Hypospadias and Epispadias

Description
 The earliest medical text describing hypospadias dates back to the second century CE and was the work of
Galen, the first to use the term.

 Hypospadias is an abnormality of anterior urethral and penile development in which the urethral opening
is ectopically located on the ventral aspect of the penis proximal to the tip of the glans penis, which, in
this condition, is splayed open.
 Epispadias is a rare congenital malformation of the male or female urogenital apparatus that consists of a
defect of the dorsal wall of the urethra.

Pathophysiology
 The pathophysiology of hypospadias and epispadias occur as follows:

1. Hypospadias is a congenital defect that is thought to occur embryologically during urethral development,
between 8 and 20 weeks’ gestation.
2. The external genital structures are identical in males and females until 8 weeks’ gestation; the genitals
develop a masculine phenotype in males primarily under the influence of testosterone.
3. The classic theory is that the urethral folds coalesce in the midline from base to tip, forming a tubularized
penile urethra and median scrotal raphe.
4. The anterior or glanular urethra is thought to develop in a proximal direction, with an ectodermal core
forming at the tip of the glans penis, which canalizes to join with the more proximal urethra at the level
of the corona.
5. The prepuce normally forms as a ridge of skin from the corona that grows circumferentially, fusing with
the glans.
6. Failure of fusion of the urethral folds in hypospadias impedes this process, and a dorsal hooded prepuce
results.
7. In males, epispadias causes impotentia coeundi, which results from the dorsal curvature of the penile
shaft, and impotentia generandi, which results from the incomplete urethra.
8. Also reported are frequent ascending infections to the prostate or bladder and kidneys and psychological
problems related to the deformity.
9. If epispadias is distal to the bladder neck, urinary continence may not be present.

Statistics and Incidences

 Hypospadias and epispadias occur across the world and may go untreated and neglected.

 Hypospadias occurs in approximately 1 in every 250 male births in the United States.
 In general, the frequency seems rather constant, at 0.26 per 1000 live births in Mexico and Scandinavia
and 2.11 per 1000 live births in Hungary.
 The incidence of hypospadias is higher in whites than in blacks, and the condition is more common in
those of Jewish and Italian descent.
 Epispadias occurs more commonly in males than in females, with a prevalence of 1 case in 10,000-50,000
persons; the male-to-female ratio is 2.3:1.
Causes
 Several etiologies for hypospadias have been suggested, including genetic, endocrine, and environmental
factors.

 Genetic. A genetic predisposition has been suggested by the eightfold increase in incidence of
hypospadias among monozygotic twins as compared with singletons.
 Endocrine. A decrease in available androgen or an inability to use available androgen appropriately may
result in hypospadias.
  Environmental. Endocrine disruption by environmental agents is gaining popularity as a possible etiology
for hypospadias and as an explanation for its increasing incidence.
Unlike hypospadias, epispadias can be explained by:

 Defective migration. There is a defective migration of the paired primordia of the genital tubercle that
fuse on the midline to form the genital tubercle at the fifth week of embryologic development.
 Abnormal development. Another hypothesis relates the defect to the abnormal development of the
cloacal membrane.

Clinical Manifestations
 Physical examination of the child with hypospadias reveal the following:

 Dorsal hood. A dorsal hood of foreskin and glanular groove are evident, but upon closer inspection, the
prepuce is incomplete ventrally and the urethral meatus is noted in a proximally ectopic position.
 Chordee. Chordee may be readily apparent or may be discernible only during erection.
 Physical examination of the child with epispadias reveal the following:

 Mucosal strip. The normal urethra is replaced by a broad mucosal strip lying on the dorsum of the
corpora cavernosa; the meatus is divided dorsally between the tip of the glans and the pubis, the penile
shaft is curved dorsally with the absence of the preputial apron, and a cleft is present on the upper
surface of the penis

Assessment and Diagnostic Findings

 Hypospadias and epispadias are diagnosed mainly through physical examination. Upper urinary tract
anomalies are rarely associated with hypospadias and do not justify routine imaging in these patients unless
other organ system anomalies are present.

Medical Management
 Hypospadias is generally repaired for functional and cosmetic reasons. Surgical repair is desirable between
the ages of 6 and 18 months.

 Tubularized incised plate (TIP). The tubularized incised plate (TIP) repair has become the most commonly
used repair for both distal and midshaft hypospadias.
 Adjuvant hormonal therapy. Although no corrective medical therapy for hypospadias is known, hormonal
therapy has been used as an adjuvant to surgical therapy in infants with exceptionally small phallic size.
 No medical treatment corrects epispadias, only surgical repair could correct it.

 Single stage procedure. In glandular epispadias with a straight penis, local flaps based from the glans are
often used to reconstruct the missing distal urethra.

Nursing Assessment

 History. Obtain a thorough history and physical examination, including any history of a familial pattern of
hypospadias, any past medical history or comorbidity, and a physical assessment focusing on the meatal
location, glans configuration, skin coverage, and chordee.
 Physical examination. Although the diagnosis of hypospadias has been made with both antenatal fetal
ultrasonography and magnetic resonance imaging (MRI), the diagnosis is generally made upon
examination of the newborn infant.

Nursing Diagnoses

 Acute pain related to physical factors: damage to the skin/tissue.

 Impaired skin integrity related to surgical trauma.
  Impaired urinary elimination related to mechanical trauma from surgery.
 Anxiety related to threat to self-concept; change in health status and environment.
 Risk for infection related to inadequate primary defenses.

Nursing Care Planning and Goals

 Child will experience decreased pain as evidenced by infrequent crying episodes and exhibit normal
sleeping pattern.
 Child will experience improved urinary elimination.
 Parent will experience less anxiety.
 Child will remain free from infection as evidenced by clean and intact wound without redness, edema,
odor or drainage and negative urine culture.

Nursing Interventions

 Relief from pain. Encourage use of relaxation techniques; apply ice compress as indicated; and educate
parents that medications will prevent pain and restlessness and allow for healing.
 Improve urinary elimination. Encourage high fluid intake after catheter removed, offer favored choice of
liquids hourly; and instruct parents to notify the physician of changes in the urinary pattern or inability to
void.
 Lessen anxiety. Encourage verbalization of concerns and allow time for parents and child to ask questions
about condition, procedures, recovery; Answer questions calmly and honestly; use pictures, drawings,
and models for information; and reassure parents and child that defect or surgery will not compromise
sexual the activity and will not affect reproductive ability.
 Prevent infection. Obtain urine specimen for culture and sensitivities as indicated; inform parents to avoid
allowing the child to straddle toys, play in a sandbox, swim, or engage in rough activities until advised by
the physician; and apply sterile technique during dressing changes, catheter care or draining urine bag.

Evaluation

 Child will experience decreased pain as evidenced by infrequent crying episodes and exhibit normal
sleeping pattern.
 Child will experience improved urinary elimination.
 Parent will experience less anxiety.
 Child will remain free from infection as evidenced by clean and intact wound without redness, edema,
odor or drainage and negative urine culture.

Documentation Guidelines

 Client’s description of response to pain.

 Acceptable level of pain.
 Current antibiotic therapy.
 Plan of care.
 Teaching plan.
 Response to interventions, teaching, and actions performed.
 Attainment or progress towards desired outcomes.
 Modifications to plan of care.