Materiały źródłowe

• #1 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 diagnosis of bladder exstrophy is clinical and does not require any ancillary investigations. […] 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. […] The diagnosis of exstrophy bladder is clinical and does not require any other additional investigations.

• #2 Current management of classic bladder exstrophy in the modern era | African Journal of Urology | Full Text

  https://afju.springeropen.com/articles/10.1186/s12301-023-00356-4

  Classic bladder exstrophy is a complex, multi-system congenital malformation affecting formation of the genitourinary system, pelvis, and abdominal wall. […] Modern advancements in the diagnosis and management of this disorder have resulted in low mortality rates and shifted clinical focus toward optimizing quality of life. […] This is an updated overview of the pathology, diagnosis, and management of this rare disorder. […] The hallmark findings for prenatal diagnosis include: (1) absence of bladder filling, (2) a low-set umbilicus, (3) widening pubic ramus, (4) diminutive genitalia, and (5) lower abdominal mass. […] The diagnosis is often missed or misdiagnosed as omphalocele or gastroschisis. […] If the infant possesses a large, elastic bladder template, free of any polyps, and under the care of an experienced exstrophy team, then, a newborn closure may be considered.

• #3 Prenatal Diagnosis of Bladder Exstrophy: Have We Shifted the Diagnostic Curve? – American Urological Association

  https://auanews.net/issues/articles/2024/november-extra-2024/prenatal-diagnosis-of-bladder-exstrophy-have-we-shifted-the-diagnostic-curve

  Two recent studies reported remarkably similar rates of prenatal diagnosis for classic bladder exstrophy: 47% (73/155) and 48% (134/280). […] These diagnostic rates are surprisingly low considering the high test sensitivity of the prenatal sonographic features of bladder exstrophy. […] A prenatal diagnosis affords families sufficient time to find experienced centers, generate a care plan, and coordinate the logistics involving postnatal care (ie, financial, travel, lodging). […] A notable finding from both US studies was an improved diagnosis rate over a 20-year period. […] The improved prenatal diagnosis rate may be secondary to a significant increased utilization trend of repeat prenatal ultrasound within the US and perhaps evolving ultrasound technology. […] However, a persistently empty/absent bladder within the pelvis after 15 weeks is considered abnormal, and pediatric urologists have a responsibility to work with referring maternal fetal medicine providers and their radiology team to spread awareness about the importance of rescanning fetuses in which the bladder is found to be empty.

• #4 Prenatal Diagnosis of Bladder Exstrophy: Have We Shifted the Diagnostic Curve? – American Urological Association

  https://www.auanews.net/issues/articles/2024/november-extra-2024/prenatal-diagnosis-of-bladder-exstrophy-have-we-shifted-the-diagnostic-curve

  Two recent studies reported remarkably similar rates of prenatal diagnosis for classic bladder exstrophy: 47% (73/155) and 48% (134/280). […] These diagnostic rates are surprisingly low considering the high test sensitivity of the prenatal sonographic features of bladder exstrophy. […] A prenatal diagnosis affords families sufficient time to find experienced centers, generate a care plan, and coordinate the logistics involving postnatal care (ie, financial, travel, lodging). […] A notable finding from both US studies was an improved diagnosis rate over a 20-year period. […] We hope that the increasing utilization of prenatal ultrasonography and our effort to improve awareness of this condition among radiologists and ultrasound technicians will build upon the current rising trend of prenatal bladder exstrophy diagnosis, thus allowing caregivers to be optimally prepared for the birth and postnatal care of the affected child.

• #5 Prenatal Diagnosis of Bladder Exstrophy: Have We Shifted the Diagnostic Curve? – American Urological Association

  https://auanews.net/issues/articles/2024/november-extra-2024/prenatal-diagnosis-of-bladder-exstrophy-have-we-shifted-the-diagnostic-curve

  Two recent studies reported remarkably similar rates of prenatal diagnosis for classic bladder exstrophy: 47% (73/155) and 48% (134/280). […] These diagnostic rates are surprisingly low considering the high test sensitivity of the prenatal sonographic features of bladder exstrophy. […] A prenatal diagnosis affords families sufficient time to find experienced centers, generate a care plan, and coordinate the logistics involving postnatal care (ie, financial, travel, lodging). […] A notable finding from both US studies was an improved diagnosis rate over a 20-year period. […] The improved prenatal diagnosis rate may be secondary to a significant increased utilization trend of repeat prenatal ultrasound within the US and perhaps evolving ultrasound technology. […] However, a persistently empty/absent bladder within the pelvis after 15 weeks is considered abnormal, and pediatric urologists have a responsibility to work with referring maternal fetal medicine providers and their radiology team to spread awareness about the importance of rescanning fetuses in which the bladder is found to be empty.

• #6 Azthena logo with the word Azthena

  https://www.news-medical.net/health/What-is-Bladder-Exstrophy-Epispadias-Cloacal-Exstrophy-Complex.aspx

  Bladder exstrophy, on the other hand, may usually be detected between the 15th and 32nd weeks of pregnancy with high-resolution real-time ultrasonography, even during regular obstetric treatment. The absence of a fluid-filled bladder, a low-set umbilicus, tiny genitalia, pubic rami expanding, and rising lower abdominal mass as the pregnancy progress have all been found to be reliable criteria for prenatal diagnosis of bladder exstrophy. […] Only ambiguous instances are subjected to fetal magnetic resonance imaging (MRI) and color Doppler ultrasonography. Although prenatal intervention is not required, early detection allows for delivery in a pediatric center equipped to handle this difficult deformity and thorough family counseling. […] In bladder exstrophy, treatment aims to close the bladder and abdominal defect while maintaining renal and sexual function. A pelvic osteotomy is always recommended in cases of failed exstrophy repair or cloacal exstrophy. Cloacal exstrophy is more difficult to treat surgically, and it usually necessitates neurosurgery, gastrointestinal, and urological treatments. […] However, urinary continence in these children is usually achieved with bladder augmentation and intermittent catheterization.

• #7 Bladder Exstrophy

  https://www.isuog.org/clinical-resources/patient-information-series/patient-information-pregnancy-conditions/abdomen/bladder-exstrophy.html

  Bladder exstrophy is often diagnosed by prenatal ultrasound, usually around 20 weeks of pregnancy at the time of the anatomy ultrasound. In fetuses with bladder exstrophy, the bladder is not seen on ultrasound while the kidneys appear normal. […] If your doctor is concerned your baby may have bladder exstrophy based on your ultrasound, you will likely be offered additional testing to help confirm the diagnosis and your babys sex. […] Tests that may be offered include: A detailed ultrasound examination: This is to carefully look at your baby for any other ultrasound findings or abnormalities. […] A MRI may be suggested to evaluate your babys bladder, bowel, spine, and genitalia for abnormalities.

• #8 Current management of classic bladder exstrophy in the modern era | African Journal of Urology | Full Text

  https://afju.springeropen.com/articles/10.1186/s12301-023-00356-4

  Classic bladder exstrophy is a complex, multi-system congenital malformation affecting formation of the genitourinary system, pelvis, and abdominal wall. […] Modern advancements in the diagnosis and management of this disorder have resulted in low mortality rates and shifted clinical focus toward optimizing quality of life. […] This is an updated overview of the pathology, diagnosis, and management of this rare disorder. […] The hallmark findings for prenatal diagnosis include: (1) absence of bladder filling, (2) a low-set umbilicus, (3) widening pubic ramus, (4) diminutive genitalia, and (5) lower abdominal mass. […] The diagnosis is often missed or misdiagnosed as omphalocele or gastroschisis. […] If the infant possesses a large, elastic bladder template, free of any polyps, and under the care of an experienced exstrophy team, then, a newborn closure may be considered.

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

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

  Exstrophy of the bladder can usually be diagnosed by fetal ultrasound before an infant is born. Bladder exstrophy is suspected when ultrasound shows that the baby’s bladder is not filling and emptying normally. […] Fetal imaging experts will look for several other indicators to confirm the diagnosis, including a low umbilical cord with an abdominal bulge below the cord insertion (representing the opened bladder halves, or bladder plate) and unclear male or female genitalia. Bladder exstrophy is not usually associated with other ultrasound findings or chromosomal or genetic syndromes. However, for gender identification, an amniocentesis may be recommended. […] If bladder exstrophy is not prenatally diagnosed, the bladder defect is easily visible after birth.

• #10 Bladder Exstrophy | Texas Children’s

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

  Bladder exstrophy may be detected during a routine prenatal ultrasound if the fetal bladder is not visible or is not functioning normally. Bladder exstrophy can be diagnosed immediately at birth since the bladder is visible outside the body. Prenatal diagnosis of bladder exstrophy should be considered any time the bladder is not visible in the ultrasound, or if the fetus shows any of the following signs: […] If bladder exstrophy is not detected during pregnancy, the condition is diagnosed at birth when the bladder is visible on the outside of the child’s body. […] If bladder exstrophy is suspected or diagnosed during pregnancy, you may be referred to a fetal center for further evaluation and specialized care, to ensure proper treatment planning. […] A diagnosis during pregnancy enables your family and your healthcare team to plan ahead for the specialized treatment and surgical expertise your baby will need at birth.

• #11 Bladder Exstrophy: Causes, Symptoms & Treatment

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

  In a baby born with bladder exstrophy, the bladder typically sticks through the abdominal wall at birth. Healthcare providers may use imaging tests such as an MRI (magnetic resonance imaging) or X-ray to confirm bladder exstrophy and check for other problems. […] Providers may spot bladder exstrophy during an ultrasound or fetal MRI before birth. Signs that your baby may have bladder exstrophy include: A bladder that doesnt empty as expected. Separated pubic bones. Smaller-than-expected genitalia. An umbilical cord that connects lower on the belly than expected. […] Babies who have bladder exstrophy at birth need surgery. […] Surgery is the only way to treat bladder exstrophy. The type and number of surgeries your baby needs will depend on the severity of their symptoms. Your babys surgeon will explain the best approach for your babys needs. As your child grows, they may need additional surgeries so they can hold their pee until its time to use the bathroom.

• #12 Bladder exstrophy | Health Library | Memorial Health System

  https://www.mhsystem.org/health-library/con-20339069/

  Bladder exstrophy may be spotted on a routine ultrasound during pregnancy. […] Diagnosis involves the steps a healthcare professional takes to find bladder exstrophy. But the condition often is found by chance during a routine pregnancy ultrasound. Other imaging tests such as MRI can help confirm the finding. Signs of bladder exstrophy seen during imaging tests include: A bladder that doesnt 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. […] Sometimes the condition cant be seen until after the baby is born. In a newborn, healthcare professionals look for: 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.

• #13 Bladder Exstrophy | Nationwide Children’s Hospital

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

  Bladder exstrophy may be diagnosed during pregnancy. Sometimes it is found during a routine ultrasound when the bladder may not be visible. In those cases, the care team will follow up with MRI (magnetic resonance imaging) for more detailed images of the bladder. […] Around 16 to 20 weeks into pregnancy, theres a routine ultrasound to see if everything is growing correctly. During this scan, doctors look for signs of exstrophy. Signs might include: […] If the doctors suspect exstrophy, more scans happen. The care team can work with the family to plan for the babys delivery and connect them with other families dealing with exstrophy. […] About half of cases are not diagnosed until after birth.

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

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

  Diagnosis involves the steps a healthcare professional takes to find bladder exstrophy. But the condition often is found by chance during a routine pregnancy ultrasound. Other imaging tests such as MRI can help confirm the finding. Signs of bladder exstrophy seen during imaging tests include: […] Sometimes the condition can’t be seen until after the baby is born. In a newborn, healthcare professionals look for: […] Mayo Clinic has a state-of-the-art fetal care center that helps in the diagnosis and prenatal management of babies with urology conditions, including BEEC. Mayo Clinic’s fetal care center has access to some of the most advanced fetal imaging, including high-resolution ultrasound and fetal MRI. Parents of babies with BEEC can meet the entire care team before their children are born.

• #15 Bladder Exstrophy | Boston Children’s Hospital

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

  Bladder exstrophy may be diagnosed during a routine fetal ultrasound or other indicated imaging techniques such as magnetic resonance imaging (MRI). Often it is diagnosed immediately after birth during initial physical examination. […] Prenatal care is an important aspect of health for every expectant mother and fetus. The prenatal diagnosis of bladder exstrophy allows time for learning about the condition and preparing for the anticipated care.

• #16 Bladder Exstrophy

  https://www.isuog.org/clinical-resources/patient-information-series/patient-information-pregnancy-conditions/abdomen/bladder-exstrophy.html

  Bladder exstrophy is often diagnosed by prenatal ultrasound, usually around 20 weeks of pregnancy at the time of the anatomy ultrasound. In fetuses with bladder exstrophy, the bladder is not seen on ultrasound while the kidneys appear normal. […] If your doctor is concerned your baby may have bladder exstrophy based on your ultrasound, you will likely be offered additional testing to help confirm the diagnosis and your babys sex. […] Tests that may be offered include: A detailed ultrasound examination: This is to carefully look at your baby for any other ultrasound findings or abnormalities. […] A MRI may be suggested to evaluate your babys bladder, bowel, spine, and genitalia for abnormalities.

• #17 📃 Bladder exstrophy

  https://thefetus.net/content/bladder-exstrophy-2

  Exstrophy of the urinary bladder is a very rare congenital deformity in which the anterior bladder wall is absent and the posterior wall of the bladder is exposed externally into the amniotic cavity. […] During the prenatal diagnosis we have to differentiate the bladder exstrophy from the other anterior abdominal wall defects, such as gastroschisis, omphalocele and cloacal exstrophy. […] Color Doppler is also an useful tool in diagnostic of bladder exstrophy. […] Ultrasound findings […] Absence of the normally filled urinary bladder […] Color Doppler shows umbilical arteries alongside the abdominal wall mass. […] We scanned the patient at 24 weeks and diagnosed a bladder exstrophy. […] Findings of the fetus confirmed our diagnosis.

• #18 Azthena logo with the word Azthena

  https://www.news-medical.net/health/What-is-Bladder-Exstrophy-Epispadias-Cloacal-Exstrophy-Complex.aspx

  Bladder exstrophy, on the other hand, may usually be detected between the 15th and 32nd weeks of pregnancy with high-resolution real-time ultrasonography, even during regular obstetric treatment. The absence of a fluid-filled bladder, a low-set umbilicus, tiny genitalia, pubic rami expanding, and rising lower abdominal mass as the pregnancy progress have all been found to be reliable criteria for prenatal diagnosis of bladder exstrophy. […] Only ambiguous instances are subjected to fetal magnetic resonance imaging (MRI) and color Doppler ultrasonography. Although prenatal intervention is not required, early detection allows for delivery in a pediatric center equipped to handle this difficult deformity and thorough family counseling. […] In bladder exstrophy, treatment aims to close the bladder and abdominal defect while maintaining renal and sexual function. A pelvic osteotomy is always recommended in cases of failed exstrophy repair or cloacal exstrophy. Cloacal exstrophy is more difficult to treat surgically, and it usually necessitates neurosurgery, gastrointestinal, and urological treatments. […] However, urinary continence in these children is usually achieved with bladder augmentation and intermittent catheterization.

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

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

  Exstrophy of the bladder can usually be diagnosed by fetal ultrasound before an infant is born. Bladder exstrophy is suspected when ultrasound shows that the baby’s bladder is not filling and emptying normally. […] Fetal imaging experts will look for several other indicators to confirm the diagnosis, including a low umbilical cord with an abdominal bulge below the cord insertion (representing the opened bladder halves, or bladder plate) and unclear male or female genitalia. Bladder exstrophy is not usually associated with other ultrasound findings or chromosomal or genetic syndromes. However, for gender identification, an amniocentesis may be recommended. […] If bladder exstrophy is not prenatally diagnosed, the bladder defect is easily visible after birth.

• #20 What Is Bladder Exstrophy?

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

  How Is Bladder Exstrophy Diagnosed? […] Bladder exstrophy is diagnosed during ultrasound of a pregnant woman. The condition often goes unnoticed since the bladder is hard to see. This is the reason why many babies are born with the condition. A specialist usually confirms the sex determination of the newborn baby. […] Amniocentesis- This can be performed for gender identification.

• #21 Bladder Exstrophy | Rady Children’s Hospital

  https://www.rchsd.org/programs-services/urology/conditions-treated/bladder-exstrophy/

  Bladder exstrophy is identified immediately after birth as the inside of the bladder is opened up on the lower part of the abdomen. […] The diagnosis is made after birth by the appearance of the bladder sitting on the lower abdomen and continuously draining urine out. The penis may appear short and testis may not be located in the scrotum. In girls, the labia may be widely separated.

• #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 diagnosis of bladder exstrophy is clinical and does not require any ancillary investigations. […] 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. […] The diagnosis of exstrophy bladder is clinical and does not require any other additional investigations.

• #23 Bladder Exstrophy – MD Searchlight

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

  Bladder exstrophy is a serious birth defect that impacts various parts of the body, including the lower part of the body wall, urinary tract, genitals, pelvis, spine, and anus. […] Doctors diagnose this condition based on physical examination and dont generally need additional tests. […] The diagnosis of bladder exstrophy (a birth defect where the bladder is turned inside out and protrudes through the abdominal wall) is usually based on clinical observations, meaning it can be identified by simply observing the physical signs and symptoms. It doesnt typically require additional special testing. […] Despite the continuous leakage of urine that is common in bladder exstrophy, damage to the upper urinary tract (which includes the kidneys and ureters) is rare before the closure of the bladder plate. […] Identifying bladder exstrophy and differentiating it from these conditions relies on a clinical examination without the need for additional tests.

• #24 Bladder Exstrophy | Children’s Hospital Colorado

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

  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: 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. […] Likely, this diagnosis will not affect your pregnancy, but delivery at the Colorado Fetal Care Center may be recommended.

• #25 Bladder Exstrophy – Fetal Health Foundation

  https://www.fetalhealthfoundation.org/fetal-syndromes/bladder-exstrophy/

  Bladder exstrophy is due to failure of the caudal aspect of the abdominal wall to form. […] 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: Part of the bladder extends out beneath the umbilical cord; In male babies, the penis is short and wide with the tip of the penis split in the middle; In females, the mons pubis, clitoris and labia are separated, and the opening of the vagina or anus may be pushed more towards the abdomen.

• #26 Bladder exstrophy | Health Library | Memorial Health System

  https://www.mhsystem.org/health-library/con-20339069/

  Bladder exstrophy may be spotted on a routine ultrasound during pregnancy. […] Diagnosis involves the steps a healthcare professional takes to find bladder exstrophy. But the condition often is found by chance during a routine pregnancy ultrasound. Other imaging tests such as MRI can help confirm the finding. Signs of bladder exstrophy seen during imaging tests include: A bladder that doesnt 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. […] Sometimes the condition cant be seen until after the baby is born. In a newborn, healthcare professionals look for: 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.

• #27 Be Informed About Bladder Exstrophy – CompactCath

  https://www.compactcath.com/blog/bladder-exstrophy/

  Bladder Exstrophy can typically be diagnosed before birth through a routine sonogram or MRI. If the condition is not detected then, it can be recognized after birth by the following characteristics: […] Bladder is inside-out and exposed to air […] Bladder doesnt form […] Abnormal shape and positioning of genitalia […] Vesicoureteral reflux: urine flows the opposite way (towards the kidneys) […] Epispadias: an underdeveloped urethra, the tube that excretes urine from the body […] Diastasis: a large gap between the sides of the rectus abdominis muscle […] Misplaced belly button […] Umbilical hernia: an unusual bulge at the belly button.

• #28 What to Expect During Care for Bladder Exstrophy | Children’s Hospital of Philadelphia

  https://www.chop.edu/centers-programs/bladder-exstrophy-program/your-childs-experience

  Expectant families that come to our hospital for an evaluation after receiving a prenatal diagnosis of a suspected bladder exstrophy/epispadias/cloacal exstrophy will have a series of outpatient appointments that will include: […] Antenatal ultrasound imaging and a fetal MRI with the team in our Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment to confirm and clarify specific details around the diagnosis […] Imaging will include renal ultrasounds and pelvic X-rays. Prior to surgery, our team will obtain a specialized, low-dose CT scan to assess pubic diastasis and potential hip issues. […] The complete primary repair of bladder exstrophy (CPRE) procedure will be performed between 4 weeks and 16 weeks of age. […] After surgery, your child will be in a spica cast. They will stay in the hospital recovering for four to six weeks. […] Follow-up appointments and testing prescribed by your child’s attending pediatric urology physician, pediatric orthopaedic surgeon or nurse practitioner will be scheduled with one of our scheduling coordinators.

• #29 Bladder exstrophy | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/bladder-exstrophy?lang=us

  Bladder exstrophy (also known as ectopia vesicae) refers to a herniation of the urinary bladder through an anterior abdominal wall defect. The severity of these defects is widely variable. […] Imaging findings include a soft-tissue mass extending from a large infra-umbilical anterior wall defect which may be close to the umbilical arterial exits. The absence of a normal urinary bladder and a low-lying umbilical cord insertion may also indicate the diagnosis. […] Failure of the pubic bones to meet in the midline (widened pubic symphysis). There will be shortening of the pubic rami and acetabular retroversion. This appearance on AP plain radiograph of the pelvis has been likened to a manta ray swimming towards you (manta ray sign).

• #30 Orphanet: Classic bladder exstrophy

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

  Classic bladder exstrophy is a subtype of exstrophy-epispadias complex (EEC) characterized by an open bladder plate, epispadias and an anterior defect of the pelvis, pelvic floor and the abdominal wall. […] Diagnosis at birth relies on clinical examination. Renal, cardial and hip ultrasounds, as well as plain X-rays in cases of wide symphysis diastasis (7 cm), are advisable. Follow-up may include urogenital ultrasound, pelvic MRI, voiding cystography, and urodynamics to evaluate renal, sphincter and detrusor function. […] The clinical picture of bladder exstrophy is unmistakable, but several types of exstrophy exist, with variable pictures of the open bladder such as covered, partial covered or duplicated exstrophic bladders. […] The classic finding is repeated non-visualizations of a normally filled fetal bladder. Other findings include a lower abdominal bulge, small penis, anterior scrotal displacement, low umbilical insertion and abnormal widening of the iliac crests.

• #31 📃 Bladder exstrophy

  https://thefetus.net/content/bladder-exstrophy-2

  Exstrophy of the urinary bladder is a very rare congenital deformity in which the anterior bladder wall is absent and the posterior wall of the bladder is exposed externally into the amniotic cavity. […] During the prenatal diagnosis we have to differentiate the bladder exstrophy from the other anterior abdominal wall defects, such as gastroschisis, omphalocele and cloacal exstrophy. […] Color Doppler is also an useful tool in diagnostic of bladder exstrophy. […] Ultrasound findings […] Absence of the normally filled urinary bladder […] Color Doppler shows umbilical arteries alongside the abdominal wall mass. […] We scanned the patient at 24 weeks and diagnosed a bladder exstrophy. […] Findings of the fetus confirmed our diagnosis.

• #32 Bladder Exstrophy – MD Searchlight

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

  Bladder exstrophy is a serious birth defect that impacts various parts of the body, including the lower part of the body wall, urinary tract, genitals, pelvis, spine, and anus. […] Doctors diagnose this condition based on physical examination and dont generally need additional tests. […] The diagnosis of bladder exstrophy (a birth defect where the bladder is turned inside out and protrudes through the abdominal wall) is usually based on clinical observations, meaning it can be identified by simply observing the physical signs and symptoms. It doesnt typically require additional special testing. […] Despite the continuous leakage of urine that is common in bladder exstrophy, damage to the upper urinary tract (which includes the kidneys and ureters) is rare before the closure of the bladder plate. […] Identifying bladder exstrophy and differentiating it from these conditions relies on a clinical examination without the need for additional tests.

• #33 Optimizing Prenatal Diagnosis of Abdominal Wall Defects — Classic Bladder Exstrophy and Its Imitators – BroadcastMed

  https://www.broadcastmed.com/urology/5225/videos/optimizing-prenatal-diagnosis-of-abdominal-wall-defects-classic-bladder-exstrophy-and-its-imitators

  Classic bladder exstrophy is a rare urologic disorder that develops between the sixth and eighth week of gestation in which the bladder and urethra are exposed. […] There are established guidelines for ultrasound, but this condition is diagnosed prenatally 46% to 47% of the time. […] The sooner this diagnosis was made prenatally, the more likely that these patients were closed at ABC Centers of excellence for the Association of Bladder Troy. […] When we look at prenatal imaging, historically, the absence of the bladder or abdominal wall defect was used for a classic bladder atrophy, but this is easy to be confused with other things such as gastroschisis or Phalle. […] We found that those that saw a specialist prenatally were much more likely to be diagnosed.

• #34

  https://link.springer.com/article/10.1007/s00404-019-05193-x

  Isolated classic bladder exstrophy (CBE) is the most common variant of the bladder-exstrophyepispadias complex (BEEC). […] We report on a series of 12 cases with CBE diagnosed prenatally and illustrate the spectrum of prenatal ultrasound findings with comparison to prior published reports on this entity. […] Median diagnosis was made with ultrasound in 24+5 (IQR25,75: 21+2, 29+0) weeks of gestation. […] All fetuses presented with the pathognomonic findings non-visualization of the fetal bladder and protruding abdominal mass below the umbilical cord insertion. […] Isolated CBE is an extremely rare prenatal sonographic finding. […] Prenatal diagnostics should exclude additional malformations within the spectrum of cloacal malformations.

• #35 Bladder Exstrophy | Boston Children’s Hospital

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

  Bladder exstrophy may be diagnosed during a routine fetal ultrasound or other indicated imaging techniques such as magnetic resonance imaging (MRI). Often it is diagnosed immediately after birth during initial physical examination. […] Prenatal care is an important aspect of health for every expectant mother and fetus. The prenatal diagnosis of bladder exstrophy allows time for learning about the condition and preparing for the anticipated care.

• #36 Bladder Exstrophy | Texas Children’s

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

  Bladder exstrophy may be detected during a routine prenatal ultrasound if the fetal bladder is not visible or is not functioning normally. Bladder exstrophy can be diagnosed immediately at birth since the bladder is visible outside the body. Prenatal diagnosis of bladder exstrophy should be considered any time the bladder is not visible in the ultrasound, or if the fetus shows any of the following signs: […] If bladder exstrophy is not detected during pregnancy, the condition is diagnosed at birth when the bladder is visible on the outside of the child’s body. […] If bladder exstrophy is suspected or diagnosed during pregnancy, you may be referred to a fetal center for further evaluation and specialized care, to ensure proper treatment planning. […] A diagnosis during pregnancy enables your family and your healthcare team to plan ahead for the specialized treatment and surgical expertise your baby will need at birth.

• #37 Bladder Exstrophy | Texas Children’s

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

  Bladder exstrophy may be detected during a routine prenatal ultrasound if the fetal bladder is not visible or is not functioning normally. […] If bladder exstrophy is not detected during pregnancy, the condition is diagnosed at birth when the bladder is visible on the outside of the child’s body. […] If bladder exstrophy is suspected or diagnosed during pregnancy, you may be referred to a fetal center for further evaluation and specialized care, to ensure proper treatment planning. […] Additional testing may include a high-resolution anatomy ultrasound, an ultra-fast MRI and a fetal echocardiogram of your baby’s heart, conducted by experts in the evaluation of fetal abnormalities. […] A diagnosis during pregnancy enables your family and your healthcare team to plan ahead for the specialized treatment and surgical expertise your baby will need at birth.

• #38 4 Things to Know About Bladder Exstrophy at Nationwide Children’s Hospital – Pediatrics Nationwide

  https://pediatricsnationwide.org/2024/11/01/4-things-to-know-about-bladder-exstrophy-at-nationwide-childrens-hospital/

  Bladder exstrophy care begins at diagnosis – even if that’s before birth. […] According to Drs. Cuckow and Jayanthi, about 50% of bladder exstrophy patients are diagnosed before birth. […] Even if bladder exstrophy isn’t diagnosed until after birth, it’s important for families and providers to know that emergency transfer is not needed, though an early visit with the bladder exstrophy team will support and educate new parents. […] The strategic framework of the Bladder Exstrophy Program enables personalized medicine and the ability to meet each patient and family where they are.

• #39 Prenatal Diagnosis of Bladder Exstrophy: Have We Shifted the Diagnostic Curve? – American Urological Association

  https://auanews.net/issues/articles/2024/november-extra-2024/prenatal-diagnosis-of-bladder-exstrophy-have-we-shifted-the-diagnostic-curve

  Two recent studies reported remarkably similar rates of prenatal diagnosis for classic bladder exstrophy: 47% (73/155) and 48% (134/280). […] These diagnostic rates are surprisingly low considering the high test sensitivity of the prenatal sonographic features of bladder exstrophy. […] A prenatal diagnosis affords families sufficient time to find experienced centers, generate a care plan, and coordinate the logistics involving postnatal care (ie, financial, travel, lodging). […] A notable finding from both US studies was an improved diagnosis rate over a 20-year period. […] The improved prenatal diagnosis rate may be secondary to a significant increased utilization trend of repeat prenatal ultrasound within the US and perhaps evolving ultrasound technology. […] However, a persistently empty/absent bladder within the pelvis after 15 weeks is considered abnormal, and pediatric urologists have a responsibility to work with referring maternal fetal medicine providers and their radiology team to spread awareness about the importance of rescanning fetuses in which the bladder is found to be empty.

• #40 Prenatal Diagnosis of Bladder Exstrophy: Have We Shifted the Diagnostic Curve? – American Urological Association

  https://www.auanews.net/issues/articles/2024/november-extra-2024/prenatal-diagnosis-of-bladder-exstrophy-have-we-shifted-the-diagnostic-curve

  Two recent studies reported remarkably similar rates of prenatal diagnosis for classic bladder exstrophy: 47% (73/155) and 48% (134/280). […] These diagnostic rates are surprisingly low considering the high test sensitivity of the prenatal sonographic features of bladder exstrophy. […] A prenatal diagnosis affords families sufficient time to find experienced centers, generate a care plan, and coordinate the logistics involving postnatal care (ie, financial, travel, lodging). […] A notable finding from both US studies was an improved diagnosis rate over a 20-year period. […] We hope that the increasing utilization of prenatal ultrasonography and our effort to improve awareness of this condition among radiologists and ultrasound technicians will build upon the current rising trend of prenatal bladder exstrophy diagnosis, thus allowing caregivers to be optimally prepared for the birth and postnatal care of the affected child.

• #41 Bladder Exstrophy Diagnosis & Treatment| UPMC

  https://www.upmc.com/services/urology/conditions/bladder-exstrophy

  Bladder exstrophy can sometimes be diagnosed with a prenatal ultrasound, but the diagnosis is usually made at the time of birth. […] Regardless of the stage at which the diagnosis is made, it is important that the child be referred to a pediatric urologist with experience in treating and managing this rare condition.

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

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

  Diagnosis involves the steps a healthcare professional takes to find bladder exstrophy. But the condition often is found by chance during a routine pregnancy ultrasound. Other imaging tests such as MRI can help confirm the finding. Signs of bladder exstrophy seen during imaging tests include: […] Sometimes the condition can’t be seen until after the baby is born. In a newborn, healthcare professionals look for: […] Mayo Clinic has a state-of-the-art fetal care center that helps in the diagnosis and prenatal management of babies with urology conditions, including BEEC. Mayo Clinic’s fetal care center has access to some of the most advanced fetal imaging, including high-resolution ultrasound and fetal MRI. Parents of babies with BEEC can meet the entire care team before their children are born.

• #43 Bladder Exstrophy and Epispadias | Riley Children’s Health

  https://www.rileychildrens.org/health-info/bladder-exstrophy

  Bladder exstrophy is sometimes diagnosed during pregnancy by prenatal ultrasound and magnetic resonance imaging (MRI), but occasionally it is not noticed until birth. […] When it is detected prenatally, doctors at the Fetal Center at Riley at IU Health carefully plan a baby’s birth to give each newborn immediate care and evaluation. Children have access to an experienced, multidisciplinary team of pediatric surgeons, urologists and fetal specialists that are prepared to treat this condition and provide lifelong care.

• #44 Bladder Exstrophy Program | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/specialties/urology/bladder-exstrophy-program

  Bladder exstrophy is a rare and complex condition, but it is not an emergency. […] Our Bladder Exstrophy Program offers a two-step treatment approach for newborns that is truly unique in the U.S. The first surgery to close the bladder takes place when your child is a few weeks to a few months old. […] Bladder exstrophy is complicated, and there are several different routes of treatment available. […] We make sure you understand the diagnosis all the options available for your child’s care. […] The Bladder Exstrophy Program at Nationwide Children’s treats all types of bladder exstrophy, including: […] Nationwide Children’s is the only hospital in the United States to offer the Kelly procedure, which is a soft tissue reconstruction approach to treating bladder exstrophy. […] When it comes to treating bladder exstrophy and epispadias in children, Nationwide Children’s is the only hospital in the United States that is able to offer the Kelly procedure.

• #45 Bladder Exstrophy | Pediatric Urologists | Duke Health

  https://www.dukehealth.org/pediatric-treatments/pediatric-urology/bladder-exstrophy

  Bladder exstrophy may be identified before birth during a routine pregnancy ultrasound, and the diagnosis is confirmed after birth. […] Once your baby is born, our pediatric urologists examine your newborn and assess your baby’s needs. They use ultrasound to evaluate the kidneys and look for spine problems that can occur with bladder exstrophy. The purpose of the exam is to determine a surgical plan that offers your child the best-possible results. […] All children with bladder exstrophy need lifelong follow-up care to prevent bladder-control problems, urinary tract infections (UTIs), or kidney damage that can be caused by the condition. They may need additional surgeries as well as medical care related to sexual or fertility issues. Our experts remain steadfast in their care for your child into adulthood.

• #46 What to Expect During Care for Bladder Exstrophy | Children’s Hospital of Philadelphia

  https://www.chop.edu/centers-programs/bladder-exstrophy-program/your-childs-experience

  Expectant families that come to our hospital for an evaluation after receiving a prenatal diagnosis of a suspected bladder exstrophy/epispadias/cloacal exstrophy will have a series of outpatient appointments that will include: […] Antenatal ultrasound imaging and a fetal MRI with the team in our Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment to confirm and clarify specific details around the diagnosis […] Imaging will include renal ultrasounds and pelvic X-rays. Prior to surgery, our team will obtain a specialized, low-dose CT scan to assess pubic diastasis and potential hip issues. […] The complete primary repair of bladder exstrophy (CPRE) procedure will be performed between 4 weeks and 16 weeks of age. […] After surgery, your child will be in a spica cast. They will stay in the hospital recovering for four to six weeks. […] Follow-up appointments and testing prescribed by your child’s attending pediatric urology physician, pediatric orthopaedic surgeon or nurse practitioner will be scheduled with one of our scheduling coordinators.

• #47 Current management of classic bladder exstrophy in the modern era | African Journal of Urology | Full Text

  https://afju.springeropen.com/articles/10.1186/s12301-023-00356-4

  A small, fibrotic bladder or one with hamartomatous polyps is unsuitable for newborn closure and further assessment under anesthesia by an experienced exstrophy surgeon should be made. […] The paramount goal of surgical management of classic bladder exstrophy is a successful primary closure as it is associated with decreased overall costs, decreased inflammation and fibrosis of the bladder, improved bladder growth, and decreased need for urinary diversion. […] Since the earliest description of staged surgical reconstruction of exstrophy by Sweetser et al. in the 1950s, debate has persisted regarding the ideal surgical management, timing, and technique of exstrophy closure. […] Starting in the 1990s, interest in combined, all-inclusive repairs of exstrophy were rekindled for older children with failed primary closures and eventually in neonatal primary closures.

• #48 Orphanet: Classic bladder exstrophy

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

  Classic bladder exstrophy is a subtype of exstrophy-epispadias complex (EEC) characterized by an open bladder plate, epispadias and an anterior defect of the pelvis, pelvic floor and the abdominal wall. […] Diagnosis at birth relies on clinical examination. Renal, cardial and hip ultrasounds, as well as plain X-rays in cases of wide symphysis diastasis (7 cm), are advisable. Follow-up may include urogenital ultrasound, pelvic MRI, voiding cystography, and urodynamics to evaluate renal, sphincter and detrusor function. […] The clinical picture of bladder exstrophy is unmistakable, but several types of exstrophy exist, with variable pictures of the open bladder such as covered, partial covered or duplicated exstrophic bladders. […] The classic finding is repeated non-visualizations of a normally filled fetal bladder. Other findings include a lower abdominal bulge, small penis, anterior scrotal displacement, low umbilical insertion and abnormal widening of the iliac crests.

• #49 Current management of classic bladder exstrophy in the modern era | African Journal of Urology | Full Text

  https://afju.springeropen.com/articles/10.1186/s12301-023-00356-4

  Classic bladder exstrophy is a complex, multi-system congenital malformation affecting formation of the genitourinary system, pelvis, and abdominal wall. […] Modern advancements in the diagnosis and management of this disorder have resulted in low mortality rates and shifted clinical focus toward optimizing quality of life. […] This is an updated overview of the pathology, diagnosis, and management of this rare disorder. […] The hallmark findings for prenatal diagnosis include: (1) absence of bladder filling, (2) a low-set umbilicus, (3) widening pubic ramus, (4) diminutive genitalia, and (5) lower abdominal mass. […] The diagnosis is often missed or misdiagnosed as omphalocele or gastroschisis. […] If the infant possesses a large, elastic bladder template, free of any polyps, and under the care of an experienced exstrophy team, then, a newborn closure may be considered.

• #50 Orphanet: Classic bladder exstrophy

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

  Management primarily focuses on surgical techniques to secure bladder, abdominal wall and pelvic closure, achieve urinary continence while preserving renal function, and ensure adequate cosmetic and functional genital reconstruction. Several methods for bladder reconstruction and epispadias repair (either staged or one-stage, during the newborn period or later) exist, with no clearly superior approach. […] Lifelong care from a multidisciplinary team of experts is crucial to improve physical, psychosocial and psychosexual outcome, and enhance quality of life (QoL).

• #51 Bladder Exstrophy | Pediatric Urologists | Duke Health

  https://www.dukehealth.org/pediatric-treatments/pediatric-urology/bladder-exstrophy

  Bladder exstrophy may be identified before birth during a routine pregnancy ultrasound, and the diagnosis is confirmed after birth. […] Once your baby is born, our pediatric urologists examine your newborn and assess your baby’s needs. They use ultrasound to evaluate the kidneys and look for spine problems that can occur with bladder exstrophy. The purpose of the exam is to determine a surgical plan that offers your child the best-possible results. […] All children with bladder exstrophy need lifelong follow-up care to prevent bladder-control problems, urinary tract infections (UTIs), or kidney damage that can be caused by the condition. They may need additional surgeries as well as medical care related to sexual or fertility issues. Our experts remain steadfast in their care for your child into adulthood.

Zobacz więcej