• MALROTATION: happens when alignment of → Excess fibrous tissue encasing the
kidney, as it assumes its final position in the pelvis, UPJ, and upper “renal” fossa NOT EXACT ureterhydronephrosis • NORMAL: orients itself so that the calyces point → Vascular compression from an laterally and the pelvis faces medially. accessory or main renal artery or • association with Turner syndrome distortion of the upper ureter or UPJ • M>F (2:1) intermittent obstruction. • Embryology. → Diagnosis 6th week: medial rotation of the diagnosis should be considered collecting system = simultaneous with in renal calculus in an abnormal renal migration completes this location process by rotating 90 degrees toward confirmation from a renal the midline by the time ascent is sonogram, CT, MRU, or complete at the end of 9 weeks’ retrograde pyelogram gestation. rotation is the result of unequal branching of successive orders of the ANOMALIES OF RENAL VASCULATURE budding ureteral tree 1. Aberrant, Accessory, or Multiple Vessels • kidney and renal pelvis normally rotate 90 2. Renal Artery Aneurysm degrees ventromedially during ascent 3. Renal Arteriovenous Fistula
1. Aberrant, Accessory, or Multiple Vessels
• Aberrant or anomalous vessels are arteries that originate from vessels other than the aorta or main renal artery • In cases of isolated malrotation, other • Accessory vessels denotes two or more arterial characteristic features may be present branches supplying the same renal segment (distortion, fibrous matting) • Multiple renal arteries are any kidney supplied • Phases of medial and reverse rotation according by more than one vessel to the position of the renal pelvis: • 5 segment of renal parenchyma based on 1. Ventral Position – MC vascular supply: apical, upper, middle, lower, 2. Ventromedial Position and posterior. 3. Dorsal Position -rarest position • Main renal artery divides into anterior and 4. Lateral Position posterior branch. • The anterior branch almost always supplies the upper, middle, and lower segments of the kidney. • posterior branch invariably supplies the posterior and lower segments • Symptoms might result from inadequate urinary drainage • Constriction in infundibulum, a major calyx, or the UPJ Pain and hematuria secondary to • Symptoms hydronephrosis, UTI, or calculus → No specific symptoms • Diagnosis: 3D power Doppler ultrasonography, CT, or MRI 2. Renal Artery Aneurysm • lesion is usually located in the upper pole (45% • Types: saccular, fusiform, dissecting, and of cases), midportion (30%) or in the lower pole arteriovenous (25%) • Saccular aneurysm - localized outpouching • Symptoms - based on the age and size of the communicating with the arterial lumen by a AVF narrow or wide opening; MC (93%) • Diagnosis - Three-dimensional Doppler • Fusiform - aneurysm located at the bifurcation of sonography and MRA are accurate and the main renal artery and its anterior and noninvasive tests; selective renal arteriography posterior divisions, or at one of the more distal or digital subtraction angiography is the most branching; considered to be congenital in origin definitive method • Acquired aneurysms - located anywhere and may • Cirsoid appearance with multiple small, result from inflammatory, traumatic, or tortuous channels; prompt venous filling; and degenerative factors. enlarged renal, and possibly gonadal vein are • localized defect in the internal elastic tissue and pathognomonic for arteriovenous malformation the media allows the vessel to dilate at that point. (AVM) • true aneurysm - composed of most of the layers • progressive alterations in the cardiovascular that make up the normal artery. system, often dictates surgical intervention. • Symptoms - silent in children, some manifest at a • congenital variant rarely behaves like acquired, later age, because the size of the aneurysm which may disappear spontaneously after increases with time. several months. • Pain (15%), hematuria (microscopic and • Nephrectomy, partial nephrectomy, vascular macroscopic) (30%), and hypertension (55%) ligation, selective embolization, and balloon secondary to compression of adjacent catheter occlusion have been used to obliterate parenchyma or to altered blood flow within the the fistula. vascular tree • Diagnosis is suspected when a pulsatile mass is ANOMALIES OF THE COLLECTING SYSTEM palpated in the region of the renal hilum or when 1. Calyceal Diverticulum a bruit is heard on abdominal auscultation. A 2. Hydrocalycosis wreathlike calcification in the area of the renal 3. Megacalycosis artery or its branches (30%) is highly suggestive 4. Pseudotumors of the Kidney • Diagnosis: color Doppler sonography, spiral CT, 5. Infundibulopelvic Stenosis 3D MRA, or digital subtraction arteriography 6. Bifid Pelvis • Intervention is indicated for RAAs larger than 2.0 cm, symptomatic individuals and high risk for 1. Calyceal Diverticulum rupture • cystic cavity within the kidney lined by transitional epithelium that communicates with a calyx or less 3. Renal Arteriovenous Fistula commonly with the renal pelvis through a narrow • Two types: isthmus a) Congenital - Fewer than 25%; present at • may be multiple birth or to result from a congenital • MC in the upper calyx aneurysm eroding into an adjacent vein • Type I diverticula - Most diverticula occur adjacent b) Acquired - secondary to trauma, to an upper or, occasionally, a lower pole calyx. inflammation, renal surgery, or • Type II diverticula are larger, communicate with the percutaneous needle biopsy) accounting for renal pelvis, and tend to be symptomatic the increase in incidence. • Congenital and acquired factors have been • identifiable by their cirsoid configuration and suggested to explain the formation of calyceal multiple communications between the main or diverticula. segmental renal arteries and the venous • tend to distend progressively with trapped urine channels • Infection, milk of calcium or true stone formation • Although congenital, rarely present clinically are complications of stasis or obstruction before 3rd or 4th decade. Symptoms (Hematuria, pain, and UTI) • F>M (3:1) • Diagnosis: ultrasonography but is confirmed on CT • right > left scan or MRU • Asymptomatic patients do not require treatment but should be followed periodically with ultrasonography • Indications for surgery (percutaneous ablation, marsupialization of the diverticulum and fulguration of the epithelial lining) included enlargement of the diverticulum associated with pain or infection, abscess formation, urosepsis, and symptomatic calculus formation