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Orbita - pathology,content,Anatomic Considerations(ocular /conal /intraconal/extraconal)Ocular pathology(Calcifications /Retinoblastoma /Melanoma /Persistent hyperplastic primary vitrous (PHPV) /Coats disease /Globe rupture /Retinal and choroidal detachment /Coloboma )Intraconal pathology (Devics syndrome /MS /Meningioma /Optic nerve glioma) Conal pathology (Thyroid eye disease /Pseudotumor )Extraconal pathology (Periorbital abscess /Orbital and periorbital cellulitis /Sphenoid wing lesions /Lacrimal gland lesions)Vascular Malformations (Capillary hemangioma /Venous Vascular Malformation/ Lymphatic malformations /Orbital varix ),Anatomic Considerations,Anatomic Considerations,Anatomic Considerations,Anatomic Considerations,Ocular pathology,Retinoblastoma Melanoma Persistent hyperplastic primary vitrous (PHPV) Coats disease Globe ruptureRetinal and choroidal detachmentColoboma,Retinoblastoma,an adolescent with bilateral retinoblastoma. In children calcifications in the globe means retinoblastoma until proven otherwise even if it is bilateral.,a 13 month old female with bilateral lesions as a result of bilateral retinoblastoma,If the patent is treated with radiation, there is a 30% chance of a second malignancy within the radiation field, due to the radiation but also due to the deficient tumor suppression gene. Outside the radiation field there is an 8% chance of malignancy. These patients are also at risk for pineal tumors and parasellar PNETs. the pineal gland does not calcify, so any calcification in this region is suspicious of retinoblastoma,but also germinoma.,Melanoma,The most common intraocular lesion in an adult is melanoma (as in this case). Number two is metastases and others like hemangioma, leiomyoma and osteoma are uncommon.,Persistent hyperplastic primary vitrous (PHPV),There is a persistent hyaloid canal when the hyaloid artery does not integrate. On the images we see a persistent canal that goes from the optic nerve to the lens. There is also retinal detachment (occurs in 30-55%) and notice the microphtalmia. PHPV is the second most common cause of leukocoria.,Coats disease,Globe rupture,the depth of the anterior chamber is decreased.there is increased density anteriorly as a result of hyphema.the lens on the right side is blurred and slightly less dense.globe rupture and specifically rupture of the anterior chamber.,Globe rupture is seen most commonly at the anterior chamber.,Retinal and choroidal detachment,Retinal detachment can be distinguished from choroidal detachment, because the retinal epithelium ends at the ora serrata,a choroidal detachment going beyond ten and two oclock (with the lens at twlve oclock) and evidently more anteriorly to the ora serrataand the optic nerve. a retinal detachment ends at the optic nerve and at the ora serrata.,Coloboma,Coloboma is a congenital malformation in which part of the eye does not form due to failure of fusion of an embryonic structure called the intraocular fissure. Often there is microphtalmia and the eye protrudes inferiorly.In 10% there are other CNS anomalies.,Coloboma,a small coloboma at the entrance of the optic nerve.,Intraconal pathology,Devics syndrome MSMeningioma Optic nerve glioma,Devics syndrome,Devics syndrome is also known neuromyelitis optica.the optic nerve is white matter tract.It has the same signal intensity as the white matter in the brain.This is therefore extra-ocular intraconal disease and we will be thinking of neoplastic versus demyelinating diseases.,Devics syndrome differs from MS: The patients are very ill, as Devics is more acute and more fulminant. Usually there is a combination of one-sided blindness and paraparalysis due to the long segment of transverse myelitis (in MS it is usually one- or two-segment). Devics is usually monophasic. Usually no brain plaques.,MS,50% of patients with optic neuritis eventually develop MS. The other 50% have isolated optic neuritis . 80% of patients with MS develop optic neuritis at some point in time.,Meningioma,The optic nerves are normal, but there is abnormal mass-like enhancement of the optic nerve sheath on the left. So this is probably a neoplasm and of the neoplasms meningioma is by far the most common optic nerve sheath tumor.,Abnormal enhancement of the optic nerve sheath,Optic nerve glioma,There is sphenoid wing hypoplasia on the right and on the left the optic nerve near the chiasma is enlarged . So the diagnosis is neurofibromatosis type I with sphenoid wing hypoplasia and an optic pathway glioma.More than 50% of patients who have an optic nerve glioma have NF1, but in NF1 only about 10% have optic nerve glioma.,Conal pathology,Thyroid eye diseasePseudotumor,Thyroid eye disease Vs Pseudotumor,The key distinction between pseudotumor and thyroid eye disease is the fact that in pseudotumor not only the muscles, but also the tendons ,fat, optic nerve, nerve sheet, lacrimal gland etc,These patients feel pain when they are moving their eyes as the tendons get irritated.,Extraconal pathology,Periorbital abscess Orbital and periorbital cellulitisSphenoid wing lesions Lacrimal gland lesions,Periorbital abscess,Do not wait for peripheral enhancement to call it an abscess!In every other location you wait for nice rim enhancement to call it an abscess and if not you say it is a phlegmone.,Orbital and periorbital cellulitis,the difference between orbital and periorbital cellulitis is the orbital septum. the inflammation includes the orbital septum and everything superficial to it, the diagnosis is periorbital cellulitis and the patient is treated with oral antibiotics on an outpatient basis. if the structures posterior to the septum are also involved. This patient will have to stay in the hospital to receive antibiotics intravenously.,Sphenoid wing lesions,There are four sphenoid wing lesions that can cause proptosis:Sphenoid wing dysplasia Fibrous dysplasia Pagets disease Meningioma with hyperostosis of the sphenoid wing,Lacrimal gland lesions,Inflammatory conditions are by far the most common lesions of the lacrimal gland (i.e. Sj?grens, TB, fungus, pseudotumor). These conditions do not cause masses. The most common mass of the lacrimal gland is lymphoma followed by pleomorphic adenoma. Epithelial tumors including adenoid cystic tumors are uncommon,Vascular Malformations,Capillary hemangioma,Mostly located in the skin, but also seen in the extraconal compartment of the eye.,Venous Vascular Malfo
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