مواضيع المحاضرة: Congenital anomaly of urinary system
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forth stage

Surgery (Urology)

Lec-2

د.ﻣﺣﻣد اﻟﺷﮭواﻧﻲ

20/10/2015

Congenital anomaly of urinary system

Congenital abnormalities of the kidney

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Its relatively uncommon                            

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Usually symtomless 

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if symptomatic its due to

     infection

     stone

    hydronephrosis  

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    Often discovered by accident                                 

Anomalies of number

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Bilateral renal agenesis ( not compatible with life )

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Unilateral renal agenesis

asymptomatic

Accidentally discovered

association with other anomalies

Anomalies of position

         Ectopia :

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Pelvic

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lumber

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Rarely thoracic

40% symptomatic, association with other anomalies 

Crossed ectopia :

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Non fused

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Fused    


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Horse shoe kidney

The commonest fusion anomalies

1/3rd of cases are symptomatic

Symptoms related to:  -Infection   -Stones   -Hydronephrosis 

Diagnosis:  May be palpable, US, IVU.   MRI

treatment:

1. Treat Infection ,stone, hydronephrosis ( If present )
2. Division of the esthmus is only indicated in the course of surgery for 

abdominal aortic aneurysm

Parenchymal anomalies

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Hypoplasia (small kidney)

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Dysplasia:    1-Cystic dysplasia       2-Polycystic renal disease(Infantile,Adult)


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                                                "dysplasia"                                                          "hypolpasia"

Polycystic kidney

A. infantile type

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     Autosomal recessive   

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     US diagnosis       

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      Early renal failure  

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      incompatable with life

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    causing obstructed labour 

B. Adult type

The most common renal cystic disease Autosomal dominant

Progressive bilateral cystic degeneration

Clinical presentation:

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Positive family history 

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Loin pain before the development of renal mass                                                         

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Hypertension, hematuria, renal mass

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Associated liver cystic disease may be seen

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Renal failure Usually in the early fifty

Imaging

1- Ultra sound is diagnostic    2-IVU 3-MRI     4-CT scan


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Treatment

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Medical management of renal failure

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Surgery :   

1. ( cyst puncturing)  
2. Renal stone
3. Cyst infection
4. Hemorrhage in the cysts
5. Ureteric obstruction by cyst

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Definitive treatment  is renal transplantation

Simple renal cyst(Solitary renal cyst)(Blue domed cyst)

Unilocular , Avascular,smooth, clear fluid content

Mostly asymptomatic

Large cyst may be felt as a mass

Incidental finding on US or other imaging

renal cell carcinoma should be ruled out

Treatment :

1. Reassurance and follow up
2. If symptomatic ---> Rovsing operation( Deroofing)  by open surgery  or 

laparoscopic surgery


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Anomalies of the collecting system

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bifid pelvis

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Hydrocalicosis 

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Calycial diverticulum 

Ureteral anomalies

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Duplication ( Partial,Complete)

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Ectopic ureter 

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Ureterocele(Orthotopic ,Ectopic)

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Pelviureteric junction obstruction

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Congenital mega ureter 

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Retrocaval ureter 

duplication of ureter :

-partial or complete
-Clinically  asymptomatic unless complicated
-Diagnosis: US. IVU . CT scan .  MCU
-treatment: Only when symptomatic

             "partial"                                                     "complete"

Ureterocele

a cystic dilatation of the intravesical sub mucosal part of the ureter

Simple : in normally placed uretric orifice

Ectopic : In lower position placed ureteric orifice , or with ureteric duplication

Diagnosis : ultrasound ,IVU (copra head) ,MCU,Cystourethroscopy

treatment: 

1-In simple ureterocele: 
a-in functioning kid--->excision & reimplantation 
b- If non functioning kidney---> nephrectomy 
2-In ectopic ureterocele  :if single--->As in simple ureterocele 


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Ureteropelvic junction obstruction

Primary  : congenital

Secondary :   to refluxing ureter 

Mechanism of obstruction:

Intrinsic smooth muscle pathology.

Adynamic segment

Congenital segmental stenosis 

Mucosal valve , web , folds

Over riding an aberrant vessel

Clinical presentation

Abdominal mass

Episodic flank pain

Pain & fever when infected

The aggravating factors: Cold, diuresis , fluid over  intake

Diagnosis

US

IVU

Diuretic IVU  , Diuretic renography 

Renal DMSA scan   ( functional).

Retrograde pyelography 

Treatment

Conservative (Treat the pain ,infection  and follow up)

Indication of surgery

1. Recurrent attack of pain,
2. stone,
3. progressive hydronephrosis 

*Surgery : Pyeloplasty,  by open surgery or laparoscopic pyeloplasty 

1.              Underson hynes 


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2.              Culp
3.              Scardino 
4.              V-Y plasty 

Endoscopically  :antegrade or retrograde  endopyelotomy 

Postcaval ureter

treatment : Surgery if causing obstruction and pain By resection of post caval 
segment and reanastomose the ureter in front of the IVC

Congenital mega ureter

Functional obstruction of lower end of the ueter leading to a progressive 
dilatation

Unilateral or bilateral

Diagnose by IVU

Treatment is by reimplantation 

                 




رفعت المحاضرة من قبل: Abdalmalik Abdullateef
المشاهدات: لقد قام 14 عضواً و 222 زائراً بقراءة هذه المحاضرة








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