13. Normal rotation – 90 degree medial rotation
of PUJ
Non rotation -Hallmark “ Some of the
calyces will be located medial to the PUJ “
Malrotation- Reverse or over-rotation
14. Renal Ectopia
Normal renal ascent – caudal to cranial
1 in 1000 M:F = 1.5:1
Location – Pelvis, iliac fossa
Anomalous blood supply
32. Abnormalities of Renal Vasculature
Failure of involution of arteries during ascent
25 % adult population
Common with pelvic / horseshoe kidney
Important for preoperative evaluation
34. Persistent Fetal
lobations
5 % of adult patients
Can be mistaken for other parenchymal
disorders
Diferentiating features
Parenchymal thickness >14 mm
Indentation must be smooth and regular
Centering of calyces
41. POLYCYSTIC KIDNEY DISEASE
AUTOSOMAL RECESSIVE
Commonly presents at birth (perinatal, neonatal, infantile, juvenile)
Renal parenchyma is replaced by numerous tiny cysts (1-8mm).
Oligohydramnios and potter’s syndrome
42. perinatal type: most common
oligohydramnios and pulmonary hypoplasia
75% have death within 24 hours of delivery
minimal hepatic fibrosis
neonatal type:
infantile type:
juvenile type: portal hypertension with splenomegaly and portosystemic varices
43. USG
Antenatal Usg : Oligohydramnios
1. Enlarged echogenic kidney with loss
of CMD.
2. Renal outline is maintained
45. IVP – Striated nephrogram
Contrast in preserved tubules next to
unopacified tubules
46. Esophageal varices with the juvenile form
of autosomal recessive polycystic kidney
disease.
47. AUTOSOMAL DOMINANT PKD
20 – 40 yrs of age.
Asymptomatic in early stage
Numerous cysts of varying sizes
48. AUTOSOMAL DOMINANT PKD
A: Early disease: numerous small intrarenal
cysts.
B: Advancing disease: Enlarged kidneys
and multiple cysts of varying sizes
throughout cortex and medulla.
49. IVP APPEARANCE-
SWISS CHEESE NEPHROGRAM
multiple well defined non perfused areas
(Cysts)
Streched out calyces
50. AUTOSOMAL DOMINANT PKD
Nonenhanced CT scan (A) shows bilateral nephromegaly.
Contrast-enhanced CT scan (B) reveals large renal cysts with enhancing residual
parenchyma between the cysts.
51. AUTOSOMAL DOMINANT PKD
Significant hepatomegaly caused by liver cysts in a 47-year-old woman with
autosomal dominant polycystic kidney disease.
52. Associations
• Cysts in liver (50%),pancreas, brain, spleen ,ovaries and testes.
• Berry aneurysm 15%
• Aneurysms of coronary arteries and aorta.
• Valvular heart disease
• Colonic diverticuli
53. MEDULLARY SPONGE KIDNEY
Due to ectasia (fusiform or cystic) of collecting duct with in renal
parenchyma.
Usually B/L may be unilateral or segmental.
Benign , asymptomatic.
Weak association with
Wilm’s disease
Pheochromocytoma
Horseshoe kidney
Hemihypertrophy
54. MEDULLARY SPONGE KIDNEY
Medullary sponge kidney. Intravenous urogram shows a
paintbrush like appearance produced by cystic dilatation of
medullary collecting ducts (arrows)
IVU
55. MEDULLARY SPONGE KIDNEY
USG
Medullary part of collecting duct -
dysplastic and dilated
80% -medullary nephrocalcinosis
Greatly increased renal medullary echogenicity
56. NCCT -clusters of
calcification around the
corticomedullary junction of
both kidneys.
62. Duplex Collecting System
- The degree of duplication is variable
- duplication is complete when there are two separate collecting systems and two
separate ureters , each with their own ureteral orifice ,
- duplication is incomplete when the ureters join and enter the bladder through a
single ureteral orifice
Three sets of excretory structures which appear in sequence to form the nephrogenic cord
Undifferentiated primitive cells//The ureteric bud ultimately gives rise to the ureter, renal pelvis, calyces, and collecting tubules of the renal medulla.//The metanephric blastema develops from the caudal portion of the nephrogenic cord and gives rise to the excretory part of the kidney.
Renal agenesis results from failure of the ureteric bud to reach the metanephric blastema because the ureteric bud fails to form or degenerates prematurely. induction of differentiation in the metanephric blastema does not occur…non contrast computed tomography image obtained for left flank pain reveals a stone in the left kidney, and the kidney is enlarged (compensatory hypertrophy).
IVU showing left renal agenesis with bowel gas within the left renal fossa. Absent renal shadow ,The large right kidney shows a bifid renal pelvis, the mildest form of renal duplication.
Axial image through the pelvis shows absence of the right seminal vesicle//absence of the ipsilateral epididymis, vas deferens, or seminal vesicle, or presence of an associated ipsilateral seminal vesicle cyst//absence or atresia of the uterus or vagina, a unicornuate
uterus with absence or atresia of the vagina// Elongated disc shaped adrenal
A, Chest radiograph in a newborn with renal agenesis demonstrates pulmonary hypoplasia with a small thorax, a right pneumothorax, and diffuse opacification of the left lung. B, An aortogram in this infant demonstrates absence of the renal arteries due to bilateral renal agenesis. C, Sonogram of the renal fossa demonstrates absence of the kidney. The adrenal gland is in its normal position, but
prominent and disk shaped (arrows).
A, Axial image through the upper abdomen shows a kidney in each renal fossa. B, Axial image of the lower abdomen shows an extra kidney in the right side of the upper pelvis (arrow).
Computed tomography image of the kidneys shows an anteriorly directed right renal hilum (nonrotation) and laterally directed left renal hilum (malrotation).
It move upto its normal position in renal fossa at 4-8 weeks of intrauterine life.///On IVP , the left kidney was found in the pelvic fossa with normal function
Coronal excretory urography, volume rendering….Left kidney is ectopic, lying in the pelvis, with a short ureter. //usually unilateral and involving left kidney./The differential for a pelvis sited kidney is post transplantation. //
Due to their pelvic location rather than being retroperitoneal, they are very prone to trauma , …. Stasis of urine in the structurally altered renal pelvis
(A) Coronal computed tomography (CT) scan shows the left kidney protruding above the diaphragm with a diaphragmatic hernia. (B) Axial CT scan shows a posterior diaphragmatic hernia with herniation of the left kidney into the thorax (arrow).
Most common type of renal fusion anomaly 90% are fused at lower pole// Contact of metanephric tissue in utero//B/l Malrotation // arrest of ascent at inferior mesenteric artery/
Kidneys are low lying//bilateral pelvicalyceal systems seen more end-on pcs show fullness //both lower poles are directed infero medially towards the spine and upper poles superolateraly //normally functioning bilateral kidneys//
Length is underestimated and it may be misdiagnosed as isthmus is not visualized
Horseshoe kidney with anteriorly oriented renal pelvis, axial contrast-enhanced MDCT image (a) shows a horseshoe, the renal isthmus is found anterior to the great retroperitoneal vessels, corresponding volume rendering (b) better show the inferior renal isthmus, the normal renal arteries, and the anterior location of the renal pelvises
coronal MR UrographyThis image demonstrates the low position ofthis horseshoe kidney, the abnormal renal axis due to medial fusion of the lower-pole renal parenchyma (arrows), and rotational anomalies in the collecting systems as indicated by calyces projecting medial to the main volume of the renal pelvis
Ureters cross midline and open at their normal location
Ureters open at normal location.
Both kidneys are located on the right side of the midline and show fusion at their poles with renal sinuses directed away from each other. The left renal fossa is empty. Features suggest a sigmoid type of Crossed fused renal ectopia.
enhanced computed tomography image through the pelvis, the bilateral pelvic kidneys are
fused in the pelvis forming a lump, discoid, or pancake kidney
(a) Intravenous pyelography showing crossed fused renal ectopia. (b) Magnetic resonance imaging scan showing crossed fused renal ectopia with hydronephrosis. (c) Ultrasonography showing both renal moieties fused with each other
renal computed tomography angiogram reveals a dominant left renal artery with smaller left lower-pole accessory artery (arrow), and three, approximately equal sized, right renal arteries (arrowheads).//or endotherapy on the infrarenal abdominal aorta, preoperative
recognition of accessory renal arteries can help to avoid damage to or ligation of these accessory branches
Lobar anatomy is evident at birth in all individuals, but with cellular multiplication, lobar anatomy is usually obscured by the age of 4
to 5.//Persistent fetal lobation is of no clinical significance,//Reflux nephropathy and renal infarcts and pailary necrosis//calyces are centered between indentations.
Failure of fusion of ureteric bud with metanephric blastemal// Multiple irregular non communicating cysts with echogenic tissue separating it.// Shadowing areas either represents dense fibrous tissue or regions of calcification.
enhanced (B) axial images through the renal beds reveal a calcified multicystic mass in the left renal fossa
Can be of two types ar and ad /Characterized by spectrum of dilated renal collecting tubules, hepatic cysts and periportal fibrosis//overall outline of kidney is maintained
Features of hepatic and periportal fibrosis keep getting more severe
Classically, oligohydramnios is detected.
Hepatic fibrosis,//A more caudal scan shows enlarged kidneys and multiple medullary cysts// (a) is present, and there is an enlarged left gastric vein (arrow) .
Pyelonephhritis /tubular necrosis/uretric obstruction/ renal vein thrombosis// Liner luscencies from medulla to cortex.
Another element unique to ARPKD is the coexistence of hepatic disease. In some patients with ARPKD, periportal hepatic fibrosis develops leading, in severe cases, to hepatic failure, portal hypertension, splenomegaly, and bleeding varices.//Esophageal varices with the juvenile form of autosomal recessive polycystic kidney disease. This film from an upper gastrointestinal series demonstrates serpiginous filling defects in the esophagus
Milder form present late.
often becoming extremely large, develop within the kidney gradually replacing renal parenchyma and eventually it presents with renal failure//HTN, Renal insufficiency //Incidental finding , abdominal mass
Distort the renal parenchyma//Cysts prone to hemorrhage and CAlcification.
surrounded by normal renal parenchyma
//Some cysts ma show calcification//
Autosomal dominant polycystic kidney disease in a 39-year-old man with hypertension and mildly impaired renal function.
The major complication associated with MSK is an increased incidence of nephrolithiasis. urinary stasis within the cystically dilated distal tubules.
Sporadic disease// patient is asymptomatic//multiple echogenic shadowing foci are seen localized to medullary pyramids.
Non-contrast CT demonstrating clusters of calcification around the corticomedullary junction of both kidneys.
Deposition of cqalcium salt within renal parenchyma
Rare benighn neoplasm//with a thick pseudocapsule//Mural or septal calcifications are occasionally seen but internal hemorrhage is rare.//variable septal enhancement //dd RCC and cystic wilms tumor
persistent metanephric blastemal//marked renal enlargement//Ultrasound may demonstrate hypoechoic nodules //low-attenuation peripheral nodules with poor enhancement
-Duplex collecting system is the most common congenital anomaly of the urinary tract
Renal agenesis with uterine anomaly. A, An enhanced computed tomography image through the upper abdomen reveals right renal agenesis and left renal compensatory hypertrophy. B, An image through the anatomic pelvis shows two apparent uterine horns in this patient with a bicornuate uterus.
