Materiały źródłowe

• #1 Bladder Exstrophy: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/21806-bladder-exstrophy

  The main symptom of bladder exstrophy is that the bladder sticks out (protrudes) through an opening in your child’s belly. But bladder exstrophy can also lead to other symptoms, including: […] If you don’t treat bladder exstrophy, your child will leak pee. With treatment, some children have ongoing challenges with their bladders or kidneys, including: […] Most babies have good long-term outcomes if they have surgery to treat bladder exstrophy. Most children who receive treatment can run, play or do any other typical activities without problems. […] Bladder exstrophy isn’t life-threatening. Children with bladder exstrophy have an average life span.

• #2 Bladder exstrophy – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/bladder-exstrophy/symptoms-causes/syc-20391299

  Bladder exstrophy can involve a spectrum of symptoms. The symptoms depend on which body parts are affected along with the bladder and stomach area, and how serious the effects are. […] Bladder exstrophy is the most common in a larger group of conditions present at birth called the bladder exstrophy-epispadias complex (BEEC). Children with BEEC have one of the following: […] Bladder exstrophy. This condition causes the bladder to form on the outside of the body. The bladder also is turned inside out. Usually, bladder exstrophy involves organs of the urinary tract, as well as the digestive and reproductive systems. Changes in the abdominal wall, bladder, genitals and pelvic bones can happen. So can changes in the end part of the large intestine called the rectum and the opening at the end of the rectum called the anus.

• #3 Bladder Exstrophy – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK563156/

  Bladder exstrophy is a rare congenital anomaly characterized by a spectrum of anomalies involving the ventral body wall, urinary tract, genitalia, bony pelvis, spine, anus, etc. Its diagnosis is clinical and does not require any additional investigations. […] The prevalence of classical bladder exstrophy is approximately 3.3 per 1,00,000 live births, and it has gender predominance in males. […] The diagnosis of bladder exstrophy is clinical and is made by the pediatrician as soon as the baby is born in the majority of cases. A fleshy, red mass prolapsing out of the suprapubic region represents the open bladder plate. There is continuous urine leakage from the mass. […] In this subset of patients, a severe excoriation in the para-exstrophy skin, hypertrophied-polypoidal bladder plate with squamous metaplasia, poor control over bowel movements, and waddling gait are common. Social problems due to the continuous wetting of clothes are also frequently seen in these children.

• #4 Bladder Exstrophy | Children’s Wisconsin

  https://childrenswi.org/medical-care/urology/conditions/exstrophy-of-the-bladder

  Bladder exstrophy is a rare complex disorder of the bladder which has not formed correctly during fetal development in the womb. In most cases, the bladder is turned inside out and is herniated outside the body through an opening in the lower belly (abdominal wall). Since the bladder is inside out and open, it does not hold or store urine. The exstrophy baby will constantly leak urine from the open bladder. […] Bladder exstrophy is rare. It happens in about one in 40,000-50,000 babies. It is more likely in boys than girls. […] There is no known cause for exstrophy. The problem occurs four to eight weeks after a woman becomes pregnant. This is when organs and tissues begin to form and specifically develop. Exstrophy is not caused by something the mother did or did not do while she was pregnant. It does not run in families (it is not hereditary).

• #5 Bladder exstrophy – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/bladder-exstrophy/symptoms-causes/syc-20391299

  Bladder exstrophy can involve a spectrum of symptoms. The symptoms depend on which body parts are affected along with the bladder and stomach area, and how serious the effects are. […] Bladder exstrophy is the most common in a larger group of conditions present at birth called the bladder exstrophy-epispadias complex (BEEC). Children with BEEC have one of the following: […] Bladder exstrophy. This condition causes the bladder to form on the outside of the body. The bladder also is turned inside out. Usually, bladder exstrophy involves organs of the urinary tract, as well as the digestive and reproductive systems. Changes in the abdominal wall, bladder, genitals and pelvic bones can happen. So can changes in the end part of the large intestine called the rectum and the opening at the end of the rectum called the anus.

• #6 Bladder Exstrophy | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/bladder-exstrophy

  The signs and symptoms of bladder exstrophy can vary from child to child, but may include: […] Abnormal development of the bladder: The bladder is open in the front and exposed on the abdominal wall (bladder is outside the body). The bladder neck has not developed properly and the bladder itself is usually small. These factors make it difficult for the bladder to hold urine initially after correction surgery until the bladder has time to grow and develop. […] Epispadias: The urethra, which is the hollow tube that drains urine from the bladder to the outside of the body, is not formed completely. In males, the urethra is open on the top of the penis and not the tip. In girls, the urethral opening may be positioned further up between the divided clitoris and labia minora. […] 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.

• #7 Bladder Exstrophy: Signs, Causes & Treatment | Baptist Health

  https://www.baptisthealth.com/care-services/conditions-treatments/bladder-exstrophy

  There are several symptoms for bladder exstrophy. These symptoms vary, depending on the type of bladder exstrophy. […] Symptoms for bladder exstrophy may include: The bladder protrudes through the skin of the belly, The bladder develops inside out, Urine will flow the wrong way (vesicoureteral reflux), Smaller bladder capacity, Anus that is lower than normal, Belly button that is lower than normal, Pelvic bones that are separated, Undescended testicles, Abnormal development of the genitals. […] In some cases, symptoms may include the development of an inguinal or umbilical hernia. Hernias occur when the stomach or intestine linings push through the abdominal wall. Hernias usually require surgery. […] Children who do not receive treatment for bladder exstrophy are at greater risk of experiencing long-term complications, such as urinary incontinence, sexual dysfunction, and bladder cancer.

• #8 Bladder Exstrophy | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/bladder-exstrophy

  Bladder exstrophy can result in challenges related to: […] Urinary incontinence (enuresis) […] Urinary tract infection […] Bladder exstrophy may be diagnosed during a routine fetal ultrasound or other indicated imaging techniques such as magnetic resonance imaging (MRI). […] After initial treatment for bladder exstrophy, your child will require lifelong follow-up. Care is ongoing and can involve a range of testing to evaluate your child’s growth and development, urinary continence, kidney function, and sexual and reproductive health. […] In some cases, additional surgery is required during childhood, adolescence, and young adulthood.

• #9 Bladder exstrophy – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/bladder-exstrophy/symptoms-causes/syc-20391299

  Children with bladder exstrophy also have a condition that causes urine to flow the wrong way. This is called vesicoureteral reflux. Urine flows backward from the bladder into tubes called ureters that connect to the kidneys. Children with bladder exstrophy have epispadias as well. […] Cloacal exstrophy (kloe-AY-kul EK-stroh-fee) is the most serious form of BEEC. In this condition, the bladder and intestine are exposed. The anus may not open and the intestine may be short. The penis or the vagina may be split. The pelvic bones are affected as well. […] Without treatment, children with bladder exstrophy won’t be able to hold urine. This is called urinary incontinence. These children also are at risk of having trouble with sexual function later in life. They have a higher risk of bladder cancer as well.

• #10 Bladder Exstrophy | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/bladder-exstrophy

  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 closed. […] 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. […] Displacement of the umbilicus (belly button) and/or an umbilical hernia. […] Our long-term goals for children with exstrophy are to optimize daytime and nighttime urinary control, to preserve normal kidney function, and to optimize the appearance and function of the external genitalia. Your child may need additional surgery as she grows older to improve continence or to complete the genital reconstruction.

• #11 Bladder Exstrophy | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/bladder-exstrophy

  The signs and symptoms of bladder exstrophy can vary from child to child, but may include: […] Abnormal development of the bladder: The bladder is open in the front and exposed on the abdominal wall (bladder is outside the body). The bladder neck has not developed properly and the bladder itself is usually small. These factors make it difficult for the bladder to hold urine initially after correction surgery until the bladder has time to grow and develop. […] Epispadias: The urethra, which is the hollow tube that drains urine from the bladder to the outside of the body, is not formed completely. In males, the urethra is open on the top of the penis and not the tip. In girls, the urethral opening may be positioned further up between the divided clitoris and labia minora. […] 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.

• #12 Bladder Exstrophy | Diagnosis, Treatment & Outlook

  https://www.cincinnatichildrens.org/health/b/bladder-exstrophy

  Children with bladder exstrophy can experience many signs and symptoms. They may include: […] Bladder problems: The bladder is outside the belly at birth. After the bladder is put back into the belly, it may leak urine. […] Split pubic bones: The pubic bones are not joined in the middle. Hips are turned outward. […] Epispadias: The urethra, which is the hollow tube that drains urine from the bladder to the outside of the body, is not formed completely. The hole that your child urinates from may be in a different place in their private area. […] Abnormal development of outer sex organs: Boys: The penis may appear shorter and curve upward (chordee). The testicles may not be in a normal position in the scrotum. A hernia may also be present. Girls: The clitoris and labia minora are split and spread apart. The vagina and urethra are shorter than usual. Usually the uterus, fallopian tubes and ovaries develop normally.

• #13

  https://www.beaumont.org/conditions/bladder-exstrophy

  The signs and symptoms vary depending on the severity of the defect, but they may include: […] a bladder that is turned inside out and exposed on the abdominal wall […] a small bladder with an abnormal bladder neck […] epispadias a birth defect in which the inner lining of the urethra (the tube that helps urine pass from the bladder out of the body) is malformed and visible either on the top surface of the penis (for boys) or between the labia minora and labia majora (for girls) […] widened pubic bones that dont join together as they should; outwardly rotated legs and feet […] abnormal genitalia […] In boys, the penis may be short and/or curved upward. The testicles may be positioned abnormally. […] In girls, the clitoris and labia minora may be separated. The vaginal opening may be narrow, and the urethra may be shorter. […] vesicoureteral reflux (VUR) a condition in which urine can travel back to the kidneys rather than draining down the ureters, into the bladder, and out through the urethra […] displaced belly button (umbilicus) and/or umbilical hernia.

• #14 Bladder Exstrophy | Children’s Hospital Colorado

  https://www.childrenscolorado.org/conditions-and-advice/conditions-and-symptoms/conditions/bladder-exstrophy/

  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. […] 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. […] At birth, the diagnosis of bladder exstrophy is made when: Part of the bladder extends out beneath the umbilical cord, Abdominal muscles go in opposite directions, In male babies, the penis is short and wide with the tip of the penis split in the middle, In females, the mons pubis (the rounded mass of fatty tissue lying over the pubic bones), clitoris and labia are separated, and the opening of the vagina or anus may be pushed more towards the abdomen.

• #15 Bladder exstrophy – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/bladder-exstrophy/symptoms-causes/syc-20391299

  Children with bladder exstrophy also have a condition that causes urine to flow the wrong way. This is called vesicoureteral reflux. Urine flows backward from the bladder into tubes called ureters that connect to the kidneys. Children with bladder exstrophy have epispadias as well. […] Cloacal exstrophy (kloe-AY-kul EK-stroh-fee) is the most serious form of BEEC. In this condition, the bladder and intestine are exposed. The anus may not open and the intestine may be short. The penis or the vagina may be split. The pelvic bones are affected as well. […] Without treatment, children with bladder exstrophy won’t be able to hold urine. This is called urinary incontinence. These children also are at risk of having trouble with sexual function later in life. They have a higher risk of bladder cancer as well.

• #16 Bladder Exstrophy – Seattle Children’s

  https://www.seattlechildrens.org/conditions/bladder-exstrophy/

  The symptoms and signs of bladder exstrophy depend on how severe it is and if other organs are involved. Children with bladder exstrophy may have some or all of these: […] The bladder may hold less urine than normal. This may cause frequent peeing (urination) or leaking. […] Some children with a more severe form of exstrophy (cloacal exstrophy) may have a fatty growth (lipoma) on their spinal cord. This may cause problems with their legs, bladder and rectum. Cloacal exstrophy also affects the genitals and bowel. […] Some children may have double kidneys or kidneys not in the usual place. […] Children with cloacal exstrophy may have bowels that are large, not well developed and without a rectum. They usually need surgery to make an opening from the intestines to outside the belly wall so poop (stool) can come out. This surgery is called a colostomy.

• #17 What Is Bladder Exstrophy?

  https://www.icliniq.com/articles/kidney-and-urologic-diseases/bladder-exstrophy

  In this condition, the penis of the boy curves upwards. […] Either side of the pubic bone in the pelvis does not meet in the front. This leads to a wide split in the pubic bone. This condition is called diastasis. […] Few kids develop a severe form of exstrophy, in which there is a fatty outgrowth or lipoma on their spinal cord. This causes problems with the legs, bladder, and rectum (the end part of the large intestine). It might also affect the genitals and bowels of the kids. […] A few kids might have more than two kidneys or displaced kidneys. […] Some kids may have large bowels that are underdeveloped and without a rectum. Surgery is usually needed to make an opening from the intestine to outside the abdominal wall for stool to come out. This surgical process is known as colostomy. […] This is the displacement of the belly button or umbilicus.

• #18 Bladder exstrophy – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/bladder-exstrophy/symptoms-causes/syc-20391299

  Children with bladder exstrophy also have a condition that causes urine to flow the wrong way. This is called vesicoureteral reflux. Urine flows backward from the bladder into tubes called ureters that connect to the kidneys. Children with bladder exstrophy have epispadias as well. […] Cloacal exstrophy (kloe-AY-kul EK-stroh-fee) is the most serious form of BEEC. In this condition, the bladder and intestine are exposed. The anus may not open and the intestine may be short. The penis or the vagina may be split. The pelvic bones are affected as well. […] Without treatment, children with bladder exstrophy won’t be able to hold urine. This is called urinary incontinence. These children also are at risk of having trouble with sexual function later in life. They have a higher risk of bladder cancer as well.

• #19 Bladder Exstrophy: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/21806-bladder-exstrophy

  The main symptom of bladder exstrophy is that the bladder sticks out (protrudes) through an opening in your child’s belly. But bladder exstrophy can also lead to other symptoms, including: […] If you don’t treat bladder exstrophy, your child will leak pee. With treatment, some children have ongoing challenges with their bladders or kidneys, including: […] Most babies have good long-term outcomes if they have surgery to treat bladder exstrophy. Most children who receive treatment can run, play or do any other typical activities without problems. […] Bladder exstrophy isn’t life-threatening. Children with bladder exstrophy have an average life span.

• #20 Bladder Exstrophy: Causes, Symptoms, and Treatment Options – The Kingsley Clinic

  https://thekingsleyclinic.com/resources/bladder-exstrophy-causes-symptoms-and-treatment-options-2/

  Bladder exstrophy is a congenital condition where the bladder forms outside the body, leading to complications such as urinary incontinence, infections, and kidney damage. […] The progression of bladder exstrophy varies depending on the severity and timing of treatment. Without intervention, patients may experience chronic urinary incontinence, recurrent infections, and kidney damage. However, early surgical intervention can improve bladder function and quality of life. […] Urinary incontinence, or the inability to control urination, is present in nearly all patients with bladder exstrophy. […] The bladder is often smaller than normal and lacks the muscle tone needed to store urine. This can lead to frequent urination, incomplete emptying of the bladder, and an increased risk of urinary tract infections (UTIs).

• #21 Bladder Exstrophy: Causes, Symptoms, and Treatment Options – The Kingsley Clinic

  https://thekingsleyclinic.com/resources/bladder-exstrophy-causes-symptoms-and-treatment-options-2/

  Urinary tract infections are common in bladder exstrophy patients, with an estimated 50-70% experiencing recurrent infections. […] Skin irritation occurs in about 80% of patients with bladder exstrophy due to constant exposure to urine. […] Difficulty with urination is common in bladder exstrophy patients due to the abnormal structure of the bladder and urinary tract. […] Recurrent urinary issues, such as frequent urination, urinary retention, and infections, are common in bladder exstrophy patients.

• #22 Bladder Exstrophy – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK563156/

  Bladder exstrophy is a rare congenital anomaly characterized by a spectrum of anomalies involving the ventral body wall, urinary tract, genitalia, bony pelvis, spine, anus, etc. Its diagnosis is clinical and does not require any additional investigations. […] The prevalence of classical bladder exstrophy is approximately 3.3 per 1,00,000 live births, and it has gender predominance in males. […] The diagnosis of bladder exstrophy is clinical and is made by the pediatrician as soon as the baby is born in the majority of cases. A fleshy, red mass prolapsing out of the suprapubic region represents the open bladder plate. There is continuous urine leakage from the mass. […] In this subset of patients, a severe excoriation in the para-exstrophy skin, hypertrophied-polypoidal bladder plate with squamous metaplasia, poor control over bowel movements, and waddling gait are common. Social problems due to the continuous wetting of clothes are also frequently seen in these children.

• #23 Bladder Exstrophy: Signs, Causes & Treatment | Baptist Health

  https://www.baptisthealth.com/care-services/conditions-treatments/bladder-exstrophy

  There are several symptoms for bladder exstrophy. These symptoms vary, depending on the type of bladder exstrophy. […] Symptoms for bladder exstrophy may include: The bladder protrudes through the skin of the belly, The bladder develops inside out, Urine will flow the wrong way (vesicoureteral reflux), Smaller bladder capacity, Anus that is lower than normal, Belly button that is lower than normal, Pelvic bones that are separated, Undescended testicles, Abnormal development of the genitals. […] In some cases, symptoms may include the development of an inguinal or umbilical hernia. Hernias occur when the stomach or intestine linings push through the abdominal wall. Hernias usually require surgery. […] Children who do not receive treatment for bladder exstrophy are at greater risk of experiencing long-term complications, such as urinary incontinence, sexual dysfunction, and bladder cancer.

• #24 Bladder Exstrophy | Texas Children’s

  https://www.texaschildrens.org/content/conditions/bladder-exstrophy

  Bladder exstrophy typically involves additional defects in the urinary tract, reproductive organs, pelvic bones, lower abdominal wall muscles and skin, and rectum. […] Common complications include: Urinary incontinence (urine leakage), Vesicoureteral reflux (backward flow of urine from the bladder up into the ureters, the tubes that connect to the kidneys), The need for repeated reconstructive surgery, Infertility. […] Even with successful surgery, patients may have long-term problems with the following: ability to hold in urine, urinary reflux (back-up of urine to the kidneys), repeated urinary tract infections (UTIs), bladder or colonic adenocarcinoma (cancer that develops in the glandular tissues of the body), uterine prolapse (the uterus slips down into or protrudes out of the vagina), self-image.

• #25 Bladder exstrophy – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/bladder-exstrophy/symptoms-causes/syc-20391299

  Children with bladder exstrophy also have a condition that causes urine to flow the wrong way. This is called vesicoureteral reflux. Urine flows backward from the bladder into tubes called ureters that connect to the kidneys. Children with bladder exstrophy have epispadias as well. […] Cloacal exstrophy (kloe-AY-kul EK-stroh-fee) is the most serious form of BEEC. In this condition, the bladder and intestine are exposed. The anus may not open and the intestine may be short. The penis or the vagina may be split. The pelvic bones are affected as well. […] Without treatment, children with bladder exstrophy won’t be able to hold urine. This is called urinary incontinence. These children also are at risk of having trouble with sexual function later in life. They have a higher risk of bladder cancer as well.

• #26 Contemporary issues relating to transitional care in bladder exstrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5926917/

  Bladder rupture is a rare, but dangerous complication of bladder augmentation in the setting of bladder exstrophy. It has often been attributed to poor compliance with clean intermittent catheterization in the adolescent group, when patients are at risk of rebelling to gain some semblance of control over their health and lives. […] The primary aim of bladder exstrophy surgery is to tubularize and close the bladder plate with or without ureteric reimplantation, with the associated, but equally important procedures of epispadias repair and bladder neck plasty. […] Patients with bladder exstrophy often have preserved sexual function, libido, and in many cases, are capable of successful fertility. […] Multiple studies have shown that men with bladder exstrophy can have preserved orgasmic function. Similarly, most men with exstrophy are capable of ejaculation, although the semen parameters and paternity seem to be significantly impacted from exstrophy reconstruction. […] Paternity rates in exstrophy are significantly reduced and range from 928%.

• #27 Bladder exstrophy: information & bladder exstrophy specialists

  https://www.leading-medicine-guide.com/en/illness/malformation/bladder-exstrophy

  In addition, kidney damage is often already present at birth. Later on, permanent kidney problems cannot be ruled out. […] The deformity also impairs sexuality. Bladder exstrophy only promotes erectile dysfunction and sexual dysfunction. It also almost always leads to a major psychological burden for the affected patients. […] The presence of bladder exstrophy is a urological emergency and must be treated surgically within 72 hours. Depending on the severity of the deformity, several operations are usually necessary until the deformity is corrected. […] The operations usually bring about a significant improvement in the problem complex. However, further operations are usually necessary to enable patients to lead a largely symptom-free life. For example, continence may subsequently disappear again.

• #28 Contemporary issues relating to transitional care in bladder exstrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5926917/

  This patient illustrates some of the issues associated with transitional care to an adult service and highlights some of the important issues surrounding the care of the adolescent exstrophy patient, such as surveillance for upper tract deterioration, management of the augmented bladder, urinary tract stones, recurrent UTIs, and surveillance for bladder cancer and testicular germ cell tumours (post-orchiopexy). […] The bladder mucosa may contain hamartomatous polyps, ectopic bowel mucosa, or in the setting of cloacal exstrophy, a segment of bowel. In addition to this, there may be squamous metaplasia with or without the presence of cystitis cystica or cystitis glandularis, the latter of which may increase the future risk of bladder adenocarcinoma. […] Recurrent stone formation is an important consideration in those with an augmented bladder, especially if the bladder neck has previously been closed or reconstructed with the creation of a Mitrofanoff channel. The presence of bladder exstrophy increases the odds ratio (17.4) of developing stones within an augmented bladder and may potentially be as a result of an underlying metabolic anomaly.

• #29 Contemporary issues relating to transitional care in bladder exstrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5926917/

  Bladder rupture is a rare, but dangerous complication of bladder augmentation in the setting of bladder exstrophy. It has often been attributed to poor compliance with clean intermittent catheterization in the adolescent group, when patients are at risk of rebelling to gain some semblance of control over their health and lives. […] The primary aim of bladder exstrophy surgery is to tubularize and close the bladder plate with or without ureteric reimplantation, with the associated, but equally important procedures of epispadias repair and bladder neck plasty. […] Patients with bladder exstrophy often have preserved sexual function, libido, and in many cases, are capable of successful fertility. […] Multiple studies have shown that men with bladder exstrophy can have preserved orgasmic function. Similarly, most men with exstrophy are capable of ejaculation, although the semen parameters and paternity seem to be significantly impacted from exstrophy reconstruction. […] Paternity rates in exstrophy are significantly reduced and range from 928%.

• #30 Bladder exstrophy – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/bladder-exstrophy/diagnosis-treatment/drc-20392198

  A bladder that doesn’t fill or empty correctly. […] An umbilical cord that is placed low on the stomach area. […] Pubic bones part of the hipbones that form the pelvis that are separated. […] Genitals that are smaller than is typical. […] Size of the portion of the bladder that is open and exposed to air. […] Position of the testicles. […] Intestine bulging through the abdominal wall, also called an inguinal hernia. […] Anatomy of the area around the navel. […] Position of the anus. […] How much the pubic bones are separated, and how easily the pelvis moves. […] After surgery, many children gain some bladder control. […] If your child had complete primary repair surgery, your child likely will need bladder neck surgery to improve bladder control. […] Sometimes, children need to have a tube inserted into their bladders to drain urine. […] More surgeries may be needed to improve urination or repair the genitals as your child grows.

• #31 Health Information Library | Rumah Sakit Pusat Pertamina

  https://rspp.co.id/dcontent.html?id=CON-20339069&n=Bladder%20exstrophy

  After surgery, many children gain some bladder control. If your child had complete primary repair surgery, your child likely will need bladder neck surgery to improve bladder control. Sometimes, children need to have a tube inserted into their bladders to drain urine. This is called catheterization. More surgeries may be needed to improve urination or repair the genitals as your child grows.

• #32 Orphanet: Classic bladder exstrophy

  https://www.orpha.net/en/disease/detail/93930

  CEB is evident at birth, with the reddish bladder mucosa visible in the lower abdomen and mucosal polyps sometimes present on the surface. […] Additionally, pubic diastasis with divergent rectus muscles and inguinal hernias are seen. In males, the penis is short and broad with a dorsal chordee. The urethral plate covers the whole dorsum from the penile tip to the open bladder. […] Females present with a bifid clitoris next to the open urethral plate. The vaginal opening appears narrow and the perineum is shortened due to the anterior displacement of the vagina and the anus. […] By adulthood, 80% of patients need additional surgery, such as bladder augmentation or creating a continent cutaneous stoma, to optimize bladder function and achieve continence or dryness. If reconstruction fails, urinary diversion is necessary. […] Life expectancy is usually normal, but complications like renal insufficiency may severely affect survival. QoL is impacted by continence, genital appearance and function, complications and their management, coping strategies and emotional well-being.

• #33 Bladder Exstrophy | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/bladder-exstrophy

  The appearance of the bladder is the most obvious sign of bladder exstrophy. The bladder sticks out of an opening in the belly or it completely forms on the outside of the body. Other signs and symptoms include: […] Children with bladder exstrophy are totally incontinent. This means they can’t control their urine flow. […] For many children (about 75%), the Kelly procedure is considered the most major operation to treat the condition. Additional treatments may include procedures to repair the appearance of the external genitalia, abdominal wall and control of their urine in the daytime. Nighttime continence may take longer to develop. […] If your child still cant stay dry, they might need another surgery. Here’s what it involves: […] Ongoing care will help to make sure your child’s bladder, kidneys and genitals develop and remain functioning in a healthy way. Many people regain continence after surgery and maintain it throughout their lifetime.

• #34 Bladder Exstrophy – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK563156/

  The diagnosis of exstrophy bladder is clinical and does not require any other additional investigations. Routine hemogram and blood chemistry are performed as a part of the preoperative work-up. […] After CPRE, it was seen in 53% of the patients during follow-up. Overall, 30% had bilateral hydronephrosis. Male gender is associated with worse renal outcomes. […] The most common complications of the surgical treatment of bladder exstrophy are wound dehiscence and bladder dehiscence. Complete dehiscence can cause bladder prolapse. […] The immediate pain is managed by the epidural analgesia. Intravenous analgesia may be required for a longer duration as the child may suffer pain from the traction and immobilization.

• #35 Bladder Exstrophy – MD Searchlight

  https://mdsearchlight.com/child-health/bladder-exstrophy/?utm_source=pubmedlink&utm_campaign=MDS&utm_content=121871

  Bladder exstrophy is a condition that is typically diagnosed by pediatricians right after a baby is born. The condition is visible as a reddish, soft mass in the area above the pubic bone, which is the opening of the bladder. This area is continuously leaking urine. The amount of the bladder that is exposed can vary, and the ureter openings are often visible. However, no attempts should be made to blindly probe these openings. […] Even though the treatment for bladder exstrophy starts from birth, it is not uncommon for some children to show symptoms later on, especially in developing countries. These symptoms may include skin inflammation around the bladder area, a bladder opening that looks like polyps and has cell changes, lack of control over bowel movements, and a distinctive gait. […] The most usual problems after bladder exstrophy surgery are wound separation and bladder coming apart. If these get worse, the bladder could slip out of place.

• #36 Bladder Exstrophy | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/bladder-exstrophy

  After surgical repair of bladder exstrophy, your child will require lifelong follow-up care. After the initial surgery, our team will follow your family closely. Your child will be scheduled for regular follow-up visits and ultrasounds to ensure her bladder and kidneys continue to develop in a healthy and safe way.

• #37 Contemporary issues relating to transitional care in bladder exstrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5926917/

  This patient illustrates some of the issues associated with transitional care to an adult service and highlights some of the important issues surrounding the care of the adolescent exstrophy patient, such as surveillance for upper tract deterioration, management of the augmented bladder, urinary tract stones, recurrent UTIs, and surveillance for bladder cancer and testicular germ cell tumours (post-orchiopexy). […] The bladder mucosa may contain hamartomatous polyps, ectopic bowel mucosa, or in the setting of cloacal exstrophy, a segment of bowel. In addition to this, there may be squamous metaplasia with or without the presence of cystitis cystica or cystitis glandularis, the latter of which may increase the future risk of bladder adenocarcinoma. […] Recurrent stone formation is an important consideration in those with an augmented bladder, especially if the bladder neck has previously been closed or reconstructed with the creation of a Mitrofanoff channel. The presence of bladder exstrophy increases the odds ratio (17.4) of developing stones within an augmented bladder and may potentially be as a result of an underlying metabolic anomaly.

• #38 Bladder Exstrophy: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/21806-bladder-exstrophy

  The main symptom of bladder exstrophy is that the bladder sticks out (protrudes) through an opening in your child’s belly. But bladder exstrophy can also lead to other symptoms, including: […] If you don’t treat bladder exstrophy, your child will leak pee. With treatment, some children have ongoing challenges with their bladders or kidneys, including: […] Most babies have good long-term outcomes if they have surgery to treat bladder exstrophy. Most children who receive treatment can run, play or do any other typical activities without problems. […] Bladder exstrophy isn’t life-threatening. Children with bladder exstrophy have an average life span.

• #39

  https://www.shrinerschildrens.org/en/pediatric-care/bladder-exstrophy

  Children born with bladder exstrophy are treated with reconstructive surgery after birth and then are provided long-term follow up care including medical management, bowel management, psychosocial support and other support services. Additional surgeries are sometimes needed as the child grows. […] Children with bladder exstrophy have normal life expectancies and the ability to live full and productive lives with working relationships and children of their own.

• #40 Bladder exstrophy in children – Children’s Health Urology

  https://www.childrens.com/specialties-services/conditions/bladder-exstrophy

  Bladder exstrophy varies widely among children with this condition. Children with bladder exstrophy often have other abnormalities, such as an unusually wide pelvic bone structure or problems with abdominal muscles. […] Children with bladder exstrophy will have very poor bladder control and may also have problems controlling other muscles in the abdominal area, including in the digestive tract. […] Bladder exstrophy is diagnosed when any one of several abnormal conditions exist in a child’s bladder, urinary tract, or abdominal area. […] Following treatment, most children with this condition are able to have normal elimination and sexual activity. […] About 75% of those treated for this condition will be able to exercise bladder control. The majority of those treated also have normal function of the bladder and sexual organs.

• #41 Bladder exstrophy – Wikipedia

  https://en.wikipedia.org/wiki/Bladder_exstrophy

  The classic manifestation of bladder exstrophy presents with: […] Females frequently have a displaced and narrowed vaginal orifice, a bifid clitoris, and divergent labia. […] The most important criterion for improving long-term prognosis is success of the initial closure. […] If a patient requires more than one closure their chance of continence drops off precipitously with each additional closure – at just two closures the chance of voiding continence is just 17%. […] Even with successful surgery, people may have long-term complications. Some of the most common include: vesicoureteral reflux, bladder spasm, bladder calculus, urinary tract infections.

• #42 Bladder exstrophy symptoms, treatments & forums | PatientsLikeMe

  https://www.patientslikeme.com/conditions/bladder-exstrophy

  Bladder exstrophy occurs when a child is born with their bladder outside of the body, protruding from the abdominal wall. The bladder and supporting structures themselves are also not properly formed. This can be repaired with surgery. […] Common symptom: Stress. […] 2 bladder exstrophy patients report severe stress (66%). […] 1 a bladder exstrophy patient reports moderate stress (33%). […] 0 bladder exstrophy patients report mild stress (0%). […] 0 bladder exstrophy patients report no stress (0%). […] Common symptom: Fatigue. […] 1 a bladder exstrophy patient reports severe fatigue (16%). […] 3 bladder exstrophy patients report moderate fatigue (50%). […] 2 bladder exstrophy patients report mild fatigue (33%). […] 0 bladder exstrophy patients report no fatigue (0%). […] Common symptom: Pain.

• #43 Bladder exstrophy symptoms, treatments & forums | PatientsLikeMe

  https://www.patientslikeme.com/conditions/bladder-exstrophy

  1 a bladder exstrophy patient reports severe pain (16%). […] 2 bladder exstrophy patients report moderate pain (33%). […] 1 a bladder exstrophy patient reports mild pain (16%). […] 2 bladder exstrophy patients report no pain (33%). […] Common symptom: Depressed mood. […] 0 bladder exstrophy patients report severe depressed mood (0%). […] 3 bladder exstrophy patients report moderate depressed mood (50%). […] 2 bladder exstrophy patients report mild depressed mood (33%). […] 1 a bladder exstrophy patient reports no depressed mood (16%). […] Common symptom: Anxious mood. […] 0 bladder exstrophy patients report severe anxious mood (0%). […] 2 bladder exstrophy patients report moderate anxious mood (33%). […] 2 bladder exstrophy patients report mild anxious mood (33%). […] 2 bladder exstrophy patients report no anxious mood (33%).

• #44 Bladder Exstrophy | Diagnosis, Treatment & Outlook

  https://www.cincinnatichildrens.org/health/b/bladder-exstrophy

  Displaced or missing belly button and/or an umbilical hernia. The child’s umbilical cord inserts low in the belly, just above the bladder. […] Kidney problems: Some children may have extra kidneys or kidneys not in the usual place. […] Vesicoureteral reflux (VUR): Urine flows backward from the bladder up to the kidneys. […] After surgery, children with bladder exstrophy will require lifelong follow-up care. This helps ensure the best health and quality of life possible. […] It is very common for children with bladder exstrophy to experience emotional issues related to their condition. Parents may struggle with complicated feelings about having a child with this condition. A psychologist can be part of the care team to help your family understand these feelings and deal with them together. […] With surgery and other treatment, children with bladder and cloacal exstrophy can have a happy, productive life with normal life expectancy. Some males with bladder exstrophy grow up to experience fertility issues, but fertility assistance is available to help them conceive a child.

• #45 Bladder exstrophy – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/bladder-exstrophy/symptoms-causes/syc-20391299

  Surgery can lower the chances of some complications. The success of surgery depends on how serious the condition is. Many children who have surgical repair are able to hold urine. Young children with bladder exstrophy may walk with their legs turned somewhat outward. This is due to the separation of their pelvic bones. Walking gets better with age. […] People born with bladder exstrophy can go on to have regular sexual function. That includes being able to have children. But pregnancy often is high risk for a pregnant person who’s had bladder exstrophy and for the unborn baby. A planned cesarean birth, also known as a C-section, may be needed.

• #46 Contemporary issues relating to transitional care in bladder exstrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5926917/

  Bladder rupture is a rare, but dangerous complication of bladder augmentation in the setting of bladder exstrophy. It has often been attributed to poor compliance with clean intermittent catheterization in the adolescent group, when patients are at risk of rebelling to gain some semblance of control over their health and lives. […] The primary aim of bladder exstrophy surgery is to tubularize and close the bladder plate with or without ureteric reimplantation, with the associated, but equally important procedures of epispadias repair and bladder neck plasty. […] Patients with bladder exstrophy often have preserved sexual function, libido, and in many cases, are capable of successful fertility. […] Multiple studies have shown that men with bladder exstrophy can have preserved orgasmic function. Similarly, most men with exstrophy are capable of ejaculation, although the semen parameters and paternity seem to be significantly impacted from exstrophy reconstruction. […] Paternity rates in exstrophy are significantly reduced and range from 928%.

• #47 Management of Bladder Exstrophy in Adulthood: A Concise Guide for Treating Urologists – American Urological Association

  https://auanews.net/issues/articles/2022/june-2022/management-of-bladder-exstrophy-in-adulthood-a-concise-guide-for-treating-urologists

  Exstrophy-epispadias complex (EEC) occurs in 1/10,000 live births and classically presents as bladder exstrophy (BE) with an estimated male:female ratio of 2.4:1–6:1. BE represents a severe congenital genitourinary anomaly which leads to many urinary, sexual and general health challenges as these patients age. […] Sexual health is a major concern, particularly among male patients who make up the sizable majority of BE patients. One of the drivers of sexual health concerns is the typically small phallus associated in men with BE due to congenitally limited corporal tissue. One study found that 79% of men reported that BE interferes with sexual or romantic relationships, while another noted that only 58% of men were moderately or very satisfied with sexual function. Among women with BE, 46% reported that BE interferes with sexual or romantic relationships. Additionally, women with BE have substantially higher rates of pelvic organ prolapse ranging from 30%–50% and at a much younger age (16) compared to the general population.

• #48 Bladder Exstrophy | Diagnosis, Treatment & Outlook

  https://www.cincinnatichildrens.org/health/b/bladder-exstrophy

  Displaced or missing belly button and/or an umbilical hernia. The child’s umbilical cord inserts low in the belly, just above the bladder. […] Kidney problems: Some children may have extra kidneys or kidneys not in the usual place. […] Vesicoureteral reflux (VUR): Urine flows backward from the bladder up to the kidneys. […] After surgery, children with bladder exstrophy will require lifelong follow-up care. This helps ensure the best health and quality of life possible. […] It is very common for children with bladder exstrophy to experience emotional issues related to their condition. Parents may struggle with complicated feelings about having a child with this condition. A psychologist can be part of the care team to help your family understand these feelings and deal with them together. […] With surgery and other treatment, children with bladder and cloacal exstrophy can have a happy, productive life with normal life expectancy. Some males with bladder exstrophy grow up to experience fertility issues, but fertility assistance is available to help them conceive a child.

• #49 Contemporary issues relating to transitional care in bladder exstrophy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5926917/

  Bladder rupture is a rare, but dangerous complication of bladder augmentation in the setting of bladder exstrophy. It has often been attributed to poor compliance with clean intermittent catheterization in the adolescent group, when patients are at risk of rebelling to gain some semblance of control over their health and lives. […] The primary aim of bladder exstrophy surgery is to tubularize and close the bladder plate with or without ureteric reimplantation, with the associated, but equally important procedures of epispadias repair and bladder neck plasty. […] Patients with bladder exstrophy often have preserved sexual function, libido, and in many cases, are capable of successful fertility. […] Multiple studies have shown that men with bladder exstrophy can have preserved orgasmic function. Similarly, most men with exstrophy are capable of ejaculation, although the semen parameters and paternity seem to be significantly impacted from exstrophy reconstruction. […] Paternity rates in exstrophy are significantly reduced and range from 928%.

• #50 Bladder exstrophy – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/bladder-exstrophy/symptoms-causes/syc-20391299

  Surgery can lower the chances of some complications. The success of surgery depends on how serious the condition is. Many children who have surgical repair are able to hold urine. Young children with bladder exstrophy may walk with their legs turned somewhat outward. This is due to the separation of their pelvic bones. Walking gets better with age. […] People born with bladder exstrophy can go on to have regular sexual function. That includes being able to have children. But pregnancy often is high risk for a pregnant person who’s had bladder exstrophy and for the unborn baby. A planned cesarean birth, also known as a C-section, may be needed.

• #51 Bladder Exstrophy | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/bladder-exstrophy

  After surgical repair of bladder exstrophy, your child will require lifelong follow-up care. After the initial surgery, our team will follow your family closely. Your child will be scheduled for regular follow-up visits and ultrasounds to ensure her bladder and kidneys continue to develop in a healthy and safe way.

• #52 Bladder Exstrophy | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/bladder-exstrophy

  Bladder exstrophy can result in challenges related to: […] Urinary incontinence (enuresis) […] Urinary tract infection […] Bladder exstrophy may be diagnosed during a routine fetal ultrasound or other indicated imaging techniques such as magnetic resonance imaging (MRI). […] After initial treatment for bladder exstrophy, your child will require lifelong follow-up. Care is ongoing and can involve a range of testing to evaluate your child’s growth and development, urinary continence, kidney function, and sexual and reproductive health. […] In some cases, additional surgery is required during childhood, adolescence, and young adulthood.

• #53 Bladder Exstrophy – Center for Young Women’s Health

  https://youngwomenshealth.org/guides/bladder-exstrophy/

  Many people with bladder exstrophy need to take medicine to help the bladder work correctly. A person may also need medicine to treat or prevent bladder and kidney infections. […] If you have bladder exstrophy its important to take your medications as prescribed by your health care provider, even if youre feeling fine. This is because many of the medications prevent problems from happening. […] Call your health care provider right away if you have any of the following symptoms: Fever greater than 101.5F, You cant empty your bladder by urinating or catheterizing, Severe pain in your abdomen (belly) or back, Nausea or vomiting that doesnt go away, Unable to have a bowel movement (poop) for more than 2 days.

• #54 Bladder Exstrophy – Center for Young Women’s Health

  https://youngwomenshealth.org/guides/bladder-exstrophy/

  Many people with bladder exstrophy need to take medicine to help the bladder work correctly. A person may also need medicine to treat or prevent bladder and kidney infections. […] If you have bladder exstrophy its important to take your medications as prescribed by your health care provider, even if youre feeling fine. This is because many of the medications prevent problems from happening. […] Call your health care provider right away if you have any of the following symptoms: Fever greater than 101.5F, You cant empty your bladder by urinating or catheterizing, Severe pain in your abdomen (belly) or back, Nausea or vomiting that doesnt go away, Unable to have a bowel movement (poop) for more than 2 days.

• #55 Bladder Exstrophy – Center for Young Women’s Health

  https://youngwomenshealth.org/guides/bladder-exstrophy/

  Many people with bladder exstrophy need to take medicine to help the bladder work correctly. A person may also need medicine to treat or prevent bladder and kidney infections. […] If you have bladder exstrophy its important to take your medications as prescribed by your health care provider, even if youre feeling fine. This is because many of the medications prevent problems from happening. […] Call your health care provider right away if you have any of the following symptoms: Fever greater than 101.5F, You cant empty your bladder by urinating or catheterizing, Severe pain in your abdomen (belly) or back, Nausea or vomiting that doesnt go away, Unable to have a bowel movement (poop) for more than 2 days.

• #56 Bladder Exstrophy | Young Men’s Health

  https://youngmenshealthsite.org/guides/bladder-exstrophy/

  Bladder exstrophy is a rare condition that affects males and females. Only one out of every 20,000 babies born will have bladder exstrophy, and there is no known cause. […] Since the bladder is exposed to the outside, urine constantly trickles onto the skin, which may cause irritation. Health care providers who treat children with bladder exstrophy work closely with both patients and caregivers to prevent skin problems. […] Call your health care provider right away if you have any of the following symptoms: Fever greater than 101.5F, You cant empty your bladder by urinating or catheterizing, Severe pain in your abdomen (belly) or back, Nausea or vomiting that doesnt go away, Youre unable to have a bowel movement (poop) for more than 2 days.

• #57 Bladder Exstrophy | Texas Children’s

  https://www.texaschildrens.org/content/conditions/bladder-exstrophy-0

  Children with bladder exstrophy require lifelong care by urologists experienced in the treatment of this rare and complex birth defect. Additional surgeries are often required throughout the child’s life to optimize bladder function and control. Ongoing emotional support is also critical to helping children and adolescents cope with the challenges of this condition.

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