Congenital anomaly of urinary system dr.mohamed fawzi alshahwani ة Congenital anomaly of urinary system dr.mohamed fawzi alshahwani

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 \

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

Anomalies of the collecting system Bifid pelvis Hydrocalicosis Calycial diverticulum Your Text Here

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

Simple : in normally placed uretric orifice 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

Ureterocele involving single system

Diagnosis uls IVU MCU Cystourethroscopy

Ureterocel: U/S

Ureterocele copra head appearance by 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