DISEASES OF URINARY SYSTEM

CONGENITAL ANOMALIES
OF KIDNEY
 1. Unilateral aplasia of hypoplasia
 2. Renal ectopia
 3. Duplication
Unilatera aplasia or hypoplasia
 A. Complete absence of kidney with absence of ureter
 B. Ureter & pelvis present with absent kidney(contralateral kidney is
hypertrophied)
 C. Grossly atrophic kidney
Renal ectopia
 A) Pelvic kidney
 B) Horseshoe kidney
 C) Crossed dystopia (unilateral fusion)
 D) Polycystic kidney
Pelvic kidney
 One kidney fail to ascend & remain in

the pelvic brim (pelvic kidney)

 Contralateral kidney is in its normal position.
 Renal artery arises from common illiac artery.
 Sometimes acute renal diseases may be misdignosed due to pelvic kidney &
during exploration kidney may be removed suspecting unexplained pelvic mass.

Horse shoe kidney



Two kidneys are fused at

their lower poles, giving a shape like horse shoe

  Commonly occurs in men

 Fusion of upper pole is uncommon

 Lower poles are fused in front of L4
 Angulated ureters behave like pelvi-urteric junction obstruction leading to urinary
stasis, secondary infection & nephrolithiasis.

Crossed dystopia (unilateral fusion)

 Both kidneys are in one loin & usually fused
 Ureter of lower kidney crosses midline to enter bladder on contralateral side

 Unilateral long kidney – both renal pelvis lie one above each other

 Unilateral S-shaped kidney – pelvis of crossed kidney faces laterally

Polycystic kidney
  Hereditary
 More in women than men
 Clinical manifestation usually after the age of 30
Pathology
 Enormously enlarged kidneys
 Cysts appears like collection of bubbles below renal capsule
 Cyst may contain clear fluid or thick brown material or coagulated blood
 18% cases associated with congenital cystic liver diseases, occasionally pancreas
& lungs may be affected
Clinical features
 Irregular upper quadrant abdominal mass
 Pain in loin
 Heamaturia
 Hypertension
 Infection
 Ureamia
Signs & symptoms
  Large volumes of urine of low specific gravity containing traces of albumin.
 Chronic renal failure coz functioning renal tissues are replaced by cysts
 Anorexia,headache,vague abdominal discomfort.drowsiness & vomitting due to
biochemical derangement
 Severe anaemia & signs of ESRF
Treatment
 Renal replacement by dialysis
 Renal transplantation
 Rovsing’s operation- rarely done. deroofing of cysts or uncaping of cyst to relieve
the pressure on parenchyma
Duplication

 A) Duplex kidney
 B) Duplex renal pelvis
 Common, asymptomatic, harmless condition
 Associated with duplex ureter
  May be associated with abnormalities of insertion of ureter into bladder, leading
to reflux or incontinence of urine
Duplex renal pelvis
 Found in 4% patients
 Usually unilateral,more common on left side
 Associated with duplication of ureters
 Ureters may open independently into bladder or may be fused in lower third of
their course before entering into bladder
CONGENITAL ANOMALIES OF URETER
 A) Duplication of ureter
 B) Congenital Megaureter
 C) Post caval ureter
 D) Ureterocele
Duplication of ureters
 Usually two ureters join before they reach the bladder, in lower third of their
course & have common ureteric orifices
 Less commonly, ureters open independently into bladder
Clinical features
 Infection, calculus formation & PUJ obstruction are more common
 Failure of normal valvular mechanism at UVJ leads to reflux & damage to renal
parenchyma

 In case of females, if ectopic ureter opens into urethra below the sphincter causes
dribbling of micturation or incontinence
 In case of males, no incontinence due to external urethral sphincter. But if it opens
at the apex of trigone, posterior urethra, seminal vesicle or in ejaculatory duct,
then it is likely to be functionally abnormal & infection is more common.
Treatment
 Asymptomatic dupliction needs no treatment
 In case of reflux causing hydronephrosis, excision or ectopic ureter
 In incontinence, implanting ectopic ureter into bladder or by joining it to its
fellow
Congenital megaureter
 Uncommon
 May be bilateral,associated with other congenital anomalies
 Functional obstruction at lower end of ureter leads to progressive dilatation &
tendency to infection
 T/t- refashioning of lower end of affected ureter
Post caval ureter
 Instead of lying right to inferior vena cava, right ureter passes behind inferior
vena cava causing obstructive symptoms
 T/t – division & rejoining in front of inferior vena cava
 Unusually there may be fibrosis of retro caval portion of the ureter
 T/t - excision
Ureterocele
 Cystic enlargement of intramural portion of the ureter, which is supposed to be
the result of congenital atresia of ureteric orifice
  Most common in women
 Remains unrecognized until adult life
 Adder head on excretory urography is typical
 Occasionally may cause obstruction to bladder outflow
 Diagnosis by cystoscopy-reveals translucent cyst emerging from ureter
 T/t –
 Endoscopic diathermy incision for symptomatic ureterocele
 In advanced cases causing hydronephrosis or pyonephrosis nephrectomy is
advisable

Congenital anomalies of bladder

 Ectopia vesicae (exstrophy of bladder)
this is due to complete ventral defect of urogenital sinus & the overlying skeletal
system, in which anterior wall of bladder & infraumbilical part of anterior abdominal
wall fail to develop, also overlying muscles & bone fail to develop.
  There is big spherical defect in anterior abdominal wall below umbilicus which is
occupied by inner surface of posterior wall of bladder
 Mucosal edges fuse with skin
 Urine spurts on abdominal wall
 The rami of pubic bones widely separated
 Epispadis is almost always associated
Clinical features
 Males more than females(4:1)
 Posterior wall of bladder protrudes through the defect which is red in colour
 Everted mucous membrane becomes ulcerated & painful frequently & may
udergo metaplastic changes froming adenocarcinoma
Treatment
 Surgical correction between 4 to 6 yrs of age
 At first diversion of urine into sigmoid colon
 Followed by excision of bladder & closure after few mnths
 Although causes recurrent infection & death of pt. ultimately