7/10/2015Congenital anomalies of the upper urinary tract
Congenital anomalies of the upper urinary tract:Anomalies of number
Anomalies of volume and structureHypoplasia
Polycystic kidney -Infantile
-Adult -Other cystic disease
-Medullary cystic disease
Anomalies of ascentSimple ectopia
Anomalies of form and fusionCrossed ectopia with and without fusion
Unilateral fused kidney (inferior ectopia)
Sigmoid or S-shaped kidney
Unilateral fused kidney (superior ectopia)
Anomalies of rotationIncomplete
Anomalies of the collecting systemCalyx and infundibulum
Anomalous calyx (pseudotumor of the kidney(
Anomalies of renal vasculatureAberrant, accessory, or multiple vessels
Renal artery aneurysm
In summerycomprise a diversity of abnormalities, ranging from: complete absence kidney, supernumerary Kidney aberrant location, orientation, and shape of the kidney ,aberrations of the collecting system, & blood supply.
Unilateral Renal Agenesis (URA)Incidence : 1: 1400 births
Found accidentally, more frequently on the left side.
Embryology :Complete absence of a ureteric bud or aborted ureteral development prevents maturation of the metanephric blastema into adult kidney tissue.
*Ipsilateral adrenal agenesis is rarely encountered with URA
*Other Genital anomalies are much more frequently observed
Diagnosis : U/S or IVU,CT scan: absent kidney on that side + compensatory hypertrophy of the contralateral kidney
Treatment : no specific treatment
Prognosis : no evidence that they have an increased susceptibility to other diseases
Bilateral agenesis: rare, incompatible with lifeSupernumerary Kidney truly an accessory organ
Incidence : very rare
Symptoms : It may not produce symptoms until early adulthood, if at all.
Diagnosis : accidentally by IVU or abdominal U/S
Treatment : no treatment
ANOMALIES OF ASCENT1. Simple Renal Ectopia
When the mature kidney fails to reach its normal location in the " renal fossa "
Incidence : The incidence is 1 in 1000
Associated Anomalies : The incidence of contralateral agenesis appears to be rather highHydronephrosis secondary to obstruction or reflux may be seen in as many as 25% of none contralateral kidneys
Clinical features : Most ectopic kidneys are asymptomatic
Diagnosis : U/S, IVU, CT scan
Prognosis : The ectopic kidney is no more susceptible to disease than the normally positioned kidney except for the development of hydronephrosis or urinary calculus formation
2.Cephalad Renal Ectopia3.Thoracic Kidney
ANOMALIES OF FORM AND FUSIONCrossed Renal Ectopia With and Without Fusion
Horseshoe Kidneyfound in 1:1000 necropsies an is commoner in men.
probably the most common of all renal fusion anomalies
The anomaly consists of two distinct renal masses lying
vertically on either side of the midline and connected at
their respective lower poles by a parenchymatous or fibrous
isthmus that crosses the midplane of the body.
Fusion of the renal masses early in embryonic life, so its ascent
will be impeded by inferior mesenteric artery.
The kidneys are low located, mal rotated and pelves lie anteriorly
Symptom When present, they are related to complications like hydronephrosis, infection, or calculus formation
Diagnosis ultrasound, IVU, CT scan
Treatment:-Medical: pain relief and to control infection
-Surgical: stone removal, PUJ stenosis correction and isthmus division in cases of
-operations on the aorta
Prognosis usually they have normal life.Cystic disease of the kidneys
Polycystic kidney disease :
The kidney is one of the most common sites in the body for cysts
AUTOSOMAL RECESSIVE ("INFANTILE") POLYCYSTIC KIDNEY DISEASE
AUTOSOMAL DOMINANT ("ADULT") POLYCYSTIC KIDNEY DISEASE
Congenital cystic kidney (polycystic kidney) (Adult cystic renal disease)Autosomal dominant, transmitted by either parents, 50% of offspring affected.
Both kidneys replaced by large no. of cysts of variable size which make the kidney of large size.
The cysts contain clear fluid but sometimes blood.
The cysts progressively increase in size causing pressure atrophy of the renal parenchyma and pressing the ureter.
15% associated with cystic disease of liver, lung, pancreas or spleen.
Etiology & PathogenesisThe cysts occur because of defects in the development of the collecting and uriniferous tubules and in the mechanism of their joining. Blind secretory tubules that are connected to functioning glomeruli become cystic.
Clinical pictures:Rarely gives clinical manifestation before 4o years
Asymptomatic: diagnosed accidentally.Pain: due to pedicle stretching, stone, ureteric obstruction, bleeding inside cyst or infection.
Hematuria: cyst distention and rupture to the collecting system.
Infection: renal or cyst infection causes fever, rigor and loin pain.
Hypertension: in 70%, Unknown cause.
Renal impairment: anorexia, headache, nausea, vomiting, drowsiness and coma.
Renal enlargement: large knobby palpable kidney
Diagnosis: Family history of polycystic disease.
U/S, IVU, CT scan, MRI
To control infection, hypertension, pain and anemia.
Renal impairment: by low protein diet and dialysis.
Rovsing’s operation (deroofing) for large cysts causing symptoms or obstruction.
Renal failure: Renal transplantation.
Infantile polycystic disease of the kidneyRare autosomal recessive, incompatible with life.
Both kidneys are large in size and replaced by large number of cysts which may obstruct labor.
The condition is due to failure of ureteric bud to fuse with metanephrose.
Simple (solitary) renal cystCommon condition.
single or multiple.
uni or bilateral.
Congenital or acquired.
In 10% symptomatic: pain, heaviness, infection, bleeding inside the cyst or
pressure effect on the ureter causing hydronephrosis.
DiagnosisExamination: usually –ve, big cyst cause painless loin mass, & painful if complicated by bleeding or infection
U/S: echo free area (cystic lesion)
KUB: soft tissue shadow.
IVU: stretched calyx, filling defect or hydronephrosis.
CT scan &MRI: are diagnostic.
Treatment: usually no treatment neededSymptomatic cases:
Aspiration and injection of sclerosing agent.
Rovsing’s operation (deroofing)
Partial or total nephrectomy in destructed kidney.
N.B. Malignant cyst: radical nephrectomy.
N.B. Hydatid cyst aspiration is contraindicated because of anaphylaxis and dissemination.
Congenital Anomalies of Renal pelvis & Ureter
Duplication of Renal Pelvis
Incidence: 4 %
More common on left side
Renorenal reflux may occur from one pelvis to the other
Duplication of the ureterIncidence : 3 %
Usually the ureters fuse & have common orifice in the bladder although they
may open independently in which case the ureters cross each other so that the
ureter that drain the upper pelvis open below (more distally) in the bladder &
Clinical features : usually asymptomatic
More prone to infections, calculus disease & hydronephrosis
Ureteral duplication: partial and complete
Is more common. Two ureters draining single
kidney for variable length, then unite together
before entering the bladder in one ureteric
Rarely the lower part is duplicated as inverted Y
-Complete duplication:Less frequent,
The whole ureter is duplicated, and each one
opens in separate orifice in the bladder.
The ureter draining the upper part opens more
distally in the bladder.
Ectopic Ureters80% are associated with a duplicated collecting system
In the male, the posterior urethra is the most common site of termination, also to semenal vesicle
In the female, the urethra and vestibule are the most common sites
Clinical features: According to the site of orifice
In females: continuous dribbling
In males: urinary tract infection
Diagnosis: IVU, U/S, CT scan, cystoscopyTreatment: Ureteric reimplantation or implantation of one ureter to the other ureter is
Ectopic ureters may drain renal moieties (either an upper pole or a single-system kidney) that have minimal function. Therefore, upper pole partial nephrectomy (or nephrectomy of single system) is sometimes recommended
Complete ureteral duplication and ectopic ureteric orifice
Grossly dilated ureter
Unilateral or bilateral
More common in male
Asymptomatic, pain, repeated UTIs
Lower ureter might be obstructed
Sometimes associated with
TreatmentInfection should be controlled
Excision of the lower stenotic segment
Ureteric tapering & reimplantation in to the
Nephroureterectomy for non functioning
Postcaval (Retrocaval) ureter (Preureteral Vena Cava )
The right ureter pass behind the inferior vena cava
This might causes obstruction
Vascular abnormalityIncidence: about 1 in 1500
Although it is congenital, most patients present at 3rd or 4th decade.
surgical correction involves ureteral division, with relocation and ureteroureteral or ureteropelvic reanastomosis,
usually with excision or bypass of the retrocaval segment, which can
Is due to congenital atresia of the ureteric orifice which causes a cystic dilatation of the intramural portion of the ureter
Women > men
Sometimes involves with ectopic ureter
More prone to stone disease & UTIs
Clinical Features : Asymptomatic
Repeated UTIs, Hematuria
IVU, cystoscopy, cystogram
The ‘adder head’ on excretory urography is typical.
Asymptomatic : no treatment
Cystoscopy with diathermy cauterization of the hole
Nephrectomy in non functioning kidney
In complicated cases, ureteral reimplantation and vesical reconstruction
Cobra (Adder) head appearance of ureterocele
Ureterocele involving single system Ureterocele involving duplicated ureter
Ureteropelvic Junction (UPJ)(PUJ) Obstruction (stenosis)
The most common cause of significant dilation of the collecting system in the fetal kidney
Boys > Girls
Left-sided lesions predominate
ETIOLOGYIntraluminal : mucosal fold that causes valve
Intrinsic (intramural) interruption in the
development of the circular musculature of the UPJ
Extrinsic An aberrant, accessory, or early-branching
lower-pole renal artery
PUJ Obstruction – gross pathology
SYMPTOMS/PRESENTATIONMost infants are asymptomatic and most children are discovered because of their symptoms
Episodic flank or upper abdominal pain, sometimes associated with nausea and vomiting
IVU: diagnostic , hydronephrosis with
fixed stenotic segment or complete
CT scan: hydronephrosis that ends
Magnetic Resonance Imaging
Radionuclide Renography: to see the
split function of each kidney
Pressure-Flow Studies: Whitaker test
Treatment:Medical: control infection and pain.
Indications for surgery:
2- UTI, and symptomatic patients.
3- Severe hydronephrotic non functioning kidney.
SURGICAL REPAIR including open surgical techniques, laparoscopic, & endoscopic approaches
Open & laparoscopic surgical techniques Anderson-Hynes dismembered pyeloplasty:
excision of the pathologic UPJ & appropriate
reanastamosis or flap technique or flap
for non functioning kidney