What is Hypospadias Urogenital Reconstruction?
Hypospadias is one of the most frequently seen issues in newborn boys, relating to their urinary and genital organs. It’s only second to undescended testis in terms of common birth defects in males. Hypospadias is defined by a few key characteristics: the opening of the urethra (where urine comes out) is on the underside of the penis rather than at the tip; there’s an unusual downward bend of the penis, known as “chordee”; and the foreskin is distributed unevenly, with a “hood” on the top and not enough on the bottom.
This condition can lead to difficulties in urination, and in grown men, it might result in sexual difficulties and infertility. It can also cause affected individuals to have a negative perception of their body image. However, early detection and timely surgery have significantly enhanced both the physical function and appearance in recent times.
What Causes Hypospadias Urogenital Reconstruction?
Hypospadias, a condition where the urethra’s opening isn’t at the tip of the penis, can have many causes.
One potential cause could be genetic and family-related factors. This is especially true for hypospadias that affects the front or middle of the penis. About 7% of individuals with hypospadias have close family members, such as a father or a brother, who also have the condition. The condition can be passed down from both the mother’s and the father’s side of the family. However, only in about 30% of cases is a clear genetic cause found. Several genetic disorders can be associated with hypospadias, the most common of which are WAGR syndrome, Denys-Drash syndrome, and Smith-Lemli-Opitz syndrome. Boys conceived through a fertility procedure called intracytoplasmic sperm injection (ICSI), which is used for severe sperm defects, may have a higher risk of developing hypospadias.
Another possible cause is hormonal factors. Hypospadias can sometimes come with conditions like undescended testicles and a small penis. Some researchers believe that an imbalance of male and female hormones may play a significant role in the abnormal development of the urethra and foreskin. Evidence has suggested that abnormal exposure to male hormones during fetal development can result in a reduced anogenital distance (the distance between the anus and the genitals), which is found in boys with hypospadias. Conditions affecting sexual development are more common in infants with hypospadias affecting the back of the penis. A condition called testicular dysgenesis syndrome (TDS), which results from abnormal development of the testes and may lead to hypospadias, undescended testicles, male infertility, and testicular cancer.
Environmental factors could also contribute to hypospadias. Some studies in animals have identified certain environmental factors that disrupt endocrine function and cause hypospadias. One recent study in humans also suggested that mothers’ exposure to certain air pollutants that disrupt hormones could contribute to hypospadias.
Lastly, some mother-related factors could contribute to severe hypospadias. These include high blood pressure in the mother, low levels of amniotic fluid, preterm delivery, and having identical twins. Some researchers propose that inadequate exposure of the fetal testes to a hormone called human chorionic gonadotropin (hCG) could lead to hypospadias. However, there still isn’t enough evidence to confirm this cause-effect relationship.
Risk Factors and Frequency for Hypospadias Urogenital Reconstruction
It’s challenging to determine the exact number of hypospadias cases at birth due to certain limitations in research methods. In the United States, hypospadias is reported to occur in about 1 in every 200 to 300 male births. However, in Europe, the rate is lower, with around 18.6 cases per 10,000 live births. Even though some research suggests an increase in hypospadias cases, other studies don’t support this claim.
Signs and Symptoms of Hypospadias Urogenital Reconstruction
Hypospadias is a condition in male infants where the urinary opening or “meatus” is placed at the wrong location. This can make it difficult for the child to urinate normally and sometimes can cause the urine to spray, wetting their clothes. There might be issues urinating while standing up. Sometimes, this condition comes with other issues, like cryptorchidism (undescended testicles) and bifid scrotum (a divided scrotum), which can make the genitals appear ambiguous. In adults, this condition can make the penis curve more than usual, especially during an erection. This could lead to problems with sexual intercourse and even infertility.
Hypospadias can be classified into three main types. These depend on where exactly the urethral meatus is located:
- Anterior hypospadias: The opening is found under the surface of the head of the penis, at the edge of the head, or at the junction of the head and the shaft. This is the most common type.
- Middle hypospadias: The opening is located on the shaft of the penis; either at the distal (near the head), midshaft, or proximal (near the base).
- Posterior hypospadias: The opening is located at the intersection of the penis and the scrotum, on the scrotum, or at the perineum (the area between the anus and scrotum).
There are two additional subtypes of the condition, namely, “megameatus with an intact prepuce” characterized by an atypically wide urethral opening and a normal foreskin, and “hypospadias sine hypospadias” characterized by a specific type of penis curvature and an unusual foreskin but a normally positioned urethral meatus. A precise classification of the condition would only be possible during surgery by considering factors such as the size of the penis and glans (head of the penis), the degree of penis curvature, and any other associated scrotal anomalies.
Testing for Hypospadias Urogenital Reconstruction
If a person has posterior hypospadias, ambiguous genitalia, or undescended testicles, known as cryptorchidism, an endocrine evaluation becomes essential. The endocrine system involves hormones, and this evaluation examines how these hormones may affect these conditions.
Your doctor may recommend specific tests, such as a karyotype test and a 17-hydroxy-progesterone test. A karyotype test looks at the number and structure of a person’s chromosomes, to check for any abnormalities. The 17-hydroxy-progesterone test, on the other hand, assesses the levels of a particular hormone in the blood that can influence sexual development.
Also, an ultrasound of the genital and urinary organs could be considered if one has posterior and more complex types of hypospadias. Hypospadias is a birth defect where the opening of the urethra is not located at the tip of the penis. The ultrasound can help the doctor see if the urinary system is working properly.
Up to 50% of perineal hypospadias cases (where the opening is near the anus) might have Mullerian remnants. These are leftover bits of female reproductive structures that can turn into a cyst or widened utriculus and could block the urinary system. A utricular cyst refers to a fluid-filled sac within the cavity of the prostate gland, and a utriculus is a small cavity in the prostate.
In addition to ultrasound, the doctor may also perform an endoscopic examination of the urethra, which involves inserting a small tube with a light and camera to see inside. This examination can help detect any potential abnormalities not visible in the ultrasound.
Treatment Options for Hypospadias Urogenital Reconstruction
Surgery for hypospadias, which is a condition where the opening of the urethra is on the underside of the penis instead of at the tip, aims to make the genitalia look more typical, prevent urine from spraying during urination, allow the individual to urinate standing up, and avoid sexual difficulties. It also facilitates the delivery of semen into the vagina during sexual intercourse. However, if the condition is mild, such as in isolated glandular hypospadias which affects just the head of the penis, it often does not require surgical correction.
Usually, hypospadias is surgically corrected early in life, typically between 6 to 18 months of age depending on how severe the condition is. Some surgeons believe that having a micropenis, which is a penis that is smaller than the standard size, could make the surgery more challenging, but there is not enough scientific evidence to support the use of testosterone supplements before surgery to enhance penile growth. Early surgical intervention also helps to alleviate psychological burdens, as children become aware of their genitalia around the age of 18 months. Moreover, surgeries performed later in life tend to have more complications.
The surgical repair of hypospadias involves several steps and typically requires high magnification equipment, fine surgical instruments, and adequate control of bleeding. The procedure usually begins with penile degloving, which involves removing the skin of the penis to assess the degree of bending (chordee). Next, orthoplasty is performed to correct the curvature and achieve a straight penis while maintaining penile length and erection. After these preliminary steps, the urethra is reconstructed— a process known as urethroplasty— in various ways as determined by the surgeon’s expertise and familiarity with the different techniques. Finally, the glans penis (the head of the penis) is reconstructed, the urinary tube is brought out through the glans, and the skin is closed to mimic the appearance of a circumcised penis.
Some methods of surgical repair for hypospadias may vary based on the location of the opening of the urethra. For the distal meatus, which means the opening is closer to the tip of the penis, Meatal Advancement and Glanuloplasty Incorporated (MAGPI), a type of outpatient surgery, is highly recommended. In addition to being generally successful, this procedure carries a low complication rate and provides satisfactory aesthetic results. Patients with certain conditions like thin or stiff skin around the meatus, or a meatus that is located excessively back from the tip or is too wide, are not suitable for the MAGPI procedure.
For non-coronal distal hypospadias, where the opening is located somewhere along the shaft of the penis, the Mathieu technique or tubularized incised plate urethroplasty (TIP) may be used. However, careful patient selection is needed to achieve optimal results.
Lastly, for persistent bending of the penis (chordee) after the skin has been removed (degloving), methods to correct the curvature are implemented in surgery. For curvatures less than 30 degrees, dorsal plication (folding or pleating the tissue on the upper surface of the penis) only might be used. For children with more severe curvatures exceeding 30 degrees, ventral corporotomy (cutting into the corpora cavernosa, the erectile tissue of the penis) is typically necessary.
What else can Hypospadias Urogenital Reconstruction be?
When dealing with a patient with hypospadias, a major first step is a thorough physical examination as well as checking over all the body’s systems. This can help rule out any possible related medical conditions. Hypospadias might be caused by a variety of different things, and the doctor has to consider all of them.
One possibility is an enzyme defect, specifically in dihydrotestosterone (DHT). DHT is involved in the development of male characteristics, so if there is a problem there, it might lead to different levels of masculinity in the patient.
The creation of DHT is closely linked to the functioning of a gene called SRD5A2. However, if there are mutations in this gene, it can lead to hypospadias and can also make the person appear more feminine. To tell this condition apart from other disorders of sexual differentiation, doctors should check for significant changes in masculinity and no evidence of breast development during puberty.
What to expect with Hypospadias Urogenital Reconstruction
Generally, the outlook is positive for both mild and proximal cases of hypospadias, especially if the condition is treated early on. Hypospadias is a condition where the opening of the urethra is on the underside of the penis, rather than at the tip.
Men who have undergone surgery to repair hypospadias often experience lower urinary tract symptoms. These symptoms can include trouble with urine flow, hesitancy in starting to urinate, and other similar issues. This is particularly true for those with a specific type of hypospadias known as posterior hypospadias.
Common complications from hypospadias surgery can include meatal stenosis (a narrowing of the urethra opening), fistula (an abnormal connection between organs), and urethral stenosis (a narrowing of the urethra). Because of these potential complications, it is important for patients to continue regular check-ups until their reproductive system is fully mature.
After surgery, about 70% of patients feel satisfied with the way their genitals look. However, those with more severe types of hypospadias, such as posterior and complex hypospadias, may not be as happy with the way their genitals look after surgery. Some men may worry about being judged by others because of the changed appearance of their genitals.
Despite this, 80% of patients report being satisfied with their sexual function after the surgery.
Possible Complications When Diagnosed with Hypospadias Urogenital Reconstruction
Early complications after surgery are usually due to technical issues during operation. These can be prevented through careful planning and gentle handling of tissues during surgery. These complications can include inflammation, formation of blood clots, wound separation, death of tissue flaps used in reconstruction, generation of an abnormal connection between the urethra and skin, twisting of the penis, and accidental removal of a tube placed in the ureter.
- Inflammation
- Formation of blood clots
- Wound separation
- Death of tissue flaps used in reconstruction
- Generation of an abnormal connection between the urethra and skin
- Twisting of the penis
- Accidental removal of a tube placed in the ureter
Controlling bleeding is essential to mitigate blood clot formation. Also, treating the tissues properly can prevent inflammation after surgery. A specific type of dressing can also limit inflammation.
To prevent the death of tissue flaps and wound separation, it’s important to mobilize the flap properly and ensure a stress-free repair. The creation of an abnormal connection between the urethra and skin is a common complication after reconstructive surgery. The reasons for the formation of this connection may include not handling the tissues properly, performing distal construction, and infection. Being aware of all these factors can help to prevent the formation of this connection.
Accidental removal of a tube placed in the ureter can be prevented through proper anchoring. Late complications can include the narrowing of the urethral opening, formation of abnormal connections, the development of bulges in the urethra, formation of strictures, persistent penile curvature, and torsion. Ignoring the early complications can lead to these later complications. Children who have wound infection, wound separation, blood clot formation etc., are at higher risk for developing further complications later. Often these complications require additional surgery for correction.
Preventing Hypospadias Urogenital Reconstruction
Hypospadias is a birth defect that occurs when the opening of the urethra is located on the underside of the penis. This condition is one of the more common birth abnormalities in children. There are several ways doctors can fix this through surgery. When this defect is fixed early, children have the best chance of a good outcome, both in terms of how they look and how well the penis functions.
However, it’s worth noting that about a quarter of children who have surgery for hypospadias will need a second operation. Out of these children, less than half will require this second surgery within a year. So, it’s really important for parents and patients to stay in touch with experienced children’s surgeons. These doctors will not just perform the surgery but also keep track of the child’s recovery to make sure everything is progressing well over the long term.
