Vascular Hamartomas and Capillary Hemangioma
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Questions and Answers

What is the common alteration in tissue structure associated with vascular hamartomas?

  • Malignant transformation of skin cells
  • Benign tumors of muscle tissues
  • Excessive growth of nerve tissues
  • Abnormal proportion of vascular elements (correct)

    • What is another name for capillary hemangioma?

  • Strawberry hemangioma (correct)
  • Cavernous hemangioma
  • Port-wine stain
  • Cherry angioma

    • What is the typical age for diagnosing a capillary hemangioma?

  • In the teenage years
  • By age 6 months (correct)
  • At birth
  • After age 1 year

    • What percentage of infants is affected by capillary hemangiomas?

    <p>1% to 2%</p> Signup and view all the answers

    What complication is most commonly associated with capillary hemangiomas in infants?

    <p>Visual loss</p> Signup and view all the answers

    Which imaging test is commonly used to rule out other hemangiomas associated with capillary hemangiomas?

    <p>MRI scans</p> Signup and view all the answers

    What is the gender ratio for infants with capillary hemangiomas?

    <p>3:2</p> Signup and view all the answers

    What could lead to the occurrence of Kasabach-Merritt syndrome in association with hemangiomas?

    <p>Large visceral lesions</p> Signup and view all the answers

    What is a common location for capillary hemangiomas to appear on the body?

    <p>Upper eyelid</p> Signup and view all the answers

    What is a potential result of amblyopia associated with capillary hemangiomas?

    <p>Optic atrophy</p> Signup and view all the answers

    What is a rare condition caused by a vascular tumor that destroys platelets?

    <p>Capillary hemangioma</p> Signup and view all the answers

    Which imaging technique is typically avoided for diagnosing conditions in children due to radiation concerns?

    <p>CT scan</p> Signup and view all the answers

    What percentage of children shows complete resolution of capillary hemangiomas by age 7?

    <p>72% to 76%</p> Signup and view all the answers

    Which of the following is NOT an indication for treatment of capillary hemangiomas?

    <p>Presence of a dermoid cyst</p> Signup and view all the answers

    What is the role of intralesional corticosteroids in the treatment of capillary hemangiomas?

    <p>To reduce inflammation and constrict blood vessels</p> Signup and view all the answers

    Which condition is considered in the differential diagnosis of capillary hemangiomas?

    <p>Rhabdomyosarcoma</p> Signup and view all the answers

    What is the recommended corticosteroid combination for intralesional injection?

    <p>Triamcinolone acetate and betamethasone</p> Signup and view all the answers

    Which of the following is a nonvisual indication for treating capillary hemangiomas?

    <p>Deep lesions with frequent bleeding</p> Signup and view all the answers

    What is the most effective age range for expecting improvement in children with capillary hemangiomas?

    <p>10 to 12 years</p> Signup and view all the answers

    What is one of the main goals when outlining management options to parents of children with these lesions?

    <p>Promoting conservative management</p> Signup and view all the answers

    What is the typical age range for patients with cavernous hemangiomas?

    <p>&lt; 18 to &gt; 78 years</p> Signup and view all the answers

    What is a common clinical feature of cavernous hemangiomas?

    <p>Painless proptosis</p> Signup and view all the answers

    Which imaging technique is used to determine the characteristics of cavernous hemangiomas?

    <p>Contrast-enhanced axial CT scan</p> Signup and view all the answers

    What histopathological feature is associated with cavernous hemangiomas?

    <p>Large, endothelium-lined blood-filled spaces</p> Signup and view all the answers

    Which management option is predominantly advised once a cavernous hemangioma diagnosis is established?

    <p>Surgical excision</p> Signup and view all the answers

    What is a distinguishing characteristic of the mass seen in cavernous hemangiomas upon MRI?

    <p>Well-defined, homogeneous intraconal mass</p> Signup and view all the answers

    What might complicate surgical excision of a cavernous hemangioma during the procedure?

    <p>Exsanguination due to low flow</p> Signup and view all the answers

    What type of hemangioma is commonly associated with visceral lesions leading to gastrointestinal bleeding?

    <p>Cavernous hemangioma</p> Signup and view all the answers

    Which of the following is NOT a characteristic of cavernous hemangiomas?

    <p>Infiltrative growth pattern</p> Signup and view all the answers

    Which of the following is a differential diagnosis for cavernous hemangiomas?

    <p>Neurofibroma</p> Signup and view all the answers

    Study Notes

    ### Vascular Hamartomas

    • Abnormal proportion of vascular elements present at a cutaneous site.
    • Growth on tissues that are supposed to be there.

    Capillary Hemangioma

    • Benign, high-flow hamartomatous proliferation of primitive vasoformative tissues.
    • AKA strawberry hemangioma.
    • Composed of a mass of small blood vessels.
    • Typically appears as a raised, red or purplish lesion on the skin, particularly on the face, scalp, or neck.
    • Most common orbital benign vascular tumor of childhood (1% to 2% of infants).
    • Female 3:2
    • Predisposing factors: maternal chorionic villus sampling and prematurity.
    • Chorionic villus sampling (or CVS) is a prenatal test that diagnoses DNA genetic conditions in a fetus by looking at cell samples from the placenta (done around 10-12 weeks of pregnancy).
    • One-third are noted at birth; virtually all are diagnosed by age 6 months
    • Largest size within 6 months and then typically involutes.
    • Most commonly a unilateral diffuse subcutaneous or circumscribed, dimpled, red dermal lesion (strawberry nevus) is noted on the upper eyelid
    • Palpebral or forniceal conjunctiva may be involved.
    • Commonly is associated with the orbital septum (variable degrees of proptosis if it extends more posteriorly).
    • Early flat, then bulky, compressible masses in weeks to months.
    • Most common ocular complications of adnexal capillary hemangiomas is visual loss, most often resulting from amblyopia or in rare cases optic atrophy.
    • Amblyopia occurs in 44% to 64% of these infants (anisometropia, visual deprivation, or both).
    • Amblyopia that can be caused by strabismus or sensorial deprivation because of anisometropia.
    • Anisometropia may result from axial myopia induced by the eyelid closure or astigmatism (plus cylinder axis points toward the tumor mass).
    • Strabismus may be present in up to 34% of patients with periorbital infantile capillary hemangioma.
    • Associated visceral hemangiomas throughout the body have been reported; with time run other imaging tests to rule out other hemangiomas elsewhere.
    • Laryngeal hemangiomas are the most frequent visceral vascular manifestation.
    • Sequestration of platelets and red blood cells leading to thrombocytopenia and bleeding diathesis (Kasabach-Merritt syndrome) can occur with large visceral lesions but are rare with isolated head and neck lesions.
    • Kasabach-Merritt syndrome is a rare, life-threatening condition that occurs when a vascular tumor destroys platelets.
    • Diagnosis typically does not involve CT scan due to radiation risk to children.
    • Can be diagnosed readily by clinical inspection.
    • Enhanced MRI (Gadolinium- pentetic acid enhancement, and fat suppression) if delineation of orbital involvement is required, or to confirm.
    • High vascular flow may be demonstrated on Doppler echography.

    Capillary Hemangioma Management

    • Outlining the natural history of these lesions to the parents (justifying conservative management).
    • Rate of complete resolution with treatment is 32% to 60% at 4 years of age, and 72% to 76% at 7 years old.
    • There is variable improvement in the remaining children until the age of 10 to 12 years.

    Capillary Hemangioma Treatment Indications

    • Amblyopia (threatened occlusion of the visual axis).
    • Compression of the optic nerve.
    • Corneal exposure secondary to severe proptosis.
    • Induced astigmatism and anisometropic refractive error.
    • Nonvisual indications: deep lesions that bleed frequently, secondary maceration and erosion of the epidermis, severe disfigurement, cardiovascular, hematologic, or obstructive complications.

    Capillary Hemangioma Treatment

    • Intralesional injections of long and short-acting corticosteroids.
    • Long-acting steroid is directed deep into the lesion to prevent deposits from being visible under the skin.
    • Short-acting corticosteroid is given subcutaneously around the periphery of the capillary hemangioma.
    • Therapeutic effectiveness results from arteriolar constriction and narrowing of precapillary sphincters
    • Separate 1- to 3-mL syringes with 25- or 27-gauge needles of: 40 mg triamcinolone acetate and 6 mg betamethasone or 40 mg methylprednisolone and 4 mg dexamethasone sodium phosphate.
    • Involution may begin in several days; usually is considerable within 2 to 4 weeks.
    • May be repeated at 6-week intervals, as needed.

    Cavernous Hemangioma

    • Benign, non-infiltrative, low-flow hamartomas.
    • Vascular malformation (not a malformation) with large blood-filled pockets.
    • They do not contain tissue of the organ in which they are situated.
    • Often cited as the most common primary orbital tumor of adults.

    Cavernous Hemangioma Clinical Features

    • Typically in the fourth and fifth decades (although the age range < 18 - > 78).
    • 60% to 70% female preference.
    • Gradually increasing painless proptosis common.
    • Less common - orbital pain, eyelid swelling, diplopia, and gaze-induced amaurosis.

    Cavernous Hemangioma Prevalence

    • Usually isolated lesions.
    • Very rarely, associated with multiple hemangiomas, involving predominantly the arms and trunk (usually evident at birth), visceral lesions commonly in the small intestine leading to gastrointestinal bleeding and iron deficiency anemia.

    Cavernous Hemangioma Imaging

    • Contrast-enhanced axial CT scan - well-demarcated, oval intraconal mass (normally at the lateral part of the middle third of the orbit).
    • Axial magnetic resonance imaging - well-defined, homogeneous intraconal mass, T1-weighted image (A), T2-weighted (B) image, displacement of the optic nerve and indented posterior globe.

    Cavernous Hemangioma Histopathology

    • Large, endothelium-lines, blood-filled spaces separated by fibrous septa.

    Cavernous Hemangioma Differential Diagnoses

    • Neurofibroma, cystic schwannoma, fibrous histiocytoma, vascular leiomyoma.
    • All can have identical clinical, CT, and MRI findings.

    Cavernous Hemangioma Management

    • As the natural history of cavernous hemangiomas is to enlarge
    • Excision is predominantly advised as soon as the diagnosis is established.

    Cavernous Hemangioma Treatment

    • Surgical excision of the lesion.
    • Blunt dissection reveals a nodular, plum-colored, encapsulated mass with vascular channels on its surface.
    • Because of its low-flow character, it can be punctured during surgery, leading to exsanguination and shrinkage of the tumor, which facilitates excision.
    • A cryo-probing may assist with extraction.
    • No evidence of recurrence from incompletely excised lesions.

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    Orbital Vascular Hamartomas PDF

    Description

    This quiz covers the characteristics and clinical aspects of vascular hamartomas and capillary hemangiomas, including their presentation, prevalence, and diagnostic factors. Learn about the benign nature of these vascular lesions and their common occurrences in infants.

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