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
What are the risk factors for developing an inguinal hernia?
Males
Premature babies
Family history
What are the signs of inguinal hernia?
Parents will notice an intermittent swelling in the groin or scrotum
What signs indicate the an inguinal hernia has become strangulated?
Irreducible
Hard
Tender
Also systemic features such as irritability and vomiting
How do we treat a strangulated hernia?
Sedation, analgesia and expert manipulation allow reduction, followed by surgical repair.
What are the complications of a strangulated inguinal hernia?
Bowel ischaemia and necrosis
What is a hydrocele?
Accumulation of fluid within the tunica vaginalis.
What are the two types of hydrocele?
Communicating: caused by patent processus vaginalis allowing peritoneal fluid to drain into the scrotum.
Non-communicating: caused by excessive fluid production within the tunica vaginalis
What are the causes of hydrocele?
Failure of processus vaginalis to obliterate at birth
Epididymo-orchitits
Testicular torsion
Testicular tumours
What are the clinical features of hydrocele?
Soft, non-tender swelling of the hemi-scrotum, usually anterior to and below the testicle.
Able to get above the mass
Transilluminates with a pen torch
Testis may be difficult to palpate if hydrocele is large
What investigations should be offered to someone who presents with a hydrocele?
Clinical examination may be enough to diagnose, however ultrasound should be done if there is any doubt or if the underlying testis cannot be palpated.
How do we manage an infant with a hydrocele?
Should be repaired surgically if they do not spontaneously resolve by the age of 1-2 years
(in adults, further investigation with USS should be done to exclude causes such as tumours)
What is the peak age of testicular torsion?
Neonatal period and early teens
What is testicular torsion?
Inadequate fixation to the tunica vaginalis allows the testis to rotate and occlude its vascular supply. This results in testicular ischaemia and necrosis.
What are the clinical features of testicular torsion?
Sudden onset, severe testicular pain
Scrotal swelling
On examination testicle is tender and pain is not alleviated by elevation (unlike in acute epididymo-orchitis)
What is the differential diagnosis for the signs and symptoms of testicular torsion?
Torsion of testicular appendix - hydatid of Morgagni
Epididymo-orchitis
Idiopathic scrotal oedema
How do we manage testicular torsion?
Surgical exploration should not be delayed
Testicular fixation of both testes
What is the deformity that increases the likelihood of developing testicular torsion?
Bell clapper deformity (where tunica vaginalis surrounds both side of testicle which makes it less fixed in one direction)
Signs include increased mobility and transverse lie
What are the most common penile abnormalities?
Hypospadias
Phimosis
What is the definition of hypospadias?
A spectrum of congenital abnormalities of the position of the urethral meatus, ranging from mild displacement to urethral opening in the scrotum.
What are the clinical features of hypospadias?
Depends on severity:
Urethral meatus opens on the ventral surface of the penis
Foreskin is incompletely closed ventrally giving a dorsal hooded appearance.
Skin tethering to hypoplastic urethra
Splayed columns of spongiosum tissue distal to the meatus
Chordee - ventral curvature of the penis
Severe forms may lead to incontinence and infertility
What proportion of the openings in hypospadias are located distally?
75%
What is the incidence of hypospadias?
1 in 300 male births
Why must infants with hypospadias not be circumcised for cultural reasons?
Because the foreskin is used in surgical correction as skin graft.
How do we manage a patient with hypospadias?
Urethroplasty
Penile reconstruction
What is phimosis?
Adhesions of the foreskin to the glans penis due to a congenital narrowing of the opening of the foreskin so that it cannot be retracted. Diagnosed after the age of 3 years as before this point it is normal for the foreskin to adhere to the glans.
What is paraphimosis?
This is where the foreskin cannot be returned to its original position once it has been retracted.
How do we manage a patient with phimosis?
Mild cases may respond to gentle periodic retraction
Topical steroids can be used to thin the skin and therefore make it more pliable and retractable
More severe cases will need surgery and circumcision
What are the complications of phimosis?
Balanitis
Balanoposthitis
What are the conditions that increase the chance of developing phimosis?
STIs
Eczema
Psoriasis
Lichen planus (balanitis xerotica obliterans)
Lichen sclerosus
Is circumcision for religious purposes available on the NHS?
No
What are the medical indications for circumcision?
Phimosis
Recurrent balanitis
Balanitis xerotica obliterans (lichen sclerosus of the penis)
Paraphimosis
Surgical correction of hypospadias.
What is balanitis xerotica obliterans?
Lichen sclerosus of the penis. Causes a thickened, scarred, white prepuce that is fixed to the glans.
What are the medical benefits of routine circumcision?
Reduces the risk of penile cancer
Reduces the risk of UTI
Reduces the risk of acquiring sexually transmitted infections including HIV
What are the complications of circumcision?
Haemorrhage
Infection
Damage to the glans
Why do prepubescent girls have an increased risk of vulvovaginitis/bacterial vaginosis?
Lack of labial development
Low oestrogen levels
More alkaline pH
What must be considered in a child with vulvovaginitis?
Sexual abuse
Foreign objects
