Download MBBS Urology Presentations 4 Congenital Anomalies of Kidney And Ureter Lecture Notes

Congenital anomalies of kidney

and ureter

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Department of Urology

?Anomalies of the Upper Urinary Tract
?Congenital cyst
?Ectopic ureter

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?Ureterocoele
Anomalies 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 AND

SYSTEM

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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depressionbetweenlowerlipandchin

Unilateral Renal Agenesis

? 1 in 1100 births

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? Kallmann syndrome, Turner syndrome, Poland

syndrome, 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 Kidney

ek ("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 omphalocoele

Thoracic 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-Brock

syndrome

? 50% asymptomatic

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? hydronephrosis, infection, or

calculus, 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 alignment

is 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 arising

from aorta

(iliac/gonadal/mesenteric

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)

? Accessory-more than one

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artery supplying same

segment

? Multiple Vessels-more

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than one artery supplying

different segment
? Nephrectomy

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? Transplantation

Renal 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 suggestive
Renal 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 ligation

ANOMALIES OF THE COLLECTING

SYSTEM

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? Calyceal Diverticulum

-a cystic cavity within the kidney lined by

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transitional epithelium communicates

with 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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ARPKD

ADPKD

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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 measures

Aspiration/ 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, rectum

Seminal vesicle, vas

deferens, prostatic

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urethra,

? Ureteroceles ?

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? cystic dilation of the

distal 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 unilaterally

In 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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Urethra

Ectopic

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