بسم الله الرحمن الرحيم
Urology Congenital anomalies of the upper urinary tract د. نعمان هادي سعيدأستاذ مساعد - فرع الجراحةM.B.Ch.B., M.R.C.S., Ph.D.(Uro), C.A.B.(Uro), F.J.M.C.(Uro).Congenital anomalies of the upper urinary tract
Anomalies of form and fusionCongenital anomalies of the upper urinary tract
In summery comprise a diversity of abnormalities, ranging from: complete absence kidney, supernumerary Kidney aberrant location, orientation, and shape of the kidney ,aberrations of the collecting system , &blood supply.Unilateral Renal Agenesis (URA)
Incidence : 1: 1400 births Found accidentally, more frequently on the left side. Embryology :Complete absence of a ureteric bud or aborted ureteral development prevents maturation of the metanephric blastema into adult kidney tissue. *Ipsilateral adrenal agenesis is rarely encountered with URA *Other Genital anomalies are much more frequently observed Asymptomatic Diagnosis : U/S or IVU,CT scan: absent kidney on that side + compensatory hypertrophy of the contralateral kidney Treatment: no specific treatment Prognosis: no evidence that they have an increased susceptibility to other diseases Bilateral agenesis: rare, incompatible with lifeSupernumerary Kidney truly an accessory organ
Incidence very rare Symptoms It may not produce symptoms until early adulthood, if at all. Diagnosis accidentally by IVU or abdominal U/S Treatment: no treatmentANOMALIES 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 1000Associated Anomalies
ANOMALIES OF FORM AND FUSION
Crossed Renal Ectopia With and Without Fusion
Horseshoe Kidney
found in 1:1000 necropsies an is commoner in men. probably the most common of all renal fusion anomalies 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. Fusion of the renal masses early in embryonic life, so its ascent will be impeded by inferior mesenteric artery. The kidneys are low located, mal rotated and pelves lie anteriorly Symptoms When present, they are related to complications like hydronephrosis, infection, or calculus formationDiagnosis ultrasound, IVU, CT scan
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.
Cystic disease of the kidneys
Polycystic kidney disease : The kidney is one of the most common sites in the body for cysts Two types: AUTOSOMAL RECESSIVE ("INFANTILE") POLYCYSTIC KIDNEY DISEASE AUTOSOMAL DOMINANT ("ADULT") POLYCYSTIC KIDNEY DISEASECongenital cystic kidney (polycystic kidney) (Adult cystic renal disease)
Autosomal dominant, transmitted by either parents, 50% of offspring affected. Both kidneys replaced by large no. of cysts of variable size which make the kidney of large size. The cysts contain clear fluid but sometimes blood. The cysts progressively increase in size causing pressure atrophy of the renal parenchyma and pressing the ureter. 15% associated with cystic disease of liver, lung, pancreas or spleen.Etiology & Pathogenesis
The cysts occur because of defects in the development of the collecting and uriniferous tubules and in the mechanism of their joining. Blind secretory tubules that are connected to functioning glomeruli become cystic.Adult polycystic renal disease
Clinical pictures:
Rarely gives clinical manifestation before 4o years Asymptomatic: diagnosed accidentally. Pain: due to pedicle stretching, stone, ureteric obstruction, bleeding inside cyst or infection. Hematuria: cyst distention and rupture to the collecting system. Infection: renal or cyst infection causes fever, rigor and loin pain. Hypertension: in 70%, Unknown cause. Renal impairment: anorexia, headache, nausea, vomiting, drowsiness and coma. Renal enlargement: large knobby palpable kidneyDiagnosis: Family history of polycystic disease.
U/S, IVU, CT scan, MRITreatment:
Medical: (Expectant) To control infection, hypertension, pain and anemia.Renal impairment: by low protein diet and dialysis.Surgical:Rovsing’s operation (deroofing) for large cysts causing symptoms or obstruction.Stone removal.Renal failure: Renal transplantation.Infantile polycystic disease of the kidney
Rare autosomal recessive, incompatible with life. Both kidneys are large in size and replaced by large number of cysts which may obstruct labor. The condition is due to failure of ureteric bud to fuse with metanephrose.Simple (solitary) renal cyst
Common condition. single or multiple. uni or bilateral. Congenital or acquired. Usually asymptomatic. - In 10% symptomatic: pain, heaviness, infection, bleeding inside the cyst or pressure effect on the ureter causing hydronephrosis.Diagnosis
Examination: usually –ve, big cyst cause painless loin mass, & painful if complicated by bleeding or infectionU/S: echo free area (cystic lesion).KUB: soft tissue shadow.IVU: stretched calyx, filling defect or hydronephrosis.CT scan &MRI: are diagnostic.Treatment: usually no treatment needed
Symptomatic cases: Aspiration and injection of sclerosing agent.Rovsing’s operation (deroofing).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.
Congenital Anomalies of Renal pelvis & Ureter
Duplication of Renal Pelvis Incidence: 4 % More common on left side Renorenal reflux may occur from one pelvis to the other 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 & hydronephrosisTreatment :expectant
Bifid renal pelvisi
Ureteral duplication: partial and complete
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
80% are associated with a duplicated collecting system In the male, the posterior urethra is the most common site of termination, also to semenal vesicle In the female, the urethra and vestibule are the most common sites Clinical features: According to the site of orifice In females: continuous dribbling In males: urinary tract infection Diagnosis IVU, U/S, CT scan, cystoscopy Treatment: Ureteric reimplantation or implantation of one ureter to the other ureter is used Ectopic ureters may drain renal moieties (either an upper pole or a single-system kidney) that have minimal function. Therefore, upper pole partial nephrectomy (or nephrectomy of single system) is sometimes recommendedComplete ureteral duplication and ectopic ureteric orifice.
Congenital Megaureter
Grossly dilated ureter Unilateral or bilateral More common in male Clinical features: Asymptomatic, pain, repeated UTIs Lower ureter might be obstructed Sometimes associated with vesicoureteral reflux Diagnosis : IVUTreatment
Infection should be controlled Excision of the lower stenotic segment (if present) Ureteric tapering & reimplantation in to the bladder Nephroureterectomy for non functioning kidneyPostcaval (Retrocaval) ureter (Preureteral Vena Cava )
The right ureter pass behind the inferior vena cava This might causes obstruction Vascular abnormality Incidence: about 1 in 1500 Although it is congenital, most patients present at 3rd or 4th decade. Diagnosis: IVU Treatment surgical correction involves ureteral division, with relocation and ureteroureteral or ureteropelvic reanastomosis, usually with excision or bypass of the retrocaval segment, which can be aperistalticUreteroceles
Is due to congenital atresia of the ureteric orifice which causes a cystic dilatation of the intramural portion of the ureterWomen > menSometimes involves with ectopic ureter More prone to stone disease & UTIsClinical Features : asymptomaticRepeated UTIs, HematuriaDiagnosisIVU, cystoscopy, cystogramThe ‘adder head’ on excretory urography is typical.TreatmentAsymptomatic : no treatmentCystoscopy with diathermy cauterization of the holeNephrectomy in non functioning kidneyIn complicated cases, ureteral reimplantation and vesical reconstructionCobra (Adder) head appearance of ureterocele
Ureterocele involving single system Ureterocele involving duplicated ureterUreteropelvic Junction (UPJ)(PUJ) Obstruction (stenosis)
The most common cause of significant dilation of the collecting system in the fetal kidney Boys > Girls Left-sided lesions predominate 15% bilateral ETIOLOGY Intraluminal : mucosal fold that causes valve like effect. Intrinsic (intramural) interruption in the development of the circular musculature of the UPJ Extrinsic An aberrant, accessory, or early-branching lower-pole renal arteryPUJ Obstruction – gross pathology
SYMPTOMS/PRESENTATIONMost infants are asymptomatic and most children are discovered because of their symptoms Episodic flank or upper abdominal pain, sometimes associated with nausea and vomiting DIAGNOSIS U/S: hydronephrosis IVU: diagnostic , hydronephrosis with fixed stenotic segment or complete obstruction CT scan: hydronephrosis that ends abruptly
Magnetic Resonance Imaging Radionuclide Renography: to see the split function of each kidney Pressure-Flow Studies : Whitaker test