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Pre-B-cell acute lymphoblastic leukemia presenting as an orbital mass in an 8-month-old.

PURPOSE: To present the case of an 8-month-old girl with undiagnosed pre-B-cell acute lymphoblastic leukemia (ALL) presenting as an orbital mass. DESIGN: Observational case report and literature review. METHODS: Review of clinical history, radiologic findings, histology of cervical lymph node and orbital biopsies, and follow-up. RESULTS: An 8-month-old girl presented with a 2-week history of right-sided tearing, lid swelling, proptosis, and rhinorrhea. A computed tomographic scan of the orbits revealed a homogeneous, retrobulbar orbital mass eroding into the ethmoid sinuses and nasal cavity, as well as cervical lymphadenopathy. Biopsy of the lesion and enlarged lymph node disclosed medium-sized, uniform, cytologically atypical lymphocytes in a starry sky pattern. Immunohistochemical analysis showed reactivity to antibodies against CD-10, CD-19, CD-34, and terminal deoxynucleotidyl transferase. Cytogenetic analysis of the tumor also revealed a gene rearrangement on chromosome 11q23. Based on these findings, the diagnosis of pre-B-cell acute lymphoblastic leukemia was made, and systemic as well as intrathecal chemotherapy was instituted, which resulted in rapid remission of the leukemia. CONCLUSIONS: The initial presentation of ALL as an orbital mass is exceedingly rare. To the best of the authors' knowledge, this is the earliest reported case of pre-B-cell ALL presenting as an orbital lesion.

Antibodies, Neoplasm↗

Whole-body positron emission tomography/computed tomography imaging and staging of orbital lymphoma.

OBJECTIVES: To report the use of whole-body positron emission tomography fused with computed tomography (PET/CT) for the diagnosis and staging of orbital lymphoma. DESIGN: Retrospective observational case series. PARTICIPANTS: Four patients with biopsy-proven orbital lymphoma were evaluated by 18-fluoro-2-deoxyglucose whole-body PET/CT imaging. METHODS: Positron emission tomography/CT images were studied for the presence of glucose uptake. Foci were considered suspicious based on their standardized uptake values (SUVs). Physiologic images (PET) and their anatomic counterparts (CT) were fused to allow form and function to be evaluated on the same diagnostic page. MAIN OUTCOME MEASURES: Positron emission tomography/CT images were assessed for foci with abnormally high SUVs that correlated with biopsy-proven lymphoma. RESULTS: Positron emission tomography/CT detected orbital lymphoma in 3 patients (75%). It also revealed systemic lymphoma in 2 of the 4 patients. The 2 patients found to have systemic lymphoma were diagnosed to have extranodal marginal zone B-cell orbital lymphoma of the mucosa-associated lymphoid tissue (MALT) type. Similarly, the 2 with negative PET/CT results also had orbital MALT-type lymphoma. We found that PET/CT imaging helped guide further management in all 4 patients. CONCLUSIONS: Positron emission tomography/CT should be considered as a new method of diagnosing, staging, and restaging patients with orbital lymphomas.

Aged↗

Anatomy of the orbit, lacrimal apparatus, and lateral nasal wall.

Endoscopic approaches to the orbit take advantage of key anatomic relationships that arise from the fact that the sinonasal tract and orbit are contiguous structures separated by thin bone. For the most part, the orbit is surrounded by air-containing sinuses for much of the length of three of its four borders. Thus, a thorough understanding of both sinonasal and orbital anatomy is essential for safe and efficacious surgery in this complex region. The structural features of the lateral nasal wall and medial orbit are highlighted, and relevant aspects of orbital, lacrimal, and paranasal sinus anatomy are reviewed.

Humans↗

Orbital decompression in graves' ophthalmopathy by medial and lateral wall removal.

OBJECTIVE: The objective of this study is to describe a technique for balanced orbital decompression and to analyze the results. METHODS AND MATERIALS: We conducted a retrospective study of 140 patients (276 orbits). Orbital decompression was carried out by removal of the medial orbital wall by ethmoidectomy and complete removal of the lateral wall by bringing out the entire sphenoid wing together with part of the zygomatic bone down to the inferior orbital fissure. RESULTS: One hundred thirty-six patients underwent bilateral decompression, 4 patients underwent monolateral decompression. Proptosis was reduced on average by 5.3 mm; 28 (20%) patients showed onset or worsening of diplopia. CONCLUSIONS: Medial and lateral approach allows a balanced orbital decompression. As some patients may present different degrees of proptosis and visual impairment, we stress the importance of carefully weighing the preoperative conditions of the individual patient when choosing the surgical approach.

Adult↗

Orbital abscess from an odontogenic infection.

An orbital abscess is a rare but serious complication of an odontogenic infection, which can lead to loss of vision or worse. This paper presents a case of orbital abscess secondary to an infection from the upper molar teeth, which extended to the retobulbar and posterosuperior region of the orbit, close to the superior orbital fissure. The infection spreaded to the pterygopalatine and infratemporal fossa and then to the orbit via the inferior orbital fissure. This paper reviews the clinical presentation, differential diagnosis, route of spread, value of serial CT scanning, treatment and possible complications.

Abscess↗

Congenital orbital varices causing extreme neonatal proptosis.

PURPOSE: To report a 6-week-old male with extreme proptosis caused by thrombosed orbital varices. METHODS: A 6-week-old male presented with advanced proptosis of his left eye. Computed tomography and magnetic resonance imaging studies of the orbits revealed a heterogeneous cystic mass that filled the entire left orbit. Extreme proptosis and corneal exposure prompted urgent surgical excision of the mass. Histopathologic review of the lesion was consistent with orbital varices. CONCLUSIONS: Although orbital varices usually are found in adults, they should be considered in the differential diagnosis of orbital lesions in children. Observation is usually warranted, but surgical intervention may be necessary in advanced cases.

Exophthalmos↗

Orbital xanthoma in a 9-month-old infant.

PURPOSE: To report a case of orbital xanthoma in a 9-month-old female. METHODS: A 9-month-old female presented with a mass in the superior temporal left orbit since birth. Clinically, a dermoid cyst was suspected. RESULTS: The orbital tumor was removed through an incision in the upper eyelid crease. Pathologic diagnosis was orbital xanthoma. CONCLUSION: Orbital xanthoma should be added to the differential diagnosis of orbital tumors in children.

Diagnosis, Differential↗

Rhino-orbital mucormycosis in a patient with acquired immunodeficiency syndrome (AIDS) and neutropenia.

PURPOSE: To present a case of rhino-orbital mucormycosis in a patient with AIDS and neutropenia managed without exenteration. METHODS: Case report. RESULTS: A 60-year-old African-American man with AIDS developed neutropenia that was probably secondary to antiretroviral therapy. He developed right rhino-orbital mucormycosis and was treated with right partial ethmoidectomy with debridement and liposomal amphotericin B. The infection was cured without need for orbital exenteration, although visual acuity in his right eye ultimately was no light perception. CONCLUSION: Rhino-orbital mucormycosis is uncommon in patients with AIDS. When rhino-orbital mucormycosis occurs, patients require a careful search for an underlying metabolic derangement such as neutropenia. Treatment should be aggressive, but these patients may not require orbital exenteration.

Acquired Immunodeficiency Syndrome↗

Simultaneous ipsilateral temporal fossa and orbital dermoid cysts.

PURPOSE: To describe a case of simultaneous dermoid cysts in the lateral orbit and temporal fossa. DESIGN: Interventional case report. METHODS: A 7-year-old boy with a lateral orbital mass and infratemporal fossa mass underwent computed tomography and surgical excision. RESULTS: Intraoperatively, two distinct cystic lesions were identified. The orbital lesion extended just beneath the lateral rim. The temporal fossa lesion extended posteriorly along the temporal fossa. No bony defect in the lateral orbital wall was identified, and each distinct lesion was completely excised. CONCLUSIONS: To our knowledge, this is the first reported case of multiple dermoid cysts in the orbital region. When imaging studies demonstrate separate cystic lesions and do not reveal a bony defect in the lateral orbital wall, multiple lesions should be suspected.

Child↗

Effect of steroid pulse therapy with and without orbital radiotherapy on Graves' ophthalmopathy.

PURPOSE: To report the effect of high-dose intravenous corticosteroid pulse therapy with and without orbital radiotherapy on Graves' ophthalmopathy. DESIGN: Nonrandomized clinical trial. METHODS: We selected 39 Japanese patients (age range, 22-64 years; mean, 48 years; 31 women, 8 men) who had active Graves' ophthalmopathy among 195 consecutive patients. In the first 20 patients, high-dose intravenous methylprednisolone pulse therapy (1 g per day for 3 successive days, repeated 3 times within 3 weeks) followed by 24-Gy orbital radiotherapy was performed. In the other 19 of the 39 patients, high-dose intravenous methylprednisolone pulse therapy without orbital radiotherapy was performed. Coronal computed tomography (CT) of the orbit and exophthalmometry were performed before the corticosteroid pulse therapy, and 1 and 6 months after the corticosteroid pulse therapy. The maximum coronal section area of the most hypertrophic rectus muscle in each eye was measured based on orbital CT imaging. RESULTS: Clinical findings at study entry were not significantly different between the two groups. No significant difference was found in the maximum coronal section area of the most hypertrophic rectus muscle and the results of exophthalmometry measurements in both groups before the therapy. Extraocular muscle hypertrophy was significantly reduced 1 month and 6 months after the therapy (P < .01) in both groups. However, no beneficial therapeutic effect on proptosis was observed in either group at 1 month and 6 months after the therapy. No significant difference in the therapeutic effect on extraocular muscle hypertrophy and proptosis was found between the two groups. CONCLUSIONS: Orbital irradiation after corticosteroid pulse therapy had no beneficial therapeutic effects on rectus muscle hypertrophy or proptosis of active Graves' ophthalmopathy during the 6-month follow-up period.

Adult↗

Retinoblastoma manifesting as orbital cellulitis.

Five children with previously undiagnosed retinoblastoma had signs of unilateral orbital cellulitis and were treated with systemic antibiotics, corticosteroids, or both. When retinoblastoma was finally diagnosed clinically, radiologists believed in every case that, on the basis of the soft-tissue swelling seen with computed tomography, there was orbital extension of the tumor. However, intraoperative and histopathologic observations disclosed no demonstrable orbital tumor involvement. The affected eyes all had neovascular glaucoma, and all harbored highly necrotic tumors that were contained within the eye. We concluded that orbital inflammation can occur with retinoblastoma confined to the eye and that signs of orbital cellulitis do not necessarily imply orbital extension of the tumor.

Cellulitis↗

Orbital metastasis from breast carcinoma presenting as neurotrophic keratitis.

CASE REPORT: This is a case report of an 81-year-old woman with previous breast cancer. The patient presented with a history of unilateral painless red eye and displayed on examination a nodule in the superior orbital rim, proptosis, and neurotrophic keratitis. Clinical and radiographic examinations were followed by biopsy of the orbital mass. Computed tomography displayed an extraconal mass adjacent to the left superior orbital rim extending to the soft tissues. Light microscopy findings suggested the diagnosis of orbital metastatic breast carcinoma that was confirmed by immunohistochemistry. The histopathologic diagnosis was metastatic adenocarcinoma of the breast to the left orbit. COMMENTS: This study emphasizes that orbital metastasis must be considered in patients with ocular complaints and neoplastic disease thought to be stable.

Aged, 80 and over↗

Ultrasound imaging in assessment of fractures of the orbital floor.

Twenty patients who had sustained facial trauma with clinical evidence of an orbital floor fracture were examined by ultrasound in a blinded prospective study to assess the utility of ultrasound in the diagnosis of orbital floor fractures. Ultrasound detected the presence of an orbital floor fracture with an overall accuracy of 86% and a sensitivity of 85% compared with computed tomography (CT) or direct surgical exploration of the orbital floor. This is lower than that quoted in previous reports. Although less sensitive than CT in the detection of orbital floor fractures, the technique appears useful as an adjunct to physical examination in the assessment of patients with facial trauma where an orbital floor fracture is suspected. The technique may also be useful in cases of coexistent cervical spine trauma or in assessment of uncooperative patients where CT is impracticable.

Humans↗

Serous retinal detachment in a patient with rhino-orbital mucormycosis.

BACKGROUND: Rhino-orbital mucormycosis is a difficult disease to treat. We report one case of rhino-orbital mucormycosis, complicated by serous retinal detachment, that responded to aggressive treatment. CASE: A 38-year-old man with diabetic ketoacidosis was referred to the emergency department of our hospital with fever, proptotic right eye, and complaint of lethargy. OBSERVATIONS: Fundus examination showed serous retinal detachment and focal lesions of retinitis with exudate at the inferior portion coincident with the position of opacification in the orbit on MRI. Fluorescein angiography showed pooling of dye in the detached retina and leakage from focal lesions of retinitis. We thoroughly removed the large necrotic materials in the orbit and sinus through the lower conjunctiva without enucleation or exenteration. Microscopic examination and culture of the necrotic materials that were removed from the orbit proved that the patient had mucormycosis. The serous retinal detachment improved 10 days after orbital debridement combined with intravenous and local (intraorbital) amphotericin B treatment. Visual acuity recovered to 20/50. CONCLUSION: We propose that inflammation of the sclera in close contact with necrotic fungi materials may cause serous retinal detachment.

Adult↗

Pediatric tumors of the eye and orbit.

Most ocular and orbital tumors of childhood are distinct from tumors that occur in adults. Many are congenital with early presentations. Most pediatric orbital tumors are benign; developmental cysts comprise half of orbital cases, with capillary hemangioma being the second most common orbital tumor. The most common orbital malignancy is rhabdomyosarcoma. The most common intraocular malignant lesion is retinoblastoma. Choroidal melanoma, which is common in adults, is extremely rare in children. The orbit is the most common location for metastases in children, whereas the choroid is the predominant site in adults. Pediatricians play a vital role in diagnosis of pediatric ocular tumors. They are the first to recognize ocular problems that may not be apparent to parents. It is therefore important to recognize the signs and symptoms of ocular tumors of childhood so that prompt ophthalmologic evaluation and treatment may be undertaken. Whereas the malignant tumors may be life-threatening, both malignant and benign tumors may be vision-threatening.

Child↗

[Orbital decompression for Graves' ophthalmopathy].

Graves' ophthalmopathy is a complex orbital condition with a controversial pathogenesis. It is the clinical expression of a discordance between the inextensible orbit and hypertrophic muscular and fatty elements within the orbit responding to immunological stimulation. The relationship between the orbital and its content can be improved by surgical expansion which increases the useful volume of the orbit. This procedure can be combined with lipectomy to decrease the volume of the orbital contents. We briefly recall the history of surgical decompression techniques and present our experience with Graves' ophthalmopathy patients.

Decompression, Surgical↗

Orbital apex leiomyoma with intracranial extension.

BACKGROUND: Leiomyoma is a rare, benign smooth muscle tumor of the orbit. It occasionally shows some histologic resemblance to other common tumors of the orbit like neurofibroma and schwannoma. Its location at the orbital apex is uncommon and only one case with intracranial extension has been reported so far. CASE DESCRIPTION: A nine-year-old boy presented to us with left orbital pain. Four years earlier he had undergone partial excision, elsewhere, of a tumor at the orbital apex, which was reported as a schwannoma. The computed tomography (CT) scan showed regrowth of the tumor with intracranial extension. The tumor was totally resected by an intracranial route. One year postoperatively there was no recurrence of the tumor. CONCLUSIONS: Leiomyoma of the orbit, though a benign tumor, does show regrowth after partial excision. Total excision, including any intracranial component, is advised. The cases reported so far are reviewed and the histopathology and possible etiopathogenesis of this tumor are discussed.

Child↗

Solitary fibrous tumor of the orbit. Case report and review of the literature.

BACKGROUND: Solitary fibrous tumor (SFT), which usually presents in the pleura and is thought to be mesothelial in nature, has been recently discovered in extrapleural sites, including the orbit. Presently ultrastructural studies show absence of epithelial-mesothelial features, and reactivity of the tumor cells to CD34 antigen on immunohistochemical analysis suggests the mesenchymal origin of such tumors. CASE DESCRIPTION: A 40-year-old woman had a 4-year history of progressive swelling of her right upper lid and a slow-growing palpable mass of the orbit. CT and MR imaging showed a well circumscribed, nonenhanced extraconal mass with mild erosion of the right orbital roof. The tumor was totally excised. Histological examination disclosed a spindle-cell tumor in a dense fibrous tissue. Immunohistochemistry showed positive staining for vimentin and CD34. We review the clinical, diagnostic, and surgical features of 22 orbital SFTs including the present case. CONCLUSIONS: Orbital SFT generally pursues a slow, indolent, and nonaggressive course, reaches a size up to 4.5 cm, and can be cured by a single excision. It must be immunohistochemically differentiated from other spindle-cell tumors of the orbit.

Adult↗