Congenital Disorders Of The genitourinary System Flashcards
Disorders of GUS
Disorder of presence/number
Disorder of location
Aetiology of bilateral agenesis
Due to lack of ________ of the ___________ by the __________
Absence of _________________
_________________ maldevelopment
induction; metanephric blastema; ureteric bud
metanephric blastema
Ureteric bud
Unilateral renal agenesis- an incidental finding with compensatory __________ of the contralateral kidney
It’s a _______ ending ____________
hypertrophy
Blind; ureteric stump
Disorder of location
————- kidney
______ or _________ kidney
__________ kidney
Pelvic
Iliac or lumbar
Thoracic
Pelvi- Ureteric Junction(PUJ) obstruction
Common abnormality of the ______
More in (boys or girls?) than (boys or girls?)
Affects (left or right?) more than (left or right?)
ureter
Boys; girls
Left; right
Ureters
Duplex-(duplication) – a kidney with two pelvi- caliceal system
Complete duplication- a kidney with ________ that drain ______ into or below the bladder
Incomplete duplication- two ureters ___________ that drains into bladder through ___________
two ureters ; separately
fuse into a single unit
a single orifice
The most common anomaly of the ureters Incomplete and complete
Incomplete – 1 in ____ individuals
Complete – 1 in _____ individuals
25
125
Ectopic ureters- ureters drain into an abnormal site
Female to male ratio- __:__
80% of all affected ureters drain the _______________ of the kidney
6;1
upper pole
Male ectopic ureters always terminate (proximal or distal?) to the external sphincter
Proximal
MEGAURETER- unusually ________ ureters
Associated features include _________ and _________
Two major problems are _________ and massive ______
Left side > Right side
dilated
tortuosity ; enlongation
obstruction ; vesicoureteral reflux
URETEROCELE- ___________ of the terminus of the ureters as it continues with the urinary bladder
80% occur in (male or female?)
cystic dilatation
female
BLADDER EXSTROPHY
Absence of the ______________ and the ____________________________
Usually associated with ____________
anterior abdominal wall
anterior wall of the urinary bladder
epispadias
Treatment for bladder exstrophy
- initial _________
-_____________ of the defect which involves bladder ________ and closure of the _______________ defect
resuscitation
primary closure; reconstruction
anterior abdominal wall
APENIA-congenital absence of the ________
penile shaft
MICROPENIS-due to ________ disturbances resulting from ________ _________
MEGALOPENIS-due to ______ levels of ___________
hormonal
hypogonadotropic hypogonadism
high; testesterone
POSTERIOR URETHRAL VALVE
Congenital __________ in the posterior urethra
Common cause of _______________
Presents as a spectrum
Management is a challenge to the clinician as well as to the surgeon
mucosal folds
bladder outlet obstruction
EPIDEMIOLOGY of posterior urethral valve
Disease unique to _______ , exclusively in ______ ; rarely, reports in _________
Increase incidence witnessed attributed to use of ___________
males; boys ; adults
antenatal USS
Prenatal diagnosis of PUV
Failure of _____________
__________ bladder wall
________________
Renal dysplasia
____hydromnios
bladder emptying
Thickened; Hydronephrosis
Oligo
Postnatal diagnosis of Posterior urethral valve
Depends largely on the _____ at presentation
age
Treatment for posterior urethral valve
PRIMARY VALVE ABLATION
BLIND
_____________________ using ________ catheter or _________________
Balloon valve avulsion; Fogarty
Mohan’s valvulotome
Treatment for posterior urethral valve
PRIMARY VALVE ABLATION
___________
______________
_________________
BLIND
ENDOSCOPIC
Renal transplantation
HYPOSPADIAS
Abnormal _________ of the _________ on _________ aspect of _________.
Incidence appears to be increasing worldwide
opening ; urethra
ventral ;penis.
Hypospadias occurs due to _________ of complete _______ of (genital) ________
failure (arrest)
fusion
Urethral folds
Hypospadias
___________ opening
______ and _______ glans (______-like)
_________
____________
Ventral urethral opening
Splayed and flattened glans (spade-like)
Hooding
Chordee
Hypospadias
Splayed and flattened glans (spade-like)
Sometimes appears rather (small or large?(
A cause of anxiety for mothers (______ acceptability)
Large
Sexual
Hypospadias
Chordee: may be due to
___________
__________ adjacent to urethra
______urethra
________ disproportion
Not present in all patients
Skin tethering
Fibrous band
Short; Corporal
Surgery for hypospadias is?
Urethroplasty
Congenital genitalia anomalies in females
Labial fusion- fused labia _______ predisposes to _______
Clitoral hypertrophy-due to ___________ in-utero
minora; recurrent UTI
exposure to testestorone
Congenital genitalia anomalies in females
Usually due to ______________ disorders of _______ synthesis
congenital enzyme
cortisol
Congenital genitalia anomalies in females
_________
___________
Labial fusion
Clitoral hypertrophy
anomalies of the testes
Undescended testis- ______ of the testis along __________________
The most common site is the _________________
Affects about 3-4% of boys and about 30% of _______ babies
arrest
its normal path of descent
superficial inguinal ring
premature babies
Clinical features of undescended testes
______ scrotum
_______________ scrotal sacs, poorly defined _________
Occasionally palpable testis which cannot be ___________________
empty; poorly developed
ruggae; coaxed into the ipsilateral scrotum
Treatment of undescended testes???
Orchidopexy
ECTOPIC TESTIS- one that has tranverses the normal path of descent but _______________
deviates to abnormal sites
