Hypospadias

Question 1

What is hypospadias?

Answer 1

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

Question 2

Epidemiology

Answer 2

1 in 300 males

Incidence is thought to be increasing

Question 3

Pathophysiology

Answer 3

Occurs due to arrest of penile development

This leads to hypoplasia of the ventral tissue of the penis

Question 4

Clinical features

Answer 4

Abnormal urinary flow i.e. not from the tip

Abnormal penile curvature during erections

Question 5

3 key features

Answer 5

Ventral opening of the urethral meatus

Ventral curvature of the penis or Chordee

Dorsal hooded foreskin

Question 6

Classifications

Answer 6

Glandular

Coronal

Shaft - distal, mid, proximal

Scrotal

Perineal

Question 7

Dx

Answer 7

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

Question 8

Ix

Answer 8

Make sure it is not a disorder of sex development

If there are concerns about DSD…

Detailed history and examination

Karyotyping

Pelvic USS

U&Es

Endocrine hormones like testosterone, 17 alpha-hydroxyprogesterone, LH FSH, ACTH, Renin Aldosterone

Question 9

Mainstay treatment

Answer 9

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

Question 10

Aims of urethroplasty

Answer 10

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.

Question 11

Short term complications

Answer 11

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

Question 12

Long term complications

Answer 12

Urethral fistula in 5-10% of distal repair and 30% of proximal

Risks of meatal or urethral stenosis which can require further intervention.