Anomalies of Genito-Urinary Tract

Urology.
Anomalies of Genito-Urinary Tract

07.10.2013 Anomalies of Genito-urinary Tract 1
Anomalies of genitourinary tract are the most wide-spread, since they are 40 % of all congenital developmental anomalies of person and 30 % of cases are combined with anomalies of genital and other organs. In accordance with the last data we observe growth of such patients number, that is explained by complicated development of genito-urinary system and impact of a good deal of unfavorable factors.
Kidneys are formed of
primordiums:
nephrogenic blastema and
Wolfian duct.
Nephrogenic blastema passes three stages of development:
pronephros, initial kidney, final kidney

and gives rise to glomuluses and one of tubules group.
The other group of
tubules, renal calices, pelvis, ureter, bladder, urethral canal

are formed from
Wolfian duct.
Kidneys formation occurs on the 6-8 weeks of embryogenesis.

That is why unfavorable factors impact such as
radiological and nuclear, chemical, medicinal, thermal etc .

in this period results in
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underdevelopment of these organs.

Anomalies of Genito-urinary Tract 6
It is worth mentioning that people with anomaly of genito-urinary organs may not even suspect, that they have a developmental anomaly.
Anatomic features of improperly generated organs

Disorder of urine passage
Frequent development of retention diseases

pyelonephritis hydronephrosis nephrolithiasis
Renal insufficiency
Anomalies of kidneys:
Anomalies in Number. Anomalies in Position. Fused kidneys Anomalies in Structure of Kidney
Anomalies in number:
Aplasia (agenesia) Duplicated kidney Supernumerary kidney
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Aplasia of kidney
is the result of halt of development on pronephros or initial kidney stage
The following methods of examination help to make a correct diagnosis of aplasia:

Aplasia of kidney
CYSTOSCOPY.
Aplasia of the right kidney (absence of ureter orifice)
The whole trigone of the bladder or the half may be absent. The ureteral orifice of anomalous kidney sometimes drains into genital tract and not into the bladder
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Aplasia of kidney
Excretory Urography.
Aplasia of right kidney (absence of pelvis)
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Aplasia of kidney
CT image.
Aplasia of the right kidney and compensatory hypertrophy of the left kidney.
Aplasia of kidney
25 % of observations of solitary ren note malformation of genital system, such as which are formed from Wolfian duct
duplication of uterus or vagina,
Treatment of congenital solitary kidney and its disease.
A solitary kidney usually requires no treatment, but it is necessary in case rens affection by disease. Only organ-preserving operations are performed: pyelolithtomy, ureterolithtomy, nephropexy and plastic surgery on urinary tract.
Supernumerary kidney
A supernumerary kidney is rather a rare anomaly.
It is the third or even fourth separate additional kidney.

Each supernumerary kidney has its independent vascular pedicle and own ureter, opening into urinary bladder (independent orifice) or a branch of main ureter of normal (main) kidney (general orifice).
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The diagnosis is made on the basis of excretory urography or retrograde pyelography, when two pelvis are seen at a distance from one another and when there are two separate kidney images.
Renal Supernumerary kidney Pneumoretroperit angiography oneum will help in provides with complicated a clearer image cases before of kidneys. surgical These methodsinterference of examination make it possible to distinguish a supernumerary kidney from a duplicated kidney.
The question of TREATMENT arises only in diseases of the supernumerary kidney. Nephrectomy is the operation of choice, since there is usually no need for organ-preserving operation. They are performed in cases of functional decrease of main rens.
Duplication of kidneys can be defined due to
extra urinary tracts
Duplicated kidneys occur often enough:
in 10,4% of all urological patients

Pathological development goes by parallel formation of 2 ureter primordiums on Wolfian duct or splitting of 1 primordium into 2 branches.
Duplication of kidneys
Shift of extra primordium to the side of urethra forming or to genital organs side results in ectopia of ureter orifice into urethra or genital organs.
In such a way complete duplication (2 ureters with separate orifices), or incomplete duplication 07.10.2013 Anomalies of Genito-urinary Tract
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Duplicated kidneys are often accompanied with

congenital ureteral defects: ectopia, ureterocele, blind ending of terminal or cranial ureteral segment, and stenosis of ureter or its valves
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Angiography.
be
Vascularization of the duplicated kidney may provided by one
or more renal arteries, which separate into branches and give separate terminal arteries to each of its parts.
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Cystoscopy An accessory ureteral orifice in the bladder is seen complete duplication of the kidney.Anomalies of Genito-urinary Tract 07.10.2013
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Excretory urography
makes recognition of the anomaly possible in most cases.

When the kidney is functioning well both pelvis and ureters are clearly visible as well as their morphological features and the function of each part;
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Treatment of duplicated kidney and their disease.
Conservative treatment spasmolytics and catheterization of renal pelvis, is aimed at improving urine flow from the upper urinary tract and relieving dyskinesia, is applied in cases when it causes pain.
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urinary tract (dyskinesiaand interureteral yo-yo reflux) by organ-preserving operations: high-level pyeloureterostomy and remove one of ureters
is eliminated development of diseases associated with poor drainage of the pelvis

In mild or moderate grade I-II of
hydronephrosis,
when the kidney keeps on functioning, plastic procedures are performed:
pyelopyelostomy or pyeloureterostomy or
ureteroureterostomy.
is Heminephrectomy
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recommended in cases of gross destruction of ren reduced function of a rens part doubtful outcome of plastic
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It is preferable to use
mechanical
tantalum suture, applied by means of the
-60 stitcher.
Duplication of kidneys and resection of accessory ureter n operation of heminephrectomy

with the ectopic orifice.
Excretory urography.
Good functioning of the operated kidney. A chain of
tantalum staples
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is seen in the
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Anomalies in Position. Kidney Distopia. is the result of disorder of normal shift to ordinary bed in lumbar area. No turn around rens own axis occurs.
Classification
Anomalies in Position. Kidney Distopia.
symmetrical asymmetrical
1. pelvic 2. iliac 3. lumbar 4. thoracic unilateral bilateral:
homolateral heterolateral
Kidney Distopia.
Diagnosis
Dystopic kidneys are predisposed to retention diseases:
hydronephrosis nephrolithiasis pyelonephritis
Kidney Distopia
is diagnosed by: abdominal pain of indefinite localization or pain in lower abdomen, in some cases dysuric disorders, symptoms of diseases of female genitals, gastro-intestinal, nervous and cardiovascular system a tumor palpated in the abdomen
Diagnosis
X-ray methods
of examination are the leading techniques in the diagnosis of dystopic kidneys.
Excretory urography | Retrograde

The lateral position of pelvis, median position of calyces and a short ureter are seen.
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Diagnosis
Hydronephrosis of dystopic kidney
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Diagnosis
Isotope renorgraphy | Kidney scintiscanning shows function of kidney and in some cases indicates the absence of function though the function is satisfactory (if the sensors are located on the place, that is to be occupied by normal kidney)
Treatment of diseases of dystopic kidney.
People with dystopic kidneys that have no clinical manifestations or diseases require no treatment.
Treatment of diseases of dystopic kidney.
Surgery should be performed

in all cases of hydronephrotic ren transformation concomitant infection disorders of renal function nephrolithiasis (frequent renal colic)
Fused Kidneys Anomalies of Interrelation
are characterized by fusion of 2 renal primordiums during 8 week of embriogenesis into 1 organ, its ureteres ending in ordinary place.
There are many variants of fusion according to form and relation to vertebra.
Horseshore kidney fused at lower pole
Cake kidney
Horseshore kidney fused at upper pole
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Fusion occurs in lower poles in 90%. Isthmus can be parenchymatous or fibrous, Pelvis located on anterior surface. The fused ren can have 1-10 arteries, enveloping and fixing organ.
Scheme of fused kidney vascularization

X-ray methods of examination
Excretory urography
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Retrograde pyelography
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Scintyscanning of horseshoe kidney.
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Preoperative renal angiography , determines many extra arteries (arterial phase ).

Knowledge of vascularization of isthmus and distribution of vessels prevents dangerous bleeding.
Shape and position of organ are

determined in parenchymatous phase.
Extraordinary anatomy favors urostasis in pre-pelvic part of ureteres and development of
pyelonephritis, hydronephrosis, nephrolithisiasis.
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Surgical treatment of fused kidney.
2 groups can be distinguished: operations without transection of renal isthmus and -operations with transection or resection of isthmus of anomalous organ.
Resections of isthmus and lower poles of horseshoe kidney
horseshoe kidney
suturation through parenchyma and renal capsule;
nephretomy and applying of superficial seams
result of operation.
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Anomalies in Structure of Kidney.

Cystic Diseases of the Kidneys
Simple cysts are divided into congenital (primary) and acquired (secondary) cyst.
Is formed as a result of single lacks of coincidence of convoluted and straight renal tubules
Is formed after infarct of ren, pyelonephritis, nephrolithiasis, (is a result of ischemia, inflammation, urostasis)
Solitary cyst
1.is either round or oval in shape 2.The contents of the cyst usually serous, rarely haemorrhagic 3.may be localized in various parts of organ: Subcapsule Intraparenchimatose Cortical Peripelvic Multilocular
4.8-10 cm in diameter
Solitary cyst
1. usually has no specific symptoms 2. Is found occasionally in urological examination Pains in lumbar Palpated tumor Total haematuria Arterial hypertension
Complications of solitary cyst
Hemorrage Disruption of cyst
Suppuration
Forming of concrements in pelvic-calicular system
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Treatment of solitary cyst

Small cysts need no treatment
Large solitary cysts in renal pole allow to p under ultrasonic control, aspiration and sclerosing solutions into its cavity (ethy
Puncture is carried out under local anaest
Percutaneous puncture of cysts of kidneys is an atraumatic method and often allows to refuse surgical interven Is often accompanied by recidivations of cystic
occur in case of pyelonephritis, nephrosclerosis(a), tumors cystadenoma (b) tuberculosis (c) of kidney hydrocalosis (d)
Acquired seconda
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Polycystic kidney disease

is frequent inherited by autosomal dominant anomaly
is always bilateral process
It may appear in children, but is usually diagnosed in adult life.
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Polycystosis is characterized by substitution of 2 rens parenchyma by multiple cysts of various size.

Defect occurs due to ill-timed conjugation of straight and convoluted tubules, formed from various primordiums in embriogenesis of secretory and excretory nephrones segments. I.e. urine drainage is violated, blindly closed tubules are dilatated and form cysts. If this tubules do not join at all, large cysts are formed.
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Squeeze of intertissue, urostasis infection and pyelonephritis. Progressing of inflammatory process, increase of cyst size, disorder of renal function
lead to
lead to
renal insufficiency
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Polycystic kidneys are often combined with polycystic liver, pancreas, spleen, lungs, ovaries, and also with aneurysm of cerebral arteries etc.
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Polycystic kidney disease has various manifestations
Dull pain in lumbar is the most common symptom due to: a) disturbed flow of urine; b) circulatory disorders in kidney; c) inflammatory processes in kidney. Pain in epigastrium may be caused by displacement of abdominal organs by large cystic kidneys and by considerably increased intraabdominal pressure.
Palpable tumor in abdomen may be patient's only complaint. Polycystic kidney can be palpated by the patient himself due to large size and mobility.
arterial pressure from 150/80 to 220/110 mm Hg in 20 per cent of cases cardiovascular disorders may be the only manifestation of polycystic kidneys Cardiovascular disorders are manifested by pain in the heart area, retrosternal pain, headache, and dizziness.
3 stages in clinical course of polycystic renal disease (1)compensation, (2)subcompensation,
(3)decompensation.
Diagnostics of a polycystosis
Scintigraphy of kidneys - to define quantity of functioning parenchyma many anechogenic zones Ultrasound research in field of kidneys and liver
CT scaning
Treatment of polycystosis is difficult,

since polycystic disease is a bilateral process, which tends to progress
Non-operative treatment:
complex measures: medicinal treatment dietetic therapy combined with physiotherapy aimed at:
elimination of infectious process in urinary tract and control of renal insufficiency, anaemia and hypertension
Operative treatment of polycysti kidneys has temporary effect Indications: (1)persisting pain in renal area; (2) large cysts, particularly in region of renal hilus; (3) recurrent total haematuria; (4) suppuration of cysts; (5) hypertension resistant to hypotensive therpy; (6) tumour or tuberculosis of polycystic kidney; (7) concrement in a kidney and ureter
1. Surgical decompression of polycystic kidney by multiple punctures and emptying of cysts ignipuncture (Rovsings operation) with omentorenopexia. 2. Percutaneous puncture of cysts under ultrasound control (operation of De La Pena).
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Thank You For Attention!
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Anomalies of Genito-Urinary Tract

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Urology.

Anomalies of Genito-Urinary Tract

Kidneys are formed of

Nephrogenic blastema passes three stages of development:

pronephros, initial kidney, final kidney

The other group of

tubules, renal calices, pelvis, ureter, bladder, urethral canal

Kidneys formation occurs on the 6-8 weeks of embryogenesis.

radiological and nuclear, chemical, medicinal, thermal etc .

underdevelopment of these organs.

Anatomic features of improperly generated organs

Frequent development of retention diseases

Anomalies of Genito-urinary Tract

The following methods of examination help to make a correct diagnosis of aplasia:

Aplasia of the right kidney (absence of ureter orifice)

Anomalies of Genito-urinary Tract

duplication of uterus or vagina,

Treatment of congenital solitary kidney and its disease.

It is the third or even fourth separate additional kidney.

Duplication of kidneys can be defined due to

extra urinary tracts

Duplicated kidneys occur often enough:

in 10,4% of all urological patients

Duplicated kidneys are often accompanied with

Anomalies of Genito-urinary Tract

Vascularization of the duplicated kidney may provided by one

Anomalies of Genito-urinary Tract

makes recognition of the anomaly possible in most cases.

Anomalies of Genito-urinary Tract

Treatment of duplicated kidney and their disease.

Anomalies of Genito-urinary Tract

is eliminated development of diseases associated with poor drainage of the pelvis

Duplication of kidneys and resection of accessory ureter n operation of heminephrectomy

Good functioning of the operated kidney. A chain of

Anomalies of Genito-urinary Tract

Anomalies in Position. Kidney Distopia.

1. pelvic 2. iliac 3. lumbar 4. thoracic unilateral bilateral:

Excretory urography | Retrograde

Hydronephrosis of dystopic kidney

Anomalies of Genito-urinary Tract

Treatment of diseases of dystopic kidney.

Treatment of diseases of dystopic kidney.

Surgery should be performed

Fused Kidneys Anomalies of Interrelation

Horseshore kidney fused at lower pole

Horseshore kidney fused at upper pole

Anomalies of Genito-urinary Tract

Scheme of fused kidney vascularization

X-ray methods of examination

Anomalies of Genito-urinary Tract

Scintyscanning of horseshoe kidney.

Anomalies of Genito-urinary Tract

Preoperative renal angiography , determines many extra arteries (arterial phase ).

Shape and position of organ are

Extraordinary anatomy favors urostasis in pre-pelvic part of ureteres and development of

pyelonephritis, hydronephrosis, nephrolithisiasis.

Anomalies of Genito-urinary Tract

Surgical treatment of fused kidney.

Resections of isthmus and lower poles of horseshoe kidney

suturation through parenchyma and renal capsule;

nephretomy and applying of superficial seams

Anomalies of Genito-urinary Tract

Anomalies in Structure of Kidney.