Duplication of Ureteric System/ Duplex ureter

Ectopic Sites:
- Male: Urethra.
- Female: Vagina (causes urinary dribbling).
Investigation:

- Intravenous Urogram (IVU):
- Urograffin dye used.

- "Drooping Lily" sign
- (duplication + pelvic malrotation).
- Mnemonic: Droop → Dupe

Management:
- Ureter reimplantation.
Horseshoe Kidney



Features:
- M > F (2:1)
- Lower poles of both kidneys are fused.
- Located at L3-L4 level.
- Ascent restricted by inferior mesenteric artery.
- Adrenal glands are in normal position (separate embryology).
Investigation:
- Known as joining hands or handshake or flower vase appearance
- Has association with Turner's syndrome.
- IVU: "Flower Vase" or "Hand Shake" sign.
- Fuse at isthmus
- Inferior mesenteric Artery → Anterior to isthmus → Prevent ascent


Complications
- ↑ Risk of RCC, Wilms Tumor, Multicystic dysplastic kidney
- UTI
- Nephrolithiasis/Hydronephrosis
Management:
- Pyeloplasty: If hydronephrosis/malrotated pelvis.
- Avoid cutting the fused portion due to devascularization risk.
Polycystic Kidney Disease (PCKD)


PRESENTATION
- HEMATURIA+ HYPERTENSION +LOIN/ FLANK PAIN ( IMP)
- Gross haematuria following trauma → due to cyst rupture.
Types:
Feature | ADPKD | Autosomal Recessive Polycystic Kidney Disease (ARPKD) |
Prognosis | Adult | Infantile → Fatal |
Chromosomes | PKD1 (Chromosome 16) PKD2 (Chromosome 4) | PKHD1 (Chromosome 6) |
Gene | PKD gene | PKHD gene |
Protein | Polycystin | Fibrocystin |
Cyst Appearance | Cysts on both outer and inner surfaces of kidney. | Only inner surface shows cysts; outer surface normal. |
Associated Organs | ABCD • Aortic aneurysms/dissections • Berry aneurysms • Cysts in pancreas/liver • Colonic Diverticula | Congenital hepatic fibrosis (H for hepatic, fibro for fibrosis). |
Clinical Presentation | More common in males. Clinical Features: • Hypertension (M/c, in 3rd decade). • Abdominal mass, hematuria, multiple cysts. • Berry aneurysm Cyst size↑↑ → Symptomatic | ㅤ |
- Extrarenal Manifestations:
- Most common: Liver cysts.
- Cysts in spleen, pancreas, lungs.
- Colonic diverticulosis.
- Mitral valve prolapse.
- Berry aneurysms in Circle of Willis (can cause subarachnoid hemorrhage).
Investigation:
- Ultrasound (can be prenatal).
Xray


ADPKD
- On CT scan (Image 1), white bone is observed.
- Both kidneys are enlarged in size and have multiple cysts.
- On MRI (Image 2), black bone is observed.
- Both enlarged kidneys have multiple cysts.
- In IVP, initial or nephrogram phase:
- Multiple cysts do not take up the contrast,
- forming filling defects in the kidney.
- Spider leg appearance.
- d/t Splayed collecting system

- Swiss cheese appearance
- On nephrogram

ARPKD
- Striated appearance or sunray appearance of the kidneys.
- The kidney appears echogenic on ultrasonography.
Management:
- Transplant.
- Dialysis.
- Note: Cyst deroofing surgery not used.
Renal Cysts

Bosniak Classification for Renal Cysts
- Bosniak classification is used to group renal cysts based on malignancy risk.


Class | Description | Work up | % Risk of malignancy |
1 | Simple cyst | Nil | 0% |
2 | Minimally complex | Nil | 0% |
2F | Minimally complex | USG/CT follow up | 5% |
3 | Indeterminate | Partial nephrectomy | 50% |
4 | Clearly malignant | Partial/Total nephrectomy | 100% |
- Anandhu → Bose now → has Renal cyst

Hydronephrosis
Features:
- Aseptic dilation of the pelvicalyceal system.
Hydronephrosis
- Left kidney
- gray color density → fluid.
- Enhancing rim → renal parenchyma.
- Rim sign seen in hydronephrosis.


Unilateral Causes:
Intraluminal:
- Stone disease (most common acquired cause).
- Sloughed papillae.
Intramural:

PUJ obstruction:


- Most common congenital cause.
- Adynamic obstruction → No physical obstruction → but PUJ is not contracting
- IVU: Clubbing of calyces.
- Surgery: Anderson-Hynes Pyeloplasty.
- Mnemonic: Anangatha (Adynamic) Andi Poya (Anderson Pyeloplasty) Pug (PUJ) → Club (clubbing) il cherth
Ureterocele:

- IVU: "Cobra Head" or "Adder Head" sign.
- Cystic dilatation of ureteric terminal end.
- Surgery: Ureter reimplantation.
Transitional cell carcinoma (pelvis/ureter):
- "Goblet sign" on IVU.
Extraluminal:
- Aberrant renal vessel
- usually unilateral,
- never cut,
- manage with pyeloplasty
- Advanced cancers (colorectal, cervical, prostate).
Retroperitoneal fibrosis (Ormond's disease)


- Can compress ureters → hydronephrosis
- "Maiden Waist" deformity, medial indrawing of ureters.
- Causes:
- Idiopathic
- post-radiotherapy
- drug-induced (Methysergide).
- Management: DJ Stenting.
- Mnemonic: Purakil ninn (retroperioneal) DJ () kalikkunna Maid () ne Ormayundo (Ormond)
Retrocaval ureter:

- Right ureter passes behind IVC.
- Hydronephrosis + hydroureter (of the proximal ureter)
- "Reversed J" or "FishHook" sign.
Bilateral Causes:
- Any unilateral cause on both sides.
- Benign Prostatic Hyperplasia (BPH).
- Bladder outlet obstruction (e.g., urethral stricture).
- Posterior urethral valves.
- Phimosis.
- Meatal stenosis.
Investigations:
- IVU: Clubbing of calyces.
Renal Scans
- Technetium-99m
- DMSA:
- Mnemonic: Morphology → Scar → Static
- Used for kidney scars or morphological changes.
- Detects if the kidney is located ectopically.
- It is a static scan.
- no GFR or tubular function
- DTPA:
- Gives the function/physiology of the kidney (GFR).
- MAG3:
- Mnemonic: Magnificient
- Helps in evaluating GFR and tubular function.
Whitaker Test – Used to differentiate between obstructive vs non-obstructive hydronephrosis
Principle:
- Measures differential pressure between the renal pelvis and bladder during fluid infusion.
Procedure:
- A percutaneous nephrostomy is created (puncture through loin into the renal pelvis).
- Normal saline or contrast is infused at a constant rate (typically 10 mL/min) into the renal pelvis.
- Simultaneous measurement of pressure in:
- Renal pelvis
- Bladder
Interpretation:
- Normal: Renal pelvic pressure stays <22 cm H₂O.
- Obstruction:
- Renal pelvic pressure >22–25 cm H₂O, or a
- pressure gradient >15 cm H₂O between renal pelvis and bladder.
- While taking water (Whitaker)
Uroflowmetry

- >15 ml/sec: Normal.
- 10-15 ml/sec: Equivocal.
- <10 ml/sec: Low flow.
Christmas or pine or fir tree appearance
- Seen in neurogenic bladder.
- The tone of the bladder is affected, causing a shape change from round to elongated.
- Multiple diverticuli are present.


Teardrop or pear shape or inverted pear shape appearance

- In an IVP image, the bladder appears to have a teardrop shape.
- A normal bladder is compressed by external forces, causing elongation.
- Causes include:
- Extrinsic compression of the bladder.
- Any pelvic cause.
- e.g., pelvic lipomatosis, pelvic abscess, pelvic hematoma, pelvic lymphadenopathy, pelvic vessel aneurysm.
Fetal skull calcification of the urinary bladder


- Seen in schistosomiasis.
- Bladder wall calcification
- Schistosoma haematobium resides in the vesical venous plexus of the bladder.
Renal Stones
Types





Crystal Type | Shape | Associated With | Mnemonic / Notes |
Tyrosine | Very thin needles | ㅤ | "Tyrosine → inject insulin → thin needle" |
Bilirubin | Thin pigmented (golden-brown) needles | ㅤ | ㅤ |
Ammonium Biurate | Apple-thorn | Laxative abuse | ㅤ |
Leucine | Lamellated (like tree bark) | ㅤ | Leucine → Lamelle |
Cholesterol | Broken chips | ㅤ | "chips rich in cholesterol" |
Calcium Carbonate | Round like car wheels | ㅤ | "carbonate, car wheels" |
Calcium Oxalate:

- Most common type.
- Radio-opaque.
- Formed in acidic urine.
- Monohydrate:
- Most common
- Dumbbell-shaped, very hard.
- SIngles use dumbell
- Dihydrate:
- Envelope-shaped, spiculated ("Mulberry" stones),
- present early with pain/hematuria
- Doubles → send letters → envelope
Triple Phosphate (Struvite/Staghorn):


- Calcium Ammonium Magnesium Phosphate.
- Associated with
- Proteus urinary tract infection.
- Parathyroid adenoma → Hypercalcemia → Calcium deposits in kidney
- Radio-opaque.
- "Coffin Lid" shaped.
- Formed in alkaline urine.
- Features
- Smooth surface, large size.
- takes up the shape of the pelvis and calyces.
- Calcification with lobulated appearance, i.e. parenchymal
- Coffin lu avathirikkan Maaninte (Staghorn) irachi thinnanam + Protein (Proteus) thinnanam→ Strong (Struvite) avanam
- DMSA scan for scarring.
Condition | IOC | Features |
Stag Horn Calculus | plain x-ray. | ㅤ |
PUJ Obstruction | IVP | Non-visualization of the ureter hydronephrosis without hydroureter affected kidney is dilated → hydronephrosis affected ureter is not seen → PUJ obstruction |
VUR | MCU / VCUG | ㅤ |


Cystine:

- "six as cis"
- Radio-opaque.
- Very hard (crystalline lattice).
- Hexagonal shape.
- Difficult to break by ESWL.
- Seen in cystinuria.
- COLA tin (cystin-uria) lost in urine
- Cystine
- Ornithine
- Lysine
- Arginine
- Management for recurrent stones: d-Penicillamine.
Uric Acid:


- Most common radiolucent stones.
- "Glass Shreds" crystals.
- ‘’Rhomboid’’ shape
- Seen in tumor lysis syndrome, gout.
- Uric acid → Universal shapes (triangular, diamond, random)
- Rx: Alkalinization of urine
Radiolucent
- Xanthine
- Orotic acid
- Urea stone
- Triamterene
- Indinavir (from anti-retroviral treatment).
- Xanthaclose (Xanthine) Urine (Uric acid) is Loose (lUcent)
Rare Stones:

Presentation
- Pain (Most common):
- Colicky pain (depends on stone site):
- Pelvis: Loin to groin radiation.
- Upper/mid ureter: Iliohypogastric nerve.
- Lower ureter: Ilioinguinal nerve.
- Impacted intramural portion: Strangury (intense urge with bloody drops).
- Fixed renal pain: At renal angle.
- Hematuria.
- Hydronephrosis.
- Dietl's Crisis:
- Stone blocks urine flow -> pain + palpable mass.
- Stone shifts -> large quantities of dilute urine.
Investigations
- CT Urography (also Radiolucent) >> Non-contrast CT (NCCT)
- Partial vs complete obstruction: Diuretic DTPA scan
Ureteric calculi
- Ureteric stone is generally vertically oval.

Renal Calculus
- A white area (right kidney) → right renal calculus
- Right kidney + psoas major muscle located posteromedially.

- On ultrasound → echogenic area + shadow behind it.
- Shadow → posterior acoustic shadowing.


B/L Nephrocalcinosis/ Medullary Nephrocalcinosis
- Lucent areas are observed between opaque areas.
- 2 causes
- A/w Hyperparathyroidism
- Medullary sponge kidney
- Functional kidney

Management
- Dietary Advice (recurrent calcium oxalate stones):
- Decrease fat content.
- Increase calcium intake.
- Increase pyridoxine dose.
- Cholestyramine: Binds oxalate in gut.
Based on stone size:

- < 5mm:
- No active intervention.
- 5 - 1.5 mm, symptomatic:
- Medical management (1st line).
- Tamsulosin (alpha blocker):
- Relaxes distal ureteric muscle.
- Fail → ESWL
- > 2 cm:
- PCNL (Percutaneous Nephrolithotomy).
Extracorporeal Shock Wave Lithotripsy (ESWL):


- Complications:
- "Stone street" (Stein strasse),
- pain (most common),
- hematuria,
- UTI.
- Ultrasonic waves fragment stone.
- Contraindications:
- Pregnancy,
- uncontrolled bleeding disorder,
- cardiac pacemaker.
- Stone > 1.5 cm.
- Children,
- obese patients.
- Very hard stones (Cystine > Calcium Oxalate Monohydrate).
- Obstructed system.
- Lower calyx stone.
Percutaneous Nephrolithotomy (PCNL):

- Indications:
- Stones > 2 cm
- lower pole stones unfavorable for SWL
- failed ESWL/RIRS,
- staghorn calculi.
- Cystine, Calcium Oxalate Monohydrate
- Mini PCNL:
- For children, tracks < 22F.
Retrograde Intrarenal Surgery (RIRS):

- Indications:
- Stones < 2 cm,
- lower pole,
- obesity,
- musculoskeletal deformities.

Ureteroscopic Removal of Stones (URS):

- Dormia basket:
- To capture stones.

- Bilateral Double J (DJ) stents:
- Facilitate passage of stone fragments,
- inserted via cystourethroscope.

Bladder Stones



Features:
- Most common in children.
- Most common type: Mixed urate.
NCCT
- Dense calcification in the center of the pelvis is observed.
- This is the bladder stone or vesical calculus.

Management:
- 1st line:
- Perurethral cystolithotomy.
- If contraindicated
- d/t urethral stricture, bladder diverticulae
- Suprapubic cystolithotomy.
Vesicoureteral Reflux (VUR)

Grades (reflux severity):


Grade | Description |
Grade 1 | Into non-dilated ureter |
Grade 2 | Into renal pelvis, no distension |
Grade 3 | Mild distension |
Grade 4 | Blunting of calyces, tortuous ureter |
Grade 5 | Severe distension, loss of papillary impressions |

Investigation:
- Micturating Cystourethrogram (MCU).

- DMSA scan for scarring.
Condition | IOC | Features |
Stag Horn Calculus | plain x-ray. | ㅤ |
PUJ Obstruction | IVP | Non-visualization of the ureter hydronephrosis without hydroureter affected kidney is dilated → hydronephrosis affected ureter is not seen → PUJ obstruction |
VUR | MCU / VCUG | ㅤ |


Treatment (due to recurrent UTIs):
- Supportive
- VUR resolves spontaneously
- Adequate fluids.
- Grades 1-5:
- Prophylactic antibiotics.
- Drugs : Cotrimoxazole/nitrofurantoin/Cephalexin.
- Regimen : Until 2 years of age + 1 year after last episode of UT.
- If no recovery for Grade 4, 5:
- STING procedure (Sub-Ureteric Teflon Injection).

Posterior Urethral Valve (PUV)
- Primary reason for bladder outlet obstruction in male children
Young's Classification
Type | Feature |
Type 1 (M/c) | Anteroinferior to verumontanum. |
Type 2 | rare |
Type 3 (Cobb’s collar). | rare |
Clinical Features
- Male child with recurrent UTI.
- A/w Oligohydramnios
Renal artery stenosis


- Affects the ostium.
- Young patient (without a family history of hypertension)
- 1st investigation is renal Doppler (Image 1)
- has a slow and blunted uptake.
- i.e. pulsus parvus et tardus waveform (Image 2)
- Normal renal artery has a quick upstroke.
- 2nd investigation is CT-angiography or MR-angiography.
- Gold standard procedure is digital subtraction angiography (DSA).
Fibromuscular dysplasia
- Beaded appearance.
- Seen in an MR-angiography.
- Indicates multiple strictures in the renal artery.
- Common in young adult females.
- Also presents with hypertension (similar to renal artery stenosis).


Ectopia Vesica (Bladder Extrophy)
- Anterior abdominal wall and anterior bladder wall
- deficient below the umbilicus.

Urethra


Length
- Female: 3-4 cm.
- Male: 18-21 cm.
Parts
- Proximal: Membranous + Prostatic Urethra.
- Distal: Penile + Bulbar Urethra.
Epithelium of Male Urethra
Part | Notes |
Pre-prostatic & Prostatic urethra | Transitional epithelium |
Membranous urethra | Pseudostratified / Stratified columnar |
Bulbar urethra | Most distensible part |
Penile (Spongy) urethra | • Proximal → Pseudostratified columnar • Distal → Stratified squamous |
Transitional epithelium / urothelium lines
- Renal pelvis and calyces,
- ureter
- urinary bladder
- pre-prostatic & prostatic parts of the urethra




Hypospadias
Features
- Most common congenital urogenital anomaly.
- Ventrally placed urethral opening.
- Associated with micropenis & undescended testis.
Types


- Most common & mildest:
- Glanular.
- Most severe:
- Perineal.
Clinical Features
- Downward-directed urine stream.
- Downward bending of penis (Chordee).
- Infertility.
- Micropenis
- H/o undescended testis
- On Examination:
- Urethral opening on the lower aspect.
- Hooded prepuce.
Hypospadias Management
Steps of Surgery:
- Orthoplasty → Urethroplasty → Meatoplasty → Glanuloplasty → Skin cover.
- Mnemonic: OUMG / OUGM
Surgical Procedures:
- Single Stage:
- Distal hypospadias:
- Mustardee, Mathieu.
- Mid hypospadias:
- Snodgrass, TIP.
- Mnemonic:
- Mastard mathai (Mustard mathieu) → does sex in single stage () → with his small grass tip (Snodgrass, TIP)
- Double Staged:
- For proximal hypospadias.
- Thiersch-Duplay,
- Dennis Brown.
- Mnemonic:
- Does sex in 2 stage () → Foreplay (Duplay) and brown dick (Dennis brown)
Renal Tuberculosis
Features:
- Secondary infection (hematogenous spread).
Presentations:



Renal

- Papillary Ulcers → Ghost calyx/Pseudo calculi → Caseous necrosis → Pus filled kidney → Calcification ("Putty" or "Cement" kidney).
Ureter

- Kirrs Kink
- Bending of ureter
- Shortening of ureter
- Golf hole ureteric orifice
- Orifice remain open
- Pale ureteric orifice
- Indicates inflammation and edema of surrounding mucosa
- Early sign
Clinical Features:
- Hematuria, pain, mass, weight loss.

Putty kidney


- Cement (amorphous calcification) + lobulated appearance = parenchymal calcification
- Moth eaten calyces
- Earliest sign in the IVP for renal TB
- irregularity of the calyx.
- Later stages of TB
- → non-functional kidney and auto-nephrectomy.
- Sterile pyuria is seen in urinary TB.
Corkscrew and Beaded appearance
- Multiple ureteric strictures

Thimble bladder


- small, contracted bladder
- Heal with fibrosis
- bladder wall calcification
Investigations:
- Urine examination: Sterile pyuria (pus cells +, culture -).
- Confirmatory:
- ZN Staining of centrifuged 3 morning urine samples.
Note:
- If genital TB
- Genital swab for polymerase chain reaction (PCR) testing
Treatment:
- Medical: Anti-tubercular treatment (ATT).
- Surgical (for complications):
- Perinephric abscess:
- Drainage.
- Ureteric kinking:
- DJ stenting.
- "Golf Hole" ureteric orifice:
- Ureteric reimplantation.
- "Thimble Bladder":
- Augmentation cystoplasty.
- Lower ureter damage:
- Boari flap repair.


Pyelonephritis
Emphysematous Pyelonephritis:


- Black areas
- Air is contained within the kidney and surrounding it.
- Organism: E.coli.
- Note: clostridium → emphysematous cholecystitis
- Common in immunocompromised, DM patients.
- Can turn fulminant and septic.
- IOC: CECT (gas in/around kidney).
- Management: Antibiotics + drainage.
Xanthogranulomatous Pyelonephritis:

- IOC: CECT
- non-functioning kidney,
- calculi,
- low-density masses
- Proteus.
- Common in middle-aged females with DM.
- Symptoms: Flank pain, pyrexia, abdominal mass.
- Management: Nephrectomy (subcapsular if management fails).
Acute pyelonephritis
- In acute pyelonephritis, the kidney is enlarged.
- In chronic pyelonephritis, the kidney is shrunken.
- Enlarged edematous kidney → Total / Focal
- A striated appearance is observed.
- Reduced areas of cortical vascularity on power Doppler

Chronic Pyelonephritis


- Cause: E. coli.
- Gross appearance: Asymmetrically contracted kidney.
- Urine microscopy: WBC cast.
Histopathological Features Chronic Pyelonephritis

- Atrophy of tubules → Lymphocytic infiltrate.
- Flattened epithelium.
- Thyroidization of tubules.
- Periglomerular fibrosis.





