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CPR Exam 3 > Congenital and Developmental Anomalies > Flashcards

Congenital and Developmental Anomalies Flashcards

1
Q

What is seen in prune belly sydrome?

A
  • Hydroureteronephrosis
  • Vesicoureteral reflux (VUR)
  • Renal dysplasia
  • Cryptorchidism
  • UTI
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2
Q

What is bilateral agenesis?

A
  • Incompatible with life and usually encountered in stillborn infants
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3
Q

What is seen in unilateral agenesis?

A
  • Uncommon and compatible with life

- Solitary kidney is usually enlarged

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4
Q

What is seen in hypoplasia?

A
  • Refers to failure of the kidneys to develop to a normal size
  • May occur bilaterally, resulting in renal failure in early childhood, but it is more commonly encountered as a unilateral defect
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5
Q

Who is affected by true renal hypoplasia?

A
  • Observed in low birth weight infants and may contribute to their increased lifetime risk for chronic kidney disease
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6
Q

Where can ectopic kidneys be found?

A
  • Pelvis
  • Iliac fossa
  • Abdominal or thoracic cavities
  • Contralateral positions
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7
Q

What are some factors that cause ectopic kidneys?

A
  • Ureteral bud mal-development
  • Defective metanephric tissue that fails to induce ascent
  • Genetic abnormalities
  • Teratogens
  • Maternal illnesses
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8
Q

What are some certain anomalies that can cause obstruction of the flow of urine?

A
  1. Vesicoureteral reflux
  2. Double or bifid ureters
  3. UPJ obstruction
  4. Diverticula
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9
Q

What is the vesicoureteral reflux?

A
  • Most common and serious congenital anomaly
  • Abnormal connections between the bladder and the vagina, rectum, or uterus may create congenital vesicouterine fistulae
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10
Q

What does vesicoureteral reflux predispose too?

A
  • Ascending pyelonephritis and loss of function
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11
Q

What is the most common cause of hydronephrosis in infants and children?

A
  • Ureteropelvic junction obstruction
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12
Q

Who is most likely affected by ureteropelvic junction obstruction?

A
  • When early in life, males
  • Bilateral in 20% of cases
  • In adults, more common in women and is unilateral
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13
Q

What is seen if there is agenesis of the contralateral kidney in ureteropelvic junction obstuction?

A
  • Hydronephrosis
  • UTI
  • Hematuria
  • FTT
  • Sepsis
  • Azotemia
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14
Q

What is the presentation in adults with ureteropelvic junction obstruction?

A
  • UTI
  • Hematuria
  • Abdominal pain and vomiting due to intermittent obstruction
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15
Q

What is ureteropelvic junction obstruction ascribed by?

A
  • Abnormal organization of smooth muscle bundles or excess stromal deposition of collagen between smooth muscle bundles at the UP
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16
Q

What are the major sites at which relative constrictions in the ureters normally appear?

A
  1. Ureteropelvic junction
  2. Crossing the external iliac artery and/or pelvic brim
  3. As the ureter traverses the bladder wall
17
Q

What causes congenital diverticula?

A
  • Due to a focal failure of development of the normal musculature or to a urinary tract obstruction during fetal development
18
Q

How do diverticula come to attention?

A
  • May come to clinical attention since they constitute sites of urinary stasis and predispose to infection and the formation of bladder calculi
19
Q

What is exstrophy of the bladder?

A
  • Developmental failure in the anterior wall of the abdomen and the bladder
20
Q

What can happen to the exposed bladder mucosa in exstrophy of the bladder?

A
  • May undergo colonic glandular metaplasia

- Subject to chronic infection that often spreads to the upper urinary tract

21
Q

What causes a urachal cyst?

A
  • Patent urachus
22
Q

What connects the urachal canal to the fetal bladder?

A
  • Allantois which is normally obliterated at birth
23
Q

What lines a urachal cyst?

A
  • Urothelial or metaplastic glandular epithelium
24
Q

What cancer is most likely to form from urachal cysts?

A
  • Adenocarcinomas
25
Q

What is hypospadias?

A
  • Abnormal opening on the ventral surface of the penis

- More common

26
Q

What is epispadias?

A
  • Abnormal opening on the dorsal surface of the penis
27
Q

What are some complications of urethral defects?

A
  • Urinary tract obstruction
  • Increased risk of ascending infections
  • Normal ejaculation and insemination are hampered and may be a cause of sterility
28
Q

What is cryptochidism?

A
  • Complete or partial failure of the intra-abdominal testes to descend into the scrotal sac
  • Associated with testicular dysfunction and increased risk of testicular cancer
  • Usually unilateral

CPR Exam 3 (16 decks)

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