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Bladder Exstrophy - Causes, Symptoms, Diagnosis, and Treatment

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Bladder exstrophy is a congenital abnormality occurring when the lower abdominal skin does not form completely. Let us understand the topic in detail.

Published At November 30, 2022
Reviewed At August 29, 2024

Education:

BDS

Professional Bio:

Dr. Antara Mukherjee completed her BDS from Kothiwal Dental College and Research Centre, Moradabad, Uttar Pradesh in 2006 and her internship in 2007. She has an experience of 16 years. During this vast range of working experience she has worked in many hospitals, dental clinics, and colleges. She has also been associated with rural dental camps and patient treatment.

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Education:

MRCS

Professional Bio:

Dr. Samer Sameer Juma Ali Altawil is an accomplished professional with more than 21 years of a successful career in preventing urologic disease with adherence to the defined surgical techniques and procedures during operations. He is highly trained in performing almost 1000 Endoscopic, Open, and Laparoscopic Surgeries, which include: ESWL, Trans-rectal Ultrasound and Guided Biopsy of Prostate, Trans-rectal Ultrasound and Guided Prostatic Abscess Aspiration, US-Guided Aspirations or Drainage and Suprapubic Catheterization, Trans-rectal Ultrasound Diagnosis, Flexible Cystoscopy Diagnosis, Flexible Cystoscopy and Biopsy, Flexible Cystoscopy and Urethral Dilatation or Catheterization, Flexible Cystoscopy and Ureteric Stent Removal, Flexible Uretero-Renoscopy and Stone Fragmentation or Extraction (RIRS), Flexible Uretero – Renoscopy Biopsy, Urethroscopy Diagnostic and Stone Fragmentation, Optical Urethrotomy, Rigid Cystoscopy Diagnostic, Rigid Cystoscopy and Stone Fragmentation, Rigid Cystoscopy and Bladder Biopsy, Rigid Cystoscopy and Cystodiathermy or Fulguration, Rigid Cystoscopy, Rigid Cystoscopy TURBT, Rigid Cystoscopy TURP/TUIP/Prostatic Abscess Drainage, Rigid Cystoscopy Bladder Neck Incision, Rigid Cystoscopy Intravesical Injections, Rigid Cystoscopy and Stent Removal, Rigid Cystoscopy Diagnostic Biopsy or Brushings. He is an energetic medical professional and top-notch consultant in assuring the delivery of quality services to all patients while keeping updates on medical regulations and advising the management team on necessary changes while completing the healthcare plan for the annual company grant, and establishing goals and criteria for the projects. He has broad experience performing Open Urological Surgery, Endoscopic Urological Surgery, Laparoscopic Surgery, Kidney Transplant Surgery, Vascular Surgery, Angioaccess for HD, Reconstructive Urological Surgery, and with Uro-oncological surgery, and Urolithiasis. He is highly skilled in diagnosing urologic problems, creating treatment plans, performing surgery, assessing patient conditions, and collaborating with other healthcare professionals. He is proficient in scientifically organizing surgical activities to facilitate the performance of various functions, with experience in teaching medical students, junior doctors, and nurses, including delivering lectures.

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Table of Contents

What Is Bladder Exstrophy?

Bladder exstrophy is a rare birth defect that occurs within the mother's body while the fetus develops. During bladder development, the skin of the abdominal wall and pelvis does not form or join completely, exposing the bladder outside the body through an opening in the lower abdominal wall. Instead of the ideal round shape, the exstrophy bladder is flat. The bladder cannot function normally or store urine, resulting in leakage.

Bladder exstrophy is a rare condition occurring in approximately one in every 50,000 births. It is more common in males than females. The disorder may also involve other organs, like the bowel, reproductive organs, and pelvic bones. Family history plays an important role in the development of bladder exstrophy. Kids born to a parent with bladder exstrophy have a one in seventy chance of having the disorder. If one child is born with this condition, then there is a one in one hundred probability of having the other child born with bladder exstrophy. Fertility treatments might play a role as well. Some studies have suggested that kids born with assisted fertility treatments are seven times more likely to have the condition.

What Causes Bladder Exstrophy?

There is no definite cause of bladder exstrophy, but this condition is thought to occur during the 11th week of pregnancy. The defect in the bladder occurs when the tissues in the lower abdominal wall develop. A temporary tissue known as the cloacal membrane covers the lower abdominal wall, which is replaced by the matured and developed abdominal muscles.

During this time, if the cloacal membrane tears before the complete formation of the abdominal muscles, it results in an exstrophy bladder.

How Does It Affect the Body?

The child’s urinary system is impacted by bladder exstrophy. Normally, when the bladder is filled with urine, nerve signals communicate with the brain. The bladder contracts and squeezes the urine into the urethra to move it out of the body. In bladder exstrophy, the cloaca fails to grow normally. The place where the reproductive, digestive, and urine systems converge is called the cloaca. Instead of using the urethra to empty their bladders, babies with bladder exstrophy do so through an orifice in their belly.

What Are the Symptoms of Bladder Exstrophy?

The signs and symptoms of bladder exstrophy depend upon the severity of the condition and the involvement of other organs.

  • Small Bladder- The development of the bladder is not proper; hence, a small bladder is formed. It cannot hold a lot of urine. Therefore, there is urine leakage or frequent urination.

  • Incomplete Formation of the Bladder Neck and Sphincter- The bladder neck is the lowermost part of the bladder. The sphincter is like a band of muscles around the neck of the bladder. They regulate the urine flow out of the bladder. The child cannot hold the urine when it is not fully formed.

  • Urine Backflow- The urine flow is from the kidneys through the ureters (tubes connecting the kidneys to the urinary bladder) into the bladder. During backflow, the urine flows from the bladder back toward the kidneys.

  • Epispadias- This is a condition in which the urethra (a tube through which the urine passes out of the body) is short and has a spit. In girls, the urethra opens between the clitoris and the labia minora (inner skin flaps around the vaginal opening). In boys, it begins on the outer surface of the penis.

  • Chordee- In this condition, the penis of the boy curves upwards.

  • Split Pubic Bone- 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.

  • Spinal Lipoma- 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.

  • Kidney Problems- A few kids might have more than two kidneys or displaced kidneys.

  • Bowel Problems- 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.

  • Umbilical Hernia- This is the displacement of the belly button or umbilicus.

What Are the Types of Bladder Exstrophy-Epispadias Complex?

A set of birth abnormalities known as the bladder exstrophy-epispadias complex (BEEC) includes bladder exstrophy as one of its conditions. Certain newborns have a single kind of BEEC, but some have more than one condition. Other forms of BEEC include epispadias and cloacal exstrophy (the genitalia, bladder, and rectum do not split apart).

How Is Bladder Exstrophy Diagnosed?

  • Fetal Ultrasound- 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.

How Is Bladder Exstrophy Treated?

Bladder exstrophy is treated with surgery. The severity of the defect determines the type of surgery.

The surgery aims to:

  • Close the bladder, the back of the urethra, and the pelvis.

  • Make the bladder hold urine till it is time to urinate without harming the kidney function.

During the early childhood years, staged reconstruction surgeries are performed-

  • The first surgery is done at birth to close the bladder and the pelvis.

  • The subsequent surgery is done after six months to rebuild the urethra and the penis.

  • When the child is around four or five years old, and the bladder has grown large enough, the surgery is performed at the neck of the bladder to achieve continence.

  • Suppose the bladder structure is good, and the penis size is also acceptable. The surgery involving the closure of the bladder and penis reconstruction can be done in a single surgery at an early age.

  • Surgeries involving both early and staged reconstruction have a good prognosis. If the bladder has good growth, then surgery can achieve continence.

  • Future surgeries are required occasionally to improve the kids' ability to pass urine.

  • Genital reconstruction surgeries are often needed to enhance the external reproductive organs.

  • Long-term management is required after completing the surgeries.

What Is the Prognosis for Infants Suffering From Bladder Exstrophy?

Children with bladder exstrophy have an excellent prognosis after the surgery. Some children may experience infections, kidney stones, and urinary incontinence. With proper treatment, children can perform their daily activities without any restrictions.

Coping Strategies and Support:

It can be incredibly distressing to have a kid with a serious and uncommon birth condition like bladder exstrophy. According to certain medical professionals, all children with BEEC should start therapy at a young age and get psychological treatment far into adulthood, along with their family.

Locating a support group of other parents who are coping with the same issue may also be beneficial. It can be beneficial to speak with people who understand what one is going through and have had comparable situations.

Conclusion:-

Bladder exstrophy occurs when the baby's urinary bladder develops inside the body. Such babies have a small bladder, split pelvic bones, or underdeveloped genitals. They need surgeries to correct the position of the bladder and close the abdominal openings. Depending upon the situation, the babies might need surgery immediately after birth and a couple more in due course of time. The kids can experience a good life and carry out all activities with prompt treatment. If left untreated, functions like urine continence and sexual functions are compromised.

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Frequently Asked Questions

How Is Bladder Exstrophy Treated?

Reconstructive surgery is used to treat newborns with bladder exstrophy to provide enough room for urine storage and to design acceptable exterior genitalia (outside sex parts), which are active both functionally and structurally.  Additionally, promote bladder control and preserve the function of the kidney to promote urination. Sometimes a child needs a tube put into their bladder to discharge pee.

Does Bladder Exstrophy Lead to Infertility?

The majority of EEC patients, both men and women have acceptable sexual functions. Although the majority of male patients have very poor fertility in comparison with female sexual health. Many individuals have irregular ejaculation and varied degrees of seminal fluid abnormalities.

Can You Manage Bladder Dysfunction?

Lifetime survival is higher in newborns. Even after being born with bladder exstrophy, an individual can still engage in normal sexual activity and become a parent. However, pregnancy is dangerous for both the mother and the unborn child, necessitating a scheduled cesarean delivery.

Which of the Following Should Be Treated First for a Newborn With Bladder Exstrophy?

At least one bladder exstrophy surgery is often performed on newborns with this illness, but most kids need numerous procedures. Closing the exposed bladder, the bottom of the urethra, and the pelvic bones are the first line of treatment, ensuring internal organs are contained within the abdomen, followed by monitoring for complications after surgery.

How Often Is Bladder Exstrophy at Birth?

About one in every 50,000 live births results in bladder exstrophy, which is more prevalent in males.  Women born with bladder exstrophy have a one-in-70 chance of becoming pregnant and having a child. The condition can affect the colon, external genitalia, pelvic bones, and other organs to variable degrees.

Can Bladder Exstrophy Be Inherited?

Each 10,000 to 50,000 live birth results in one newborn with this inherited or congenital birth abnormality. Bladder exstrophy affects one out of every 100 babies. If both parents have exstrophy, the child has a one in 70 chance of inheriting the disease, and siblings are more likely to be born with the disorder if another sibling has bladder exstrophy.

What Is Associated With a Male Child Who Was Born With Bladder Exstrophy?

The exposed bladder's inability to hold urine or perform its regular functions leads to urine leakage. The other signs are a bladder sticking out from a belly hole and undescended testicles; the testicles are not in their usual placement, and the anus is typically lower than usual.

What Are the Three Inherited Abnormalities That Comprise the Bladder Exstrophy Complexity?

Epispadias (improper development of the urethra within a tube, resulting in excretion of urine from other parts of the body), classic bladder exstrophy (defects of the abdominal wall, bladder, genitalia, pelvic bones, rectum, and anus), and cloacal exstrophy (underdevelopment and exposure of an internal organ of the abdomen) are the three congenital defects associated with the bladder exstrophy complex.

What Are the Abdominal Abnormalities?

Diaphragmatic hernia (the protrusion of an organ into the chest cavity), exomphalos (the protrusion of an organ through the navel), and gastroschisis (the protrusion of an organ through the abdominal wall) are three congenital or inherited abnormalities that affect the abdomen.

What Is a Small Bladder?

The functionally small bladder is unable to store a significant amount of pee, which results in frequent urination. The hyperactive bladder muscles or the sphincter muscles., infections like urinary tract infections (UTI), and nerve injury are the most typical reasons for frequent urination.

Is Bladder Exstrophy Treatable?

A complete primary repair of bladder exstrophy involves closure of the bladder, abdomen, and outer sex organs, along with reconstruction of the urethra. This can be done as soon as the infant is born or when it is about two to three months old.

How Do People With Bladder Exstrophy Urinate?

The exposed bladder results in urine leakage or incontinence. The severity of the problems caused by bladder exstrophy varies. Before urination, relax the bladder muscles by rinsing the perineum and leaning forward, which promotes urination. Squeezing the pelvic floor or tapping on the bladder after urination aids in bladder contraction.

How to Tell if the Child Is on the Bladder?

The uterus lies beneath the bladder. The bladder flattens or compresses as the baby grows and leaves less room for urination. The bladder and pelvic floor may experience pressure from the baby's head, which results in the desire to urinate more frequently than usual because of the added pressure. This condition recovers within a few weeks after a child's birth.

Can a Baby Cause a Bladder to Rupture?

The bladder ruptures due to the abrupt rise in abdominal pressure and weakening of the bladder during delivery or postpartum, although it is an extremely rare condition. The common symptoms are hematuria, pelvic and lower abdominal pain.
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Dr. Samer Sameer Juma Ali Altawil from iCliniq
Dr. Samer Sameer Juma Ali Altawil

Urology

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