Urology

Congenital anomaly ofurinary systemdr.mohamed fawzi alshahwani

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Congenital abnormalities of the kidney

Its relatively uncommon Usually symtomless if symptomatic its due to infection stone hydronephrosis Often discovered by accident

Anomalies of number

Bilateral renal agenesis not compatible with life Unilateral renal agenesis asymptomatic Accidentally discovered association with other anomalies

Anomalies of position

Ectopia Pelvic lumber Rarely thoracic 40% symptomatic, association with other anomalies

Ectopic kidney

Thoracick kidney



Lumber ectopic kidney
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Crossed ectopia : Non fused Fused

Cross ectopia
Non fused

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

Horse shoe kidney

Horse shoe normal

treatment

Treat, Infection stone hydronephrosis Division of the esthmus is only indicated in the course of surgery for abdominal aortic aneurysm

Parenchymal anomalies

Hypoplasia ..small kidney Dysplasia: Cystic dysplasia Polycystic renal disease: Infantile Adult


dysplasia


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Polycystic kidney

A. infantile type Autosomal recessive US diagnosis Early renal failure incompatable with life causing obstructed labour

Adult polycystic renal disease

The most common renal cystic diseaseAutosomal dominant Progressive bilateral cystic degeneration

Adult polycystic renal disease

Clinical presentation
Positive family history Loin pain before the development of renal mass Hypertension, hematuria, renal mass Associated liver cystic disease may be seen Renal failure Usually in the early fifty

Imaging

Ultra sound is diagnostic Ivu: MRI CT scan

Treatment

Medical management of renal failure Surgery : ( cyst puncturing) Renal stone Cyst infection Hemorrhage in the cysts Ureteric obstruction by cyst Definitive treatment, renal transplantation

Simple renal cystSolitary renal cystBlue 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

Reassurance and follow up If symptomatic.. Rovsing operation : Deroofing by open surgery or laparoscopicaly

Anomalies of the collecting system

Bifid pelvis Hydrocalicosis Calycial diverticulum



Bifid renal pelvis

Ureteral anomalies

Duplication : Partial Complete Ectopic ureter Ureterocele: Orthotopic Ectopic Pelviureteric junction obstruction Congenital mega ureter Retrocaval ureter

MRI: Partial duplication

Complete ureteral duplication and ectopic ureteric orifice.

IVU: ureteric duplication

Clinically asymptomatic unless complicatedDiagnosis: US. IVU . CT scan . MCUtreatment: Only when symptomatic

UreteroceleA 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



Ureterocele involving single system

Diagnosis

uls IVU MCU Cystourethroscopy

Ureterocel: U/S

Ureterocelecopra head appearanceby IVU

treatment

In simple ureterocele: in functioning kid, excision & reimplantation If non functioning kidney,,,nephrectomy In ectopic :if single As in simple ureterocele

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

IVU: PUJO

pujo

Treatment

Conservative Treat the pain ,infection and follow up Indication of surgery Recurrent attack of pain, stone, rogressive hydronephrosis

Surgery

Pyeloplasty, by open surgery or laparoscopic pyeloplasty Underson hynes Culp Scardino V-Y plasty

Treatment,cont

Endoscopically

antegrade or retrograde endopyelotomy

Endopyelotomy

Postcaval ureter

Post caval 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 Uni or bilateral Diagnose by IVU Treatment is by reimplantation