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4.1 · Hypertension
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a
b
. Fig. 4.1.3 Color Doppler sonogram of the renal arteries demonstrates normal arterial waveform of the right renal artery ( a ) and the left renal
artery ( b )
Signs on CT
5 Arterial cut-off sign : the course of the renal artery is
seen interrupted on contrast-enhanced images. It
is a sign of renal artery obstruction (. Fig. 4.1.4 ).
5 Rim sign of vascular compromise : the kidney fails to
enhance on contrast-enhanced images, with a thin
rim of subcapsular enhancement seen paralleling the
renal margin (. Fig. 4.1.4 ). This sign is caused by
renal medullary arterial perfusion interruption in
cases of renal artery obstruction, with preserved
perfusion of the renal cortex by the capsular
perforating vessels. This sign can be seen in cases of
renal vein thrombosis, renal arterial occlusion, and
acute tubular necrosis.
5 Reverse rim sign : this sign is seen as hypodense
renal cortex against a contrast-enhanced
background of intact medullary arterial perfusion
(. Fig. 4.1.4 ). This sign is seen in cases of
compromised renal arterial perfusion in cases of
cortical necrosis.
. Fig. 4.1.4 Axial postcontrast-enhanced CT illustration shows
the arterial cut-o sign ( solid arrowhead ), rim sign of vascular
compromise ( open arrowhead ), and reverse rim sign ( arrow )
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Signs on MRA
5 Magnetic resonance angiography (MRA)
demonstrates the aortic vessels clearly, and it is
mostly used after detection of RAS on Doppler
sonography for surgical planning (. Fig. 4.1.5 ) .
4
Signs on Chest Radiograph
5 In chronic cases of aortic coarctation, the ribs
show irregular lower border representing rib
notching (. Fig. 4.1.6 ). This condition can be also
seen in other conditions affecting the intercostals
neurovascular bundle like superior vena cava
obstruction syndrome and neurofibromatosis of
the intercostals nerves.
5 The aortic arch is often bulging with dilatation of
the aortic knuckle (. Fig. 4.1.7 ).
5 Pseudocoarctation of the aorta is seen as
elongated aortic knuckle appearing like a
posterior mediastinal mass.
. Fig. 4.1.5 MRA image shows >50 % stenosis of the left renal
artery ( arrowhead ) in a patient with peripheral vascular disease
Coarctation oftheAorta
Coarctation of the aorta is a condition characterized by aortic lumen narrowing, most commonly located below the origin of the le subclavian artery (the aortic isthmus near the
ligamentum arteriosum), causing hypertension below the
level of the narrowing.
Patients with aortic coarctation o en present with severe
hypertension that does not respond to antihypertensive medication and chest pain that radiates to the back. Patients with
aortic coarctation carry the risk of aortic dissection, which is
characterized by aortic wall intimal tear and leaking of the
blood in between the aortic wall layers.
When it is chronic, aortic coarctation causes dilatation of the
internal mammary and intercostals arteries. Chronic high-pressure pulsation of the intercostals arteries over the inferior aspect
of the ribs may result in rib notching. Coarctation of the aorta can
be seen in up to 30 % in patients with bicuspid aortic valve.
Pseudocoarctation of the aorta is a relatively rare condition
characterized by kinking of the aorta at its isthmus near
the ligamentum arteriosum without lumen narrowing.
Pseudocoarctation resembles true coarctation; di erentiation is
possible by looking for changes of the collateral circulation,
where there will be no enlarged peripheral vessels, signs of pressure gradients, or rib notching. e condition is symptomless
and can be associated with congenital bicuspid aortic valve.
. Fig. 4.1.6 Plain chest radiograph shows rib notching in a
patient with severe aortic coarctation since 12 years
( arrowheads )
. Fig. 4.1.7 Posteroanterior plain chest radiograph in a
patient with coarctation of the aorta shows bulging of the
aortic knuckle
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Signs on CT and MRI
5 Aortic coarctation classically is seen in sagittal
reformatted images as a narrowing of the aortic
lumen located at the area of the aortic isthmus,
giving the classic “inverted shape of 3”
(. Fig. 4.1.8 ).
5 Aortic dissection is seen as hypodense layer found
within the aortic lumen on postcontrast-enhanced
images representing the false lumen (. Fig. 4.1.9 ).
Stanford type A dissection involves dissection of the
ascending aorta and the arch up to the origin of the left
subclavian artery. Stanford type B dissection involves
dissection of the descending aorta below the origin of
the subclavian artery. Type A is managed surgically,
while type B is managed medically, providing evidence
of end-organ ischemia not being present.
5 Aortic pseudocoarctation is seen on axial CT images as
higher than normal located arch, with a well-de ned
enhancing vascular mass located near the aortic arch,
representing the kinked portion of the arch.
. Fig. 4.1.8 Sagittal MRA of a patient with coarctation of the
aorta shows the inverted shape of 3, which is a characteristic of
this condition ( arrowhead )
Polyarteritis Nodosa
Polyarteritis nodosa is a rare disease characterized by aneurysmal, nodular lesions that a ect the medium-sized and
small-sized arteries due to brinoid necrotizing vasculitis.
Vasculitides are a diverse group of diseases characterized
by in ammation and necrosis of all three coats of the vessels
(intima, media, and adventitia). ey are divided into largesized vessel vasculitis (e.g., Takayasu and giant cell arteritis),
. Fig. 4.1.9 Axial CT angiography shows the classical intimal
ap in a patient with aortic dissection Stanford type A
medium-sized vessel vasculitis (e.g., polyarteritis nodosa and
Kawasaki disease), and small-sized vessel vasculitis (e.g.,
Wegener’s granulomatosis and Henoch–Schönlein purpura).
Polyarteritis nodosa commonly causes multiple arterial
aneurismal wall formation, plus fragmentation and degeneration of the adventitia layer. Necrosis starts in the media layer
and spreads to involve the entire width of the vascular wall.
is in ammation and necrosis are associated with eosinophilic in ltration, brin deposition, and destruction of the
elastic tissue and the intima layer. Vascular thrombosis or an
aneurysm, mainly at the vessels bifurcation or the hilar region
of viscera, may occur. Later, a granuloma is formed at the area
of previous in ammation with broblast proliferation. A
healed stage is characterized by vascular recanalization and
formation of a nonvascularized brous mass that completely
replaces a section of the vessel wall. e disease rapidly progresses once started, and death may result from strokes or
myocardial infarctions within 2–3 months a er onset.
Any organ can be a ected by PAN, including the central
and peripheral nervous system. e kidney is the most
commonly involved (80–90 %), followed by the gastrointestinal (GI) tract (50–70 %). Clinically, patients present with
vague signs and symptoms like fever, myalgia, headache, and
malaise. Renal artery involvement, especially at the renal
hilum, o en results in rapidly progressing hypertension.
Involvement of the GI tract may present with lower GI bleeding, abdominal pain, and vomiting (6 % of cases). Peripheral
nervous system involvement compromises the nervous tissue
blood supply causing polyneuropathy. Other lesions involve
pericarditis, myocardial infarction, and purpuric skin rash.
Up to 30 % of patients test positive to hepatitis B surface antigens, and up to 50 % of patients have arthralgias.
Laboratory investigations usually show leukocytosis with
eosinophilia (4 %), anemia, uremia, and high erythrocyte
sedimentation rate.
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in ammatory changes are responsible for the nonspeci c,
Signs on Angiography or MRA
Polyarteritis nodosa is suggested by detection of
multiple aneurysms up to 1cm in diameter within the
renal, mesenteric, hepatic, or central venous vasculature.
This nding is not pathognomonic since it can be seen in
patients with necrotizing angiitis associated with drug
abuse. The history and the high suspicion of PAV with
4
this angiographic picture help to di erentiate the two
entities.
systemic in ammatory symptoms. In contrast, the late phase
is characterized by arterial symptoms with no systemic symptoms. e absence of the systemic symptoms in the late phase
is attributed to the development of systemic collaterals; however, this is not seen in all patients. In the late phase, the coronary arteries might be a ected. Hypertension, arterial
stenosis, aneurysms, and dissection all can be seen in late
phase of TA.
Criteria forTA Diagnosis (TA Diagnosis
Requires atLeast Three Criteria)
Signs on CT
5 The bowel walls are thickened and show
white-attenuated target sign after contrast injection.
5 CT angiography shows multiple aortic aneurysms
in the branches of the SMA or the celiac trunk.
5 I n spotted nephrogram , small vessel occlusion in
cases of PAN may cause patchy perfusion of the
kidney on IVU or contrast-enhanced CT due to
multiple infarctions (. Fig. 4.1.10 ). This sign can be
also seen in scleroderma and hypertensive
nephrocalcinosis.
5 Age of presentation <40years.
5 Limbs claudication.
5 Decrease one or both brachial arteries pulse.
5 Blood pressure di erence of the extremities >10mmHg.
5 Bruit heard over the aorta or the subclavian arteries.
5 Angiographic abnormalities include arterial occlusion,
stenosis, or aneurysms of the aorta or its main branches.
Commonly, these abnormalities are observed bilaterally.
Signs on CT and CT Angiography
5 In the acute phase, there is thickening of the great
vessel wall without signs of calcification due to
inflammation and intramural hematomas
(especially the aorta) (. Fig. 4.1.11 ).
5 The thickened vascular wall may show
enhancement in early contrast phase due to
inflammatory hyperemia.
5 Aneurysmal dilatation of the aortic root or its
major branches is mainly observed in late chronic
phase of the disease.
5 Signs of dissection, stenosis, or occlusion of the
major vessels may be seen (. Fig. 4.1.12 ) .
. Fig. 4.1.10 Axial CT illustration demonstrates the spotted
nephrogram that may be seen in patients with polyarteritis
nodosa
Takayasu Arteritis
Takayasu arteritis is a rare, granulomatous disease characterized by in ammation of the large vessel walls, leading to progressive stenosis, aneurysms, or limb ischemia. e disease
a ects mainly the aorta and its major branches.
TA a ects mainly young females between 20 and 30 years
of age, who present with nonspeci c systemic in ammatory
symptoms like fever, night sweat, weight loss, myalgia, and
arthralgia. TA has two main phases with di erent clinical presentations, an early and a late phase. e early phase is characterized by nonspeci c in ammation in the blood vessels walls
a ecting the media and the adventitia layers. ese nonspeci c
. Fig. 4.1.11 Axial CT angiography in a 50-year-old female
patient presented with nonspeci c systemic in ammatory
symptoms with absent right arm pulsation and limb claudication.
Patient was suspected to have Takayasu arteritis by the attending
physician. CTA con rmed the acute thickening of the aortic wall
( arrowheads ), with occlusion of the right subclavian artery and
multiple aneurysms a ecting the carotid arteries (not shown)
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. Fig. 4.1.12 Aortic-renal angiography in a patient with
chronic Takayasu arteritis shows multiple small stenoses and
dilatations a ecting the right renal artery and the abdominal
aorta in a milder degree
Midaortic Syndrome
Midaortic syndrome (MAS) is a nonspeci c arteritis that
a ects the midportion of the abdominal aorta and its main
branches.
MAS classically starts from the infrarenal part of the
aorta and progresses proximally to involve the renal arteries
(80 %), SMA, and the celiac artery (25 %). MAS mainly a ects
children and young adults who typically present with renovascular hypertension due to bilateral RAS.If untreated, the
disease is fatal by the age of 30, with many patients experiencing intracranial bleeding due to malignant hypertension.
e inferior mesenteric artery (IMA) and the common iliac
arteries are almost never involved.
How Can You Di erentiate Between MAS and TA?
z
5 Takayasu arteritis o en presents with systemic symptoms
of fever, malaise, and weight loss. ese features are not
part of MAS.
5 MAS a ects mainly children and young adults, while
Takayasu arteritis mainly a ects females between 20 and
30 years of age.
5 MAS exclusively a ects the mid-abdominal aorta, while
Takayasu arteritis involves any vessels and can even a ect
the pulmonary artery, causing pulmonary hypertension.
Signs on Doppler Sonographs
5 Stenosis of the mid-abdominal aorta.
5 Renal artery stenosis is often seen unilaterally or
bilaterally.
Signs on CT Angiography
5 The aorta shows stenosis typically from the
infrarenal portion and extends proximally to
involve the renal arteries, IMA, or the celiac.
5 The IMA and the common iliac vessels are spared.
Preeclampsia
Preeclampsia is a pregnancy-related condition characterized
by hypertension, lower leg edema, and proteinuria. In contrast, eclampsia is a life-threatening condition characterized
by the same symptoms as preeclampsia plus tonic–clonic
seizures.
Hypertensive disorders occur in about 3–10 % of all pregnancies, and the incidence of preeclampsia ranges between 10
and 15 % in primigravida ( rst birth) and 5.7–7.3 % in multiparas (multiple pregnancies). Hypertension in preeclampsia
is diagnosed a er 20 weeks of gestation by a diastolic blood
pressure >90mmHg stable over 4h or one measurement of
diastolic blood pressure >110mmHg. Proteinuria is de ned
as a concentration of protein of 0.1g/L or more in at least two
random urine samples collected 4 h or more apart or as
0.3g/L in a 24h urine collection in the absence of urinary
tract infection.
Patients with preeclampsia usually present with hypertension (hallmark of the disease), lower leg edema, proteinuria, headache, visual symptoms, and epigastric pain. e
absence of hypertension in the presence of edema and proteinuria does not exclude the diagnosis of preeclampsia. e
liver is uncommonly a ected by preeclampsia (10 % of cases).
When liver dysfunction occurs, mild elevation of serum
enzymes is common.
HELLP syndrome is a disease characterized by hemolytic
anemia (Hb <11g/dL), elevated liver enzymes, low platelet
count that predisposes to thrombocytopenia (<100,000/μL),
and subcapsular liver hematoma. e incidence of HELLP
syndrome is 2–12 % of preeclampsia cases. Patients o en
present with epigastric pain (65 %), nausea and vomiting
(50 %), and nonspeci c symptoms. Severe hypertension is
not a constant or a frequent nding in HELLP syndrome.
Signs on CT or MRI
5 In patients with HELLP syndrome, the imaging
findings include subcapsular hematoma,
hepatomegaly with bulging of the left lobe, fatty
liver, free abdominal ascites, bilateral pleural
effusions, or bilateral basal lobes atelectasis
(. Fig. 4.1.13 ) .
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. Fig. 4.1.13 Axial abdominal postcontrast CT illustration
demonstrates signs of HELLP syndrome. There is hepatic
subcapsular hematoma ( solid arrowhead ), fatty liver changes
( open arrowhead ), and ascites ( arrow )
Signs on CT and MRI
5 On CT, there are symmetrical, noncontrast-enhancing
hypodensities located in the posterior region of the
occipital and the parietal lobes.
Syndrome (Hypertensive Encephalopathy)
Reversible posterior leukoencephalopathy syndrome
(RPLES) is a disease with unknown cause characterized by
cerebral demyelination in the posterior white matter areas of
the brain (occipital lobes). PRLES is thought to be caused by
increased permeability of the blood–brain barrier in the posterior circulation.
PRLES is typically seen in patients with hypertension and
eclampsia and patients on immunosuppressive and cytotoxic
drugs like cephalosporin and methotrexate. PRLES is a
reversible condition once the cause is removed (e.g., control
hypertension). If the cause persists, it will lead to cerebral
infarction. Patients will present with headache, vertigo, vomiting, seizures, and altered mental status.
5 On MRI, symmetric low T1 signal intensity with high
T2 and FLAIR signal intensities in the region of the
occipital and the parietal lobes (. Fig. 4.1.14 ).
5 There is cytotoxic edema and restricted water di usion
(high DWI signal intensity) in cases of infarction.
Reversible Posterior Leukoencephalopathy
a
b
. Fig. 4.1.14 Axial T1W ( a ) and T2W ( b ) MR illustrations demonstrate bilateral almost symmetrical low T1 and high T2 signal intensity
lesions located in the posterior lobes. This sign with a history of hypertension is diagnostic of RPLES
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Nephroptosis (Floating Kidney)
Nephroptosis, also known as oating or wandering kidney, is
a condition characterized by renal descent of 5cm or more
(or two vertebral bodies) when the patient moves from
supine to an upright position.
Nephroptosis occurs more commonly in slim women
(ten times more common than in males) and a ects the right
kidney more than the le (20 % of cases). Causes of nephroptosis include multiple pregnancies, rapid loss of retroperitoneal fat, variation in the shape of the spinal cord, shallow
Gerota’s fossa, and direct renal trauma.
Patients with nephroptosis are rarely symptomatic.
Symptomatic patients typically present with history of ank
pain in the upright position that reduces or is relieved by
lying down. e pain is attributed to intermittent functional
excretory obstruction, forceful traction of the renal artery
causing renal ischemia, or traction of the perirenal nerves.
e most severe manifestation of nephroptosis is Dietl’s cri-
sis. Dietl’s crisis is a condition characterized by violent paroxysmal colicky ank pain, tachycardia, nausea, chills, oliguria,
hypertension, and transient hematuria or proteinuria. e
condition is caused by acute hydronephrosis due to kinking
or vascular obstruction of the ureters.
On physical examination, the lower pole of the kidney
can be palpated on deep inspiration. e examiner’s nger
should reach over the upper pole of the kidney and push it
down to the navel. On Dietl’s crisis, the kidney is tender on
palpation and may be enlarged.
Historically, nephroptosis is used to be corrected by neph-
ropexy , a surgical procedure characterized by suturing part of
the renal capsule to the surrounding abdominal wall and vertebral column.
examinations (e.g., on supine it is 0.5, and on erect
it is 0.6). The other normal kidney shows no
change in the RI values on both supine and erect
examinations. These findings are explained by the
renal artery tension occurring due to downward
movement of the kidney.
Riley–Day Syndrome (Familial
Dysautonomia)
Riley–Day syndrome (RDS) is a rare inherited disorder characterized by infantile hypertension, postural hypotension,
and recurrent attacks of unexplained fever due to autonomic
nervous system dysfunction.
As a rule, RDS manifests in infancy, which is important to
assume the diagnosis. e major features are o en seen in an
infant or a child with recurrent attacks of unexplained fever,
hypertension, and vomiting. Infants commonly have excessive drooling with swallowing di culties, making them
prone to recurrent aspiration pneumonia. Aspiration pneumonia is the main cause of death in patients with RDS.
Hypertension of RDS is characteristically associated
with excitement. Intermittent attacks of hypertension with
vomiting may cause RDS to be confused with infantile
pheochromocytoma. Postural hypotension can be demonstrated in most patients beyond 2 years of age, and it can be
so marked as to give rise to “blackout spells” when the
patient stands.
Stafne’s Bone Defect oftheMandible
Signs on IVU
5 IVU is the classical diagnostic investigation for
nephroptosis. The patient is injected with the
contrast intravenously, and the kidney is imaged
after opaci cation of the renal parenchyma and
ureters while the patient is supine. The patient is
then imaged while he is on erect position. The kidney
is clearly seen descending caudally from its normal
position between the two lms (e.g., from the level of
L2 to the level of L5). The normal kidney is located
between the lumbar vertebral levels L1 to L4.
Signs on Doppler Sonography
5 In suspected cases of nephroptosis, the resistive
indexes (RIs) of the interlobar parenchyma renal
arteries should be measured on both supine and
erect positions. Both kidneys should be imaged for
comparison. In the ptotic kidney, there is a change
in the RI values >0.1 between the erect and supine
Stafne’s bone defect of the mandible is a rare cyst-like bony
defect with cortical bone thickening with continuity from
the base of the mandible around the gonial angle of the
mandible, under the mandibular canal on panoramic radiography or cone-beam CT.Most cases are seen in hypertensive patients from 40 to 60 years old. e bony defect is
symptomless.
Stafne’s mandibular bony defect is considered as a complication of long-standing hypertension and thought to be
caused by high-pressure exertion by the facial artery over the
mandible (. Fig. 4.1.15 ).
Hypertensive Heart Disease
Patients with long-standing hypertension develop le ventricle hypertrophy due to raised le ventricular wall tension,
which may lead to coronary microangiopathy of the midwall portion of the le ventricle wall. Hypertensive heart dis-
ease is a term used to describe a hypertensive patient with
cardiac failure due to diastolic heart dysfunction with normal
systolic heart function (normal ejection fraction).
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Chapter 4 · Nephrology
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. Fig. 4.1.15 Axial CT illustration of the mandible demonstrates
Stafne’s bone defect of the mandible on the right side ( arrowhead )
Le ventricular hypertrophy can be generalized, reducing
the internal cavity (concentric hypertrophy), or localized to
the interventricular septum (eccentric hypertrophy). Le
ventricular hypertrophy in hypertensive patients is usually
concentric and typically found in moderate to severe
hypertension in middle-aged and elderly patients. Le ventricular hypertrophy can cause atrial brillation and arrhythmias. Also, le ventricular hypertrophy in hypertensive
patients is associated with three- to fourfold increase in the
risk of stroke, a two- to threefold increase in coronary heart
disease, and a threefold increase in peripheral arterial disease.
Signs on Cardiac MRI
5 Patients with left ventricular hypertrophy due to
hypertensive heart disease can show intramural,
mid-wall, or subendocardial delayed contrast
enhancement (e.g., >15min), mostly due to
myocardial ischemia, necrosis, or fibrosis
(. Fig. 4.1.16 ). Patients with delayed contrast
enhancement on cardiac MRI may show
ST-segment depression or T-wave inversion on
electrocardiogram.
. Fig. 4.1.16 Short-axis dark-blood postcontrast cardiac MR
illustration demonstrates left ventricular concentric
hypertrophy with intramural enhancement representing MR
ndings in hypertensive heart disease
Further Reading
Akpunonu BE, et al. Secondary hypertension: evaluation and
treatment. Dis Mon. 1996;42(10):609.
Andersen K, et al. Myocardial delayed contrast enhancement
in patients with arterial hypertension: initial results of cardiac MRI. Eur J Radiol. 2009;71:75–81.
Ando H, et al. Abnormal collateral arterial system in Takaya-
su’s arteritis and Lariche’s syndrome evaluated by whole
body acquisition using multislice computed tomography.
Int J Cardiol. 2007;121:306–8.
Applegate KE, et al. Spontaneous colonic ischemia in a
patient with Riley-Day syndrome. Pediatr Radiol.
1995;25:312–3.
Barber NJ, et al. Nephroptosis and nephropexy - hang up on
the past? Eur Urol. 2004;46:428–33.
Bulum J, et al. Takayasu’s arteritis and chronic autoimmune
thyroiditis in a patient with type 1 diabetes mellitus. Clin
Rheumatol. 2005;24:169–71.
Canyigit M, et al. Imaging characteristics of Takayasu arteri-
tis. Cardiovasc Intervent Radiol. 2007;30:711–8.
Chen P, et al. Color and power Doppler imaging of the kid-
neys. World J Urol. 1998;16:41–5.
Das BB, et al. Midaortic syndrome presenting as neonatal
hypertension. Pediatr Cardiol. 2008;29:1000–1.
4.2 · Polycystic Kidney Disease
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Dineen R, et al. Imaging of acute neurological conditions in
pregnancy and the puerperium. Clin Radiol.
2005;60:1156–70.
Dyer RB, et al. Classic signs in uroradiology. Radiographics.
2004;24:S247–80.
Ferrazzani S. Hypertension in pregnancy. Saudi J Kidney Dis
Transpl. 1999;10(3):298–312.
Fujita T, et al. Takayasu arteritis evaluated by multi-slice com-
puted tomography in old man. Int J Cardiol. 2008;125:286–7.
Ha HK, et al. Radiologic features of vasculitis involving the
gastrointestinal tract. Radiographics. 2000;20:779–94.
Hartman RP, et al. Evaluation of renal causes of hyperten-
sion. Radiol Clin North Am. 2003;41:909–29.
Hoenig DM, et al. Nephroptosis: a “disparaged” condition
revisited. Urology. 1999;54:590–6.
Lewis III VD, et al. e midaortic syndrome: diagnosis and
treatment. Radiology. 1988;167:111–3.
Lip GYH, et al. Hypertensive heart disease. A complex
syndrome or a hypertensive ‘cardiomyopathy’? Eur
Heart J. 2000;21:1653–65.
Moss SW. Floating kidneys: a century of nephroptosis and
nephropexy. J Urol. 1997;158:699–702.
Olivier H, et al. Renovascular disease: Doppler ultrasound.
Semin Ultrasound CT MRI. 1997;18(2):136–46.
Rooholamini SA, et al. Imaging of pregnancy-related compli-
cations. Radiographics. 1993;13:753–70.
Shimizu M, et al. CT analysis of the Stafne’s bone defects of
the mandible. Dentomaxillofac Radiol. 2006;35:95–102.
Son JS, et al. Pseudocoarctation of the aorta associated with
the anomalous origin of the le vertebral artery: a case
report. Korean J Radiol. 2008;9:283–5.
Soulez G, et al. Imaging of renovascular hypertension:
respective values of renal Doppler US, and MR angiography. Radiographics. 2000;20:1355–68.
Stadlmaier E, et al. Midaortic syndrome and celiac disease: a
case of local vasculitis. Clin Rheumatol. 2005;24:301–4.
Strohmeyer DM, et al. Changes of renal blood ow in neph-
roptosis: assessment by color Doppler imaging, isotope
renography and correlation with clinical outcome a er
laparoscopic nephropexy. Eur Urol. 2004;45:790–3.
Taneja K, et al. Pseudocoarctation of the aorta: complemen-
tary ndings on plain lm radiography, CT, DSA, and
MRA. Cardiovasc Intervent Radiol. 1998;21:439–41.
Van Hoe L, et al. Liver involvement in HELLP syndrome: CT
and MRI ndings in two patients. Eur Radiol. 1995;5:331–4.
4.2 Polycystic Kidney Disease
Polycystic kidney disease (PKD) is a disease characterized by
the development of multiple cysts within the kidneys in bilateral fashion. A cyst is de ned as a uid- lled sac lined with a
single layer of tubular epithelium. A cystic kidney is de ned
as a kidney that contains three or more cysts.
Simple renal cyst is the most common renal anomaly. Up
to 22 % of symptomless patients over 70 years old or older
have at least one renal cyst. ere are three types of PKD:
autosomal dominant (adult) PKD, autosomal recessive
(infantile) PKD, and acquired PKD.
Autosomal Dominant Polycystic Kidney
Disease
Autosomal dominant polycystic kidney disease (ADPKD) is
the fourth cause of chronic renal failure throughout the
world. e disease has an autosomal dominant mode of
inheritance as its name states, with a positive family history
of ADPKD elicited in 60 % of patients.
ADPKD is typically seen in adults, with both kidneys
a ected in a bilateral, almost symmetrical, fashion. e kidneys are enlarged in size as the disease progresses. In a patient
<30years old with a positive family history of ADPKD, the
presence of two cysts, either unilateral or bilateral, is su cient to make the diagnosis. In patients >30years old with a
positive family history of ADPKD, at least two cysts in each
kidney are su cient to make the diagnosis.
Patients with ADPKD present with bilateral renal cysts
with enlarged kidneys (100 %), renal pain (60 %), hematuria
(42 %), hypertension (75 %), colonic diverticuli (80 %), and
hepatic cysts (57 %). Potential causes of hematuria in ADPKD
include renal stones formation (20 %) and glomerulonephritis.
Hypertension arises in 75 % of ADPKD with normal
renal functions. Cyst expansion is believed to alter blood ow
by glomerular compression, which results in the release of
rennin, leading to the formation of angiotensin II.
Hepatic cysts occur in 57%of patients with ADPKD, and
they are rare before puberty. Hepatic cysts are believed to
originate from cystic dilatation of the bile ducts. Patients may
present with right upper quadrant pain due to liver capsule
stretching and hepatomegaly.
Colonic diverticuli are seen in up to 80 % of patients with
ADPKD, usually with end-stage renal disease. Patients with
ADPKD are at risk of cerebral aneurysm rupture, which has
a prevalence of <5 %. ADPKD patients with positive family
history of cerebral arteries aneurysm have an increased incidence of developing cerebral aneurysm (22 %) than ADPKD
patients with no family history of cerebral aneurysm (5 %).
ADPKD patients with aneurismal rupture present with signs
of intracranial bleeding or subarachnoid hemorrhage such as
severe headache, neck sti ness, and altered consciousness,
with nausea and vomiting.
Rare manifestations of ADPKD include coronary arteries
or abdominal aorta aneurysm, mitral valve prolapse, aortic
regurgitation, pancreatic cysts (10 %), splenic cysts (5 %), and
inguinal hernias. Patients with ADPKD may develop seminal
vesicle cysts, which present clinically as painful ejaculation,
prostatitis, urinary tract obstruction, or epididymitis.
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Chapter 4 · Nephrology
Signs on US
5 The kidneys show multiple echo-free parenchymal
cysts with typical posterior shadowing.
a
5 Liver, pancreatic, or splenic cysts may be seen
(. Fig. 4.2.1 ) .
b
4
. Fig. 4.2.1 Axial CT postcontrast ( a ) with liver ultrasound ( b ) images show multiple liver cysts in a patient with ADPKD
Signs on CT
5 Typically, both kidneys are enlarged with multiple
cysts of variable sizes (. Fig. 4.2.2 ).
5 Hyperdense calculi may be seen within the renal
pelvis or the ureters.
5 Hepatic, pancreatic, or splenic cysts may be seen
(. Fig. 4.2.1 ). Intrahepatic cystic bile duct
dilatation (Caroli’s disease) can be associated with
PKD in up to 70 % of cases.
. Fig. 4.2.2 Axial CT urography image in a patient with
ADPKD shows bilateral mildly enlarged kidneys with multiple
c y s t s
Signs on MRI
5 Cerebral MR angiography should be performed for
ADPKD patients with positive family history of
cerebral aneurysms as a screening examination.
These aneurysms are classically saccular aneurysms
that occur at the bifurcation of cerebral vessels and
resemble a berry in size and shape ( berry aneurysm ).
Up to 80 % of berry aneurysms arise from the circle
of Willis, and 20 % arise from the posterior fossa.
5 Seminal vesicle cyst is detected as unilocular cyst
with fluid signal located at the posterolateral
aspect of the urinary bladder. The cyst may be
associated with ipsilateral ejaculatory duct
dilatation that may protrude into the urinary
bladder mimicking ectopic ureterocele.
Autosomal Recessive Polycystic Kidney
Disease
Autosomal recessive polycystic kidney disease (ARPKD) is a
rare genetic disease with prevalence of 1:20,000 live births.
ARPKD typically starts in neonates and infants as early renal
failure. Infant’s death usually occurs within the rst year of
life, unless renal transplantation is considered.
e kidneys are massively enlarged with numerous cysts.
Hepatic brosis is very common in ARPKD (60 %). Hypertension occurs in almost all cases. Pregnant women with an
infant with ARPKD typically display oligohydramnios.
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