Congenital Disorders Of The genitourinary System Flashcards

1
Q

Disorders of GUS

Disorder of presence/number

Disorder of location

A
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2
Q

Aetiology of bilateral agenesis

Due to lack of ________ of the ___________ by the __________

Absence of _________________

_________________ maldevelopment

A

induction; metanephric blastema; ureteric bud

metanephric blastema

Ureteric bud

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3
Q

Unilateral renal agenesis- an incidental finding with compensatory __________ of the contralateral kidney

It’s a _______ ending ____________

A

hypertrophy

Blind; ureteric stump

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4
Q

Disorder of location

————- kidney
______ or _________ kidney

__________ kidney

A

Pelvic

Iliac or lumbar

Thoracic

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5
Q

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?)

A

ureter

Boys; girls

Left; right

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6
Q

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 ___________

A

two ureters ; separately

fuse into a single unit

a single orifice

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7
Q

The most common anomaly of the ureters Incomplete and complete

Incomplete – 1 in ____ individuals

Complete – 1 in _____ individuals

A

25

125

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8
Q

Ectopic ureters- ureters drain into an abnormal site

Female to male ratio- __:__

80% of all affected ureters drain the _______________ of the kidney

A

6;1

upper pole

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9
Q

Male ectopic ureters always terminate (proximal or distal?) to the external sphincter

A

Proximal

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10
Q

MEGAURETER- unusually ________ ureters

Associated features include _________ and _________

Two major problems are _________ and massive ______

Left side > Right side

A

dilated

tortuosity ; enlongation

obstruction ; vesicoureteral reflux

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11
Q

URETEROCELE- ___________ of the terminus of the ureters as it continues with the urinary bladder

80% occur in (male or female?)

A

cystic dilatation

female

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12
Q

BLADDER EXSTROPHY

Absence of the ______________ and the ____________________________

Usually associated with ____________

A

anterior abdominal wall

anterior wall of the urinary bladder

epispadias

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13
Q

Treatment for bladder exstrophy

  • initial _________
    -_____________ of the defect which involves bladder ________ and closure of the _______________ defect
A

resuscitation

primary closure; reconstruction

anterior abdominal wall

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14
Q

APENIA-congenital absence of the ________

A

penile shaft

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15
Q

MICROPENIS-due to ________ disturbances resulting from ________ _________

MEGALOPENIS-due to ______ levels of ___________

A

hormonal

hypogonadotropic hypogonadism

high; testesterone

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16
Q

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

A

mucosal folds

bladder outlet obstruction

17
Q

EPIDEMIOLOGY of posterior urethral valve

Disease unique to _______ , exclusively in ______ ; rarely, reports in _________

Increase incidence witnessed attributed to use of ___________

A

males; boys ; adults

antenatal USS

18
Q

Prenatal diagnosis of PUV

Failure of _____________

__________ bladder wall

________________

Renal dysplasia

____hydromnios

A

bladder emptying

Thickened; Hydronephrosis

Oligo

19
Q

Postnatal diagnosis of Posterior urethral valve

Depends largely on the _____ at presentation

20
Q

Treatment for posterior urethral valve

PRIMARY VALVE ABLATION
BLIND
_____________________ using ________ catheter or _________________

A

Balloon valve avulsion; Fogarty
Mohan’s valvulotome

21
Q

Treatment for posterior urethral valve

PRIMARY VALVE ABLATION

___________
______________
_________________

A

BLIND
ENDOSCOPIC
Renal transplantation

22
Q

HYPOSPADIAS
Abnormal _________ of the _________ on _________ aspect of _________.

Incidence appears to be increasing worldwide

A

opening ; urethra

ventral ;penis.

23
Q

Hypospadias occurs due to _________ of complete _______ of (genital) ________

A

failure (arrest)

fusion

Urethral folds

24
Q

Hypospadias

___________ opening
______ and _______ glans (______-like)
_________
____________

A

Ventral urethral opening
Splayed and flattened glans (spade-like)
Hooding
Chordee

25
Hypospadias Splayed and flattened glans (spade-like) Sometimes appears rather (small or large?( A cause of anxiety for mothers (______ acceptability)
Large Sexual
26
Hypospadias Chordee: may be due to ___________ __________ adjacent to urethra ______urethra ________ disproportion Not present in all patients
Skin tethering Fibrous band Short; Corporal
27
Surgery for hypospadias is?
Urethroplasty
28
Congenital genitalia anomalies in females Labial fusion- fused labia _______ predisposes to _______ Clitoral hypertrophy-due to ___________ in-utero
minora; recurrent UTI exposure to testestorone
29
Congenital genitalia anomalies in females Usually due to ______________ disorders of _______ synthesis
congenital enzyme cortisol
30
Congenital genitalia anomalies in females _________ ___________
Labial fusion Clitoral hypertrophy
31
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
32
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
33
Treatment of undescended testes???
Orchidopexy
34
ECTOPIC TESTIS- one that has tranverses the normal path of descent but _______________
deviates to abnormal sites