Hypospadias and Epispadias

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Hypospadias and Epispadias

Gino, a 3-month-old baby, was discovered to have hypospadias. His parents worry that this will affect his elimination in the future. Their physician assured them that by the age of 6 to 18 months, Gino would be prepared for the reconstruction of his penis. 

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 episadias 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 Management


Nursing management of a child with hypospadias or epispadias inlude:

Nursing Assessment

Assessment of a child with hypospadias or epispadias include the following:

  • 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

Based on the assessment data, the major nursing diagnoses are:

Nursing Care Planning and Goals

Main Article: 4 Hypospadias and Epispadias Nursing Care Plans

The major nursing care plan goals for a child with hypospadias and/or epispadias are:

  • 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

Nursing interventions for the child are:

  • 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

Goals are met as evidenced by:

  • 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

Documentation in a patient with hypospadias and epispadias include:

  • 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.

Practice Quiz: Hypospadias and Epispadias


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1. The parents of a newborn male with hypospadias want their child circumcised. The best response by the nurse is to inform them that:

A. Circumcision is delayed so the foreskin can be used for the surgical repair.
B. This procedure is contraindicated because of the permanent defect.
C. There is no medical indication for performing a circumcision on any child.
D. The procedure should be performed as soon as the infant is stable.

1. Answer: A. Circumcision is delayed so the foreskin can be used for the surgical repair.

  • Option A: Even if mild hypospadias is suspected, circumcision is not done in order to save the foreskin for surgical repair, if needed.
  • Options B, C, D: Options B, C, and D are incorrect.

2. Niklaus was born with hypospadias; which of the following should be avoided when a child has such condition?

A. Surgery.
B. Circumcision.
C. Intravenous pyelography (IVP).
D. Catheterization.

2. Answer: B. Circumcision.

  • Option B: Hypospadias refers to a condition in which the urethral opening is located below the glans penis or anywhere along the ventral surface (underside) of the penile shaft. The ventral foreskin is lacking, and the distal portion gives an appearance of a hood. Early recognition is important so that circumcision is avoided; the foreskin is used for the surgical repair.
  • Option A: Surgery is the procedure of choice to improve the child’s ability to stand when urinating, improve the appearance of the penis, and preserve sexual adequacy.
  • Option C: IVP is contraindicated if the child has an allergy to iodine or shellfish.
  • Option D: Catheterization may be used to ensure urinary elimination.

3. An 18-month-old is being discharged following hypospadias repair. Which instruction should be included in the nurse’s discharge teaching?

A. The child should not play on his rocking horse.
B. Applying warm compresses to decrease pain.
C. Diapering should be avoided for 1–2 weeks.
D. The child will need a special diet to promote healing.

3. Answer: A. The child should not play on his rocking horse.

  • Option A: The child will need to avoid straddle toys, swimming, and rough play until allowed by the surgeon.
  • Options B, C, D: Answers B, C, and D do not relate to the post-operative care of the child with hypospadias; therefore, they are incorrect.

4. Assessment of a newborn male reveals that the infant has hypospadias. The nurse knows that:

A. The infant should not be circumcised.
B. Surgical correction will be done by 6 months of age.
C. Surgical correction is delayed until 6 years of age.
D. The infant should be circumcised to facilitate voiding.

4. Answer: A. The infant should not be circumcised.

  • Option A: The infant with hypospadias should not be circumcised because the foreskin is used in reconstruction.
  • Options B&C: Answer B and C are incorrect because reconstruction is done between 16 and 18 months of age, before toilet training.
  • Option D: Answer D is incorrect because the infant with hypospadias should not be circumcised.

5. A full-term male has hypospadias. Which statement describes hypospadias?

A. The urethral opening is absent.
B. The urethra opens on the dorsal side of the penis.
C. The penis is shorter than usual.
D. The urethral meatus opens on the underside of the penis.

5. Answer: D. The urethral meatus opens on the underside of the penis. 

  • Option D: Hypospadias is a congenital abnormality in which the urethral meatus is on the underside of the penis.
  • Option A, B, C: Options A B, and C are incorrect.

See Also


Related topics to this study guide:

Further Reading


Recommended resources and books for pediatric nursing:
  1. PedsNotes: Nurse's Clinical Pocket Guide (Nurse's Clinical Pocket Guides)
  2. Pediatric Nursing Made Incredibly Easy
  3. Wong's Essentials of Pediatric Nursing
  4. Pediatric Nursing: The Critical Components of Nursing Care
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