CONGENITAL

UROLOGICAL
CONDITIONS
Dr. Hawkar A. Kak-Ahmed Khayyat
Principles of Pediatric Urology
1. If one urinary tract anomaly is present, a second anomaly is
likely.
2. The wide range of urogenital disorders and diseases, in terms
of presentation, the patient’s age, damage to renal function, and
other functional problems, make it necessary for these patients to
be cared for by specialized pediatric urologists and pediatric
nephrologists' teams, preferably in a specialist center.
3. Urinary tract infection (UTI) and urinary tract obstruction
(UTO) are common and dangerous problems in childhood.
4. The first aim is to protect renal function.
5. In the 1st year of life VUR and a very high pressure during
micturition are physiological, and its coordination by the
nervous system is still developing. For this reason, bladder
surgery should, if possible, be avoided during the 1st year of
life if necessary.
6. For most problems there are guidelines for parents available
from appropriate pediatric and urological associations.
7. Functional problems often lead to social and psychological
problems.
Hematuria
The differential diagnosis of gross hematuria and microscopic hematuria
may be carried out according to the following four criteria:
1. The most common causes of hematuria are in accordance with the
pediatric nephrologists:
a. glomerulonephritis,
b. urinary tract infection,
c. injury of kidneys and urinary tract system, and
d. idiopathic hematuria.
2. According to the following subgroups of hematuria:
a. unconfirmed hematuria
b. isolated hematuria
c. hematuria combined with bacteriuria
d. hematuria combined with proteinuria
3. According to the organs of origin of hematuria: The methods of
determination of the origin of erythrocyturia by the distribution of
the erythrocyte volumes and occurrence of dysmorphic
erythrocytes are of minor significance in pediatric urology and
surgery.
4. The differential diagnosis according to pathoanatomical
criteria is the most appropriate for pediatric urology and surgery.
Differential Diagnosis of
Hematuria
Urinary tract malformations:
• Pelvicureteric junction anomalies (obstruction)
• Retrocaval ureter
• Obstructive megaureter
• Ureterocele
• Diverticulum (renal pelvis, bladder, urethra)
• Ureteral duplications
• Renal fusion
• Vesicoureteral reflux
• Neurogenic bladder
• Lower urinary tract obstructions
Tumors:
• Nephroblastoma and other renal tumors
• Rhabdomyosarcoma (bladder trigonum, prostate)
• Polyp (pelvicureteric junction, ureter, bladder, urethra)
• Hemangioma, neuro fibroma, etc.
Injuries:
• Renal injury
• Bladder, urethral, and ureteral rupture
• Other types of renal trauma and injuries to external genitals and
urethra
• Post interventional and operative hematuria
• Penis manipulations
Urinary lithiasis, foreign bodies
• Urolithiasis
• Foreign Bodies of bladder and urethra
Urinary tract infections
• Urogenital schistosomiasis (bilharziosis)
Pediatric disorders of pediatric urological relevance
• Renal vein thrombosis
• Shunt nephritis
• Hemolytic-uremic syndrome
Ureteropelvic Junction
Obstruction
• Inadequate drainage of urine from the renal pelvis into the upper
ureter, resulting in hydrostatic distention of the renal pelvis and
calyces.
• Causes:
a. intrinsic stenosis (failure of recanalization, accounts for 75% of
cases)
b. Congenital kinking
c. A lower pole vessel crossing the ureter as it joins the renal pelvis
(20%)
• More common in boys and on the left side.
Clinical Presentation
• Most hydronephrotic kidneys are detected antenatally.
• Palpable mass in the abdomen as a result of hydronephrosis.
• Hematuria (after minor trauma or exercise)
• Urinary tract infection
• Renal hypertension
• Cyclic or acute abdominal, flank, or back pain associated with nausea
and vomiting.
• Associated malformations [VATER/VACTERL syndrome (syndrome
with a wide range of anatomical births defects, i.e., vertebral, anal,
cardiac, tracheo-esophageal, renal and limb abnormalities), multicystic
kidneys, reflux]
• Nephrolithiasis (long-standing obstruction and infection)
Investigations
• Antenatal and postnatal ultrasonographic examination
• Urine examination (including urine culture)
• 24-hour creatinine clearance
• Isotope studies (diuretic renogram DTPA or MAG3) to distinguish
between obstructing and non-obstructing hydronephrosis
• Voiding cystourethrography (rule out vesicoureteral reflux and
subvesical obstruction)
• Intravenous pyelography (only in selected cases)
 Treatment

Pyeloplasty: excising the stenotic area of the renal pelvis or uretero-pelvic junction and
creating a more capacious conduit using the tissue of the remaining ureter and renal pelvis.
Differential Diagnosis of UTI
Obstructive uropathies upper urinary tract (about 40%):
• Ureteropelvic junction obstructions
• Obstructive megaureter
• Retrocaval ureter
• Prune-belly syndrome
• Ureteroceles
• Polyps in urinary tract, nephrolithiasis
• Extrinsic obstructions
Ureteral duplications (about 1%):
• Complete ureteral duplication
• Incomplete ureteral duplications
Renal fusions, ectopia, and a-, hypo-, and dysplasia (about 2.5%):
• Horseshoe kidney
• Renal ectopia
• Renal agenesis, a-, hypo-, dysplasia (about 35 %)
Cystic renal diseases (about 14%):
• Multicystic renal dysplasia [AR polycystic kidney disease]
• Solitary renal cyst
• Other cystic renal diseases, megacalycosis
Vesicoureteral reflux
Bladder diverticula
Cystitis
• Granular cystitis
Neurogenic bladder, functional voiding disorders, obstructive
uropathies lower urinary tract
• Phimosis
• Other disorders
Disorders of Micturition
Obstructive uropathies of the lower urinary tract:
• Bladder neck obstructions
• Müllerian duct remnants, utricle cyst
• Posterior urethral valves
• Other rare urethral pathologies
• Urethral stricture
• Meatal stenosis
• Urethral diverticulum
• Phimosis
• Labial synechia
• Interlabial masses
Neurogenic bladder, functional voiding disorders:
• Neurogenic bladder
• Dysfunctional voiding
Acute and chronic urinary retention:
• Acute urinary retention
• Chronic urinary retention
Clefts of bladder and/or urethra, ureteral duplication:
• Bladder exstrophy
• Epispadias
• Complete ureteral duplication
• Other pathologies
Genital Bleeding and Vaginal Discharge
Physiologic Hemorrhages:
• They occur in the first week of the neonatal period as withdrawal
bleeding after the discontinuation of the maternal estrogens: A
vaginal discharge with occult blood is observed in one third of
the newborns and a visible hemorrhage starting the second day
after birth in about 3%.
Menarche
• Menarche at the beginning of the maturation period and later
menstruation belong to the physiological bleedings.
• Menarche starts at about 12 years of life with a variation of 8(6)–16
years.
• Menarche before 8 years of age corresponds to a precocious
puberty .
• If it starts after 16 years, to a delayed puberty.
• If it is absent, to a primary amenorrhea.
Causes of Pathological Genital
Bleedings in the Girl
Neonatal period:
• Birth injuries
• Congenital tumors
• Malformations
First month of life until menarche:
• Vulvovaginitis
• Foreign bodies of the vagina
• Trauma (falling astride including impalement injuries, sexual abuse)
• Tumors (rhabdomyosarcoma, endodermal sinus tumor)
• Precocious puberty
Menarche and thereafter:
• Trauma, multisystemic organ injury, sexual abuse, defloration
bleeding
• Vulvovaginitis
• Foreign bodies of the vagina
• Dysfunctional bleedings (e.g., anovulation [estrogenmbreakthrough
bleeding])
• Tumors (cervical or endometrial polyps, submucosal leiomyomas,
uterine and cervical carcinoma)
• Hypothyroidism, bleeding disorders
Differential Diagnoses of Testicular and
Scrotal Swelling (pain)
• Testicular torsion
• Epididymitis
• Torsion of the testicular and paratesticular appendages
• Scrotal injury, zipper entrapment
• Testicular hydrocele or hydrocele of the cord and complete inguinal
hernia
• Acute idiopathic scrotal edema
• Insect sting and bite
• Scrotal swelling in Henoch-Schönlein purpura
• Orchitis
• Other (inflammatory) disorders of the scrotum
• Peritoneal disorders with scrotal swelling
• Varicocele
• Testicular tumor
• Idiopathic infarction of testis and tunica vaginalis,
spontaneous thrombosis of the plexus pampiniformis, torsion
of a cyst of the tunica vaginalis
Cryptorchidism (Empty Scrotum)
• Classification:
• Undescended testis:
• Palpable
• Impalpable
• Retractile testis
• Ectopic testis
• Testicular descent:
• Gubernaculum
• Intra-abdominal pressure
• Local hormones
• Processus viginalis
• Diagnosis is clinical.
• Needs to be taken down because of:
• Malignancy
• Infertility
• Torsion
• Treatment
• No role for hormone therapy.
• If palpable then do open surgery.
• If impalpable do laparoscopically.
• If high do orchiectomy
• If low do one-stage (fowler-stephens) or two-stage (Shehata) orchidopexy.
Differential Diagnosis of Conspicuous
and Abnormal External Genitals in Boys
• Epispadias (bladder exstrophy complex)
• Hypospadias
• Micropenis, pseudomicropenis
• Penis torsion and lateral deviation
• Rare malformations of the penis
• Priapism
Circumcision
• Relative indications:
1. Paraphimosis.
2. Balanoposthitis and balanitis.
3. Preputial “pearls” and redundant foreskin.
4. Phimosis.
5. Prevention of sexually transmitted
6. infections.
7. Prevention of penile cancer.
8. Prevention of UTI in boys with urological
9. abnormalities.
10.Trauma.
Circumcision Complications
1. Bleeding.
2. Infection.
3. Phimosis (insufficient inner skin removal)
4. Insufficient Foreskin Removed
5. Excessive Foreskin Removed.
6. Adhesions / Skin Bridges.
7. Inclusion Cysts.
8. Abnormal Healing.
9. Meatitis.
10.Glans amputation
Circumcision Technique
• Surgical
• Gomco clamp
• Plastibell
• Sutureless (thermal coagulation)
Hypospadias
Differential Diagnosis of Conspicuous
External Genitals in a Girl
Malformations:
• Bladder exstrophy, epispadias
• Hypospadias
• Interlabial masses a
• Imperforate hymen
• Other vaginal obstructions (vaginal atresia), vaginal agenesis
• Duplications of vagina and uterus
Variations of normality, acquired disorders:
• Pseudohypertrophy of clitoris and/or labia, synechia of clitoris
frenulum
• Pseudoimperforate hymen
• Labial synechia
• Injuries, female genital mutilation
• Tumors of the external genitals
Differential diagnosis of
ambiguous external genitals
Newborns
1. 46, XX disorders of sex differentiation
• Congenital adrenal hyperplasia (CAH), complicated CAH
• Ovotesticular DSD (true hermaphroditism)
• Testicular DSD (XX-male)

2. 46, XY disorders of sex differentiation

• Complete and incomplete androgen insensitivity syndrome (cAIC, pAIC)
• Defects of testosterone synthesis and dihydrotestosterone transformation
• Disorders with abnormal differentiation of the testis
• Oviduct persistence (persistent Müllerian duct syndrome)

3. Chromosomal disorders of sex differentiation

• Asymmetrical mixed gonadal dysgenesis
Infants and older children
1. 46, XX disorders of sex differentiation
• Congenital adrenal hyperplasia (CAH), late-onset CAH, CAH in boys

2. 46, XY disorders of sex differentiation

• Complete androgen insensitivity syndrome, testicular feminization
• Pure gonadal dysgenesis
• Oviduct persistence (persistent Müllerian duct syndrome)

3. Chromosomal disorders of sex differentiation

• Turner syndrome
• Klinefelter’s syndrome