Lecture 3 - Growths of the Orbit and Adnexa

What growths of the orbit and adnexa are covered in this lecture?

Capillary hemangioma, port wine stain, cavernous hemangioma, orbital venous varix, neurofibromatosis, rhabdomyosarcoma, and lacrimal gland tumors

What does the Greek root "hem-" mean?

Blood

What does the Greek root "-angi-" mean?

Vessel

What does the suffix "-oma" mean?

Tumor or mass

What is a capillary hemangioma?

A benign vascular tumor of the orbit and periocular tissues composed of numerous small capillaries

What is another name for a capillary hemangioma?

Strawberry nevus

What is the structure of a capillary hemangioma?

Numerous normal-sized capillaries tightly packed together and held by thin connective tissue

Can capillary hemangiomas cause ocular damage despite being benign?

Yes

Are capillary hemangiomas usually present at birth?

No, they are typically absent at birth and develop during infancy

What is the most common benign tumor of infancy?

Capillary hemangioma

What percentage of births are affected by capillary hemangiomas?

Approximately 1-2%

What are the two phases of capillary hemangioma development?

Proliferative and involutional

What occurs during the proliferative phase of a capillary hemangioma?

Rapid growth over 8-18 months

What occurs during the involutional phase of a capillary hemangioma?

Slow spontaneous regression over several years

Who is more commonly affected by capillary hemangiomas?

Females (3:1)

Are capillary hemangiomas typically painful?

No

How do capillary hemangiomas typically present clinically?

A unilateral red-to-purple lesion of the superonasal eyelid or brow

How large can capillary hemangiomas become?

They can range from less than 1 cm to greater than 10 cm

How extensive can capillary hemangiomas become?

They may involve the entire upper lid, both lids, surrounding skin, or deeper tissues

What are the classifications of capillary hemangioma?

Superficial cutaneous, subcutaneous, and deep

What are the characteristics of superficial cutaneous capillary hemangiomas?

Bright red, nodular lesions that blanch with pressure

Which type of capillary hemangioma is most common?

Superficial cutaneous

What are the characteristics of subcutaneous capillary hemangiomas?

Dark blue or purple lesions with a spongy consistency

What are the characteristics of deep capillary hemangiomas?

Proptosis, globe displacement, and potential vision loss

What ocular complications are associated with capillary hemangiomas?

Mechanical blepharoptosis, proptosis, exposure keratopathy, decreased visual acuity, amblyopia, anisometropia, and conjunctival involvement

What percentage of patients with capillary hemangiomas develop amblyopia?

43-60%

Why is amblyopia a particular concern in capillary hemangiomas?

Especially in children younger than 2 years old

Why can capillary hemangiomas cause decreased visual acuity?

Changes in corneal curvature

Why can capillary hemangiomas cause anisometropia?

Mass effect on the cornea

What imaging study confirms a capillary hemangioma?

MRI

What does MRI show in a capillary hemangioma?

A strongly enhancing, lobulated mass

Why is imaging particularly important in deep capillary hemangiomas?

To determine lesion extent and differentiate tumor type

What is the most common management approach for capillary hemangiomas?

Observation because most undergo spontaneous involution

At what age do capillary hemangiomas typically begin to regress?

Around 18 months

What percentage of capillary hemangiomas regress by age 3?

30%

What percentage of capillary hemangiomas regress by age 7?

70%

How are superficial capillary hemangiomas treated medically?

Topical or injectable corticosteroids

When are systemic corticosteroids used for capillary hemangiomas?

For amblyogenic or life-threatening lesions

What surgical options are available for capillary hemangiomas?

CO2 laser, primary excision, vessel ligation, and vascular embolization

When is CO2 laser treatment used for capillary hemangiomas?

For superficial lesions less than 2 mm thick

What is vessel ligation?

Closing off a blood vessel with a ligature or clip

What is vascular embolization?

Artificial formation of an embolus to block blood flow

When is vascular embolization used for capillary hemangiomas?

Large extraorbital hemangiomas only

What specialists should be consulted for capillary hemangiomas?

Pediatric ophthalmology and pediatric dermatology

What is a port wine stain?

A congenital flat intradermal capillary malformation

What is another name for a port wine stain?

Nevus flammeus

What is a capillary malformation?

A congenital structural abnormality of blood vessels

What is the vascular structure of a port wine stain?

Dilated blood-filled vascular spaces

Are port wine stains present at birth?

Yes, they are always present at birth

How can a port wine stain be differentiated from a capillary hemangioma?

Port wine stains do not blanch with pressure and are permanent

Do port wine stains blanch with pressure?

No

Are port wine stains permanent?

Yes

How are port wine stains distributed anatomically?

They follow dermatomes but may be bilateral

What syndrome is associated with port wine stains?

Sturge-Weber syndrome

What is another name for Sturge-Weber syndrome?

Encephalotrigeminal angiomatosis

What ocular disease may be associated with port wine stains?

Congenital glaucoma

What is a cavernous hemangioma?

A benign vascular tumor of the orbit and periocular tissues composed of vascular channels separated by fibrous tissue

Can cavernous hemangiomas involve tissues outside the orbit?

Yes, they can affect a wide variety of tissues

How do cavernous hemangiomas typically grow?

Slowly

Can pregnancy affect cavernous hemangioma growth?

Yes, growth may accelerate due to hormonal changes

What is the most common vascular orbital tumor in adults?

Cavernous hemangioma

What percentage of adult angiomatous malformations are cavernous hemangiomas?

80%

What percentage of primary orbital expanding masses are cavernous hemangiomas?

9.5-15%

What percentage of all orbital tumors are cavernous hemangiomas?

5-7%

Who is most commonly affected by cavernous hemangiomas?

Females

What age group is most commonly affected by cavernous hemangiomas?

4th-5th decade (30s-40s)

Are cavernous hemangiomas common before age 20?

No

Are cavernous hemangiomas typically painful?

No, except in advanced stages

What symptoms are associated with cavernous hemangiomas?

Diplopia, cosmetic concern over proptosis, and vision loss from optic nerve compression

What signs are associated with cavernous hemangiomas?

Proptosis, ocular misalignment, ophthalmoplegia, RAPD, and choroidal folds

What imaging findings are seen in cavernous hemangiomas?

A strongly enhancing, well-circumscribed, homogeneous mass

How are small asymptomatic cavernous hemangiomas managed?

Observation once or twice annually

Is there a medical treatment for cavernous hemangiomas?

No

What is the treatment of choice for cavernous hemangiomas?

Surgical orbitotomy

What does the Latin root "varic-" mean?

Dilated, twisted, swollen vein

What is an orbital venous varix?

A congenital venous malformation of the orbit caused by weakness of the postcapillary venous wall

What is the vascular structure of an orbital venous varix?

A plexus of thin-walled, distensible, low-flow vessels

What does plexus mean?

A braided or branching network

What is the plural of varix?

Varices

How common are orbital venous varices?

Very uncommon

Are orbital venous varices congenital?

Yes

When do orbital venous varices usually become symptomatic?

After puberty

Do orbital venous varices have a specific age, gender, or racial predilection?

No

When do symptoms of orbital venous varices usually begin?

Early adulthood

Are orbital venous varices usually unilateral or bilateral?

Usually unilateral

What symptoms are classically associated with orbital venous varices?

Sudden, intermittent, and transient diplopia with proptosis

What triggers proptosis in orbital venous varices?

Increased venous pressure from Valsalva maneuver or postural changes

What is paradoxical enophthalmos in orbital venous varix?

The eye sinks into the orbit when the patient is supine

What severe complications can occur in orbital venous varix?

Pain, orbital hemorrhage, thrombosis, and optic nerve compression

What imaging studies can diagnose orbital venous varices?

MRI, MRV, CT, or ultrasound

What imaging findings are associated with orbital venous varices?

A lobulated mass with variable enhancement

How are small asymptomatic orbital venous varices managed?

Observation once or twice annually

When is surgery indicated for an orbital venous varix?

Severe pain, disfiguring proptosis, hemorrhage, thrombosis, or compressive optic neuropathy

What surgical options are available for orbital venous varices?

CO2 laser, vascular embolization, and primary excision

What is the most common treatment for orbital venous varix?

Primary excision

What is neurofibromatosis?

An autosomal dominant genetic disorder characterized by neurofibromas

What is another name for neurofibromatosis?

Von Recklinghausen disease

What is a neurofibroma?

A tumor arising from nerve tissue

What tissues are affected in neurofibromatosis?

Neural crest-derived tissues including Schwann cells, endoneurial fibroblasts, melanocytes, chondrocytes, and osteoblasts

Why can neurofibromas cause tissue damage?

Compression of adjacent structures