1. CONGENITAL DEFECTS OF THE BLADDER

2. Ectopia vesicae (exstrophy of the bladder)
MECHANISM
Caused by the incomplete development of the infra-umbilical part of the anterior abdominal wall, with incomplete development of the anterior wall of the bladder.
Clinical features of ectopia vesicae:
One in births (four male: one female)
The presence of the viscera behind it
Edges of abdominal wall can be felt
Umbilicus is absent
In the male
the completely epispadiac penis is broader and shorter than normal,
bilateral inguinal hernia .
the prostate and seminal vesicles are rudimentary,
the testes are normal and usually descended.

3. In female In both sexes, the clitoris is bifid
the labia minora are separated anteriorly, exposing the vaginal orifice.
In both sexes,
there is separation of the pubic bones, which are connected by a strong ligament.
linea alba is broad.
In the rare, incomplete form of penile epispadias or female epispadias, the pubes are united and the external genitalia are almost normal, although in the female the clitoris is bifid.

8. TREATMENT Staged reconstruction:
Iliac osteotomy, closure of the bladder and closure of abdominal wall.
In the first year of life, the bladder is closed following osteotomy of both iliac bones just lateral to the sacroiliac joints.
Later reconstruction of the bladder neck and sphincters is required.
In some patients, the reconstructed bladder remains small and requires augmentation.
One-stage reconstruction is being practiced in some major centers.

9. TREATMENT Urinary diversion:
Less satisfactorily, urinary diversion can be carried out by means of ureterosigmoid anastomosis, an ileal or colonic conduit, or continent urinary diversion.
Long-term complications include:
(1) stricture at the site of anastomosis with bilateral hydronephrosis and infection;
(2) hyperchloraemic acidosis;
(3) an increased (20-fold) risk of tumour formation (adenoma and adenocarcinoma) at the site of a ureterocolic anastomosis.

10. CONGENITAL ANOMALIES OF URETHRA & PENIS

11. CONGENITAL URETHRAL LESIONS
Anatomy
Male urethra is a tubular structure extending from the bladder neck to the external urethral meatus(EUM) at the tip of the glans penis. It is composed of 4 parts:
Prostatic urethra posterior urethra
Membranous urethra

12. Bulbar urethra anterior urethra
Penile urethra
Prostatic urethra contain verumontanum(important landmark).
The external urethral sphincter is composed of striated muscle within the urethral wall .

14. Urethral stricture
rare, may be associated with duplication of urethra.
Symptoms are delayed till adolescence when it may not be differentiated from traumatic urethral injury.
Rx.:
Optical urethrotomy
Urethral dilation.

16. 2. Congenital urethral valves
Anterior urethral valves
rare, cause urethral dilation &diverticulum. They are manifested by UTI, urine incontinence. They are treated by endoscopic fulgration.
b. Posterior urethral valves
congenital mucosal folds located just distal to the verumontanum or within the prostatic urethra. They are flap valves therefore urethral catheterization can be done inspite of reduced urine stream.

18. Clinically, Prenatally diagnosed by U.S.(as hydronephrosis).
2. Recurrent UTI in neonates.
3. Urine incontinence in toddlers with urine dribbling.
4. Delayed presentation in adolescence as urinary tract obstruction ,hydroureteronephrosis &renal failure.

19. Investigations:
U.S. , IVU, MCUG( it shows the valve folds, the trabeculated bladder with diverticulae & even vesicoureteral reflux).
Cystourethroscopy confirm the Dx.
Rx.:
Endoscopic valve resection.
Pt. with renal failure & hydrureteronephrosis need vesicostomy till stabilizing renal function then endoscopic valve ablation.

21. Hypospadias incidence : 1 in 300
the commonest congenital urethral disease.
Definition: the external urethral meatus(EUM)opens on the ventral side of the penis prximal to the tip of the glans penis or on the scrotum or perineum.
There may be poorly developed ventral part of the prepuce( hooded prepuce).
There may be ventral penile curvature(chordee).

23. Aetiology Classification
Congenital incomplete fusion of the urethral folds.
Esrogens & progestins given in prgnancy increase its incidence.
Classification
According to the position of the meatus:
Glanular hypospadias
the commonest type.
2. Coronal hypospadias
the EUM lies on the ventral side of the junction of the glans & penile shaft.

24. Rx. 3. Penile & penoscrotal hypospadias 4. Perineal hypospadias
the most severe type associated with bifid scrotum, maldescended testes & difficult sex determination.
Rx.
The glanular type needs no Rx. Unless the meatus is stenosed which needs meatotomy, however surgery is needed for the other types.
Time of surgery: montha of age.

26. Aim of surgery
improve sexual function.
Improve urine stream.
Cosmotic reasons.
Steps of surgery:
Orthoplasty(correction of penile chordee).
Urethroplasty(correction of the site of the EUM).
Glanuloplasty(correction of the shape of the glans).
There are many types of operations for hypospadias, most of them use the prputial skin therefore circumcision should be

27. Delayed till hypospadias repair succeeded.
4. Epispadias
very rare. The EUM lies on the dorsum of the penis & associated with upward curvature of the penis.
It might coexists with bladder exstrophy & other severe congenital defects.

29. 5. Diverticulum of male urethra
Congenital partial duplication of the urethra. It is uncommonly acquired (where it is due to increased intraurthral pressure behind a urethral stricture or due to the lonstanding presence of a stone in the urethra).
It is treated by excision & treating the cause.

30. CONGENITAL ANOMALIES OF THE PENIS
Phimosis
a scaring prepuce which becomes tight & cant be retracted over the glans.
It can cause difficult urination or may hide a stenosed meatus, so it can cause back pressure effect on the ureters& kidneys. It should be differentiated from the physiologic adhesions between the the foreskin & glans.

32. It is caused by chronic infection under the prepuce with poor local hygiene & may arise because of balanitis xerotica obliterans( a thickened foreskin which doesn’t retract over the glans).
Rx. : circumcision.
Indications of circumcision:
Religious or cultural habits.
Phimosis & paraphimosis.
Recurrent UTI or balanoposthitis
4. Obstruction of urine flow.
5. In adults for inability to retract the prepuce for intercourse.

34. 2. Paraphimosis
A tight retracted foreskin that act as a ring & is difficult to return back normally over the glans. It is caused by chronic infection under the prepuce . This ring causes venous & lymphatic obstruction of the glans which becomes swollen & this will further increase the pressure effect of the ring.
Rx.:
Gentle manual squeezing of the glans+ icebags.
Circumcision(if the first step fails).

35. Dorsal slitting of the prepuce under lacal anaesthesia