Urogenital Abnormalities Flashcards
Renal agenesis embryology
Lack of ureteric bud or failure to interact with metaphoric blastema
Bilateral renal agenesis S+S
Potter faces
Oligohydramnios
Pulmonary hypoplasia
May void in 1st 24 hours - thereafter no void
Majority are stillborn and demise within 48hours
Screening for bilateral renal agenesis
First degree relatives of patients with congenital solitary kidneys or infants with bilateral renal agenesis
Unilateral renal agenesis diff dx
Congenital agenesis(colon malposition in renal fossa)
Vs
Involution of dysplasic kidney or MCDK (colon usually in correct position )
Short notes unilateral renal agenesis
Males to females 2:1
More common on left
2ndary ureteric bud formation +RET mutation. Contralateral renal hypertrophy - 1/3 will have VUR
-HTN/renal insufficiency/proteinuria
Associated conditions
Males-absent ipsilateral body/tail epididilymus,vas,ampulla,sv,Ejacduct
Females-abnormal uterus(unicornate)
Supernumerary kidney notes
2distinct metaphoric diverticulum form 2 separate ureteric buds OR
Ureteric bud branches and each meets an independent metanephric blastema
Bifid/duplex ureter
Usually incidental finding
Crossed renal ectopia notes
90%fused
10%non fused to ipsilateral kidney
Likely one of the ureteric buds crosses over to contralateral blastema - usually fuses to inferior pole of normal position kidney
Male:female 2:1
Left to right 2:1
Ureter usually implants normally into trigone (ie crossed)
Horseshoe kidney notes
Metanephric blastema fuses at inferior pole resulting in an inability to ascend superior to the IMA
Males to female 2:1
Notochord needed for proper renal positioning
Sonic hedgehog gene:criteria for renal positioning and mutation may lead to fusion
Calyces directed posteriorly
1/3 have UPJO with HUN
- abnormally high ureter insertion
Variable blood supply - multiple arteries
Increased incidence of HK in Edward’s syndrome:trisomy 18
Turner’s syndrome :45XO
Increased incidence of wilms tumor in HK
Pcnl access more medial
Ectopic kidney notes
50% HUN
Urethral obstruction 50%
Significant VUR 25%
Malrotation 25%- results in anterior renal pelvis
Increased rate of concomitant genital abnormalities
Thoracic kidney
-ascent through foramen of bochdalek into posterior mediastinum by 8th week gestation
Adrenal gland in normal position
Usually incidental finding on cxr
Renal malrotation notes
Normal development : hilum is posterior with outer concavity anterior
As kidney ascends it rotates ventromedially (hilum faces aorta medically , outer concavity faces ribs laterally)
May under or over rotate
Classification of Calyceal diverticulum - outpouching of calyx/renal pelvis lined by urothelium
Type 1: upper or lower pole calyx outpouching
Type 2 : outpouching with thin connection connections directly to the renal pelvis
Calyceal diverticulum presentation
Variable
Asymptomatic
UTI
Nephrolithiasis
Milk of calcium
Hematuria
Calyceal diverticulum Mx
Dx:CT/MRI
Tx:periodically monitor asymptomatic with u/s
If symptoms/uti/stones
-diverticulum ablation and stone extraction
Posterior- PCNL
Anterior superior pole-URS
Anterior mid/lower pole - laparoscopy ablation
Megacalycosis notes
Embryologic malformation of renal papillae that results in enlarged and increased number of calyces without evidence obstruction
Males 6:1
Tx: none- no evidence for long term renal injury
Renal pseudotumor notes
Hupertrophied column of bertin
Usually between upper and middle calyces
-high suspicious on in solitary kidney with a central tumor
Dromedary hump
Solitary, renal parenchyma bulge seen laterally and usually on the left kidney a normal variant
Dx: DMSA: normal or increased isotope update
UPJ-O causes
Intrinsic : dynamic segment 2ndary to collagen deposition
poor circulature musculature
Mucosal folds
Extrinsic: aberrant, lower pole vessel cross anterior to ureter and cause kinking
Associated : contra lateral upjo
Renal dysplasia
Multicystoc kidneys
Tx upj n-o
Endopyelotomy - 85% success rate if no lower pole crossing vessel/good renal function, segment <2cm, mild:mod hun
If fails -Pyeloplasty
If failed pyeloplasty - endopyelotomy
If failed endophelotomy - ureterocalicostomy
Dietls crisis
Patients with upjo have acute renal colic secondary to diuretics eg alcohol ingestion
Bifid ureter /ureter fissus notes
2 ureters enter the renal pelvis with shared UO
Partial division of the ureteric bud prior to contact with metanephric blastema
Triple and quadruple ureters also seen
Duplicates ureters
D2 ureters enter the renal pelvis , and 2 completely separate ureters enter their own UO
Complete division of ureteric bud
Wegner’s Meyer rule
Upper pole - UO is inferior/medial + ureterocele + ectopic
S
Lower pole - UO is superior/lateral + VUR + UPJO
Rx mag 3 determine function and drainage
symptomatic + decreased function =heminephrectomy
symptomatic/not +function=correct obstruction
Asymptomatic and +- function =observe
