Congenital Anomalies of the Upper Urinary Tract (Lec 2) 2024 PDF

Urosurgery [Congenital anomalies] 1 Urosurgery [Congenital anomalies] 2 Urosurgery [Congenital anomalies] Congenital anomalies of the upper urinary tract:...

Urosurgery [Congenital anomalies] 1 Urosurgery [Congenital anomalies] 2 Urosurgery [Congenital anomalies] Congenital anomalies of the upper urinary tract: ¿ Anomalies of number ® Agenesis: Unilateral Bilateral ® Supernumerary kidney ¿ Anomalies of volume and structure ® Hypoplasia ® Multicystic kidney Criteria defining PCK ; ® Polycystic kidney 15-30 year age : at least one cyst in each kidney -Infantile 30-60 year age : 2 or more cysts in each kidney -Adult -Medullary cystic disease ¿ Anomalies of ascent ® Simple ectopia ® Cephalad ectopia ® Thoracic kidney ¿ Anomalies of form and fusion Crossed ectopia with and without fusion ® Unilateral fused kidney ® Sigmoid or S-shaped kidney ® Lump kidney ® L-shaped kidney ® Horseshoe kidney ¿ Anomalies of rotation ¿ Anomalies of the collecting system Calyx and infundibulum ® Calyceal diverticulum ® Hydrocalyx ® Megacalycosis Pelvis ® Extrarenal pelvis ® Bifid pelvis ¿ Anomalies of renal vasculature ® Aberrant, accessory, or multiple vessels ® Renal artery aneurysm ® Arteriovenous fistula 3 Urosurgery [Congenital anomalies] Unilateral Renal Agenesis (URA) o Incidence : 1: 1400 births o Found accidentally, more frequently on the left side. o Embryology :Complete absence of a ureteric bud or aborted ureteral development prevents maturation of the metanephric blastema into adult kidney tissue. o *Ipsilateral adrenal agenesis is rarely encountered with URA o *Other Genital anomalies are much more frequently observed o Asymptomatic o Diagnosis : U/S or IVU,CT scan: absent kidney on that side + compensatory hypertrophy of the contralateral kidney o Treatment : no specific treatment Bilateral agenesis: rare, incompatible with life ANOMALIES OF ASCENT: Simple Renal Ectopia When the mature kidney fails to reach its normal location in the " renal fossa " Incidence : The incidence is 1 in 1000 o Associated Anomalies : contralateral agenesis / Hydronephrosis ( 25%) o Clinical features : asymptomatic o Diagnosis : U/S, IVU, CT scan o Prognosis :no more susceptible to disease, but hydronephrosis or urinary calculus formation is more 4 Urosurgery [Congenital anomalies] ANOMALIES OF FORM AND FUSION Crossed Renal Ectopia With and Without Fusion; Horseshoe Kidney ` o found in 1:1000 necropsies an is commoner in men. o probably the most common of all renal fusion anomalies o The anomaly consists of two distinct renal masses lying vertically on either side of the midline and connected at their respective lower poles by a parenchymatous or fibrous isthmus that crosses the midplane of the body. o It is impeded by inferior mesenteric artery , lying in front of the fourth lumbar vertebra o The blood supply of the horseshoe comes unpredictably from nearby major vessels. o The kidneys are low located, mal rotated and pelvis lie anteriorly o Symptom: Horseshoe kidneys are liable to pelviureteric obstruction, infection and stone o Diagnosis ultrasound, IVU, CT scan o Treatment: -Medical: pain relief and to control infection -Surgical: stone removal, PUJ stenosis correction and isthmus division in cases of operations on the aorta Prognosis usually they have normal life 5 Urosurgery [Congenital anomalies] Polycystic kidney disease : The kidney is one of the most common sites in the body for cysts Two types: o AUTOSOMAL RECESSIVE ("INFANTILE") POLYCYSTIC KIDNEY DISEASE o AUTOSOMAL DOMINANT ("ADULT") POLYCYSTIC KIDNEY DISEASE Congenital cystic kidney (polycystic kidney) APKD o Autosomal dominant, 50% of offspring affected. o Both kidneys replaced by large no. of cysts of variable size which make the kidney of large size. o The cysts contain clear fluid but sometimes blood. o The cysts progressively increase in size causing pressure atrophy of the renal parenchyma and pressing the ureter. o 15% associated with cystic disease of liver, lung, pancreas or spleen. Clinical pictures: o Rarely gives clinical manifestation before 4o years o Asymptomatic: diagnosed accidentally. o Pain:?? o Hematuria: cyst distention and rupture to the collecting system. o Infection;: o Hypertension: in 70%, Unknown cause. o Renal impairment: Diagnosis: Family history of polycystic disease , U/S, IVU, CT scan, MRI Treatment: Radiology Medical: (Expectant) o To control infection, hypertension, pain and anemia. o Renal impairment: by low protein diet and dialysis. Surgical: o deroofing for large cysts causing symptoms or obstruction. o Stone removal. Renal failure: Renal transplantation 6 Urosurgery [Congenital anomalies] Infantile polycystic disease of the kidney o Rare autosomal recessive, incompatible with life. o Both kidneys are large in size and replaced by large number of cysts which may obstruct labor. o The condition is due to failure of ureteric bud to fuse with metanephrose Simple (solitary) renal cyst o Common condition. o single or multiple. o uni or bilateral. o Usually asymptomatic. o Diagnosed on ultrasound o Rarely require treatment In 10% symptomatic: pain, heaviness, infection, bleeding inside the cyst or pressure effect on the ureter causing hydronephrosis. Treatment: usually no treatment needed Symptomatic cases: o Aspiration and injection of sclerosing agent. o Rovsing’s operation (deroofing) o Partial or total nephrectomy in destructed kidney. N.B. Malignant cyst: radical nephrectomy. N.B. Hydatid cyst aspiration is contraindicated because of anaphylaxis and dissemination. ý DDx?? Congenital Anomalies of Renal pelvis & Ureter Duplication of Renal Pelvis Incidence: 4% More common on left side Duplication of the ureter Incidence : 3% Usually the ureters fuse & have common orifice in the bladder although they may open independently in which case the ureters cross each other so that the ureter that drain the upper pelvis open below (more distally) in the bladder & vise versa. Clinical features : usually asymptomatic More prone to infections, calculus disease & hydronephrosis Treatment: expectant 7 Urosurgery [Congenital anomalies] -Partial duplication: Is more common. Two ureters draining single kidney for variable length, then unite together before entering the bladder in one ureteric orifice. Rarely the lower part is duplicated as inverted Y ureter. -Complete duplication: Less frequent, The whole ureter is duplicated, and each one opens in separate orifice in the bladder. The ureter draining the upper part opens more distally in the bladder. Ectopic Ureters o 80% are associated with a duplicated collecting system o In the male, the posterior urethra is the most common site of termination o In the female, the urethra and vestibule are the most common sites Clinical features: According to the site of orifice o In females: continuous dribbling o In males: urinary tract infection Diagnosis: IVU, U/S, CT scan, cystoscopy Treatment: Ureteric reimplantation or upper pole partial nephrectomy (or nephrectomy of single system)>>> if they have minimal function Congenital Megaureter. > 7 mm o Grossly dilated ureter o Unilateral or bilateral o More common in male Clinical features: o Asymptomatic, pain, repeated UTIs o Lower ureter might be obstructed o Sometimes associated with vesicoureteral reflux Diagnosis: IVU Treatment o Infection should be controlled o Excision of the lower stenotic segment (if present)/ Ureteric tapering & reimplantation in to the bladder o Nephroureterectomy for non functioning kidney 8 Urosurgery [Congenital anomalies] Postcaval (Retrocaval) ureter (Preureteral Vena Cava ) The right ureter pass behind the inferior vena cava This might causes obstruction Ureteroceles o Is due to congenital atresia of the ureteric orifice which causes a cystic dilatation of the intramural portion of the ureter o Women > men o Sometimes involves with ectopic ureter o More prone to stone disease & UTIs Clinical Features : Asymptomatic Repeated UTIs, Hematuria Diagnosis: IVU {Cobra (Adder) head appearance of ureterocele} , cystoscopy , cystogram Treatment: o Asymptomatic : no treatment o Cystoscopy with diathermy cauterization of the hole o Nephrectomy in non functioning kidney In complicated cases, ureteral reimplantation and vesical reconstruction 9 Urosurgery [Congenital anomalies] Ureteropelvic Junction (UPJ)(PUJ) Obstruction (stenosis) o The most common cause of significant dilation of the collecting system in the fetal kidney o Boys > Girls o Left-sided lesions predominate o 15% bilateral ETIOLOGY o Intraluminal : mucosal fold o Intrinsic (intramural) interruption in the development of the circular musculature of the UPJ o Extrinsic An aberrant, accessory, or early-branching lower-pole renal artery SYMPTOMS/PRESENTATION o asymptomatic o Episodic flank or upper abdominal pain, sometimes associated with nausea and vomiting DIAGNOSIS o U/S: hydronephrosis o IVU: diagnostic , hydronephrosis with fixed stenotic segment or complete obstruction o CT scan: hydronephrosis that ends abruptly o Magnetic Resonance Imaging o Radionuclide Renography: to see the split function of each kidney o Pressure-Flow Studies: Whitaker test Treatment: o Medical: control infection and pain. o Surgical: o Indications for surgery: o 1-progressive hydronephrosis. o 2- UTI, and symptomatic patients. o 3- Severe hydronephrotic malfunctioning kidney. 10 Urosurgery [Congenital anomalies] Surgical: o Open & laparoscopic surgical techniques Anderson-Hynes dismembered pyeloplasty: excision of the pathologic UPJ & appropriate reanastamosis or flap technique o Endoscopic Approaches balloon dilatation Antegrade endopyelotomy o Nephrectomy for non functioning kidney Anderson-Hynes dismembered pyeloplasty 11