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Anomalies of the
Upper Urinary Tract
By
Hassaan Ali Gad
Assistant lecturer of urology and Andrology
Aswan University
Dr.Hassaan Ali 2014
Embryology of kidney.
Normal Anatomy of kidney.
Congenital abnormalities of the kidney.
Complication.
Dr.Hassaan Ali 2014
kidneys develop from the intermediate
mesoderm(Kidneys, Adrenals and Gonads)
3 embryonic kidneys, in order of appearance
pronephros .
mesonephros
metanephros
first two regress in utero,
and the third becomes the permanent kidney
Kidney Development
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
Embryology of kidney
Dr.Hassaan Ali 2014
Embryology of kidney
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
The nephron (glomerulus, proximal tubule, loop of Henle, and
distal tubule) is thought to derive from the metanephric
mesenchyme
The collecting system (collecting ducts, calyces, pelvis, and
ureter) formed from the ureteric bud
Development of Nephrons/Collecting
System
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
- From 6th to 9th weeks: kidneys ascend to a lumbar site just
below adrenals
- the differential growth of the lumbar and sacral regions of the
embryo plays a role renal ascent.
as the kidneys migrate, they are vascularized by a succession of
transient aortic sprouts that arise at progressively higher levels
final pair forms in the upper lumbar region and becomes the
definitive renal arteries
Renal Ascent
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
Normal Anatomy
Dr.Hassaan Ali 2014
Developmental anomalies of the
kidney
Abnormalities of the kidney number
Abnormalities of the kidney position
Abnormalities of the kidney rotation
Collecting system abnormalities
Cystic abnormalities of the kidney
Anomalies of renal vasculature
Dr.Hassaan Ali 2014
Bilateral renal agenesis
Unilateral renal agenesis
Supernumerary kidney
abnormalities of the kidney number
Dr.Hassaan Ali 2014
1 : 4800 births
Origin ureteral bud failure or absence of the
nephrogenic ridge
Associated findings :
- absent renal arteries
- complete ureteral atresia (50%)
- bladder atresia (50%)
- low birth weight, oligohydramnion
Bilateral renal agenesis
Dr.Hassaan Ali 2014
1 : 1500, 2 : 1 male, left kidney more often
Origin ureteral bud failure; familial trend
Associated findings :
- absent ureter with hemitrigone (50%)
- adrenal agenesis (10%)
- genital anomalies
Unilateral renal agenesis
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
Origin combined defect of ureteral bud &
metanephros
Associated findings :
- hydronephrosis (50%)
- common ureter (40%)
- duplex ureter (40%)
- ectopic ureter (20%)
Supernumerary kidney
Dr.Hassaan Ali 2014
Simple ectopia
Thoracic ectopia
Crossed ectopia
ectopiaFusion
Abnormalities of the kidney position
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
Incidens 1 : 900, left side favored
Associated findings :
- Anomalous vasculature
- Contralateral agenesis
- VUR
- undescended testes, hypospadia
- urethral duplication (10-20% male)
- skeletal & cardiac anomalies (20%)
Simple ectopia
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
< 5% of ectopic kidney
Origin is delayed closure of diaphragmatic
Adrenal may or may not be thoracic
THORACIC ECTOPIA
Dr.Hassaan Ali 2014
1 : 1000 to 1 : 2000, 90% crossed with fusion
2 : 1 male, 3 : 1 left crossed
Origin abnormal migration of ureteral bud
or rotation of caudal end
CROSSED ECTOPIA & FUSION
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
1 : 400, 2:1 males
Origin fusion of lower poles before or during
rotation (4 ½ - 6 wks of gestation)
Associated findings :
- anomalous vessels
- skeletal, CV, CNS anomalies
- hypospadias & cryptorchidism, UTI, stone.
HORSESHOE KIDNEY
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
Rotation of the kidney during its ascent from the
pelvis
rotation during its ascent to the adult site.
A, Primitive embryonic
position; hilus faces ventrad (anterior)
B, Normal adult
position;
hilus faces mediad
C, Incomplete rotation.
D, Hyper-rotation;
hilus faces dorsad (posterior).
E, Hyper-rotation; hilus faces
laterad.
F, Reverse rotation; hilus faces laterad.
Dr.Hassaan Ali 2014
Rotation of the kidney during its ascent from the pelvis.
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
1. Autosomal dominant polycystic kidney disease
2. Autosomal recessive polycystic kidney disease
3. Medullary sponge kidney (tubular ectasia)
4. Medullary cystic disease .
5. Unilateral multicyctic dysplastic kidney
Cystic abnormalities of the kidney
Dr.Hassaan Ali 2014
Adult type is the most common cystic disease in
humans
1 : 1250, 10 % of all ESRD
Present at age 30 – 50 yrs, can occur in children
Pain, hematuria, progressive renal impairment
IVU irregular renal enlargement + calyceal
distortion
Assoc. findings : liver cysts, berry aneurism
Autosomal dominant polycystic
kidney disease
Dr.Hassaan Ali 2014
Infantile type, rare (1 : 10.000)
IVU streaked appearance (sunburst
pattern)
Usually die within the first 2 mo of life
Autosomal recessive polycystic
kidney disease
Dr.Hassaan Ali 2014
Adult disease
Enlarged tortuous collecting ducts
1 : 20.000
IVU bristles on a brush
Complication : infection, stones, distal renal
tubular acidosis, hematuria
1/3 pat with hypercalcemia
Medullary sponge kidney (tubular
ectasia)
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
Bilateral small kidney, amedullary cysts
Progress to ESRD by age 20
Juvenile type 20% of childhood renal failure
deaths
Polydipsia & polyuria in 80%
Retinitis pigmentosa in 16%
Medullary cystic disease
Dr.Hassaan Ali 2014
Most common cystic disease of the newborn
Second most common abdominal mass in
infant after hydronephrosis
Left kidney is more common, =
Unilateral multicyctic dysplastic
kidney
Dr.Hassaan Ali 2014
Ureteropelvic junction obstruction (UPJO)
Calyceal diverticulum
Hydrocalycosis
Megacalycosis
Infundibulopelvic stenosis
Collecting system abnormalities.
Dr.Hassaan Ali 2014
Usual cause of the most common abdominal
mass in children (hydronephrosis)
Male: Female = 2 : 1 (in child), left side
predominance
Episodic flank pain, flank mass, hematuria,
infection, nausea & vomiting, uremia.
UPJO (uretero pelvic junction
obstruction)
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
4,5 : 1000
Origin failure of degeneration of 3rd & 4th
order branches of ureteral bud
In 1/3 patients stones will be form
Th/ : removal stones, drainage of pus,
marsupialization to the renal surface
CALYCEAL DIVERTICULUM
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
Rare
Involving vascular compression, cicatrization
or achalasia of the infundibulum
Rarely requires any intervention
HYDROCALICOSIS
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
Rare, one or both kidney
Dilated unobstructed calyces, > 25 / kidney
(N : 8 – 10)
Faulty uretral bud division, hypoplasia of
juxtamedullary glomeruli & maldevelopment
of calyceal musculature
: = 6 : 1, only in Caucasian
X-linked recessice
MEGACALYCOSIS
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
May involve part or all of one or both kidney
Calyces quite large
No progressive functional deterioration
Maybe with dysplasia & lower tract anomalies
Common with vesicoureteral reflux
INFUNDIBULOPELVIC STENOSIS
Dr.Hassaan Ali 2014
Aberrant, Accessory, or Multiple Vessels
Renal Artery Aneurysm
Renal Arteriovenous Fistula
Dr.Hassaan Ali 2014
Anomalies of renal vasculature
Dr.Hassaan Ali 2014
Dr.Hassaan Ali 2014
THANK YOU

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Renal anomalis

  • 1. Anomalies of the Upper Urinary Tract By Hassaan Ali Gad Assistant lecturer of urology and Andrology Aswan University Dr.Hassaan Ali 2014
  • 2. Embryology of kidney. Normal Anatomy of kidney. Congenital abnormalities of the kidney. Complication. Dr.Hassaan Ali 2014
  • 3. kidneys develop from the intermediate mesoderm(Kidneys, Adrenals and Gonads) 3 embryonic kidneys, in order of appearance pronephros . mesonephros metanephros first two regress in utero, and the third becomes the permanent kidney Kidney Development Dr.Hassaan Ali 2014
  • 8. The nephron (glomerulus, proximal tubule, loop of Henle, and distal tubule) is thought to derive from the metanephric mesenchyme The collecting system (collecting ducts, calyces, pelvis, and ureter) formed from the ureteric bud Development of Nephrons/Collecting System Dr.Hassaan Ali 2014
  • 10. - From 6th to 9th weeks: kidneys ascend to a lumbar site just below adrenals - the differential growth of the lumbar and sacral regions of the embryo plays a role renal ascent. as the kidneys migrate, they are vascularized by a succession of transient aortic sprouts that arise at progressively higher levels final pair forms in the upper lumbar region and becomes the definitive renal arteries Renal Ascent Dr.Hassaan Ali 2014
  • 13. Developmental anomalies of the kidney Abnormalities of the kidney number Abnormalities of the kidney position Abnormalities of the kidney rotation Collecting system abnormalities Cystic abnormalities of the kidney Anomalies of renal vasculature Dr.Hassaan Ali 2014
  • 14. Bilateral renal agenesis Unilateral renal agenesis Supernumerary kidney abnormalities of the kidney number Dr.Hassaan Ali 2014
  • 15. 1 : 4800 births Origin ureteral bud failure or absence of the nephrogenic ridge Associated findings : - absent renal arteries - complete ureteral atresia (50%) - bladder atresia (50%) - low birth weight, oligohydramnion Bilateral renal agenesis Dr.Hassaan Ali 2014
  • 16. 1 : 1500, 2 : 1 male, left kidney more often Origin ureteral bud failure; familial trend Associated findings : - absent ureter with hemitrigone (50%) - adrenal agenesis (10%) - genital anomalies Unilateral renal agenesis Dr.Hassaan Ali 2014
  • 18. Origin combined defect of ureteral bud & metanephros Associated findings : - hydronephrosis (50%) - common ureter (40%) - duplex ureter (40%) - ectopic ureter (20%) Supernumerary kidney Dr.Hassaan Ali 2014
  • 19. Simple ectopia Thoracic ectopia Crossed ectopia ectopiaFusion Abnormalities of the kidney position Dr.Hassaan Ali 2014
  • 21. Incidens 1 : 900, left side favored Associated findings : - Anomalous vasculature - Contralateral agenesis - VUR - undescended testes, hypospadia - urethral duplication (10-20% male) - skeletal & cardiac anomalies (20%) Simple ectopia Dr.Hassaan Ali 2014
  • 24. < 5% of ectopic kidney Origin is delayed closure of diaphragmatic Adrenal may or may not be thoracic THORACIC ECTOPIA Dr.Hassaan Ali 2014
  • 25. 1 : 1000 to 1 : 2000, 90% crossed with fusion 2 : 1 male, 3 : 1 left crossed Origin abnormal migration of ureteral bud or rotation of caudal end CROSSED ECTOPIA & FUSION Dr.Hassaan Ali 2014
  • 30. 1 : 400, 2:1 males Origin fusion of lower poles before or during rotation (4 ½ - 6 wks of gestation) Associated findings : - anomalous vessels - skeletal, CV, CNS anomalies - hypospadias & cryptorchidism, UTI, stone. HORSESHOE KIDNEY Dr.Hassaan Ali 2014
  • 33. Rotation of the kidney during its ascent from the pelvis rotation during its ascent to the adult site. A, Primitive embryonic position; hilus faces ventrad (anterior) B, Normal adult position; hilus faces mediad C, Incomplete rotation. D, Hyper-rotation; hilus faces dorsad (posterior). E, Hyper-rotation; hilus faces laterad. F, Reverse rotation; hilus faces laterad. Dr.Hassaan Ali 2014
  • 34. Rotation of the kidney during its ascent from the pelvis. Dr.Hassaan Ali 2014
  • 37. 1. Autosomal dominant polycystic kidney disease 2. Autosomal recessive polycystic kidney disease 3. Medullary sponge kidney (tubular ectasia) 4. Medullary cystic disease . 5. Unilateral multicyctic dysplastic kidney Cystic abnormalities of the kidney Dr.Hassaan Ali 2014
  • 38. Adult type is the most common cystic disease in humans 1 : 1250, 10 % of all ESRD Present at age 30 – 50 yrs, can occur in children Pain, hematuria, progressive renal impairment IVU irregular renal enlargement + calyceal distortion Assoc. findings : liver cysts, berry aneurism Autosomal dominant polycystic kidney disease Dr.Hassaan Ali 2014
  • 39. Infantile type, rare (1 : 10.000) IVU streaked appearance (sunburst pattern) Usually die within the first 2 mo of life Autosomal recessive polycystic kidney disease Dr.Hassaan Ali 2014
  • 40. Adult disease Enlarged tortuous collecting ducts 1 : 20.000 IVU bristles on a brush Complication : infection, stones, distal renal tubular acidosis, hematuria 1/3 pat with hypercalcemia Medullary sponge kidney (tubular ectasia) Dr.Hassaan Ali 2014
  • 42. Bilateral small kidney, amedullary cysts Progress to ESRD by age 20 Juvenile type 20% of childhood renal failure deaths Polydipsia & polyuria in 80% Retinitis pigmentosa in 16% Medullary cystic disease Dr.Hassaan Ali 2014
  • 43. Most common cystic disease of the newborn Second most common abdominal mass in infant after hydronephrosis Left kidney is more common, = Unilateral multicyctic dysplastic kidney Dr.Hassaan Ali 2014
  • 44. Ureteropelvic junction obstruction (UPJO) Calyceal diverticulum Hydrocalycosis Megacalycosis Infundibulopelvic stenosis Collecting system abnormalities. Dr.Hassaan Ali 2014
  • 45. Usual cause of the most common abdominal mass in children (hydronephrosis) Male: Female = 2 : 1 (in child), left side predominance Episodic flank pain, flank mass, hematuria, infection, nausea & vomiting, uremia. UPJO (uretero pelvic junction obstruction) Dr.Hassaan Ali 2014
  • 48. 4,5 : 1000 Origin failure of degeneration of 3rd & 4th order branches of ureteral bud In 1/3 patients stones will be form Th/ : removal stones, drainage of pus, marsupialization to the renal surface CALYCEAL DIVERTICULUM Dr.Hassaan Ali 2014
  • 50. Rare Involving vascular compression, cicatrization or achalasia of the infundibulum Rarely requires any intervention HYDROCALICOSIS Dr.Hassaan Ali 2014
  • 52. Rare, one or both kidney Dilated unobstructed calyces, > 25 / kidney (N : 8 – 10) Faulty uretral bud division, hypoplasia of juxtamedullary glomeruli & maldevelopment of calyceal musculature : = 6 : 1, only in Caucasian X-linked recessice MEGACALYCOSIS Dr.Hassaan Ali 2014
  • 54. May involve part or all of one or both kidney Calyces quite large No progressive functional deterioration Maybe with dysplasia & lower tract anomalies Common with vesicoureteral reflux INFUNDIBULOPELVIC STENOSIS Dr.Hassaan Ali 2014
  • 55. Aberrant, Accessory, or Multiple Vessels Renal Artery Aneurysm Renal Arteriovenous Fistula Dr.Hassaan Ali 2014 Anomalies of renal vasculature