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Infants with bladder exstrophy have defects involving the bladder, abdominal wall, perineum (the area between the anus and the scrotum or vulva), genitals and pelvis. With this condition, the bladder protrudes from the body, which can affect muscular, neurological and urinary function if not treated.
There is good news for parents who find out their baby has bladder exstrophy, though. The expert surgeons at the Colorado Fetal Care Center can use advanced surgical techniques to close the abdominal wall, fix the bladder and rebuild the urethra and genitals. Our fetal care specialists aim to provide the best outcomes and, in most cases, babies treated for bladder exstrophy are able to achieve continence and normal genital function.
The Colorado Fetal Care Center is a national leader in diagnosing and treating bladder exstrophy. Our fetal care specialists are here to address your concerns and help you and your family maximize your child's long-term health.
In babies with bladder exstrophy, the skin, muscle and pelvic hip bones of the lower belly are malformed, causing the bladder to protrude from the skin. The genitals can also be deformed and the muscles surrounding the bladder and urethra fail to function properly. Without normal muscle and neurological function, future incontinence – the inability to control urination – is at risk without surgery.
This condition occurs in as few as 1 in 50,000 live births. However, a family history makes it more likely. Children born to a parent with bladder exstrophy have about a 1-in-70 chance of having it, and if parents have one child with this condition, they have a 1-in-100 chance of having another child with it.
Recent studies also suggest that children born as the result of fertility treatments are 7 times more likely to have bladder exstrophy than those conceived without fertility assistance. There is a slightly increased rate of bladder exstrophy in mothers who use progestins (a natural or synthetic steroid hormone, such as progesterone, to maintain pregnancy). Males are five times more likely than females to be diagnosed.
In some cases, bladder exstrophy is detected from a routine prenatal ultrasound. In other cases, it isn't diagnosed until birth, when physicians can clearly see the exposed bladder.
Prenatally, ultrasound and MRI together often assist in making this diagnosis.
At birth, the diagnosis of bladder exstrophy is made when:
Likely, this diagnosis will not affect your pregnancy, but delivery at the Colorado Fetal Care Center may be recommended. This way, our highly trained team will be available to care for your newborn child. A unique treatment plan will be created by your fetal care team to ensure the best outcomes for your child during and after pregnancy.
The treatment for fetal bladder exstrophy is different with each child. The Colorado Fetal Care Center offers the experience and expertise of our fetal care team to provide the widest range of treatment options for this condition.
Babies born with this condition generally receive at least one bladder exstrophy surgery, but most children require multiple operations. The first priority is to close the bladder, the back of the urethra and the pelvic bones, while ensuring that the internal organs are enclosed within the abdomen. Surgeons also rebuild the external sex organs and fix the bladder so it can provide urinary continence.
The number of surgeries depends on the details of each case, but physicians often follow a phased approach to bladder exstrophy surgery. This means they start at birth by closing the bladder and pelvis and repair the urethra and genitals later in infancy. Then, when a child is ready for potty training and his or bladder is large enough, physicians perform the bladder exstrophy surgery necessary for continence (usually around age 4).
Reconstructive surgery to correct the defects associated with bladder exstrophy have positive results, with most patients achieving functional use of their bladder, urethra, pelvic bones and genitals by potty-training-age. Even in more complex cases that require longer-term management, reconstructive surgery can allow patients to reach puberty with successful results.
Exstrophy of the bladder has been recognized for centuries but it was not until the 19th century that surgical correction was first attempted (Hall et al., 1953). The first attempts at diversion of urine into the colon were made in 1850 and the first successful closure of bladder exstrophy was performed in 1862 (Canning et al., 1996). In contrast to patients with cloacal exstrophy that have other unrelated abnormalities, infants with bladder exstrophy have defects confined to the bladder, abdominal wall, perineum, genitalia and bony pelvis.
At birth, the diagnosis of bladder exstrophy is easily made by the presence of characteristic findings. The bladder plate protrudes immediately beneath the umbilical cord. The rectus muscles are divergent due to separated pubic bones. There is an outward rotation of the innominate bones and eversion of the pubic rami (Sponsellor et al., 1991). The phallus is short with a dorsal urethral plate, splayed glans and dorsal chordee. In females, the mons pubis, clitoris and labia are separated and the vaginal orifice may be displaced anteriorly. Bilateral inguinal hernias are commonly seen at birth because of the large internal and external inguinal ring caused by the splaying of the rectus musculature and lack of obliquity of the inguinal canal. Hussman et al. (1990) reported that 56% of males and 15% of females have inguinal hernias. In a report by Peppas et al. (1995) in patients presenting with hernia within 1 year of primary closure, 10 to 53% of the hernias were incarcerated.
In bladder exstrophy, the umbilical cord inserts low on the abdomen and the anus and scrotum tend to be more anteriorly placed than normal. Although the rectus abdominus muscles insert normally at the pubic tubercles, the diastasis of the pubic symphysis and lateral displacement of the iliac bones causes splaying of the rectus muscles. This lateral displacement of the rectus muscles widens the inguinal canal, predisposing to indirect inguinal hernias in these patients (Connor et al., 1989; Hussman et al., 1990; Stringer et al., 1994). Because the bladder is external, the peritoneal reflection is deeper in the pelvis than normal and the ureters course deeply through the pelvis and enter the bladder with almost no submucosal tunnel, which predisposes them to vesicoureteral reflux (Nisonson and Lattimer, 1968).
The penis in males with bladder exstrophy is short and broad because of pubic bone separation, which prevents the midline joining of the corpora cavernosa. The overall length of the corpora cavernosa is shortened but reasonable length may be obtained with epispadias repair from the deep corporal bodies (Woodhouse et al., 1984). There is usually a marked dorsal chordee and the penile curvature is compounded by shorter dorsal tunica albuginea. Females with bladder exstrophy have a hemiclitoris on each side of the bladder and the vaginal orifice may be duplicated and displaced anteriorly (Damario et al., 1994). The uterus may be duplicated but the fallopian tubes and ovaries are usually normal.
All patients with bladder exstrophy have some degree of pubic diastasis with the hips rotated outward. Many patients have a waddling gait in early childhood but long-term hip or gait problems are rare.
In normal development, the cloacal membrane occupies the infraumbilical position of the abdominal wall and this bilaminar membrane is infiltrated by mesenchyme to form the lower abdominal musculature. The genital folds fuse superiorly to form the genital tubercle. The most widely accepted theory for the cause of exstrophy is based on the work of Muecke (1964) in chick embryos. In this model, overgrowth or persistence of a thickened cloacal membrane results in truncated mesenchymal migration. Later rupture of the membrane without the mesenchymal reinforcement results in exstrophy. Bladder exstrophy results if the rupture occurs after the descent of urorectal septum. If rupture occurs in the absence of the urorectal septum, cloacal exstrophy occurs. The prenatal sonographic observation by Langer et al. of an intact cloacal membrane that subsequently ruptured during the second trimester suggests that the presence or absence of the urorectal septum, not timing of membrane rupture, distinguishes bladder exstrophy from cloacal exstrophy (Langer et al., 1992).
Cardiology - Pediatric, Pediatrics
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Surgery - Pediatric, Surgery, Surgical Critical Care