Download MBBS (Bachelor of Medicine, Bachelor of Surgery) 1st Year, 2nd Year, 3rd Year and Final year Urology 5 Congenital Anomalies of Kidney And Ureter PPT-Powerpoint Presentations and lecture notes
Congenital anomalies of kidney
and ureter
Department of Urology
?Anomalies of the Upper Urinary Tract
?Congenital cyst
?Ectopic ureter
?Ureterocoele
Anomalies of the Upper Urinary Tract
? ANOMALY OF NUMBER
? ANOMALIES OF ROTATION
? Bilateral Renal Agenesis
? ANOMALIES OF RENAL
? Unilateral Renal Agenesis
VASCULATURE
? Supernumerary Kidney
? Aberrant, Accessory, or
? ANOMALIES OF ASCENT
Multiple Vessels
? Simple Renal Ectopia
? Renal Artery Aneurysm
? Cephalad Renal Ectopia
? Renal Arteriovenous Fistula
? Thoracic Kidney
? ANOMALIES OF THE COLLECTING
? ANOMALIES OF FORM AND
SYSTEM
FUSION
? Calyceal Diverticulum
? Hydrocalycosis
? Crossed Renal Ectopia with
? Megacalycosis
and without Fusion
? Infundibulopelvic Stenosis
? Horseshoe Kidney
? Bifid Pelvis
? ANOMALY OF NUMBER
? Bilateral Renal Agenesis
? Unilateral Renal Agenesis
? Supernumerary Kidney
Bilateral Renal Agenesis
? Very rare, 500 cases reported in the literature
? no single etiology
? esophageal atresia, cryptophthalmos or
Fraser syndrome, Klinefelter syndrome and
Kallmann syndrome
? 40% -stil born.
? born alive do not survive beyond 48 hours because of
respiratory distress associated with pulmonary
hypoplasia.
? Potter facies ,oligohydramnios-pathognomonic.
? The ureters -absent,
? Bladder- absent or hypoplastic.
? The adrenal glands are usual y positioned normal y.
? MD anomalies are commonly observed.
? prominentfoldandskincreasebeneatheacheye,
bluntednose,
depressionbetweenlowerlipandchin
Unilateral Renal Agenesis
? 1 in 1100 births
? Kallmann syndrome, Turner syndrome, Poland
syndrome, Fraser syndrome, branchio-oto-
renal (BOR) syndrome, DiGeorge syndrome
? Anomaly of other organ
Supernumerary Kidney
? distinct encapsulated parenchymal mass with
its own col ecting system, blood supply
? Very rare
? Asymptomatic/symptomatic
? T/t- if symptom
ANOMALIES OF ASCENT
? Simple Renal Ectopia
? Cephalad Renal Ectopia
? Thoracic Kidney
ek ("out") and topos ("place")
MC
? Mostly asymptomatic
? renal pelvis is usual y anterior
(instead of medial)
? Hydronephrotic
? PUJO
? VUJO
? Reflux
? Malrotation
? Ureter- not redundent
? Vascular supply-
? Not from abdominal aorta
? Associated anomaly:
? Female
? Contralateral renal agenesis
? bicornuate or unicornuate uterus
? rudimentary or absent uterus and
proximal and/or distal vagina
? duplication of the vagina.
? Male
? undescended testes, duplication of the urethra, and
hypospadias, cloacal malformation
? Dx- Incidental
? USG/CT/MRI/Nuclear scan
? Clinical implication-
? Calculus(Obstruction-malrotation, High insertion,
crossing vessel)
? RVH
? No difficult labour
Cephalad Renal Ectopia (ascent
anomaly)
? In omphalocoele
Thoracic Kidney
? Exceedingly Rare
? Asymptomatic
? incidental
? ANOMALIES OF FORM AND FUSION
? Crossed Renal Ectopia with and without Fusion
? Horseshoe Kidney
Crossed Renal Ectopia
with and without Fusion
Horseshoe kidney
? most common of al renal fusion anomalies
? 1 in 400 persons
? skeletal, cardiovascular, and central nervous
system anomaly
? trisomy 18, Turner syndrome, Townes-Brock
syndrome
? 50% asymptomatic
? hydronephrosis, infection, or
calculus, UPJ obstruction
? high insertion of ureter, angulation of ureter
over isthmus, aberrant blood vessel
? Wilms tumor
ANOMALIES OF ROTATION
The kidney and renal pelvis normal y rotate 90 degrees ventromedial y
during ascent so that the calyces point lateral y and the pelvis faces
medial y.
When this alignment
is not exact, the
condition is known
as malrotation
It is frequently associated with Turner syndrome
ANOMALIES OF RENAL VASCULATURE
? Aberrant- not arising
from aorta
(iliac/gonadal/mesenteric
)
? Accessory-more than one
artery supplying same
segment
? Multiple Vessels-more
than one artery supplying
different segment
? Nephrectomy
? Transplantation
Renal Artery Aneurysm
? Incidence-0.1% - 0.3%.
Most -silent,
? diagnosed during an evaluation of hypertension.
? pulsatile mass in the
region of the renal hilum or
? abdominal bruit
? calcifcation in the area of the renal
artery or its branches (30%) is highly suggestive
Renal Arteriovenous Fistula
? congenital
? acquired
? trauma, inflammation, renal surgery, or
percutaneous needle biopsy)
? Hypertension, Cardiac failure
? CD/MRA/CTA/DSA
? Angioembolisation/Surgical ligation
ANOMALIES OF THE COLLECTING
SYSTEM
? Calyceal Diverticulum
-a cystic cavity within the kidney lined by
transitional epithelium communicates
with a calyx or, less commonly, with the renal
pelvis through a narrow isthmus.
Dx-CT or MR urography.
T/t-marsupialization of the diverticulum,
fulguration of the epithelial lining,
? Hydrocalycosis ?obstruction/VUR
? Megacalycosis-Nonobstructive
? Extrarenal Calyces
? Infundibulopelvic Stenosis
? Extrarenal Pelvis
? Bifid pelvis
Congenital cyst
Congenital/Acquired
? Autosomal Recessive (Infantile) Polycystic Kidney Disease
? Autosomal Dominant (Adult) Polycystic Kidney Disease
? Juvenile Nephronophthisis and Medul ary Cystic Disease Complex
? Other Inheritable Renal Cystic Diseases (Congenital Nephrosis)
? Familial Hypoplastic Glomerulocystic Kidney Disease (Cortical Microcystic
? Disease)
? Multiple Malformation Syndromes with Renal Cysts
? Multicystic Dysplastic Kidney
? Benign Multilocular Cyst (Cystic Nephroma)
? Simple Cysts
? Medul ary Sponge Kidney
? Sporadic Glomerulocystic Kidney Disease
? Acquired Renal Cystic Disease
? Calyceal Diverticulum (Pyelogenic Cyst)
? Parapelvic and Renal Sinus Cysts
ARPKD
ADPKD
ARPKD
ADPKD
ARPKD
ADPKD
? PKDH1 gene
PKD 1 and PKD 2 gene
? Firocystin (polyductin)
Hypertension, ESRD
? Renal/ hepatic failure and portal hypertension
Anti hypertensive( ACE inhibitors)
? Most die early
Renoprotective measures
Aspiration/ Deroofing / Nephrectomy
Ectopic ureter and Ureterocoele
Ectopic Ureter does not enter the
trigonal area
vagina, uterus,
Perineum,
urethra, rectum
Seminal vesicle, vas
deferens, prostatic
urethra,
? Ureteroceles ?
? cystic dilation of the
distal ureter
? Located either within the
bladder or spanning the
bladder neck and
urethra.
? Single / duplex system
? Unilaterally/ bilaterally
? MC duplex system unilaterally
In a duplex system the ectopic ureter is
inevitably the upper pole ureter due to its
budding from the mesonephric duct later
(more cephalad) than the lower pole ureteral
bud.
? C/F-
? Incidental
? Vague abdominal discomfort
? Recurrent UTI
? Incontinence
? Prolapsed ureterocoele
Urethra
Ectopic
ureter
? Diagnosis:
? USG
? IVP
? CT Urography
? MRU
? Endoscoppy
Ureterocoele
? Transurethral incision
? Ureterocoele excision
? Upper pole nephrectomy
? Ureteric reimplantation
This post was last modified on 08 April 2022