Urinary congenital anomalies Flashcards
What are the types of cystic diseases of the kidney?
GENETIC
- adult polycystic kidney disease (autosomal dominant)
- infantile polycystic kidney disease (autosomal recessive)
NON-GENETIC
- simple cyst
- multicystic kidney
- medullary sponge kidney
ACQUIRED
- develops in long-term dialysis
What is the investigation of choice of a mass in the kidney discovered incidentally & is asymptomatic?
SIMPLE CYST OF KIDNEY
- US or CT
- a cyst in the hilum of the kidney (PARAPELVIC CYST) presses on the pelviureteric junction & causes obstruction
What is the cause of pain in a simple cyst of the kidney?
hemorrhage into the cyst & infection
How is the simple cyst of the kidney treated?
LAPAROSCOPIC KIRWIN’S OPERATION
- kidney is exposed -> cyst is aspirated & a portion of the cyst wall is removed & cavity is filled with perinephric fat
if cyst is in one of the poles -> partial nephrectomy
A hereditary autosomal dominant disease of the kidney that is potentially lethal & does not manifest before 30 is?
POLYCYSTIC KIDNEY DISEASE
- kidneys are huge -> cysts distort the renal capsule -> cysts contain clear fluid or coagulated blood
- may be associated with cystic disease of the liver
What are the clinical features of polycystic kidney disease?
- RENAL ENLARGEMENT
- PAIN -> dull loin pain
- RECURRENT HEMATURIA -> rupture of a cyst into renal pelvis
- PYELONEPHRITIS -> urinary stasis
- HYPERTENSION -> in up to 75%
- UREMIA -> sign of end-stage renal failure, begins suddenly during middle life
What radiological investigation should be used in polycystic kidney disease?
CT
- multiple cysts in both kidneys
- sometimes cysts in liver & other organs
do FNA is there is doubt about the diagnosis
IVP
- renal shadows are enlarged in all directions
- renal pelvis is compressed & elongated
- calyces are stretched over the cysts (big, bizarre & bilateral)
What is the treatment of polycystic kidney disease?
- hemodialysis & renal transplant
- infection, anemia, hypertension & disturbances of calcium metabolism need appropriate treatment
What are the clinical features of a horseshoe kidney?
- FIXED NON MOBILE FIRM MASS IN THE MIDLINE AT L4
- picture of complications -> infection, stone formation & hydronephrosis
- ectopic kidneys fused at their lower poles -> lying in front of the fourth lumbar vertebrae & great vessels
- ureters angulate as they pass over the fused isthmus -> urinary stasis with infection & nephrolithiasis
What are the diagnostic radiological investigations used in case of horseshoe kidney?
- CT -> diagnostic
- IVP -> medialisation of lower calyces & curving of ureter like a FLOWER VASE
How should a horseshoe kidney be treated?
- symptomatic treatment
- division of the isthmus between kidneys ONLY in surgery for abdominal aortic dissection
What is crossed dystopia?
Unilateral fusion of kidneys
- both kidneys are fused at one loin
- the ureter of the lower kidney crosses the midline to enter the bladder on the other side
- can be a unilateral long kidney or an S-shaped kidney
What are the clinical features of duplication of the renal pelvis or ureter?
- unilateral & left sided
- infection
- calculus formation
- pelviureteric junction obstruction
- children -> refluxing lower pole ureter & upper pole ureter terminating in ureterocele
- female -> ectopic ureter opens into urethra OR vagina (voids normally but dribbles urine since young age)
Which method of investigation is diagnostic for duplication of the renal pelvis or ureter?
IVU -> diagnostic
- US -> look for complications
- cystoscopy -> double ureteric orifices on the same
- DTPA scan -> see function
How should duplication of the renal pelvis or ureter be treated?
- asymptomatic -> no treatment
- severely diseased or atrophic -> partial nephrectomy
- refluxing ureter -> reimplanting
- ectopic ureter in female -> frequently drains hydronephrotic & chronical infected renal tissue -> best excised
