SM_198b: Congenital Pediatric GU Anomalies Flashcards
Congenital anomalies of the kidney and collecting system are ____, ____, and ____
Congenital anomalies of the kidney and collecting system are multicystic dysplastic kidney, antenatal hydronephrosis / urinary tract dilation, and ureteropelvic junction obstruction
Congenital anomalies of the ureters are ____ and ____
Congenital anomalies of the ureters are vesicoureteral reflux and ureteral duplication
Congenital anomalies of the urethra are ____ and ____
Congenital anomalies of the urethra are posterior urethral valves and hypospadias
Congenital anomalies of the scrotum are ____ and ____
Congenital anomalies of the scrotum are undescended testes and pediatric hydroceles / hernia
Three stags of renal development are ____, ____, and ____
Three stags of renal development are
- Pronephros
- Mesonephros (Wolffian ducts)
- Metanephros
Proper development of the kidney and ureters requires interaction of the ____ with the ____
Proper development of the kidney and ureters requires interaction of the ureteric bud (mesonephric duct) with the metanephric blastema
- Failure leads to congenital anomalies of the GU tract
____ and ____ must interact for the kidney and ureters to properly develop
Ureteric bud (mesonephric duct) and metanephric blastema must interact for the kidney and ureters to properly develop
____ occurs if the ureteric bud does not connect / contact the metanephric blastema
Multicystic dysplastic kidney occurs if the ureteric bud does not connect / contact the metanephric blastema
____ is the most common cystic renal disease in children, is more common on the left and in boys, and is diagnosed with ultrasound
Multicystic dysplastic kidney is the most common cystic renal disease in children, is more common on the left and in boys, and is diagnosed with ultrasound
Ultrasound finding of multicystic dysplastic kidney is ____
Ultrasound finding of multicystic dysplastic kidney is lack of reniform shape
Describe pathology of multicystic dysplastic kidney
Multicystic dysplastic kidney pathology
- Multiple non-communicating cysts
- No identifiable normal renal parenchyma
- Ureteral with or without pelvic atresia
- Immature glomeruli are often present
Multicystic dysplastic kidney pathophysiology involves ____ and ____
Multicystic dysplastic kidney pathophysiology involves malunion of the ureteric bud with the metanephric blastema and early obstruction of the ureter
Multicystic dysplastic kidney workup involves ____ and identifying contralateral pathology using ____ and ____
Multicystic dysplastic kidney workup involves ultrasound and identifying contralateral pathology using voiding cystourethrogram and radionucleotide renal scan
One-third of patients with mutlicystic dysplastic kidney have ___
One-third of patients with mutlicystic dysplastic kidney have contralateral vesicoureteral reflux
(voiding cystourethrogram)
Multicystic dysplastic kidney natural history is typically ____
Multicystic dysplastic kidney natural history is typically involution
(nephrectomy is seldom necessary)
____ results if there is an abnormality with ureteral development after the ureteric bud joins the metanephric blastema
Hydronephrosis / urinary tract dilation results if there is an abnormality with ureteral development after the ureteric bud joins the metanephric blastema
Describe embyrological ureteral development
Embyrological ureteral development
- Days 28-35: ureter patent due to urine from mesonephros
- Days 37-40: ureter loses its lumen
- Day 40: ureter regains its lumen (midpoint outwards to distal and proximal ureter, last segments to recanalize are ureteropelvic and ureterovesical junctions), ureter invests into the UG sinus (bladder) separated by Chawalla’s membrane (rupture leads to open communication between the ureter and bladder)
- Week 9: fetal kidney produces urine, ureteral lumenal patency
Last segments of ureter to recanalize at day 40 are ____ and ____
Last segments of ureter to recanalize at day 40 are ureteropelvic and ureterovesical junctions
Hydronephrosis is ____
Hydronephrosis is dilation of the urinary tract as a consequence of some degree of obstruction
(use the term urinary tract dilation)
Antenatal urinary tract dilation is most often diagnosed ____ and ____ is the prenatal ultrasound standard of care
Antenatal urinary tract dilation is most often diagnosed antenatally and 20 week is the prenatal ultrasound standard of care
Describe urinary tract dilation grading
Urinary tract dilation grading
- Grade 0: no hydro
- Grade 1: slight pelvic dilatation, no calyceal dilatation
- Grade 2: moderate pelvic dilatation, slight calyceal dilatation (major calyces)
- Grade 3: large pelvis, dilated calcyes (minor calyces), normal parenchyma
- Grade 4: large pelvis, dilated calyces, thinned parenchyma
Antental hydronephrosis most commonly presents with ____, ____, and ____
Antental hydronephrosis most commonly presents with isolated antenatal hydronephrosis, ureteropelvic junction obstruction, and vesicoureteral reflux
Describe ureteropelvic junction obstruction
Ureteropelvic junction obstruction
- Males > females
- Left > right
- May lead to renal deterioration
- 10-40% bilateral
Describe etiologies of ureteropelvic junction obstruction
Ureteropelvic junction obstruction etiologies
- Intrinsic: aperistaltic segment, intrinsic narrowing, ureteral polyps
- Extrinsic: high insertion, kinking secondary to periureteral fibrosis, and crossing vessel
Ureteropelvic junction obstruction presents in infants with ____, ____, and ____
Ureteropelvic junction obstruction presents in infants with prenatal ultrasound, abdominal masses, and febrile UTI
Ureteropelvic junction obstruction presents in older children with ____, ____, ____, and ____
Ureteropelvic junction obstruction presents in older children with Dietl’s crisis (intermittent pain episodes), febrile UTI, hematuria, and stones
Describe ureteropelvic junction obstruction workup
Ureteropelvic junction obstruction workup
- Renal ultrasound
- Diuretic renogram: MAG 3, differential function, drainage curve
- VCUG: 10% chance of reflux
Ureteropelvic junction obstruction is treated with ____
Ureteropelvic junction obstruction is treated with pyeloplasty
Vesicoureteral reflux is ____ and the most common cause of ____
Vesicoureteral reflux is reflux of urine up one or both ureters up to the kidney and the most common cause of renal scarring
- Associated with bladder and bowel dysfunction
- Incidence inversely related to age, Caucasians, girls
Vesicoureteral reflux presents as ____ or is found ____
Vesicoureteral reflux presents as symptomatic UTI or is found incidentally on evaluation of antenatal urinary tract dilation
- Associated with bladder and bowel dysfunction
Describe causes of vesicoureteral reflux
Causes of vesicoureteral reflux
- Abnormality of the ureterovesical junction: intramural tunnel, detrusor support
- Due to caudal displacement of the ureteric bud
- Lateral displacement of the ureteral orifice
Gold standard for diagnosis of vesicoureteral reflux is ____
Gold standard for diagnosis of vesicoureteral reflux is voiding cystourethrogram
- Fluorscopic vs nuclear, bottoms up
Vesicoureteral reflux is graded from ___ to ___
Vesicoureteral reflux is graded from I to V
(higher numbers indicate greater reflux)
Vesicourteral reflux has a high rate of ____
Vesicourteral reflux has a high rate of symptomatic resolution
(higher rate as grade decreases)
Management of vesicoureteral reflux involves medical management to ____ and surgical management to ____
Management of vesicoureteral reflux involves medical management to prevent UTIs and associated renal scars and surgical management to eliminate vesicoureteral reflux
____ results if there are two ureteral buds interacting with the metanephric blastema
Ureteral duplication results if there are two ureteral buds interacting with the metanephric blastema
Weigert-Meyer rule states that ____
Weigert-Meyer rule states that the ureters invert their positions as they fuse with the urogenital sinus
- Upper moiety: ureteral orifice inferiorly and medially
- Lower moiety: ureteral orifice superiorly and laterally
Describe the effect of the Weigert-Meyer rule in ureteral duplication
Weigert-Meyer rule in ureteral duplication
- Upper moiety: ureteral orifice inferiorly and medially, more commonly obstructs, can present with incotinence
- Lower moiety: ureteral orifice superiorly and laterally, more commonly refluxes
Upper moiety involves a ureteral orifice positioned ____ and more commonly ____
Upper moiety involves a ureteral orifice positioned inferiorly and medially and more commonly obstructs
- Obstructs: ectopic ureter, uterocele
Lower moiety involves a ureteral orifice positioned ____ and more commonly ____
Lower moiety involves a ureteral orifice positioned superiorly and laterally and more commonly refluxes
Anomalies of the urethra are ____ and ____
Anomalies of the urethra are posterior urethral valves and hypospadias
Describe posterior urethral valves
Posterior urethral valves
- Most common cause of bladder outlet obstruction in boys
- Reported incidence between 1:4000 live male births
- Due to abnormal insertion of wolffian ducts into the posterior urethra
- Important etiology for renal failure in children
Posterior urethral valve most commonly presents ____
Posterior urethral valve most commonly presents prenatally
Posterior urethral valve presents prenatally as a male fetus with ____, ____, ____, and ____
Posterior urethral valve presents prenatally as a male fetus with bilateral hydronephrosis, thick-walled bladder, dilated posterior urethra, oligohydramnios
Potter’s syndrome is ____, ____, ____, ____, and ____
Potter’s syndrome is flattened nose, low set ears, recessed chin, small chest, and limb deformities
Posterior urethral valve presents postnatally as ____, ____, ____, ____, ____, and ____
Posterior urethral valve presents postnatally as urinary retention, renal insufficiency, pulmonary insufficiency, UTIs, poor urinary stream, incontinence
Posterior urethral valve diagnosis involves ____ and ____
Posterior urethral valve diagnosis involves ultrasound and VCUG
Describe management of posterior urethral valve
Posterior urethral valve management
- Initial: catheterization and medical stabilization
- Early: endoscopic valve ablation vs diversion
- Late: management of clinical sequelae (ESRD, valve bladder)
Hypospadias is ____
Hypospadias is embryologic failure of urethral plate to tubularize
Hypospadias etiology is ____, including ____, ____, and ____
Hypospadias etiology is multifactorial, including environmental and / or endocrine disruptors, native endocrine / local tissue disruption, and arrested development
Hypospadias diagnosis involves ____, ____, ____, and ____
Hypospadias diagnosis involves dorsal hood, chordee, deviation of median raphe, and flattened ventral glans
Hypospadias is associated with ____, ____, and ____
Hypospadias is associated with undescended testis, hernia, and disorder of sexual differentiation (hypospadias + undescended testis)
Hypospadias surgery involves native tissue and is indicated if ____, ____, ____, and ____
Hypospadias surgery involves native tissue and is indicated if deviated urinary stream, significant chordee (> 30 degrees), psychosocial concerns, or comesis
(usually at 6-9 months of age)
Congenital anomalies of the scrotum are ____ and ____
Congenital anomalies of the scrotum are undescended testis and hernia / hydroceles
Undescended testis is most common in ____, spontaneous descent is ____ after 6 months of age, and diagnosis is made by ____
Undescended testis is most common in premature children, spontaneous descent is rare after 6 months of age, and diagnosis is made by physical exam
Describe undescended testis terminology
Undescended testis terminology
- Acquired testis: a testis previously noted in the scrotum
- Ascended testis: becomes extra-scrotal over time
- Entrapped testis: becomes extra-scrotal after inguinal surgery
- Vanishing testis: initially present at one time but dispapeared secondary to spermatic cord torsion or vascular compromise
- Retractile testis: normally descended testis that moves intermittently in and out of the scrotum due to a hyperactive cremasteric reflex, testis can be pulled into scrotum without significant amount of tension on physical exam
Management of undescended testis occurs due to ____, ____, ____, and ____
Management of undescended testis occurs due to fertility, cancer risk (seminoma), cosmesis, and decrease risk of testicular torsion
(at 6 months of age or at time of diagnosis)
Pediatric hernia and hydrocele result from a ____
Pediatric hernia and hydrocele result from a patent processus vaginalis
- Hydrocele: fluid only
- Hernia: intraperitoneal contents
Pediatric hernia / hydrocele are must common in ____ children
Pediatric hernia / hydrocele are must common in premature children
Pediatric hernia / hydrocele diagnosis involves ____, ____, and ____
Pediatric hernia / hydrocele diagnosis involves history, physical exam, and radiological studies
Pediatric hernia / hydrocele indications for surgery are ____, ____, and ____
Pediatric hernia / hydrocele indications for surgery are true hernia, evidence of communicating hydrocele, and lack of spontaneous resolution of hydrocele by 18 months
- True hernia: look for inguinal bulge
- Communicating hydrocele: waxes and waves in size over course of the day, progressive enlargement
This is ____
This is multicystic dyplastic kidney
Most common cause of unilateral urinary tract dilation in a newborn is ____
Most common cause of unilateral urinary tract dilation in a newborn is idiopathic
____ is the most likely diagnosis in a male fetus with high grade bilateral hydronephrosis
Posterior urethral valves is the most likely diagnosis in a male fetus with high grade bilateral hydronephrosis
