congenital anomalies ppt

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Congenital Genitourinary anomalies Urology Department Undergraduate courses

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Congenital anomalies ppt

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Page 1: Congenital anomalies ppt

Congenital Genitourinary

anomalies

Urology Department

Undergraduate courses

Page 2: Congenital anomalies ppt

For our Lectures and Scientific resources visit our web sites,

Uroainshams.blogspot.com

Uronotes2012.blogspot.com

©

Page 3: Congenital anomalies ppt

Common congenital anomalies

Common Renal anomalies

• Abnormal number: agenesis

• Abnormal form or position:

horseshoe kid.

Common Bladder anomalies

• Bladder Extrophy.

Common Urethral & penile anomalies

• Hypospadias.

• Epispadias.

Common ureteral & renal pelvis anomalies

• UPJ obstruction.

• Vesico-uretral reflux.

• Duplication.

• Uretrocele.

• Ectopic ureter.

Common genital anomalies

• Undescended testis.

• Intersex (disorders of

sexual differentiation).

Page 4: Congenital anomalies ppt

Renal agenesis

Bilateral renal agenesis

• both mesonephric ducts fail to

develop.

• Incompatible with life.

Unilateral renal agenesis

• the mesonephric duct fails to develop.

• Usually there is absent ureter, trigone,

kidney and (in boys) vas deferens.

Page 5: Congenital anomalies ppt

Horseshoe kidney

• both metanephros are fused together.

• both kidneys rotated & their lower poles

are joined in the shape of a horseshoe.

• As the fetus grows, the joined kidneys

are held up by the inferior or superior

mesenteric arteries at L3.

Page 6: Congenital anomalies ppt

Pelviureteric junction

obstruction Obstruction of the junction

between the renal pelvis & ureter.

Aetiology

• aperistaltic segment of

ureter due to absent muscles.

or

• crossing vessels over UPJ.

Page 7: Congenital anomalies ppt

• may present at any time

(before birth, in childhood, or in

adulthood) by:

• abdominal mass.

• abdominal pain.

• Haematuria after fairly minor abdominal

trauma.

Pelviureteric junction

obstruction- Presentation

Page 8: Congenital anomalies ppt

Pelviureteric junction

obstruction- Evaluation • IVU shows delay in

appearance of contrast and

dilated renal pelvis and calices.

• Renal scan shows differential

renal function and confirms

obstruction.

Page 9: Congenital anomalies ppt

• Surgery is indicated for:

1.obstructive symptoms,

2.stone formation,

3. recurrent urinary infection,

4.progressive renal impairment.

• Pyeloplasty is the treatment of choice

• Nephrectomy is performed if the affected

kidney is <10% of total renal function.

Pelviureteric junction

obstruction- Treatment

Page 10: Congenital anomalies ppt

• Minimally invasive alternative techniques:

1. antegrade endopyelotomy.

2. Laparoscopic pyeloplasty is becoming more used now.

Pelviureteric junction

obstruction

Page 11: Congenital anomalies ppt

Vesicoureteric junction reflux

• Reflux can be defined as the retrograde

flow of urine into upper urinary tract.

• incidence of reflux is equal in both sexes.

• Reflux can be classified into 5 grades

according to severity.

Page 12: Congenital anomalies ppt

Vesicoureteric junction reflux-

evaluation • Micturating cystourethrography

is the gold standard for diagnosis

and evaluation of VUR grade.

• Diuretic Renal scan (DMSA) is

used to visualize scarring and

quantify differential renal function.

Page 13: Congenital anomalies ppt

Vesicoureteric junction reflux-

Management • antibiotic prophylaxis is recommended for

children with reflux of grades I-II.

• Surgery (uretro-vesical reimplantation or endoscopic injection) is recommended in

reflux of grades III-V and persistent reflux

despite a trial of antibiotics.

Page 14: Congenital anomalies ppt

Duplication of urinary system

• Ureteral duplication is the most

frequent anomaly of urinary tract

• Female: male = 2 : 1

• ureteral orifice of the upper renal segment

drains inferiorly and medially to the orifice

of the lower segment (Weigert –Myer law).

Page 15: Congenital anomalies ppt

Duplication of urinary system

• The orifice draining the upper

segment is often obstructed.

• The orifice of the lower segment

generally refluxes.

• Duplication is usually discovered

on an IVU.

• Management is according to

segment affected and its function.

Page 16: Congenital anomalies ppt

Ectopic ureter

• An ectopic ureter is one that opens in

some location other than the bladder.

80% associated with duplicated system.

20% associated with single system.

• Most common sites (in female): urethra,

vestibule, and vagina

• In female present as urinary incontinence.

• Most common sites (in male): posterior

urethra and seminal vesicles.

Single system

duplex system

Page 17: Congenital anomalies ppt

Uretrocele • A congenital cystic ballooning of the

terminal submucosal ureter.

• It is classified as simple or ectopic.

• Simple (Orthotopic) Ureterocele:

in trigone.

• Ectopic Ureterocele: can obstruct

bladder neck or even prolapse from

female urethra.

Page 18: Congenital anomalies ppt

Undescended testis

• The incidence ranges from 3.4% to 5.8%

in full term boys

• It decreases to 0.8% in one year old boys.

• Cause is unclear, but

androgens may have an

important role.

Page 19: Congenital anomalies ppt

Undescended testis vs Ectopic

• Undescended testis can be classified by its

location:

1. upper scrotum,

2. superficial inguinal pouch,

3. inguinal canal,

4. abdomen.

• In 80% of cases, the undescended testis will be

palpable in the inguinal canal.

Page 20: Congenital anomalies ppt

Undescended testis vs Ectopic

• Ectopic testis (testis outside path of normal descent) may be:

1. penile,

2. femoral,

3. perineal,

4. retrovesical.

5. Superficial inguinal pouch.

Page 21: Congenital anomalies ppt

Undescended testis-

Complications • Patients with undescended testes have

two major concerns:

1. increased incidence of testicular cancer:

up to 10 times higher than in a normal

testis

2. subfertility.

• Spontaneous descent of undescended

testis is rare after the age of one year.

Page 22: Congenital anomalies ppt

Undescended testis-

• Examination (esp, under anesthesia) is

more accurate than imaging.

• Laparoscopy is the investigation of

choice for non-palpable testes to

differentiate intra-abdominal from vanished

from inguinal testis.

• Imaging: Ultrasound, CT, MRI (inaccurate).

• chromosomal and endocrine evaluation if

bilateral non-palpable testes.

Page 23: Congenital anomalies ppt

Undescended testis

Patent internal ring

Spermatic vessels

Vas

Laparoscopy showing intra-abdominal testis

Page 24: Congenital anomalies ppt

Undescended testis-

Management

• Orchidopexy if testis is felt in inguinal

canal or below, it should be performed as

soon as possible.

• Orchidectomy If an atrophic intra-

abdominal testis is detected especially after

puberty, as the testis is incapable of

spermatogenesis and the risk of

malignancy.

Page 25: Congenital anomalies ppt

Retractile testis

• Commonly confused with undescended

testis.

• the testis can be delivered into bottom of

scrotum.

• low undescended testis will immediately

pop back to its undescended position after

being released.

Page 26: Congenital anomalies ppt

Hypospadias • congenital condition results

in underdevelopment of urethra.

• affects 3 per 1000 male infants.

• Consists of 3 anomalies:

(1) Abnormal ventral opening of the urethral meatus.

(2) Ventral curvature of the penis (chordee).

(3) Deficient prepuce ventrally.

Page 27: Congenital anomalies ppt

Hypospadias- Evaluation

- Site Of the meatus.

- Circumcised or not.

- Associated anomalies:

meatal stenosis, or

undecended testis.

- Penile curvature.

Page 28: Congenital anomalies ppt

Hypospadias- Examples

Page 29: Congenital anomalies ppt

Hypospadias- Treatment

• The child should be referred for urological

assessment and surgical treatment.

• The ideal age for surgery is 6–12 months.

Page 30: Congenital anomalies ppt

Epispadias

• Congenital condition in

which the urethra opens

on dorsal surface of penis..

• Usually associated with bladder extrophy

(ectopia vesicae).

Page 31: Congenital anomalies ppt

Bladder Extrophy (Ectopia vesicae)

• Failure of development of the lower abdominal wall.

• Anomaly include defect in anterior abdominal wall, defect in anterior bladder wall and epispadias (dorsal

penile opening).

Page 32: Congenital anomalies ppt

Posterior Urethral Valves

• Posterior urethral valves are congenital

membranel like structures located in the distal

prostatic urethra.

• most common cause of BOO in male children.

• Diagnosis is made on voiding

cystourethrography (VCUG) &

cystourethroscopy.

Page 33: Congenital anomalies ppt

Posterior Urethral Valves

Treatment

• Fulgration of posterior valves by endoscopy.

• Urinary diversion.

• Management of renal failure.

Page 34: Congenital anomalies ppt

Thank You