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congenital abnormalities

. Let us have a review of the normal anatomy of the urinary system before having a knowledge of the congenital abnormalities. . . . . . . . . . . The most frequent congenital defects and abnormalities of the genitourinary tract are hydronephrosis, undescended testicles (cryptorchidism), hypospadias and epispadias.

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congenital abnormalities

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    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

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