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On 7 July 1993, a 61 year-old female presented a seven-day history of double vision and retro-ocular pain. Neurological examination on admission demonstrated right oculomotor palsy and pain in the right frontal nerve. Routine CT scan and MRI disclosed no abnormalities. Her symptoms deteriorated progressively and increased in severity, resulting in complete ptosis and visual disturbance in the right eye, for which she was hospitalized. Angiography also failed to demonstrate any lesion. Herrical CT scan demonstrated a small mass lesion at the right orbital apex. A frontotemporal extradural approach operation was performed on July 6, 1993. The anterior clinoid process and minor wing of the sphenoid were drilled to expose the optic canal and the superior orbital fissure. When the periorbita just beside the optic sheath was opened, a large vein was observed to be impinging on the optic nerve and nerves which pass through the superior orbital fissure. This vein was carefully dissected, coagulated and removed. The postoperative course was uneventful. Her severe retro-ocular pain completely disappeared immediately after the operation. Although complete remission of ptosis and return of eye movements to normal were noted, her visual acuity is at present limited to hand movement. Orbital varix is a common cause of unilateral intermittent proptosis. However, there has been no report in the literature of orbital varix with orbital apex syndrome as the initial clinical appearance. In this type of patient with orbital varix and retrobulbar hemorrhage or impairment of intraorbital nerves, surgical intervention using a transcranial approach to the lesion should be undertaken.
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PMID:[Orbital varix presenting orbital apex syndrome]. 787 40

A 61-year-old woman had gradual onset of ptosis and painless proptosis of her left eye of 1-year duration. Orbital computed tomography (CT) showed a well-circumscribed superior nasal orbital tumor with uniform density. The mass was removed by anterior orbitotomy and histopathologically diagnosed as a conjunctival cyst. Recent reports have increased awareness of this entity. This case typifies findings of conjunctival orbital cysts. These cysts should be considered when a differential diagnosis is formulated for orbital tumors with a similar presentation.
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PMID:Conjunctival orbital cyst. 794 52

A surgical case of monostotic fibrous dysplasia of the left frontal and sphenoidal bone in a 14-year-old girl is described. This girl was admitted to our hospital in March, 1992, with a chief complaint of facial deformity and asymmetry due to a painless and progressive bony bulging over the left fronto-orbital region. But she denied any symptoms such as proptosis, diplopia, optic atrophy and visual loss. Other data found on neurological examination and laboratory tests were normal. In addition, she had no history of skin lesions, precocious puberty or other endocrine abnormalities. Plain craniogram showed remarkable thickening of the left frontal bone and of the anterior cranial fossa of the sphenoidal bone with irregular stenosis of the left optic canal. CT scan showed the diffuse enlargement of the affected bone and involvement of the paranasal sinuses. Angiography revealed no positive findings. On December 10, 1992, orbito-cranial reconstruction and unilateral optic canal release were performed using an extradural approach through a left fronto-temporal craniectomy. Histological findings confirmed the lesion to be typical fibrous dysplasia. She recovered completely one month after the operation, but she suffered transient blurred vision, diplopia and left ptosis. Most of the decreased vision caused by fibrous dysplasia cannot be reversed after surgical treatment. So, if optic canal stenosis is evident, even when visual loss is not clear, release of the optic canal stenosis should be done as early as possible in association with experienced neurosurgeons and with meticulous dissection.
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PMID:[Orbito-cranial reconstruction and unilateral optic canal release in a patient with left fronto-orbital fibrous dysplasia: a case report]. 796 64

Partial ophthalmoplegia due to third nerve palsy with an intact pupil is a frequent cause of diploplia observed in diabetic patients. Pupillary muscle involvement, such as anisocoria and loss of light reflex, is usually uncommon in this diabetic cranial mononeuropathy. A 65-year-old woman with non-insulin dependent diabetes mellitus (NIDDM) suddenly developed a severe headache and diplopla. Right oculomotor nerve palsy was observed in association with anisocoria, ptosis of the right lid, and a defective light reflex. No exophthalmos or vascular bruit was observed in the right orbital region. Computed tomography and magnetic resonance images of the head were negative. Cerebral angiography revealed a carotid cavernous sinus fistula (CCF). The patient was successfully treated with external carotid artery embolization combined with radiation. It is well known that pupil sparing in oculomotor nerve palsy predicts an extraaxial ischemic lesion, while pupil involvement predicts an extraaxial compression lesion. Therefore, pupillary involvement in oculomotor nerve palsy in diabetic patients necessitates cerebrovascular investigation to rule out ICPC aneurysm or tumor. In this circumstance, a variant type of CCF without characteristic ocular signs should be included in the differential diagnosis.
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PMID:[A case of NIDDM associated with oculomotor palsy due to atypical carotid cavernous sinus fistula]. 827 44

Noonan syndrome was first described over 20 years ago by Noonan and Ehmke. They defined a specific group of nine patients with valvular pulmonary stenosis who, in addition, had short stature, mild mental retardation, hypertelorism and unusual facies. The incidence of Noonan syndrome has been estimated to be between 1 in 1000 and 1 in 2500 live births. The primary biochemical defect in Noonan's syndrome is unknown. We analyzed 9 patients (5 males and 4 females) in an age range of 6 months to 10 years and 3 months with Noonan syndrome. Patients were diagnosed as having the syndrome if they had characteristic facies and a normal karyotype, plus one of the following signs: cardiac defects, short stature or undescended testes. All patients have ocular anomalies (epicanthal folds, ptosis of eyelids, hypertelorism, downslanting palpebral fissures and ocular proptosis). Congenital heart malformations are present in 8 patients and the more frequent cardiopath is pulmonary valve stenosis due to a dysplastic or thickened valve. Short stature is present in 6 patients and 3 of them are actually on treatment with rhGH. A moderate-mild mental retardation is present in 6 patients. Case n. 9 had a syringomyelia and tethered cord. These malformations are rarely reported in Noonan's syndrome.
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PMID:[Nosologic evaluation of Noonan syndrome and description of nine cases]. 830 30

We report 14 cases of extranodal sinus histiocytosis with massive lymphadenopathy involving a variety of head and neck sites. The patients ranged in age from 3 to 70 years (median, 43 years). Nine cases occurred in women and five occurred in men. The clinical presentation varied depending on the site of occurrence and included nasal obstruction, stridor, proptosis, ptosis, decreased visual acuity, facial pain or tenderness, cranial nerve deficits, mandibular tenderness, and mass lesions. Head and neck sites involved by disease included the nasal cavity, paranasal sinuses, nasopharynx, parotid gland, submandibular gland, larynx, temporal bone, infratemporal fossa, pterygoid fossa, meninges, and orbital region. The majority of patients presented with involvement of more than one site. Nodal involvement was identified in four patients. Special stains for microorganisms were negative. The sinus histiocytosis with massive lymphadenopathy cells demonstrated an immunophenotypic profile supporting derivation from macrophage/histiocytic lineage. Treatment varied and included surgical excision with or without adjuvant therapy (chemotherapy, radiotherapy) or steroids. Several patients required more extensive surgery as a result of extension of their disease to adjacent structures or due to recurrent disease. Twelve patients are alive and either free of disease or have persistent disease. Two patients died, one as a result of complications of disease.
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PMID:Extranodal sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) of the head and neck. 849 88

A 55-year-old man reported a severe headache of 3 days' duration, left ptosis and left lid swelling before examination. The ocular examination revealed left eye proptosis, severe edema of the left bulbar conjunctiva and lid, increasing intraocular pressure of the left eye and ptosis on the left side with decreased extraocular movement. The right eye was normal. Hematologic studies indicated mild inflammation. An enhanced computed tomography scan revealed proptosis of the left globe and enlargement of the superior ophthalmic vein and cavernous sinus of the left side. Angiography revealed an area of interrupted blood flow in the left cavernous sinus. Enhanced magnetic resonance imaging (MRI) with Gd-DTPA revealed a low-intensity area that was suspected to be a blood clot in the enlarged left cavernous sinus. This case indicates the efficacy of enhanced MRI examination in the early diagnosis of cavernous sinus thrombosis.
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PMID:Magnetic resonance imaging in the early diagnosis of cavernous sinus thrombosis. 857 Jan 57

The ocular manifestations of 60 cases with tumors of accessory nasal sinuses were reported. They were proptosis, impairment of ocular movement, diplopia, decrease or loss of visual acuity, ptosis, etc. Of them, 42 patients came to the ophthalmology department first. All the patients were diagnosed by computerized tomography (CT scan) and pathological findings. Operative treatment was applied in 48 cases. 32 cases had improvement in ocular symptoms. Particular attention was paid to the analysis of the causes of the patients, ocular symptoms and signs. They are due to the close anatomical relationship between accessory nasal sinuses and the orbit and optic nerve canal. CT scan is helpful to the early diagnosis of the disease.
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PMID:[An analysis of ocular manifestations in cases with tumors of accessory nasal sinuses]. 874 23

Rhinocerebral mucormycosis is a well-described fulminant fungal infection that typically presents acutely in patients with diabetic ketoacidosis or immunosuppression. Chronic presentations of rhinocerebral mucormycosis have also been described. In the chronic infection, the disease course is indolent and slowly progressive, often occurring over weeks to months. The authors report 2 cases of chronic rhinocerebral mucormycosis (CRM) treated at their institution and review 16 other cases reported in the English-language literature. In these cases, the median time from symptom onset to diagnosis was 7 months. The most common presenting features of CRM are ophthalmologic and include ptosis, proptosis, visual loss, and ophthalmoplegia. CRM occurs predominantly in patients with diabetes and ketoacidosis. The incidence of internal carotid artery and cavernous sinus thrombosis is higher in CRM patients than in those with the acute disease, although the overall survival rate for CRM patients is 83%. CRM is clinically distinct from chronic Entomophthorales infection.
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PMID:Chronic rhinocerebral mucormycosis. 884 4

Rhino-orbital-cerebral mucormycosis is a disease that is frequently fatal. A 39-year-old man with diabetic ketoacidosis was referred to the authors' ophthalmic service with fever, orbital apex syndrome in the right eye, lethargy, and a black eschar in the palate. He was treated with systemic and local (intraconal) amphotericin B and his ketoacidosis was controlled; exenteration was not performed. Biopsy of the palate proved mucormycosis. Eighteen months later the patient was still alive and had a blind, anatomically preserved right eye with ptosis and intact extraocular muscle function without proptosis or pain. The authors propose this alternative means of treatment to achieve higher doses of the drug at the site of infection and better cosmetic and psychological results.
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PMID:Intraconal amphotericin B for the treatment of rhino-orbital mucormycosis. 885 37


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