What They Are
Hypospadias is an anterior urethral and penile development defect: the urethral opening sits on the ventral aspect of the penis, proximal to the tip of the glans, with the glans splayed open. Epispadias is a rarer urogenital malformation, a defect of the dorsal wall of the urethra.
Pathophysiology
Hypospadias develops embryologically during urethral formation, between 8 and 20 weeks gestation. External genitalia are identical in both sexes until 8 weeks gestation; the masculine phenotype then develops primarily under testosterone. Normally the urethral folds coalesce in the midline from base to tip, forming a tubularized penile urethra and median scrotal raphe, while the anterior (glanular) urethra develops proximally as an ectodermal core canalizes at the level of the corona, and the prepuce grows circumferentially from the corona to fuse with the glans. In hypospadias the urethral folds fail to fuse, the process stalls, and a dorsal hooded prepuce results.
In males, epispadias causes impotentia coeundi from dorsal curvature of the penile shaft and impotentia generandi from the incomplete urethra. Expect frequent ascending infections to the prostate, bladder, and kidneys, plus psychological burden from the deformity. When epispadias is distal to the bladder neck, urinary continence may be absent.
Statistics and Incidences
Hypospadias occurs in about 1 in 200 male births in the United States (CDC). Frequency is fairly constant worldwide, from 0.26 per 1000 live births in Mexico and Scandinavia to 2.11 per 1000 live births in Hungary. It is more common in whites than in blacks and in those of Jewish and Italian descent. Epispadias is more common in males, with a prevalence of 1 case in 10,000 to 50,000 persons and a male-to-female ratio of 2.3:1.
Causes
Hypospadias has genetic, endocrine, and environmental drivers. The eightfold higher incidence in monozygotic twins versus singletons points to genetic predisposition. Reduced available androgen, or an inability to use it appropriately, can also cause it, and endocrine disruption by environmental agents is increasingly proposed as a cause and as an explanation for its rising incidence.
Epispadias is explained instead by defective migration of the paired primordia of the genital tubercle, which normally fuse in the midline at the fifth week of embryologic development, or by abnormal development of the cloacal membrane.
Clinical Manifestations
In hypospadias, look for a dorsal hood of foreskin and glanular groove; on closer inspection the prepuce is incomplete ventrally and the meatus sits in a proximally ectopic position. Chordee may be obvious or apparent only on erection.
In epispadias, 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 shaft curves dorsally with no preputial apron, and a cleft sits on the upper surface of the penis.
Assessment and Diagnostic Findings
Both are diagnosed on physical examination. Upper urinary tract anomalies are rarely associated with hypospadias and do not justify routine imaging unless other organ system anomalies are present.
Medical Management
Hypospadias is repaired for function and cosmesis, ideally between 6 and 18 months of age. The tubularized incised plate (TIP) repair is now the most common technique for both distal and midshaft hypospadias. No corrective medical therapy exists, but hormonal therapy has been used as a surgical adjuvant in infants with exceptionally small phallic size.
Epispadias has no medical treatment; only surgical repair corrects it. In glandular epispadias with a straight penis, a single-stage procedure using local flaps based from the glans reconstructs the missing distal urethra.
Nursing Management
Nursing Assessment
Take a thorough history and physical, including any familial pattern of hypospadias and any comorbidity, with the exam focused on meatal location, glans configuration, skin coverage, and chordee. Diagnosis has been made with antenatal ultrasonography and MRI, but it is usually made on examination of the newborn.
Nursing Diagnoses
- Acute pain related to skin and tissue damage.
- Impaired skin integrity related to surgical trauma.
- Impaired urinary elimination related to mechanical trauma from surgery.
- Anxiety related to threat to self-concept and change in health status and environment.
- Risk for infection related to inadequate primary defenses.
Nursing Care Planning and Goals
- Child reports or shows decreased pain (infrequent crying, normal sleep pattern).
- Child shows improved urinary elimination.
- Parents show less anxiety.
- Child stays free from infection (clean, intact wound without redness, edema, odor, or drainage; negative urine culture).
Nursing Interventions
Relief from pain: use relaxation techniques, apply ice compress as indicated, and teach parents that scheduled medication prevents pain and restlessness and lets healing proceed.
Improve urinary elimination: push fluids once the catheter is out, offer preferred liquids hourly, and tell parents to report any change in urinary pattern or inability to void.
Lessen anxiety: let parents and child voice concerns and ask about the condition, procedure, and recovery; answer calmly and honestly; use pictures, drawings, and models; and reassure them the defect and surgery will not compromise sexual activity or reproductive ability.
Prevent infection: obtain urine for culture and sensitivity as indicated; tell parents to keep the child from straddling toys, sandbox play, swimming, or rough activity until cleared; and use sterile technique for dressing changes, catheter care, and emptying the drainage bag.
Evaluation
Goals are met when the child shows decreased pain and a normal sleep pattern, improved urinary elimination, less parental anxiety, and remains infection-free with a clean intact wound and negative urine culture.
Documentation Guidelines
Document the child's description of and acceptable level of pain, current antibiotic therapy, plan of care, teaching plan, response to interventions and teaching, progress toward outcomes, and any modifications to the plan.
Frequently Asked Questions
What is the difference between hypospadias and epispadias? In hypospadias the urethral opening sits on the ventral (underside) of the penis; in epispadias it sits on the dorsal (upper) surface, or near the clitoris in females. Both are congenital defects in where the urethra opens.
How common is hypospadias? It is one of the most common birth defects in boys, occurring in about 1 in 200 male births in the United States (CDC).
When is hypospadias repaired? Surgery is usually done between 6 and 18 months of age for function and appearance. The tubularized incised plate (TIP) repair is now the most common technique (Hypospadias, StatPearls).
Will hypospadias affect sexual function or fertility? Families worry about this, but parents can be reassured that the defect and its surgical repair are not expected to compromise sexual activity or reproductive ability.
What does the nurse watch for after repair? Protect the surgical repair and keep any urinary stent or catheter patent, watch for infection, support a return to voiding, use sterile technique for dressing and catheter care, and keep the child from straddling toys or rough play until cleared.