Vascular Embryology

Jim Kaan, PGY 1

08/07/2017
Review of Terminology
Vasculogenesis = de novo formation of embryonic blood
vessels
Angiogenesis = creation of new blood vessels from
existing blood vessels
Arteriogenesis = growth of collateral vessels

Neovascularization = formation of new blood vessels

Vasculogenesis
Vasculogenesis
Mesoderm

Hem-angioblasts

HSCs Angioblasts

Hematopoietic Endothelial
Cells Cells
 Day 17

Splanchnic
Mesodermal
Cells
- Indian Hedgehog
- Bone Morphogenic
Protein
- TGF-b
- VEGF
- Angiopoietin-2
- Nitric Oxide Synthase
- Angiopoietin-1 Tight Junctions
- VEGF fenestrations
Vasculogenesis
Aortic Arch Development
 Normal Arch Development - Review
Truncus arteriosus: proximal part of the
ascending aorta and pulmonary trunk
Aortic sac Ascending aorta and right
brachiocephalic trunk
1st and 2nd arches dont worry about it
3rd arch left and right common and
internal carotid arteries
4th arch
R part of the right subclavian
L part of the aortic arch
5th arch non-existent in humans
6th arch pulmonary trunk
R right pulmonary trunk
L left pulmonary artery & ductus
arteriosus
Dorsal aorta
R part of the right subclavian
L arch of aorta and descending
aorta
Normal Arch Development - Review
 Aortic Arch Anomalies
Normal/classic variant:
65% of the population

Bovine Arch:
Left common carotid from the
brachiocephalic
22% of population (73% of arch
anomalies)

Other arch anomalies (less the 3%)

Shortened brachiocephalic
Isolated left vertebral
Left brachiocephalic
Aberrant Right Subclavian
Aortic Arch Anomalies
Patent Ductus PDA

Arteriosus (PDA)
Most common vascular Pulmonary
anomaly R-sided Aorta
Arteries
From left distal 6th arch
Constricts at birth
Normally obliterated by 1
month
Prolonged patency pHTN
cor pulmonale
 Coarctation of the Aorta
Usually at level of ligamentum
arteriosum
MC location: Postductal
Etiology: Unclear
Ectopic oxygen sensitive
muscle tissue from ductus
arteriosus
Ischemia below constriction
collateralization through
intercostals
Notching of ribs 3-8 on CXR
Progresses to LVH, CHF, pulmonary
and upper extremity HTN
Associated with: PDA, VSD, AS
 Double Aortic Arch
Right Dorsal Aorta Distal to the right
7th intersegmental artery persists
Becomes part of the vascular ring
around the esophagus and aorta
Double Aortic Arch
 Right Aortic Arch
Involution of left dorsal aorta distal to
the left 7th intersegmental, with
persistence of the right
Ligmamentum arteriosum arises from
the distal right sixth arch instead of
the left
 Aberrant Right Subclavian
aka Retroesophageal Right Subclavian
aka Lusorian Artery

Incidence = 0.5%-2%
Mostly asymptomatic
5% esophageal
compression
Dysphagia Lusoria
Ortners Syndrome
Aneurysmal changes
= Kommerell Diverticulum
Aberrant Right Subclavian
 Development of the Extremities
Limbs start as limb buds, with
an apical ectodermal ridge in
the anteroposterior plane

Gremlin
Fgf4
Hoxd13
Bmp2
Gli1
Hox genes
 Development of the Extremities - Upper
Initially, a capillary network
provides blood supply to the
limb
A single large central artery
then develops
Feeds into a peripheral
capillary bed & then collects in
a marginal sinus beneath the
apical ectodermal ridge
 Development of the Extremities - Upper
The primary axial artery
becomes the brachial artery
Gives rise to the branches in
the newly developing forearm
Large central artery
primary axial artery
brachial artery
 Development of the Extremities - Upper
High origin of the radial artery above
the level of the elbow or cubital fossa
Occurs in 14% of the population
Persistence of the median artery into
the palm
Seen in up to 12% of the
population

Persistent Median Artery

 Development of the Extremities - Lower

Sciatic artery = Sciatic regress and The sciatic involutes

continuation of External Iliac
the internal iliac develops into CFA
arteries or of the
umbilical arteries
 Persistent Sciatic Artery (PSA) - Background
Normal development:
Sciatic Artery continuation of internal
iliac in early development
Atrophies as femoral artery develops

Sciatic artery fails to regress

hypoplastic SFA
PSA becomes principal blood supply

Incidence = 0.05%
First described in 1832
First account of aneurysmal rupture
resulting in death 1864
Persistent Sciatic Artery (PSA) - Variations

Complete
PSAs
 Persistent Sciatic Artery (PSA) - Anatomy
Passes through the sciatic notch
with the sciatic nerve
Remains posterior to the adductor
magnus
 Aneurysmal PSA: Symptoms
Most frequent symptoms
Pulsatile buttock mass
Sciatic neuropathy
Acute Limb Ischemia

Most frequent symptoms

Pulsatile buttock mass
Sciatic neuropathy
Acute Limb Ischemia
Popliteal Entrapment Syndrome
 Popliteal Entrapment Syndrome - Background
Embryology:
Competition for space in
the popliteal fossa
Delayed migration of
the medial head of the
gastrocnemius
Pathophys:
Hypertrophy of
surround muscles
Repeated arterial
compression =
Repetitive trauma
early atherosclerosis
stenosis
aneurysmal changes
Incidence < 3%
mostly asymptomatic
Bilateral in 60% of cases Normal Popliteal
Popliteal Entrapment Syndrome - Types
 Popliteal Entrapment Syndrome - Clinical

History
Younger individuals < 40 years old
Athletes
Hx of aching pain, numbness,
tiredness
Periods of cramps of LE coldness
Relieved by rest

Physical Exam
Normal limb appearance
Posterior calf cold to touch
Diminished pulses with active plantar
flexion
Questions
The origin of a persistent sciatic artery is:
a. a right-sided aortic arch
b. the left vertebral artery arising directly from the aortic arch
c. bovine anatomy of the left common carotid artery
d. an aberrant right subclavian artery
e. an aberrant innominate artery

The persistent sciatic artery is most commonly at risk of:

a. No sequela
b. Acute occlusion
c. Aneurysmal degradation
d. Source of distal arterial emboli
e. Sciatic nerve Ischemia
The correct answer is C: The internal iliac artery

- In early development, sciatic artery is a continuation of the

internal iliac artery
- Primary blood supply to the lower limb bud during early fetal
development
- Normally involutes during fetal development,
- Persistence of the sciatic artery is a rare vascular anomaly that
may be of surgical significance.
- Failure to appreciate the persistent sciatic artery as the major
inflow into the lower extremity may lead to inappropriate bypass
of apparent occlusive disease of the superficial femoral artery.
- Also frequently aneurysmal:
- May cause critical limb ischemia resulting from thrombosis
or embolization of aneurysm thrombus.
A 50 year old woman with left calf claudication for 40 years has developed
similar right-sided symptoms in the past year. Symptoms occur after walking 4-5
blocks. She has HTN, HLD. On physical exam, she has 1+ femoral pulses
bilaterally, and 2+ popliteal and pedal pulses bilaterally. No foot ulcers or other
cutaneous changes are evident on the lower extremities.
ABIs:
R: 0.68 ; 0.4 (after exercise)
L: 0.74 ; 0.71 (after exercise)

For the diagnosis of popliteal artery entrapment syndrome, what is the best
imaging test?
a. Stress position CTA
b. Stress position MRA
c. Stress position arteriogram
d. Treadmill ABI test
e. EMG
The correct answer is B: Stress position MRA

- Angiograms will show a short-segment occlusion of the popliteal

- A stress positional MRA/MRI can confirm the presence of anomalous
musculotendinous bands and the suspected diagnosis of anatomic PAES
- With forced plantar flexion, a band-like compression of the popliteal artery
may occur
- Check both sides as these anatomic anomalies are frequently bilateral

Treatment/Intervention
- Patients with documented musculotendinous anomalies in the popliteal
fossa require surgical intervention
- Approach: retrogeniculate approach
A 47 year old man presents with difficulty swallowing.
Esophagogastroduodenoscopy (EGD) reveals a pulsatile mass impinging on the
posterior wall of the esophagus. A CT scan of the chest reveals an arterial variant
which is most likely to be:

a. a right-sided aortic arch

b. the left vertebral artery arising directly from the aortic arch
c. bovine anatomy of the left common carotid artery
d. an aberrant right subclavian artery
e. an aberrant innominate artery
The correct answer is D: Aberrant Right
Subclavian Artery (aka Lusorian artery)
Dysphagia lusoria = dysphagia 2/2 to
esophageal compression by the aberrant right
subclavian

- Can also cause Ortners Syndrome

Rare cardiovocal syndrome referring to vascular
compression of the recurrent laryngeal nerve.
- Aberrant right subclavian
- Dilated Left atrium
- Pulmonary hypternsion
- Thoracic aortic aneurysms
- Enlarged pulmonary artery