care of children with Epispadias,hypospadias,ectopia vescica

HYPOSPADIAS

HYPOSPADIAS

• The term hypospadias refers to a urethral opening that is on the ventral surface of the penile shaft, proximal to the end of the glans. The meatus may be located anywhere along the shaft of the penis, from the glans to the scrotum, or even in the perineum.

• Some boys with hypospadias, particularly those with proximal hypospadias, have chordee, in which there is ventral penile curvature during erection.

Incidence

• Hypospadias occurs in up to 4 in 1,000 newborn boys.

• The reason why the penis does not develop properly is still not clear.

• The development of the penis whilst the baby is growing in the womb is partly dependent on the male sex hormones such as testosterone.

• The effects of testosterone on the growing penis may be blocked in some way.

• Although it is not a genetic condition, hypospadias can run in some families

Causes

• present at birth (congenital). The exact reason this defect occurs is unknown.

• A defect in the androgen stimulation of the developing penis

• the deficient androgen production by the testes and placenta.

Risk factors

• Age and weight: Mothers who were age 35 years or older and who were considered obese had a higher risk of having a baby with hypospadias.

• Fertility treatments: Women who used assisted reproductive technology to help with pregnancy had a higher risk of having a baby with hypospadias.

• Certain hormones: Women who took certain hormones just before or during pregnancy were shown to have a higher risk of having baby with hypospadias.

• Exposure to smoking and chemicals: There is some speculation about an association between a mother's exposure to pesticides and hypospadias.

• Family history: This condition is more common in infants with a family history of hypospadias.

Classification

by Duckett in 1996.

He divided them into anterior (50%), middle (30%) and posterior (20%) hypospadias.

– The anterior form: glandular, coronal and distal penile.

– The middle form: "midshaft” and proximal penile.

– The posterior form: penoscrotal, scrotal and perineal

Glanular hypospadias : the urethra is within the head of his penis (glans).

Coronal: The opening of the urethra is just below the head of his penis.

–Distal penile: the opening of the urethra is in the distal portion of the shaft of the penis

• Midshaft: The opening of the urethra is located along the shaft of the penis.

• Proximal penile: the opening of the urethra is in the proximal portion of the shaft of the penis

• Penoscrotal: The opening of the urethra is located where the penis and scrotum meet.

• Scrotal hypospadias: hypospadias with the urethral opening on the scrotal surface.

• perineal hypospadias: the urethral meatus opens in the perineum near the anus; the scrotum is usually cleft.

Clinical manifestations

• Mild hypospadias usually does not cause symptoms, especially in newborns and young children.

• This condition may cause a downward curve of the penis during an erection.

• Erections are common in infant boys.

Other symptoms include:• Abnormal spraying of urine• Having to sit down to urinate• Foreskin that makes the penis looks like it has a

"hood"• The farther the opening is from the tip of the

glans, the more likely curvature in the penis (chordee) is present.

Diagnostic measures

• prenatal ultrasound

• Physical exam of a newborn: upon examination, the foreskin is usually incomplete and the misplaced urethral opening is located.

• Excretory urogram: This test uses X-rays to provide pictures of the urinary tract, and other congenital anomalies

Treatment

• Medical management

• Minor cases of hypospadias, in which the meatus is located up toward the tip of the glans, may not require surgical repair and may simply be managed with observation.

Adjuvant hormonal therapy

• Presurgical treatment with testosterone injections or creams, as well as HCG injections, to promote penile growth, and for the improvement in chordee with lessening in the severity of the hypospadias.

Surgical management

• Management begins in the newborn period.

• Circumcision should be avoided, because the foreskin often is used in the repair.

• The ideal age for repair in a healthy infant is 6–12 months

– There is no greater risk of general anesthesia at this age compared to 2–3 yr

– Penile growth over the next several years is slow

– The child does not remember the surgical procedure

– Postoperative analgesic needs are less than in older children

DIFFERENT SURGERIES

• Glandular hypospadias requires a glandular meatotomy

• Coronal hypospadias requires a meatal advancement and glanduloplasty (MAGPI operation)

• Proximal hypospadias without a chordee can be treated by a skin flap advancement

• If chordee present it should be excised and an island flap urethroplasty performed

EPISPADIASIS

• Epispadias is a congenital malformation in which the opening of the urethra is on the dorsum of the penis.

• 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.

Incidence

• Epispadias occurs in 1 in 117,000 newborn boys and 1 in 484,000 newborn girls.

Causes

• Unknown• Related to improper development of the pubic

bone• Failures of abdominal and pelvic fusion in the

first months of embryogenesis• Epispadias can be associated with

bladder exstrophy, an uncommon birth defect in which the bladder is inside out, and sticks through the abdominal wall

• Also occur with other defects

Classification

Classification of epispadias is based on the location of the meatus the penis. It can be positioned:

• On the glans (glanular)

• Along the shaft of the penis (penile)

• Near the pubic bone (penopubic).

• The position of the meatus is important because it predicts the degree to which the bladder can store urine (continence). The closer the meatus is to the base of the penis, the more likely the bladder will not hold urine

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 infection

Diagnostic measures

• Prenatal diagnosis - rare

• 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

Surgical technique in males:

• The modified Cantwell technique

It involves partial disassembly of the penis and placement of the urethra in a more normal position.

Treatment

The primary goals of treatment of epispadias are to:

• maximize penile length and function by correcting dorsal bend and chordee; and

• create functionality and cosmetically acceptable external genitalia with as few surgical procedures as possible.

• If the bladder and bladder neck are also involved, surgical treatment is required to establish urinary continence and preserve fertility.

• The second technique is the Mitchell technique.»It involves complete disassembly of

the penis into its three separate components.

»Following disassembly, the three components are reassembled such that the urethra is in the most functional and normal position and dorsal chordee is corrected.

Surgical technique in females

• The urethra and vagina may be short and near the front of the body and the clitoris is in two parts.

• If diagnosed at birth, the two parts of the clitoris can be brought together and the urethra can be placed into the normal position.

• If repaired early enough, lack of urinary control (incontinence) may not be a problem.

• If the diagnosis is missed or if early repair is not performed, then incontinence can be surgically corrected at the time of diagnosis.

• If the vaginal opening is narrow in older girls or younger women, reconstruction can be performed after puberty.

UNDESCENDED TESTIS

Definition

• Undescended testis is the failure of one or both testes to reach the normal position in the scrotal sac through the inguinal canal.

Incidence

• Cryptorchidism is the most common genital abnormality in boys, affecting approximately 30% of baby boys born prematurely and about 4% born at term.

• Around 1 in 20 male babies born at term also has cryptorchidism. Many of these will become descended in time. However, for around 1 in 70 cases, the testis remains undescended after the child is 1 year old.

Etiological factors

• The exact cause of an undescended testicle isn't known.

• A combination of genetics, maternal health and other environmental factors might disrupt the hormones, physical changes and nerve activity that influence the development of the testicles.

contd..

• Impairment of the hypothalamic pituitary gonadal axis: block in the hormonal axis to stimulate the testes to descend or the testes may fail to respond o the stimulus due to some inherent defects

• Anatomical obstruction: there may be an obstruction in the pathway of descend or failure of intra abdominal pressure to rise

• Heredity or chromosomal anomalies: absence of one or both testes

• Short spermatic cord and artery mechanically prevent the descend

• Ectopic attachment of the testes

Risk Factors

• Factors that might increase the risk of undescended testicle in a newborn include:

• Low birth weight• Premature birth• Family history of undescended testicle or other

problems of genital development• Conditions of the fetus that can restrict growth,

such as Down syndrome or an abdominal wall defect

• Alcohol use by the mother during pregnancy

Contd….

• Cigarette smoking by the mother or exposure to secondhand smoke

• Obesity in the mother• Diabetes in the mother — type 1 diabetes, type

2 diabetes or gestational diabetes• Parents' exposure to some pesticides

Pathogenesis.

• The process of testicular descent is regulated by an interaction between hormonal and mechanical factors.

• The testis develops at 7–8 wk of gestation. At 10–11 wk, the Leydig cells produce testosterone, which stimulates differentiation of the wolffian (mesonephric) duct into the epididymis, vas deferens, seminal vesicle, and ejaculatory duct.

• At 32–36 wk, the testis, which is anchored at the internal inguinal ring by the gubernaculum, begins its process of descent. The gubernaculum distends the inguinal canal and guides the testis into the scrotum. Following testicular descent, the patent processus vaginalis (hernia sac) normally involutes.

Types

• Retractile or pseudo cryptorchidism: This is NOT an undescended testicle but is often mistaken for one. It is caused byoveractive muscles that pull the testicle(s) out of the scrotum. In this type the testicles can be placed in the scrotum manually and stay there for a short period of time. This is a type of normal and does not need treatment.

• Palpable (80%): In this type, (also called prepubic or inguinal) the testicle is located anywhere from just above the scrotum to high in the groin.

Contd….

• Nonpalpable (15%): This means the testicle is in the boy’s abdomen or is absent and not felt in the scrotum or inguinal canal.

• Ectopic (5%): In this case, the testicle has taken the wrong path and ended in an unusual location in the groin area.

Clinical features

• A nonpalpable testis (unable to feel on examination) is the most common symptom of cryptorchidism.

• However, each child may experience symptoms differently.

• Symptoms of cryptorchidism may resemble other conditions or medical problems.

Diagnostic measures

• Soon after the baby is born or during a routine check-up when they are six to eight weeks old.

• Physical examination• The first stage in diagnosing undescended

testicles is to carry out a physical examination to see whether the testicles can be felt near the scrotum (palpable) or if they cannot be felt at all (unpalpable).

Contd……

• Further tests• These tests may include an ultrasound scan, CT

scan, a diagnostic laparoscopy, hormonal assay and urine and blood tests.

• Open surgery. • Direct exploration of the abdomen or groin

through a larger incision may be necessary in some cases.

Treatment

• In most cases, the testicle will descend without treatment during the child’s first year. If this does not occur, treatment may include:

• Hormone injections (B-HCG or testosterone) to try to bring the testicle into the scrotum. It also helps in the enlargement of the testis.

• Surgery (orchiopexy) to bring the testicle into the scrotum. This is the main treatment. If there is an associated hernia, the herniotomy along with orchidoplexy is indicated.

• Having surgery early may prevent damage to the testicles that can cause infertility. An undescended testicle that is found later in life may need to be removed. This is because the testicle is not likely to function well and could pose a risk for cancer.

Complications

• In order for testicles to develop and function normally, they need to be slightly cooler than normal body temperature. The scrotum provides this cooler environment. Until a boy is 3 or 4 years old, the testicles continue to undergo changes that affect how well they function later.

• Complications of a testicle not being located where it is supposed to be include:

• Testicular cancer• Fertility problems• Other complications related to the abnormal

location of the undescended testicle include:• Testicular torsion.• Trauma • Inguinal hernia.

BLADDER EXSTROPHY/ ECTOPIA VESCICA

• Exstrophy of the bladder or ectopia vesicae is a congenital malformation of the urinary bladder in which a part of the wall of the bladder in front is absent and the inner part of the bladder is exposed.

Incidence

• It is a rare occurrence. • The incidence of bladder exstrophy is

approximately 2.07 per 100,000 live births and is slightly more common in males than females.

• The risk of a family having more than one child with this condition is approximately one in 100,

• And children born to a parent with exstrophy have a risk of approximately one in 70 of having the condition

Etiology

• There is no known cause for this condition but there are many theories.

• Some experts believe during the 11 th week of pregnancy the embryo undergoes structural changes including ingrowths of tissue in the lower abdominal wall, which stimulates development of muscles and pelvic bones.

• Up to this point the primitive bladder and rectum are contained within tissue called the cloacal membrane.

• The rectum then separates from the bladder, and if migration of tissue towards the midline over the primitive bladder fails the cloacal membrane may rupture, creating an exstrophied bladder.

• The exact timing of premature rupture of the membrane determines whether the child is born with isolated epispadias, classic bladder exstrophy or cloacal exstrophy.

Risk factors

• The disorder has no known risk factors, although it is more common in boys and in infants of mothers who had tobacco exposure or were young when giving birth. Children whose parents were also born with bladder exstrophy have a 1 in 70 chance of having it themselves.

Associated problems

• Bladder: The bladder is turned inside out and exposed on the abdominal wall. The bladder neck has not developed properly and the bladder itself is usually small.

Contd…

• Epispadias: The urethral opening, which is the hollow tube that drains urine from the bladder to the outside of the body, is in an abnormal location. In males, the urethral opening is usually on the topside of the penis and not the tip. In girls, the urethral opening may be positioned further up between the divided clitoris and labia minora.

Contd…

• Widening of the pubic bones: The pubic bones usually join to protect and support the bladder, urethra and abdominal muscles. In children with exstrophy, the pubic bones do not join, leaving a wide opening. This causes the hips to be outwardly rotated.

Contd…

• Vesicoureteral reflux (VUR): Normally the kidneys make urine and drain down the ureters (drainage tubes) into the bladder. VUR is a condition where urine travels back up into the kidneys. This may develop after the bladder is reconstructed.

• Contd……

Abnormal Development of Genitalia:

– Boys: The penis may appear shorter and curved in an upward direction. The testicles may not be in a normal position in the scrotum and a hernia may be seen.

– Girls: The clitoris and labia minora are separated and spread apart; the vagina and urethra are shorter. The uterus, fallopian tubes and ovaries are generally normal.

– Contd…..

• Displacement of the umbilicus and/or an umbilical hernia.

Clinical features

• Constant urinary dribbling through the defect• Skin excoriation• Infection • Ulceration of bladder mucosa• Ambiguous genitalia• Waddling unsteady gait• Urinary tract infections • Growth failure

Diagnostic measures

• fetal ultrasound

• cystoscopic examination• X ray• USG• IVP

• Urodynamic studies

• All these will help to determine the extent of anomaly and other associated problems

Treatment

• Management of bladder exstrophy should start at birth.

• Cover the bladder with plastic wrap to keep the bladder mucosa moist.

• Avoid application of gauze or petroleum-gauze to the bladder mucosa.

SURGICAL MANAGEMENT

Prompt closure of the exstrophic bladder

• During this procedure the abdominal wall is mobilized and the pubic rami are brought together in the midline.

• (If the bladder closure is performed during the first 48 hr of life, often there is sufficient mobility of the pubic rami to allow approximation of the pubic symphysis. If the procedure is delayed, the pelvic bones must be broken (pelvic osteotomy) to allow the pubic rami to be brought together and create a pubic symphysis to support the bladder closure.)

Early bladder closure

• It can be applied to almost all neonates with classic bladder exstrophy. Treatment should be deferred in selected situations when surgical therapy would be excessively risky or complex, such as in a premature baby or when it would have to be performed by inexperienced surgeons.

Total reconstruction

• It includes closure of the bladder, closure of the abdominal wall, and, in boys, correction of epispadias using a technique of penile disassembly

Creation of a sphincter muscle for bladder control and correction of the

vesicoureteral reflux

• The final stage of reconstruction involves creation of a sphincter muscle for bladder control and correction of the vesicoureteral reflux.

Post operative care

• Bladder capacity is monitored every 12–24 mo using cystoscopy under anesthesia.

• Monitor for the development of hydronephrosis and infection.

• Most infants with bladder exstrophy have vesicoureteral reflux and should receive antibiotic prophylaxis.

• In boys, if the epispadias is not corrected at birth, epispadias repair usually is performed between 1–2 yr of age

NURSING MANAGEMENT FOR CONGENITAL

GENITOURINARY MALFORMATIONS

Preoperative

• It is important to address parents’ concerns at the time of birth. Preoperative teaching can relieve some of their anxiety about the future appearance and functioning of the penis.

Postoperative nursing diagnoses

• Pain (acute / chronic) related to physical factors like damage to the skin / tissue (incision)

• Impaired skin integrity related to surgical trauma

• Impaired urinary elimination related to surgical diversion, tissue trauma