A/2. Developmental disorders of the kidney, the ureter and the bladder

Question 1

Renal agenesis

Answer 1

• (unilateral/bilateral)
• Agenesis means absent organ due to absent primordial tissue

Question 2

unilateral renal agenesis symptoms

Answer 2

If unilateral, majority of cases are asymptomatic with compensatory hypertrophy of the contralateral kidney;
but abnormalities of the contralateral kidney are
common

Question 3

Renal hypoplasia

Answer 3

Kidney is smaller than normal (hypoplastic), usually structurally normal.

• Associated with reduced number of functional nephrons, with increased risk of developing kidney failure

Question 4

DEVELOPMENTAL DISORDERS OF THE KIDNEY

Answer 4

1. Renal agensis
2. renal hypoplasia
3. Multicystic
   dysplastic kidney
4. horseshoe kidney
5. potter sequence

Question 5

Multicystic
dysplastic kidney - pathology

Answer 5

Ureteric bud fails to induce differenciation of metanephric mesenchyme -> non-functional kidney consisiting of cysts and Connective tissue

• Predominantly non-hereditary and unilateral; bilateral leads to potter sequence

Question 6

multicystic dysplastic kidney- it unilateral or bilateral?

Answer 6

• Predominantly non-hereditary and unilateral;
• bilateral leads to potter sequence

Question 7

unilateral multicystic dysplastic kidney is it hereditary?

Answer 7

predominanly Non-hereditary

Question 8

Horseshoe kidney def

Answer 8

Inferior poles of both kidneys fuse abnormally > as they ascend from the pelvis (during normal embryogenesis)
–>horseshoe kidney get trapped under the inferior mesenteric artery

Question 9

how is renal function in horseshoe kidney

Answer 9

Renal function is usually normal

Question 10

horseshoe kidney is associated with ?
increases risk for

Answer 10

• hydronephrosis (ureteropelvic junction obstruction)
• stone
• infections
• increased risk of cancer

Question 11

Potter sequence what is it

Answer 11

oligohydramnios
> compression of developing fetus
> limb deformities
> facial anomalies
> compression of chest and lack of amniotic fluid aspiration into fetal lung
> pulmonary hypoplasia

Question 12

causes of potter sequence

Answer 12

• ARPKD
• obstructive uropathy
• bilateral renal agenesis
• chronic placental insufficiency
• BILATERAL MULTICYSTIC dysplastic kidney

Question 13

DEVELOPMENTAL DISORDERS OF THE COLLECTING SYSTEMS AND URETERS

Answer 13

1. Duplex collecting system
2. Ureteropelvic junction obstruction (UPJ)
3. Vesicoureteral reflux (VUR
4. Ureterocele
5. Posterior urethral
   valve

Question 14

Duplex collecting
system what is it

Answer 14

• ## Y-shaped bifid ureter

Question 15

duplex collecting system what is associated with

Answer 15

Associated with
* vesicoureteral reflux (VUR)
* and/or ureteral obstruction
* increased risk of UTI

Question 16

Ureteropelvic
junction obstruction etiology

Answer 16

• Congenital (inadequate canalization)
  or
• acquired (trauma, fibrosis)

Question 17

Ureteropelvic
junction obstruction presentation

Answer 17

• May be asymptomatic,
• or present with recurrent UTIs
• stone disease
• palpable mass
• hydronephrosis

Question 18

Ureteropelvic
junction obstruction treatment

Answer 18

Pyeloplasty is the treatment of choice,

but if the affected kidney possesses < 10%
of total renal function, nephrectomy should be performed

Question 19

when is nephrectomy indicated in ureteropelvic junction obstruction

Answer 19

Pyeloplasty is the treatment of choice,

but if the affected kidney possesses < 10%
of total renal function, nephrectomy should be performed

Question 20

Vesicoureteral
reflux (VUR) definition

Answer 20

• Retrograde regurgitation of urine from the urinary bladder up the ureter and into the collecting system of the kidneys

Question 21

Vesicoureteral
reflux (VUR) is a end result of

Answer 21

several anomalies related to the functional integrity of the
* ureter
* bladder dynamics
* and the anatomic composition of the ureterovesical junction (UVJ)

Question 22

Vesicoureteral
reflux (VUR) presents with

Answer 22

• recurrent UTIs,
• chronic pyelonephritis > may develop CKD (‘reflux nephropathy’)

Question 23

Ureterocele def

Answer 23

Cystic out-pouching of the distal ureter into the urinary bladder

Question 24

Ureterocele presentation

Answer 24

• May be asymptomatic
• or present with recurrent cystitis
• bladder outlet obstruction; eventually may develop CKD

Question 25

Posterior urethral valve defintion

Answer 25

Membranous remnant in the posterior urethra in males; it's persistence can lead to urethral obstruction

Question 26

persistance of posterior urethral valve can lead to

Answer 26

urethral obstruction

Question 27

Posterior urethral valve can be diagnosed prenatally how?

Answer 27

can be diagnosed prenatally by * bilateral hydronephrosis * and dilated or thickened bladder wall on US

Question 28

Most common cause of bladder outlet obstruction in male infants

Answer 28

posterior urethral valve

Question 29

posterior urethral valve is associated with

Answer 29

oligohydramnios in cases of severe obstruction

Question 30

DEVELOPMENTAL DISORDERS OF THE URINARY BLADDER

Answer 30

1. Urachus anomalies * Patent urachus * Urachal cyst * Vesicourachal diverticulum 2.Bladder exstrophy

Question 31

Urachus- what is it

Answer 31

duct between the fetal bladder and umbilicus

Question 32

Urachus anomalies are associated with increased risk of

Answer 32

associated with : * UTI and * increased risk of bladder adenocarcinoma

Question 33

Patent urachus - what is it

Answer 33

Total failure of urachus to obliterate > urine discharge from umbilicus

Question 34

Urachal cyst definition

Answer 34

* fluid-filled cavity lined with uroepithelium, between umbilicus and bladder - Cyst can become infected and present as painful mass below umbilicus

Question 35

Urachal cyst etiology

Answer 35

Partial failure of urachus to obliterate; fluid-filled cavity lined with uroepithelium, between umbilicus and bladder

Question 36

complication of urachal cyst and its presentation

Answer 36

Cyst can become infected and present as painful mass below umbilicus

Question 37

Vesicourachal diverticulum - definition

Answer 37

Slight failure of urachus to obliterate > outpouching of bladder

Question 38

Bladder exstrophy def

Answer 38

Protrusion of the urinary bladder through a defect in the abdominal wall

Question 39

Bladder exstrophy presentation

Answer 39

* Usually present with a wide range of congenital anomalies, affecting the 1. pelvic bones, 2. genitals, 3. reproductive tract, 4. and gastrointestinal tract