Congenital anomaly ofurinary systemdr.mohamed fawzi alshahwani
Congenital abnormalities of the kidneyIts 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 anomaliesAnomalies of position
Ectopia Pelvic lumber Rarely thoracic 40% symptomatic, association with other anomaliesEctopic kidney
Thoracick kidneyLumber ectopic kidney
\Crossed ectopia : Non fused Fused
Cross ectopiaNon 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. MRIHorse shoe kidney
Horse shoe normaltreatment
Treat, Infection stone hydronephrosis Division of the esthmus is only indicated in the course of surgery for abdominal aortic aneurysmAnomalies of rotation
Malrotation Accidental discovery BizarreParenchymal anomalies
Hypoplasia ..small kidney Dysplasia: Cystic dysplasia Polycystic renal disease: Infantile Adultdysplasia
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Polycystic kidney
A. infantile type Autosomal recessive US diagnosis Early renal failure incompatable with life causing obstructed labourAdult polycystic renal disease
The most common renal cystic diseaseAutosomal dominant Progressive bilateral cystic degenerationAdult polycystic renal disease
Clinical presentationLoin pain before the development of renal mass Hypertension, hematurea, renal mass Positive family history. Associated liver cystic disease may be seen Renal failure Usually in the early fifty
Imaging
Ultra sound is diagnostic Ivu: MRI CT scanTreatment
Medical management of renal failure Surgery : ( cyst puncturing) Renal stone Cyst infection Hemorrhage in the cysts Ureteric obstruction by cyst Definitive treatment, renal transplantationSimple 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 outTreatment
Reassurance and follow up If symptomatic.. Rovsing operation : Deroofing by open surgery or laparoscopicalyAnomalies of the collecting system
Bifid pelvis Hydrocalicosis Calycial diverticulumBifid renal pelvis
Ureteral anomalies
Duplication : Partial Complete Ectopic ureter Ureterocele: Orthotopic Ectopic Pelviureteric junction obstruction Congenital mega ureter Retrocaval ureterMRI: Partial duplication
Complete ureteral duplication and ectopic ureteric orifice.IVU: ureteric duplication
Clinically asymptomatic unless complicatedDiagnosis: US. IVU . CT scan . MCUtreatment: Only when symptomaticEctopic ureter
The ureter opens away from the normal orifice Single ureter : 20% hemitrigone > males Duplex system 80% > females The ectopic opening: In female: urethra, vagina, Vestibule, Presents as incontinence. In male: Post urethra, seminal vesicle . Often asymptomaticUreteroceleA 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 duplicationUreterocele involving single system
ureteroceleClinical Features asymptomatic Repeated UTIs, Hematuria retention of urin
Diagnosis
uls IVU MCU CystourethroscopyUreterocel: U/S
Ureterocelecopra head appearanceby IVUtreatment
In simple ureterocele: in functioning kid, excision & reimplantation If non functioning kidney,,,nephrectomy In ectopic :if single As in simple ureteroceleTreatment,contectopic ureterocele ,duplex
Upper partial nephroureterectomy: when the upper pole is non functioning Pyelopyelostomy with distal ureterectomy of the upper pole ureter if the upper pole is functioningUreteropelvic junction obstruction
Primary : congenital Secondary : to refluxing ureterMechanism of obstruction
Intrinsic smooth muscle pathology. Adynamic segment Congenital segmental stenosis Mucosal valve , web , folds Over riding an aberrant vesselClinical presentation
Abdominal mass Episodic flank pain Pain & fever when infected The aggravating factors: Cold, diuresis , fluid over intakeDiagnosis
US IVU Diuretic IVU , Diuretic renography Renal DMSA scan ( functional). Retrograde pyelographyIVU: PUJO
pujo
Treatment
Conservative Treat the pain ,infection and follow up Indication of surgery Recurrent attack of pain, stone, rogressive hydronephrosisSurgery
Pyeloplasty, by open surgery or laparoscopic pyeloplasty Underson hynes Culp Scardino V-Y plastyTreatment,cont
Endoscopicallyantegrade or retrograde endopyelotomy
EndopyelotomyPostcaval ureter
Post caval ureter