Bladder vs. Cloacal Exstrophy

Bladder Exstrophy

Exstrophy of the bladder is an abnormality of the bladder and urethra, where the bladder is turned "inside out", not formed completely, and is open to the surface.  The urethra and genitalia are not formed completely (epispadius), and the anus and vagina may not be placed in their usual places.  Additionally, the pelvic bones are widely separated (diastasis) and the baby has a low set umbilicus.

Exstrophy includes a spectrum of urologic abnormalities that range from mild to severe. Epispadias, the milder form, is a condition where the urethra (the tube that carries urine out of the body from the bladder) opens on the top surface of the penis instead of on the tip. In classic bladder exstrophy, the bladder and related structures are open and located outside the body. The pubic bones, which are normally joined to form in the front of the pelvis, are separated. Cloacal exstrophy, the most complex and severe form of bladder exstrophy, also involves the bowel. (health – cares.net)

Q: What causes exstrophy?

A: Obviously, exstrophy is the result of a problem that occurred during the development of the embryo.  What exactly that problem was is unclear.  One theory says that something went wrong with the separating, dividing and folding of the cells during the development of the embryo.  A second theory says that the muscle and connective tissue over the bladder did not form correctly and the thin layer of skin is not able to hold the bladder and pulls apart. 

In the first days, after my daughter’s birth it was suggested that perhaps her exstrophy was a result of a twining process that went wrong.  During the first trimester of my pregnancy, I experienced spotting.  It went on for about four to five weeks.  I continually went to my obstetrician and was monitored closely.  At first, my obstetrician thought that I was miscarrying and put me on bed rest.  The bed rest helped, but it did not go away entirely.  My obstetrician could not find any reason for the spotting.  Once it was apparent that I was not miscarrying, my obstetrician wrote the spotting off as, “some women just spot during their pregnancy.”   Since that episode occurred around the time that the midsection (the areas that exstrophy affects) was forming, and since there was a history of twins in my family some medical personnel theorized that I had actually become pregnant with twins.  Around the time I was spotting, it was theorized that I lost one twin.  Instead of my body actually “getting rid” of the twin, my daughter’s developing body absorbed the twin into her own.  Because of this “disruption” during this particular time in my pregnancy medical personnel felt that the folding, dividing and separation process of cells was interrupted and altered.

This, of course, is just a theory; no one could say for sure what happened in my case or in any exstrophy birth.   One thing is clear, exstrophy is not caused by anything the mother did or did not do during pregnancy.   The feeling of guilt is common in every mother.  I went over and over what I ate, what I drank, what I did.  Remember that poor genetics Dr?   It took a while for me to accept that my daughter’s exstrophy was not a result of anything I did.  With time knowing this did help to put my mind a little more at ease.

Q: What orthopedic aspects are associated with exstrophy?

A: Each area of the body develops together during a certain time before birth.  This includes organs, boney structures, etc.  Development of one structure affects another.  Since the wall of the bladder and abdomen are opened, so are the bones of the pelvis.

The normal pelvis develops as a ring.  It is closed in the front and back and protects the lower portions of the intestinal, urinary and reproductive systems.   It also contains the muscles of the pelvic floor, which help to stop urination and provides continence. It is composed of three bones, which begin as flexible cartilage in the embryo, and gradually develops into calcified bone as the baby grows.  That is why the surgeons wanted to wrap my daughter’s pelvis and legs.  They wanted to take advantage of this window of time and hopefully bring together the pelvic bones before calcification  The two pubic bones that join in front are joined with a connective tissue to protect the bladder.

 

This is one of the affected parts in the anatomy in a child with exstrophy.  Since the bladder and the muscles of the abdominal wall are open in front, the pelvis is also open in front.   Therefore, this whole area needs to be closed either at the same time or in staged reconstructions.

Cloacal Exstrophy

Babies born with cloacal exstrophy have a larger opening in their abdominal wall and bladder, and the lower intestines are exposed.  In addition, they may have abnormalities in the back.  They may also have weakness in the feet and legs.  The pelvises in these babies are more widely open and sometimes unstable.  The hips may also be dislocated.  Luckily, my daughter did not have any of the abnormalities in the back, feet or legs.

Q: What surgical procedures are associated with Cloacal Exstrophy?

A: Cloacal exstrophy is on the most severe side of the exstrophy spectrum.  It includes abnormalities in the bladder, urethra, and genitalia, large intestine, spine, and lower extremities.  Babies also present with an “exstrophy hernia” which is a defect in the abdominal wall above the bladder.  This is called an omphalocele.

In general, during the first procedure, soon after birth, the omphalocele is closed, the intestine (the hindgut or large bowel) is removed from the two bladder halves, a colostomy is made, and the two bladder halves are sewn together.  Every effort is made to save the entire large bowel (hindgut) because this tissue is very important in fluid and electrolyte conservation.  This large bowel segment is added onto the end of the small bowel; it usually is not left with the bladder.  Sometimes, it is also possible to close the bladder and omphalocele in the first procedure.  This depends on the size of the omphalocele. 

With my daughter they were able to close the omphalocele, close the bladder, create a colostomy, but not totally remove the large bowel from the bladder.  It was still connected through a fistula.  In time, their goal was to separate this completely, but they wanted to allow time for her internal organs, as well as, her body in general to grow.

After this operation, my daughter was back in the NICU and on a respirator.  She was sedated pretty heavily the first few days as they were trying to limit her movement and allow her body to heal.  It was probably a good thing because once she was weaned from sedation she was her feisty self, pulling at any tubes she could get her hands on and trying to squirm out of the traction they placed her in.

This movement was the cause of one of her complications of the surgery.  She was so active and constantly rubbing her legs together and trying to pull them out of traction that she developed pressure sores around her knees.  They were deep sores where the skin broke down. 

The nurses and surgeons tried several things to try and “wait out” her traction period.  These things included putting padding between her knees, trying different ointments, cleaning and wrapping her sores.  None of this helped and they ended up taking her down out of traction a week early. 

It took weeks to heal those sores.  They pussed, drained and were very ugly, angry looking sores.  We needed to clean them twice a day and I remember my daughter just wailing.  That kind of cry that babies cry where they can’t even take a breath.  I imagined it hurt her very much by the way they looked and it made my heart hurt.

 Even after we came home we continued cleaning and changing the dressings on her sores.  They eventually healed but even now, over a decade later, she still has scars around her knees.  My daughter always mentions, “Many people don’t know about my exstrophy, but everyone thinks I’ve had surgery on my knees.”

Q:  What additional challenges are associated with Cloacal Exstrophy

A: Besides the obvious urinary and genital problems of cloacal exstrophy, there may be skeletal and gastrointestinal abnormalities, as well.  The skeletal problems include vertebral, tethered spinal cord, a myelocystocele (a lesion similar with those seen in spina bifida), and limb abnormalities (which may affect overall growth and/or sensation in some limbs).  The gastrointestinal problems include: inguinal hernia, ostomy care, and the possibility of short-gut syndrome which affects nutrition.

For more information go to:

Bladder Exstrophy Links

http://www.upmc.com/services/urology/conditions/pediatric/pages/bladder-exstrophy.aspx

http://www.diseasesdatabase.com/ddb33377.htm

http://www.umm.edu/ency/article/002997.htm

http://www.nlm.nih.gov/medlineplus/ency/article/002997.htm

http://www.mens-health.health-cares.net/bladder-exstrophy.php

http://www.wrongdiagnosis.com/b/bladder_exstrophy_epispadias_cloacal_exstrophy_complex/intro.htm

https://www.everettclinic.com/kbase/nord/nord195.htm

http://www.childrenshospital.org/clinicalservices/Site1697/mainpageS1697P0.html

http://www.ibis-birthdefects.org/start/epispadi.htm

http://www.health.state.mn.us/divs/fh/mcshn/bd/bladder.htm

http://urology.jhu.edu/pediatric/diseases/DEx1.php

http://www.childrens.com/specialties/template.cfm?gropid=4&pageid=171

http://www.bladderexstrophy.com/

Cloacal Exstrophy Links:

http://www.urologyhealth.org/print/index.cfm?topic=311

http://www.cigna.com/healthinfo/nord195.html

http://www.jpedsurg.org/article/s0022-3468(04)00270-2/abstract

http://www.humpath.com/cloacal-exstrophy

http://www.childrenshospital.org/az/Site2187/mainpageS2187P0.html

http://www.ncbi.nlm.nih.gov/sites/ppmc/articles/pmc1421517/

http://search.medscape.com/emedicine-search?queryText=cloacal%20exstrophy

http://www.ejbjs.org/cgi/reprint/73/4/551.pdf

http://www.medgle.com/rw/procedures/closure+for+cloacal+exstrophy

http://urology.jhu.edu/pediatric/diseases/DEx2.php

http://www.isna.org/node/564

http://www.tripdatabase.com/search?criteria=cloacal+exstrophy

Glossary of Terms

At this time I think it would be beneficial to share a glossary of common terms used in relation to exstrophy.  I will list the terms and meanings here for ease of cross-referencing.  This glossary can also be found at The Association for the Bladder Exstrophy Community - THE ABC.  The ABC is an international support group for individuals who were born with a form exstrophy and their families.  They can be found at http://www.bladderexstrophy.com/.

GLOSSARY

Agenesis
Embryologically speaking, absence or incomplete development of an organ or body part.

Anastomosis surgically sewing two structures together, whether it is two ends of the bowel, which is bowel anastomosis, or reimplanting and sewing the ureter to the bladder, which is a ureteroneocystostomy anastomosis. This term simply means sewing two structures together.

Anomalies Another name for birth defect. More properly, it is termed congenital anomaly in the case of our exstrophy patients, which implies a developmental defect that happened during fetal life.

Anterior
Anatomically located in front of another body part; for example, the anterior surface of the bladder is the front wall of the bladder.

Appliance An adhesive bag worn to collect waste

Augmentation To enhance. In terms of exstrophy patients, augmentation typically means to enhance the size of the bladder with a piece of intestine-typically, the small intestine, large intestine(colon) or in very rare cases, the stomach.

Bifid Forked or split into two parts (such as the ureter or the penis).

Bilateral Occurring in or affecting both sides of the body(ie. both the left and right kidney) .

Bladder augmentation Surgical procedure to enlarge the capacity of urine the bladder can hold. It lowers the pressure in the bladder. Most commonly, a piece of intestine (bowel) is moved, cut open, and added to the opened bladder to make a larger urine container.

Bladder closure The surgical procedure to create a hollow sphere of the exstrophy bladder plate and to place it within the abdomen. It may be the first attempt (primary bladder closure) or the second or subsequent attempt (redo bladder closure).

Bladder neck plasty Another name for bladder neck reconstruction, the muscle floor of the bladder is rolled into a tight tube to create a sphincter-like affect to help increase the strength of the bladder neck area to render the child continent of urine.

Bladder neck suspension The surgical procedure in which stitches are used to permanently sew the bladder neck to the back of the bony pelvis. This helps achieve urinary continence.

Bryant's traction A type of immobilization of the pelvis using pulleys and weights. This is used on patients with classic bladder exstrophy closures who were closed within 48-72 hours of life and without osteotomies.

Calyces The smallest part of the collecting system of the kidneys. The urine is filtered in the cortex or "meat" of the kidney and filters through tiny ducts in the calyces where it begins to be collected and passes down into the pelvis of the kidney before it passes down the ureter to the bladder.

Catheter A hollow, flexible tube inserted into a body cavity, duct, or vessel to allow the passage of fluids.

Catheterization The passage of a tube, either through the urethra into the bladder, or through a stoma in the abdominal wall into the bladder.

Caudal Anatomically more toward the tail than another part of the body.

Cephalad Anatomically more toward the head than another body part.

Chordee A bend in the penis. In the exstrophy/epispadias group, chordee is typically upward in the direction of the abdominal wall. This is part of the total birth defect in this complex. This bend is fixed at the time of epispadias repair.

Classic exstrophy The most common birth defect seen in the exstrophy/epispadias complex. It makes up to 60-70% of the patients seen in this complex. This occurs in one in 40,000 live births, with a ratio of 3:1 male to female. The bladder is open from the top of the bladder through the bladder neck and prostate area, completely through the urethra, to the tip of the penis. The pubic bones are often times widely split.

Clean intermittent catheterization (CIC)
The process of passing a clean (not sterile) catheter, emptying all the fluid, and then removing the catheter. This process is repeated once every 2-8 hours to empty urine.

Cloacal exstrophy Occurs one in 400,000 live births. This is one of the most severe birth defects that are still compatible with life. There is typically an omphalocele, which is a membrane-covered area on the abdominal wall which contains intestinal contents. The bladder is divided into two halves and in the middle of the abdominal wall between the bladder halves lies part of the intestine. In the male patient with cloacal exstrophy the penis is split in two halves, one lying on either side at the base of the bladder. The pubic bones in cloacal exstrophy are typically more widely split than in bladder exstrophy. In females with cloacal exstrophy, the clitoris is divided in two halves, again at the base of the bladder, and the pubic bones are widely split. Oftentimes in cloacal exstrophy there may be two vaginal openings, which is different than in classic bladder exstrophy.

Colostomy Surgical construction of an artificial excretory opening from the colon to the skin.

Continence(relative to exstrophy)
Urinary continence can be defined in many ways. Continence is the ability for the child to hold urine for three to four hours without leakage from the urethra. Continence can be achieved through bladder neck reconstruction and voiding through the urethra which is the preferred manner, or in other cases continence can be achieved by bladder augmentation and the establishment of a continent stoma on the abdominal wall which is catheterized every three to four hours to achieve urinary dryness.

Cystogram An x-ray of the inside of the bladder. This is typically accomplished by passing a catheter through the child's urethra and filling the bladder. Most cystograms are done to look for the presence of reflux of urine back into the kidneys, which is very common in the exstrophy patients, and to measure bladder capacity. Cystograms may be done under anesthesia with gently passing the contrast through the catheter under gravity to accurately measure the bladder capacity and look for reflux.

Cystometrogram A catheter is passed into the bladder and oftentimes a small catheter is passed into the rectum. Gel-like pads are fastened to the abdomen, which are known as electrodes. A cystometrogram is used to look at the pressure of the bladder, the size of the bladder, and whether or not the bladder muscle acts normally.

Dehiscence A wound that has split apart. A dehiscence in the exstrophy population typically means that the pubic bones and abdominal wall closure have split apart and the bladder is reexstruded onto the abdomen wall.

Detrusor muscle The actual muscle of the bladder that is involved in contraction to expel the urine from the bladder.

Diastasis (of the pubic symphysis)
The distance between the left and right pubic bones in a patient with bladder exstrophy or cloacal exstrophy. If it is a big bladder the diastasis tends to be wider, and if the bladder is small the diastasis, or separation, tends to be more narrow.

Distal Anatomically located further from a point of reference than another body part.

Diverted The urine typically does not tend to pass through the urethra. In the past, diverted simply meant that the ureters were placed into a piece of bowel and this was brought to the abdominal wall and placed into a bag. This is still done on rare occasions. The most common diversion these days is a bladder augmentation with a continent stoma placed in a hidden position on the abdomen, which does not leak and which is catheterized at regular intervals. This is known as continent urinary diversion, which is a form of diversion.

Diverticula 
A weak spot in the wall of the bladder where a bit of the lining of the bladder extrudes out through this weakness and you see a small "pocket-like" lesion on the cystogram.

Dorsal Anatomically located in front of another body part.

Dorsal chordee An upward bend in the penis when the penis is erect(rigid). In exstrophy children, this is typically part of the epispadias abnormality with a shortened urethra.

Enuresis
The involuntary or uncontrolled discharge of urine.

Epispadias Another part of the bladder exstrophy/epispadias complex. Epispadias by itself occurs in one in 112,000 live male births. Epispadias can also occur in females, occurring one in 400,000 live births.In male epispadias the urethra is open from the tip of the penis, back to under the pubic bone. In those patients which are known as complete epispadias, or penopubic epispadias, the penis is totally incontinent, just as in the exstrophy population. However, the epispadias defect sometimes is not as severe and the urethra can exit out on the shaft of the penis. In these patients there is no urinary incontinence. In the typical female epispadias patient, the clitoris is divided into two halves, just as in female exstrophy.

Fistula
A communication between an inside structure and an outside structure. The typical application of a fistula in the exstrophy/epispadias patient is a small undesired leakage between the surgically reconstructed urethra and the outside of the penis. This is known as a urethrocutaneous fistula. In addition, fistulas can occur between the bladder and the abdominal skin. This is called a vesicocutaneous fistula and is more rare than a fistula on the penis.

Genitoplasty The reconstruction of the female external genitalia in the female exstrophy patient. This typically involves bringing thee clitoral halves together which are apart, as mentioned above, bringing the urethra out as far as one can in the vagina, and reconstructing the fatty tissue over the clitoris to look like a normal mons pubis, which has hair in the adult female.

Glans The tip. The glans penis is the tip, or head, of the male penis. The glans clitoris is the tip, or head, of the clitoris in the female.

Gut (foregut,midgut and hindgut)
Terms which describe the early regions of the developing gut in the embryo. These terms are important in children with cloacal exstrophy, which involves malformation of the junction of the midgut and the hindgut.

Hernia An outpouching of an organ or other body part through a weak spot in the wall that normally contains it.

Hydronephrosis Dilation of the internal collecting system of the kidney. This can be secondary to obstruction at the level where the ureter joins the kidney, or it can be secondary to reflux. More precisely put, the appropriate term oftentimes in the exstrophy population, is hydroureteronephrosis, which typically means some swelling of the ureter from the bladder up to and including the inside of the kidney. Some hydronephrosis is normal and is not uncommon after surgery, but significant amounts of hydronephrosis can be dangerous.

Hypoplasia
Under developed. For example, a hypoplastic glans penis in the male would mean that the tip of the penis is poorly developed.

Hypospadias This is not part of the exstrophy/epispadias complex. Hypospadias is a very common birth defect in males, occurring up to eight per 1000 live births. In hypospadias, the urethra is not at the tip of the penis but is somewhere on the bottom of the penis and can be anywhere from near the anus, on the perineum, in the scrotum, at the base of the penis, on the midshaft of the penis, or even slightly off the mark on the tip of the penis.

Ileostomy The surgical construction of a stoma made of ileum(small intestine) on the skin. Typically in urological patients, a small segment of ileum is separated away from the rest of the stool stream. The ureters are attached to the internal end of the blind-ending tube of ileum. Therefore, urine can flow to the outside of the body through the ileostomy and must be collected into a bag(appliance) since this is incontinent. In the cloacal exstrophy patient, an ileostomy is a stoma made of ileum which is in continuity with the stool stream. Therefore, this ileostomy shunts stool to the appliance; the ureters are not surgically connected to this ileostomy.

Incontinence The inability to prevent leakage of urine or stool.

Inferior Situated more below than another body part.

Inguinal hernia
83% of male patients with classic bladder exstrophy have inguinal hernias. An inguinal hernia is communication of the membrane in the abdomen which carries through the groin and oftentimes into the scrotum. This is a congenital anomaly that is easily fixed by tying off the hernia sac where it exits from the abdomen into the groin.

Lateral
Situated more away from the central line of the body than another body part.

Medial
Situated more toward the central line of the body than another body part.

Mitrofanoff
Paul Mitrofanoff is a French surgeon who in 1980 first described an operation where he disconnected the appendix from the large intestine and connected one end to the skin and the other to the bladder. He specifically tunneled the appendix in the bladder so it would be continent. Therefore CIC could be performed through this channel, the patient would be dry and the patient would not have to wear a bag. The 'idea' is called the Mitrofanoff principle and can be applied in several ways with tubes other than the appendix.  Also known as a continent appendicovesicostomy.

Mucosa lining
In the exstrophy/epispadias population the bladder mucosa is the pink tissue that one sees in a newborn exstrophy that covers the detrusor muscle of the bladder, which is mentioned above.

Osteotomy
(osteo=bone; tomy=to cut) cutting of the bones. In the bladder exstrophy population, osteotomies are used to help reconstruct the pelvic bones and the pelvic floor which removes tension from the abdominal wall and bladder closure thereby aiding in the success of the exstrophy reconstruction. The three bones of the pelvic bone are the ishium, the ilium and the pubic. In exstrophy surgery, iliac and pubic osteotomies are commonly performed.

Ostomy
The surgical construction of a new 'mouth' or opening (stoma). This surgery may be done as a temporary or a permanent endeavour. Unless specified otherwise, the stoma connects an organ to the surface of the skin.

Penopubic
Describing something located at the junction of the penis and the pubic(ie. penopubic epispadias).

Posterior
Anatomically located behind another body part.

Proximal
Anatomically located closer to a point of reference than another body part (whereas distal means away from).

Pubic symphysis
The pelvis or pelvic ring is made of two halves. Anteriorly the left and right pubic bone of the pelvic ring is joined by a ligamentous band known as the pubic symphysis.

Pyelonephritis Kidney infection. This is dangerous and requires immediate treatment. It can damage the kidneys, cause scars in the kidneys, and can lead to high blood pressure later in life if the kidneys are damaged.

Rectus fascia
The main abdominal fascia which extends from the lower part of the ribs down to the pubic bones. When the pubic bones are brought into good approximation in the midline, the rectus fascia is brought back to the middle of the abdomen wall in its normal position.

Reflux
The appropriate term is vesicoureteral reflux. This is the abnormal passage of urine from the bladder, up the ureter, and into the kidney. Reflux is graded from grade I to V. Almost all children with bladder exstrophy will have low grade vesicoureteral reflux. This part of the birth defect, as the ureters come through the bladder wall in a very short tunnel and thus an appropriate "flap" valve is not present as in a normal kid, giving urine access to the ureter and kidney. Low grade reflux can be easily controlled with small daily doses of an appropriate antibiotic.

Renal
Pertaining to the kidneys

Retrograde cystoscopy The passage of a cystoscope from the urethra through the bladder neck, and into the bladder.

Retrograde ejaculation
During ejaculation, the bladder neck closes allowing the ejaculate to be expelled at the tip of the urethra. In patients with epispadias, the bladder neck may be unable to close. All or part of the ejaculate enters the bladder and is not expelled. The semen therefore will be mixed with urine and is expelled when the patient voids the next time.

Sacrum
A group of fused backbones located at the caudal end of the spine. They are triangular shaped and are located in the posterior portion of the pelvic ring. These bones are connected to the last vertebrae, the coccyx, and the hipbone on each side. The three bones of the pelvic bone are the ishium, the ilium and the pubis.

Sphincter
The ring-like muscle that maintains constriction of a body passage or orifice and that relaxes by normal physiological functioning(e.g. the sphincter controls urination and keeps us dry between urinations). In the bladder exstrophy population(those patients with bladder exstrophy, cloacal exstrophy, and complete epispadias), there is no development of the urinary sphincter and thus, even after closure of the bladder or urethra, or both, there is still dripping of urine from the urethra. A sphincter has to be constructed from bladder muscle in order for the child to be rendered dry of urine.

Spina bifida
A congenital defect in which the spinal column is opened posteriorly so that part of the meninges or spinal cord protrude. This results in varying severity of neurological loss.

Stenosis
Narrowing or stricture of a duct or passage, usually by scar tissue, such that substances have difficulty passing.

Stoma
A surgically constructed opening, especially one made in the abdominal wall to permit the passage of waste. The stoma is usually on the abdominal wall in a hidden position, but occasionally can be down in the urethra.

Stricture
Narrowing. In the exstrophy/epispadias population, a stricture can involve a narrowing of the urethra after it has been reconstructed to the tip of the penis, or can involve a narrowing of the posterior urethra and bladder neck at the time of exstrophy closure, which is more dangerous.

Superior
Situated more above than another body part.

Suprapubic tube
Also known as suprapubic catheter. A tube that passes through the abdominal wall and into the bladder, allowing drainage of urine from the bladder while healing occurs after surgery.

Symphyseal separation
If the pubic symphysis is surgically brought together, it can grow or spread apart.

Ureter
A muscular tube that brings the urine from the kidney to the bladder.

Ureterosigmoidostomy
This is an older type operation which is not typically done in the United States. In this procedure the bladder is removed and the ureters are reimplanted (moved) into the anterior wall of the sigmoid colon. These patients pass their urine along with their feces, approximately 8 to 10 times per day. This operation is not used much in modern times because there is an increased risk of cancer when there is a mixture of urine and feces in the same chamber.

Ureterostomy
Surgical construction of an artificial excretory opening from the ureter to the skin.

Urethral groove
Another name for the urethral plate, or urethra, that extends from the base of the penis to the tip.

Urethrotomy
Cutting of the urethra internally. This is done with an instrument for the treatment of a urethral stricture.

Urinary diversion
The process of altering the normal channels of urine flow. This can be by using tubes to change the course of urine or by doing one of several surgical operations which can temporarily or permanently alter the course of urine flow.

Ventral
Anatomically located behind or at the bottom of another body part. For example, the top of the penis would be known as the dorsum and the bottom side of the penis is the ventral aspect.

Vesicostomy
Surgical construction of an artificial excretory opening from the urinary bladder to the skin.


LINKS:
http://www.bladderexstrophy.com/

The First Surgery

Finally, Monday rolled around.  With all of our hospitalizations, one of the things I have grown to hate is weekends in the hospital.  In our experience, the weekend “doctors” usually consists of residents.  Not that they are bad people or Dr.’s, but when you are dealing with a rare condition to begin with, residents do not have the knowledge to advise you.  In some cases, they cannot even give you confidence that the hospital knows what it is doing.  During our journey there has been many times where I received misguided or misinformed information from residents.  There have even been episodes where I have thrown residents out of my daughter’s hospital room, more on that later.

Upon consultation with surgeons in the areas of urology, general surgery, genetics and orthopedics we found out several bits of information.  First, our daughter was indeed a girl!!!  Second, she had exstrophy (OK, we sort of knew that already), but they did not yet know how severe it was (ie. They didn’t know what her insides looked like).  And the final bit of news was that she would be having surgery later that day.

In the last couple of days, we learned that the effects of exstrophy can be “repaired”.  Not “cured,” as in make it go away completely, but “repaired” to improve the quality of life for the child.  Surgical repair of exstrophy is referred to as “staged reconstruction.”   This means that several different surgeries are required to accomplish the best possible results.  The actual number of surgeries varies from child to child and is also dependent on the severity of the exstrophy.   

The first goal of my daughter’s first surgery was to find out what her exact anatomy looked like.  Tests that had been run, up to this point, were inconclusive as to how everything fit together and looked inside. The surgeons also hoped to close the bladder, urethra and abdominal wall and to create a colostomy.

The surgeons explained to us that after surgery our daughter was going to be placed in traction.  Her legs would be placed together, wrapped and then positioned in a 90 degree angle to her body and held there with weights.  They explained that this would bring the pelvic bones together and would be done for two reasons.  One reason was to hold together the closure of the bladder and abdominal wall after surgery so that it could heal. 

The second reason was to try and correct one of the effects of exstropy, which was wide set pelvic bones.  The pelvic bones provide protection of the lower part of the bladder, urethra, and the muscles of the wall of the abdomen.  The muscles of the pelvic floor, which help to stop urination and provide continence, also are located there.  The surgeon’s explained that the opening in the pelvic bones would get bigger as my daughter grew and would not provide the protection and support needed for my daughter’s internal organs  and muscles in that area.  They explained that trying to close the pelvic bones, in this way, at this time was important because the pelvic bones were still soft.  They hoped that this attempt would prevent my daughter from having a hip osteomy later.

At about 7pm that night my daughter went in for what was supposed to be a couple hour “exploratory” surgery.  Five hours later, the two surgeons (urologist and general surgeon) emerged with good news.  They reported that my daughter had all her internal organs (with the exception of one kidney) including all her female organs.  They closed the bladder and internalized it, reconstructed a portion of the urethra and did some cosmetic work on the outside where only one tiny opening existed before.  Her abdomen cavity was closed and a colostomy was created.   They said they did a little more than expected because they found more to work with than they expected.  They did say that the bladder was still attached to the intestines through a fistula and would need to be separated later in life, but because she was so small they wanted to allow her time to grow and gain strength before the next reconstruction and further separation.

All in all it was good news.  I already knew that there would be more surgeries in the future so that was not a surprise. Everyone already knew that she had a form of bladder exstrophy and that the severity could not be determined until after this surgery.  Her diagnosis was now cloacal exstrophy, but the surgeons felt confident that, with further reconstructive surgeries, her outcome was going to be good. The surgeons prepared us for the worse and it was finally a pleasant surprise to find out that something was better than expected. 

Day 2

I spent the night in the hospital, away from my baby.  The next morning, as waves of consciousness washed over me, I thought, for those few blissful moments, that everything was alright. I was still pregnant and everything was alright.  A few moments later I opened my eyes and it all hit me like a dump truck of bricks.

I knew my baby was in Children’s Hospital of Wisconsin and, from the phone call from the hospital the night before, she had settled in well.  There was nothing for me to do in a hospital 40 miles away.  I wanted to be with my baby.  I wanted to find out what was wrong and what I could do to make her life all it could be.  They had given me the number to the NICU and I called it right away.  They informed me she was doing well, but she was an ornery little girl.  They told me she had pulled out all of her tubes, iv’s, etc.  They had to shave a portion of her head (yes, she had thick beautiful brown hair – and it was long in the back!) to insert an iv into her head.  I remember half laughing at her my daughter’s antics and half crying about the still unknown situation we faced.

My obstetrician came in later in the morning and fully understood that I wanted to be released and wanted to be with my baby.   He informed me, before signing my discharge papers, that he reviewed every ultrasound I had during my pregnancy looking for something that would indicate my baby was going to be born with exstrophy.  He said the way the pictures were taken and the way she was positioned there was just no way to see it.  That may or may not be true, but at this point I figured what happened, happened and we will learn to deal with it.

A nurse came in and gave me my discharge papers, a prescription for pain meds, and the new baby packet.  It contained the card with my daughter’s footprints on it, coupons for baby items, a pacifier, a soft baby book, and the memento birth certificate.  I looked at the memento certificate and saw that they left the “sex” of the baby blank.  The nurse saw me looking at the certificate and again indicated, “We cannot mark the sex of the baby because we don’t know.”  I thought to myself, yeah, you and everyone else in this hospital have said that.  The packet was also supposed to come with a little baby bracelet, beaded in either blue or pink beads, reading either “boy” or “girl”.  They had omitted that altogether.

When we left there were no Congratulations or good wishes that I had anticipated for the last nine months.  It was, “I’m sorry” and “Good Luck” said solemnly and with pity. It was at that point that I said to myself I will not allow my daughter’s life to be filled with “I’m sorry” and pitiful “good luck” wishes and glances.  She was beautiful and she was feisty.  With all the problems I had during my pregnancy (almost miscarrying at 9 nine weeks among other things) and now with the complications she was born with, she was still fighting and giving the NICU at Children’s Hospital hell.  If she was going to fight so was I and I decided that she was going to be all that she could be.  At that point, I was not sure what that was, how much she could do, or quite frankly, how long she would be around to do it, but she was going to be all that she was set on this earth to be.

What I found at the Children’s Hospital of Wisconsin was miraculous.  Up until that point in my life, I had never had any reason to experience the inside workings of a major medical center, especially an NICU.  The monitors, machines, beeps, buzzes, wires, tubes, incubators, warming lights and many other forms of equipment met us.   The smell was medicinal and chemical.  I’ll never forget that smell. The incubators stood in rows and some of them were covered with baby blankets and quilts, but some were open and you could see the tiniest little beings grasping on to life.  I tried not to look, because really all I wanted to see was MY baby.

She was toward the back and when I came up to her incubator I was surprised.  Not shocked as the nurse told me some of what to expect but still surprised.  She had an ng (nasogastric) tube, and the iv in the side of her head.  She had little arm boards on as she was being ornery about pulling at her tubes.  She was supposed to be covered with a blanket or quilt, but as she was to become known for, she had kicked them off.  Her exposed bladder was covered with a dressing and she had a catheter inserted.  She had a diaper fastened loosely around her waist but I think it was more cosmetic because she had no openings down below (no anal or vaginal opening) except one tiny one sitting about an inch below her exposed bladder where they had the catheter inserted.  She was awake and alert when we got there and was taking in all of her surroundings.

We were allowed to visit with her awhile and then the information barrage started.  They told us what tests they had run and what they knew upon initial examination. I got to talk to the genetics Dr. (the Dr. not the residents on duty during the weekend) though and finally someone told me that my daughter’s condition was not fatal.  She was going to make it!!!  He said that it was too early to tell the degree of exstropy she was born with as everyone was still examining x-rays and ultrasounds but that she was going to make it.  That was another time where I felt myself let out my breath when I hadn’t even realized I had been holding it.  I remember him telling me, the concern should not be whether or not she is going to have a life because she will.  The question was what quality of life she will have based on the severity of her condition.  I made him repeat it again.  I don’t really know why other than to re-enforce in me that my daughter was going to have the best quality of life that I could provide her with her current condition.  We were not going to be beat down by this. We were going to learn and grow with this and make the best we could out of it.

Did I still have those “lost” dreams of a healthy child?  Yes, they crept up here and there especially as we entered more and more surgeries but I didn’t dwell on them.  My job, as I saw it, was to arm myself with the strength, knowledge and the tools necessary to provide my daughter with the love, support, knowledge and tools in order for her to have the best quality of life she could have in spite of her condition.

The other memorable moment I remember about talking with the genetics Dr. was the question of why did this happen?  I hounded the man with everything I could think of.  I asked about the foods I ate, the medications I took, the work environment I was in, the water,  the air, the area I lived in, what I used to clean the house, the activities I participated in, the color of the sky, whatever entered my mind as to what I possibly did to cause this.  He assured me that NOTHING environmental “caused” this condition.  IT WAS NOTHING I DID.  AND THERE WAS NOTHING I COULD HAVE DONE TO PREVENT IT.

Birthday!

Welcome

Hello, if you are reading this, you may have just discovered that your child, or a child in your life, was born with bladder, cloacal or another of the various forms of exstrophy.  Well, I am writing this because I am the parent of a daughter born with cloacal exstrophy over 10 years ago.  Exstrophy is a rare condition requiring specialized medical care and expertise.  It does not go away, your child will not “grow out of it” and while it can “repaired” through staged surgeries, it will affect your child for a lifetime.

At this point, you may be looking for information about this unique condition.  “Ignorance is bliss” may or may not be comforting to you at this point but it will not carry you very far into this journey.  So get up, dust yourself off and get back in the saddle.  My aim is to arm you with some information.  As this journey continues YOU will be the source of information concerning your child and exstrophy.

The diagnosis of bladder exstrophy is frightening, in the beginning.  Many parents first learn about exstrophy soon after the birth of their child, as we did.  The news came as a surprise even though I had a difficult pregnancy.  I had a feeling through my whole pregnancy that something was wrong with my daughter, but not something as drastic as exstrophy.  I had been through several prenatal ultrasounds because of my insistent urging to my obstetrician, but everything “looked good”.  He wrote me off as a nervous new Mom – to – be.  After my daughter’s birth, I, like other parents, was catapulted into the world of not only having the normal hopes and dreams for my newborn daughter, though at that particular point in time I questioned them, but also into a maze of a rare, complex and chronic health condition.  I was disoriented.  It was one of those times in my life where everything looked different, sounded different, felt different and even smelled different with the accompanying feeling of when am I going to wake up to realize this is not real.  Later I found that to maneuver through the maze I had to learn a new language (medicalese) and start drawing a new map.

After months of reading pregnancy and baby books, counting the weeks on the calendar and comparing what my daughter might look like at any given week with the in utero drawings in the books, this was not the birth experience that I dreamt of.  My feelings and emotions were already heightened due to a difficult pregnancy, anticipation, worry, having to be induced and then finally going through labor.  Yep.  I delivered naturally and with no epidural.  With the induction, my daughter and my body decided that it was going to sluggishly go through the whole day with me only being able to dilate to 3.  Then within ½ I went from 3 to PUSH!!! 

When my daughter finally arrived the whole atmosphere of the delivery room changed.  Everything went quiet.  My daughter wasn’t crying and no one was saying anything.  I began to worry.  Were my instincts throughout the pregnancy correct?  After a few seconds my daughter let out a howl and then never stopped crying.  I let out my breath (I hadn’t even realized I had been holding it).  Now I waited for news from the Dr.  Nothing.  Wasn’t there supposed to be the … BIG ANNOUCEMENT… The congratulations it is a boy or girl?  Nothing.  So I finally asked?  Is it a girl or a boy?  Is everything alright?  A nurse came to hold my hand as the Dr. announced there is a problem with the baby.  My whole line of vision went black, and then, as the images in the room started to reappear I asked again, “Is it a girl or a boy?”  My Dr. simply said, “We don’t know.  There is an area of the abdomen where the internal organs are on the outside and the “deformity” extends down to the organs of gender and we cannot tell.”  What?  You cannot tell?  I had never heard of such a thing.

My child was born with a “deformity” as the Dr. announced.  He said it was very rare and he wasn’t sure if this is what the “deformity” was, but it was called exstropy.  I don’t remember too much more about what was said at that time.  All I remember is asking over and over again, “Is she going to be OK?”  I didn’t yet know for sure if my daughter was indeed even a girl but I had a gut feeling she was.  I remember the nurse patting my hand and telling me, “Everything is going to be OK.”  However, I wasn’t sure if she meant it or if she was just trying to keep me from becoming hysterical.  I don’t think she knew.

My daughter was over being “attended” to and she was wailing.  A testament to the feisty attitude she still holds on strong to today.  I heard the nurses say that her apgar scores were all 9’s and 10’s.  I thought to myself. 9’s and 10’s, that's good right?  From what I heard and read that was good.  She couldn’t be that bad.  My husband was over looking at her and then came to my bedside and said, “You were right, it is a girl.”  The nurse quickly reprimanded him and said, “We don’t know that.  What you are seeing is the birth defect; the absence of a penis does not indicate it is not a boy.”

They wrapped my daughter up tight and I got to hold her for a minute and then she was whisked away to the intensive care unit.  Well, what little one they had.  They informed me that she would be transported sometime that night to a bigger facility because they did not have the expertise to deal with that type of birth defect.

The Dr. continued working on me and a little while later the neonatologist came in and said that my daughter was born with bladder extrophy.  They were not sure of the extent, however, as exstrophy is a spectrum defect.  He said he tried to “feel” around and suspicion that my daughter was a girl but that genetic tests would have to be performed to be able to tell for sure.  He said that my baby was going to be sent to the Children’s Hospital of Wisconsin in Milwaukee, WI, about 40 miles away. 

I felt overwhelmed, sad, frightened and confused.  I was grieving the loss of my notion of the perfect delivery and healthy baby.  I feared what the future held for my baby, as well as, for my family.  All that was wrapped up in the guilt I felt of what did I do wrong? At this time, the professional were the experts and I was the bystander. 

Since exstrophy is a rare birth condition, there are few doctors experienced in the surgical repair of the newborn with exstrophy.  Accessing the best care for my daughter on my own, at this point, was impossible.  So I entrusted the care of my precious newborn daughter to people I didn’t even know.  It was a very difficult time to think about the types of questions that should be asked.  This was just the beginning of the many stages of my daughter’s development that would be challenging and altered because of the condition of exstrophy.

By the time I was able to get up and go to the nursery to see my daughter the whole extended family was there.  My, now ex- husband, in his panicked state called everyone and since everyone was local they all came to the hospital.  In a sense, it was nice to have them there for support and also that way I wouldn’t have to explain everything over and over again to everyone.  Everyone was already there.  However, in another sense it was overwhelming and stressful too.  I wanted time to see my daughter and be alone with her before they transported her and I had to stop and have everyone question me as to how I was feeling, what I should be feeling and how I was suppose to act.  My, now ex sister-in-law actually said to me, “Do you know what is going on?  You are acting so out of it.”  Then again that family is so overly emotional that if you are not wailing in the hallways, you are not acting “appropriately.”  To do it over again, I would not have had everyone there.

Some parents have found that by limiting phone calls and visits from family and friends in the first 24 hours they can save themselves explanations about the unknown.  Once you have solid information about your baby’s health status you can determine how you will disseminate this information.  You might want to set up an account with caringbridge.org.  This website allows families, who are experiencing health challenges, connect with family and friends, privately, to give regular updates.  This way you can control the information that goes out and don’t have to deal with well meaning but sometimes intrusive questions.

I got to go into the nursery for a few minutes before the ambulance came to transport my baby.  She was still wailing up a storm.  At first, I was afraid to even touch her.  Finally, I held out my finger and put it in her hand and started to talk to her and as soon as she heard my voice she stopped crying, grasped my finger tight, and looked me in the eyes.  She was lying in the bassinet with her diaper open and I could, for the first time, see her abdomen and the protruding red mass of internal organs slipping out the opening.  To be honest, I didn’t know what to think, I had half heard so much in the last hour or so, I was still trying to process all that was happening and then the transport personnel came to take her.  I do have to say they were a wonderful bunch of people.  They didn’t rush in and ram you over while busily going about their business.  They explained what they knew to me and they described everything they were doing to my baby and why.  After they had her situated for transport they let me have time to say good-bye and then took a couple of Polaroid pictures for me to hold unto.  They gave me a time estimate of what it would take to transport my baby to The Children’s Hospital in Milwaukee and get her settled in the Neonatal Intensive Care unit up there.  They also said that someone would call from the hospital to let me know how she did on the trip.

I took everything in and then watched as they wheeled her out of the nursery, down the hall and into the elevators.  I stood in the hallway, holding my Polaroid pictures and finally at that point allowed myself to cry.


LINKS:
http://www.caringbridge.org/