Cases reported "Exophthalmos"

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1/20. Unilateral proptosis resulting from giant-cell arteritis.

    BACKGROUND: Giant-cell arteritis (GCA) is a systemic, inflammatory vasculopathy that affects small- to medium-sized arteries. Arterial wall inflammation results in reduction of blood flow and subsequent ischemia. Arteries of the head and neck are particularly susceptible, including the ophthalmic and posterior ciliary arteries. The eye care provider is in a position to assist with the ultimate diagnosis of GCA. CASE REPORT: A 79-year-old black man was referred to the eye clinic for evaluation of exophthalmos of the left eye. The patient reported increasing proptosis over the previous 6 months; a history of sudden, permanent vision loss of the affected eye (approximately 2 years earlier); and generalized malaise and chronic frontal headache. Examination did reveal an exophthalmic eye of approximately 8-mm difference by Hertel exophthalmometry. Fundus examination revealed optic nerve pallor O.S. CT scan revealed chronic inflammatory changes of orbital tissue, including the extraocular muscles. No compressive lesions were present. Laboratory testing indicated an elevated erythrocyte sedimentation rate. A tentative diagnosis of giant-cell arteritis was made, which was confirmed with temporal artery biopsy. CONCLUSIONS: patients with ocular complications secondary to GCA manifest several different ocular symptoms, including unilateral and bilateral intermittent blur, sudden complete vision loss, double vision, etc. This was an unusual case of GCA because the initially manifested ocular sign was unilateral proptosis. The patient probably had initial ocular complications of GCA 2 years previously, with sudden loss of vision in the left eye. The patient never sought medical attention at that time, and the unilateral exophthalmic eye resulted from chronic inflammatory orbital changes associated with GCA.
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2/20. Giant prolactinoma presented as unilateral exophthalmos in a prepubertal boy: response to cabergoline.

    We report the case of a giant prolactinoma in a 7-year-old boy, which was complicated by unilateral exophthalmos. The initial levels of prolactin (PRL) were about 80,000 microU/ml. Treatment with cabergoline (CAB) resulted in rapid normalization of serum PRL (6 weeks after initiation of treatment) and reduction of tumor size. In particular, magnetic resonance imaging (MRI), which was done 2.5 months after the patient was put on CAB, revealed tremendous improvement with a decrease in the size of the tumor which now showed no extrasellar extension. Subsequent MRI studies demonstrated further improvement. exophthalmos, anisocoria and visual fields improved. In summary, this patient represents the first report of the therapeutic use of CAB as the primary mode of treatment in a 7-year-old boy with infiltrative giant prolactinoma complicated by unilateral exophthalmos. It is a noninvasive treatment that can preserve and restore vision, as well as pituitary function, and is preferable to surgery or radiation in the treatment of prolactin-secreting macroadenoma in childhood and adolescence.
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3/20. A giant prolactinoma presenting with unilateral exophthalmos: effect of cabergoline and review of the literature.

    We report the case of a 45-year-old male presenting with unilateral exophthalmos due to a large tumoral mass invading the skull base. Ophthalmologic examination did not show any visual field defects. Imaging techniques demonstrated extension of a huge tumor (approx. 8x8x8 cm) into the right orbit and nasopharynx. Endocrine work-up revealed grossly elevated serum prolactin (PRL) levels (26,466 microg/l, nl. < 12), pointing to a large, invasive macroprolactinoma. Stimulation tests indicated associated partial adrenal and growth hormone deficiencies. Planned surgery was abandoned, and the patient was instead treated with the long-acting dopamine agonist, cabergoline. Over a period of one year, serum PRL dropped to 131 microg/l, while the tumor mass shrank to less than 50% of its original volume (with 3.5 mg/week of cabergoline). The exophthalmos disappeared, and the patient did not develop rhinorrhea or any other side effects from treatment with cabergoline. The efficacy was maintained throughout the second year (ultimate serum PRL 74 microg/l, and final size less than 10% of the original). With reference to this case, we review other macroprolactinomas reported in the recent literature for associated exophthalmos, grossly elevated serum PRL levels (> or = 15,000 microg/l), and/or "giant" size (> or = 4 cm in maximum diameter). We highlight the use of dopamine agonists in the treatment of prolactinomas with such unusual characteristics.
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4/20. erdheim-chester disease: two cases of orbital involvement.

    erdheim-chester disease (ECD) is an increasingly recognised form of fibro-inflammatory process characterised by xanthomatous histiocytes containing large amounts of ingested lipid, plasma cells, macrophages and Touton-type giant cells. Ophthalmic involvement in ECD has been reported in only 22 cases. We describe two patients, one presenting with diabetes insipidus and subsequently developing orbital pseudotumours and retroperitoneal fibrosis, the other presenting with exophthalmos and diplopia. The first patient was treated with cladribine and subsequently developed sudden onset of bilateral blindness while the second required radiation therapy for the retro-orbital process and developed radiation retinopathy. These cases typify the variable presentation and course in patients with ECD.
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5/20. Juvenile xanthogranuloma of the orbit in an adult.

    We present a case of juvenile xanthogranuloma (JXG) with unilateral involvement of the orbit and eyelid and proptosis, histologically confirmed in a 32-year-old man with a 1-year history of a pansinusitis and dacryoadenitis with rhinitis. Nine months later an infiltration of the anterior upper part of the right orbit and right eyelid appeared. Computed tomography scan and magnetic resonance imaging studies confirmed the presence of pansinusitis and infiltration. The patient underwent a blepharoplasty and excision of the infiltrated tissues of the orbit, eyelid, and levator muscle. hematoxylin-eosin and immunohistochemical studies revealed features consistent with a diagnosis of JXG (Touton giant cells). JXG, a non-Langerhans'-type benign proliferation, is a rare condition in adulthood.
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6/20. Orbital intradiploic giant epidermoid cyst.

    We report a case of an intradiploic epidermoid cyst of the lateral orbital wall that resulted in proptosis and downward displacement of the left eye. Computed tomography and magnetic resonance imaging revealed a bone-destroying mass involving the frontal, sphenoid, and zygomatic bones that extended into the orbit, temporalis, and anterior cranial fossa. Complete surgical removal was performed through a lateral orbitotomy. The lateral wall was reconstructed with a porous polyethylene sheet. The cyst has not recurred after 18 months of follow-up.
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7/20. Giant ependymal cyst of the temporal horn -- an unusual presentation. Case report with review of the literature.

    Primary benign cystic lesions in the brain are uncommon. However, extracerebral cysts like arachnoid cyst, epidermoid cyst and craniopharyngiomas are fairly common lesions. Also, colloid cyst in the third ventricle, dermoid cyst and endodermal cyst in the extracerebral location are not uncommon. On the contrary, intraventricular ependymal and choroidal cysts in the intraventricular location are infrequent. Surgical intervention is warranted in cysts, which produce a mass effect and raised intracranial pressure. We present an interesting case of a giant intraventricular ependymal cyst in the temporal horn in a 14-year-old boy, who presented with recent onset of headaches and epilepsy. He also had long-standing progressive proptosis of the left eye and left temporal bossing. Excision of this cystic lesion was curative. Interesting clinical and neuroimaging features are presented.
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8/20. Mixed mesenchymal differentiation in meningiomas.

    Five intracranial meningiomas showing mixed mesenchymal differentiation are described. Three contained cartilage, three contained bone, four contained hyaline fibrous strands or nodules which in two instances were calcified, three contained angiomatous areas, one contained pericytoma-like areas, four contained pleomorphic and sometimes multinucleate giant cells, and one contained a mucoid matrix including spheroidal cells superficially resembling chordoma. The importance of recognising such tumours is emphasised as they can be misdiagnosed as metastatic deposits, particularly in a frozen section.
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9/20. Giant frontal sinus mucocoele with intracranial extension and orbital displacement in an elderly Nigerian.

    Mucocoele of the frontal sinus presents with initial sign of forehead swelling in about 10% of cases, and cases with huge intracranial extension have been reported to be rare. We present a case of a giant frontal sinus mucocoele with intra-cranial and intra-orbital extensions exerting a mass effect on the anterior cranial fossa in a 78 year old Nigerian female with resultant forehead swelling, proptosis and total blindness of the left eye. diagnosis was made on clinical and radiological basis, and enucleation using coronal flap approach was done. Immediate repair of the resultant frontal bone defect was effected through the use of a curved 0.5mm stainless steel wire fixed in 3 layers across the defective frontal bone over which the soft tissues of the forehead were undermined for primary closure on sound bone. This approach was considered more appropriate than a split rib graft in view of the patient's age. No evidence of recurrence was recorded during a one-year post-surgery follow-up, suggesting that mucocoele, regardless of size can be treated with conservative surgical approach provided all cystic lining and mucocoele are removed.
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10/20. Giant osteoma of the frontoethmoidal sinus associated with two cerebral abscesses.

    A 36-year-old woman presented with severe frontal headache, fever, left palpebral swelling, and proptosis. Radiographic studies showed a giant frontoethmoidal osteoma, that extended intracranially into the frontal lobe and was associated with two abscesses, one within the lesion and the other in the right frontal lobe. The tumour was excised and the abscesses drained.The patient made a full recovery.
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