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Mucocoeles are cyst-like lesions of the paranasal sinuses, which may rarely erode through the bony orbit and so displace or compress orbital contents. Such patients may present to ophthalmologists and other health care professionals with diverse problems including facial pain and swelling, proptosis, double vision, ptosis, infra-orbital anaesthesia, epiphora and optic nerve dysfunction with reduced vision. We present an illustrated interventional case report of an 81-year-old gentleman with a 2-week history of swelling around the left lower eyelid and malar region associated with double vision both in the primary position and particularly downgaze. We discuss the aetiology, diagnosis and contemporary management of maxillary mucocoeles and also the Caldwell-Luc procedure. The article serves as a reminder that maxillary mucocoeles may slowly and covertly invade the orbit and should be considered in the differential diagnosis in patients presenting with proptosis, globe displacement and an orbital or adnexal mass.
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PMID:Diplopia due to maxillary sinus mucocoele. 1748 96

A 52-year-old patient presented with an orbital swelling and exophthalmos that enlarged over a period of about 40 years. The clinical examination showed massive exophthalmos and ptosis of the right eye without diplopia. The radiological investigation (MRI, CT and ultrasound) showed an unclear intraorbital mass with erosion of the orbital floor, infraorbital rim and orbital roof. The lesion was diagnosed histologically as a plexiform neurofibroma. The patient did not present any features of neurofibromatosis type 1 (NF-1) and molecular genetic analysis was unable to uncover a pathogenic sequence alteration in the NF-1 gene. Owing to the absence of clinical and ophthalmologic symptoms and the improbability of complete removal, the patient refused surgical intervention.
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PMID:Intraorbital plexiform neurofibroma in an NF-1-negative patient. 1753 94

A 65-year-old woman presented with severe periorbital pain and swelling of the left eye, with complete ptosis, proptosis, and conjunctival chemosis. The eye was in a hypotropic position, and activity in the left superior rectus was inadequate. A firm, elastic, 2-cm mass was palpated near the superior orbital rim. Computed tomography revealed a subperiosteal abscess (SPA) at the superior portion of the orbit and a large frontoethmoidal sinus osteoma. After the SPA had been surgically drained and the osteoma completely removed, the patient recovered, with resolution of proptosis, ptosis, and motility limitations. Osteomas of the paranasal sinuses are usually asymptomatic and rarely cause SPA and orbital cellulitis. Therefore, osteoma cases must be closely followed to ensure that early diagnosis and treatment of SPA are successful in preventing severe visual loss and rescuing the patient's vision.
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PMID:Frontoethmoid sinus osteoma as a cause of subperiosteal orbital abscess. 1766 Jan 65

Cholesteatoma and cholesterol granuloma are relatively rare lesions of the orbit. Both may involve the superior orbit and present with globe ptosis, proptosis, and double vision developing over weeks to years. In addition to their etiology being unknown, the nomenclature regarding these tumors historically has been confusing, with a variety of names being used to describe them. Their histopathology shares numerous similarities but has one key distinguishing feature: The cholesterol granulomas lack an epithelial lining, whereas the cholesteatomas have an epithelial lining. Surgical removal is the treatment of choice for both tumors. It is important to differentiate the two as the prognosis with recurrence differs. Cholesteatoma may recur with the possibility of malignancy found at the time of re-exploration. We present a case of each tumor type, illustrating their similarities and highlighting the histopathologic findings and treatment recommendations.
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PMID:Orbital cholesterol granuloma and cholesteatoma: significance of differentiating the two. 1788 99

Two Caucasian males (57 and 70 years old) were referred to our attention with parasinus mucoceles, maxillary and frontal mucocele, respectively, that had eroded the orbital rim and caused swelling of the eyelids and brow. Invasion of the orbital space caused several ophthalmic symptoms such as diplopia, proptosis, ptosis, and the formation of a palpable mass. Ophthalmic involvement was the first sign of the mucocele. The mucoceles were completely excised through a skin incision and the diseased mucosa of the sinuses was removed: endonasal fibre optic surgery and the Caldwell-Luc procedure were used in the patient with maxillary mucocele. The cases are described with retrospective review.
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PMID:Two anomalous localizations of mucocele: clinical presentation and retrospective review. 1795 52

Tolosa Hunt syndrome is a rare disorder caused by nonspecific inflammation in the cavernous sinus/superior orbital fissure and/or orbital apex. It is clinically characterized by alternating remissions and exacerbations, and manifested as diplopia associated with unilateral periorbital hemicranial headache. The symptoms include blepharoptosis, which is usually mild if present, bulbomotor paresis involving the pupil, and loss of sensation in the area supplied by the first division of the trigeminal nerve. Therapy for Tolosa-Hunt syndrome are systemic steroids. The course of disease in a 25-year-old man hospitalized for painful ophthalmoplegia and diplopia is presented. The history included severe pain on rightward eye movement and parabulbarly on the right, considerable defect in the area supplied by the first division of the trigeminal nerve, right hemicrania, and diplopia on looking to the left, right, upward and downward that developed after four days. A month before, the patient was observed at neurology department for severe right hemicrania. Current status included severe pain parabulbarly on the right, discrete proptosis with mild ptosis on the right eye, restricted right eye bulbar motoricity on looking nasally, nasally upward and downward, and loss of sensation in the area supplied by the first division of the trigeminal nerve. Pupilar motoricity was normal. Upon admission, neuroradiologic examination (orbit CT) and brain MR were performed, and therapy with systemic corticosteroids was initiated ex iuvantibus, in consultation with a neurologist. At 24 hours of corticosteroid therapy, the pain subsided, whereas diplopia disappeared almost completely after 5 days, now being only occasionally recorded on looking to the left and upward.
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PMID:[Painful ophthalmoplegia--Tolosa-Hunt syndrome]. 1804 75

To describe the clinical characteristics of orbital pseudotumor, a retrospective analysis was performed on patients with orbital pseudotumor at Siriraj Hospital for ten years. Forty-nine patients (24 males and 25 females; 62 eyes) with a mean age of 43.75 years were included (a mean follow-up of 25 months). Thirty-six patients (73.5%) had unilateral disease. The clinical features were proptosis (79.6%), ocular motor deficit (61.2%), pain (51%), lid swelling or lid mass (44.9%), ptosis (24.5%), and chemosis (18.4%). The most common presenting sign was proptosis (49%). All were treated with corticosteroids with clinical improvement in 40 (81.6%) patients. Ten (83.3%) of 12 patients with visual loss improved with mean recovery time of 10.3 days. Ocular motility recovered in 24 (80%) patients, occurring an average of 17.8 days after initiation of therapy. It is concluded that the clinical features of orbital pseudotumor are varied. Most patients were improved with corticosteroids treatment.
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PMID:Orbital pseudotumor: clinical features and outcomes. 1840 94

Frontal mucocele usually presents with visual complaints like diplopia, diminution of vision, visual field defect, ptosis, orbital swelling, retro-orbital pain, displacement of eye-globe and proptosis. Very rarely it can present as a subcutaneous swelling. To the best of the authors' knowledge, only two cases of frontal mucocele presenting with a forehead mass have been reported in the western literature. Authors report an unusual case of frontal mucocele in a 53-year- old female who presented with painless slowly progressive subcutaneous swelling of five-year duration on the forehead. The case is discussed and the pertinent literature is reviewed.
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PMID:Frontal mucocele presenting with forehead subcutaneous mass: an unusual presentation. 1859 39

Metastasis to the orbital soft tissues is relatively uncommon. We report a rare case of renal cell carcinoma with orbital metastasis as the first clinical manifestation. A 48-year-old-man presented with left proptosis and complete ptosis of three weeks duration. Radiological examination revealed a left intraconal heterogeneous cyst-like lesion with rim enhancement immediately deep to the left superior rectus muscle. Diagnosed as having orbital cysticercosis, he was prescribed oral albendazole and prednisolone. But there was no clinical improvement. An incisional biopsy performed showed metastatic poorly differentiated carcinoma. The patient complained of backache and weight loss in the interim. Magnetic resonance imaging (MRI) of the spine showed extensive vertebral metastasis to the thoracic and lumbosacral spine and the iliac bone, with an incidental detection of a large mass from the right kidney. Further MRI of abdomen and chest showed a large right renal mass presumed to be a renal cell carcinoma with extension into the right renal vein, intra-abdominal lymph nodes, and peritoneum. There were small nodules in the lung suggesting the possibility of pulmonary metastatic deposits. Renal cell carcinoma does not respond to chemotherapy, immunotherapy, or radiation; because of the disease's advanced stage, the patient received palliative treatment. There have been only two other reports in the literature of metastatic renal cell carcinoma in the orbit where the proptosis was the initial presenting feature similar to our case.
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PMID:Orbital metastasis of renal cell carcinoma masquerading as cysticercosis. 1883 35

Rosai-Dorfman disease is a rare benign idiopathic histioproliferative disorder usually manifesting as massive painless adenopathy. Extranodal involvement of the Central Nervous System (CNS) mimicking a skull base meningioma is rare. A 42-year-old male presented with painless, progressive left visual loss of 4 months duration. Clinically, he had a left ptosis, proptosis and ophthalmoplegia. Magnetic Resonance Imaging (MRI) of the brain with gadolinium revealed a destructive lesion of the left orbital apex, middle cranial fossa and cavernous sinus. He was treated with corticosteroids and underwent debulking. Pathology showed inflammatory infiltrate in the absence of an infectious agent, emperipolesis and a positive S100 stain was consistent with Rosai-Dorfman disease. As there was no improvement following steroids and debulking, he underwent radiation therapy with significant improvement of his symptoms. Although a rare entity, Rosai-Dorfman disease should be considered in the differential of a skull base lesion.
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PMID:Rosai-Dorfman disease manifesting as intracranial and intraorbital lesion. 1902 Aug 7


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