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Anomalies of the lower Urinary Tract Vesicoureteral Reflux Definition: Retrograde flow of urine from the bladder to the upper urinary tract. Etiology of Vesicoureteral Reflux Primary Reflux primary reflux represents a congenital defect in the structure and function of the ureterovesical junction.. Secondary Reflux -Bladder dysfunction e.x neurogenic bladder. -Bladder outlet obstruction e.x posterior urethral valve. Grading of Reflux: by the voiding cystourethrogram GRADE 1 2 3 4 5 DESCRIPTION Into a nondilated ureter. Into the pelvis and calyces without dilatation. Mild to moderate dilatation of the ureter, renal pelvis, and calyces. Moderate ureteral tortuosity and dilatation of the pelvis and calyces Gross dilatation of the ureter, pelvis, and calyces and sever ureteral tortuosity Complication of VUR: -Infectious pyelonephritis: reflux facilitates the ascension of microorganisms from the bladder to the kidneys. -postpyelonephritic scarring: -Hypertension.: VUR is the primary cause of hypertension in children. -Renal Failure: high grade bilateral VUR may cause renal failure by back pressure and infection. 1 Diagnosis of Vesicoureteral Reflux The voiding cystourethrogram (VCUG): the gold standard for reflux detection. Renal ultrasonography: it is used for serial follow-up of renal growth and development. Ultrasonography also images the degree of corticomedullary differentiation in the kidney. Loss of corticomedullary differentiation, or an increase in the overall echogenicity of the kidney, is associated with some degree of renal functional impairment. Renal Scintigraphy: The main test for scar detection. Management of VUR: Medical Management: - Spontaneous resolution of reflux is very common (90% for grade 1, 80% of grade 2 , 50% for grade 3 , 25% for grade 4 and 5% for grade 5). -. The classic approach is daily low-dose prophylactic antibiotic suppression of infections as the first line of treatment under the principle that every case of reflux should be offered time to resolve spontaneously.It is reasonable to wait until approximately 5 years of age assuming no intercurrent breakthrough infections occur. Beyond this age, it is commonly believed that the kidneys become less prone to scarring after pyelonephritis . Endoscopic Treatment of Vesicoureteral Reflux: use of injectable bulking agents to narrow the ureteral orifice, thereby preventing vesicoureteral reflux. Surgical treatment: by ureteric reimplantation,indicated in patient with recurrent breakthrough infections . Posterior Urethral Valves It is mucosal fold at posterior urethra causing varying degree of obstruction to voiding without interfering to retrograde catheterization. The classic posterior urethral valves occur only in males. Pathophysiology: The effects of congenital obstruction of the urethra are reflected in the entire urinary tract above the level of obstruction. -Bladder: hypertrophy and hyperplasia of the detrusor muscle along with increased connective tissue. The bladder neck is rigid and hypertrophied. -prostatic urethra: High voiding pressures distend and thin the prostatic urethra. -Ureter: The ureteral wall is thickened and the lumen massively dilated. -Kidney: renal injury is caused by persistent high pressure. 2 Clinical Presentation: it depend on age of patient and degree of obstruction. In utero: today, most patients with posterior urethral valves are diagnosed with prenatal ultrasound . The observation of marked hydroureteronephrosis, a distended bladder, and a thickened bladder wall and oligohydramnios in utero strongly support the diagnosis of valves . newborn : usually presents with signs of severe systemic illness such as failure to thrive, lethargy, and poor feeding. The infants may be pale and have poor muscle tone. Examination of the abdomen may reveal masses due to hydroureteronephrosis and a distended bladder. Older Children : present with urinary tract infection and difficulty in urination with poor urinary stream. Diagnosis: Ultrasound : The classic ultrasound findings in patients with valves include bilateral hydroureteronephrosis, distended bladder, dilated posterior urethra, and a thickened bladder wall. Voiding Cystourethrography: The voiding cystourethrogram (VCUG) remains the most important study in diagnosing posterior urethral valves because it defines the anatomy and gross function of the bladder, bladder neck, and urethra. Cystoscope: for direct visualize the valve. Laboratory Evaluation: serum Creatinine and blood urea to assess renal function. Management: Bladder Drainage: Initial management of all patients with posterior urethral valves requires the immediate urinary catheter drainage from the bladder. Valve Ablation After successful initial bladder drainage and when the patient’s medical condition has stabilized, the next step is to permanently destroy the valves by using pediatric resectoscope with a hook or cold knife to incise the valves. 3 Hypospadias Hypospadias is considered arrested penile development in which the urethral meatus open on the ventral side of the penis proximal to the tip of the glans usually associated with ventral curvature of the penis and deficient of foreskin ventally. Hypospadias occurred in 1 of 300 males. ETIOLOGY: Unknown: the underlying etiology is unknown for most cases. Genetic Factors: Familial aggregation is found in 10% of hypospadias cases, including first-, second-, and third-degree relatives. Endocrinopathies Defects in the testosterone biosynthetic or androgen receptor may be a cause of hypospadias. Assisted reproduction is associated with an increased hypospadias risk, which has been attributed to hormonal manipulations during and after the procedures. Other Associations low birth weight, preterm birth (<37 weeks), maternal obesity and diabetes, and maternal hypertension. Both decreased (<24 years) and increased (>40 years) maternal age . Classification :according to the location of urethral meatus -glanular -coronal -penile -Penoscrotal -perineal DIAGNOSIS: Hypospadias is diagnosed by physical examination, first suspected by the ventrally deficient prepuce and confirmed by the proximal meatus. ASSOCIATED ANOMALIES Cryptorchidism : approximately 10% of hypospadias patients also have cryptorchidism. Disorders of Sex Development: hypospadias with cryptorchidism increases the likelihood for DSD and need karyotyping (chromosomal assessment). Inguinal hernia : occur in 10% of cases Management. The therapeutic objectives are to correct the penile curvature,, to bring the urethral meatus to the tip of the glans. The age at surgery for hypospadias repair is usually 6-18 months. 4