There are more than a dozen different reproductive disorders that include external genitalia, internal organs, and hormone levels in both male and females. Some are identified in utero while others may not present until puberty. Most reproductive disorders are congenital and are caused by abnormalities in fetal development. While many do not require intervention but may cause sterility or the inability to reproduce, there are some that require surgical intervention at, or shortly after birth.
Follow along today as we discuss some of the neonatal reproductive disorders that require surgical intervention.
Bladder exstrophy, also referred to as ectopia vesicae, is the congenital disorder where the bladder is located outside the abdominal wall. This occurs in one out of 50,000 live births. Sometimes this disorder can be identified in a prenatal ultrasound, but in the majority of pregnancies, it is not identified until the baby is born. Most of the time, this abnormality is easily identified and obvious if the bladder is completely on the outside of the abdomen. Sometimes, if the bladder is under the skin, but through the abdominal wall, it may be less obvious, but easily detectable on assessment. In males, it may result in a small penis and displaced scrotum, and commonly females will have divergent labia and a displaced vaginal orifice. Surgical intervention is required to correct the disorder. Depending on the severity of the condition and what structures are involved, the treatment may require several surgeries over a few years as structures develop. Initially, the goal will be to make sure that the organs are placed back inside the body and the abdominal wall is closed. Your pediatric surgical team will discuss options with you based on your baby’s conditions. For more information about bladder exstrophy and treatment options, read more here.
Cloacal anomaly, deformity, or malformation are terms used for conditions that affect the exit of tracts in the female. There are several different variations that involve the merging of the anus, vagina, and the urethra. In some cases, these anomalies can be identified on prenatal ultrasound and others may not be obvious until a newborn assessment is completed. Cloacal deformities vary depending on which tracts are involved and where these tracts merge. Generally, these abnormalities do not affect the internal reproductive organs including the uterus or ovaries. In conditions where urine and bowel can freely exit the body, surgical intervention can be delayed, however, in conditions where either cannot exit the body, it is considered an emergent surgical situation. Surgical treatment options include reconstruction of all of the organs and creating the appropriate openings. Your neonatal surgical team can begin planning corrective surgery as soon as the condition is identified. For more information about these anomalies and treatment options, read here.
Hypospadias and Epispadias
Hypospadias and epispadias are birth defects in males where the urethra does not exit out of the head of the penis, but rather on the inferior surface (hypospadias) or the upper aspect of the penis (epispadias). These defects occur in one out of 300 and one out of 117,000 live births, respectively. The severity of the condition will depend on where they urethra exits and whether or not it creates a tunneling effect. This condition can happen in girls in rare cases (one in 484,000) but may not be noticed until potty training results in continual urinary leaking. Surgery is not an emergency as long as the urethra is patent (open and allowing the urine to exit the body). Surgical treatments are aimed at making the genitals look and work as “normal” as possible. Your pediatric surgeon will discuss the options with you to create a plan. For more information about these conditions, read here.
At Alaska Pediatric Surgery, our pediatric surgical team is skilled at conducting neonatal and pediatric surgeries that help children live normal lives. Whether your baby has an abnormality that is discovered while you are still pregnant, at birth, or later in life, contact us so we can begin planning as soon as possible. We will work with you and the rest of the pediatric team to create a treatment plan with your baby’s quality of life in mind. Schedule your consult in Anchorage, Fairbanks, or Wasilla today!