CONGENITAL MALFORMATION OF URINARY TRACT

Question 1

RENAL AGENESIS - UNILATERAL
A. COMMONLY AFFECTED RIGHT KIDNEY
B ASYMPTOMATIC
C. THE OTHER KIDNEY WILL UNDERGOES COMPENSATORY HYPERTROPHY
D. A/W OLIGOHYDRAMNIOS
E. FEMALE > MALE

Answer 1

A. FALSE (LEFT KIDNEY IS MORE COMMON)
B. TRUE
C. TRUE
D. FALSE (RENAL AGENESIS - BILATERAL)
E. FALSE ( MALE > FEMALE)

Question 2

BRIEFLY DESC. REGARDING BILATERAL RENAL AGENESIS.

Answer 2

• INCOMPATIBLE IN POST NATAL LIFE
• USUALLY INFANT DIE RIGHT AFTER BIRTH OR AFTER THE FIRST MONTH OF LIFE
• A/W OLIGOHYDRAMNIOS BCZ NO OR VERY LITTLE AMOUNT OF URINE EXCRETED INTO THE AMNIOTIC CAVITY
• IT IS THE RESULT FROM THE FAILURE OF URETIC BUD DEVELOPMENT
• URETERIC BUD IS UNABLE TO PENETRATE THE METANEPHRIC BLASTEMA.

Question 3

STATE THE POTTER SEQUENCE.

Answer 3

1. ANURIA
2. OLIGOHYDROMNIOS
3. HYDROPLASTIC LUNG
4. POTTER FACIES (LOW SET OF EARS, BROAD NASAL BRIDGE, EPICANTHIC FOLD)
5. CLUB FEET
6. A/W CONGENITAL ANOMALIES (CARDIAC ABNORMALITIES, CLEFT LIP OR PALATE, BRAIN ABNORMALITIES)

Question 4

STATE THE EXAMPLE OF RENAL DYSPLASIA AND BRIEFLY DESC.

Answer 4

• RENAL DYSPLASIA: MULTICYSTIC DYSPLASTIC KIDNEY
• NUMEROUS DUCT IS SURROUNDED BY UNDIFFERENTIATED CELLS -> (CAUSING) URETERIC BUD FAILS TO BRANCH AND NEPHRON FAIL TO BE DEVELOPED. -> (AS A RESULT) COLLECTING DUCTS NEVER FORM -> (HENCE) INVOLUTION OF THE KIDNEY RESULTING IN RENAL AGENESIS.

Question 5

REGARDING CONGENITAL MALFORMATION OF URINARY TRACT:
A. RENAL HYPOPLASIA CAN BE UNILATERAL AND BILATERAL
B. RENAL HYPOPLASIA ALWAYS A/W NORMAL MORPHOLOGY BUT WITH REDUCED NUMBER OF NEPHRONS
C. ARPKD IS MUCH MORE COMMON THAN ADPKD.
D. ARPKD IS MORE PROGRESSIVE THAN ADPKD.
E. ADPKD FORM FROM ALL SEGMENTS OF NEPHRON AND DOES NOT CAUSE RENAL FAILURE TILL ADULTHOOD.

Answer 5

A. TRUE
B. TRUE
C. FALSE
D. TRUE
E. TRUE

Question 6

BRIEFLY DESC ARPKD.

Answer 6

ARPDKD
- 1/5000 BIRTHS
- PROGRESSIVE DISORDER
- CYST FORM FROM CD
- KIDNEY BECOME VERY LARGE
- RENAL FAILURE IN INFANCY
- D/T MUTATION OF GENES THT ENCODE PROTEINS IN CILIA

Question 7

BRIEFLY DESC ADPKD.

Answer 7

ADPKD
- 1/500 TO 1/1000
- FORM FROM ALL SEGMENTS OF NEPHRON
- DOES NOT CAUSE RF TILL ADULTHOOD
- LESS PROGRESSIVE
- D/T MUTATION OF GENES THAT ENCODE PROTEIN IN CILIA

Question 8

MALROTATED KIDNEY:
- IF KIDNEYS FAILS TO ROTATE ->
- IF KIDNEYS ROTATE LATERALLY ->
- IF KIDNEYS ROTATE TOO FAR ->
- COMMONLY A/W ->

Answer 8

• HILUM FACES ANTERIORLY
• HILUM FACES LATERALLY
• HILUM FACES POSTERIORLY
• ECTOPIC KIDNEY

Question 9

STATE EXAMPLES OF ECTOPIC KIDNEY.

Answer 9

• PELVIC KIDNEY
• CROSS RENAL ECTOPIA.

Question 10

BRIEFLY DESC PELVIC KIDNEY AND CROSS RENAL ECTOPIA.

Answer 10

PELVIC KIDNEY:
- RESULT FROM FAILURE OF KIDNEY TO ASCEND
- RECEIVE BLOOD SUPPLY FROM ARTERIES NEAR THEM

CROSS RENAL ECTOPIA:
- A KIDNEY CROSSES TO THE OTHER SIDE

Question 11

BRIEFLY DESC THE HORSESHOE KIDNEY.

Answer 11

• 1/600 BIRTH
• DURING ASCEND: KIDNEYS ARE PUSHED SO CLOSE TGT DURING THEIR PASSAGE THROUGH ARTERIAL FORK FORMED BY THE UMBILICAL ARTERY -> INF. POLE OF KIDNEY TO FUSE TGT
• FURTHER ASCEND IS PREVENTED BY THE ROOT OF INF. MESENTERIC ARTERY
• LOCATED AT LOWER LUMBAR LEVEL

Question 12

STATE THE DISEASE.
- LUMEN OF URACHUS PERSIST -> URINE DRAIN FROM UMBILICUS:
- LUMEN OF ONLY UPPER PART OF URACHUS PERSIST:
- LOCAL PART OF URACHUS PERSIST, SECRETORY ACTIVITY OF ITS LINING -> CYSTIC DILATION:

Answer 12

• LUMEN OF URACHUS PERSIST -> URINE DRAIN FROM UMBILICUS: URACHUS FISTULA
• LUMEN OF ONLY UPPER PART OF URACHUS PERSIST: URACHUS SINUS
• LOCAL PART OF URACHUS PERSIST, SECRETORY ACTIVITY OF ITS LINING -> CYSTIC DILATION: URACHUS CYST

Question 13

REGARDING EXTROPHY OF URINARY BLADDER:
A. VERY COMMON
B. EPISPADIAS IS A COMMON FEATURE
C. VENTRAL BODY WALL DEFECT -> EXPOSURE OF BLADDER MUCOSA
D. GENITAL SWELLING WIDELY SPACE -> DEFECT EXTERNAL GENITALIA
E.FAILURE OF POSTERIOR BODY WALL FOLDS TO CLOSE IN MIDLINE IN PELVIC REGION

Answer 13

A. FALSE (RARE: 2/100 000 BIRTH)
B. TRUE
C. TRUE
D. FALSE (EXTROPHY OF CLOACA)
E. FALSE (FAILURE OF LATERAL BODY WALL FOLDS TO CLOSE IN MIDLINE IN PELVIC REGION

Question 14

BRIEFLY DESC EXTROPHY OF CLOACA.

Answer 14

• RARE, 1/30 000 BIRTH
• MORE SEVERE VENTRAL BODY WALL DEFECT -> GREATER DEGREE OF DISRUPTION OF LATERAL BODY WALL FOLDS
• ALTERED URORECTAL SEPTUM DEVELOPMENT -> ANAL CANAL MALFORMATION, IMPERFORATED ANUS
• GENITAL SWELLING WIDELY SPACED -> DEFECT EXTERNAL GENITALIA

Question 15

BRIEFLY DESC ECTOPIC URETER.

Answer 15

• URETER OPENS ANYWHERE EXCEPT INTO URINARY BLADDER.
• IN MALE, URETER OPENS INTO NECK OF BLADDER, PROSTATIC URETHRA, DUCTUS DEFERENS, PROSTATIC UTRICLE, SEMINAL GLAND
• IN FEMALE, URETER OPENS INTO NECK OF BLADDER, URETHRA, VAGINA, VESTIBULE
• AS A RESULT, INCONTINENCE, URINE CONTINUOUSLY DRIBBLES FROM URETHRA/ VAGINA

Question 16

BRIEFLY DESC HYPOSPADIAS.

Answer 16

• 3/5/ 1000 BIRTHS
• FUSION OF URETHRAL FOLD IS INCOMPLETE.
• ABNORMAL OPENING OF URETHRA ALONG THE INFERIOR ASPECT OF PENNIS, NEAR THE GLAND, ALONG THE SHAFT, OR NEAR THE BASE OF PENIS.

Question 17

BRIEFLY DESC EPISPADIAS.

Answer 17

• RARE, 1/ 30 000 BIRTHS
• URETHRAL MEATUS ON DORSUM OF PENIS
• COMMONLY A/W EXTROPHY OF BLADDER AND ABNORMAL CLOSURE OF VENTRAL BODY WALL.