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
What is the cause of a congenital megaureter?
functional obstruction at the lower end of the ureter -> leads to progressive dilatation & infection
- reflux is inevitable if ureteric orfice is opened endoscopically
- infection or deteriorating function -> requires refashioning & reimplantation
right ureter passing behind vena cava instead of lying to the right of it is called?
POST-CAVAL URETER
- if it causes obstructive symptoms -> ureter can be divided & rejoined in front of the cava
a cystic enlargement of the intramural ureter is thought to result from?
CONGENITAL ATRESIA OF THE URETERIC ORIFICE -> Ureterocele
- usually present from childhood but is unrecognized till adult life
What investigations are done for a suspected ureterocele? How is it treated?
IVP -> adder head on excretory urography
CYSTOSCOPE -> translucent cyst enlarging & collapsing as urine flows in from above
Treated by -> endoscopic diathermy (for symptomatic ureterocele)
-> nephrectomy (in advanced unilateral cases with hydronephrosis or pyonephrosis)
What is ectopia vesica?
congenital anomaly characterized by
- absent lower anterior abdominal wall, anterior bladder wall, wide separation of the symphysis pubis
complications -> severe UTI & metaplasia (PRECANCEROUS)
What is the difference between a congenital & acquired diverticulum?
Congenital Acquired
- less common - more common
- solitary - multiple
- true (contains muscle) - false (mucosa only) —> seen due to bladder neck obstruction
What are the features of urinary bladder diverticulum?
- clinically -> double micturation (second is predisposed by change of posture)
- complications -> stasis -> recurrent infections, stone formation, back pressure, & metaplasia
- investigations -> cystoscope & cystography
- treatment -> diverticulotomy & deal with the cause
External meatus opening on the underside of the penis or perineum with a poorly developed prepuce (hooded prepuce) is known as?
HYPOSPADIAS
- has a ventral deformity of the erect penis due to CHORDEE
types
- glanular -> proximal to normal site
- coronal -> at junction of the underside of glans & body of penis
- penile & penscrotal -> underside of the penile shaft
- perineal ->makes determination of child’s sex difficult
urethral opening on the dorsum of the penis & upward curvature of the penis is known as?
epispadias
What is phimosis?
- congenital contraction of the prepuce -> pinhole opening
What is paraphimosis?
compression on the shaft by retracted prepuce
A congenital valve that leads to bladder outlet obstruction is treated by?
- immediate relieve of the obstruction -> catheter
- definitive line -> endoscopic resection
POSTERIOR URETHRAL VALVE
