Uro Emergencies In Kiddies

Question 1

Prune belly syndrome

Triad, pathology& management

Answer 1

Triad: absence of abdominal wall musculature, UDT, anomalies of urogenital tract.

Pathology: abdominal wall( shrivel and prune-like) ureters(dilated and tortuous, VUR) kidneys (small +/- hydronephrosis) bladder ( large volume, +/- patent urachus) hypo plastic prostate,megalourethra, bilateral UDT,dimple on Lat sides of knees.

Management: bladder prophyl antibiotic for UTI, +/- surgery, UDT orchidopexy, abdo wall (poor cough reflex but abdo wall gets better with age)

Question 2

Female pseudo hermaphrodite

Answer 2

Chromosome 46XX
Two ovaries and normal female internal genitalia
Ambiguous ext genitalia
CAUSE congenital adrenal hyperplasia CAH
Congen enzyme deficiencies of gluco +/- mineralo-corticosteroids
There is no enzyme defect in androgen pathway causing excess precursors into androgens
Present: salt and water wasting, hpt, hyperpigmentation.
Diagnostic: high 17 hydroxyl progesterone
Mx: replacement steroid therapy.

Question 3

Neonatal testicular tortion

Answer 3

Testis become fixed to scrotum 7-10 days of life.
Extravaginal torsion, entire testis complex twists en bloc
Most common 85% prenatal 32 weeks gestation
Hard swollen non tender testis with a dusky hemi scrotum.
Immediate surgical exploration and contralateral testes orchidopexy

Question 4

Posterior urethral valves PUJ

Answer 4

Thin membrane obstructing urethra just distal to verumontanum.
The more severe the problem, the earlier the presentation.
2/3 of pts

Question 5

Neonatal Anuria:
Causes of anuria vs retention

NB‼️ by 24 hours all normal neonates must pass urine, if not investigate!

Answer 5

Anuria
Bilateral renal agenesis (suggested by Potters faces)
Vascular catastrophe: renal vein thrombosis, renal art thrombosis, renal cortical necrosis.

Retention
Obstruction: PUV, neuropathic bladder (myelomeningocele)
Maternal drug administration: magnesium sulphate for pre-eclampsia