MOST COMMON

PEDIATRIC
UROLOGICAL
EMERGENCIES
Mohamed Zawky
Resident of urology
Mansoura urology and nephrology center
1-Antenatal hydronephrosis
2 –Bladder exstrophy
3 – Acute scrotum
4 - PARAPHIMOSIS
5- Congenital adrenal hyperplasia
 Antenatal
hydronephrosis
 It ranges from 0.6 to 5.4%.
 Bilateral in 17‑54% .
 Ultrasound screening during pregnancy has resulted in increasing its recognition ,
although ANH resolves by birth or during infancy in 41‑88% patients, urological
abnormalities requiring intervention are identified in 4.1‑15.4%.
 It is important to distinguish infants with significant illness that require long ‑term
follow‑up or surgery, from those with transient hydronephrosis and minimum need for
invasive investigations.
Etiology
1. Transient hydronephrosis
2. Pelviureteric junction obstruction
3. Vesicoureteric reflux
4. Vesicoureteric junction obstruction, megaureter
5. Multi cystic dysplastic kidney
6. Duplex kidneys (± ureterocele)
7. Posterior urethral valve
8. Others: Urethral atresia, urogenital sinus, prune belly syndrome, tumors
How to approach ( Diagnosis & Management)
Posterior urethral
valve
It represents 1 in 4000–6000 live births .
 Presentation:

1. The fetus
 80% of cases are detected on prenatal ultrasound
 dilatation of the urinary tract may not develop until much later in pregnancy.
 55% of cases of prenatally detected posterior urethral valves are picked up on
routine maternal ultrasound performed between 16 and 20 weeks.
 Ultrasound features of posterior urethral valves in the fetus :
1. Bilateral upper tract dilatation
2. Persistently distended full bladder

 Predictors of poor functional outcome and early-onset renal failure:

1. Detection before 24 weeks’ gestation
2. Bladder wall thickening
3. Echo-bright kidneys (renal dysplasia)
4. Oligohydramnios
2- The neonate :

 Symptoms usually relate to bladder outflow obstruction or, less often, to the effects of
impaired renal function.
 Listlessness, poor feeding, irritability and failure to thrive are common.
 The urinary stream, if witnessed, is usually poor.
 The bladder is palpable in most instances; the kidneys may also be palpated.
 Urinary ascites occurs as an occasional complication.
3-The infant :

 Presentation in infancy is generally with a urinary infection and in the majority of cases
physical signs are absent.
 Gram-negative sepsis and renal failure with gross electrolyte disturbance were common forms
of presentation but with increasing detection by prenatal ultrasound and heightened awareness
of urinary infection among infants they have become unusual.
 Chronically impaired renal function is usually manifest as poor growth
Investigations :

1. Prenatal

 The presence of posterior urethral valves can only be inferred from the ultrasound
appearances of a distended fetal bladder and dilated upper tracts

 Diagnosis can be made with more certainty if there is dilatation of the posterior urethra – the
so-called ‘keyhole sign’
2. Postnatal:
 Ultrasonography :
- is the initial investigation
- The relevant findings comprising upper tract dilatation (which is

sometimes only unilateral), perinephric urinoma (occasionally),

thickening of the bladder wall, with or without residual urine.
 Micturating cystourethrography (MCU) :
- provides the definitive diagnosis.
- Vesicoureteric reflux is present in 40–60% of cases at the time of initial
evaluation and is unilateral in approximately two-thirds of cases.
Management
1. The fetus

 The rationale for fetal intervention derives from experimental studies.

 prenatal intervention, in the form of vesicoamniotic shunting and intrauterine valve ablation are very rare.
 Termination of pregnancy is the most common form of prenatal intervention, particularly when severe
dilatation and associated oligohydramnios are detected in early pregnancy
 Elective preterm delivery represents another form of intervention, and it may be beneficial in cases of
rapidly progressing late-onset dilatation. It is important to balance lung maturity with the risk of
progressive renal damage in deciding the optimum timing for preterm delivery.
1. The neonate

To minimise risk of electrolyte disturbances or of urinary infection , the obstruction should

be relieved promptly:

- Urethral or suprapubic bladder drainage : The usual primary treatment, this will remain for 7–10
days to obtain baseline renal function.

- Endoscopic valve ablation : The valve leaflets are incised at the 5 , 7 and 12 o’clock positions .

- Vesicostomy : Vesicostomy drainage may be preferred in those with markedly impaired renal function,
especially if deteriorating despite catheterization, and/or in those with gross bilateral
vesicoureteric reflux.
Bladder exstrophy
 Embryology

 Failure of migration of mesoderm to the area of the cloacal membrane,

and subsequent lack of development of an intermediate layer between its inner
(endodermal) and outer (ectodermal) layers, leads to rupture and exposure of an
open bladder plate and urethra.

 These structures occupy a triangular space between a low-set umbilicus superiorly,

split rectus abdominis muscles on each side and an open pelvic ring inferiorly.
 Incidence:
1 : 50 000 live births
Male to female ratio: 3:1

 Antenatal diagnosis:
The detection rate of cases of bladder exstrophy by antenatal
ultrasound is currently around 40% at 18 weeks’ gestation.
 Diagnostic features include:
- inability to visualise urine in the fetal bladder
- low set umbilical cord
- a short, wide penis
- bulging bladder plate
Presentation and
clinical features
 If not detected antenatally, classic bladder exstrophy presents at birth with a visible bladder plate
below a low-set umbilical cord.

 The mucosa may become inflamed and polypoid, especially after a few
hours’ exposure following delivery.

 The shaft of the penis is usually short and thick with a good-sized glans.
 The scrotum is present in boys with the distinctive upwards direction of the rugae and the testes are
usually palpable within it.

 Classic bladder exstrophy has few associated anomalies and babies are usually born at or close to
term and are otherwise well.

 Inguinal hernias are present in up to 80% of boys and 15% of girls but may not become apparent until
after primary closure.
Management
Initial neonatal repair : it carried out during the first days of the fetal life to avoid a
additional orthopedic procedures such as bilateral iliac
ostieotomies .
multiple factors such as anasthetic hazards and deficiency of
clotting factors development make this maneuver rarely
use nowdays.
Late infancy repair: there are 2 schools regarding the technique of the repair
1-complete primary closure : which include closure of the
bladder with anterior abdominal wall defect, repair of the
epispadias , bladder neck surgerey and bilateral iliac ostieotomies.

2- modern staged closure : it includes closure of the

bladder with anterior abdominal wall defect, and bilateral iliac
ostieotomies with later on epispadias and bladder neck surgery.
Acute scrotum
Differential diagnosis of acute scrotum
Presentation of acute scrotum
Scrotalpain
Swelling
Redness
Oedema
Generalized illness
Diagnosis and management
Rapid notes about testicular torsion
 It is the most common cause of rapid onset testicular pain and swelling in
people under 18 years old .
 It occurs in about 1 in 4,000 to 1 per 25,000 males per year before 25
years of age
 t it can occur at any age, including infancy.
 it has different variants :
Intravaginal testicular torsion

 Intravaginal testicular torsion occurs when the testicle rotates on the

spermatic cord within the tunica vaginalis.

 more commonly occurs in older children and adults.

Extravaginal testicular torsion

 Torsion occurring outside of the tunica vaginalis .

 when the testis and gubernaculum can rotate freely, is termed an
extravaginal testicular torsion. This type occurs exclusively in newborns,
however, newborns can be affected by other testicular torsion variants as
well.
 Management of testicular torsion

 Urgent surgical exploration is the keystone of management.

 Following exposure of the testis via a scrotal incision, the spermatic cord is untwisted and testicular viability
assessed.
 Factors influencing the decision to conserve or remove the testis include the duration of the history, the
appearance of the testis and arterial bleeding on incising the tunica albuginea
 When exploration has been undertaken within the first few hours and the testis is clearly viable it should be
fixed to prevent recurrent torsion.
 Regardless of the outcome on the affected side, fixation of the contralateral testis is mandatory in view of the
bilateral nature of the anatomy predisposing to torsion.
Paraphimosis
 Paraphimosis is urologic emergency, occurring in uncircumcised males, in
which the foreskin becomes trapped behind the corona and forms a tight
band of constricting tissue
Etiology

 The cause of paraphimosis is most often iatrogenic; the condition

frequently occurs after penile examination, urethral catheterization
or cystoscopy. Paraphimosis typically occurs after Foley catheter
placement.
 During the insertion of a urethral catheter, the foreskin is retracted to
prepare and drape the glans penis. After insertion of the catheter, the
practioner may not return the retracted foreskin to its normal
position.
Management of paraphimosis
MANUAL REDUCTION
Manual pressure may reduce edema. A gloved hand is circled around the distal penis
to apply circumferential pressure and disperse the edema. Ice packs are also useful in
reducing swelling of the penis and prepuce
PHARMACOLOGIC THERAPY

 Injection of hyaluronidase into the edematous prepuce is effective in

resolving edema and allowing the foreskin to be easily reduced
MINIMALLY INVASIVE THERAPY

 The “puncture” technique is a minimally invasive therapy in which a hypodermic

needle is used to directly puncture the edematous prepuce. Puncture sites permit
safe and effective evacuation of the trapped fluid. External drainage of the
trapped fluid allows for manual reduction of paraphimosis.

paraphimosis.
 SURGICAL THERAPY
If a severely constricting band of tissue precludes all forms of conservative or
minimally invasive therapy, an emergency dorsal slit should be performed. This
procedure should be performed with the use of a local anesthetic by a physician
experienced with the technique.
Congenital adrenal
hyperplasia
(CAH)
 CAH is the most common cause of 46XX DSD (female pseudo-hermaphroditism)

 It also accounts for approximately 85% of all infants with ambiguous genitalia in Western countries.

 The disorder can arise as a result of one of three enzymatic defects occurring at different points on the
biosynthetic pathways for the production of cortisol and aldosterone in the adrenal gland.

 All three defects prompt over production of adrenocorticotrophic hormone (ACTH), which in turn stimulates
overproduction of androgenic precursors by the adrenals.
Classification :
 21-Hydroxylase deficiency (most common)

 11β-Hydroxylase deficiency

 17α-Hydroxylase deficiency

 3β-Hydroxysteroid dehydrogenase deficiency

 Lipoid CAH
 21- Hydroxylase Deficiency

 is the most common form of CAH ( 95% of all cases)

 Incidence 1:15 000 births

 It is an autosomal recessive disorder associated with a mutation of a gene located on chromosome 6

 50% of cases are complicated by a salt-losing state which may pose a potentially life-threatening neonatal
emergency.
21- Hydroxylase Deficiency

 Classic type
1- salt wasting form
2- simple virilizing form

 Non classic type

Salt wasting form :
 Most lethal form of 21 hydroxylase deficiency
 Manifests as salt water crisis at 2 to 6 weeks of life

Clinical findings :
 Vomiting
 Lethargy
 FFT
 Shock with decreased urine output
 Abnormal genital appearance (clitoral enlargement , scrot-labial mass and labial fusion )

Biochemical findings:
 Hyponatremia
 Hyperkalemia
 Hpoglycemia
 Metabolic acidosis
 Important Lab Investigations

 Serum electrolytes
 Arterial blood gas
 Serum cortisol
 Serum ACTH
 17α-hydroxy-progesterone
 Karyotyping
 The diagnostic feature is elevation of plasma 17α-hydroxyprogesterone.

 Prenatal diagnosis is possible and treatment has been described using dexamethasone to suppress the fetal
pituitary–adrenal axis from the 9th to the 17th weeks of gestation .

 Management comprises gluco- and mineralocorticoid replacement therapy, female gender assignment and
consideration of feminising genitoplasty .
Thank you