Structural Anomalies

Question 1

Features of Potter’s syndrome (7)

Answer 1

(collection of symptoms due to oligohydramnios)

pulmonary hypoplasia

clubbed feet

cranial anomalies

parrot nose, flat nose, low ears

dry, loose skin

skin fold from medial canthus to cheek

various GI atresias

Question 2

Causes of Potter’s syndrome (9)

Answer 2

BILATERAL RENAL AGENESIS

ureteric/urethral atresia

PCKD: AR presents early in childhood, AD in adulthood

bilateral multicystic kidney disease

renal hypoplasia

dysplastic kidney

amniotic rupture

toxaemia

uteroplacental insufficiency

Question 3

Other renal anomalies (4)

Answer 3

duplex collecting system

horseshoe kidney: may predispose to infection/impaired urinary drainage

bladder exstrophy: may occur w. epispadias

vesico-ureteric junction obstruction

Question 4

Features and presentation of posterior urethral valves (4)

Answer 4

only in boys

congenital membrane-like structure located in the distal prostatic urethra

antenatally: progressive bilateral hydonephrosis, oligohydramnios, pulmonary hypoplasia
postnatally: distended and palpable bladder

Question 5

Dx of posterior urethral valves

Answer 5

MCUG

Question 6

Mx of posterior urethral valves (3)

Answer 6

propylactic Abx to prevent UTI

catheterisation

endoscopic valve ablation

Question 7

Mx of labial adhesions (3)

Answer 7

can be left alone if asymptomatic> often lyse spontaneously

if symptomatic e.g. UTIs/soreness: oestrogen creams can lyse

surgery rarely required

Question 8

Causes of vulvovaginitis/vaginal discharge (4)

Answer 8

bacterial/fungal abuse

specific irritants

poor hygiene

sexual abuse

(v. rarely threadworm infestation)

Question 9

Mx of vulvovaginitis/vaginal discharge in children (2)

Answer 9

avoid bubble baths/scented soaps

oestrogen creams increase resistance to infection

Question 10

Mx of bilateral undescended testes (3)

Answer 10

NEED TO RULE OUT CAH:

• MRI+/- USS
• laparoscopy
• endocrine tests

Question 11

general Mx of undescended testes (3)

Answer 11

if undescended by 3mo, cryptorchidism diagnosed

refer to surgery by 6mo

orchidoplexy between 1-2yrs

Question 12

Long term consequences of cryptorchidism (3)

Answer 12

increased risk of:

• infertility
• torsion
• testicular ca.
• psychological problems

Question 13

Other features of hypospadias (2)

Answer 13

underdeveloped foreskin

downward bending penis

Question 14

Feature and assoc. of hypospadias (2)

Answer 14

urethral meatus not in correct place-most are distal i.e still in glans

assoc. w. undescended testes

Question 15

Advice to parents, Mx of hypospadias and its complications

Answer 15

advise not to circumcise as skin may be needed for reconstruction

surgery can be carried out >3mo

risk of stenosis of new meatus

Question 16

Features of torsion of the Hydatid of Morgagni (3)

Answer 16

like torsion but:

• longer Hx
• cremaster reflex preserved
• transillumination>blue dot visible

(most common cause of acute scrotum in children)

(HoM=embryological remnant at top of testes)

Question 17

Mx of torsion of the Hydatid of Morgagni

Answer 17

benign, no Rx required