Anomalies of Rotation

• MALROTATION: happens when alignment of kidney, as it assumes its

final position in the “renal” fossa  NOT EXACT
• NORMAL: orients itself so that the calyces point laterally and the
pelvis faces medially.
• association with Turner syndrome
• M>F (2:1)
• Embryology.
6th week: medial rotation of the collecting system occurs simultaneously with
renal migration  completes this process by rotating 90 degrees toward the
midline by the time ascent is complete at the end of 9 weeks’ gestation.
postulated that rotation is actually the result of unequal branching of
successive orders of the budding ureteral tree
Anomalies of Rotation
• kidney and renal pelvis
normally rotate 90 degrees
ventromedially during ascent
• In cases of isolated malrotation,
other characteristic features
may be present (distortion,
fibrous matting)
• Abnormal phases of medial and reverse rotation according to
the position of the renal pelvis (Weyrauch (1939):

a) Ventral Position
b) Ventromedial Position
c) Dorsal Position
d) Lateral Position
• Symptoms
→ No specific symptoms
→ Excess fibrous tissue encasing the pelvis, UPJ, and upper
ureterhydronephrosis
→ Vascular compression from an accessory or main renal artery or
distortion of the upper ureter or UPJ  intermittent obstruction.
• Diagnosis
 diagnosis should be considered in renal calculus in an abnormal
location
 confirmation from a renal sonogram, CT, MRU, or retrograde
pyelogram
Anomalies of Renal Vasculature
1. Aberrant, Accessory, or Multiple Vessels
2. Renal Artery Aneurysm
3. Renal Arteriovenous Fistula
Aberrant, Accessory, or Multiple Vessels
• Aberrant or anomalous vessels are arteries that originate from vessels
other than the aorta or main renal artery
• Accessory vessels denotes two or more arterial branches supplying the
same renal segment
• Multiple renal arteries is any kidney supplied by more than one vessel
• Five segment of renal parenchyma based on vascular supply: apical, upper,
middle, lower, and posterior.
• Main renal artery divides into anterior and posterior branch.
• The anterior branch almost always supplies the upper, middle, and lower
segments of the kidney. The posterior branch invariably supplies the
posterior and lower segments
Aberrant, Accessory, or Multiple Vessels
• Symptoms might result from inadequate urinary drainage
→Constriction in infundibulum, a major calyx, or the UPJ  Pain
and hematuria secondary to hydronephrosis, UTI, or calculus
• Diagnosis. Precise anatomic resolution of vascular variants and
associated disease states through 3D power Doppler
ultrasonography, CT, or MRI
• Prognosis. Hydronephrosis secondary to a vascular anomaly such as
a lower pole crossing vessel is a very rare finding, especially when
one considers the relative frequency of all renal vascular variations.
Hypertension is no more frequent in patients with multiple renal
arteries than in those with a single vessel
Renal Artery Aneurysm
• Classification as follows: saccular, fusiform, dissecting, and arteriovenous
a) The saccular aneurysm - localized outpouching that communicates with the
arterial lumen by a narrow or wide opening; is the most common type (93%)
b) Fusiform type is When the aneurysm is located at the bifurcation of the main renal
artery and its anterior and posterior divisions, or at one of the more distal
branchings, it is considered to be congenital in origin The presence of similar
aneurysms at branching points in the vasculature of other organ systems attests to
this possible origin
c) Acquired aneurysms may be located anywhere and may result from inflammatory,
traumatic, or degenerative factors. A localized defect in the internal elastic tissue
and the media allows the vessel to dilate at that point. It is a true aneurysm,
because its walls are composed of most of the layers that make up the normal
artery. The outpouchings may vary in size from 1 to 2 cm up to 10 cm, but 90% are
smaller than 2 cm. There is no absolute predilection for side, but the right appears
to be favored, and bilateral aneurysms are seen in 15%
RAA
• Symptoms - silent in children, some manifest at a later age, because
the size of the aneurysm increases with time.
Pain (15%), hematuria (microscopic and macroscopic) (30%), and
hypertension (55%) secondary to compression of adjacent
parenchyma or to altered blood flow within the vascular tree
• Diagnosis is suspected when a pulsatile mass is palpated in the region of the
renal hilum or when a bruit is heard on abdominal auscultation. A wreathlike
calcification in the area of the renal artery or its branches (30%) is highly
suggestive
• RAAs can be evaluated with color Doppler sonography, spiral CT, 3D MRA, or
digital subtraction arteriography
RAA
• High risk for rupture - associated with rapidly expanding RAAs and
pregnant females or those who are considering pregnancy.
• Intervention is indicated for RAAs larger than 2.0 cm or symptomatic
individuals (uncontrolled hypertension from renal artery stenosis,
flank pain, hematuria, or renal ischemia/infarction resulting from
embolization from the aneurysm).
• The likelihood of spontaneous rupture (about 10%), with its dire
consequences, dictates emergency treatment. Open surgical or
endovascular techniques should be performed electively for those at
high risk for rupture
Renal Arteriovenous Fistula
• Two types:
a) Congenital - Fewer than 25%; present at birth or to result from a congenital
aneurysm eroding into an adjacent vein
b) Acquired - secondary to trauma, inflammation, renal surgery, or percutaneous
needle biopsy) accounting for the increase in incidence.
• identifiable by their cirsoid configuration and multiple communications
between the main or segmental renal arteries and the venous channels
• Although congenital, rarely present clinically before 3rd or 4th decade.
• F>M (3:1)
• right > left
• lesion is usually located in the upper pole (45% of cases), but not
infrequently it may be found in the midportion (30%) or in the lower pole
(25%)
RAF
• Symptoms - based on the age and size of the AVF
• Diagnosis - Three-dimensional Doppler sonography and MRA are accurate and
noninvasive tests but selective renal arteriography or digital subtraction
angiography is the most definitive method for diagnosing the lesion.
• Cirsoid appearance with multiple small, tortuous channels; prompt venous filling;
and an enlarged renal, and possibly gonadal, vein are pathognomonic for
arteriovenous malformation (AVM)
• The symptomatic nature which causes progressive alterations in the
cardiovascular system, often dictates surgical intervention.
• congenital variant rarely behaves like its acquired counterpart, which may
disappear spontaneously after several months.
• Nephrectomy, partial nephrectomy, vascular ligation, selective embolization, and
balloon catheter occlusion have been used to obliterate the fistula.
Anomalies of the Collecting System
1. Calyceal Diverticulum
2. Hydrocalycosis
3. Megacalycosis
4. Pseudotumors of the Kidney
5. Infundibulopelvic Stenosis
6. Bifid Pelvis
Calyceal Diverticulum
• cystic cavity within the kidney lined by transitional epithelium that
communicates with a calyx or less commonly with the renal pelvis
through a narrow isthmus
• may be multiple, with the upper calyx most frequently affected.
• Type I diverticula - Most diverticula occur adjacent to an upper or,
occasionally, a lower pole calyx.
• Type II diverticula are larger, communicate with the renal pelvis, and
tend to be symptomatic
• Congenital and acquired factors have been suggested to explain the
formation of calyceal diverticula.
Calyceal Diverticulum
• tend to distend progressively with trapped urine
• Infection, milk of calcium or true stone formation are complications of
stasis or obstruction  Symptoms (Hematuria, pain, and UTI)
• Diagnosis is suggested on ultrasonography but is confirmed on CT scan or
MRU
• Asymptomatic patients do not require treatment but should be followed
periodically with ultrasonography
• Indications for surgery (percutaneous ablation, marsupialization of the
diverticulum and fulguration of the epithelial lining)
included enlargement of the diverticulum associated with pain or infection, abscess
formation, urosepsis, and symptomatic calculus formation