Renal Pathology 2: Congenital and Devel Anomalies (Dobson)

Question 1

Prune belly syndrome

Answer 1

predominantly in males, loss of abdominal musculature; so babies cannot cough, cannot do valsalva maneuver. Forms prune belly appearance. Babies present with hydroureteronephrosis (mostly bilateral) and males present with bilateral cryptorchidism; 75% of babies have vesicoureteral reflux (VUR) mostly bilateral; 80% will have UTI;
1/3 will have renal dysplasia
1/3 will develop pyelonephritis

Question 2

Bilateral Renal agenesis

Answer 2

incompatible with life (stillborns). Often associated with other congenital disorders.

Question 3

Unilateral renal agenesis

Answer 3

uncommon and compatible with life. The solitary kidney is enlarged (hypertrophy). Some patients develop glomerular sclerosis in the remaining kidney as a result of hypertrophied nephrons. Hyperfiltration injury.

Question 4

Renal Hypoplasia

Answer 4

small sized kidneys; bilateral - renal failure early in childhood; unilateral (more common). Observed in low birth weight infants.

Question 5

Ectopic kidney

Answer 5

arrested ascent of kidneys during development; can be found in pelvis, iliac fossa, abdominal or thoracic cavities or c/l positions.

Question 6

Horseshoe kidney

Answer 6

fusion of the lower poles (90%) produces a horseshoe-shaped structure that is continuous across the great midline anterior to the great vessels; doesn’t cause abnormal renal function during life; usually found incidental; stopped by IMA

Question 7

What are the four congenital abnormalities of the ureters?

Answer 7

1. Vesicoureteral reflux (VUR)
2. Double or bifid ureters
3. UPJ obstruction
4. Diverticula

Question 8

Vesicoureteral reflux (VUR)

Answer 8

most common and serious congenital anomaly of the ureters; marked dilation of the ureters and hydronephrosis; predisposes to ascending pyelonephritis and loss of renal function. Abnormal connections to the vagina, rectum, or uterus may create congenital vesicouterine fistulae.

Question 9

Double or bifid ureters

Answer 9

uncommon developmental anomaly that results from an abnormal branching pattern of ureteric bud. No clinical significance.

Question 10

Ureteropelvic junction (UPJ) obstruction

Answer 10

most common cause of hydronephrosis in infants (males); bilateral; agensis of the c/l kidney; presents with hydronephrosis, UTI, hematuria, failure to thrive (FTT), sepsis, azotemia.

in adults (females); unilateral; most commonly ascribed to abnormal organization of smooth muscle bundles or excess stromal deposition of collagen between smooth muscle bundles at UPJ

Question 11

Urinary or Bladder Diverticula

Answer 11

pouch-like invaginations in the ureters, more commonly seen in the bladder; can be congenital or acquired; most are asymptomatic; usually a secondary dx after infection or stone.

Complication: can become the source of tumor development (carcinomas are on average in a more advanced stage due to thin or absent muscularis propria layer of diverticula.

Question 12

In which congenital anomaly is there a risk for a vesicouterine fistulae to form?

Answer 12

Vesicoureteral reflux (VUR); most common and serious congenital anomaly of the ureters; Abnormal connections to the vagina, rectum, or uterus may create congenital vesicouterine fistulae.

Question 13

What is the most common cause of hydronephrosis in infants.

Answer 13

Ureteropelvic junction (UPJ) obstruction

Question 14

Major (rare) complication of urinary or bladder diverticula.

Answer 14

Complication: can become the source of tumor development (carcinomas are on average in a more advancs stage due to thin or absent muscularis propria layer of diverticula.

Question 15

Exstrophy of the bladder

Answer 15

developmental failure in the anterior wall of the abdomen and the bladder; exposed bladder is subject to chronic infection that often spreads to the upper UT; can be seen w epispadias

Complication: mucosa may undergo colonic glandular metaplasia - carcinoma

Question 16

Major complication with extrophy of the bladder

Answer 16

mucosa may undergo colonic glandular metaplasia - carcinoma

Question 17

Urachal remnant

Answer 17

can develop into a urachal cyst; at risk for bladder adenocarcinomas

Question 18

Hypospadias

Answer 18

malformation of the ureteral groove with an abnormal opening on the VENTRAL side of the penis; more common than epispadias; can result in urinary tract obstruction, and increased risk of ascending infections.

Question 19

Epispadias

Answer 19

malformation of the ureteral groove with an abnormal opening on the DORSAL side of the penis; less common than epispadias; can result in urinary tract obstruction and increased risk of ascending infections.

Question 20

Cryptorchidism

Answer 20

a complete or partial failure of the intra-abdominal testes to descend into the scroal sac and is associated with testicular dysfxn and increased for testicular cancer

Question 21

Cryptorchidism gives you the increased for what type of cancer?

Answer 21

Testicular cancer.

Question 22

Which vessel prevents the ascension of a horseshoe kidney?

Answer 22

Inferior mesenteric artery (IMA); prevents kidneys from ascending; stuck in lower abdomen

Question 23

Oligohydramnios

Answer 23

decreased/low amniotic fluid; due to bilateral renal agenesis; incompatible with life; Potter’s sequence can result

Question 24

Potter’s Sequence

Answer 24

Lung hypoplasia, flat face with low set ears, and developmental defects of extremities. Likely as the result of bilateral renal agenesis (oligohydramnios)