20 - 118: VASCULAR TUMORS Flashcards
most significant risk factor for development of infantile hemangioma (IH)
LBW <2500g
Characteristic of infantile hemangiomas, except
a. presence at birth
b. presence of a nascent IH
c. presence of area of pallor
d. presence of telangiectasias
a. presence at birth – absence at birth
S1 IH corresponds to anomalies in the
a. cerebral, cerebrovascular, ocular
b. less associated with extracutaneous features
c. airway involvement
d. cardiovascular abnormalities
a. cerebral, cerebrovascular, ocular
most common extracutaneous features of PHACE
cardiac anomalies
Areas most highly associated with PHACE
S3 – and S1
area that carries 60% of symptomatic airway disease
S3
Multifocal IH are considered if the patient presents with ___ or more IH
c. 6 p 2045
MC complication of IH
a. GI bleeding
b. early onset vascular stroke
c. Ulceration
d. DVT
c. Ulceration
rare complication in infants with diffuse infantile hemangiomas
a. Hypothyroidism
b. Hyperthyroidism
c. Hypercalcemia
d. Hypocalcemia
a. Hypothyroidism
Alternative drug to propranolol that lacks bronchial reactivity
Atenolol
most common tumor of infancy
Infantile hemangiomas
most significant risk factor for IH development
Low birth weight
characteristic features of IH
- ABSENT at birth or presence of a nascent IH often an area of pallor, telangiectasias or duskiness
There is _____% risk increase of infantile hemangioma for every _______ g decrease in birth weight
40%
500g
peak Infantile hemangioma growth period
5.5 and 7.5 weeks of age
Most common location of IH
Head and neck
but can be found on any cutaneous or mucosal site
Fill in the blanks.
Almost all IHs with a superficial component become apparent in the _______ month of life, and most will at least double in size within the first _______months of life.
- become apparent in the First month of life
- double in size within the first 2 months of life
While 80% of growth has occurred by _____months of age, 80% of IHs have completed all growth by ______ months of age
- 80% of growth has occurred by 3 months of age
- 80% of IHs have completed all growth by 5 months of age
The late proliferative stage of ongoing slower growth that occurs after peak rapid growth typically ends by _______ months of age
9 months
Evidence of involution, often referred to as ________
graying
- involves change to a dull red, then gray or milky-white color, followed by flattening and softening
Evidence of involution, often referred to as graying, is usually apparent by what age?
usually apparent by 1 year of age
More than 90% of IHs have completed involution by what age?
3.5 to 4 years of age
True or False:
DEEP IHs are more likely to develop residual skin changes following involution compared with SUPERFICIAL IHs
FALSE
Superficial IHs are more likely to develop residual skin changes following involution compared with deep IHs
A less-common subtype of IH is distinguished by its minimal-to-absent proliferation and telangiectatic surface
IH with minimal or abortive growth
the presence of facial segmental IH is associated with risk of what?
PHACE (posterior fossa brain malformations, hemangiomas of the face, arterial anomalies, cardiac anomalies, and eye abnormalities)
Segmental IHs on the lower body confer risk of what syndrome?
myelopathy and genitourinary anomalies in LUMBAR (lower body hemangioma and other cutaneous defects, urogenital anomalies, ulceration, myelopathy, bony deformities, anorectal malformations, arterial anomalies, and renal anomalies) syndrome
term used to those that are not clearly identifiable as localized or segmental IH; some authors consider them to be “subsegmental”
Indeterminate IHs
Multifocal (multiple localized) IHs are less common, with fewer than 3% of infants presenting with _________ or more IHs.
6
This subtype of IHs are 11 times more likely to experience complications and 8 times more likely to receive treatment
segmental hemangiomas
possible complications of infantile hemangioma
ulceration, severe bleeding, scarring, pain and infection
High-risk Infantile Hemangioma Features
Enumerate the 4 segments of facial segmental IH
S1: frontotemporal
S2: maxillary
S3: mandibular
S4: frontonasal
this segment of facial segmental IH is associated with cerebral, cerebrovascular, and ocular anomalies
S1: frontotemporal segment
this segment of facial segmental IH is less-frequently associated with extracutaneous involvement
maxillary segment (S2)
this segment of facial segmental IH is correlated with airway IH, ventral developmental defects, and cardiovascular abnormalities
mandibular segment (S3)
this segment of facial segmental IH encompasses the high-risk territory of the nose, including the nasal tip, 30 and confers risk of cerebral and cerebrovascular involvement
frontonasal segment (S4)
facial segments most highly associated with PHACE
frontotemporal (S1) and mandibular (S3) segments
most common extracutaneous features in PHACE
Congenital vascular anomalies
segmental IHs on the lower body involving the perineum or lumbosacral area are risk factors for associated spinal, bony, and genitourinary anomalies
LUMBAR syndrome describes the constellation of lower body hemangioma and other cutaneous defects, urogenital anomalies, ulceration, myelopathy, bony deformities, anorectal malformations, arterial anomalies, and renal anomalies
most commonly associated extracutaneous abnormality in LUMBAR syndrome presenting as tethered cord or lipomyelo(meningo)cele
Myelopathy
most common ocular complication, occurring in 40% to 60% of infants with untreated periocular IH
Amblyopia
IHs larger than ______in diameter confer greater risk of amblyopia, significantly lowering the threshold to initiate treatment.
1 cm
The most favorable prognostic sign to herald normal vision following involution
absence of asymmetrical refractive error
most common extracutaneous site for IH
Liver
T/F.
Focal hepatic hemangiomas are not true IHs.
Multifocal and diffuse hepatic hemangiomas are true IHs.
True
most common complication of IH
ulceration
- occurring in greater than 20% of infants in a referral setting, typically during the proliferative phase, with a median age of onset of 4 months
sensitive marker of impending ulceration of IH
Early gray-white discoloration in patients younger than 3 months
most reliable histologic marker of IH, which is expressed in all stages of IH
GLUT1, a red blood cell glucose transporter protein
only medication approved by the United States Food and Drug Administration (FDA) for the treatment of complicated IH
Propranolol
