Congenital abnormities of kidney ad ureter 30 3-10

Dr Ahmed RehmanFCPS UrologyAssistant Professor Urology

30-march, 2010, tuesday 1

Learning Objectives

Enlist congenital abnormalities of kidney & ureter

Describe clinical significance of these abnormalities, ie What problems these can cause (symptoms) What are risks to health ( complications)

Enlist diagnostic investigation plan– history, examination, investigations

Suggest treatment plan in these conditions


Congenital abnormalities of kidney Uncommon ( <1:1000) Commonly Symptomless – found

incidentally – US,IVU, CT. Why ????

sometimes detected only when have caused sufficient damage

Endanger kidneys to various complications


Agenesis / aplasia of kidney Bilateral – fatal Unilateral –

compatable with normal life, contralateral kidney hypertrophed

Failure of mesonephric duct to bud

Ureteric orifice absent on cystoscopy

Rarely ureters & pelvis may be present but renal tissue is absent or so


Hypoplasia-dysplasia


Renal Ectopia / pelvic kidneys Doesn't ascend, formed

near pelvic brim No symptom Present with pain or mass

which one may tempt to remove as unexplained pelvic mass

pose diagnostic problems in case of disease or surgery

May be source of stone, infection

Liable to trauma30-march, 2010, tuesday 6

Crossed Ectopia / Crossed Dystopia

Both kidneys lie in one loin

May be fused with each other or separate

Ureter of lower crosses midline to open into bladder on its normal side


Crossed Ectopia contrasted with normal IVU


Mal rotated kidneys

Calyces face anteriorly or antrolaterally

Have some element of obstruction causing inadequate drainage – leading to infection & stone formation


Horse shoe kidney

low lying – ascent impeded by inferior mesenteric artery

Lower poles fused in mid line in front of 4th lumber vertebra.(isthemus).

Longitudinally lie medially and downwards,

instead of laterally and down wards,

Part or whole of pelvicaliceal system is malrotated ( facing medially),

Ureters curve over fused poles.


Horse shoe kidney

Pain, hematuria, fever, mass

Exam : fixed mass below umbilicus Diagnosis: US & IVU Significance: Liable to disease

angulated ureters + PUJ obstruction urinary stasis stones, infection & obstruction CRF


Horse shoe kidney

Treatment Asymptomatic = nothing doing Mild sypmtoms = treat accordingly PUJ or ureteric obstruction, recurrent

infections, Stones surgery (pyelolithotomy + /-reconstruction)

ISTHEMECTOMY with straightening of ureters – less commonly done.


Polycystic kidneys

Hereditary – autosomal dominant Not manifested before 30 Kidneys enlarged, studded with cysts Unyeilding capsule compresses renal

parenchyma causing atrophy Liver,lungs and pancreas may be

affected Defact : not clear, many theories


Polycystic kidneys

Loin pain- weight dragging upon peddicle or capsule stretch, hemorhage in cyst, stone

abdominal mass- confused with cystic tumor

hematuria- cyst rupture in pelvis,moderate, episodic.

hypertention, infection, & uremia/CRF.

Nonspecific symptoms: anorexia, headache, vague abdominal discomfort, vomiting, drowsiness, anemia.

ESRD: suddenly in middle age, survival without RRT ( dialysis/ transplant) unlikely


Polycystic kidneys

US and CT: cysts in kidneys,liver & others Simple (aquired) cysts: solitary, smooth

walled & homogeneous contants Blood & debris – cystic adenocarcinoma FNA- cytology – differentiates

IVU Enlarged renal shadow, Renal pelvis – compressed & elongated Calyces – narrow, stretched over cysts

( spider legs / bell shaped)


Polycystic kidneys

Nephrologist : BP control, infection,anemia, disturbances

of Ca metabolism, low protein diet to delay need for DIALYSIS

Urologist: Surgical / laproscopic deroofing of cysts

( Rovsing’s operation) Relieves pain & pressure saving kidneys Rarely performed / not preserve function

Renal transplant/ pretransplant bilateral native kidneys nephrectomy


solitary / Simple (acquired) renal cysts Common, may be multiple ( not

always 1 ) Incidentally found– no treatment

needed Rarely symptomatic; pain- stretch,

bleed in cyst, infection,mass papa-pelvic cyst at hilum presses

PUJ obs IVU: filling defect. US, CT :smooth, homogeneous

contant DD: hydatid, cystic

adenocarcinoma Percuteneous FNA Cytology Treat only in case obstructing


Infantile polycystic kidneys Rare Inheritance- autosomal recessive Enlarged kidneys – may obstruct

labour, Many stillborn Die of renal failure in in early

childhood


Unilateral Multicystic kidney More common Presents as nonfunctioning mass Exploration & removal is treatment

of chioce DD: wilm’s, neuroblastoma,

congenital hydronephrosis


Aberrant vessels

Variation in no of vessels – common Arteries – END arteries, damage

ischemia Veis – extensive colaterals = can be

ligated are not cause of hydronephrosis-

though the bulging renal pelvis in between them makes them noticeable


Abnormalities of renal pelvis and ureter Most common, harmless,

asymptomatic

DUPLICATION OF RENAL PELVIS

Common,4%, usually unilateral – left

Upper pelvis – small, drains upper calyx

Asymptomatic no treatnment

If one moity severly damaged – partial nephrectomy


Duplex kidney, double moiety


DUPLICATION OF URETER. 3%

Often join before reaching bladder, suffer obstruction ( esp from stones) & YOYO reflux

May open independently, ureter from upper moity opens distal and medial to its fellow.

Uppermoity ureter suffers ureterocele.

Lower moity ureter suffers VUR

Infection, calculus formation, PUJ obst and VUR, ectopic opening


Abnormalities of ureter Ectopic uretric opening

Female – into urethra below sphincter on vagina = incotinance since childhood with desire and passage of urine normally as well. IVU and cystoscopy ( indigocarmine)

Male.continent as opening is above sphincter Opening in trigone apex, post. Urethra, seminal vesical

or ejaculatory duct – functionally abnormal, infection common

Treatment: Frequently ectopic ureter drains hydronephrotic,

chronically infected moity --- best excised - nephrectomy Incontinence can be cured and renal function preserved

by implanting ureter into bladder ( tunneling) or joining its fellow. (URETERO-NEOCYSTOSTOMY, URETERO-URETEROSTOMY)


Abnormalities of ureter CONGENITAL MEGA URETER

Uncommon, bilateral FUNCTIONAL

obstruction at lower end dilatation & infection

Ureteric orifices normal, ureteric cath passes easily

Reflux not feature till opened endoscopically

Treatment: refashioning and tunneled reimplant


Abnormalities of ureterPOST (retro) CAVAL URETER

Right ureter passes behind IVC instead of lying to the right of it (laterally)

If causing obstruction, can be devided and joined in front of cava – long oblique ETE anastomosis


Abnormalities of ureter URETEROCELE

Cystic enlargement of intramural portion of ureter

Thought to result from congenital atresia of ureteric orifice

Though present since childhood, unrecognised till adulthood

More common in female, cause BOO by obstructing / prolapsing into internal urinary meatus. May even prolapse out of urethra


Adder head on IVU Cyst wall composed of

urothelium only confirmed on cystoscopy

Translucent cyst, enlerging and collapsing as urine flows

Treatment avoided unless symptoms of infection / stone

Endoscxopic diahermy incision / deroofing Postoperatove MCUG to

see VUR Ureteral reimplant Sever hydronephrosis,

pyonephrosis nephrectomy


Hydronephrosis

Aseptic dilatation of pelvicaliceal system due to complete or partial obstruction.

Unilateral hydronephrosis Epsilateral

ureteric obstruction

( unilateral supravesical obstruction)


Hydronephrosis:Bilateral

Bilateral ureteric obstruction ( bilateral supravesical obstruction) Urethral obstruction

( bladder outlet obstruction, infravesical obstruction )

Detrusor hypertrophy intramural ureteric obstruction

VUR Pregnancy –physiologic

dilatation - progesterone, early pregnancy – 20 weeks marked reverts 12 week of delivery

Infection, diagnostic difficulty in acute abdominal pain in pregnancy


Causes of Ureteric Obstruction

Extramural Tumors of cervix, ovary, uterous, vagina, urinary bladder,

prostate, rectum, colon, caecum & lymphomas Idiopathic retroperitoneal fibrosis Retrocaval ureter Pararenal cysts Aberent vessels

Intraluminal Calculus, sloughed papilla, clot, ureteric malignancy

Intramural Congenital PUJ obstruction or stenosis Ureterocele and congenital small ureteric orifice Strictures ( stone, repair, tuberculosis, schistosomiasis) Ureteric / vecsical malignanncy Kenks & adhesions ( sec to VUR)


Bladder outlet obstruction Phemosis / fused synichae, Ext. meatal

stenosis Urethral Stone / foreign body impaction, Enlarged prostate--------- benign /

malignant / inflammatory/abscess bladder neck stenosis, Post urethral,valve

Urethral stricture neoplasm of bladder, urethra, prostate and

penis vesical calculus, foreign body Neurogenic

Detrusor sphincter dys-synergia ,neurogenic bladder ,spine trauma,

multiple seclerosis. DM Stones , vesical , urethral,


Congenital (idiopathic)pelvi-ureteric obstruction Right side effected more Female :male = 2:1 Clinical features

Insidious onset mild loin pain / dull ache / Sensation of dragging heaviness made worse by fluid intake

Little to call attention to renal damage

Kidney may / may not be palpable – renal failure intervenes before kidneys dilate

Intermittent hydronephrosis / Dietl’s crisis

Pain, swelling passage of large volume urine pain & swelling disappears

Trauma Mass – obstructed kidney hypertention


Congenital(idiopathic) pelvi-ureteric obstruction Pathogenesis

Adynamic segment of ureter Polyps, valves, kiks, angulation High origin, abnormal relation to vessels

Narrowing, strecture, Pathology

Pelvicaliceal system dilates at the expense of parenchyma which is compressed & destroyed by surrounding unyielding capsule. ( pressure atrophy)

Resultant nonfunctioning Kidney consists of thined out cortex making lobulated sac containing pale low specific gravity uriniferous fluid

Extrarenal pelvis Renal damage delayed and prolonged % lesser

Intrarenal pelvis Renal damage rapid and severe

Stasis leads to infection and stone formation – pain, fever , hematuria


Workup

Urine RE Urea criatinine USG---------- hydronephrosis /cortical thickness, in utero

diagnosis IVU------------ provided RFTs normal

Shows degree of obtrusion / level of, (delayed films) Normal calyceal cupping lost, -- clubbed Pelvis dilated, if intrarenal parenchymal damage severe, as

compared to extrarenal In advance stage a faint nephrogram obtained around dilated

calyces == soap bubble appearances Renal isotope scans (DTPA, MAG 3)= obstruction

Diuretic ( frusimide) increases degree of obstruction Whitaker test : fluid innfused thru percuteneoous puncture

annd intra renal pressuures monitered Retrograde pyelogrphhy – level of obstruction

Deranged RFTs / contrast sensitivity MCUG


Treatment

Indications Recurrent pain Increasinng hydronephrosis / renal damage Infection, stones

Aims Relieving obstruction Preserving renal tissue Preventing infection, stones


Treatment - Options

Mild cases – follow up with USG, operate only if hydronephrosis increasing

Corrective / reconstructive surgery – pyelo-reteroplasty – Anderson-Hynes, VY plasty, Clup’s

Anastomosis protected by stent / nephrostomy

Nonfunctioning kidney – nephrectomy Avoid if DMSA SCAN SHOWS >20% FUNCTION

Endoscopic ballon dilatation under image intensifier ( pyelolysis)

Percuteneous / retrograde Endopyelotomy Laproscopic pyeloplasty


Assignment

Define IVU Give indications (5 atleast) Precautions? (3) Detail procedure with preparation Adverse reactions –

? To prevent, ? To treat Contra-indications What does it tell how to read how to interpret


Congenital abnormities of kidney ad ureter 30 3-10

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