Vascular Anomalies Flashcards
Vascular malformations are present after birth
F Vascular malformations are present at birth
Sclerotherapy is one potential intervention for venous and
lymphatic malformations.
T
Hemangiomas are the most common tumor of infancy
T Hemangiomas are the most common tumor of infancy and occur
at a rate of 11 in 10 infants
Hemangiomas present at birth
not present at birth
and are commonly visualized at approximately 2 weeks after birth
These tumors are more common in females (3:1) and Caucasians (1
in 10) with the most common location occurring in the head and
neck region
T
Infantile hemangiomas can be diagnosed with urine test?
Infantile hemangiomas are positive for glucose transporter 1 (GLUT-I) on biopsy of the lesion as well as within the urine
Imaging play an important role in hemangioma diagnoses
F. imaging is not commonly required.
The pulsed dye laser reducing bleeding from the site of haemangioma
T. the pulsed dye laser to lighten the color of the hemangioma and improve skin texture while reducing bleeding from the site
What the doses of predinsilon and for how long is given?
2-3mg /kg /day. For 4-6 week in proliferate phase
The initial response of steroid start in 1-2 week of therapy
T
The intial responce to systemic steriod in haemangioma start after 1-2 week
T
Intralesional corticosteroid vs systemic steriod
Intralesional corticosteroid delivery is another method of drug administration that has lower risks of systemic side effects, but similar potential to prevent further growth and induce tumor regression.
topical corticosteroid application is also effective in hemangioma
F. Although injectable steroid is known to be effective, topical corticosteroid application is ineffective.
Beta blocker in mor effective than steriod
F. it remains unclear whether beta blockers are more or less effective than steroids
Topical beta blockers such as timolol are also effective
T
Beta blockers, most commonly propranolol,
are administered at a dose of2 mg/kg/d and are most effective when
initiated during the proliferative phase
T
Responce to nterferon alpha-2A considred very fast in haemngioma
F. response is usually seen within 6 to 10 months
Beta blockers have a role . for Kasabach-Merritt syndrome.
F. Interferon alpha-2A is indicated for Kasabach-Merritt syndrome.
vincristine has preferable side effect
F. . As this is a chemotherapeutic agent, side effects include peripheral neuropathy, infections, and hair loss. Although the response rate is thought to approach over 80%, patients are required to have a central line for administration
Interferon alpha-2A is considered a second-line treatment At a subcutaneous dose of 2 to 3 mU/
m2
T
Haemangioma lesions may remain highly vascular even in the resolution phase
T
congenital hemangiomas are negative for GLUT-I, whereas infantile hemangiomas are positive.
T
agents used to treat hemangiomas including beta blockers and steroids are usually can be use in haemangioendothiloma
F. agents used to treat hemangiomas including beta blockers and steroids are usually ineffective for this indication. Vincrstin only
the main goal of care. Hemangioendotheliomas is surgical excision
F. necessitating symptomatic management as the main goal of care.
Hemangioendotheliomas can present at birth and post nataly
T
Hemangioendotheliomas mor common in female
F. are equally common in males and females
platelet transfusions may exacerbate swelling so should be avoided unless indicated for uncontrolled bleeding in this circumstance. In haemangioendothilioma
T
, resection is commonly possible in haemangioendothlioma
F. , resection is not commonly possible
All hemangioendothelioma regress by 2 years of age.
F some hemangioendotheliomas regress by 2 years of age, many of these lesions do not
involute with time,
Capillary Malformations influenced by the autonomic nervous system
T For example,
Sturge-Weber syndrome is defined by a capillary malformation
within the Vl trigeminal nerve distribution.
Capillary malformations occur equally in males and females.
F Capillary malformations occur with greater frequency
among female children
Isolated cutaneous capillary malformations do not require imaging
T unless uncertainty exists with regard
to the presence of visceral lesions or to more clearly define the extent
of these lesions
Capillary malformation in Ultrasound show high flow
T
MRI finding on Lymphatic Malformations show
MRI findings include
a soft-tissue mass with low signal on the Tl window and high signal with fluid levels on the T2 window
Microcystic lesions are commonly treated
with sclerosing agents such as doxycycline, ethanol
F Macrocystic lesions are commonly treated
with sclerosing agents such as doxycycline, ethanol
Venous malformations are composed of numerous thin-walled
veins, many of which lack valves and smooth muscle control
F lack valves and normal smooth muscle control
MRI finding of venous malformation
MRI demonstrates isointense
signal within the lesion on the Tl window and high signal with signal
voids at sites of phleboliths on the T2 window
MRI demonstrates soft tissue thickening with flow voids on the Tl window with a variable increase in
signal and similar flow voids in the T2 in AVM
T
lesions with an arterial
component are fast flow, whereas all other lesions including venous, capillary, and lymphatic malformations are
slow flow
T
Does imaging is useful in VM?
Imaging is useful when determining the extent
of the lesion as well as the amount of flow, particularly for vascular malformations
Infantile Hemangiomas Phases?
proliferation (0 to 12 months), involution (12 months to 10 years), and finally, regression (after 10 years).
When we should do a surgical intervention for IH?
hemangiomas that result in amblyopia, distortion of critical anatomic
structures, ulceration and bleeding complications, or airway compromise
Hormonal alterations over time can affect the involution face of haemangioma
T
imaging may further complicate the diagnosis in IH
T imaging may further complicate the diagnosis given that these
are not the only lesions that demonstrate fast flow on ultrasound
MRI finding in IH ?
proliferating hemangiomas are isointense on
Tl view and hyperintense on T2 view, with confluent enhancement
and flow voids with contrast.
most hemangiomas are treated nonoperatively
T
We can use PDL for. deep haemangioma
This is particularly effective
for superficial hemangiomas as the depth of
penetration of the pulsed dye laser is approximately 0.75 to 1.2 mm
Patients must also pass a
thorough cardiopulmonary evaluation prior to drug initiation of beta blocker?
T
Side effect of interferon-alpha 2A side effect
spastic diplegia, fevers, transaminitis, neutropenia, and anemia
the first technique for surgical excision of IH ?
Circular excision with purse-string closure is one potential technique
Hemangiomas ofthe lip can also cause significant deformity that even after involution results in soft-tissue laxity and discoloration.
T
Tissue expansion can be used in IH ?
For larger lesions, concomitant tissue expansion may be required to facilitate soft-tissue coverage
Tissue expanders in the back and extremities are excellent option for
vascular anomalies in these locations in pediatric patient populations
T
preoperative embolization always required in IH
preoperative embolization may be required
Difference between congenital haemangioma and IH ?
Characteristic appearance associated with their development, which includes a surrounding whitish halo, the pale central portion of the lesion, and an otherwise reddish hue
Congenital haemangioma is present e within the fetus and are
fully grown at birth.
Hemangioendothelioma can be metastasize
F these
lesions do not metastasize are locally
aggressive enlarge beyond 5 cm in diameter
When Hemangioendotheliomas lesions are
suspected, biopsy should be considered to rule out malignancy
T
biopsy should be considered always for VM ?
biopsy should be considered for any lesion when there
is uncertainty regarding the diagnosis
Von Hippel-Lindau
disease associated with developmental delay
T
Option of Capillary malformation Managment?
Observation
PDL for ulcerated and bleeding type
Debulking
Microcystic lesions are commonly treated
with sclerosing agents such as doxycycline, ethanol, and sodium tetradecyl sulfate
F Macrocystic lesions are commonly treated
with sclerosing agents such as doxycycline, ethanol, and sodium tetradecyl sulfate
Direct excision and
liposuction are potential techniques utilized for operative intervention.for lymphatic malformation
T
Causes of pain wih Venous Malformations
phleboliths and chronic inflammation
Options of Treatment in Venoue Malformation
Treatment of venous malformations is similar to that of lymphatic malformations. Options include observation, compression wraps, sclerotherapy, and excision for debulking. Low-dose aspirin therapy is helpful for pain due to microthrombi
Stages of Arterial Venous Malformation?
-Quiescent lesions may or may not be evident at birth
- Stage 2 consists of an expansion of the AVM, which can manifest as a thrill or bruit on physical examination
- stage 3 destructive phases, episodes of bleeding, pain, and local-tissue damage become apparent.
- Stage 4 is associated with heart failure and continued local-tissue destruction
Osler-Weber-Rendu disease
Cutaneous telangiectasias, frequent
nosebleeds, AVMs of viscera
There are two main types of congenital
hemangiomas: noninvoluting congenital hemangiomas (NICH)
and rapidly involuting congenital hemangiomas. Although both
commonly present within the head and neck region, noninvoluting
hemangiomas demonstrate persistent fast flow and do not regress,
whereas rapidly involuting hemangiomas substantially regress
within a few months of birth.
t
AVMs begin as quiescent lesions that may or may not
be evident at birth
T
