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Core > Pediatrics: Genitourinary, Congenital > Flashcards

Pediatrics: Genitourinary, Congenital Flashcards

1
Q

Bilateral renal agenesis is associated with…

A

Potter sequence (no fetal urine output -> pulmonary hypoplasia)

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2
Q

Unilateral renal agenesis is associated with…

A

70% of females will have genital anomalies (e.g. unicornuate uterus or rudimentary horn)

20% of males will have genital anomalies (missing epididymus/vas deferens on that side, also with a seminal vesicle cyst on that side)

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3
Q

Prenatal ultrasound of the aorta demonstrates an absent right renal artery…

A

Right renal agenesis (look for right-sided genital anomalies)

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4
Q

Potter sequence

A

An initial insult (e.g. ACE inhibitors) causes bilateral renal agenesis, which leads to oligohydramnios and pulmonary hypoplasia

Note: No kidneys -> no urine -> oligohydramnios -> unable to “breath” amniotic fluid -> pulmonary hypoplasia.

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5
Q

How can you differentiate between a congenitally absent and a surgically absent kidney?

A

Look for the “pancake adrenal sign” in the ipsilateral adrenal gland

Note: If the kidney is congenitally absent, the adrenal gland will look elongated/flattened because it was not molded by the ipsilateral kidney in utero.

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6
Q

Right adrenal gland is elongated and lying flat posteriorly (not the usual “Y” morphology)…

A

Think renal agenesis (kidney should be absent on that side)

Note: You don’t see this “pancake adrenal sign” when the kidney is surgically absent.

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7
Q

What is the most common renal fusion anomaly?

A

Horseshoe kidney

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8
Q

A horseshoe kidney is more inferior because it gets stuck on the ____ during ascent

A

Inferior mesenteric artery

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9
Q

Pts with a horseshoe kidney are at a higher risk for…

A
  • Traumatic injury by compression against the spine (kids shouldn’t play football or wrestle)
  • Kidney stones
  • Kidney infections
  • Renal cancers (due to chronic inflammation)
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10
Q

Pts with a horseshoe kidney are at a higher risk of which cancers?

A
  • Wilms
  • Transitional cell carcinoma
  • Renal carcinoid
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11
Q

Horseshoe kidney is associated with…

A

Turner’s syndrome

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12
Q

Cross fused renal ectopia

A

When one kidney crosses the midline and fuses to the bottom of the other kidney (the inferior one is the ectopic one)

Note: There will still be two ureters.

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13
Q

Crossed fused renal ectopic is associated with…

A

VACTERL

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14
Q

Pts with crossed fused renal ectopia are at a higher risk for…

A
  • Kidney stones
  • Kidney infections
  • Hydronephrosis (50%)
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15
Q

What is the most common congenital anomaly of the genitourinary tract in neonates?

A

Congenital UPJ (ureteropelvic junction) obstruction

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16
Q

Treatment for congenital UPJ obstruction

A

Surgery (pyeloplasty)

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17
Q

What should the radiologist look for prior to surgical correction of a congenital UPJ obstruction?

A

Are there vessels crossing the UPJ (this changes surgical management)

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18
Q

How can you tell the difference between a prominent extrarenal pelvis and a congenital UPJ obstruction?

A

Whitaker test (a urodynamics study combined with an antegrade pyelogram)

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19
Q

Are dilated ureters common in congenital UPJ obstructions?

A

No, congenital UPJ obstructions do not have hydroureters

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20
Q

Teenager with flank pain after drinking a lot of fluids…

A

Think congenital UPJ obstruction

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21
Q

Large, hyperechoic kidneys with loss of the corticomedullary differentiation in a child with hypertension and renal failure…

A

Think autosomal recessive polycystic kidney disease (ARPKD)

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22
Q

Clinical manifestations of autosomal recessive polycystic kidney disease

A
  • Hypertension
  • Renal failure
  • Hepatic fibrosis (portal hypertension is a common cause of death in these pts)
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23
Q

Which form of polycystic kidney disease is always associated with congenital hepatic fibrosis?

A

Autosomal recessive polycystic kidney disease

Note: Portal hypertension is a common cause of death in these pts.

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24
Q

Risk factors for neonatal renal vein thrombosis

A

Maternal diabetes, sepsis, and dehydration

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25
Q

Clinical manifestations of neonatal renal vein thrombosis

A
  • Renal enlargement (acute)
  • Renal atrophy (chronic)
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26
Q

Clinical manifestations of neonatal renal artery thrombosis

A

Severe hypertension

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27
Q

Risk factors for neonatal renal artery thrombosis

A

Umbilical artery catheters

Note: This is why the positioning of these catheters (either above or below the renal arteries) is so important.

28
Q

Prune Belly Syndrome (Eagle Barrett syndrome)

A

A malformation triad that occurs in males:

  • Deficiency of abdominal musculature (leading to the widened belly)
  • Hydroureteronephrosis
  • Cryptorchidism (bladder distention interferes with descent of testes)
29
Q

Congenital megaureter

A

Any enlarged ureter that is due to an intrinsic problem (not due to a distal obstruction)

Note: There is usually no hydronephrosis.

30
Q

Common causes of congenital megaureter

A
  • Distal dynamic segment
  • Reflux at the UVJ
  • Idiopahtic
31
Q

Retrocaval/circumcaval ureter

A

A developmental issue of the IVC that grows in a way that pins down the ureter, predisposing to partial obstructions and recurrent UTIs

32
Q

“reverse J” or “fishhook” appearance of the ureter…

A

Retrocaval ureter

Note: Higher risk of partial obstruction and recurrent UTIs.

33
Q

What is the Weigert-Meyer rule for duplicated collecting systems?

A

The upper pole inserts inferior an medially (and is prone to ureterocele formation and obstruction)

The lower pole inserts superior and lateral (and is prone to reflux)

34
Q

What clinical manifestation of a duplicated collecting system happens in females but never males?

A

Urinary incontinence (sometimes one of the ureters may insert into the vagina rather than the bladder)

35
Q

Ureterocele

A

A cystic dilatation of the intravesicular ureter secondary to an obstruction at the ureteral orifice

36
Q

Ectopic ureter

A

When the ureter inserts distal to its normal insertion on the trigone of the bladder

Note: This is often asymptomatic, but may cause urinary incontinence in females, recurrent infections, and obstruction.

37
Q

What is the most common cause of urethral obstruction in male infants?

A

Posterior urethral valves

38
Q

Posterior urethral valve

A

A fold in the posterior urethra that leads to outflow obstruction and eventual renal failure if not fixed

39
Q

What comprises the valve of a posterior urethral valve?

A

A fold of Wolfing duct tissue remnant

40
Q

VCUG of a male infant demonstrates an abrupt caliber change of the urethra from dilated proximally to normal distally…

A

Posterior urethral valve

41
Q

Prenatal ultrasound demonstrates hydronephrosis, bladder dilatation, and oligohydramnios…

A

Think posterior urethral valve (if male)

42
Q

Why are perirenal fluid collections common in the setting of a posterior urethral valve?

A

Urinary tract obstruction can eventually lead to forniceal rupture, leading to perirenal fluid

43
Q

Next step: hydronephrosis on routine prenatal ultrasound

A

Repeat ultrasound after birth

Note: Most of the time it just goes away.

44
Q

Next step: Persistent hydronephrosis on ultrasound after birth

A

Voiding cystourethrogram (to evaluate anatomy)

Note: In addition, you can also do a MAG3 scintigraphy to evaluate function/drainage.

45
Q

Causes of non-obstructive hydronephrosis in male infants

A
  • Vesicoureteral reflux
  • Primary megaureter
  • Prune belly syndrome
46
Q

Causes of obstructive hydronephrosis in male infants

A
  • Posterior urethral valve
  • UPJ obstruction
  • Ureteral ectopia
47
Q

Pathophysiology of vesicoureteral reflux

A

Ureter inserts on the bladder at an unusual angle (e.g. 90 degrees horizontal), rather than the normal oblique angle that allows a functional “valve” to form

48
Q

Grading system for vesicoureteral reflux

A
  • 1 (reflux halfway up ureter)
  • 2 (reflux to non-dilated renal pelvis)
  • 3 (hydronephrosis)
  • 4 (mildly tortuous ureter)
  • 5 (severely tortuous ureter)
49
Q

Echogenic mound adjacent to the ureterovesicular junction in a pt with a history of vesicoureteral reflux…

A

Think “Reflux” injection (treatment for vesicoureteral reflux by injecting a synthetic material near the UVJ to create an artificial partial obstruction to minimize reflux)

50
Q

Complications of vesicoureteral reflux

A
  • Recurrent UTIs
  • Renal scarring leading to chronic renal failure and hypertension
51
Q

Common causes of secondary vesicoureteral reflux

A
  • Ureterocele
  • Posterior urethral valve
  • Neurogenic bladder
52
Q

How can you differentiate primary from secondary vesicoureteral reflux on imaging?

A

Secondary VUR is usually associated with a “hutch” diverticulum (a bladder diverticulum at or adjacent to the ureterovesicular junction)

53
Q

Treatment for primary vesicoureteral reflux

A
  • None (most resolve by age 5-6)
  • Reflux injection (injection of a synthetic material near the UVJ to create an artificial partial obstruction, limiting reflux)
54
Q

Treatment for secondary vesicoureteral reflux

A

Surgery

55
Q

Hutch diverticulum

A

A bladder diverticulum at or just above the ureterovesicular junction that is associated with secondary vesicoureteral reflux. Best seen on voiding cystogram, harder to see on US.

Note: These are often surgically resected.

56
Q

Urachus

A

A connection between the bladder and the umbilicus (normally atrophies to become the umbilical ligament)

57
Q

What are the types of urachal remnant?

A
  • Urachal diverticulum
  • Urachal cyst
  • Urachal sinus
  • Patent urachus
58
Q

What is the most common complication of a urachal remnant?

A

Infection

59
Q

Are urachal remnants more common in males or females?

A

Males (2:1)

60
Q

What is the most common cancer to develop in a urachal remnant?

A

Adenocarcinoma (90%)

61
Q

Mass arising from a midline bladder structure…

A

Think adenocarcinoma (most common cancer to arise in a urachal remnant)

62
Q

Bladder exstrophy

A

Herniation of the urianry bladder through a hole in the anterior infra-umbilical abdominal wall

63
Q

Bladder exstrophy is associated with an increased risk of…

A

Malignancy (in the extruded bladder)

64
Q

What type of cancer is most common within the extruded bladder of bladder exstrophy?

A

Adenocarcinoma

Note: This is the same as with urachal remnants.

65
Q

Unfused pubic bones on radiograph…

A

Bladder exstrophy

Note: This is the “manta ray sign” on an AP pelvic radiograph.

66
Q

Cloacal malformation

A

When the GI tract and GU tract both drain into a common opening (only happens in females)

67
Q

Causes of neurogenic bladder in a child

A

Spinal dysraphism (tethered spinal cord, sacral agenesis, etc.)

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