1. Congenital anomalies of Renal system

2. Objectives To give an over view of common congenital renal anomalies.
Implications of these anomalies in childhood and beyond.

5. Development Three sets of excretory organs:
Pronephros (rudimentary & non functional)
Mesonephros (Briefly functional)
Metanephros (Permanent) initiated by an interaction between ureteric bud(branch of mesonephric duct) & metanephric mesoderm. Collecting system is formed by branching of ureteric bud.

6. Developmental Abnormalities
Developmental process is disturbed
Normal development
Kidneys arise from mesoderm, develop in pelvis, ascend to final position
Bladder derived from lower end of intestinal tract
Excretory ducts (ureters, calyces, pelvis) develop from ureteric buds that extend from bladder into the developing kidneys

7. Nomenculature Malformation of parenchyma Agenesis/Aplasia Hypoplasia
Dysplasia
Cystic Disease.
Aberrant migration of renal parenchyma
Renal ectopia, Fusion anomalies.

8. Agenesis/Aplasia/Hypoplasia
Due to failure of mesonephric duct to bud.
Bilateral fatal (oligohydramnios) 1 in 3000 births.
Unilateral in 1000 new borns.
compatible with normal life. Other kidney hypertrophies.

9. Multicystic/Polycystic dysplastic kidneys
Failure of ureteric bud derivatives to join the tubules of metanephric mesoderm. Kidneys consists of groups of cysts with minimal parenchyma. 1in 2400 births.

10. Ectopic Kidneys Aberrant migration of renal parenchyma
One or both kidneys may be in an abnormal position.
Pelvic kidney due to failure of kidney to ascend. If both are close to each other---pancake or discoid.
Horseshoe kidney 1 in 500 Lower poles fuse. Usually lies in hypogastrium because they are caught by root of inferior mesenteric A. U shaped.
May remain asymptomatic.

12. Fusion anomalies
Crossed renal ectopia Kidney may cross to the opposite & fuses or remains unfused with contralateral kidney.

16. Abnormalities of development of collecting system.
Pelvi ureteric junction obstruction (Hydronephrosis)
Ureteric
Mega ureter, Ectopic ureter, Duplication, Vesico ureteric reflux.
Urinary Bladder
Exstrophy (ectopia vesicae).
Urethra
Posterior urethral valves, Hypospadias, Epispadias.

17. Hydronephrosis

18. Hydronephrosis

19. Duplication of ureter
Results from division of the ureteric bud or separately from two buds.
Complete.
Incomplete.

21. Ectopic ureter
Results when ureteric bud is not incorporated into the posterior part of urinary bladder. It may open at a place other than in the bladder at two upper angles of trigone. May be a part of duplex system.

22. Ectopic ureter
Males Neck of the bladder, prostatic urethra, seminal vesicle or vas deferens. May cause epididymo orchitis or urinary incontinence. Females Bladder neck, urethra, Vagina or Vestibule.

23. Mega Ureter Larger than normal size ureter.
Obstructive due to some congenital obstruction like stenosis or atresia.
Non obstructive due to vesico ureteral reflux.

24. Mega Ureter

25. Vesicoureteral Reflux
Urine is normally prevented from flowing back into the ureters during micturation by a valve like mechanism at ureteovesical junction.
Failure of mechanisms allows bladder urine to reflux into ureter during voiding.
Predisposes to urinary tract infection
Predisposes to pyelonephritis

26. Vesicoureteral Reflux

28. Urinary Bladder Exstrophy ( Ectopia vesicae) 1 in 40,000 births.
Protrusion of posterior bladder wall due to incomplete medial closure of inferior part of anterior abdominal wall & also involves anterior bladder wall. Results from failure of migration of mesenchymal cells between cectoderm of anterior abdominal wall and cloaca.

30. Posterior Urethral Valves
Most common form of obstructive uropathy leading to renal failure in newborn boys. These leaf like structures arise from the distal portion of verumontanum, course in the anterolateral direction & in more severe cases fuse with the opposite valve in the anterior midline resulting in outlet obstruction

32. Urachal anomalies
Urachal Fistula
Patent Urachus
Cyst
Diverticulum

33. Urethral anomalies
Hypospadias means below & spadon means defect or rent. Incidence is 8.3 per 1000 male births.
It results from failure of fusion of urethral folds on ventral aspect of penis.

34. Urethral anomalies
Epispadias External ure meatus is on the dorsal aspect of the shaft of the penis

35. Take home message
Congenital renal anomalies may present beyond neonatal period.
Bilateral involvement, solitary kidney etc. require urgent evaluation.
Long term follow up of renal & bladder functions are needed.

36. THANK YOU FOR YOUR PATIENCE

37. QUESTIONS