?Anomalies of the Upper Urinary Tract
?Congenital cyst
?Ectopic ureter
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?UreterocoeleAnomalies of the Upper Urinary Tract
? ANOMALY OF NUMBER
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? ANOMALIES OF ROTATION? Bilateral Renal Agenesis
? ANOMALIES OF RENAL
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? Unilateral Renal Agenesis
VASCULATURE
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? Supernumerary Kidney? Aberrant, Accessory, or
? ANOMALIES OF ASCENT
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Multiple Vessels
? Simple Renal Ectopia
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? Renal Artery Aneurysm? Cephalad Renal Ectopia
? Renal Arteriovenous Fistula
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? Thoracic Kidney
? ANOMALIES OF THE COLLECTING
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? ANOMALIES OF FORM ANDSYSTEM
FUSION
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? Calyceal Diverticulum
? Hydrocalycosis
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? Crossed Renal Ectopia with? Megacalycosis
and without Fusion
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? Infundibulopelvic Stenosis
? Horseshoe Kidney
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? Bifid Pelvis? ANOMALY OF NUMBER
? Bilateral Renal Agenesis
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? Unilateral Renal Agenesis? Supernumerary Kidney
Bilateral Renal Agenesis
? Very rare, 500 cases reported in the literature
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? no single etiology? esophageal atresia, cryptophthalmos or
Fraser syndrome, Klinefelter syndrome and
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Kallmann syndrome? 40% -stil born.
? born alive do not survive beyond 48 hours because of
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respiratory distress associated with pulmonary
hypoplasia.
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? Potter facies ,oligohydramnios-pathognomonic.? The ureters -absent,
? Bladder- absent or hypoplastic.
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? The adrenal glands are usual y positioned normal y.
? MD anomalies are commonly observed.
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? prominentfoldandskincreasebeneatheacheye,
bluntednose,
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depressionbetweenlowerlipandchinUnilateral Renal Agenesis
? 1 in 1100 births
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? Kallmann syndrome, Turner syndrome, Polandsyndrome, Fraser syndrome, branchio-oto-
renal (BOR) syndrome, DiGeorge syndrome
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? Anomaly of other organ
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Supernumerary Kidney? distinct encapsulated parenchymal mass with
its own col ecting system, blood supply
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? Very rare
? Asymptomatic/symptomatic
? T/t- if symptom
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ANOMALIES OF ASCENT
? Simple Renal Ectopia
? Cephalad Renal Ectopia
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? Thoracic Kidneyek ("out") and topos ("place")
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MC? Mostly asymptomatic
? renal pelvis is usual y anterior
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(instead of medial)
? Hydronephrotic
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? PUJO? VUJO
? Reflux
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? Malrotation
? Ureter- not redundent
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? Vascular supply-? Not from abdominal aorta
? Associated anomaly:
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? Female? Contralateral renal agenesis
? bicornuate or unicornuate uterus
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? rudimentary or absent uterus and
proximal and/or distal vagina
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? duplication of the vagina.? Male
? undescended testes, duplication of the urethra, and
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hypospadias, cloacal malformation
? Dx- Incidental
? USG/CT/MRI/Nuclear scan
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? Clinical implication-? Calculus(Obstruction-malrotation, High insertion,
crossing vessel)
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? RVH
? No difficult labour
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Cephalad Renal Ectopia (ascent
anomaly)
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? In omphalocoeleThoracic Kidney
? Exceedingly Rare
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? Asymptomatic? incidental
? ANOMALIES OF FORM AND FUSION
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? Crossed Renal Ectopia with and without Fusion
? Horseshoe Kidney
Crossed Renal Ectopia
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with and without Fusion
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Horseshoe kidney? most common of al renal fusion anomalies
? 1 in 400 persons
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? skeletal, cardiovascular, and central nervous
system anomaly
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? trisomy 18, Turner syndrome, Townes-Brocksyndrome
? 50% asymptomatic
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? hydronephrosis, infection, orcalculus, UPJ obstruction
? high insertion of ureter, angulation of ureter
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over isthmus, aberrant blood vessel
? Wilms tumor
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ANOMALIES OF ROTATION
The kidney and renal pelvis normal y rotate 90 degrees ventromedial y
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during ascent so that the calyces point lateral y and the pelvis faces
medial y.
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When this alignmentis not exact, the
condition is known
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as malrotation
It is frequently associated with Turner syndrome
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ANOMALIES OF RENAL VASCULATURE
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? Aberrant- not arisingfrom aorta
(iliac/gonadal/mesenteric
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)
? Accessory-more than one
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artery supplying samesegment
? Multiple Vessels-more
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than one artery supplying
different segment
? Nephrectomy
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? TransplantationRenal Artery Aneurysm
? Incidence-0.1% - 0.3%.
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Most -silent,
? diagnosed during an evaluation of hypertension.
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? pulsatile mass in the
region of the renal hilum or
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? abdominal bruit
? calcifcation in the area of the renal
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artery or its branches (30%) is highly suggestiveRenal Arteriovenous Fistula
? congenital
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? acquired? trauma, inflammation, renal surgery, or
percutaneous needle biopsy)
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? Hypertension, Cardiac failure
? CD/MRA/CTA/DSA
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? Angioembolisation/Surgical ligationANOMALIES OF THE COLLECTING
SYSTEM
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? Calyceal Diverticulum
-a cystic cavity within the kidney lined by
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transitional epithelium communicateswith a calyx or, less commonly, with the renal
pelvis through a narrow isthmus.
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Dx-CT or MR urography.
T/t-marsupialization of the diverticulum,
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fulguration of the epithelial lining,? Hydrocalycosis ?obstruction/VUR
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? Megacalycosis-Nonobstructive? Extrarenal Calyces
? Infundibulopelvic Stenosis
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? Extrarenal Pelvis
? Bifid pelvis
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Congenital cyst
Congenital/Acquired
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? Autosomal Recessive (Infantile) Polycystic Kidney Disease? Autosomal Dominant (Adult) Polycystic Kidney Disease
? Juvenile Nephronophthisis and Medul ary Cystic Disease Complex
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? Other Inheritable Renal Cystic Diseases (Congenital Nephrosis)
? Familial Hypoplastic Glomerulocystic Kidney Disease (Cortical Microcystic
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? Disease)? Multiple Malformation Syndromes with Renal Cysts
? Multicystic Dysplastic Kidney
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? Benign Multilocular Cyst (Cystic Nephroma)
? Simple Cysts
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? Medul ary Sponge Kidney? Sporadic Glomerulocystic Kidney Disease
? Acquired Renal Cystic Disease
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? Calyceal Diverticulum (Pyelogenic Cyst)
? Parapelvic and Renal Sinus Cysts
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ARPKDADPKD
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ARPKD
ADPKD
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ARPKD
ADPKD
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? PKDH1 gene
PKD 1 and PKD 2 gene
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? Firocystin (polyductin)Hypertension, ESRD
? Renal/ hepatic failure and portal hypertension
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Anti hypertensive( ACE inhibitors)
? Most die early
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Renoprotective measuresAspiration/ Deroofing / Nephrectomy
Ectopic ureter and Ureterocoele
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Ectopic Ureter does not enter the
trigonal area
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vagina, uterus,
Perineum,
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urethra, rectumSeminal vesicle, vas
deferens, prostatic
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urethra,
? Ureteroceles ?
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? cystic dilation of thedistal ureter
? Located either within the
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bladder or spanning the
bladder neck and
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urethra.? Single / duplex system
? Unilaterally/ bilaterally
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? MC duplex system unilaterallyIn a duplex system the ectopic ureter is
inevitably the upper pole ureter due to its
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budding from the mesonephric duct later
(more cephalad) than the lower pole ureteral
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bud.? C/F-
? Incidental
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? Vague abdominal discomfort? Recurrent UTI
? Incontinence
? Prolapsed ureterocoele
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UrethraEctopic
ureter
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? Diagnosis:
? USG
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? IVP? CT Urography
? MRU
? Endoscoppy
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Ureterocoele
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? Transurethral incision
? Ureterocoele excision
? Upper pole nephrectomy
? Ureteric reimplantation
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? Thank you