What is hypospadias?
A congenital defect causing the urethral meatus to be located at an abnormal site
This is usually on the under side of the penis rather than tipe
1 in 300 males
Incidence is thought to be increasing
Occurs due to arrest of penile development
This leads to hypoplasia of the ventral tissue of the penis
Abnormal urinary flow i.e. not from the tip
Abnormal penile curvature during erections
3 key features
Ventral opening of the urethral meatus
Ventral curvature of the penis or Chordee
Dorsal hooded foreskin
Shaft - distal, mid, proximal
If associated with unilateral or bilateral undescended testis CAH might be diagnosis that must not be missed
If not detected early it can lead to salt wasting crisis due to cortisol and aldosterone deficiency with androgen excess
Make sure it is not a disorder of sex development
If there are concerns about DSD...
Detailed history and examination
Endocrine hormones like testosterone, 17 alpha-hydroxyprogesterone, LH FSH, ACTH, Renin Aldosterone
Urethroplasty either as single stage or 2-stage repair using a graft.
This is preferentially by preputial graft harvested from the foreskin.
This means they need advise against circumcision
Aims of urethroplasty
Bring the meatus to the glans of the penis
Chordee is corrected to straighten the penis
Dorsal foreskin is managed with either circumcision or reconstruction.
Note it is important to make sure family understand that circumcision should not be performed until after discussion about reconstruction with a specialist has occured. Since it can form a vital part of reconstruction.
Short term complications
Urethral cathether is usually needed to protect surgical reconstruction, and this can block.
The cath can also cause pain and bladder spasms, however urine must be freely draining before assigning symptoms to bladder spasms.
Bleeding and infection can also occur
Long term complications
Urethral fistula in 5-10% of distal repair and 30% of proximal
Risks of meatal or urethral stenosis which can require further intervention.