Anomalies of theUpper 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 appearancepronephros .

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 adultposition;

hilus faces mediadC, 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 polycystickidney 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 polycystickidney 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 (tubularectasia)

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 dysplastickidney

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 junctionobstruction)

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