Part 9:

GENITOURINARY &
TRANSPLANTATION
Chapter 48:
The Kidneys & Ureters:
“A Healthy Outside Starts from the Inside”

------ Robert Urich

Congenital Abnormalities:
I. Horseshoe Kidney:
 It is a congenital disorder in which the kidneys are fused together.
 It occurs when the medial subdivisions of the mesonephric bud meet and fuse.
 As the kidneys ascend during fetal development, they get trapped under inferior mesenteric artery
and remain low in the abdomen.
 Features:
o Incidence is 1: 1000
o More common in men
o 90% are fused at the lower pole; 10 % are fused at the upper pole
o Lie in front of 4th lumber vertebra and great vessels.

 Clinical Features:
o Pelviureteric obstruction
o Infection – angulated ureters cause urinary stasis
o Stone formation (nephrolithiasis) – angulated ureters cause urinary stasis
o It is not a contraindication to pregnancy
o Associated with Turner syndrome.

 Diagnosis & Treatment:

o It is usually a radiological diagnosis.
o Urogram shows lower pole calyces on both sides being directed towards the midline.
o Division of isthmus between the kidneys is only indicated in the course of surgery for
abdominal aortic aneurysm.
o Surgical management of complications, e.g.:
 Pelviureteric Obstruction – Pyelouretroplasty
 Kidney stones – ESWL (see below) or open surgery

Horseshoe Kidney
II. Adult Polycystic Kidney Disease (APKD):
 Genetics:
o Trait = autosomal dominant.
o Mutations = genes PKD1 (85%) and PKD2 (15%).
o Women > men

 Clinical Features:
o Women > men
o Bilaterally enlarged kidneys – irregular upper quadrant abdominal mass
o Dull loin pain
o Hematuria – recurrent hematuria due to cyst rupture
o Infection – pyelonephritis
o Hypertension (caused by increased renin production) –70% of patients over the age of 20.
o Uremia – sign of end-stage renal failure.

 Associations:
o Berry aneurysms
o Mitral valve prolapse
o Urinary tract infections
o Benign hepatic cysts

 Diagnosis:
o Cytologic examination of cyst fluid obtained by FNAC will rule out cancer.
o Urography confirms diagnosis:
 Renal shadows are enlarged in all directions
 Renal pelvis is compressed and elongated.
 Calyces are stretched over the cysts.
 Giving the typical “big, bizarre and bilateral” appearance.

 Treatment:
o Low protein diet
o Antibiotics for infection
o Antihypertensives
o Surgery to uncap the cysts (Rovsing’s procedure) is rarely indicated.
o Renal replacement therapy:
 Dialysis
 Renal transplantation

III. Childhood Polycystic Kidney Disease:

 Genetics:
o Trait = autosomal recessive
o Mutations = gene PKHD1

 Clinical Features:
o Many patients are stillborn (significant renal failure in utero can lead to Potter’s
syndrome).
o Renal failure in early life
o Hypertension
o Anemia
o Kidney stones
o Associations:
 o Congenital hepatic fibrosis
o Splenomegaly and portal hypertension

 Management:
o Infection – antibiotics
o Hypertension – antihypertensives (ACE-inhibitors are the drugs of choice)
o Anemia – iron and erythropoietin
o Dialysis
o Renal transplantation

IV. Simple Renal Cyst:

 It is a benign non-hereditary cystic disease.
 It is characterized by single or multiple cysts in normal-sized kidneys.
 It is diagnosed on ultrasound, and can be differentiated from renal tumors by:
o Smooth thin walls
o Homogenous contents
o Avascularity
o Fluid rather than solid signals.

 Treat only if causing obstruction.

 Percutaneous cyst puncture for cytology is rarely necessary.

V. Duplication of Renal Pelvis or Ureter:

 Duplication of a renal pelvis is found in about 4% of patients.
 Duplication of a renal pelvis is usually unilateral and left-sided.
 Duplication of a ureter is found in about 3% of urograms.
 Duplicated ureters often join in the lower third of their course and have a common Ureteric orifice.
 Clinical Features:
o Often asymptomatic and harmless
o Pelviureteric obstruction
o Infection
o Stone formation
o Ectopic second ureteric opening:
 In women, ectopic ureter opens into the urethra BELOW the sphincter, therefore
incontinence can occur.
 In men, ectopic ureter opens into the urethra ABOVE the sphincter, therefore
incontinence doesn’t occur.

 Treatment:
o Usually doesn’t require treatment.
o If one ureter is severely disease = partial nephrectomy
o If causing urinary reflux = re-implanting
o If causing incontinence:
 Excision, OR
 Implanting the ectopic ureter into the bladder or contralateral ureter.

VI. Uretrocele:
 It refers to cystic enlargement of the intramural ureter.
 It results from congenital atresia of the ureteric orifice.
 It is more common in women.
 Diagnosis:
 o Urography : Typical adder head appearance.
o Cystoscopy : Translucent cyst, enlarging and collapsing as urine flows in from above.

 Treatment:
o Asymptomatic : No treatment
o Symptomatic : Endoscopic diathermy incision.

Typical Case:
 A 32-year old man presents to the ER with frank hematuria. He has several
month history of bilateral loin pain and recurrent UTI. On examination, his BP
is 165/100 mmHg and is tender in both renal angles. Cardiac auscultation
reveals a systolic murmur with mid-systolic click.

 Diagnosis: Adult Polycystic Kidney Disease (hint: mitral valve prolapse)

Renal Calculi:
I. Introduction:
 It affects men more than women, with peak age of onset between 20 – 30 years.
 It is unilateral in majority of cases.
 Risk Factors:
o Dehydration
o Decreased urinary citrate (e.g. during menstruation)
o Renal infection
o Prolonged immobilization
o Deficiency vitamin A
o Hyperparathyroidism

II. Types of Stone:

 Calcium Oxalate Calculus:
o It is the most common stone.
o It is irregular in shape, and covered with sharp projections.
o It can cause bleeding.
o It is radio-opaque and precipitates at acidic pH.

 Magnesium Ammonium Phosphate:

o Also known as “struvite” or “staghorn” calculus.
o It is smooth and dirty white.
o It is radio-opaque and precipitates at alkaline pH.
o It is caused by infection with urease-positive organism (Proteus, Staphylococcus) that
hydrolyze urea to ammonia causing urinary alkalinization.
o It may be clinically silent even when very large.

 Uric Acid & Urate Calculi:

o These are hard, smooth, and often multiple.
o These are radio-lucent (not visible on x-rays) and precipitate at acidic pH.
o These stones appear on urogram as a filling defect; confirmed by CT.
o Strong association with hyperuricemia (e.g. gout)
o Most uric acid stones contain some calcium, so they cast a faint radiological shadow.

 Cystine & Xanthine:

o Cystine calculi are radio-opaque, hexagonal, translucent, white crystals.
o Cystine calculi are radio-opaque and precipitate at acidic pH.
 o Xanthine calculi are extremely rare.
o Xanthine calculi are smooth, round, and brick-red in color.

III. Clinical Features & Investigations:

 Pain is the leading symptom in 75% of people.
o Fixed pain is located anteriorly in the hypochondrium.
o Fixed pain is located posteriorly in the renal angle.
o Pain is aggravated on movement, particularly on climbing stairs.
o Ureteric colic:
 Severe pain on a background of continuing pain.
 Pain radiates to the groin, penis, scrotum, or labium.
 Severity of pain is not related to the size of the stone.
 Pain is almost invariably associated with hematuria.
 Pain is not associated with fever.
 Rigidity of lateral abdominal muscles but not, as a rule, of the rectus abdominis.

 Investigations:
o Complete blood count
o X-ray KUB (kidney, ureter, bladder):
 Branched opacity = calculus
 Opacity that maintains its position relative to urinary tract during respiration =
calculus

o Contrast-enhanced CT scan – mainstay of investigation for acute ureteric colic.

o Excretion Urography – confirms presence and site of calculus
o Ultrasound - most valuable for locating stones for treatment by ESWL

IV. Treatment:
 Renal stones that are small (< 0.5 cm) may be managed expectantly - most will pass spontaneously.
 Renal stones if bilateral; the kidney with better function is treated first unless the other kidney is
more painful or there is pyonephrosis.
 Extracorporeal Shockwave Lithotripsy (ESWL):
o It is the preferred treatment option for stones ≤ 2 cm.
o It involves using focused, externally generated ultrasonic shock-waves.
o It causes disintegration of stones into fragments that are then voided.
o It can be done without general anesthesia.
o The results with harder stones, especially cystine stones are less satisfactory.
o Complications:
 Infection (most common)
 Ureteric colic
 Obstruction by bulky fragments of a large stone.
 Show-waves can cause capillary damage.

 Percutaneous Nephrolithotomy (PCNL):

o Indications:
 Stones > 2 cm
 Stones > 1 cm in renal pelvis (in the lower pole) – due to poor clearance rates
 In conditions where ESWL are contraindicated:
 Pregnancy
 Patients with abdominal aortic aneurysm (AAA)
 o It involves placement of a hollow needle into renal collecting system using fluoroscopic
guidance.
o Nephroscope is then inserted and the calculus visualized.
o Small stones may be grasped under vision and extracted whole.
o Larger stones must be fragmented by an ultrasound or laser and removed in pieces.
o Complications:
 Hemorrhage from punctured renal parenchyma.
 Perforation of collecting system
 Perforation of colon
 Perforation of pleural cavity

 Open Surgery:
o Pyelolithotomy  for stones in the renal pelvis.
o Extended pyelolithotomy  avoids major vessels; even large stones to be removed intact
o Nephrolithotomy  for complex calculus branching into peripheral calyces.
o Partial Nephrectomy  for stone in lowermost calyx + infective damage to adjacent
parenchyma.

 Prevention of Recurrence:
o Increase oral fluid intake
o Decrease calcium intake
o Treat infections promptly
o Urinary alkalization, e.g. sodium bicarbonate 5-10 g/day PO in water
o Thiazide diuretics (for idiopathic hypercalciuria)

Ureteric Calculus:
I. Introduction:
 Ureteric stone usually comes from the kidney.
 Stones pass spontaneously from ureter, but there are sites of narrowing where the stone may be
arrested.
 Normal anatomical narrowing of ureter where a stone gets arrested are:
o Ureteropelvic junction
o At point crossing the iliac artery
o Juxtaposition of vas deferens (broad ligament in females)
o At point entering the bladder wall
o At ureteric orifice.

 Clinical Features:
o Ureteric colic (see above)
o Hematuria
o Symptoms and signs of renal failure (if obstruction)
o Must be differentiated from appendicitis:
 The patient is in greater pain than appendicitis.
 The patient is less systemically ill than in appendicitis.

II. Investigations & Treatment:

 Investigations:
o Abdominal X-ray (KUB)
o Intravenous Urography during pain confirms diagnosis.
o Spiral CT-scan
o Retrograde ureterography
o Cystoscopy
  Treatment:
o NSAIDs for pain such as diclofenac and indomethacin.
o Expectant treatment for small stones.
o Indications for surgical removal:
 Repeated attacks of pain and the stone is not moving
 Stone is enlarging
 Complete obstruction of the kidney
 Urine is infected
 Stone is too large to pass

 Treatment Options:
o Endoscopic removal using Dormia stone-catching basket
o Ureteric meatotomy – endoscopic opening of ureter via an incision using a diathermy
knife.
o Ureteroscopic removal, which is a long thin endoscope passed transurethrally.
o Push Bang:
 A stone in the middle or upper part of ureter can be flushed back into kidney using
a ureteric catheter.
 A J-stent secures the repositioned calculus in the kidney for subsequent treatment
with ESWL.

o Open Surgery i.e. ureterolithotomy:

 Calculi in the upper-third : through loin incision.
 Calculi in the middle-third : through muscle-cutting iliac fossa incision
 Calculi in the lower third : through a Pfannenstiel incision.

Renal Infections:
I. Introduction:
 Renal infections arise either via hematogenous route or ascending route (most common).
 Renal infections are commonly caused by bacteria E. coli (most common) and other Gram-negative
organisms.
 E. coli and streptococcal infections produce acidic urine.
 Proteus and staphylococcal infections produce alkaline urine (urea-splitting property)

II. Acute Pyelonephritis:

 It is acute inflammation of tubules and interstitium.
 Risk Factors:
o UTI
o Female (shorter urethra)
o Pregnancy
o Diabetes
o Immunosuppression
o Instrumentation
o Vesicoureteral reflux (VUR).

 Clinical Features:
o Acute pain in flank and hypochondrium
o Fever
o Dysuria, frequency, and urgency
o Risk of life-threatening septicemia.
 o Childhood:
 Cloudy or offensive urine
 Pain or screaming on micturition
 Urinary frequency and secondary enuresis
 50% with urinary infection have an underlying anatomic abnormality, e.g. VUR.
 A single proven UTI in child should prompt referral to a pediatric urologist.

 Investigations:
o Urine culture & sensitivity
o Complete blood count
o Urea and electrolytes
o Blood cultures
o Renal tract ultrasonography
o Intravenous Urography (IVU)

 Treatment:
o Adequate fluid intake
o Alkalinisation of urine by potassium citrate
o NSAIDs or Morphine analgesics for pain
o Antibiotics

III. Chronic Pyelonephritis:

 Also known as “reflux nephropathy” because it’s so often associated with Vesicoureteral reflux.
 It is characterized by:
o Asymmetrical contraction of kidneys.
o Tubules show areas of atrophy and dilation.
o The dilated tubules are filled with colloid casts i.e. thyroidization.
o Glomeruli retain their normal structure until the final stage of the disease.

 Clinical Features:
o More common in women.
o Lumbar pain, dull and non-specific in character.
o Hypertension in 40%.
o Increased urinary frequency and dysuria.
o Attacks of low-grade fever.

 Investigations:
o Urinalysis
o Urea and electrolytes
o Renal tract US
o Intravenous Urography
o Voiding cystourethrogram – investigation of choice for Vesicoureteral reflux.

 Treatment:
o Treat the predisposing factors, e.g. removal of stones or obstruction.
o Antibiotics
o Surgical treatment is indicated only when the disease confined to one kidney.
o Patients with end-stage renal failure require renal replacement therapy.
o Nephrectomy or partial nephrectomy may control infections and make hypertension easier
to control.
IV. Renal Tuberculosis:
 It arises from hematogenous infection from a distant focus.
 It results in chronic inflammation and scarring throughout the urinary tract.
 It is usually confined to one kidney.
 It may be bilaterally affected as part of the generalized process of miliary TB.
 It is often associated with TB of the bladder.
 Clinical Features:
o It occurs between 20 – 40 years of age.
o It is twice as common in men as in women
o It affects the RIGHT kidney more than the left.
o Urinary frequency is often the first symptom.
o The patient complains of a progressive increase in both daytime and night-time frequency.
o It is a cause of sterile pyuria.
o Painful micturition is a feature as soon as TB cystitis sets in.
o Renal pain (dull ache in the loin)
o Hematuria.
o Malaise, weight loss, and a low-grade evening fever is typical.

 Investigations:
o Bacteriologic examination of at least 3 specimens of early-morning urine.
o Ziehl-Neelsen staining of urine.
o Lowenstein-Jensen culture of urine
o Abdominal X-ray
o Chest X-ray
o Intravenous urography
o Cystoscopy

 Treatment:
o Anti-tuberculous therapy (ATT).
o Surgery is aimed at removing large foci of infection and correction of obstruction.
o The optimum time for surgery is between 6 and 12 weeks after the start of ATT.
o An obstructed lower pole calyx may be drained into the upper ureter.
o A strictured renal pelvis needs a pyeloplasty.
o Ureteric stenosis and shortening may require Boari operation.
o If the kidney has no function it is best to perform a nephroureterectomy.
Neoplasms of the Kidney:
I. Renal Cell Carcinoma:
 Also known as “hypernephroma” or “Grawitz’s tumor”.
 It is an adenocarcinoma; and the most common neoplasm of the kidney.
 It arises from renal tubular cells.
 Features:
o More common in men.
o Most common site = upper pole
o Classic triad of costovertebral pain, palpable mass and hematuria.
o Rapidly developing left-sided varicocele is a rare but impressive sign.
o Tumor is prone to grow into the renal vein.
o Distant metastasis to lung is most common giving rise to cannonball appearance.
o It can involve bones; and via lymph nodes in the hilum of kidney to the para-arotic nodes
and beyond.

 Atypical Presentations:
o No local symptoms in 25% of cases.
o Persistent pyrexia with no evidence of infection is sometimes the only symptom.
o Pyrexia after nephrectomy suggests metastases.
o Polycythemia occurs in 4% of cases due to production of erythropoietin by tumor cells.
o Hypercalcemia is common.
o Nephrotic syndrome is rare presentation.

 Investigations:
o Intravenous Urography
o Plain abdominal X-ray (KUB)
o CT-scan
o Full blood count
o Urea creatinine and electrolytes
o Chest X-ray
o Isotope bone scan

 Treatment:
o Nephrectomy is the only curative treatment if the tumor is confined to the kidney.
o Nephrectomy can be performed through a loin or transabdominal incision.
o Radical nephrectomy is recommended for large tumors.
o Partial nephrectomy may be suitable for peripheral tumors < 4cm in size.
o Important points to know during nephrectomy:
 Transabdominal approach gives better exposure than the loin approach.
 The vascular pedicle should be ligated BEFORE the kidney is mobilized, to prevent
spilling of the tumor cells into the circulation.
 The first step in the procedure is to clean the renal artery and ligate it in
continuity.
 The renal vein should be gently palpated to ensure that there is no tumor in its
lumen.
 The renal vein, and then the renal artery are ligated and divided.
 The kidney is then mobilized within its coverings.
 Adenocarcinoma of the kidney responds poorly to radiotherapy or conventional
chemotherapy.
II. Wilms’ Tumor:
 Also known as “nephroblastoma”.
 It is a mixed tumor containing blastemal, stromal, and epithelial elements arising from embryonic
nephrogenic tissue.
 It is the most common primary malignant renal tumor of childhood.
 It is thought to be due to loss of tumor suppressor gene on chromosome 11 (WT-1 gene)
 Features:
o It is usually discovered during first 5 years of life.
o It usually unilateral.
o It typically presents with abdominal mass, which may be rapidly growing in size.
o Hematuria denotes extension of tumor into the renal pelvis and is associated with poor
prognosis.
o Metastasis to the lungs occurs early.
o Metastasis to liver, bone, and brain are rare.
o Lymphatic spread is uncommon.

 Investigations:
o CT abdomen
o CT chest (lung metastases)
o US with Doppler to exclude tumor in the renal vein or vena cava.
o Diagnosis is by needle biopsy to exclude other rarer renal tumors
o Open biopsy should be avoided.

 Treatment:
o Unilateral tumors = chemotherapy followed by nephrectomy.
o Bilateral tumors = partial nephrectomy (in order to preserve some renal tissue)
o Prognosis = survival rate is ≥ 80%
o Prognosis = is poor if metastases present.