- •Hematuria II: causes and investigation
- •Hematospermia
- •Lower urinary tract symptoms (LUTS)
- •Nocturia and nocturnal polyuria
- •Flank pain
- •Urinary incontinence in adults
- •Genital symptoms
- •Abdominal examination in urological disease
- •Digital rectal examination (DRE)
- •Lumps in the groin
- •Lumps in the scrotum
- •2 Urological investigations
- •Urine examination
- •Urine cytology
- •Radiological imaging of the urinary tract
- •Uses of plain abdominal radiography (KUB X-ray—kidneys, ureters, bladder)
- •Intravenous pyelography (IVP)
- •Other urological contrast studies
- •Computed tomography (CT) and magnetic resonance imaging (MRI)
- •Radioisotope imaging
- •Post-void residual urine volume measurement
- •3 Bladder outlet obstruction
- •Regulation of prostate growth and development of benign prostatic hyperplasia (BPH)
- •Pathophysiology and causes of bladder outlet obstruction (BOO) and BPH
- •Benign prostatic obstruction (BPO): symptoms and signs
- •Diagnostic tests in men with LUTS thought to be due to BPH
- •Why do men seek treatment for their symptoms?
- •Watchful waiting for uncomplicated BPH
- •Medical management of BPH: combination therapy
- •Medical management of BPH: alternative drug therapy
- •Minimally invasive management of BPH: surgical alternatives to TURP
- •Invasive surgical alternatives to TURP
- •TURP and open prostatectomy
- •Indications for and technique of urethral catheterization
- •Indications for and technique of suprapubic catheterization
- •Management of nocturia and nocturnal polyuria
- •High-pressure chronic retention (HPCR)
- •Bladder outlet obstruction and retention in women
- •Urethral stricture disease
- •4 Incontinence
- •Causes and pathophysiology
- •Evaluation
- •Treatment of sphincter weakness incontinence: injection therapy
- •Treatment of sphincter weakness incontinence: retropubic suspension
- •Treatment of sphincter weakness incontinence: pubovaginal slings
- •Overactive bladder: conventional treatment
- •Overactive bladder: options for failed conventional therapy
- •“Mixed” incontinence
- •Post-prostatectomy incontinence
- •Incontinence in the elderly patient
- •Urinary tract infection: microbiology
- •Lower urinary tract infection
- •Recurrent urinary tract infection
- •Urinary tract infection: treatment
- •Acute pyelonephritis
- •Pyonephrosis and perinephric abscess
- •Other forms of pyelonephritis
- •Chronic pyelonephritis
- •Septicemia and urosepsis
- •Fournier gangrene
- •Epididymitis and orchitis
- •Periurethral abscess
- •Prostatitis: presentation, evaluation, and treatment
- •Other prostate infections
- •Interstitial cystitis
- •Tuberculosis
- •Parasitic infections
- •HIV in urological surgery
- •6 Urological neoplasia
- •Pathology and molecular biology
- •Prostate cancer: epidemiology and etiology
- •Prostate cancer: incidence, prevalence, and mortality
- •Prostate cancer pathology: premalignant lesions
- •Counseling before prostate cancer screening
- •Prostate cancer: clinical presentation
- •PSA and prostate cancer
- •PSA derivatives: free-to-total ratio, density, and velocity
- •Prostate cancer: transrectal ultrasonography and biopsies
- •Prostate cancer staging
- •Prostate cancer grading
- •General principles of management of localized prostate cancer
- •Management of localized prostate cancer: watchful waiting and active surveillance
- •Management of localized prostate cancer: radical prostatectomy
- •Postoperative course after radical prostatectomy
- •Prostate cancer control with radical prostatectomy
- •Management of localized prostate cancer: radical external beam radiotherapy (EBRT)
- •Management of localized prostate cancer: brachytherapy (BT)
- •Management of localized and radiorecurrent prostate cancer: cryotherapy and HIFU
- •Management of locally advanced nonmetastatic prostate cancer (T3–4 N0M0)
- •Management of advanced prostate cancer: hormone therapy I
- •Management of advanced prostate cancer: hormone therapy II
- •Management of advanced prostate cancer: hormone therapy III
- •Management of advanced prostate cancer: androgen-independent/ castration-resistant disease
- •Palliative management of prostate cancer
- •Prostate cancer: prevention; complementary and alternative therapies
- •Bladder cancer: epidemiology and etiology
- •Bladder cancer: pathology and staging
- •Bladder cancer: presentation
- •Bladder cancer: diagnosis and staging
- •Muscle-invasive bladder cancer: surgical management of localized (pT2/3a) disease
- •Muscle-invasive bladder cancer: radical and palliative radiotherapy
- •Muscle-invasive bladder cancer: management of locally advanced and metastatic disease
- •Bladder cancer: urinary diversion after cystectomy
- •Transitional cell carcinoma (UC) of the renal pelvis and ureter
- •Radiological assessment of renal masses
- •Benign renal masses
- •Renal cell carcinoma: epidemiology and etiology
- •Renal cell carcinoma: pathology, staging, and prognosis
- •Renal cell carcinoma: presentation and investigations
- •Renal cell carcinoma: active surveillance
- •Renal cell carcinoma: surgical treatment I
- •Renal cell carcinoma: surgical treatment II
- •Renal cell carcinoma: management of metastatic disease
- •Testicular cancer: epidemiology and etiology
- •Testicular cancer: clinical presentation
- •Testicular cancer: serum markers
- •Testicular cancer: pathology and staging
- •Testicular cancer: prognostic staging system for metastatic germ cell cancer
- •Testicular cancer: management of non-seminomatous germ cell tumors (NSGCT)
- •Testicular cancer: management of seminoma, IGCN, and lymphoma
- •Penile neoplasia: benign, viral-related, and premalignant lesions
- •Penile cancer: epidemiology, risk factors, and pathology
- •Squamous cell carcinoma of the penis: clinical management
- •Carcinoma of the scrotum
- •Tumors of the testicular adnexa
- •Urethral cancer
- •Wilms tumor and neuroblastoma
- •7 Miscellaneous urological diseases of the kidney
- •Cystic renal disease: simple cysts
- •Cystic renal disease: calyceal diverticulum
- •Cystic renal disease: medullary sponge kidney (MSK)
- •Acquired renal cystic disease (ARCD)
- •Autosomal dominant (adult) polycystic kidney disease (ADPKD)
- •Ureteropelvic junction (UPJ) obstruction in adults
- •Anomalies of renal ascent and fusion: horseshoe kidney, pelvic kidney, malrotation
- •Renal duplications
- •8 Stone disease
- •Kidney stones: epidemiology
- •Kidney stones: types and predisposing factors
- •Kidney stones: mechanisms of formation
- •Evaluation of the stone former
- •Kidney stones: presentation and diagnosis
- •Kidney stone treatment options: watchful waiting
- •Stone fragmentation techniques: extracorporeal lithotripsy (ESWL)
- •Intracorporeal techniques of stone fragmentation (fragmentation within the body)
- •Kidney stone treatment: percutaneous nephrolithotomy (PCNL)
- •Kidney stones: open stone surgery
- •Kidney stones: medical therapy (dissolution therapy)
- •Ureteric stones: presentation
- •Ureteric stones: diagnostic radiological imaging
- •Ureteric stones: acute management
- •Ureteric stones: indications for intervention to relieve obstruction and/or remove the stone
- •Ureteric stone treatment
- •Treatment options for ureteric stones
- •Prevention of calcium oxalate stone formation
- •Bladder stones
- •Management of ureteric stones in pregnancy
- •Hydronephrosis
- •Management of ureteric strictures (other than UPJ obstruction)
- •Pathophysiology of urinary tract obstruction
- •Ureter innervation
- •10 Trauma to the urinary tract and other urological emergencies
- •Renal trauma: clinical and radiological assessment
- •Renal trauma: treatment
- •Ureteral injuries: mechanisms and diagnosis
- •Ureteral injuries: management
- •Bladder and urethral injuries associated with pelvic fractures
- •Bladder injuries
- •Posterior urethral injuries in males and urethral injuries in females
- •Anterior urethral injuries
- •Testicular injuries
- •Penile injuries
- •Torsion of the testis and testicular appendages
- •Paraphimosis
- •Malignant ureteral obstruction
- •Spinal cord and cauda equina compression
- •11 Infertility
- •Male reproductive physiology
- •Etiology and evaluation of male infertility
- •Lab investigation of male infertility
- •Oligospermia and azoospermia
- •Varicocele
- •Treatment options for male factor infertility
- •12 Disorders of erectile function, ejaculation, and seminal vesicles
- •Physiology of erection and ejaculation
- •Impotence: evaluation
- •Impotence: treatment
- •Retrograde ejaculation
- •Peyronie’s disease
- •Priapism
- •13 Neuropathic bladder
- •Innervation of the lower urinary tract (LUT)
- •Physiology of urine storage and micturition
- •Bladder and sphincter behavior in the patient with neurological disease
- •The neuropathic lower urinary tract: clinical consequences of storage and emptying problems
- •Bladder management techniques for the neuropathic patient
- •Catheters and sheaths and the neuropathic patient
- •Management of incontinence in the neuropathic patient
- •Management of recurrent urinary tract infections (UTIs) in the neuropathic patient
- •Management of hydronephrosis in the neuropathic patient
- •Bladder dysfunction in multiple sclerosis, in Parkinson disease, after stroke, and in other neurological disease
- •Neuromodulation in lower urinary tract dysfunction
- •14 Urological problems in pregnancy
- •Physiological and anatomical changes in the urinary tract
- •Urinary tract infection (UTI)
- •Hydronephrosis
- •15 Pediatric urology
- •Embryology: urinary tract
- •Undescended testes
- •Urinary tract infection (UTI)
- •Ectopic ureter
- •Ureterocele
- •Ureteropelvic junction (UPJ) obstruction
- •Hypospadias
- •Normal sexual differentiation
- •Abnormal sexual differentiation
- •Cystic kidney disease
- •Exstrophy
- •Epispadias
- •Posterior urethral valves
- •Non-neurogenic voiding dysfunction
- •Nocturnal enuresis
- •16 Urological surgery and equipment
- •Preparation of the patient for urological surgery
- •Antibiotic prophylaxis in urological surgery
- •Complications of surgery in general: DVT and PE
- •Fluid balance and management of shock in the surgical patient
- •Patient safety in the operating room
- •Transurethral resection (TUR) syndrome
- •Catheters and drains in urological surgery
- •Guide wires
- •JJ stents
- •Lasers in urological surgery
- •Diathermy
- •Sterilization of urological equipment
- •Telescopes and light sources in urological endoscopy
- •Consent: general principles
- •Cystoscopy
- •Transurethral resection of the prostate (TURP)
- •Transurethral resection of bladder tumor (TURBT)
- •Optical urethrotomy
- •Circumcision
- •Hydrocele and epididymal cyst removal
- •Nesbit procedure
- •Vasectomy and vasovasostomy
- •Orchiectomy
- •Urological incisions
- •JJ stent insertion
- •Nephrectomy and nephroureterectomy
- •Radical prostatectomy
- •Radical cystectomy
- •Ileal conduit
- •Percutaneous nephrolithotomy (PCNL)
- •Ureteroscopes and ureteroscopy
- •Pyeloplasty
- •Laparoscopic surgery
- •Endoscopic cystolitholapaxy and (open) cystolithotomy
- •Scrotal exploration for torsion and orchiopexy
- •17 Basic science of relevance to urological practice
- •Physiology of bladder and urethra
- •Renal anatomy: renal blood flow and renal function
- •Renal physiology: regulation of water balance
- •Renal physiology: regulation of sodium and potassium excretion
- •Renal physiology: acid–base balance
- •18 Urological eponyms
- •Index
558 CHAPTER 15 Pediatric urology
Cystic kidney disease
Congenital cystic kidney disease can be classified into genetic and nongenetic types.
Genetic
Autosomal recessive polycystic kidney disease (ARPKD)
ARPKD is a disease of infancy and childhood, where renal collecting tubules and ducts become cystically dilated and numerous small cysts form in the renal cortex and medulla. Severe forms present early and have a poor prognosis.
Prenatal US demonstrates oligohydramnios (amniotic fluid <200 mL) and large, “bright” homogeneously hyperechogenic kidneys, which can cause obstructed labor and respiratory problems.
Neonates have large flank masses and limb and facial anomalies. All cases are associated with congenital hepatic fibrosis. Infants develop fatal uremia and respiratory failure; older children present with renal failure, hypertension, and portal hypertension. Most develop end-stage renal failure by adulthood, requiring hemodialysis and renal transplantation.
Autosomal dominant polycystic kidney disease (ADKD)
ADKDA Typically presents in adulthood, although older children can present with complications of hematuria, flank pain, flank mass, UTI, proteinuria, hypertension, and intracerebral bleeds (secondary to berry aneurysm rupture).
It is characterized by multiple expanding cysts of both kidneys that ultimately destroy the intervening parenchyma, and accounts for 10% of all chronic renal failure.
Familial juvenile nephronophthisis
This is an autosomal recessive disorder that develops in early childhood and accounts for up to 20% of pediatric renal failure. Medullary cystic disease is a similar (autosomal dominant) condition that develops in later childhood.
Histology shows interstitial nephritis associated with medullary and corticomedullary cysts. Disease progression causes a reduction in kidney size. Features include polyuria, polydipsia, anemia, growth retardation, and chronic renal failure.
Renal cysts are also a feature of autosomal dominant conditions, including Von–Hippel–Lindau syndrome (cerebellar hemangioblastomas, pheochromocytoma, renal cell carcinoma) and tuberous sclerosis (adenoma sebaceum, epilepsy, learning difficulties).
Nongenetic
Multicystic dysplastic kidney (MCDK)
MCDK is the most common cystic kidney disorder and second most common cause of abdominal mass in newborns (after UPJ obstruction). The cysts of a multicystic kidney are not due to dilatation of renal collecting ducts (as in polycystic disease); instead, the entire kidney is dysplastic, with immature dysplastic stroma and cysts of various sizes. The kidney does
CYSTIC KIDNEY DISEASE 559
not have a renoform shape or a calyceal drainage system—the ureter is atretic.
Bilateral disease is incompatible with life. Unilateral disease is often associated with reflux (20–40%) or UPJ obstruction (10%) in the contralateral kidney. Affected kidneys may undergo renal aplasia, where they spontaneously shrink to a tiny remnant.
Ultrasound helps to distinguish this condition (noncommunicating cysts) from hydronephrosis. Renal scanning shows no function.
Most patients can be treated conservatively with close surveillance for the associated risks of hypertension and Wilms’ tumor, which would be indications for surgery. It may involute spontaneously.
Multilocular cystic nephroma
This condition presents in young children, with unilateral flank mass. It is included in a spectrum of diseases that are closely associated with Wilms tumor, so the recommended treatment is partial or full nephrectomy.
There is bimodal distribution—male infants <2 and female adults >40 years of age.
560 CHAPTER 15 Pediatric urology
Exstrophy
Bladder exstrophy results from defective development of the anterior bladder and lower abdominal walls, leaving the posterior bladder wall lying exposed on the abdomen.
Epidemiology
Incidence is 3.3 cases per 100,000 live births, with a male-to-female ratio >2:1. There is and increased risk in offspring of affected patients and with younger maternal age and increased parity.
Embryology
An embryological malformation results in the abnormal overdevelopment of the cloacal membrane, which prevents in-growth of lower abdominal tissues. The cloacal membrane normally perforates to form the urogenital and anal openings, but in exstrophy there is premature rupture, resulting in a triangular defect below the umbilicus.
The timing of this rupture determines the type of defect (bladder exstrophy, cloacal exstrophy, OR epispadias).
Associated anomalies
•Bone defects: diastasis (widening) of the symphysis pubis due to outward rotation of the pelvic bones along the sacroiliac joints (Fig. 15.8)
•Musculofascial defects: umbilical hernias, inguinal hernias, abnormal pelvic floor
•Genital defects: Males have a short, broad epispadiac penis with lateral splaying of the corporal cavernosa, and short urethral plate. Females have a bifid clitoris, stenotic vaginal orifice, short vaginal canal, and vaginal prolase.
•Urinary tract defects: Most patients suffer vescioureteric reflux (VUR) due to lateral displacement of the ureteric orifices.
•GI defects: anteriorly displaced anus, rectal prolapse; abnormal anal sphincter contributes to incontinence.
Investigation
Typical features seen on prenatal ultrasound scan include a lower abdominal wall mass, absent bladder filling, low-set umbilicus, small genitalia, and abnormal iliac crest widening. Diagnosis can help in planning of delivery in a center with facilities to perform early surgical correction.
Management
At birth, cover the bladder with plastic film and irrigate regularly with sterile saline. Trauma to the bladder mucosa can eventually result in squamous metaplasia, cystitis cystica, or adenocarcinoma and squamous cell carcinoma after chronic exposure.
EXSTROPHY 561
Selected cases are suitable for one-stage repair, but most require a three-stage procedure:
•Newborn: pelvic osteotomy (cutting bone to correct deformity) with external fixation with closure of bladder, abdominal wall, and posterior urethra
•6–12 months: epispadias repair
•4–5 years: Bladder neck reconstruction (Young–Dees–Leadbetter procedure) and anti-reflux surgery (ureteric reimplantation) are performed when there is adequate bladder capacity and children can participate in voiding protocols. Where bladder capacity is too small, bladder augmentation or urinary diversion is required.
Figure 15.8 KUB from exstrophy patient shows characteristic wide separation of pubes.