Congenital and Developmental Anomalies Flashcards
What is seen in prune belly sydrome?
- Hydroureteronephrosis
- Vesicoureteral reflux (VUR)
- Renal dysplasia
- Cryptorchidism
- UTI
What is bilateral agenesis?
- Incompatible with life and usually encountered in stillborn infants
What is seen in unilateral agenesis?
- Uncommon and compatible with life
- Solitary kidney is usually enlarged
What is seen in hypoplasia?
- 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
Who is affected by true renal hypoplasia?
- Observed in low birth weight infants and may contribute to their increased lifetime risk for chronic kidney disease
Where can ectopic kidneys be found?
- Pelvis
- Iliac fossa
- Abdominal or thoracic cavities
- Contralateral positions
What are some factors that cause ectopic kidneys?
- Ureteral bud mal-development
- Defective metanephric tissue that fails to induce ascent
- Genetic abnormalities
- Teratogens
- Maternal illnesses
What are some certain anomalies that can cause obstruction of the flow of urine?
- Vesicoureteral reflux
- Double or bifid ureters
- UPJ obstruction
- Diverticula
What is the vesicoureteral reflux?
- Most common and serious congenital anomaly
- Abnormal connections between the bladder and the vagina, rectum, or uterus may create congenital vesicouterine fistulae
What does vesicoureteral reflux predispose too?
- Ascending pyelonephritis and loss of function
What is the most common cause of hydronephrosis in infants and children?
- Ureteropelvic junction obstruction
Who is most likely affected by ureteropelvic junction obstruction?
- When early in life, males
- Bilateral in 20% of cases
- In adults, more common in women and is unilateral
What is seen if there is agenesis of the contralateral kidney in ureteropelvic junction obstuction?
- Hydronephrosis
- UTI
- Hematuria
- FTT
- Sepsis
- Azotemia
What is the presentation in adults with ureteropelvic junction obstruction?
- UTI
- Hematuria
- Abdominal pain and vomiting due to intermittent obstruction
What is ureteropelvic junction obstruction ascribed by?
- Abnormal organization of smooth muscle bundles or excess stromal deposition of collagen between smooth muscle bundles at the UP
What are the major sites at which relative constrictions in the ureters normally appear?
- Ureteropelvic junction
- Crossing the external iliac artery and/or pelvic brim
- As the ureter traverses the bladder wall
What causes congenital diverticula?
- Due to a focal failure of development of the normal musculature or to a urinary tract obstruction during fetal development
How do diverticula come to attention?
- May come to clinical attention since they constitute sites of urinary stasis and predispose to infection and the formation of bladder calculi
What is exstrophy of the bladder?
- Developmental failure in the anterior wall of the abdomen and the bladder
What can happen to the exposed bladder mucosa in exstrophy of the bladder?
- May undergo colonic glandular metaplasia
- Subject to chronic infection that often spreads to the upper urinary tract
What causes a urachal cyst?
- Patent urachus
What connects the urachal canal to the fetal bladder?
- Allantois which is normally obliterated at birth
What lines a urachal cyst?
- Urothelial or metaplastic glandular epithelium
What cancer is most likely to form from urachal cysts?
- Adenocarcinomas
What is hypospadias?
- Abnormal opening on the ventral surface of the penis
- More common
What is epispadias?
- Abnormal opening on the dorsal surface of the penis
What are some complications of urethral defects?
- Urinary tract obstruction
- Increased risk of ascending infections
- Normal ejaculation and insemination are hampered and may be a cause of sterility
What is cryptochidism?
- 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
