Incidence

• Hypospadias is a very common congenital anomaly (1 in 300 male births). It is most often an isolated finding but may be associated with other abnormalities. ¹
• The incidence is increased if first degree relatives are affected. Up to 26% of male offspring of an affected father may have hypospadias, and the risk in subsequent siblings is 12%. ²
• It is more common in male infants who are growth restricted and premature. Other risk factors include parental subfertility. ³

Definition and Description

Hypospadias (hypo = below; spadon = a fissure or a ‘hole’) consists of some or all of the following features: Ventral displacement of the urethral meatus (hypospadias). Incomplete formation of the prepuce (dorsal 'hooding') Ventral curvature (chordee). Urethral meatal openings are generally described as being: Anterior – where the meatus is near the tip of the penis Middle – where the meatus is along the shaft of the penis Posterior – where the meatus is near the base of the penis or in the scrotum   Early recognition and paediatric urological referral is useful for counselling and planning timing of surgery

Investigations

• The baby requires a complete examination to determine whether other external abnormalities are present.
• Penile length should be determined
  • Normal stretched penile length at term >2.5cm
  • If penile length <2.5cm, refer to the Paediatric Endocrinology service for assessment. Testosterone may be indicated.
• Renal ultrasound is not indicated for simple anterior or middle hypospadias unless there are other features of concern (for example, dysmorphic features).
  • Other conditions, such as an enlarged utricle, may be present particularly with posterior hypospadias
  • Renal abnormalities are more common with more posterior hypospadias.  Renal ultrasound is the investigation of choice initially.
• Posterior hypospadias – particularly in the absence of palpable gonads – should be regarded as an intersex disorder and investigated appropriately.

Surgical Management

• Parents should be reassured that hypospadias is a common condition which can be corrected with surgery.
• Surgery is performed by the Paediatric Urologists at Starship Children’s Hospital.
• Surgery is usually undertaken between 6 and 18 months, although timing will depend on the surgeon and other factors. Often more than one procedure is required and it is preferable to complete all stages in early childhood.
• It is critical that parents are told that circumcision should not be performed as the foreskin remnant is required for surgical repair.
• The surgical principles are:
  • To reposition the meatus on to the head of the penis (meatoplasty and glanduloplasty)
  • To straighten the chordee (othoplasty)
  • To correct the hooded foreskin (by circumcision)
  • To achieve all of this with an aesthetically acceptable result

Referrals

• Babies should be referred on discharge to the paediatric surgical service at Starship Children’s Hospital.
• Send referrals to the Department of Paediatric Surgery – Fax 307 8952
• Alternatively, contact a paediatric urologist directly.

References