Development And Cystic Diseases Flashcards
What is hydronephrosis? What is the most common cause?
= Dilation of the renal pelvis by urine
-Most common cause: Ureteropelvic junction obstruction
Ureteropelvic junction obstruction results from what abnormalities? What are the clinical signs?
=the result of incomplete canalization of ureteric bud at 12 weeks gestation and/or local abnormality of smooth muscle fibers with increased fibrosis impeding peristalsis
Signs: Abdominal mass, pain, UTI
Which is the most common renal abnormality: hydroureter or ureteral duplication?
Ureteral duplication (about 1% of population)
-signs & symptoms include failure to toilet train or continuous drip incontinence
Complete ureteral duplication can cause obstruction of upper pole and vesicoureteral reflux in the lower pole. What is vesicoureteral reflux?
Urine traveling back up the ureter upon contraction of the detrusor muscle of the bladder, usually resulting in hydroureter
What is a ureterocele?
= a cystic dilatation of the terminal intravesical (portion within the bladder wall) ureter.
-An ectopic ureterocele is at the bladder neck or the urethra, regardless of the position of the orifice
How is a uterocele diagnosed?
- Diagnosed prenatally when associated with hydronephrosis or during work up of UTI,
- or may prolapse through urethra causing bladder outlet obstruction, usually in girls
What is a congenital disorder that is diagnosed when there is clear fluid accumulating in the umbilicus with micturition? Describe three anomalites that can result from this?
Urachal Remnant
- Urachus: structure that connects fetal bladder to umbilical cord (allantois)
Can cause
- Cyst: Painful midline mass
- Sinus or 3. Fistula: leads to drainage of clear or purulent urine at umbilicus and sometimes UTI
What do you call a chronic abnormal distension of the bladder by urine due to bladder outlet obstruction?
Megalocystis (Megacystis)
What is the most common cause of bladder outlet obstruction in boys that is usually diagnosed prenatally with anuria and bladder distension?
Posterior urethral valves
Older boy may have a poor urine stream, UTI or urinary incontinence
What is bladder diverticulum?
Outpouching of the bladder mucosa through a weakness in the muscular wall
What is hypospadias? (hint: its only in boys)
The orifice (pee hole) of the penile urethra is at a location along the ventral aspect of the penis
- results from abnormal fusion of urogenital folds from androgen insufficiency
What do you call a fibrous band that causes the penis to curve toward its location?
Chordee
- usually associated with hypospadias or epispadias
And since we’re talking about penises, there are two things called epispadias and exstrophy that often go together. What are they?
Epispadias-location of the urethral opening on the dorsal aspect of the penis
Exstrophy-exposure of the bladder mucosa due to absence of the abdominal wall
What causes the exstrophy-epispadias complex? Is it more common in boys or girls?
=Arises from failure of separation by the urorectal septum of the primitive cloaca into the urogenital sinus and anorectal canal at six weeks gestation
- Boys: 2-6M:1F
IMPORTANT: list the consequences of fetal urinary tract obstruction or renal agenesis?
- Inability to excrete urine
- Decreased amount of amniotic fluid
- Oligohydramnios (deficiency of amniotic fluid)
- Less room for fetal movement
- Loss of “normal” in utero respiration and impaired lung development
What happens to a babies face when in too little of amniotic fluid? What do you call the condition?
Potter’s Facies
- Large, flattened ears
- Flattened nose
- Infraorbital skin folds
With deficient amniotic fluid, what happens to the amniotic membrane? The lungs?
Amnion Nodosum: Nodules of squamous cells on amniotic membrane
Lungs: Pulmonary hypoplasia
- Leads to underdeveloped lungs
- Respiratory insufficiency is the cause of death
What is the problem with prune belly (Eagle-Barrett) syndrome?
Atrophy of anterior abdominal muscles due to megalocystis
-also, undescended testes (cryptorchidism)
