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

Download MBBS (Bachelor of Medicine, Bachelor of Surgery) 1st Year, 2nd Year, 3rd Year and Final year Urology 4 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
? Thank you

This post was last modified on 08 April 2022