History

Fact Explanation
Family history of hypospadias. The cause of hypospadias is polygenic. The risk of ocurrence in an infant is 8% if his father has hypospadias and 14% if a sibling has the conditon; if two members of the same family have hypospadias, the risk is about 21% [1].
History of infertility and treatment for infertility. In-vitro fertilization is known to be associated with this condition [2].
References
  1. DUCKETT J. W.. Hypospadias. Pediatrics in Review [online] 1989 August, 11(2):37-42 [viewed 21 August 2014] Available from: doi:10.1542/pir.11-2-37
  2. ERICSON A., KALLEN B.. Congenital malformations in infants born after IVF: a population-based study. Human Reproduction [online] 2001 March, 16(3):504-509 [viewed 21 August 2014] Available from: doi:10.1093/humrep/16.3.504

Examination

Fact Explanation
A ventrally incomplete, dorsally hooded prepuce and a urethral meatus situated proximal to the tip of the glans penis (most commonly in the subcoronal position). If the genital folds fail to fuse in the midline, the preputial tissues do not develop ventrally. Consequently, they form in an excessive manner dorsally. Because the distal penile urethra ends normally at the subcoronal area and the glans channel from the tip fuses downward, this is the most common anomaly making up 65% to 70% of cases [1].
Chordee- ventral curvature of the penis. It is caused by atrophy of the corpus spongiosum, fibrosis of the tunica albuginea and fascia over the tunica, tightness of the ventral skin and Buck's fascia, tethering of the penile shaft skin onto the underlying structures, or tethering of the urethral plate onto the corpora cavernosa [2].
Detailed examination assessing for other abnormalities. hypospadias (especially proximal hypospadias) may be one of the features of over 200 different syndromes. Therefore other associated anomalies such as undescended testes, inguinal hernia, etc are common [3].
References
  1. DUCKETT J. W.. Hypospadias. Pediatrics in Review [online] 1989 August, 11(2):37-42 [viewed 21 August 2014] Available from: doi:10.1542/pir.11-2-37
  2. LEUNG ALEXANDER K. C., ROBSON WILLIAM L. M.. Hypospadias: an update. Asian J Andrology [online] 2007 January, 9(1):16-22 [viewed 21 August 2014] Available from: doi:10.1111/j.1745-7262.2007.00243.x
  3. MANZONI GIANANTONIO, BRACKA AIVAR, PALMINTERI ENZO, MARROCCO GIACINTO. Hypospadias surgery: when, what and by whom?. [online] 2004 November, 94(8):1188-1195 [viewed 23 August 2014] Available from: doi:10.1046/j.1464-410x.2004.05128.x

Differential Diagnoses

Fact Explanation
Ambiguous genitalia. It also could present with the urethra opening at an ectopic site but the testes will not be palpable [1].
Penile torsion. It also presents with an abnormally placed urethra but it is due to abnormal penile rotation which is usually counterclockwise and more common on the left side [2].
References
  1. OGILVY-STUART A L. Early assessment of ambiguous genitalia. Archives of Disease in Childhood [online] 2004 May, 89(5):401-407 [viewed 22 August 2014] Available from: doi:10.1136/adc.2002.011312
  2. MONTAG SYLVIA, PALMER LANE S.. Abnormalities of Penile Curvature: Chordee and Penile Torsion. The Scientific World JOURNAL [online] 2011 December, 11:1470-1478 [viewed 22 August 2014] Available from: doi:10.1100/tsw.2011.136

Investigations - Followup

Fact Explanation
Uroflowmetry and bladder ultrasonography performed at several stages of life, following surgery. To assess the quality of micturition which could be affected by the surgical correction [1].
References
  1. MANZONI GIANANTONIO, BRACKA AIVAR, PALMINTERI ENZO, MARROCCO GIACINTO. Hypospadias surgery: when, what and by whom?. [online] 2004 November, 94(8):1188-1195 [viewed 23 August 2014] Available from: doi:10.1046/j.1464-410x.2004.05128.x

Investigations - Screening/Staging

Fact Explanation
Renal ultrasonography for patients with posterior hypospadias. Associated malformations of the urinary tract are most common in proximal or complex hypospadias [1].
Karyotype. If one or both testes are impalpable, it may signify the presence of an intersex condition such as adrenogenital syndrome or a mixed gonadal dysgenesis [1].
References
  1. MANZONI GIANANTONIO, BRACKA AIVAR, PALMINTERI ENZO, MARROCCO GIACINTO. Hypospadias surgery: when, what and by whom?. [online] 2004 November, 94(8):1188-1195 [viewed 23 August 2014] Available from: doi:10.1046/j.1464-410x.2004.05128.x

Management - General Measures

Fact Explanation
Testosterone enanthate : 25mg administered intramuscularly 1 month before surgery or topical dihydrotestosterone cream, applied daily for 1 month. It is administered when micropenis poses as a problem or if there is insufficient foreskin for repair [1].
References
  1. LEUNG ALEXANDER K. C., ROBSON WILLIAM L. M.. Hypospadias: an update. Asian J Andrology [online] 2007 January, 9(1):16-22 [viewed 21 August 2014] Available from: doi:10.1111/j.1745-7262.2007.00243.x

Management - Specific Treatments

Fact Explanation
Surgical correction between 6 to 12 months of age. The advantages of early surgery include easier after care, which includes better restraint for hygienic purposes and less likelihood of urinary catheter dislodgement, less separation anxiety, less need for analgesia, less postoperative emotional disturbance, and better parent-infant bonding. After 6 months of age, the anesthetic risk is no greater than when the child is older [1].
Urethral plate tubularization. When the urethral plate does not require transection and its axial integrity can be maintained, it is possible to tubularize it [2].
Urethral plate augmentation. it is done in the presence of narrow and inelastic urethral plates and when the glans configuration is more conical, with a minimal groove and lacking the usual external rotation of the glans wings [2].
Urethral plate substitution. In the presence of severe proximal forms, with significant ventral chordee, urethral plate transection becomes inevitable, and a full circumferential substitution urethroplasty is required [2].
References
  1. LEUNG ALEXANDER K. C., ROBSON WILLIAM L. M.. Hypospadias: an update. Asian J Andrology [online] 2007 January, 9(1):16-22 [viewed 21 August 2014] Available from: doi:10.1111/j.1745-7262.2007.00243.x
  2. MANZONI GIANANTONIO, BRACKA AIVAR, PALMINTERI ENZO, MARROCCO GIACINTO. Hypospadias surgery: when, what and by whom?. [online] 2004 November, 94(8):1188-1195 [viewed 23 August 2014] Available from: doi:10.1046/j.1464-410x.2004.05128.x