Congenital Anomalies Of The Lower Urinary System dr,mohamed fawzi alshahwani
Hypospadias Urethra normally opens at the tip of the glans penis, & the penis is straight during erection. In hypospadias the external urethral meatus opens on the under surface of the penis proximal to the tip of the glans penis. The missing part of the urethra is replaced by a fibrous band causing ventral curvature (cordee).
Types According to the position of the opening. Glandular. Coronal. Penile: distal, penile shaft or proximal. Penoscrotal. Perineal. The scrotum is bifid (inter sex).,
Hypospadias is due to failure of fusion of urethral folds.
Important clinical points The abnormal hooded prepuce shouldn’t be circumcised to be used for later reconstruction. Increased incidence of undescended testes. Meatal stenosis usually present may result in repeated UTI.
HYPOSPADIAS: meatal stenosis
Treatment For psychological reasons hypospadias should be repaired before school age. Repair traditionally in the first 2 years
Stages of operations Orthoplasty: straitening of the penis. Urethroplasty: formation of neourethra MAGPI. Flip-flap. Mathieu.
Complications Stricture. Fistula. Failure of operations.
ECTOPIA VESICAE (BLADDER EXTROPHY)
Bladder Extrophy (Ectopia Vesicae) Rare anomaly 1:50,000. affects males more. Absent anterior wall of the bladder. Absent anterior abdominal wall from the umbilicus downward. Widening of symphysis pubis causing waddling gate. Associated epispadias with short & wide penis.
Pelvic X ray: pubic diastasis
EPISPADIAS
Undescended testis: Normally the testis developed in the abdomen under the kidney, then descends down to the scrotum through inguinal canal just before birth, guided by gubernaculum under hormonal control. Undescended testis referred to testicular arrest during descent in it’s path to the scrotum. .
facts: It affects 1% of all males. In 20% bilateral. Right side more than left side. Incidence is higher in premature infants. Associated with inguinal hernia in 75%. Testicular descent may be completed at first year of life.
Types: Intra abdominal. Intra-canalicular. At external ring. At scrotal neck.
Pathophysiology: *Lower temp is required for spermatogenesis. *Hormonal function remains normal. secondary sex characters develop normally.
Differential diagnosis: of absent testis Anorchia: testicular vanishing syndrom. Retractile testis: normal phenomenon in young children due to strong cremasteric reflex, the scrotum is well developed & the testicle can be brought down to the bottom of the scrotum, examin at squatting position. no treatment is required. Orchedectomy Ectopic testis Undescended testis:
Complications: Sterility in bilateral cases. Torsion of the spermatic cord. High liability for malignancy. Liability for trauma and infection. Associated hernia complications. Psychologic disturbance.
Investigations: Hormonal assay to exclude anorchia. U/S & CT scan for localization. Laparoscope.
Treatment: Hormonal: Surgical: ( orchidopexy): Beter to be done befor two years old. Staged orchiopexy. Laparoscopic orchiopexy. For intra abdominal Orchiectomy: Specially in adulthood.
Sub dartous pouch fixation
Ectopic testis The position of the testis is outside the normal pathway of the testicular descent. Penile root. Femoral triangle. Crossed ectopia. Perineal.
Treatment Replacement the testicle to the scrotal sac for proper development.
Posterior urethral valve The most common obstructive urethral lesion in infants and newborns, occures only in males and are found at the distal prostatic urethra. The valves are mucosal folds (thin membrane).
PUV may cause varying degrees of obstruction when the child attempts to void mild, moderate or severe. BOO result in retention and variable degrees of hydronephrosis.
MCUG
Cystoscope: is diagnostic by direct visualization of the valve and therapeutic to destruct the valve by electrocautary.