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7th > Chapter 147 - Vascular malformations > Flashcards

Chapter 147 - Vascular malformations Flashcards

1
Q

Vascular malformations arise due to errors of development of vessels that occur during the ____ weeks of intrauterine life

A

4th - 10th

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2
Q

Capillary lymphatic malformation with limb hypertrophy

A

Klippel Trenaunay syndrome

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3
Q

Multiple endochondromas associated with multiple venous anomalies and high incidence of malignancy

A

Maffucci syndrome

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4
Q

High flow vascular malformation of extremity with soft tissue hypertrophy

A

Parkes Weber syndrome

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5
Q

Vascular malformations grow proportionately with the patient and usually regresses spontaneously.
True or False

A

False, does not regress

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6
Q

Very useful noninvasive radiologic exam for differentiating various types

A

Doppler ultrasonography

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7
Q

Congenital slow flow malformations of the capillary bed

A

Capillary malformation

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8
Q

Port wine stain can be present in (2) syndromes

A

Beckwith Wiedemann

Rubinstein Taybi

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9
Q

Capillary malformations persist lifelong.

True or False

A

True

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10
Q

Embryogenic anomaly involving both the vasomotor nerves and the melanocytes (both derived from the neural crest), often located on the trunk/ extremities, in association with pigmented cutaneous lesions

A

Phakomatosis pigmentovascularis

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11
Q

Neuro-oculo-cutaneous syndrome involves CN V1 with homolateral leptomeningeal capillary-venous malformation and choroid CVM.

A

Sturge Weber syndrome

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12
Q

It is associated with high risk of mental retardation, epilepsy, glaucoma, buphthalmos, and retinal detachment

A

Sturge Weber syndrome

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13
Q

Hemihypertrophy of entire body that does not follow lines of Blaschko

A

Diffuse Capillary Malformation with Overgrowth

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14
Q

Well-delineated CM if vermilion border, tip of nose with macrocephaly is pathognomonic for this condition

A

Macrocephaly-Capillary Malformation

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15
Q

Macrocephaly- Capillary Malformation have no risk for mental retardation.
True or False

A

False, at risk

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16
Q

50% of capillary malformations are located on the

A

Face

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17
Q

White cutaneous spots surrounded by pale halo of redness seen in both congenital forms of Capillary Malformation

A

Bier spots

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18
Q

Inherited CMs are seen at birth and blanch on pressure with diascopy.
True or False

A

True

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19
Q

In which inherited CM is CM more telangiectatic in appearance, esp. periorally and on the upper thorax.

A

CM-AVM2

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20
Q

Phakomatosis pigmentovascularis, Sturge Weber syndrome, facial CM with hypertrophy have mutations in

A

GNAQ or GNA11

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21
Q

Macrocephaly-Capillary Malformation have a gene defect in

A

AKT3, PIK3CA, PIK3R2

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22
Q

Mutation of CM-AVM1?

CM-AVM2

A

1-RASA1

2- EPHB4

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23
Q

Major concern for patient with CM is

A

Cosmetic

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24
Q

In Atopic dermatitis, psoriasis, or acne, lesions are worse in the area of CM

A

Meyerson phenomenon

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25
Q

Histologically, CM has

A

Dilated capillaries of papillary and upper reticular dermis with areas of increases number of normal looking capillaries

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26
Q

Stain for CM

A

S100

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27
Q

Imaging studies is mandatory for CM.

True or False

A

False, not mandatory except in rare situations (painful, warm, spontaneously bleeds)

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28
Q

If CM is painful, warm, or spontaneously bleeds; ____ is indicated to exclude Dx of fast flow malformation

A

Doppler UTZ

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29
Q

In SWS, ophthalmologic and neurologic examination is done during the first months of life and repeated ___ until puberty, even if normal at younger age

A

Once a year

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30
Q

___ should be done to evaluate occurrence of leptomeningeal CVM

A

Brain MRI

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31
Q

In DCMO, ____ is needed to evaluate progressive discrepancy around the age of _____ years

A

Scaniometry (leg length films)

4-6 years old

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32
Q

___ is indicated for multifocal lesions

A

Genetic testing

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33
Q

Pale, uncharacteristic, and inconspicuous CMs can be a sign of

A

Phosphatase and Tensin Homolog Hamartoma Tumor Syndrome

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34
Q

In the presence of leg length discrepancy over ___ cm, ____ is the management to prevent compensatory tilting of pelvis.

A

1.5cm, early - adapted shoe lift

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35
Q

___ is necessary for orthopedic considerations when child is 11-13 years old

A

Epiphysiodesis

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36
Q

Gold standard treatment for most CM

A

PDL 585-595nm, short pulse duration (400-1500ms) for 6-12 sessions

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37
Q

Laser treatment is more efficient on cervicofacial and trunk area than on extremities.
True or False

A

True

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38
Q

Is used to treat pyogenic granuloma or lip hypertrophy

A

Contour resection

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39
Q

Most common referral to specialized centers for vascular anomalies

A

Venous anomalies

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40
Q

Associated with consumptive coagulopathy in ___% of cases

A

40

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41
Q

Congenital lesion made up of venous type vessels of the skin or mucosa but can involve any structure or organ

A

Venous malformation

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42
Q

___% of VMs are located in cervicofacial area; ___% on extremities

A

50%; 37%

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43
Q

Inherited VMs reaches its maximum by ___ years of age

44
Q

60% Mutations in VM, mucocutaneous venous malformation (VMCM), and blue rubber bleb nevus (BRBN)

45
Q

It is characterized by one large ‘dominant’ VM lesion associated with multiple small dark blue nipple-like lesions on palms and soles

A

Blue rubber bleb nevus syndrome

46
Q

Extracutaneous manifestations of BRBN include GIT, predominantly the ___. Complication: ____

A

Small bowel, acute hemorrhage —> death

47
Q

Complications associated with BRBN and abdominal pain

A

Intussusception
Volvulus
Bowel infarction

48
Q

In BRBN, ___ should be perfomed to detect GI lesions

A

Endoscopy
Colonoscooy
Wireless capsule endoscopy

49
Q

Management of GI BRBN lesions

A

Iron supplementation
Blood transfusions
Endoscopic coagulation with Nd:Yag laser, bipolar or argon plasma coagulation

50
Q

Rare, characterized by multiple endochondromas associated with subcutaneous VMs of distal extremities

A

Maffucci syndrome

51
Q

Maffucci syndrome is caused by somatic mutations in ___

A

IDH1 and IDH2

52
Q

Patients with Maffucci syndrome have a high incidence of malignancies

A

40% chondrosarcoma, glioma, fibrosarcoma, angiosarcoma

53
Q

The most common location of VM

A

Head & neck

54
Q

Small (<5cm) multifocal, asymptomatic VMs present at birth involving the lips, tongue, and buccal mucosa

A

Mucocutaneous venous malformation (VMCM)

55
Q

VMCM is an ___ mutation of ___

A

AD; TIE2 (angiopoietin receptor)

56
Q

Similar to VMCM but lack family history

A

Multifocal venous malformations (MVMs)

57
Q

MVM is caused by mutation in

58
Q

Bluish to purple, raised venous anomaly characterized by multifocality, hyperkeratosis, nodularity with a cobblestone surface present at birth

A

Glomuvenous malformation

59
Q

It is painful on palpation and noncompressible often located on the extremities

A

Glomuvenous malformation

60
Q

Patient with GVM have normal mental and physical development

True or False

61
Q

GVm is caused by dominat, loss-of-fxn mutation in

A

Glomulin gene

62
Q

Histologically, GVM is characterized by

A

Distended venous channels with surrounding mural glomus cells

63
Q

Glomus cells stain positive for (2)

A

Smooth muscle alpha actin

Vimentin

64
Q

GVM has a more superficial location than VM

True or False

65
Q

In patients with GVM, management is

A

Skin graft with excision

66
Q

Rare congenital vascular anomaly with immunohistochemical profile similar to vascular neoplasms (GLUT1 positive) on the legs

A

Verrucous venous malformation (VVM)

67
Q

VVM involves muscles/tissues

True or False

A

False, never

68
Q

Complication of VVM

A

Ulceration

69
Q

VVM is caused by somatic mutations of

70
Q

Histologically, VVM is characterized by

A

Hyperkeratotic epidermis on top of small blood vessels with multilaminated membrane located in the dermis and subcutis

71
Q

VVM is best treated with

A

Surgical excision

72
Q

Common feature when VM is located in the temporal muscle

73
Q

Local thrombosis is responsible for acute pain which resolves as

A

Phleboliths

74
Q

VM produces more severe leg length discrepancy than Klipple Trenaunay syndrome
True or False

A

Less severe

75
Q

Localized intravascular coagulopathy, associated with VMs, presents with ____ in 40% of patients

A

Elevated D dimer level (>500ng/ml)

76
Q

Severe Localized IV Coagulopathy is more common in ___ and presents with low fibrinogen levels

A

Large VMs of extremities

77
Q

Triggers conversion of LIC to DIC

A

Surgery
Sclerotherwpy
Hormonal influences

78
Q

Pathognomonic of severe LIC

A

Low fibrinogen level

79
Q

Pathognomonic of LIC in a VM in plain radiography

A

Calcifications of phleboliths

80
Q

__ is present in 20% of patients with extensive head and neck VMs that presents as headaches

A

Cerebral developmental venous anomaly

81
Q

Major complications of VM

A

GI hemorrhage

Airway compression

82
Q

Most common indications for Tx of VM

A

Pain

Functional impairment

83
Q

Compression decreases pain and swelling in most VM, except for

84
Q

Treatment for LIC

A

LMWH (100IU antiXa/kg/day)24 hours before and 5 days after surgery.
Usually given for 2 weeks

85
Q

For difficult to treat VM not amenable to surgery

A

Rapamycin (MTOR)

86
Q

Rapamycin should not be given to ___ VMs that respond to standard of care

A

Small, localized asymptomatic

87
Q

Primary treatment for VM

A

Percutaneous IL sclerotherapy

88
Q

Most efficient sclerosing agent

89
Q

Alternatives for ethanol sclerotherapy

A

Sodium tetradecyl sulphate foam

lauromacrogol

90
Q

Side effects in 2% of detergent sclerosants

A

Neurologic complications

91
Q

Single sclerotherapy session is needed to completely treat VM
True or False

A

False, multiple due to propensity to recanalize and recur

92
Q

Worldwide prevalence of vascular malformations is 0.3%, mostly accounted for by___

A

Capillary malformation

93
Q

Vascular malformation are histologically characterized by

A

Enlarged, tortuous cells with quiescent endothelium

94
Q

Most common vascular tumor

A

Hemangioma

95
Q

For __ mutation, biopsy of affected tissue is needed; for __ mutation, blood test is sufficient to make a diagnosis

A

Somatic

Inherited

96
Q

Can be cutaneous hallmark of occult spinal dysraphism, if located on lumbosacral area

A

Capillary malformation

97
Q

Blanchable pink patches often located on the nape (81%), eyelids (45%), and glabella (33%)

A 
Nevus flammeus neonatorum
Storks bite
Salmon patch
Angel’s kiss
Nevus simplex
98
Q

Nevus simplex disappear spontaneously by __

A

1-4 years old

99
Q

Phakomatosis pigmentovascularis is associated with the ff lesions

A

CALMs
atypical Mongolian spot not on sacrum
nevus spilus
Pigmented nevus

100
Q

Macrocephaly-Capillary malformation has also been associated with reports of

A

Wilms tumor

101
Q

In inherited CM-AVM, the risk of having a fast flow lesion is more frequent with

102
Q

It has associated toe anomalies in 30% and prominent veins

103
Q

In CM-AVM, there is __% risk of having brain or spine AVM

104
Q

___ is indicated to rule out presence of Wilms tumor

A

Renal ultrasound

105
Q

During the first few weeks of life, CM may slightly fade due to decreased level of Hgb.
True or False

106
Q

Early treatment during childhood leads to less laser sessions.
True or False

A

False, no effect

107
Q

Laser can be used to treat hypertrophy.

True or False

7th (23 decks)

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