Urological Congenital Anomalies Dr. TAMER M. ABD ELRHMAN
Congenital anomalies of the kidney • 1) Anomalies of Number: - agenesis (unilateral or bilateral). - super-numerary kidney. • 2) Anomalies due to abnormal fusion: - Sigmoid and L-shaped kidney. - Discoid shaped kidney. - Horse-shoe kidney (commonest). • 3) Anomalies due to failure of communication: - solitary renal cyst. - polycystic kidney. • 4) Anomalies due to failure of migration: - ectopic kidney
anomalies of the kidney • 5) Anomalies in size: - hypo-plastic kidney. - hypertrophied kidney. • 6) Anomalies of the renal pelvis: - bifid (double) pelvis. - pelvi-uriteric j. obstruction. • 7) Anomalies of renal vessels: - renal artery stenosis, Aneurysm. - aberrant renal artery.
Congenital anomalies of ureter • - single ureter. • - double ureter (common). • - post-cavalureter. • - congenital mega-ureter. • - ureterocele. anomalies of urinary bladder • 1- Ectopia vesica (exstrophy). • 2- Congenital anomalies of urachus: ( urachal diverticulum, cyst,sinus,fistula). • 3- Congenital diverticulum. • 4- Septate, Bipartite & Lobulaled bladder.
anomalies of urethra • - Phimosis,Paraphimosis. • - Meatalstenosis. • - Urethral values. • - Congenital urethral diverticulum. • - Hypospadius, Epispadius.
Solitary Cyst of the Kidney • Etiology • Localized polycystic kidney Not communicating with pelvi-calyceal system. • Localized hydronephrosis Related to calyces, communicating with pelvis. • Pathology:- cyst Contains fluid, Lined by epithelium, Surrounded by a fibrous tissue & compressed renal tissue. • Clinical picture: • dull aching pain in the loin, a swelling may be felt in the loin. • Investigations IVP Smooth amputation of a calyx, Localized smooth spider leg appearance. Renal angiography, U\S. • Treatment: Deroofingoperation (Rovesing operation). Partial nephrectomy.
Polycystic Kidney • Etiology Failure of fusion between metanephros & mesonephros retention cysts. It might be a part of cystic changes of the body. • Pathology kidney is enlarged with multiple cysts. Cysts are not intercommunicated & are not connected to renal pelvis. Cysts compress renal tissue atrophy. • Clinical picture Obstructed labor Still birth. Infantile type vitD resistant rickets. Adult type: • Renal symptoms Renal pain, renal swelling, polyuria, haematuria, uremia. • Cardiovascular symptoms Renal hypertension, heart failure. • Investigations IVP Bilateral smooth spider leg appearance. • Treatment Rovsing operation. Nephrectomy if renal transplantation is possible.
Hypospadius • A congenital anomaly of the urethra in which the urethra open on the under surface of penis or perineum. Types • Glandular :- EUM opens on under surface of the glans. • CORONAL . • Penile :- failure of fusion of inner uretheral folds EUM opens on under surface of the penis, distal part of urethra is replaced by fibrous band (urethral cordae). It may be: anterior,mid or posterior penile. • Penoscrotal, SCROTAL. • Perineal:- failure of development of whole penile urethra, The scrotum is splitted, The urethra opens between its 2 halves, The penis is rudimentary.
Hypospadius 2 Complications • Psychological trouble. • Bowing during erection. • Sterility. Treatment • circumcision should be avoided in all cases. • plastic surgery can be performed at the age of one year • the aim is to release chordee and a new urethra is fashioned using neighboring skin from the prepuce or the penile skin. • if glandular hypospadiusmagpi operation. • if penile hypospadiusdukette operation. • if perineal mucosal bladder flap operation + phaloplasty.