RENAL AND GENITOURINARY ABNORMALITIES Flashcards
What are the congenital abnormalities affecting the kidneys?
Complete bilateral renal agenesis
Abnormalities of ascent and rotation
Duplex kidney
Horseshoe kidney
Cystic disease of the kidney
Renal dysplasia
What is renal agenesis?
Absence of both kidneys. Results in Potter syndrome (or oligohydramnios syndrome, due to lack of fetal urine) where oligohydramnios leads to lung hypoplasia and postural deformities. Results in still birth in almost all cases.
What is Duplex kidney?
This is where there are two ‘ureters’ emerging from one kidney. The upper pole ureter (emerging from higher up the kidney) may be ectopic, draining into the urethra or vagina. The lower pole normally goes to the bladder but will often reflux.
What is horseshoe kidney?
This is where is a single elongated kidney as the two kidneys are fused together by an isthmus. Symptoms include nausea, abdominal discomfort, frequent renal stones and increased susceptibility to UTI.
What are the 3 most common conditions associated with cystic kidneys?
Autosomal recessive polycystic kidney disease - seen in children and infants
Autosomal dominant polycystic kidney disease - seen in adults
Tuberous sclerosis
What are the possible sites of obstructive lesions of the urinary tract?
Pelviureteric (PU) junction
Vesicoureteric (VU) junction
Bladder
Urethra - posterior urethral valves
What are the presenting complaints of an obstructive lesions of the urinary tract?
UTI
Abdominal or loin pain
Haematuria
A palpable kidney or bladder
What may be seen on the scan of someone with obstructive lesions of the urinary tract?
Hydronephrosis (PU junction obstruction)
Hydroureters (VU junction obstruction)
Full bladder
What is vesicoureteric reflux?
Condition in which urine refluxes up the ureter during voiding, predisposing to infection and exposing kidneys to bacteria and high pressure.
What are the short term complications of ureteric reflux?
Pyelonephritis
What are the long term complications of ureteric reflux?
Scarring - reflux nephropathy
CKD
Hypertension
What is primary vesicoureteric reflux?
This is when the reflux is caused by a developmental anomaly of the vesicoureteric junction.
In a normal bladder, the ureters enter the bladder wall at an angle with a large section of the ureter with the muscular wall which is compressed with bladder contraction.
In primary VUR, the ureters enter the bladder perpendicularly, hence the segment of ureter within the bladder wall is abnormally short and there is inadequate ureter closure.
How do we grade the severity of vesicoureteric reflux?
Mild Grade I - refluxed urine does not quite enter kidney
Moderate Grade III - Moderate dilatation of the ureter and renal pelvis due to reflux
Severe Grade V - Gross dilatation of the ureter, renal pelvis and calyces of kidney.
What are the features of vesicoureteric reflux?
Often asymptomatic and picked up on ultrasound
UTI
Pyelonephritis
How do we definitively diagnose vesicoureteric reflux?
Micturating cystourethrogram
How do we manage vesicoureteric reflux?
Mild VUR resolves spontaneously
Surgery is indicated if there are recurrent UTIs or grade IV-V
Prophylactic trimethoprim can be given to prevent infection in more severe cases.
SIblings of children should be investigated as there is a strong genetic component.
What are the different types of undescended testes (cryptorchidism)?
Retractile - normally descended with exaggerated cremasteric reflex. Can be coaxed in the scrotum. May become ascended and require monitoring until puberty
Arrested descent - found along the normal pathway but not as far the scrotum
Ectopic - deviated from normal pathways
When are the testes routinely examined?
At birth and at 6-8 week check
How should someone with an impalpable testicle be managed?
Referred to surgeon for orchidopexy (moving testicle down into scrotum and fixing it there) between the age of 1 and 2 years (normally at about 18 months)
Orchidectomy is indicated for unilateral intra-abdominal testis that is not amenable to orchidopexy.
What might bilateral undescended testis in the newborn indicate?
Congenital adrenal hyperplasia
What further investigations might be done for a neonate with undescended testes?
USS +/- MRI
Laparoscopy
Endocrine investigations
What are the complications of untreated undescended testis?
Increased risk of malignancy (40 times more likely to develop seminoma)
Increased of risk of subfertility
Increased risk of torsion
During descent of the testis what structure does the testis take with it into the scrotum?
The processus vaginalis. This normally becomes obliterated at or around birth.
What happens if the processus vaginalis fails to become obliterated at birth?
Inguinal hernia or hydrocele