congenital abnormalities

1. Congenital Abnormalities Of the Genitourinary System Dr.V.Kasiviswanathan MS Formerly Sr.DMO / S.Rly. / Palakkad

13. The most frequent congenital defects and abnormalities of the genitourinary tract are hydronephrosis, undescended testicles (cryptorchidism), hypospadias and epispadias

14. Absence of one kidney Congenital aplasia Failure to develop one kidney Can be found during ulatrasound examination, CT scanning and Pyelogram studies Ureter absent No ureteric orifice found during cystoscopy Or ureter and renal pelvis are present but the kidney absent

15. Absent left kidney

16. CT Urography CT Urography has almost completely replaced conventional excretory urography, popularly called IVU. With the current multi-slice scanner, especially 64-slice CT scanners, it is possible to obtain high-quality images of the kidneys, ureters and bladder, Angiographic, parenchymal and excretory phases.

17. Renal Ectopia Kidney does not ascend Usually near the pelvic brim ; usually left If it is not symptomatic the only problem is that during the abdominal operations the pelvic kidney should not be mistaken for any abnormal tumour and be injured

18. Renal ectopia – pelvic kidney

19. Horseshoe Kidney Situated usually in front of fouth lumbar vertebra Fused lower poles common Ureters angulated Infection Nephrolithiasis Fixed mass below umbilicus

20. Horseshoe Kidney

21. Horseshoe Kidney - pyelogram

22. Horseshoe Kidney – CT scan

23. Horseshoe Scintigram

24. Horse shoe kidney

25. Unilateral Fusion Both kidneys are in one loin Usually fused Ureter of the lower kidney crosses the midline to enter the bladder on the contralateral side. Both renal pelves may lie one above each other medial to the renal parenchyma(unilateral long kidney - or the pelvis of the crossed kidney faces laterally (unilateral S-shaped kidney)

26. Crossed fused kidneys

27. Congenital cystic kidneys – polycystic kidneys Hereditary Autosomal dominant trait Not usually detectable until the second or third decades of life and never manifests before the age of 30 Irregular upper quadrant mass Loin pain Haematuria Infection Hypertension Uraemia CT image : multiple cysts in both kidneys

28. Bilateral polycystic kidneys ct

29. Polycystic kidney

30. Simple Renal Cyst Common Multiple Diagnosed on ultrasound Rarely require treatment Treat only if causing obstruction

31. Renal Cyst

32. Aberrent renal vessels Two or more renal arteries are most common on the left Functional end arteries – infarction if divided Veins can be divided because they have collaterals

33. Normal Renal Arteries

34. Two Right Renal Arteries

35. Congenital abnormalities of the renal pelvis and ureter Duplication of a renal pelvis Common Usually unilateral

36. Duplication of a ureter The ureters usually join before they reach the bladder Less commonly the ureters open indepedently into the bladder Congenital megaureter

37. Duplication of the ureter

38. Congenital defects of the bladder Ectopia vesicae – exstrophy of the bladder Easily recognised at birth Umbilicus absent, protruding due to the intraabdominal pressure In addition epispadias Mons and clitoris bifid In the neonate the bladder should be covered with Saran Wrap or clingfilm to prevent trauma to the delicate mucosa

39. Exstrophy of the bladder

40. Congenital abnormalities of the urethra and penis Meatal stenosis Congenital stricture Congenital valves Hypospadias epispadias

41. Meatus Congenital stenosis of the external urethral meatus – normally the narrowest part of the male urethra Associated with phimosis – at times pin hole meatus Back pressure effects Spraying, dribbling

42. Congenital Urethral Stricture Rare

43. Congenital valves of the posterior urethra Folds of urothelium Obstuction in boys Within prostatic urethra Catheter will pass easily Micturating cystourethrogram Pass catheter

44. Posterior Urethral Valve

45. Micturating cystourethrogram

46. Posterior Urethral Valve with proximal dilatation of the prostatic urethra

47. Hypospadias Most common urethral abnormality Glandular hypospadias Coronal hypospadias Penile and penoscrotal hypospadias Perineal hypospadias Avoid circumcision

48. Normal External Urethral Meatus

49. Hypospadias types

50. Hypospadias

51. Abnormalities of the testes and scrotum Incompletely Descended Testis Testis is not present in the scrotum In about 4 % of all newborns 50% descend during the first month of life The genitals all newborns must be examined May be associated with inguinal hernia Should be corrected well before puberty Otherwise atrophies

52. Various Positions of the incompletely descended testis

54. Retractile Testis Sometimes the testis intermittently disappears upwards. This phenomenon is called 'retractile testis'. wait for the boy to grow careful followup if the testis prefers to stay higher or if the testis is under tension when brought down, surgical correction is recommended.

55. Ectopic testis The sites of ectopic testes are At the superficial inguinal ring. In the peruneum At the root of the penis In the feroral ring

56. Phimosis At birth foreskin adherent to the surface of the glans penis Separate spontaneously with time Can wait for 4 years to separate Gentle retraction at bath permitted Forcible retractions injure

57. Phimosis