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