HYPOSPADIASIS AND EPISPADIASIS

INTRODUCTION

Congenital malformations of the male genitalia include a wide range of clinical conditions such as hypospadias, epispadias (in females, bifid clitoris) with bladder exstrophy, and hidden penis. Epispadias is an uncommon congenital malformation of the penis and is often part of the condition termed epispadias-exstrophy of the bladder.

HYPOSPADIASIS

DEFINITION

Hypospadias is a somewhat common birth (congenital) defect in which the opening of the urethra is on the underside, rather than at the end, of the penis.

INCIDENCE

Hypospadias affects up to 4 in 1,000 newborn boys.

CAUSES

Some cases are passed down through families. In other cases the cause is unknown.

CLASSIFICATION

Hypospadias is classified according to the position of the urethral meatus after taking into account whether chordee is present ( Fig. 544-1 ). The deformity is described as glanular (on the glans penis), coronal, subcoronal, midpenile, penoscrotal, scrotal, or perineal. Approximately 60% of cases are distal, 25% are subcoronal or midpenile, and 15% are proximal. In the most severe cases, the scrotum is bifid and sometimes extends to the dorsal base of the penis (scrotal engulfment). There also is a megameatal variant, in which the foreskin is developed normally, but there is either distal or subcoronal hypospadias with a “fish mouth” meatus. These cases may not be diagnosed until after a circumcision is performed.

SYMPTOMS

The condition varies in severity. In most cases, the opening of the urethra is located near the tip of the penis on the underside. More severe forms of hypospadias occur when the opening is at the midshaft or base of the penis. Occasionally, the opening is located in or behind the scrotum.

Males with this condition often have a downward curve (chordee) of the penis during an erection. (Erections are common with infant boys.)

Other symptoms include:

Abnormal spraying of urine

Having to sit down to urinate

Malformed foreskin that makes the penis look "hooded"

DIAGNOSIS

A physical examination can diagnose this condition. Imaging tests may be needed to look for other congenital defects.

COMPLICATIONS

(1) deformity of the urinary stream, either ventral deflection or severe splaying

(2) sexual dysfunction secondary to penile curvature

(3) infertility if the urethral meatus is proximal

(4) meatal stenosis (congenital),

Treatment

Infants with hypospadias should not be circumcised. The foreskin should be preserved for use in later surgical repair.

URETHROPLASTY; MEATOPLASTY; GLANULOPLASTY

Surgery is usually done before the child starts school. Today, most urologists recommend repair before the child is 18 months old. During the surgery, the penis is straightened and the hypospadias is corrected using tissue grafts from the foreskin. The repair may require multiple surgeries.

Hypospadias repair is surgery to correct a birth defect in boys in which the urethra (the tube that carries urine from the bladder to outside the body) does not end at the tip of the penis. Instead, it ends on the underside

Description

Hypospadias repair is usually done when boys are between 6 months and 2 years old. It is done on an outpatient basis. It rarely requires an overnight stay in the hospital.

Boys who are born with hypospadias are not circumcised at birth, so their foreskin can be used for the repair during surgery.

Before surgery, your child will receive general anesthesia. This will make him sleep and unable to feel pain during surgery. Mild defects may be repaired in one procedure. Severe defects may need two or more procedures.

The surgeon will use a small piece of foreskin or tissue from another site to create a tube that increases the length of your son's urethra. Extending the length of the urethra will allow it to open at the tip of the penis.

During surgery, the surgeon may place a catheter (tube) in the urethra to make it hold its new shape. The catheter may be sewn or fastened to the head of penis to keep it in place. It will be removed 1 - 2 weeks after surgery.

Most of the stitches (sutures) used during surgery will dissolve on their own and will not have to be removed later.

.

If repair is not done, child may have

Problems controlling and directing his urine stream A curve in the penis during erection Problems with fertility

Surgery is NOT needed if the condition does not affect normal urination while standing, sexual function, or the deposit of semen.

Risks

Risks for any anesthesia are:

Allergic reactions to medicines Breathing problems

Risks for any surgery are:

Bleeding Blood clot Infection

Other risks for hypospadias:

A hole that leaks urine (fistula) Large blood clot (hematoma) Scarring or narrowing of the repaired urethra

Before the Procedure

Your child's surgeon may ask for a complete medical history and physical exam of your child before the procedure.

Always tell your child's doctor or nurse:

What drugs your child is taking Include drugs, herbs, and vitamins you bought without a prescription. Tell them about any allergies your child has to medicine, latex, tape, or skin cleaner.

Ask your child's doctor which drugs your child should still take on the day of surgery.

On the day of the surgery:

Your child will usually be asked not to drink or eat anything after midnight the night before surgery.

Give your child any drugs your doctor told you to give your child with a small sip of water.

Your child's doctor or nurse will tell you when to arrive for the surgery. The doctor will make sure your child is healthy enough for surgery. This means your son

should not have any signs of illness. If your son is ill, the surgery may be delayed.

After the Procedure

Right after surgery, your son's penis will be taped securely to his belly so that it does not move.

Often, a bulky dressing or plastic cup is placed over the penis to protect the surgical area. A urinary catheter (a tube used to drain urine from the bladder) will exit the dressing to allow urine to flow freely into the diaper.

Your child will be encouraged to drink fluids so that he will urinate. Urinating will keep pressure from building up in the urethra.

Your son may be given medicine to relieve pain.

You will probably be able to take your child home the same day as the surgery. If you live a long way from the hospital, you might want to stay in a hotel near the hospital the first night.

Outlook (Prognosis)

This surgery lasts a lifetime. Most children do well after this surgery. Your son's penis will look almost or completely normal. It will also work almost or completely normally.

If your child has a complicated hypospadias, he may need more operations to improve the penis' appearance or to repair a hole or narrowing in the urethra.

Follow-up visits with a urologist (a doctor who specializes in the treatment and surgery of the urinary system) may be needed once your son has healed from surgery. Sometimes a visit is needed when boys reach puberty.

Expectations (prognosis)

Results after surgery are typically good. In some cases, additional surgery is needed to correct fistulas or a return of the abnormal penis curve.

Complications

If hypospadias is untreated, a boy may have difficulty with toilet training and problems with sexual intercourse in adulthood. Urethral strictures and fistulas may form throughout the boy's life, requiring surgery.

EPISPADIASISINTRODUCTION

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. The extent of the defect can vary from a mild glandular defect to complete defects as are observed in bladder exstrophy, diastasis of the pubic bones, or both. Simple epispadias occurs less commonly than the more severe form associated with exstrophy of the bladder

DEFINITION

Epispadias is a rare congenital (present from birth) defect located at the opening of the urethra.

In this condition, the urethra does not develop into a full tube and the urine exits the body from an abnormal location.

INCIDENCE

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

The causes of epispadias are unknown at this time. Itmay be related to improper development of the pubic bone.

In boys with epispadias, the urethra generally opens on the top or side of the penis rather than the tip. However, it is possible for the urethra to be open along the entire length of the penis.

In girls, the opening is usually between the clitoris and the labia, but may be in the belly area.

Epispadias can be associated with bladder exstrophy, an uncommon birth defect in which the bladder is inside out, and sticks through the abdominal wall. However, epispadias can also occur with other defects.

Epispadias occurs in 1 in 117,000 newborn boys and 1 in 484,000 newborn girls. The condition is usually diagnosed at birth or shortly thereafter.

Pathophysiology

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.

Also reported are frequent ascending infections to the prostate or bladder and kidneys and psychological problems related to the deformity. If epispadias is distal to the bladder neck, urinary continence may not be present.

Symptoms

In males:

Abnormal opening from the joint between the pubic bones to the area above the tip of the penis

Backward flow of urine into the kidney (reflux nephropathy)

Short, widened penis with an abnormal curvature

Urinary tract infections

Widened pubic bone

In females:

Abnormal clitoris and labia

Abnormal opening where the from the bladder neck to the area above the normal urethral

opening

Backward flow of urine into the kidney (reflux nephropathy)

Widened pubic bone

Urinary incontinence

Urinary tract infections

DIAGNOSIS

Blood test to check electrolyte levels

Intravenous pyelogram (IVP), a special x-ray of the kidneys, bladder, and ureters

MRI and CT scans, depending on the condition

Pelvic x-ray

Ultrasound of the urogenital system

TREATMENT

Surgical repair of epispadias is recommended in patients with more than a mild case. Leakage of urine (incontinence) is not uncommon and may require a second operation.

PROGNOSIS

Surgery generally leads to the ability to control the flow of urine and a good cosmetic outcome.

POSSIBLE COMPLICATIONS

Persistent urinary incontinence can occur in some persons with this condition even after several operations.

Upper urinary tract (ureter and kidney) damage and infertility may occur.

NURSING MANAGEMENT

NURSING DIAGNOSES

PRE-OPERATIVE:-1.Impaired Urinary Elimination related to anatomical defectGoal: Facilitating Urine Output Preoperatively

Nursing Interventions

Protect the bladder area from trauma and infection.o Keep the infant in an Isolette to avoid irritation from clothing and blankets.

Position on back or side.o Humidity to exposed bladder using hood: may cover area with wet gauze, when

out of Isolette, for feeding.o Change bed linen frequently to maintain skin integrity.

Observe the infant closely for signs of infection. Involve other members of the health care team for parental support because of the

psychosocial implications of a child who has special needs. Assist the parents in dealing with their emotional reactions regarding the child's defect. Prepare child and parents for the proposed surgery

2.Knowledge deficit of the parents regarding surgical procedure secondary to the congenital anomaly.

Goal: improve the level of knowledge

Ascertain parents’ understanding of the procedure. Determine cultural/religious influences.

Review information about the advantages and disadvantages of surgery Discuss anticipated infant behaviors following procedure.

Note any special requests made by parents. Provide information about the healing process and proper care (e.g., cleaning, diapering,

positioning, use of petroleum gauze dressing or bacterial ointment

Discuss potential complications, e.g., hemorrhage, infection, or other signs warranting notification of healthcare provider.

o .

3..Risk for Injury related to complications of surgery

Goal :Providing Postoperative Care to Prevent ComplicationsNursing interventions:

Provide care for the ureteral and urethral catheters. Observe and record the amount of urinary drainage, catheter positions, and bladder spasms.

Care for the child, who is placed in a body cast for several weeks (see page 1059) or in a traction system (see page 1061).

Provide care and instruction for an ileal conduit as necessary (see page 766). Observe for complications.

o Urinary or incisional infectionso Fistulae in the suprapubic or penile incisions

Recommend long-term support for children and families to help them deal with such fears as appearance of genitalia, potential inability to reproduce, rejection by peers, and sexual function. Ongoing discussion groups for parents and children may be helpful.

Teach the parents how to care for the child at home and make appropriate referrals.

. POST-OPERATIVELY

1.Acute pain related to the surgical procedure as evidenced by the pain score reading of five.

Goal:Improve comfort

Provide pacifier (dipped in sugar, if desired), stroke lightly, and talk gently to infant during procedure. Observe infant response.

Remove infant from restraints immediately following procedure.

Calm infant by holding, cuddling, dressing, and talking to him.Encourage parents to feed and cuddle infant.

Position infant on side or back, not on abdomen. .

Administer analgesics as per order

2.Impaired tissue integrity related to the incision secondary to the surgical management of the congenital anomaly

Goal:Maintain skin integrity

Assess the condition of the skin.

Check for any leaking , redness or rashes

Provide a dry dressing near the site

Always keep the wound dry and clean.

Check in between for any leakage of the catheter.

3.Imbalanced nutrition less than the body requirement related to the surgical procedure and theNPO status.Goal; maintain the nutritional status

Identify children at risk for malnutrition

Determine ability to swallow, taste; presence of mechanical barriers;

Determine child’s current nutritional status using age-appropriate measurements, including weight and body build desires/influences on dietary choices.

Determine whether infant is breastfed or formula-fed and typical pattern of feedings during a 24-hr period. Note type and amounts of solid foods an infant/young toddler eats.

Auscultate bowel sounds. Note characteristics of stool (color, amount, frequency, and so on).

Emphasize importance of well-balanced, nutritious intake. Provide information regarding individual nutritional needs and ways to meet these needs within financial constraints. Avoid arguing over food intake. Provide food without comment

4.. Anxiety of the parents regarding the surgical out come secondary to the congenital malformation.

Goal:Relieve anxiety

Nursing Interventions:

Assess the level of anxiety of the parents

Assess the awareness of the parents regarding the disease condition ,surgical management amd outcome of the surgery.

Allow the parents to express their concerns and worries.

Provide need based information and psychological support to the child.