Anterior urethral valve
- Less frequent than PUV, it is a semi-lunar band of tissue on the ventral aspect of urethra, often at the bulbar or penile urethra
- May present with poor stream, ballooning, UTI or haematuria
- May be confused with or have concurrent urethral diverticulum
- May present with similar obstructive findings to PUV – trabeculated bladder, VUR, dilated posterior urethra
- Treat with endoscopic resection of the valve
Syringocele
- Cystic dilation of the Cowper’s glands (bulbourethral glands) which sit either side just distal to the urethral sphincter
- May be congenital, or acquired after trauma or infection
- Can present with post void dribble, urethral discharge, UTIs, perineal pain, haematuria, obstructive LUTS, dysuria or urinary retention
- Urethrogram shows a filling defect distal to the prostate, and US/MRI may also be used
- Endoscopic deroofing is usually effective
Labial adhesions
- Relatively common finding in young girls
- Thin, atrophic labial skin
- Reassurance and conservative approach is standard. Never seen into adolescents or adulthood.
- Labial traction and topical oestrogen probably unnecessary but may help parental anxiety
- May be associated with vaginal reflux/pooling of urine
Denys-Drash syndrome
- Triad of congenital nephropathy, Wilms tumour and DSD
- Mutations in WT1 gene
Zinner syndrome
- Mesonephric (Wolffian) duct abnormality
- Triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst and ejaculatory duct obstruction
Neuroblastoma
- Malignant tumour in young children under 5
- Most common extracranial solid tumour in kids
- Neuroendocrine tumour, often in adrenal medulla or sympathetic chain
- Often metastatic at diagnosis
- Elevated urinary VMA
- Manage with excision +/- chemo
Principles of operating on children
- Balance between need for operation and safe age to have general anaesthesia, and painful memories/co-operation of child.
- Magnification with Loupes useful.
- Specialist paediatric anaesthesia and nursing staff.
- Day cases whenever possible.
Whitaker test
- First described in 1973 – might be useful in differentiated a dilated non obstructed system from an obstructed one
- Percutaneous needle placed in collecting system, and contrast infused at 10 mL / min
- Urodynamic catheter placed in the bladder, and intravesical pressures monitored and subtracted from intrapelvic pressures during infusion
- Pressures recorded when contrast passes PUJ and VUJ
- Intrapelvic pressure < 15 cmH2O normal, > 22 cmH2O obstructed, 15 – 22 indeterminate