Urology Flashcards by Carol Lam

Definition of unresolved congenital hydronephrosis based on 3rd trimester antenatal ultrasound

> = 7mm

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What could a single umbilical artery signify re: the kidney?

Unilateral renal agenesis

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Incidence of unilateral renal agenesis

approx 1/750

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Potter syndrome

Bilateral renal agenesis
Incompatible with life
Pulmonary hypoplasia
Potter facies (widely set eyes, low set ears, broad/flat nose)
Limb anomalies

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What is multicystic dysplastic kidney

Usually unilateral, NOT inherited condition causing replacement of the kidney by cysts

May be due to ureteral atresia

Most common cause of abdominal mass in the newborn!

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What is the most common cause of an abdominal mass in the newborn?

Multicystic dysplastic kidney

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Risks associated with multicystic dysplastic kidney

Hypertension (0.2-1.2%)
Wilms Tumour (1/333)

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Ask-Upmark kidney

aka segmental hypoplasia

small kidneys with grooves
pts have severe hypertension
resolves w/ nephrectomy

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Anatomic kidney anomalies with increased risk of Wilms

Multicystic dysplastic kidney

Horseshoe kidney

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Complication of horseshoe kidney

Wilms tumour
stone disease
hydronephrosis (due to UPJ obstruction)

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Things that predispose to urinary reflux

kidney duplication (with an extra ureter inserting into bladder on one side)

ureterocele (cyst of the intramural portion of the distal ureter)

ureter diverticulum

neuropathic bladder (in myelomeningocele, sacral agenesis)

posterior urethral valves

renal agenesis, MCDK (predisposes to reflux on the contralateral side)

constipation

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Reflux is more likely to resolve if….

lower grade
unilateral
younger age at diagnosis

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AAP recommends to treat what grade of VUR?

Grade IV + V

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Diagnosis of UTI

Urinanalysis demonstrating pyuria and culture growing > 50,000 CFU/mL (from a cath/suprapubic aspirate)

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When do you do a VCUG?

VCUG is indicated if U/S shows hydronephrosis,
scarring, or other findings suggestive of high-grade VUR or obstructive
uropathy, and in other atypical or complex
clinical circumstances

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Is there a role for antimicrobial prophylaxis if you are concerned about VUR?

No. The evidence does not support that antimicrobial prophylaxis prevents renal scarring or UTI

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How should you counsel parents of a child with VUR?

Prompt assessment when febrile (within 48 hrs) is important - early treatment is key in preventing renal scarring!

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Complications of VUR

UTI
hypertension
CKD

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How can you manage severe VUR with frequent UTIs?

Can try prophylaxis (Not recommended though - septra or nitrofurantoin)

Deflux injections into ureter

Reimplantation of ureter

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symptoms of obstructive renal insufficiency

non-specific!
nausea, vomiting
mild abdo pain
diarrhea
poor urine stream

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walnut-sized palpable mass just above the pubic symphysis in a newborn boy

posterior urethral valves

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palpable abdominal mass in newborn

most likely multicystic dysplastic kidney

could also be hydronephrosis

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AP diameter grading system for hydronephrosis

mild = 6-9mm

moderate = 9-15mm

severe = > 15 mm

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For what degree of antenatal hydronephrosis would you perform a post-natal RBUS?

> = 10mm in the 3rd trimester

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When should do you an urgent post-natal ultrasound?

If severe bilateral hydronephrosis OR if severe single kidney hydronephrosis

When should you start a baby with antenatally detected hydronephrosis on prophylactic antibiotics after birth?

when RPD > 10mm in 3rd trimester OR SFU grade IV

When does a baby require a post-natal VCUG?

If postnatal U/S shows persistent hydronephrosis RPD >10mm

If the post-natal VCUG does not show reflux, then what do you do?

If RPD 10-15mm, stop antibiotics and repeat U/S at 3 mos & 1 yr If RPD > 15mm, do diuretic scan to look for obstruction

Gene/protein affected in autosomal recessive polycystic kidney disease

PKHD1, which encodes fibrocystin

What other organ is involved/affected in AR PCKD?

the liver - can cause bile duct proliferation and fibrosis | indistinguishable from congenital hepatic fibrosis

Presentation of AR PCKD?

bilateral flank masses during neonatal period or infancy

DDX PCKD?

Multicystic dysplastic kidney Wilms Hydronephrosis Bilateral renal vein thromboses

Most common hereditary kidney disease

Autosomal dominant polycystic kidney disease (1/500 to 1/1000)

mutations/protein causing autosomal dominant polycystic kidney disease

PKD1/2, encodes polycystin 1/2

Autosomal dominant PCKD - when does it present?

ESRD in 4th/5th decade

Extra-renal symptoms of AD polycystic kidney disease

Cysts in liver, pancreas, spleen, ovaries Intracranial aneurysms Mitral valve prolapse Hernias, intestinal diverticula Renal cell carcinoma

Repair of UPJ obstruction

pyeloplasty (stenotic segment at UPJ is excised)

Prune-belly syndrome - TRIAD

Deficient abdominal muscles Undescended testes Urinary tract abnormalities

What is the likely underlying etiology of prune belly syndrome?

severe urethral obstruction in fetal life

urinary tract abnormalities in prune belly syndrome

massive dilation of ureters / upper tracts / gigantic bladder

how do you decompress suspected PUV?

insertion of an NG tube (NOT a foley catheter - the balloon may cause bladder spasm, inducing severe obstruction)

Unfavourable prognostic factors relating to PUV

- hydronephrosis before 24 weeks GA - persistently elevated serum creatinine after decompression - cortical cysts in kidneys - incontinence > 5 yrs old

What is the bell clapper deformity?

Redundant tunica vaginalis, allowing excessive mobility of the testis - predisposing to torsion (often bilateral)

What reflex should you check if you suspect testicular torsion?

cremasteric (stroke inner thigh, testis retracts) - is almost always absent

Most common cause of testicular pain in boys aged 2-10yrs

torsion of the appendix testis

PE sign for torsion of appendix testis

blue dot sign

management torsion of the appendix testis

bed rest x 24 hrs, NSAIDs x 5 ds

what side do varicocele usually present on?

LEFT | if right-sided, should work up for abdominal mass

Why do you treat varicocele?

if significantly large, can affect fertility! need surgical excision/management

What should you do if a boy has bilateral, non-palpable testes?

Refer to specialist for possible DSD!

When should you refer a child with unilateral undescended testis for surgical management?

at 6 months CGA | will NOT descend after 4 mos

risk factor for cryptorchidism?

prematurity (testes descend at 7-8 mos GA) secondary to inguinal hernia repair

risks of cryptorchidism

``` infertility testicular cancer testicular torsion associated hernia psychologic effects of undescended testis ```

do boy with retractile testes have an increased risk of infertility or cancer?

POTENTIALLY! | 1/3 of retractile testis become "acquired undescended" and then have the same risk of infertility/cancer as undescended

how do you manage non-palpable testes?

referral for diagnostic laparotomy | U/S not sensitive to find testes if they are not located within inguinal canal/ring

what is priapism?

a penile erection at least 4 hrs in duration, unrelated to sexual stimulation can be ischemic, non-ischemic (high-flow) or intermittent

most common cause of priapism in children

sickle cell disease!

management of priapism in children with HbSS

exchange transfusion hydration pain management oxygen

cause of high-flow, non-ischemic priapism?

usually perineal injury causing caverous arterial laceration/flow

management of urethral prolapse

sitz baths, topical estrogen x 2 weeks (girls) | may require surgery if persistent

flank mass, hematuria, thrombocytopenic

renal vein thrombosis

risk factors for renal vein thrombosis

``` maternal diabetes prematurity dehydration sepsis polycythemia CHD ```

most common cause of renal stones in kids

hypercalciuria

most common types of stones in kids

calcium oxalate

increase your risk of stones

``` hypercalciuria oxaluria stasis infection cystinuria lasix ```

inhibit formation of stones

citrate phosphate magnesium high flow urine

If struvite stones, think of....

upper UTI with klebsiella or proteus

indications for surgical removal of kidney stone

``` obstruction infection (Urologic emergency!) ```

ways to avoid formation of kidney stones...

``` increase fluid intake decrease meat/protein intake drink lemonade (citrate) - inhibits stone formation limit salt consumption (decreases calcium excretion) ```

cystinosis causes....

Fanconi syndrome

cystinuria causes...

cystine stones