Congenital abnormities of kidney ad ureter 30 3-10


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lecture by Dr. Ahmed Rehman

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Congenital abnormities of kidney ad ureter 30 3-10

  1. 1. Dr Ahmed Rehman FCPS Urology Assistant Professor Urology 30-march, 2010, tuesday
  2. 2. Learning Objectives <ul><li>Enlist congenital abnormalities of kidney & ureter </li></ul><ul><li>Describe clinical significance of these abnormalities, ie </li></ul><ul><ul><li>What problems these can cause (symptoms) </li></ul></ul><ul><ul><li>What are risks to health ( complications) </li></ul></ul><ul><li>Enlist diagnostic investigation plan– history, examination, investigations </li></ul><ul><li>Suggest treatment plan in these conditions </li></ul>30-march, 2010, tuesday
  3. 3. Congenital abnormalities of kidney <ul><li>Uncommon ( <1:1000) </li></ul><ul><li>Commonly Symptomless – found incidentally – US,IVU, CT. Why ???? </li></ul><ul><li>sometimes detected only when have caused sufficient damage </li></ul><ul><li>Endanger kidneys to various complications </li></ul>30-march, 2010, tuesday
  4. 4. Agenesis / aplasia of kidney <ul><li>Bilateral – fatal </li></ul><ul><li>Unilateral – compatable with normal life, contralateral kidney hypertrophed </li></ul><ul><li>Failure of mesonephric duct to bud </li></ul><ul><li>Ureteric orifice absent on cystoscopy </li></ul><ul><li>Rarely ureters & pelvis may be present but renal tissue is absent or so </li></ul>30-march, 2010, tuesday
  5. 5. Hypoplasia-dysplasia 30-march, 2010, tuesday
  6. 6. Renal Ectopia / pelvic kidneys <ul><li>Doesn't ascend , formed near pelvic brim </li></ul><ul><li>No symptom </li></ul><ul><li>Present with pain or mass which one may tempt to remove as unexplained pelvic mass </li></ul><ul><li>pose diagnostic problems in case of disease or surgery </li></ul><ul><li>May be source of stone, infection </li></ul><ul><li>Liable to trauma </li></ul>30-march, 2010, tuesday
  7. 7. 30-march, 2010, tuesday
  8. 8. Crossed Ectopia / Crossed Dystopia <ul><li>Both kidneys lie in one loin </li></ul><ul><li>May be fused with each other or separate </li></ul><ul><li>Ureter of lower crosses midline to open into bladder on its normal side </li></ul>30-march, 2010, tuesday
  9. 9. Crossed Ectopia contrasted with normal IVU 30-march, 2010, tuesday
  10. 10. Mal rotated kidneys <ul><li>Calyces face anteriorly or antrolaterally </li></ul><ul><li>Have some element of obstruction causing inadequate drainage – leading to infection & stone formation </li></ul>30-march, 2010, tuesday
  11. 11. Horse shoe kidney <ul><li>low lying – ascent impeded by inferior mesenteric artery </li></ul><ul><li>Lower poles fused in mid line in front of 4 th lumber vertebra.(isthemus). </li></ul><ul><li>Longitudinally lie medially and downwards, </li></ul><ul><li>instead of laterally and down wards, </li></ul><ul><li>Part or whole of pelvicaliceal system is malrotated ( facing medially), </li></ul><ul><li>Ureters curve over fused poles. </li></ul>30-march, 2010, tuesday
  12. 12. Horse shoe kidney <ul><li>Pain, hematuria, fever, </li></ul><ul><li>mass </li></ul><ul><li>Exam : fixed mass below umbilicus </li></ul><ul><li>Diagnosis: US & IVU </li></ul><ul><li>Significance: Liable to disease </li></ul><ul><ul><li>angulated ureters + PUJ obstruction  urinary stasis  stones, infection & obstruction  CRF </li></ul></ul>30-march, 2010, tuesday
  13. 13. Horse shoe kidney <ul><li>Treatment </li></ul><ul><ul><li>Asymptomatic = nothing doing </li></ul></ul><ul><ul><li>Mild sypmtoms = treat accordingly </li></ul></ul><ul><ul><li>PUJ or ureteric obstruction, recurrent infections, Stones  surgery ( pyelolithotomy + /-reconstruction) ISTHEMECTOMY with straightening of ureters – less commonly done. </li></ul></ul>30-march, 2010, tuesday
  14. 14. Polycystic kidneys <ul><li>Hereditary – autosomal dominant </li></ul><ul><li>Not manifested before 30 </li></ul><ul><li>Kidneys enlarged, studded with cysts </li></ul><ul><li>Unyeilding capsule compresses renal parenchyma causing atrophy </li></ul><ul><li>Liver,lungs and pancreas may be affected </li></ul><ul><li>Defact : not clear, many theories </li></ul>30-march, 2010, tuesday
  15. 15. Polycystic kidneys <ul><ul><li>Loin pain- weight dragging upon peddicle or capsule stretch, hemorhage in cyst, stone </li></ul></ul><ul><ul><li>abdominal mass- confused with cystic tumor </li></ul></ul><ul><ul><li>hematuria- cyst rupture in pelvis,moderate, episodic. </li></ul></ul><ul><ul><li>hypertention, infection, & uremia/CRF. </li></ul></ul><ul><ul><li>Nonspecific symptoms: anorexia, headache, vague abdominal discomfort,  vomiting, drowsiness, anemia. </li></ul></ul><ul><ul><li>ESRD: suddenly in middle age, survival without RRT ( dialysis/ transplant) unlikely </li></ul></ul>30-march, 2010, tuesday
  16. 16. 30-march, 2010, tuesday
  17. 17. Polycystic kidneys <ul><li>US and CT: </li></ul><ul><ul><li>cysts in kidneys,liver & others </li></ul></ul><ul><ul><li>Simple (aquired) cysts: solitary, smooth walled & homogeneous contants </li></ul></ul><ul><ul><li>Blood & debris – cystic adenocarcinoma </li></ul></ul><ul><ul><li>FNA- cytology – differentiates </li></ul></ul><ul><li>IVU </li></ul><ul><ul><li>Enlarged renal shadow, </li></ul></ul><ul><ul><li>Renal pelvis – compressed & elongated </li></ul></ul><ul><ul><li>Calyces – narrow, stretched over cysts ( spider legs / bell shaped) </li></ul></ul>30-march, 2010, tuesday
  18. 18. Polycystic kidneys <ul><li>Nephrologist : </li></ul><ul><ul><li>BP control, infection,anemia, disturbances of Ca metabolism, low protein diet to delay need for DIALYSIS </li></ul></ul><ul><li>Urologist : </li></ul><ul><ul><li>Surgical / laproscopic deroofing of cysts ( Rovsing’s operation) </li></ul></ul><ul><ul><ul><li>Relieves pain & pressure  saving kidneys </li></ul></ul></ul><ul><ul><ul><li>Rarely performed / not preserve function </li></ul></ul></ul><ul><ul><li>Renal transplant/ pretransplant bilateral native kidneys nephrectomy </li></ul></ul>30-march, 2010, tuesday
  19. 19. solitary / Simple (acquired) renal cysts <ul><li>Common, may be multiple ( not always 1 ) </li></ul><ul><li>Incidentally found– no treatment needed </li></ul><ul><li>Rarely symptomatic; pain- stretch, bleed in cyst, infection,mass </li></ul><ul><ul><li>papa-pelvic cyst at hilum presses PUJ  obs </li></ul></ul><ul><li>IVU: filling defect. </li></ul><ul><li>US, CT :smooth, homogeneous contant </li></ul><ul><ul><li>DD : hydatid, cystic adenocarcinoma </li></ul></ul><ul><li>Percuteneous FNA Cytology </li></ul><ul><li>Treat only in case obstructing </li></ul>30-march, 2010, tuesday
  20. 20. Infantile polycystic kidneys <ul><li>Rare </li></ul><ul><li>Inheritance- autosomal recessive </li></ul><ul><li>Enlarged kidneys – may obstruct labour, Many stillborn </li></ul><ul><li>Die of renal failure in in early childhood </li></ul>30-march, 2010, tuesday
  21. 21. Unilateral Multicystic kidney <ul><li>More common </li></ul><ul><li>Presents as nonfunctioning mass </li></ul><ul><li>Exploration & removal is treatment of chioce </li></ul><ul><li>DD: wilm’s, neuroblastoma, congenital hydronephrosis </li></ul>30-march, 2010, tuesday
  22. 22. Aberrant vessels <ul><li>Variation in no of vessels – common </li></ul><ul><li>Arteries – END arteries , damage ischemia </li></ul><ul><li>Veis – extensive colaterals = can be ligated </li></ul><ul><li>are not cause of hydronephrosis- though the bulging renal pelvis in between them makes them noticeable </li></ul>30-march, 2010, tuesday
  23. 23. Abnormalities of renal pelvis and ureter <ul><li>Most common, harmless, asymptomatic </li></ul><ul><li>DUPLICATION OF RENAL PELVIS </li></ul><ul><ul><li>Common,4%, usually unilateral – left </li></ul></ul><ul><ul><li>Upper pelvis – small, drains upper calyx </li></ul></ul><ul><ul><li>Asymptomatic no treatnment </li></ul></ul><ul><ul><li>If one moity severly damaged – partial nephrectomy </li></ul></ul>30-march, 2010, tuesday
  24. 24. Duplex kidney, double moiety 30-march, 2010, tuesday
  25. 25. DUPLICATION OF URETER . 3% <ul><ul><li>Often join before reaching bladder, suffer obstruction ( esp from stones) & YOYO reflux </li></ul></ul><ul><ul><li>May open independently, ureter from upper moity opens distal and medial to its fellow. </li></ul></ul><ul><ul><li>Uppermoity ureter suffers ureterocele . </li></ul></ul><ul><ul><li>Lower moity ureter suffers VUR </li></ul></ul><ul><ul><li>Infection, calculus formation, PUJ obst and VUR, ectopic opening </li></ul></ul>30-march, 2010, tuesday
  26. 26. Abnormalities of ureter Ectopic uretric opening <ul><ul><li>Female – into urethra below sphincter on vagina = incotinance since childhood with desire and passage of urine normally as well. </li></ul></ul><ul><ul><ul><li>IVU and cystoscopy ( indigocarmine) </li></ul></ul></ul><ul><ul><li>Male.continent as opening is above sphincter </li></ul></ul><ul><ul><ul><li>Opening in trigone apex, post. Urethra, seminal vesical or ejaculatory duct – functionally abnormal, infection common </li></ul></ul></ul><ul><ul><li>Treatment: </li></ul></ul><ul><ul><ul><li>Frequently ectopic ureter drains hydronephrotic, chronically infected moity --- best excised - nephrectomy </li></ul></ul></ul><ul><ul><ul><li>Incontinence can be cured and renal function preserved by implanting ureter into bladder ( tunneling) or joining its fellow . (URETERO-NEOCYSTOSTOMY, URETERO-URETEROSTOMY) </li></ul></ul></ul>30-march, 2010, tuesday
  27. 27. 30-march, 2010, tuesday
  28. 28. Abnormalities of ureter CONGENITAL MEGA URETER <ul><ul><li>Uncommon, bilateral </li></ul></ul><ul><ul><li>FUNCTIONAL obstruction at lower end  dilatation & infection </li></ul></ul><ul><ul><li>Ureteric orifices normal, ureteric cath passes easily </li></ul></ul><ul><ul><li>Reflux not feature till opened endoscopically </li></ul></ul><ul><ul><li>Treatment: refashioning and tunneled reimplant </li></ul></ul>30-march, 2010, tuesday
  29. 29. Abnormalities of ureter POST (retro) CAVAL URETER <ul><ul><li>Right ureter passes behind IVC instead of lying to the right of it (laterally) </li></ul></ul><ul><ul><li>If causing obstruction, can be devided and joined in front of cava – long oblique ETE anastomosis </li></ul></ul>30-march, 2010, tuesday
  30. 30. Abnormalities of ureter URETEROCELE <ul><ul><li>Cystic enlargement of intramural portion of ureter </li></ul></ul><ul><ul><li>Thought to result from congenital atresia of ureteric orifice </li></ul></ul><ul><ul><li>Though present since childhood, unrecognised till adulthood </li></ul></ul><ul><ul><li>More common in female, cause BOO by obstructing / prolapsing into internal urinary meatus. May even prolapse out of urethra </li></ul></ul>30-march, 2010, tuesday
  31. 31. <ul><ul><li>Adder head on IVU </li></ul></ul><ul><ul><li>Cyst wall composed of urothelium only </li></ul></ul><ul><ul><li>confirmed on cystoscopy </li></ul></ul><ul><ul><ul><li>Translucent cyst, enlerging and collapsing as urine flows </li></ul></ul></ul><ul><ul><li>Treatment avoided unless symptoms of infection / stone </li></ul></ul><ul><ul><li>Endoscxopic diahermy incision / deroofing </li></ul></ul><ul><ul><ul><li>Postoperatove MCUG to see VUR </li></ul></ul></ul><ul><ul><li>Ureteral reimplant </li></ul></ul><ul><ul><li>Sever hydronephrosis, pyonephrosis  nephrectomy </li></ul></ul>30-march, 2010, tuesday
  32. 32. Hydronephrosis <ul><ul><li>Aseptic dilatation of pelvicaliceal system due to complete or partial obstruction . </li></ul></ul><ul><ul><li>Unilateral hydronephrosis </li></ul></ul><ul><ul><ul><li>Epsilateral ureteric obstruction </li></ul></ul></ul><ul><ul><ul><li>( unilateral supravesical obstruction) </li></ul></ul></ul>30-march, 2010, tuesday
  33. 33. Hydronephrosis: Bilateral <ul><ul><ul><li>Bilateral ureteric obstruction ( bilateral supravesical obstruction ) </li></ul></ul></ul><ul><ul><ul><ul><li>Urethral obstruction ( bladder outlet obstruction , infravesical obstruction ) </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Detrusor hypertrophy  intramural ureteric obstruction </li></ul></ul></ul></ul><ul><ul><ul><ul><li>VUR </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Pregnancy –physiologic dilatation - progesterone, early pregnancy – 20 weeks marked  reverts 12 week of delivery </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Infection, diagnostic difficulty in acute abdominal pain in pregnancy </li></ul></ul></ul></ul>30-march, 2010, tuesday
  34. 34. Causes of Ureteric Obstruction <ul><li>Extramural </li></ul><ul><ul><ul><li>Tumors of cervix, ovary, uterous, vagina, urinary bladder, prostate, rectum, colon, caecum & lymphomas </li></ul></ul></ul><ul><ul><ul><li>Idiopathic retroperitoneal fibrosis </li></ul></ul></ul><ul><ul><ul><li>Retrocaval ureter </li></ul></ul></ul><ul><ul><ul><li>Pararenal cysts </li></ul></ul></ul><ul><ul><ul><li>Aberent vessels </li></ul></ul></ul><ul><li>Intraluminal </li></ul><ul><ul><ul><li>Calculus, sloughed papilla, clot, ureteric malignancy </li></ul></ul></ul><ul><li>Intramural </li></ul><ul><ul><ul><li>Congenital PUJ obstruction or stenosis </li></ul></ul></ul><ul><ul><ul><li>Ureterocele and congenital small ureteric orifice </li></ul></ul></ul><ul><ul><ul><li>Strictures ( stone, repair, tuberculosis, schistosomiasis) </li></ul></ul></ul><ul><ul><ul><li>Ureteric / vecsical malignanncy </li></ul></ul></ul><ul><ul><ul><li>Kenks & adhesions ( sec to VUR) </li></ul></ul></ul>30-march, 2010, tuesday
  35. 35. Bladder outlet obstruction <ul><li>Phemosis / fused synichae, Ext. meatal stenosis </li></ul><ul><li>Urethral Stone / foreign body impaction, </li></ul><ul><li>Enlarged prostate- -------- benign / malignant / inflammatory/abscess </li></ul><ul><li>bladder neck stenosis, Post urethral,valve Urethral stricture </li></ul><ul><li>neoplasm of bladder, urethra, prostate and penis </li></ul><ul><li>vesical calculus, foreign body </li></ul><ul><li>Neurogenic </li></ul><ul><li>Detrusor sphincter dys-synergia , </li></ul><ul><li>neurogenic bladder ,spine trauma, multiple seclerosis. DM </li></ul><ul><li>Stones , vesical , urethral, </li></ul>30-march, 2010, tuesday
  36. 36. Congenital (idiopathic)pelvi-ureteric obstruction <ul><li>Right side effected more </li></ul><ul><li>Female :male = 2:1 </li></ul><ul><li>Clinical features </li></ul><ul><ul><li>Insidious onset mild loin pain / dull ache / Sensation of dragging heaviness made worse by fluid intake </li></ul></ul><ul><ul><li>Little to call attention to renal damage </li></ul></ul><ul><ul><li>Kidney may / may not be palpable – renal failure intervenes before kidneys dilate </li></ul></ul><ul><ul><li>Intermittent hydronephrosis / Dietl’s crisis </li></ul></ul><ul><ul><li>Pain, swelling  passage of large volume urine  pain & swelling disappears </li></ul></ul><ul><ul><li>Trauma </li></ul></ul><ul><ul><li>Mass – obstructed kidney </li></ul></ul><ul><ul><li>hypertention </li></ul></ul>30-march, 2010, tuesday
  37. 37. 30-march, 2010, tuesday
  38. 38. Congenital(idiopathic) pelvi-ureteric obstruction <ul><li>Pathogenesis </li></ul><ul><ul><li>Adynamic segment of ureter </li></ul></ul><ul><ul><li>Polyps, valves, kiks, angulation </li></ul></ul><ul><ul><li>High origin, abnormal relation to vessels </li></ul></ul><ul><ul><li>Narrowing, strecture, </li></ul></ul><ul><ul><li>Pathology </li></ul></ul><ul><ul><ul><li>Pelvicaliceal system dilates at the expense of parenchyma which is compressed & destroyed by surrounding unyielding capsule. ( pressure atrophy) </li></ul></ul></ul><ul><ul><ul><li>Resultant nonfunctioning Kidney consists of thined out cortex making lobulated sac containing pale low specific gravity uriniferous fluid </li></ul></ul></ul><ul><ul><ul><li>Extrarenal pelvis </li></ul></ul></ul><ul><ul><ul><ul><li>Renal damage delayed and prolonged % lesser </li></ul></ul></ul></ul><ul><ul><ul><li>Intrarenal pelvis </li></ul></ul></ul><ul><ul><ul><ul><li>Renal damage rapid and severe </li></ul></ul></ul></ul><ul><ul><ul><li>Stasis leads to infection and stone formation – pain, fever , hematuria </li></ul></ul></ul>30-march, 2010, tuesday
  39. 39. Workup <ul><li>Urine RE </li></ul><ul><li>Urea criatinine </li></ul><ul><li>USG ---------- hydronephrosis /cortical thickness, in utero diagnosis </li></ul><ul><li>IVU- ----------- provided RFTs normal </li></ul><ul><ul><ul><li>Shows degree of obtrusion / level of, (delayed films) </li></ul></ul></ul><ul><ul><li>Normal calyceal cupping lost, -- clubbed </li></ul></ul><ul><ul><li>Pelvis dilated, if intrarenal parenchymal damage severe, as compared to extrarenal </li></ul></ul><ul><ul><li>In advance stage a faint nephrogram obtained around dilated calyces == soap bubble appearances </li></ul></ul><ul><li>Renal isotope scans (DTPA, MAG 3)= obstruction </li></ul><ul><ul><li>Diuretic ( frusimide) increases degree of obstruction </li></ul></ul><ul><li>Whitaker test : fluid innfused thru percuteneoous puncture annd intra renal pressuures monitered </li></ul><ul><li>Retrograde pyelogrphhy – level of obstruction </li></ul><ul><ul><li>Deranged RFTs / contrast sensitivity </li></ul></ul><ul><li>MCUG </li></ul>30-march, 2010, tuesday
  40. 40. Treatment <ul><li>Indications </li></ul><ul><ul><li>Recurrent pain </li></ul></ul><ul><ul><li>Increasinng hydronephrosis / renal damage </li></ul></ul><ul><ul><li>Infection, stones </li></ul></ul><ul><li>Aims </li></ul><ul><ul><li>Relieving obstruction </li></ul></ul><ul><ul><li>Preserving renal tissue </li></ul></ul><ul><ul><li>Preventing infection, stones </li></ul></ul>30-march, 2010, tuesday
  41. 41. Treatment - Options <ul><ul><li>Mild cases – follow u p with USG, operate only if hydronephrosis increasing </li></ul></ul><ul><ul><li>Corrective / reconstructive surgery – pyelo-reteroplasty </li></ul></ul><ul><ul><ul><li>– Anderson-Hynes, </li></ul></ul></ul><ul><ul><ul><li>VY plasty, </li></ul></ul></ul><ul><ul><ul><li>Clup’s </li></ul></ul></ul><ul><ul><li>Anastomosis protected by stent / nephrostomy </li></ul></ul><ul><ul><li>Nonfunctioning kidney – nephrectomy </li></ul></ul><ul><ul><ul><ul><li>Avoid if DMSA SCAN SHOWS >20% FUNCTION </li></ul></ul></ul></ul><ul><ul><li>Endoscopic ballon dilatation under image intensifier ( pyelolysis) </li></ul></ul><ul><ul><li>Percuteneous / retrograde Endopyelotomy </li></ul></ul><ul><ul><li>Laproscopic pyeloplasty </li></ul></ul>30-march, 2010, tuesday
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  46. 46. Assignment <ul><li>Define IVU </li></ul><ul><li>Give indications (5 atleast) </li></ul><ul><li>Precautions? (3) </li></ul><ul><li>Detail procedure with preparation </li></ul><ul><li>Adverse reactions – </li></ul><ul><ul><li>? To prevent, ? To treat </li></ul></ul><ul><li>Contra-indications </li></ul><ul><li>What does it tell </li></ul><ul><li>how to read </li></ul><ul><li>how to interpret </li></ul>30-march, 2010, tuesday