Ординатура / Офтальмология / Английские материалы / Rapid Diagnosis in Ophthalmology Series Oculoplastic and Reconstructive Surgery_Nerad, Carter, Alford_2008

Neoplasms• 11 SECTIONOrbit: the of Disorders

Pediatric Metastatic Orbital Disease (Continued)

•CT shows soft tissue mass (Fig. 11.40)

•Lateral orbital wall bone involvement with destructive lesions common

•Urine test for elevated:

•homovanillic acid

•vanillylmandelic acid

•3-methoxy-4-hydroxyphenylethyleneglycol

•Treatment is chemotherapy and orbital radiation

•Guarded prognosis for survival

Orbital Involvement with Leukemia

•Orbital manifestation of a systemic malignancy, usually appearing in late stages of leukemia

•May present with axial proptosis or other globe displacement, may be bilateral

•Occurs in acute lymphoblastic leukemia most commonly

•Can be seen in myelogenous leukemia; the orbital infiltrate is known as a granulocytic sarcoma or chloroma

•CT or MRI used to characterize the lesion (Fig. 11.41)

•Incisional biopsy

•Histopathology: Leder stain confirms presence of esterase and lysosomal enzymes that are seen in granulocytic process

•Chemotherapy for systemic disorder

•Survival is better if orbital lesion identified before advanced systemic disease occurs

•Prognosis improved with current chemotherapeutic regimens

Fig. 11.40 Abdominal CT showing left supraadrenal mass with calcification.

Fig. 11.41 MRI T2-weighted image showing infiltrative mass in the right lateral orbit including the lateral rectus muscle in a patient with myelogenous leukemia.

(continued) Disease Orbital Metastatic Pediatric

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B

Fig. 11.42 (A) A 57-year-old woman with gradual onset of diplopia. Right hypotropia in primary gaze. (B) Limited motility in upgaze of the right eye.

Fig. 11.43 Whitish choroidal mass in the temporal retina of the right eye. There is subretinal fluid around the mass.

Adults in Disease Orbital Metastatic

Neoplasms• 11 SECTIONOrbit: the of Disorders

Metastatic Orbital Disease in Adults (Continued)

•Usually imaged by CT but can use MRI (Fig. 11.44)

•Extraocular muscle or soft tissue infiltration, or bony destruction

•MRI findings are a hypointense mass on T1-weighted images and hyperintense signals on T2-weighted or contrast studies

•Differential diagnosis

Differential diagnosis includes thyroid eye disease

•Both are common in women and can have a restrictive ocular motility defect Incisional biopsy is performed if known metastatic disease does not exist

•Work-up includes hormone receptor studies and systemic oncology evaluation

•Treatment

Treatment options include:

•hormone therapy (tamoxifen)

•chemotherapy or radiation to orbit

•Prognosis

Metastatic disease often occurs many years after initial diagnosis

•Onset of metastatic disease in one location often suggests widespread disease

Bronchogenic Carcinoma

•Seen in middle-aged to older patients

•Time interval between diagnosis of primary disease and metastasis is months

•Metastasizes to the choroid more frequent than to the orbit

•Proptosis from tumor mass effect

•Motility deficits, pain, and chemosis are frequent (Fig. 11.45)

•CT and MRI usually show an infiltrative mass

•Bone destruction is common

•Incisional biopsy is performed, then systemic work-up by oncologist

•Palliation is the rule:

•orbital radiation in most cases, chemotherapy in some cases

•Metastatic disease has a poor prognosis

•Survival is usually <1 year

Prostate Carcinoma

•Proptosis, diplopia, and ptosis most common presenting signs (Fig. 11.46)

•Equal or second to lung as primary site in men

•CT and MRI—most common orbital site is bone, with osteoclastic and osteoblastic response (Fig. 11.47)

•Uncommonly can present as a solitary lesion of extraocular muscle mass (Fig. 11.48)

•Serology:

•prostate specific antigen levels

•Other metastatic sites include spine and long bones

•Incisional biopsy, especially if no other metastatic disease is known

•Oncologist evaluation

•Treatment:

•orbital radiation in most patients

•hormonal therapy or other chemotherapy for systemic spread

•Patients usually live a long time after metastases first diagnosed

Fig. 11.44 Coronal CT showing an enlarged right inferior rectus muscle from metastatic breast disease.

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B

Fig. 11.45 (A) Marked chemosis in this 68-year-old man with known bronchogenic carcinoma. (B) Axial CT showing the metastasis involving the right lateral rectus muscle and proptosis of the eye.

(continued) Adults in Disease Orbital Metastatic

Neoplasms• 11 SECTIONOrbit: the of Disorders

Metastatic Orbital Disease in Adults (Continued)

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C

Fig. 11.46 (A) A 68-year-old male presented with globe ptosis and proptosis. (B) Profi le view of the left eye in normal position (C) Profi le view showing marked proptosis of the right eye.

Fig. 11.47 Axial CT showing osteoblastic changes along the lateral wall and sphenoid bone in the right orbit. This bony change was the result of metastatic prostate carcinoma.

Fig. 11.48 Diffuse enlargement of the right medial rectus muscle from recurrent metastatic prostate process. The muscle was biopsied for confirmation of metastatic disease in a patient previously in remission.

(continued) Adults in Disease Orbital Metastatic

Abnormalities Vascular• 12 SECTIONOrbit: the of Disorders

Arteriovenous Fistula

Key Facts

•Abnormal direct connection between arterial and venous circulations, without an intervening capillary net

•Ocular signs due to rupture of internal carotid or smaller branches of carotid system into cavernous sinus, leading to reversed flow of venous system supplying the eye

•Two types of carotid cavernous sinus fistulas:

1.high flow, with branches of the carotid flowing into the cavernous sinus

2.low flow, caused by dural arteries flowing into cavernous sinus; sometimes also called indirect fistulas, because the miscommunication is not directly from the carotid artery but from its meningeal branches

•Traumatic cause in 70–90% of high-flow carotid-sinus fistulas

•Spontaneous etiology more common in low-flow dural-sinus fistulas

•Uncommon

•Spontaneous low-flow fistulas more common in females than in males

Clinical Findings

•Decreased visual acuity in 50–75%

•Orbital congestion:

•dilated conjunctival corkscrew vessels, chemosis

•proptosis (may be pulsatile if flow is high)

•increased IOP

•central retinal artery or vein occlusion

•Corneal exposure

•Cranial neuropathy including 3, 6, and 7

Imaging

• Cerebral angiogram is the gold standard

•CT or MRI show:

•dilated superior ophthalmic vein • enlarged extraocular muscles • fullness of cavernous sinus

Differential Diagnosis

• Orbital vascular tumors, inflammations, orbital infection

Treatment

•Medical therapy:

•antiglaucoma medications for increased IOP

•many (20–50%) dural fistulas close spontaneously

•lubricants to protect the cornea

•Surgical:

•endovascular closure using balloons, wires, or coils via direct carotid or transvenous (superior ophthalmic vein) approach

•Complications:

•cerebral angiogram and interventional radiologic procedures are associated with risks, including stroke and death

Prognosis

•Treatment is effective at reversing all signs and symptoms

• Vision loss usually improves