Epispadias: Characteristics, Causes, and How to Treat It

Epispadias is a congenital abnormality that causes urine to exit the body through an abnormal location or opening.

PossibleParentsever heard the term hypospadias. Just like hypospadias , this condition also affects the urethra and meatus or urinary opening. The meatus in hypospadias sufferers is located at the bottom of the penis (in men), while the meatus in epispadias sufferers is located at the top.

Let’s get to know more about epispadias through the following review.

Definition

Source: Freepik

Reporting fromCleveland Clinic, epispadias is a rare birth defect that affects the urethra, the ‘tube’ that carries urine out of the body. In babies with epispadias, their urethra does not develop into a ‘tube’ so urine exits the body from an abnormal location.

In most cases, this condition is associated with other congenital urological problems, including bladder exstrophy. Children with bladder exstrophy are born with their bladder outside the abdomen.

Epispadias is classified based on the location of the urinary outlet (meatus). The location of the meatus will affect how well the bladder can store urine.

In male infants, the closer the meatus is to the base of the penis, the more likely the condition is to also affect the bladder sphincter and urinary control. The bladder sphincter opens and closes to release or hold urine.

Types of epispadias in males include:

Glanular epispadias : This is the most common type. The meatus is located above the glans (head of the penis). Epispadias penis : The meatus is located along the shaft of the penis. Penopubic epispadias : The meatus is near the pubic bone. The pelvic bones are not fused, and the sphincter cannot close completely.

Epispadias is more common in males. Although less common, it can also occur in females. How does this condition affect females?

Baby girls with epispadias may have:

A urethral opening leading to the clitoris or even in the lower abdomen (belly). The pubic bones are separated so that the clitoris cannot connect in the middle. Labia (folds of skin outside the vagina) that are not formed properly. Problems with the bladder neck, resulting in problems controlling urine. Symptoms of Epispadias

Most symptoms or signs of epispadias are visible during a physical examination of a newborn. In mild cases of epispadias, the condition may go unnoticed until parents notice a tendency for urinary incontinence during toilet training (toilet training).

Symptoms of Epispadias in Men There is an abnormal opening of the joint between the pubic bone to the area above the tip of the penis. The urethra may open along the length of the penis Backflow of urine into the kidneys (reflux nephropathy) The penis is short and wide with an abnormal curvature. Urinary tract infection Pubic bone widening Symptoms of Epispadias in Women Abnormal looking clitoris and labia Abnormal opening from the neck of the bladder to the area above the normal urethral opening Backflow of urine into the kidneys (reflux nephropathy) The pubic bone widens, causing the clitoris to not connect in the middle, so that the clitoris appears to be divided into 2 parts. Urinary incontinence Urinary tract infection Reason

Source: Freepik

Researchers still do not know exactly what causes epispadias. However, some believe that epispadias occurs when a developing fetus has problems with the development of the cloacal membrane.

This membrane or membrane covers the cloaca, which normally develops into the gastrointestinal, urinary, and reproductive organs. This condition may also occur because the pubic bone is not properly developed.

The way organs form in a baby during pregnancy is very complicated. Certain steps must occur in the right order. However, if there is a defect in one organ, there will be defects in other organs.

It is rare for epispadias to occur as a stand-alone case or without other defects. Those with epispadias generally have bladder problems, and in rare cases can also have defects in the colon.

Frequency of Occurrence

According to the American Urological Association , epispadias is a fairly rare condition, affecting only 1 in 117,000 males. It is much rarer in girls, affecting only 1 in 484,000 cases.

Risk Factors

Risk factors for this birth defect are:

1. Genetics

The risk is higher in children of parents who also have epispadias, with a 1 in 70 chance of being born with this type of congenital problem.

2. Gender

Men are four times more likely to develop this condition than women.

3. Race

Caucasians who are firstborns are more likely to be born with this condition.

Diagnosis Physical examination

It is usually diagnosed shortly after birth by physical examination of the genitals that appear abnormal. This diagnosis often prompts additional testing to determine if additional areas of the urinary tract are involved or affected.

Laboratory Test

Doctors may need to check kidney and urinary tract function for babies born with epispadias with imaging studies including:

USG (USG) CT scan X-ray Other urology tests

If a colon defect is involved, a colonoscopy or other procedure to examine the spinal cord may be necessary.

In more severe cases involving the bladder or spine, diagnosis can be made during a prenatal ultrasound or before the baby is born. However, mild cases usually cannot be detected by a routine ultrasound.

Handling

Source: Freepik

Surgical repair of epispadias is recommended in patients with moderate cases. Surgery generally leads to better control of urinary flow and better cosmetic results.

When surgeons perform surgery at birth or early in life, children with epispadias can have a better quality of life. According to research, 1 in 3 of them will have good urinary control without the need for further surgery.

A child born with another condition such as bladder exstrophy may require additional procedures to repair his bladder.

The risks of all epispadias surgeries include the general risks of surgery and the risks associated with general anesthesia. The risk of urinary incontinence is high because many people already have this condition before surgery, and the hope is that surgery will correct the incontinence.

Recovery time depends on the complexity of the repair required. Children who have minor surgery will recover quickly. For children with bladder exstrophy, more than one surgery may be needed. Recovery will take longer.

Male Epispadias Surgery

There are several surgical options for epispadias. Your doctor’s recommendation will depend on the type of epispadias you have. A child with complex exstrophy-epispadias may require more complex surgery.

Types of surgery for men include:

Modified Cantwell-Ransley Technique
This technique is done to reconstruct part of the penis. The surgeon can reconstruct part of the penis and move the urethra to a better location. Mitchell Technique
This technique is performed by completely reconstructing the penis, to ensure the urethra is in the best position. Female Epispadias Surgery

Girls usually require less complicated epispadias surgery than boys. The type of surgery may depend on how early the doctor diagnoses epispadias.

If the condition is diagnosed at birth, urine control is unlikely to be a problem. The surgeon may:

Connecting the two parts of the clitoris. Placing the urethra in the correct location.

If the problem is not diagnosed or treated early, later in life girls may need surgery to:

Incontinence or lack of urinary control. Reconstruction of a vaginal opening that has grown too narrow. Possible Complications of Epispadias Complications of Epispadias Surgery

Sometimes after major surgery, a hole called a fistula can open from the urethra to the skin. However, fistulas as a complication are very rare because the surgical techniques used today are generally sophisticated.

If a fistula occurs, it may close on its own or the child may require another procedure to close the fistula.

Long Term Risk

Long-term usually occurs in adulthood. Patients will be at higher risk of developing the following conditions:

Epididymitis Vaginal prolapse Rectal prolapse Bladder cancer Renal cell carcinoma Sexual Function

Sexual function usually remains normal after surgery, even when additional surgery is required. However, men may experience a decreased sperm count or low ejaculate volume, resulting in decreased fertility, but not infertility.

Women with previous epispadias are at higher risk for postpartum hemorrhage. There is also a small risk of ureteral damage and fistulas that can occur after delivery.

Prevention

Unfortunately, because the exact cause of this condition is still unknown, there is no known effective way to prevent it.

IfParentsIf you have a family history of epispadias, it is a good idea to consult a doctor when planning a pregnancy.

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Epispadias is usually diagnosed in infancy and can be corrected by surgery. Hope this information is helpful.

Epispadias
https://www.hopkinsmedicine.org/health/conditions-and-diseases/epispadias#

Epispadias: Symptoms, Diagnosis, & Treatment
https://www.urologyhealth.org/urology-a-z/e/epispadias

Epispadias: What is it, Causes, Diagnosis & Treatment
https://my.clevelandclinic.org/health/diseases/21551-epispadias

Epispadias
https://medlineplus.gov/ency/article/001285.htm

Epispadias: Symptoms, Diagnosis, Treatment, and Outlook
https://www.verywellhealth.com/epispadias-overview-4177870#

Republished with permission from theAsianParent Indonesia