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Infantile myofibromatosis of orbital bone. A case report with computed tomography, magnetic resonance imaging, and histologic findings.

OBJECTIVE: The authors report the clinical, radiographic, histologic, and surgical findings of a rare presentation of infantile myofibromatosis. A mass presenting at the inferolateral orbital rim of a 4-year-old child was imaged with both computed tomography (CT) and magnetic resonance imaging (MRI), excised surgically and analyzed histologically to confirm the diagnosis. Infantile myofibromatosis is a rare benign disorder of childhood consisting of well-demarcated nonencapsulated tumors. The overall prognosis depends on the number and location of tumors. Isolated infantile myofibromatosis of the head and neck is the most common form of this generally benign disease. DESIGN: A case review study is presented and discussed with regard to clinical, radiographic, histologic, and interventional findings. The literature is reviewed and the clinical relevance discussed. INTERVENTION: Surgical extirpation of the tumor was undertaken after sufficient localizing radiographic information was obtained. RESULTS: The tumor showed erosion of the orbital bone and orbital extension with reactive hyperostosis on CT imaging. The MRI finding showed an inhomogeneous well-demarcated dark mass on T1 images with increased signal intensity on T2 images. Gadolinium contrast showed significant vascular enhancement. Histologic sections showed a spindle-cell tumor of whorled myofibroblasts surrounding a hemangiopericytoma-like center. The tumor was excised completely. There has been no evidence of recurrence or adverse ocular sequelae in the first 6 months after surgery. CONCLUSIONS: Cases involving the orbit or cranial bones are particularly rare but can have profound secondary effects on local structures. Isolated tumors can occur in all age groups and can mimic more aggressive or malignant neoplasms. The CT and MRI findings are beneficial in establishing a differential diagnosis, plan of treatment, and prognosis. Early diagnosis is important to rule out other neoplasms. The appropriate treatment is excisional biopsy whenever possible and is considered curative. The authors present what to our knowledge is the first reported case of an isolated infantile myofibroma involving an orbital bone.

Child, Preschool↗

Repair of exposed hydroxyapatite orbital implant by a tarsoconjunctival pedicle flap.

OBJECTIVE: This study aimed to determine a surgical method of management of exposed hydroxyapatite orbital implants. DESIGN: Noncomparative small case series. PARTICIPANTS: Four patients with exposures of hydroxyapatite orbital implants are described. INTERVENTION: A superiorly based tarsoconjunctival pedicle flap is fashioned from the upper eyelid; this flap is inverted and placed over the scleral-covered, exposed hydroxyapatite orbital implant. The flap is divided as a second-stage procedure 4 weeks later. MAIN OUTCOME MEASURE: Mucosal coverage of the previously exposed orbital implant was measured. RESULTS: Four cases are presented with excellent postoperative results at 3 to 18 months' follow-up. CONCLUSION: A suitably fashioned pedicle flap from the upper eyelid is an effective method of managing exposed hydroxyapatite orbital implants.

Adult↗

Penetrating orbital injury with organic foreign bodies.

OBJECTIVE: The authors reviewed the clinical features, diagnostic workup, and management of patients of penetrating orbital injuries with retained organic foreign bodies. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Nineteen patients (15 males, 4 females) with penetrating orbital injuries due to organic foreign bodies. RESULTS: The series included 15 (78.9%) males and 4 (21.1%) females who ranged in age from 6 months to 40 years (mean = 14.6 years); 12 (63.2%) patients were younger than 12 years of age. Twelve (63.2%) right and 7 (36.8%) left orbits were involved. Time between injury and presentation varied from a few hours to 9 months. Most common injury site was the superior orbit in 11 (57.9%) patients leading to abnormal extraocular motility (84.2%), proptosis (68.4%), and upper lid ptosis (47.4%). Associated pathologies also included acute cellulitis in 11, orbitocutaneous fistula in 5, and osteomyelitis in 2 patients. Preoperative computed tomography (CT) and magnetic resonance imaging (MRI) identified the foreign bodies in 42% and 57% of the patients, respectively. CONCLUSION: Preoperative identification of the foreign material in the orbit was found to be very helpful for patient management but was only possible in approximately 50% of our cases with the use of CT and MRI. The vision in our patients usually improved shortly after treatment; the long-term complications more often included extraocular muscle and eyelid motility problems and periorbital scarring.

Adolescent↗

Orbital infarction and melting in a patient with systemic lupus erythematosus.

OBJECTIVE: To present a patient with systemic lupus erythematosus who developed infarction and melting of the orbit secondary to her systemic disease. DESIGN: A case report. PARTICIPANT: A 61-year-old white woman with a 5-year history of systemic lupus erythematosus. METHODS: The patient presented with left orbital pain, limitation of extraocular movements, and a fistula from the ethmoid sinus to the upper eyelid. A detailed examination with computerized tomography, ultrasound, and a comprehensive medical evaluation with laboratory testing was performed. Histopathologic analysis with special stains of the orbital tissues was also performed. RESULTS: Histopathologic examination of the biopsy specimens revealed the features of an inflammatory process involving the orbit, similar to a panniculitis. These include a lymphocytic reaction with a predominance of plasma cells, vasculitis with occlusion, and thickening of the vessel walls, necrosis, and hyalinization of fat. CONCLUSION: This is a unique case in which infarction and melting of the entire orbital structures occurred in the presence of systemic lupus erythematosus. The underlying disease process is a lupus-related panniculitis. The authors stress that this is a very rare entity and that other diseases should be ruled out before entertaining this diagnosis.

Eye Diseases↗

Management of medial subperiosteal abscess of the orbit in children--a 5 year experience.

A medial subperiosteal abscess (SPA) of the orbit is the most common serious complication of sinusitis in children. The distinction between SPA and the more benign pre-septal disease is difficult to make especially in a young child in whom an ophthalmological evaluation is often difficult. Computerised tomography (CT) is the investigation of choice in making this distinction. Subperiosteal inflammatory disease of the orbit is initially treated with intravenous antibiotic therapy with surgery reserved for those patients who do not respond to medical treatment and in whom a medial SPA is confirmed by CT. Conventionally, the abscess is drained via an external incision and an ethmoidectomy is performed at the same time. More recently, successful drainage of SPA's has been accomplished endoscopically via a intranasal approach with less morbidity and superior cosmesis. We present a 5 year experience of 24 patients with CT scans suggestive of medial SPA who underwent endoscopic exploration of the medial subperiosteal orbital space. We discuss the current management of medial subperiosteal disease of the orbit in children and include a review of the literature. Also included is a clinical staging system which aids the management of orbital complications of sinusitis.

Abscess↗

[Orbital meningoencephalocele: two case studies].

Primitive orbital meningocele is a rare congenital malformation. It is defined as a herniation of meninges into the orbit through a congenital defect in the orbital bones. In meningoencephalocele, there is atrophic brain tissue in the herniated meningeal sac. We report the cases of two babies who presented with a medial orbital tumoral syndrome. Computed tomography showed a cystic tumor close to a bony defect in the anterior frontoethmoidal junction. A transcranial approach confirmed the diagnosis of meningoencephalocele; it was resected and the bony and dural defects were closed. The outcome was uneventful. Orbital meningoencephalocele is probably linked to an abnormal closure of the rostral neuropore. It generally presents in the first months of life with very suggestive radiological and clinical features. Surgical treatment using a transcranial approach is indicated in all cases and provides definitive recovery.

Encephalocele↗

Nontraumatic prolapse of the orbital contents into the ethmoid sinus: evaluation with screening sinus CT.

PURPOSE: The purpose of this study is to evaluate the incidence and CT features of nontraumatic prolapse of the orbital content into the ethmoid sinus, which may be a cause of orbital complication of ethmoidectomy. MATERIALS AND METHODS: CT of 1,024 consecutive patients with subjective sinonasal symptoms who underwent screening sinus CT were reviewed and analyzed. The 573 men and 451 women were between the ages of 5 and 79 years old. The patients with a history of sinus surgery of facial trauma were excluded from this study. The incidence and CT features of dehiscence and/or bowing of the lamina papyracea with prolapse of the orbital content in each age group and differences between the age groups were analyzed. RESULTS: Ethmoidal prolapse of the orbital content was found in 67 patients (6.5%). There was a tendency of increasing incidence with increasing age. The incidence in the juvenile age group was significantly low (1.9%) as compared with that of adult. Four patients (0.4%) had bilateral lesions. Twenty-nine lesions showed prolapse of moderate to severe degree, and deformity of the medial rectus muscle on coronal CT image was associated in 21 lesions. Inflammatory change of adjacent ethmoid sinus was indicated in 19 lesions, and there was no statistical difference in incidence from that of the opposite side. CONCLUSIONS: The incidence of nontraumatic prolapse of the orbital content is far beyond general expectations, and detailed structural information with CT for this anatomic alteration may be important before endoscopic sinus surgery to avoid possible complication. The higher incidence in adult may suggest age-related acquired etiologic factors besides congenital dehiscence or weakness of the lamina papyracea.

Adolescent↗

Blow-out fracture of the orbit. Diagnostic and therapeutic considerations, and results in 90 patients treated.

On the basis of 90 patients with blow-out fracture of the orbit the authors present diagnostic methods, the treatment and follow-up results, mainly as regards visual function (diplopia) and aesthetics (enophthalmos). Surgery was performed in 77 cases, 6 patients were treated conservatively by eye muscle training, and 7 patients refused surgery. Surgery consisted in primary reconstruction of the orbit, exploration of the fracture site, freeing the entrapped soft tissues (periorbital tissues, eye muscles) and reconstruction of the defect with autogenous bone graft, followed by eye muscle surgery in cases of failure. After surgery no complications were observed. Results of treatment were evaluated by established methods elaborated by the authors, in the same order before and after surgery. Of 77 patients treated surgically recovery was obtained in 63.6%, marked improvement in 23.4%, slight improvement in 13.0%. In conclusion the authors state that: 1) in all cases ophthalmological examination is most important, 2) approach to the orbital floor or medial wall is best obtained by transconjunctival or subciliary incision, 3) in cases with a defect with a defect of the orbital floor the best material is autogenous bone graft. In linear fracture of the orbital floor and medial wall it is sufficient to free the entrapped tissues, 4) in all surgical patients eye muscle training proved effective.

Adolescent↗

Primary radiotherapy for localized orbital MALT lymphoma.

PURPOSE: To define the natural history, prognosis, and radiocurability of localized orbital extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT). METHODS AND MATERIALS: Clinical records and pathologic material of 40 patients treated with local radiotherapy for localized orbital lymphoma were reviewed. Treatment consisted of 30-40 Gy in 1.8-2-Gy fractions (mean 34 Gy) of irradiation using 9-20-MeV electrons for conjunctival lesions, or 6-MV photons with complex treatment planning for retrobulbar lesions. The lens was routinely shielded with the use of a suspended eye bar. RESULTS: Upon pathologic review, 31 cases of orbital MALT lymphoma were identified. With the median follow-up of 5.9 years (range 9 months-0.3 years), the actuarial 10-year overall survival was 73%. Local control was 100%. Five distant failures resulted in a projected 10-year freedom from relapse of 71%. Most of the failures were extranodal in sites where MALT lymphoma has previously been shown to arise. No difference in outcome was observed among patients treated to less than or equal to 34 Gy vs. those treated to higher radiation doses. Two patients experienced clinically significant retinal damage after doses > or = Gy. CONCLUSION: In this study, localized orbital MALT lymphoma was well controlled with radiotherapy. Even following relapse, patients with orbital MALT lymphoma exhibited an indolent course. Relapse occurred predominantly in extranodal mucosal sites, implying a possible homing mechanism for MALT lymphoma cells. Given the excellent local control rates, our current treatment recommendation is to use a radiation dose of 30-30.6 Gy in 1.5-.8-Gy fractions to minimize risk of late toxicity.

Adult↗

Radiotherapy in the management of orbital lymphoma.

PURPOSE: This retrospective study reviews the treatment technique, disease outcome, and complications of radiotherapy used in the management of lymphoma involving the orbits. PATIENTS & METHODS: Thirty-eight patients were treated between May 1969 and January 1995, with a median follow-up of 8.3 years. All patients had biopsy-proven orbital lymphoma. Twenty patients who had limited disease were treated with curative intent, and 18 patients who had known systemic disease were treated with palliative intent. Of the 20 patients treated with curative intent, 14 had low-grade and 6 had intermediate- or high-grade disease. None received chemotherapy. Most patients received treatment with 250 kVP or 60Co radiation, using either an en face anterior field or wedged anterior and lateral fields. Median treatment dose was 25 Gy. Lens shielding was performed if possible. For patients treated for cure, cause-specific survival and freedom from distant relapse were calculated using the Kaplan-Meier method. RESULTS: Control of disease in the orbit was achieved in all but 1 patient, who developed an out-of-field recurrence after irradiation of a lacrimal tumor and was salvaged with further radiotherapy. In the patients treated curatively, the 5-year rate of actuarial freedom from distant relapse was 61% for those with low-grade and 33% for those with intermediate/high-grade disease (p = 0.08). Cause-specific survival at 5 years was 89% for patients with low-grade and 33% for those with intermediate/high-grade disease (p = 0.005). Two patients with low-grade disease had contralateral orbital failures; both were salvaged with further irradiation. Acute toxicity was minimal. Cataracts developed in 7 of 21 patients treated without lens shielding and 0 of 17 patients treated with lens shielding. No patient developed significant late lacrimal toxicity. CONCLUSION: Radiotherapy is a safe and effective local treatment in the management of orbital lymphoma.

Adolescent↗

Subperiosteal orbital hemorrhage associated with chronic sinusitis: a case report and review of the literature.

A case of subperiosteal orbital hemorrhage associated with chronic sinusitis is reported. A 57-year-old woman had a sudden onset of severe orbital pain and exophthalmos in the left eye. Computed tomography revealed a discrete round mass in the left retro-orbital space. An external frontoethmoidectomy was performed to evacuate the hematoma of the subperiosteal space. Additionally, the mucosa was edematous in the left ethmoid sinus. It appears that subperiosteal orbital hemorrhage in this case was caused by rupture of vessels which were inflamed as a result of sinusitis. Orbital hemorrhage associated with chronic sinusitis has rarely been reported. Diagnosis and treatment are discussed and etiologic factors are reviewed.

Ethmoid Sinus↗

Systemic diseases involving the orbit.

A variety of systemic pathological processes may involve the eye and the surrounding orbital adnexae. CT and MRI of the orbit have become useful clinical adjuvants, not only in establishment of the diagnosis, but also in suggesting the appropriate clinical treatment, sometimes preventing needless biopsy. Grave's disease, Wegener's granulomatosis, and sarcoidosis may all present initially with orbital disease before the onset of systemic manifestations. Idiopathic inflammatory disease of the orbit and lymphoproliferative disease of the orbit continue to remain radiological and clinical challenges. The article discusses the CT and MRI appearance of many of the common systemic diseases in adults with attention to features useful in clinical practice as well as in differential diagnosis.

Adult↗

The Groote Schuur hospital classification of the orbital complications of sinusitis.

The complications of sinusitis have been well described. The most common classifications used for orbital complications have been that of Chandler et al. (1970) and Moloney et al. (1987). With the ready availability of high-resolution computed tomography (CT) scanners, limitations of these classifications have become apparent. The aims of this study were to determine the relative frequency of the various complications associated with acute sinusitis, to determine which groups of sinuses were most frequently involved and to correlate the orbital signs with a new proposed classification of orbital complications. Over a five-year period, 87 consecutive patients were admitted with acute sinusitis. Sixty-three patients (72.4 per cent) had one or more complications. When orbital complications were classified under the proposed classification, all patients with proptosis and/or decreased eye movement had post-septal infection. Visual impairment occurred only in the post-septal group. Most complications had a combination of sinus involvement with the maxillary/ethmoid/frontal combination being the most common. The authors propose a modification of Moloney's classification for orbital complications of acute sinusitis that allows a clear differentiation between pre- and post-septal infection and a radiological differentiation to be made between cellulitis/phlegmon and abscess formation. The latter is of importance when a decision is made on whether surgical intervention is appropriate or not.

Acute Disease↗

Endoscopic orbital decompression for dysthyroid eye disease.

Orbital decompression for dysthyroid eye disease is traditionally performed either through an external approach or transantrally. The introduction of endoscopic sinus surgery over the last decade has led to a better understanding of the intranasal surgical landmarks of orbital walls and the development of endoscopic orbital decompression. We have adopted the endoscopic technique as the treatment of choice for orbital decompression in dysthyroid eye disease for the last two years. The surgical technique is described and illustrated, and the results of our first series of 15 patients (30 orbits) are presented.

Adult↗

Primary palpebral and orbital ossification in pseudo-pseudohypoparathyroidism.

PURPOSE: To present a case of ossification of the eyelid, episclera and orbit in a patient with pseudo-pseudohypoparathyroidism (pPHP). METHODS: A 20-year-old woman diagnosed with pseudo-pseudohypoparathyroidism underwent clinical and histopathological examination of calcified plaques of the right eyelid and orbit. The patient presented with a round face, tousled short hair and retarded speech. She had been diagnosed with pPHP at 3 years of age. During her first decade, calcified plaques developed in the right eyelid and orbit. Gradually, she developed horizontal diplopia, pseudo-ptosis and periorbital pain. Vertical eye movements were reduced to 10 mm, although levator function remained intact. Computer tomography scans of the orbit showed three separate dense structures. Radiographic findings also showed bilateral shortening of the fourth metacarpus and a calcified subcutaneous plaque in the left thigh. The patient's blood status revealed an elevated level of thyroid stimulating hormone, but was otherwise normal. The patient was treated with eltroxin and shortly afterwards regained normal hair and normal speech function. RESULTS: The calcified structures were removed surgically and almost normal eye movements were re-established. Histological examination of the excised tissue demonstrated bone formation. CONCLUSION: This is the first reported case of ossification of the eyelid and orbit in a patient with pseudo-pseudohypoparathyroidism.

Adult↗

Ocular morbidity after radiotherapy of orbital and conjunctival lymphoma.

One hundred and fifteen patients were treated for lymphoma of the conjunctiva and orbit between 1970 and 1984. One hundred and twelve patients received radiotherapy to the orbit, of whom 73 (65 per cent) had at least one year of follow-up by an ophthalmic surgeon. Patients with low-grade lymphomas (97) mostly received 30 Gy in 15 fractions to the orbit over a period of 3 weeks. Those with high-grade lymphomas (18) mostly received 40 Gy in 20 fractions over 4 weeks. Most were treated using anterior and lateral radiation fields to the orbit with shielding of the cornea and lens. This technique delivered a mean lens dose of 15 Gy. The early and late ocular and orbital morbidity in these patients was low. Eight radiation-induced cataracts developed of which only six interfered with vision. None has needed cataract surgery. No patients developed radiation retinopathy and only 5 had disorders of ocular lubrication.

Adolescent↗

Orbital ultrasound in the ocular ischaemic syndrome.

The ocular ischaemic syndrome illustrates well the effects of hypoperfusion of the globe and is a useful model for studying disorders of the orbital circulation. Recent advances in orbital ultrasound techniques have provided a non-invasive method of examining flow velocities in the orbital vessels, particularly the ophthalmic artery. Orbital ultrasound studies were performed on 3 cases of the ocular ischaemic syndrome. Continuous or intermittent reversal of blood flow in the ophthalmic artery was seen in all cases. How this phenomenon relates to the symptoms and signs in affected patients, and the role of orbital ultrasound in the investigation of the ocular ischaemic syndrome, are discussed.

Aged↗

The incidence of diplopia following coronal and translid orbital decompression in Graves' orbitopathy.

PURPOSE: Firstly, to assess the incidence of induced diplopia following orbital decompression in patients with Graves' orbitopathy. Secondly, to assess patient satisfaction after orbital decompression. Thirdly, to determine the factors that contribute to the variable reported incidence of diplopia complicating decompression surgery. METHODS: We present a retrospective analysis of the alterations of ocular motility in a consecutive series of 81 patients with Graves' orbitopathy who underwent orbital decompression by either a coronal or a translid approach. We assessed patient satisfaction by a telephone survey, and we reviewed the literature. RESULTS: Eleven patients underwent decompressive surgery for dysthyroid optic neuropathy (DON); 5 of them had a three-wall coronal decompression, the other 6 had a two-wall translid decompression. One of the 5 (20%) coronal versus 2 of the 6 (33%) traslid patients experienced worsening of their existing diplopia. Seventy patients underwent surgery for disfiguring proptosis; 41 of them had a coronal decompression and 29 had a translid decompression. Eight of the 41 coronal patients (20%) and 4 of the 29 translid patients (14%) experienced aggravation of their motility impairment. There was no statistically significant difference between these percentages (chi-squared, p > 0.05). Three of 26 coronal patients (12%) without pre-operative motility impairment developed diplopia in all directions. Twenty-five per cent needed strabismus surgery (9% multiple times). High satisfaction scores were noted after both types of orbital decompression. Through a review of the literature, several factors that may add to heterogeneous results were identified, including definition of diplopia, inclusion criteria and type of surgery. CONCLUSIONS: Induced diplopia is seen after any type of orbital decompression (19% overall), and its incidence is determined by various factors. To facilitate comparative studies between decompression techniques, a standardised protocol for orthoptic evaluation should be developed.

Adult↗