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Questions and Answers
What is a primary method for diagnosing cases suspected to involve vascular tumors in children?
What is a primary method for diagnosing cases suspected to involve vascular tumors in children?
Which imaging technique can be utilized to delineate orbital involvement more clearly?
Which imaging technique can be utilized to delineate orbital involvement more clearly?
Which condition is NOT listed as a differential diagnosis for vascular tumors in the provided information?
Which condition is NOT listed as a differential diagnosis for vascular tumors in the provided information?
What is the observed rate of complete resolution for vascular tumors with treatment by the age of 4?
What is the observed rate of complete resolution for vascular tumors with treatment by the age of 4?
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Indications for treatment of vascular tumors include which of the following?
Indications for treatment of vascular tumors include which of the following?
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What is the primary mechanism by which corticosteroid treatment is effective?
What is the primary mechanism by which corticosteroid treatment is effective?
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Which of the following is NOT a nonvisual indication for treating vascular tumors?
Which of the following is NOT a nonvisual indication for treating vascular tumors?
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Which steroid combination is recommended for intralesional injection?
Which steroid combination is recommended for intralesional injection?
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What is a noted complication of untreated vascular tumors potentially leading to treatment considerations?
What is a noted complication of untreated vascular tumors potentially leading to treatment considerations?
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What is a characteristic feature of cavernous hemangiomas?
What is a characteristic feature of cavernous hemangiomas?
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Which imaging technique reveals a well-defined, homogeneous intraconal mass in cavernous hemangiomas?
Which imaging technique reveals a well-defined, homogeneous intraconal mass in cavernous hemangiomas?
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In which age group are cavernous hemangiomas most commonly diagnosed?
In which age group are cavernous hemangiomas most commonly diagnosed?
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What is the preferred management approach for cavernous hemangiomas upon diagnosis?
What is the preferred management approach for cavernous hemangiomas upon diagnosis?
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What is the main characteristic of capillary hemangiomas?
What is the main characteristic of capillary hemangiomas?
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Which symptom is most commonly associated with cavernous hemangiomas?
Which symptom is most commonly associated with cavernous hemangiomas?
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What is the consequence of puncturing a low-flow cavernous hemangioma during surgery?
What is the consequence of puncturing a low-flow cavernous hemangioma during surgery?
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At what age are virtually all capillary hemangiomas diagnosed?
At what age are virtually all capillary hemangiomas diagnosed?
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What is the gender ratio associated with capillary hemangiomas in infants?
What is the gender ratio associated with capillary hemangiomas in infants?
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Cavernous hemangiomas are primarily characterized by which type of histopathological finding?
Cavernous hemangiomas are primarily characterized by which type of histopathological finding?
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Which of the following is NOT a common ocular complication of capillary hemangiomas?
Which of the following is NOT a common ocular complication of capillary hemangiomas?
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What is a common differential diagnosis for cavernous hemangiomas?
What is a common differential diagnosis for cavernous hemangiomas?
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What is a common predisposing factor for the development of capillary hemangiomas?
What is a common predisposing factor for the development of capillary hemangiomas?
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What age group is associated with a higher female preference for cavernous hemangiomas?
What age group is associated with a higher female preference for cavernous hemangiomas?
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What is the potential outcome for incompletely excised cavernous hemangiomas?
What is the potential outcome for incompletely excised cavernous hemangiomas?
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What is the typical appearance of a capillary hemangioma?
What is the typical appearance of a capillary hemangioma?
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Which condition can occur in infants with large visceral hemangiomas?
Which condition can occur in infants with large visceral hemangiomas?
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What is the typical size progression of capillary hemangiomas during the first 6 months?
What is the typical size progression of capillary hemangiomas during the first 6 months?
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What is a common risk factor for developing amblyopia in infants with capillary hemangiomas?
What is a common risk factor for developing amblyopia in infants with capillary hemangiomas?
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Which of the following best describes vascular hamartomas?
Which of the following best describes vascular hamartomas?
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Study Notes
Vascular Hamartomas
- Abnormal proportion of vascular elements 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
- Most common orbital benign vascular tumor of childhood (1% to 2% of infants)
- Female 3:2 ratio
- Predisposing factors: maternal chorionic villus sampling and prematurity
- 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 orbital septum, with variable degrees of proptosis if it extends more posteriorly
- Early flat, then bulky, compressible masses in weeks to months
- Most common ocular complications are 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)
- Anisometropia may result from axial myopia induced by eyelid closure or astigmatism
- Strabismus may be present in up to 34% of patients with periorbital infantile capillary hemangioma
- Associated visceral hemangiomas throughout the body have been reported, consider additional imaging 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
- Diagnosis can be made 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
- Treatment: Intralesional injections of long and short-acting corticosteroids
- Involutioin may begin in several days, usually is considerable within 2-4 weeks
- May be repeated at 6-week intervals, as needed
Cavernous Hemangioma
- Benign, non-infiltrative, low-flow hamartomas
- Vascular 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
- 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
- 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
- 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, with displacement of the optic nerve and indented posterior globe
- Histopathology - Large, endothelium-lines, blood-filled spaces separated by fibrous septa
- Ddx - Neurofibroma, cystic schwannoma, fibrous histiocytoma, vascular leiomyoma
- Management consideration - Cavernous hemangiomas have a tendency to enlarge, therefore, excision is predominantly advised as soon as the diagnosis is established
- Treatment - Surgical excision of the lesion
- Blunt dissection reveals a nodular, plum-colored, encapsulated mass with vascular channels on its surface
- A cryo-probing may assist with extraction
- No evidence of recurrence from incompletely excised lesions
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Description
This quiz covers the characteristics and clinical significance of vascular hamartomas and capillary hemangiomas. Explore the benign nature of capillary hemangiomas, their prevalence in infants, and associated clinical features. Test your understanding of these vascular lesions and their management.
