Download Orbital Tumors

眼科學 Ophthalmology
眼眶及眼整形概論
國防醫學院醫學系眼科學科 助理教授
三軍總醫院眼科部 視力保健科主任
江尚宜 博士
Outline
• Orbital Cavity
• Eyelids
• Lacrimal System
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Outline
• Orbital Cavity
• Eyelids
• Lacrimal System
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Bony socket
• This consists of 7 bones
- Frontal
- Ethmoid
- Lacrimal
- Sphenoid
- Maxillary
- Palatine
- Zygomatic
• Volume:30 mL
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Various bony openings into the orbital
cavity
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CN II, III, IV, V1, VI 通過
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Examination Methods
• Cardinal symptoms: Unilateral or bilateral enophthalmos or
exophthalmos
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Visual acuity
Ocular motility
Examination of the fundus
Exophthalmometry
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Orbital Involvement in Autoimmune
Disorders: Graves Disease
• Autoimmune disorder with orbital involvement is frequently
associated with thyroid dysfunction.
• Histologic examination reveals inflammatory infiltration of the
orbital cavity.
• Epidemiology. Women are affected eight times as often as men.
Sixty percent of patients have hyperthyroidism. Ten percent of
patients with thyroid disorders develop Graves disease during
the course of their life.
• Graves disease is the most frequent cause of both unilateral
and bilateral exophthalmos.
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Graves Disease
• Symptoms: painless disorder between the ages of 20 and 45.
Patients complain of reddened dry eyes with a sensation of
pressure (symptoms of keratoconjunctivitis sicca) and of cosmetic
problems. Ocularmotility is also limited, and patients may
experience double vision.
• Diagnostic:
- characteristic eyelid sign
- Thickening of the muscles (primarily the rectus inferior and
medial) and subsequent fibrosis lead to limited motility and
double vision.
- Elevation is impaired; this can lead to false high values when
measuring intraocular pressure with the gaze elevated.
- The tentative clinical diagnosis of Graves disease is supported by
thickening of the extraocular muscles identified in ultrasound or CT
studies.
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Graves Disease
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Graves Disease
Treatment:
• Acute stage: management of the thyroid dysfunction, systemic
cortisone (initially 60–100mg of prednisone) and radiation therapy
of the orbital cavity.
• Surgical decompression of the orbital cavity is indicated in recurrent
cases that do not respond to treatment to avoid compressive optic
neuropathy.
• Exposure keratitis (keratitis due to inability to close the eye) should
be treated with artificial tears or tarsorrhaphy (partial or complete
suture closure of the upper and lower eyelid to shorten or close the
palpebral fissure).
• In the postinflammatory stage of the disease, eye muscle surgery
can be performed to correct strabismus.
Clinical course and prognosis. Visual acuity will remain good if
treatment is initiated promptly. In the postinflammatory phase,
exophthalmos often persists despite the fact that the underlying
disorder is well controlled
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Orbital Inflammation
• Definition: Acute inflammation of the contents of the orbital
cavity with the cardinal symptoms of limited motility and general
malaise.
• Orbital cellulitis is the most frequent cause of exophthalmos
in children.
• Etiology: usually spread from surrounding tissue. Over 60% of
cases (as high as 84% in children) can be classified as originating
in the sinuses, especially the ethmoidal air cells and the frontal
sinus. In infants, tooth germ inflammations may be the cause.
Less frequently, this clinical picture occurs in association with facial
furuncles, erysipelas, hordeolum, panophthalmitis, orbital injuries,
and sepsis.
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Orbital Inflammation
• The crucial characteristic feature of orbital cellulitis for differential
diagnosis is the significantly limited ocular motility (“cemented”
globe).
• A rhabdomyosarcoma should also be considered in children.
• Treatment. This consists of high-dose intravenous antibiotic
therapy. Treatment of underlying sinusitis is indicated in
applicable cases.
• Orbital cellulitis can progress to a life-threatening situation
(cavernous sinus thrombosis).
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Orbital Pseudotumor
• Definition: Lymphocytic orbital tumor of unknown origin.
• Symptoms and findings. Painful, moderately severe inflammatory
reaction with eyelid swelling, chemosis, and unilateral or bilateral
exophthalmos.
• Involvement of the ocular muscles results in limited motility with
diplopia.
• Diagnostic considerations. The CT and MR images will show
diffuse soft-tissue swelling.
• A biopsy is required to confirm the diagnosis.
• Occasionally the CT image will simulate an infiltrative tumor.
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Orbital Pseudotumor
• Differential diagnosis. Various disorders should be excluded.
These include Graves disease and orbital cellulitis, which is
usually bacterial.
• Special forms of orbital pseudotumor include myositis and
Tolosa–Hunt syndrome (painful total ophthalmoplegia produced by
an idiopathic granuloma at the apex of the orbit).
• Treatment. High-dose systemic cortisone (initially 100mg of
prednisone) usually leads to remission.
• Orbital radiation therapy or surgical intervention may be indicated in
patients with no response to treatment.
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Pulsating Exophthalmos
• Etiology. An abnormal communication between the cavernous
sinus and the internal carotid artery (a direct shunt) or its
branches (indirect shunt) results in distention of the orbital venous
network. 80% are attributable to trauma; less frequently the disorder
is due to syphilis or arteriosclerosis.
• Diagnostic considerations. The increased venous pressure leads
to dilation of the episcleral and conjunctival vessels, retinal signs of
venous stasis with bleeding, exudation, and papilledema. Intraocular
pressure is also increased. The increased pressure in the cavernous
sinus can also result in oculomotor and abducent nerve palsy.
• Doppler ultrasound studies can confirm a clinical suspicion. Only
angiography can determine the exact
location of the shunt.
• Treatment. Selective embolization
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Orbital Tumors-Hemangioma
• The most common benign orbital tumors in both children and adults.
• Usually occur in a nasal superior location.
• Capillary hemangiomas are more common in children (they swell
when the child screams), and cavernous hemangiomas are more
common in adults.
• Treatment is only indicated where the tumor threatens to occlude
the visual axis with resulting amblyopia or where there is a risk of
compressive optic neuropathy.
• Capillary hemangiomas in children can be treated with cortisone or
low-dose radiation therapy.
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Orbital TumorsDermoid and Epidermoid Cyst
• These lesions are the most common orbital tumors in children.
• Etiologically, they are choristomas—i.e., dermal or epidermal
structures that have been displaced into deeper layers. However,
they usually are located anterior to the orbital septum (and
therefore are not in the actual orbit itself).
• Lesions located posterior to the orbital septum usually become
clinically significant only in adults.
• Treatment consists of complete removal.
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Orbital Tumors
• Meningioma : proceed from the optic nerve (meningioma of the optic
nerve sheath) or from within the cranium (sphenoid meningioma).
• Histiocytosis X: proliferation of Langerhans cells of undetermined,
all three of the following types can cause exophthalmos where there is
orbital involvement:
- Letterer–Siwe disease (malignant).
- Hand–Schuller–Christian disease (benign).
- Eosinophilic granuloma (rare and benign).
• Lymphoma: treated by radiation therapy or chemotherapy. Usually
these tumors are only slightly malignant. The highly malignant Burkitt
lymphoma, which has a high affinity for the orbital cavity.
• Rhabdomyosarcoma: the commonest primary malignant tumor in
children.
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Blunt Ocular Trauma (Ocular Contusion)
• Epidemiology and etiology: resulting from blunt trauma such as a
fist, ball, champagne cork, stone, falling on the eye, or a cow’s horn
are very common. Significant deformation of the globe can result
where the diameter of the blunt object is less than that of the bony
structures of the orbit.
• Clinical picture and diagnostic considerations: Deformation
exerts significant traction on intraocular structures and can cause
them to tear. Often there will be blood in the anterior chamber, which
will initially prevent the examiner from evaluating the more posterior
intraocular structures.
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Blow-Out Fracture
• This fracture usually occurs where the bone is thinnest, along
the paper-thin floor of the orbit over the maxillary sinus.
• The ring-shaped bony orbital rim usually remains intact. The fracture
can result in protrusion and impingement of orbital fat and the
inferior rectus and its sheaths in the fracture gap. Where the
medial ethmoid wall fractures instead of the orbital floor,
emphysema(眼瞼皮下氣腫) in the eyelids will result.
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Outline
• Orbital Cavity
• Eyelids
• Lacrimal System
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Structure of the eyelids
•Superficial layer (supplied by NV1, V2):
– skin, modified sweat glands (ciliary
glands or glands of Moll) and
sebaceous glands (glands of Zeis),
– orbicularis oculi muscle (supplied by
the CNVII),
– levator palpebrae muscle, which
actively opens the eye (supplied by the
CNIII).
•Deep layer:
– tarsal plate, tarsal muscle (supplied
by the sympathetic nervous system),
– palpebral conjunctiva is firmly
attached to the tarsal plate.
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Eyelid position & Width of the
palpebral fissure
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Ptosis
• Definition: Paralysis of the levator palpebrae muscle with resulting
drooping of one or both upper eyelids
• The following forms are differentiated according to their origin:
• Congenital ptosis.
• Acquired ptosis:
– Paralytic ptosis.
– Sympathetic ptosis.
– Myotonic ptosis.
– Traumatic ptosis.
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Congenital ptosis
• Usually hereditary and is primarily autosomal-dominant as opposed
to recessive.
• The cause is frequently aplasia in the core of the oculomotor
nerve (neurogenic) that supplies the levator palpebrae muscle; less
frequently it is attributable to an underdeveloped levator
palpebrae muscle (myogenic).
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Acquired ptosis
• Neurogenic causes:
– Oculomotor palsy (paralytic ptosis).
– Lesions in the sympathetic nerve (sympathetic ptosis) in Horner’s
palsy (ptosis, miosis, and enophthalmos).
• Myotonic ptosis: myasthenia gravis and myotonic dystrophy.
• Traumatic ptosis can occur after injuries.
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Methods of surgical retraction of the upper eyelid
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Entropion
• Definition: Entropion is characterized by inward rotation of the
eyelid margin. The margin of the eyelid and eyelashes or even the
outer skin of the eyelid are in contact with the globe instead of only
the conjunctiva.
• The following forms are differentiated according to their origin:
- Congenital entropion: Usually the lower eyelid is affected.
- Spastic entropion: affects only the lower eyelid
- Cicatricial entropion: frequently the result of postinfectious or
post-traumatic tarsal contracture (such as trachoma; burns and
chemical injuries).
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Ectropion
• Definition: Ectropion refers to the condition in which the margin of
the eyelid is turned away from the eyeball. This condition almost
exclusively affects the lower eyelid.
• The following forms are differentiated according to their origin:
- Congenital ectropion.
- Senile ectropion.
- Paralytic ectropion.
- Cicatricial ectropion.
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Blepharospasm
• Definition: involuntary spasmodic contraction of the orbicularis oculi
muscle supplied by the facial nerve.
• Etiology: Causes of the disorder include extrapyramidal disease
such as encephalitis or multiple sclerosis. Trigeminal neuralgia or
psychogenic causes may also be present.
• Treatment: depends on the cause of the disorder.
- Mild cases: muscle relaxants.
- Severe cases: transection of the fibers of the facial nerve supplying
the orbicularis oculi muscle.
The disorder may also be successfully treated with repeated local
injections of botulinum toxin.
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Disorders of the Skin and Margin
of the Eyelid
• Edema
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Disorders of the Skin and Margin of the
Eyelid
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•
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Seborrheic Blepharitis
Herpes Simplex of the Eyelids
Herpes Zoster Ophthalmicus
Eyelid Abscess
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Disorders of the Eyelid GlandsHordeolum
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• Epidemiology and etiology. Staphylococcus aureus is a
common cause of hordeolum. External hordeolum involves
infection of the glands of Zeis or Moll. Internal hordeolum
arises from infection of the meibomian glands.
• Hordeolum is often associated with diabetes, gastrointestinal
disorders, or acne.
• Differential diagnosis. Chalazion (tender to palpation) and
inflammation of the lacrimal glands (rarer and more painful).
• Treatment. Antibiotic ointments and application of dry heat
(red heat lamp) will rapidly heal the lesion.
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Disorders of the Eyelid GlandsChalazion
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• Definition: Firm nodular bulb within the tarsus.
• Epidemiology and etiology. Chalazia occur relatively frequently
and are caused by a chronic granulomatous inflammation due to
buildup of secretion from the meibomian gland.
• Symptoms. The firm painless nodule develops very slowly. Aside
from the cosmetic flaw, it is usually asymptomatic.
• Differential diagnosis. Hordeolum (tender to palpation) and
adenocarcinoma.
• Treatment. Surgical incision.
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Eyelids Benign Tumors
• Ductal Cysts:
The round cysts of the glands of Moll are usually located in the
angle of the eye. Their contents are clear and watery and can be
transilluminated. Gravity can result in ectropion.Therapy consists of
marsupialization. The prognosis is good.
• Xanthelasma
Local fat metabolism disorder that produces lipoprotein deposits.
These are usually bilateral in the medial canthus. Postmenopausal
women are most frequently affected. A higher incidence in patients
with diabetes, increased levels of plasma lipoprotein, or bile duct
disorders.
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Eyelids Malignant Tumors
Basal Cell Carcinoma
• frequent, moderately malignant, fibroepithelial tumor that can cause
severe local tissue destruction but very rarely metastasizes.
• Approximately 90% of malignant eyelid tumors are basal cell
carcinomas. Their incidence increases with age. In approximately
60% of cases they are localized on the lower eyelid. Dark-skinned
people are affected significantly less often.
• Increased exposure to the sun’s ultraviolet radiation, carcinogenic
substances (such as arsenic), and chronic skin damage can also
lead to an increased incidence.
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Eyelids Malignant Tumors
Basal Cell Carcinoma
• Loss of the eyelashes in the vicinity of the tumor always suggests
malignancy.
• Treatment. The lesion is treated by surgical excision within a
margin of healthy tissue.
• The earlier a basal cell carcinoma is detected, the easier it is to
remove.
• Prognosis. The chances of successful treatment by surgical
excision are very good. Frequent follow-up examinations are
indicated.
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Eyelids Malignant Tumors
Squamous Cell Carcinoma
• This is the second most frequently encountered malignant eyelid
tumor.
• The carcinoma arises from the epidermis, grows rapidly and
destroys tissue. It can metastasize into the regional lymph nodes.
Distant metastases are rarer.
• The treatment of choice is complete surgical removal.
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Eyelids Malignant Tumors
Adenocarcinoma
•
•
•
•
•
Arises from the meibomian glands or the
glands of Zeis.
The firm, painless swelling is usually located in
the upper eyelid and is mobile with respect to
the skin but not with respect to the underlying
tissue.
In its early stages it can be mistaken easily for
a chalazion.
The lesion can metastasize into local lymph
nodes.
An apparent chalazion that cannot be removed
by the usual surgical procedure always
suggests a suspected adenocarcinoma.
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Outline
• Orbital Cavity
• Eyelids
• Lacrimal System
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Anatomy of the lacrimal system
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外
內
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Evaluation of Tear Drainage
• Conjunctival fluorescein dye test.
• Probing and irrigation
A probe can be used to determine the site
of the stricture, and possibly to eliminate
obstructions
• Radiographic contrast studies
• Digital subtraction dacryocystography
• Lacrimal duct endoscopy
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Disorders of the Lower Lacrimal System
• Dacryocystitis
Inflammation of the lacrimal sac is the most frequent disorder of
the lower lacrimal system. It is usually the result of obstruction of the
nasolacrimal duct and is unilateral in most cases.
- Acute Dacryocystitis
- Chronic Dacryocystitis
- Neonatal Dacryocystitis
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Acute Dacryocystitis
• Epidemiology: most frequently affects adults between 50 and 60.
• Etiology: The cause is usually a stenosis within the lacrimal sac.
The retention of tear fluid leads to infection from staphylococci,
pneumococci, Pseudomonas, or other pathogens.
• Symptoms. Clinical symptoms include highly inflamed, painful
swelling in the vicinity of the lacrimal sac.
• Treatment. Acute cases are treated with local and systemic
antibiotics according to the specific pathogens detected.
• Treatment after acute symptoms have subsided often requires
surgery (dacryocystorhinostomy) to achieve persistent relief.
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Chronic Dacryocystitis
• Etiology. Obstruction of the nasolacrimal duct is often secondary to
chronic inflammation of the connective tissue or nasal mucosa.
• Symptoms and diagnostic considerations. The initial characteristic
is increased lacrimation. Signs of inflammation are not usually present.
Applying pressure to the inflamed lacrimal sac causes large quantities
of transparent mucoid pus to regurgitate through the punctum.
• Treatment. Surgical intervention is the only effective treatment in the
vast majority of cases. This involves either a dacryocystorhinostomy
(DCR 淚囊鼻腔吻合術) (creation of a direct connection between the
lacrimal sac and the nasal mucosa) or removal of the lacrimal sac.
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Neonatal Dacryocystitis
• Etiology. Approximately 6% of newborns have a stenosis of the
mouth of the nasolacrimal duct due to a persistent mucosal fold
(lacrimal fold or valve of Hasner). The resulting retention of tear
fluid provides ideal growth conditions for bacteria, particularly
staphylococci, streptococci, and pneumococci.
• Symptoms and diagnostic considerations. Shortly after birth
(usually within 2–4weeks), pus is secreted from the puncta.
• Treatment: During the first few weeks, the infant should be
monitored for spontaneous opening of the stenosis. During this
period, antibiotic and antiinflammatory eyedrops and nose drops
(such as erythromycin and xylometazoline 0.5% for infants) are
administered.
• If symptoms persist, irrigation or probing under short-acting
general anesthesia may be indicated
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Tumors of the Lacrimal Sac
• Epidemiology. Tumors of the lacrimal sac are rare but are primarily
malignant . They include papillomas, carcinomas, and sarcomas.
• Symptoms and diagnostic considerations. Usually the tumors
cause unilateral painless swelling followed by dacryostenosis.
• Diagnostic considerations. The irregular and occasionally bizarre
form of the structure in radiographic contrast studies is typical.
Ultrasound, CT, MRI, and
• biopsy all contribute to confirming the diagnosis.
• Differential diagnosis. Chronic dacryocystitis, mucocele of the
ethmoid cells.
• Treatment. The entire tumor should be removed.
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Disorders of the Lacrimal Gland
Acute Dacryoadenitis
• Definition: Acute inflammation of the
lacrimal gland is a rare disorder
characterized by intense inflammation
and extreme tenderness to palpation.
• Etiology: Pneumococci and staphylococci, and less frequently to
streptococci. There may be a relationship between the disorder and
infectious diseases such as mumps, measles, scarlet fever,
diphtheria, and influenza.
• Symptoms: Usually occurs unilaterally. The inflamed swollen
gland is especially tender to palpation. The upper eyelid exhibits a
characteristic S-curve.
• Treatment. Depend on the underlying disorder. Moist heat,
disinfectant compress, and local antibiotics are helpful.
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Disorders of the Lacrimal Gland
Chronic Dacryoadenitis
• Etiology: result of an incompletely healed acute dacryoadenitis.
Diseases such as tuberculosis, sarcoidosis, leukemia, or
lymphogranulomatosis.
• Symptoms: Usually there is no pain. Less pronounced than in the
acute form. However, the S-curve deformity of the palpebral fissure
resulting from swelling of the lacrimal gland is readily apparent.
• Treatment. This will depend on the underlying disorder. Systemic
corticosteroids may be effective in treating unspecific forms.
• Prognosis: good when the underlying disorder can be identified.
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Tumors of the Lacrimal Gland
• Epidemiology. Tumors of the lacrimal gland account for 5–7% of
orbital neoplasms. Lacrimal gland tumors are much rarer in children.
• The most frequent benign epithelial lacrimal gland tumor is the
pleomorphic adenoma. Malignant tumors include the adenoid
cystic carcinoma and pleomorphic adenocarcinoma.
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謝謝
2017/5/8
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