is most likely to yield significantly positive results in this study. Blood cultures were not helpful.

Adolescent↗
PubMed2000

Non-traumatic orbital haemorrhage.

PURPOSE: To establish the incidence of underlying orbital vascular anomalies, the presence of systemic associations and predisposing factors, the natural history and appropriate management of patients with non-traumatic orbital haemorrhage presenting in an orbital clinic. METHODS: The records of 115 patients with a diagnosis of non-traumatic orbital haemorrhage were reviewed with regard to clinical findings, investigations, management and outcome. RESULTS: Associated orbital vascular malformations were present in 104 patients (90%). Thirteen (11%) had additional or other predisposing factors (childbirth, prolonged headstands, hypertension or coagulopathies). Six patients (5%) had no predisposing factor. Acute onset painful proptosis, associated with lid swelling or a mass, was the most common presentation. Visual acuity was reduced in 37 patients (32%) at presentation. Excluding eight patients (7%) who underwent surgery for optic nerve compression, spontaneous resolution of the haemorrhage was complete in 62%, partial in 27%, while 4% had no resolution. Final visual acuity was reduced in 23 patients (20%). CONCLUSION: The majority of bleeds are associated with some form of orbital vascular anomaly. Where no such anomaly can be demonstrated a search for an underlying systemic cause should be performed. Haemorrhages in the young were usually localized whereas those in older patients were diffuse. Orbital imaging, with a combination of computed tomography and magnetic resonance imaging, was helpful in the assessment of these lesions. Most bleeds are venous and self-limiting. Surgical intervention was rarely necessary and should be confined to those with optic nerve compromise or a localized lesion which persists.

Adolescent↗
PubMed2001

Primary liposarcoma of the orbit: a clinicopathologic study of seven cases.

Liposarcoma, the most common soft tissue sarcoma in adults, will rarely involve the orbit, either primarily or as a metastasis. We describe seven primary orbital liposarcomas, representing the largest documented series of primary orbital liposarcoma to date. Affected patients were three males and four females ranging in age from 28 to 69 years (median, 51 years). Five patients presented with painless proptosis, one patient had painful proptosis, and no details of presenting symptoms are available in one case. The site distribution was retrobulbar (3 cases), lateral orbital wall (2 cases), medial wall (1 case), and unspecified (1 case). Radiologic impression included hemangioma, lipoma, and an inflammatory process. Lesional size ranged from 2.8 to 4 cm. Five liposarcomas were purely well-differentiated, one was dedifferentiated, and one was pleomorphic in type. The well-differentiated cases comprised the following subtypes: spindle cell (2 cases), adipocytic (2 cases), and combined adipocytic/sclerosing/inflammatory (1 case). Five patients underwent orbital exenteration (one followed by radiation) and two had marginal/partial excision of their tumors. Follow-up was available for five patients, ranging from 13 to 204 months (median 65 months). Four patients showed no evidence of recurrence, including the patient with pleomorphic liposarcoma who had a long, disease-free survival (65 months) following marginal excision. One patient has had multiple recurrences following initial partial excision. One patient died of an unrelated cause with no clinical evidence of recurrence. Despite the difficulty in obtaining wide surgical margins, the small tumor size at presentation and the apparent predominance of the well-differentiated type means that the prognosis for orbital liposarcoma is generally good. In view of the various morphologic patterns that may occur, liposarcoma should be considered in the differential diagnosis of any histologically unusual mesenchymal lesion in the orbit.

Adult↗