Chapter 130 - Renovascular and aortic developmental disorders

Question 1

Mid aortic syndrome first used by

Answer 1

1963 Sen

Question 2

Classification of mid aortic syndrome and its relative prevalence

Answer 2

Suprarenal 69%
Intrarenal 23%
Infrarenal 8%

Diffuse hypoplasia of abdominal aorta - also along spectrum of disease

Question 3

Embryology in mid aortic syndrome

evidence in this theory

Answer 3

Two dorsal aorta over-fuse

1) single lumbar instead of paired
2) multiple renal accessory arteries (2x likelihood)

Question 4

Genetic syndromes associated with mid aortic syndrome

Answer 4

1) Neurofibromatosis NF-1 (13-25%)
2) William syndrome
3) Alagille syndrome
4) Tuberous sclerosis

Question 5

Inflammatory/infectious etiology of mid aortic syndrome

Answer 5

1) maternal rubella in 1st trimester
2) takayasu aortoarteritis
3) umbilical artery catheterization (also associated with aortic thrombosis and mycotic aneurysms)

Question 6

Aortic coarctation association with splanchnic and renal artery stenosis

Answer 6

Splanchnic 87%

Renal 62%

Question 7

Etiology of pediatric renal artery stenosis in NA and Asia/Africa/SA

Answer 7

NA: developmental (NF-1)

Asia/Africa/SA: inflammatory, Takayasu

Question 8

Embryologic origin of CA and SMA

Answer 8

Vitelline arteries

Question 9

Rate of renovascular HTN as a cause of 2ndary HTN in kids

Answer 9

8-10%

Question 10

Intetinal ischemia due to mid aortic syndrome

Answer 10

6% of mid-aortic syndrome patients only

Question 11

Symptoms of pediatric renovascular HTN

Answer 11

1) HTN
2) headache, seizure, visual disturbance
3) epistaxis
4) renal insufficiency
5) Bell’s palsy
6) hemorrhagic stroke
7) hypertensive encephalopathy
8) flash pulmonary edema

Question 12

Renal US to detect aortorenal stenosis sen and spe

Answer 12

90% sen

68% spe

Question 13

Rate of cerebral vascular abnormalities in mid aortic syndrome

Answer 13

3-13%

occlusive and aneurysmal

Question 14

Goal of HTN therapy in children

Answer 14

Reduce to 95percentile

If LVH or target organ identified then reduce to 90th percentile

Question 15

Classes of antihypertensives

Answer 15

1) ACEi
2) ARBs
3) combined alpha beta blocker
4) beta blocker
5) diuretic
6) central alpha agonist
7) calcium channel blocker
8) peripheral alpha antagonist
9) vasodilators

Question 16

Principle of antihypertensive meds

Answer 16

Use one until max dose or side effect

Question 17

Diuretic use in controlling pediatric blood pressure

Answer 17

Used in combination with something else or else increase renin release will exacerbate HTN

Question 18

Surgical treatment of mid aortic syndrome

Answer 18

1) Patch angioplasty
2) thoracoabdominal bypass
3) retroperitoneal tissue expander

Question 19

Conduit for thoracoabdominal bypass in mid aortic syndrome

Answer 19

ePTFE > dacron because less likely to have postimplantation dilatation

Question 20

When does patch not work in mid aortic syndrome

Answer 20

Aorta too small such that the suture lines overlap

Question 21

Diameter of grafts to use in thoracoabdominal bypass for mid aortic syndrome

Answer 21

Young children: 8-12 mm
Early adolescent 12-16 mm
Late adolescent/adults 14-20 mm

Question 22

When is it ok to not leave graft redundancy in thoracoabdominal bypass of youth

Answer 22

after age 9 because axial growth is limited

Question 23

Rate of reoperation in mid aortic syndrome

Answer 23

10% in 5-12 years

Question 24

At what age is renal revasc more durable

Answer 24

after 3 years of age

defer repair whenever possible until age 3

Question 25

Conduit in pediatric renal reconstruction

Answer 25

Internal iliac artery

GSV not good - aneurysmal degeneration in 50%

Question 26

Why are splenorenal reconstructions not idea

Answer 26

Chance of developing celiac stenosis in this patient population

Question 27

Indication for nephrectomy in pediatric

Answer 27

1) Non-reconstructable disease (multiple intrarenal stenosis)
2) diminutive non-functioning kidney 2-3cm in size
3) contralateral kidney assumed to be adequate

Question 28

Large series of pediatric renovascular HTN

Answer 28

1) University of Michigan
2) Hospital Beaujon (France)
3) Cleveland clinic
4) Vanderbilt University (philadelphia)

Question 29

Cure rates of HTN with renovascular revasc in children

Answer 29

66-70% cure
23-27% improvement
3-11% fail

Question 30

Rate of complication following endovascular treatment of mid aortic syndrome

Answer 30

30%

Question 31

Endovascular treatment success for mid aortic syndrome

Freedom from reintervention at 1 and 5 years

Answer 31

1 year 55%

5 year 33%

Question 32

Remedial open revasc of renal artery in pediatric after failed endo vs primary open revasc

cure rate and improvement rate of HTN

Answer 32

Remedial 25% cure, 54% improve

primary 70% cure, 27% improve

Question 33

Principle of endovascular treatment of congenital reno stenosis

Answer 33

1) no stent - intimal hyperplasia
2) only at high centers
3) high risk of rupture, pseudoaneurysm, recurrence