1/36. eosinophilic granuloma (Kimura's disease) of the orbit: a case report.BACKGROUND: eosinophilic granuloma of the soft tissue, Kimura's disease, is a benign slow-growing tumor that is manifested clinically by one or more inflammatory nodules involving mainly the face and scalp, but rarely the eye. CASE REPORT: The patient was a 32-year-old male with swelling of the left lower eyelid, marked peripheral blood eosinophilia and increased serum immunogloblin E. MRI revealed swelling of all rectus muscles of the left eye, but no tumor mass. Corticosteroid treatment reduced the swelling of the eyelid, but it recurred after corticosteroid was discontinued. Eight years later the patient returned with a complaint of increased swelling of the left lower eyelid. An elastic, nontender, soft tumor mass was palpable subcutaneously in the left lower eyelid extending into the orbit. MRI revealed a tumor mass in the left orbital space. The parotid gland was also swollen and palpable. Both tumors were resected surgically, and histopathological study revealed prominent proliferation of lymphoid follicles with germinal centers showing interfollicular infiltration by eosinophils. The pathological findings in the parotid gland were similar. The diagnosis was Kimura's disease. CONCLUSION: This patient is unique in that he had no tumor at the first examination, only swelling of the rectus muscles, and a tumor mass appeared many years later. Unilateral swelling of the rectus muscles may be one of the first signs of Kimura's disease. Not only tumor but also swelling of the rectus muscles limited ocular movement.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/36. Kimura disease of the orbit and ocular adnexa.Kimura disease (KD) is a distinct clinicopathologic entity that has been the subject of considerable confusion and debate. Although common in Asia, KD rarely occurs in non-Asian patients. Kimura disease shares both clinical and histopathologic features with angiolymphoid hyperplasia with eosinophilia (ALHE). Because of this overlap and the rarity of KD in europe and the united states, KD and ALHE have been used synonymously in the Western medical literature, as they were thought to represent variations of the same disease. Some pathologic reports have called for distinguishing KD and ALHE as two separate entities, based on their histologic features. Kimura disease occurs most commonly in the head and neck region and has been described in the orbit, eyelids, and lacrimal gland more frequently than ALHE. Because both diseases can cause proptosis, lid swelling, ocular dysmotility, or a palpable mass, they should be considered in the differential diagnosis of orbital lesions occurring in adults. We report two cases of KD involving the orbit and ocular adnexa, and review additional cases reported in the literature. The ophthalmic literature does not clearly reflect the current understanding that KD and ALHE are best considered two separate clinicopathologic entities.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
3/36. Orbital wegener granulomatosis without systemic findings.PURPOSE: To describe a case of orbital wegener granulomatosis without systemic disease. METHOD: Case report. RESULTS: A 69-year-old patient with bilateral inflammatory lacrimal gland masses underwent multiple biopsies that showed a nonspecific lymphoplasmacytic infiltrate consistent with orbital pseudotumor. After unsuccessful treatment with systemic corticosteroids and radiation, severe orbital disease rapidly progressed and the patient underwent unilateral enucleation. The enucleated specimen showed multifocal vasculitis, tissue necrosis, and granulomas consistent with wegener granulomatosis (WG). Elevated antineutrophil cytoplasmic antibody titers supported the diagnosis of WG. The patient did not have any extraocular signs of WG and continues to be disease-free systemically. CONCLUSION: The authors believe this is the first report of bilateral lacrimal gland masses presenting as a localized form of WG in the total absence of systemic disease.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
4/36. Radiological and clinicopathological features of orbital xanthogranuloma.BACKGROUND: Orbital xanthogranuloma, a diagnosis confirmed histologically, occurs rarely in adults and children. With its characteristic macroscopic appearance the adult form may be associated with a spectrum of biochemical and haematological abnormalities including lymphoproliferative malignancies. METHOD: The clinicopathological features and imaging appearances on computed tomography and magnetic resonance imaging of this condition are described in eight adults and a child. RESULTS: Radiological evidence of proptosis was present in seven patients. In all nine patients an abnormal infiltrative soft tissue mass was seen, with increased fat in six cases. All patients had associated enlargement of extraocular muscles suggestive of infiltration and five had lacrimal gland involvement. Encasement of the optic nerve, bone destruction, and intracranial extension was present only in the child with juvenile xanthogranuloma. Haematological and/or biochemical abnormalities were detected in seven patients and seven patients had other systemic diseases which were considered to have an immune basis. One patient subsequently developed non-Hodgkin's lymphoma. CONCLUSION: The investigation and management of orbital xanthogranulomas requires a multidisciplinary approach even though the diagnosis may be suspected clinically. Imaging delineates the extent of disease and involvement of local structures and may influence the differential diagnosis. The juvenile form may be more locally aggressive, causing bone destruction with consequent intracranial extension.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
5/36. Orbital drainage from cerebral arteriovenous malformations.OBJECTIVE: To describe the neuro-ophthalmic findings in patients with orbital drainage from cerebral arteriovenous malformations (AVMs). methods: We reviewed the records of 100 consecutive adult patients with cerebral AVMs who presented to our institution during a 4-year period. All patients with orbital drainage were identified, and their neuro-ophthalmic evaluations were reviewed. RESULTS: Three patients (3%) were identified with orbital drainage from a cerebral AVM. The first patient presented with typical chiasmal syndrome (reduced visual acuity, bitemporal hemianopia, and optic atrophy). magnetic resonance imaging demonstrated a large left temporal and parietal lobe AVM with compression of the chiasm between a large pituitary gland and a markedly enlarged carotid artery. The second patient presented with headaches and postural monocular transient visual obscurations. Examination revealed normal visual function with minimal orbital congestion and asymmetrical disc edema, which was worse in the left eye. magnetic resonance imaging revealed a large right parietal and occipital lobe AVM without mass effect or hemorrhage and an enlarged left superior ophthalmic vein. The third patient had no visual symptoms and a normal neuro-ophthalmic examination; a right parietal lobe AVM was discovered during an examination for the cause of headaches. CONCLUSION: Orbital drainage from cerebral AVMs is rare. Manifestations may include anterior visual pathway compression, dilated conjunctival veins, orbital congestion, and asymmetrical disc swelling.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
6/36. Ectopic lacrimal gland cyst of the orbit.Lacrimal duct cysts are not common. It is extremely rare when a lacrimal duct cyst and an ectopic lacrimal gland develop in the orbital cavity. A unique case of an ectopic lacrimal gland cyst of the orbit is presented. A 33-year-old man had a palpable mass above the inferior medial orbital rim for nearly two years. An ocular examination was normal except for a movable, firm mass found in the anterior nasal inferior orbit of the right eye. An echogram revealed a homogeneous, hypoechoic cystic mass. Computed tomography of the orbit showed a well-encapsulated lesion in the lower orbit of the right eye near the inferior rectus muscle, without bony erosion. A tense, thin-walled, clear fluid-filled cyst measuring 15 x 12 x 13 mm in size was completely enucleated without rupture by anterior orbitotomy. Pathologic examination disclosed a small nest of normal gland tissue surrounded by a cystic lesion lined with two layers of lacrimal duct epithelium cells. No recurrent signs were noticed during a 12-month period of follow-up.- - - - - - - - - - ranking = 3.5keywords = gland (Clic here for more details about this article) |
7/36. Lid swelling and diplopia as presenting features of orbital sarcoid.Sarcoid is an idiopathic multisystem non-caseating granulomatous disease with protean clinical manifestations. In the eye, the common sites of involvement are the skin of eyelid, conjunctiva, uveal tract, retina, optic nerve and lacrimal gland.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
8/36. Bilateral orbital Kimura's disease in a young Asian man.A 16-year-old Asian man adolescent presented with bilateral eyelid swelling with multiple palpable mass lesions, which waned after treatment with corticosteroids but waxed after medications were discontinued, for about 1 year. He was otherwise healthy except that bilateral postauricular lymphadenopathy had developed for about 9 years. Laboratory study revealed peripheral eosinophilia with an elevated IgE level. The tumor masses of the left orbit were completely excised through an incision similar to that used in blepharoplasty, which gave good cosmetic results. Postoperative computed tomography scan showed no residual tumor mass in the left orbit but contralateral homogeneous soft-tissue mass lesions around the lacrimal gland and extending deep into the orbit, between the superior and lateral rectus muscles. pathology reported numerous lymphoid follicles with active germinal centers and extensive lymphocyte and eosinophil infiltration, which characterize Kimura's disease (KD). The tumor mass in the right orbit was also excised during a second elective surgery 10 days later. No evidence of recurrence was noted after follow-up for 7 months. KD of the orbit is rare and usually occurs in middle-aged to elderly Asian men but can also be present in young adolescent. Complete excision with simultaneous blepharoplasty gives satisfactory cosmetic results.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
9/36. Ocular scrofuloderma with unilateral proptosis.Proptosis due to an extraconal orbital abscess of tubercular origin with lacrimal gland involvement, representing ocular scrofuloderma, is a rare entity. This association has not been reported earlier in the literature. We describe a 7-year-old boy who presented with nodulo-ulcerative lesions of tubercular etiology with discharging sinuses on right side of the face and a similar lesion on the right lower eyelid along with proptosis of 4 months duration. Computerized tomography (CT) scan of the head confirmed the extraconal, intraorbital, hyperdense, homogeneously enhancing mass separated from the lateral rectus muscle and further revealed involvement of lacrimal gland along with erosion of the temporal bone. The patient showed marked improvement of his dermatological and ophthalmological lesions with anti-tubercular treatment. Subsequent ultrasound examinations of the orbit revealed regression in the size of the abscess from 10.7 mm to 5.0 mm and then complete disappearance of the abscess obviating surgical intervention.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
10/36. Orbital sinus histiocytosis (Rosai-Dorfman disease): a lacrimal gland involvement.We report the clinical and histopathological features of 2 cases of orbital Rosai-Dorfman disease with lacrimal gland involvement but no lymphadenopathy or cutaneous involvement. The first case was a 7-year-old boy, who was referred to the ophthalmology department with a proptosis in the left eye. The second case involved a 57-year-old African man, who developed oedema of the right upper eyelid over a 4-month period. Ocular involvement in Rosai-Dorfman disease is rare (10%). The diagnosis is histological in combination with immunohistology and is often delicate, especially in extranodal localizations. Histopathologically, normal gland morphology was altered by fibrosis and inflammatory cells. To our knowledge, this is the first report on lacrimal involvement in incomplete, thus uncommon sinus histiocytosis with massive lymphadenopathy.- - - - - - - - - - ranking = 3keywords = gland (Clic here for more details about this article) |
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