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Sacral agenesis

Sacral agenesis (or hypoplasia of the sacrum) is a little known and rather infrequent congenital condition of spinal deformity affecting the sacrum - the caudal partion of the spine. It occurs at a rate of approximately 1 of 25,000 live births. more...

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Etiology

The condition arises from a set of conditions present during approximately the 3rd week to 7th week of fetal development. Formation of the sacrum/lower back and corresponding nervous system is usually nearing completion by the 4th week of development. While the exact etiology is unknown, the condition may be associated with certain dietary deficiencies including a lack or insuffient amounts of folic acid or other developmental aids. The condition may also be associated with or resultant of maternal diabetes.

Prognosis

There are four levels (or "types") of malformation. The least severe indicates partial formation (unilateral) of the sacrum. The second level indicates a billateral (uniform) deformation. And the most severe types involve a total absence of the sacrum.

Depending on the type of sacral agenesis - bowel or urinary bladder deficiencies may be present. A permanent colostomy may be necessary in the case of imperforate anus. Incontinence may also require some type of continence control system (e.g. self-catheterization) be utilized. Occasionally if deformities of the knees, legs or feet would prove unresponsive to corrective action - amputation at the knee may be proposed.

Before more comprehensive medical treatment was available, full amputation of the legs at the hip was often performed.

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Surgical Management of Vesicoureteral Reflux - Statistical Data Included
From AORN Journal, 3/1/00 by Pamela I. Ellsworth

Vesicoureteral reflux (VUR) is a urologic condition in which there is a retrograde flow of urine from the bladder through the ureter back up to the upper urinary tract. Vesicoureteral reflux may be classified as primary or secondary. Primary reflux, a congenital anomaly of the ureterovesical junction, is due to a deficiency of the longitudinal muscle of the intravesical ureter, which results in an inadequate flap valve mechanism. Secondary reflux is a result of decompensation of the valvular mechanism as a result of elevated intravesical pressures associated with voiding dysfunction and bladder outlet obstruction.(1)

The incidence of VUR ranges from 1% to 18.5% in children and is as high as 70% in infants who present for treatment with symptomatic urinary tract infections.(2) Eighty-five percent of children are females, with Caucasian females most frequently affected.(3) The definitive reason that VUR occurs more frequently in Caucasian females is unknown, but it is believed to be genetic.

Vesicoureteral reflux appears to have a multifactorial origin. It appears that the genetic component exists, and this is supported by the increased incidence (ie, 45%) of VUR among siblings.(4) Sterile reflux after birth does not appear to cause significant renal damage; however, reflux of infected urine may lead to pyelonephritis, renal scarring, and in severe cases, significant loss of renal function and subsequent hypertension. The primary goal in the management of VUR is the prevention of pyelonephritis and subsequent renal scarring.

HISTORICAL PERSPECTIVES

Vesicoureteral reflux and the concept of an incompetent ureterovesical junction were first described by Leonardo da Vinci and Galen.(5) One researcher noted that renal infection may be the result of an abnormal ureterovesical configuration.(6) Another researcher was the first to demonstrate that the length of the intravesical ureter and the muscular development of the trigone affected the incidence of VUR.(7) In 1952, it was proposed that there was a causal relationship between reflux and pyelonephritis and renal scarring.(8) Restoration of a normal length submucosal tunnel, which was five times the diameter of the ureter, became the focus of surgical repair.

In 1967, researchers demonstrated that the normal ureteral diameter ranges from 1.4 mm to 2.0 mm and varies in size at the ureterovesical junction with the child's age. The normal intravesical ureteral length varies from 7 mm to 12 mm and also varies with the child's age. In children with reflux, the ureter is often dilated up to several millimeters in diameter, varying with the degree of reflux. Based on these findings, restoration of a normal submucosal (ie, intramural) tunnel, five times the diameter of the ureter, became the focus of the repair.(9)

The excellent success of open procedures (ie, 98%), fueled the interest in devising less invasive techniques to surgically correct VUR.(10) Currently, a variety of surgical techniques are used to treat VUR, including laparoscopic, endoscopic, and open techniques.

EVALUATION AND MANAGEMENT

Most children with VUR present with symptoms of a urinary tract infection (UTI). Newborns often are diagnosed with a febrile illness, failure to thrive, or a history of pelvicaliectasis (ie, dilation of the renal pelvis and calyces) on a prenatal ultrasound.(11) Complete evaluation, including a voiding cystourethrogram (VCUG) and renal ultrasound, is indicated in four groups of children. The first group consists of any child less than five years of age with a documented UTI. In the second group are children with a febrile UTI, regardless of their age. The third group consists of male children with a UTI, unless the male child is sexually active or has a significant urologic history.(12) The fourth group consists of newborns with a history of prenatal pelvicaliectasis.(13)

Vesicoureteral reflux may be identified on a contrast VCUG or by radionuclide VCUG. Both VCUGs are performed in the radiology department. The contrast VCUG is performed in the fluoroscopy suite, and the radionuclide VCUG is performed in the nuclear medicine department. Preparation for both studies involves antiseptic cleansing of the glans and perimeatal area and the placement of a small caliber lubricated feeding tube into the bladder. Usually, a 5-Fr catheter is used in newborns and infants, and an 8-Fr catheter is used in children. The postvoid urine residual is recorded, and a urine sample is sent for urine culture. The child's expected bladder capacity is determined using the formula of the child's age in years plus two, times 30.

For the contrast study, diluted water-soluble contrast is instilled into the catheter by gravity that does not exceed 100 ml water. An x-ray is obtained before instilling the contrast to confirm catheter position and to rule out any abnormalities of the lower spine (eg, sacral agenesis, spina bifida occulta). While the contrast medium is being instilled, intermittent fluoroscopic scanning is performed to assess for reflux during the filling. Fluoroscopic scanning also is performed while the child is voiding. If reflux is present, films are taken at intervals (ie, delayed) to assess upper urinary tract drainage. A postvoid film also is obtained to assess bladder emptying. Repeating the filling and voiding cycle during the same examination increases the accuracy of the contrast voiding cystourethrogram VCUG by 12%.(14) A contrast VCUG usually is used for the initial radiologic study as it allows for grading of the reflux and visualization of the urethral and bladder anatomy. Reflux is routinely graded according to the International Classification of VUR (Table 1, Figure 1).(15)

[Figure 1 ILLUSTRATION OMITTED]

Table 1

INTERNATIONAL CLASSIFICATION OF VESICOURETERAL REFLUX

* Grade I: Reflux that occurs in the ureter only.

* Grade II: Reflux that extends into the renal pelvis and calyces.

* Grade III: Reflux that extends into the renal pelvis and calyces and is associated with mild to moderate ureteral dilation and minimal blunting of the fornices.

* Grade IV: Reflux that extends into the renal pelvis and calyces and is associated with moderate ureteral, renal pelvic, and calyceal dilation.

* Grade V: Reflux that is associated with significant dilation of the ureter, pelvis and calyces, and ureteral tortuosity.

Follow-up studies in children with documented reflux may be either by a contrast VCUG or a radionuclide VCUG. The radionuclide VCUG is performed using technetium-99m pertechnetate mixed in normal saline to fill the bladder. Scanning is performed using a gamma camera, which is used in nuclear medicine departments, that scans patients who have been injected with small amounts of radioactive materials.

The advantage of the radionuclide VCUG is that it provides radiation doses that are 100 times lower than the radiation doses of a standard VCUG.(16) In addition, it allows for prolonged examination under the gamma camera, which increases its sensitivity. Disadvantages of the radionuclide study include difficulty in grading the reflux and not providing the anatomic detail that is provided by a contrast VCUG.

The VUR grades I to III eventually resolve spontaneously in many children. The rate of resolution of the VUR depends on the initial grade of the VUR and the age of the child at the time of diagnosis. Low-grade reflux (ie, grades I and II) resolve spontaneously in 80% to 85% of children, and moderate-grade reflux (ie, grade III) (Figure 2) resolves spontaneously in 50% of children.(17) High-grade reflux (grade IV or V) (Figure 3) is much less likely to resolve spontaneously.(18) Reflux tends to resolve more often in children who present for treatment at a younger age. If resolution occurs, it usually occurs within four to five years after the diagnosis.(19)

[Figures 2-3 ILLUSTRATION OMITTED]

TREATMENT

The American Urologic Association has developed guidelines for the management of primary VUR. Children with VUR who are toilet-trained should be evaluated for voiding dysfunction. The presence of voiding dysfunction associated with holding of urine and constipation may delay the resolution of the reflux and may affect the success of ureteral reimplantation. These children should be treated with appropriate behavioral and medical therapy before consideration for ureteral reimplantation.

As sterile reflux does not appear to cause significant renal damage and prophylactic antibiotics are well tolerated by children, most children with VUR can be managed medically. Even infants with high-grade reflux may be initially managed with medical therapy, thus avoiding surgical treatment in infancy. Low dose prophylactic antibiotic therapy is the mainstay of medical treatment. Amoxicillin or ampicillin is given to newborn patients up to six weeks of age. After six weeks of age, the child's liver is mature enough to use trimethoprim and sulfamethoxazole. Nitrofurantoin monohydrate is another reasonable prophylactic antibiotic that may be used in children who are older than eight weeks of age.

Technetium-99m dimercaptosuccinic acid (DMSA) renal scans are taken to detect renal scarring and assess renal function. A DMSA scan is obtained in children with a history of several febrile UTIs and/or if there is a discrepancy in renal size or a suggestion of renal scarring seen on renal ultrasound. Parents obtain a urine sample for culture whenever the child has a temperature of greater than 101.5 [degrees] F (38.56 [degrees] C), dysuria, foul-smelling urine, or a change in voiding habits. The child remains on prophylactic antibiotics until the VUR is resolved or is surgically corrected.

Indications for surgical intervention (ie, ureteral reimplantation) in children are UTIs that occur despite prophylactic antibiotics; noncompliance with medical therapy; high-grade reflux (ie, grades IV and V), particularly when associated with renal scarring; reflux (ie, grade II and higher) that persists in females who are nearing puberty; VUR associated with congenital abnormalities at the ureterovesical junction (eg, bladder diverticula); and reflux that increases in grade.(20) These indications correspond with the guidelines of the American Urologic Association.(21)

PATIENT ASSESSMENT

The pediatric urologist discusses surgical correction of VUR (ie, ureteral reimplantation) with the child and family members when one or more of the criteria for surgical correction are met. The preoperative assessment initially involves a thorough assessment of the child's voiding habits to rule out coexistent voiding dysfunction. If a voiding dysfunction is present, this is treated before surgical intervention. We review all of the previous radiographic studies to determine the location of the reflux, the grade of reflux, and bladder capacity. The goal of the surgery is to treat reflux to prevent renal scarring. It is important to emphasize that UTIs may recur after surgery but usually involve only the bladder.(22) The general principles of patient teaching apply to patients who are scheduled for ureteral reimplantation. An age-related understanding of the surgical procedure must be considered when preparing the child for surgery. In older children, the use of a videotape or a guided tour of the OR familiarizes the child with the waiting area, the OR, and the postanesthesia care unit (PACU) and can alleviate some of the child's and parents' anxieties.

A complete history and physical examination, including a history of medical illness; previous surgeries; allergies; medications; family history, including anesthesia-related problems; and potential bleeding problems should be performed within one month before surgery. A urine culture is performed one week before surgery and, if it is positive, the pediatric urologist is notified and the child is treated with appropriate antibiotic therapy. An active infection at the time of surgery causes the bladder mucosa to be more friable and difficult to work with, and it increases the risk of infectious complications in the postoperative phase.

The surgeon discusses the planned surgical repair method with the parents, including the fact that the child will remain in the hospital for approximately three to four days. The child is included in the discussion if the child is old enough to understand what is being discussed. The parents are informed that a Foley catheter will be placed in the child's bladder during the procedure. The Foley catheter remains in place for approximately three days postoperatively for an open procedure and one day if an extravesical procedure is performed.

Ureteral stents usually are not inserted during the procedure unless there is diminished urine flow through the ureteral orifice at the completion of the ureteral reimplantation. If a ureteral stent is placed, it exits through the bladder wall and the lateral aspect of the incision and is usually removed on the patient's third postoperative day.

PREOPERATIVE CARE

The perioperative nurse meets the patient and family members the morning of surgery. The nurse weighs the child, assesses vital signs, and reviews and verifies the surgical consent form to ensure that it has been properly completed and signed. The perioperative nurse confirms with the family members the laterality of the ureter that is to be reimplanted. If there is any discrepancy between what is scheduled and the parents' response, the pediatric urologist is notified. The child's medical history and allergies are confirmed, and the urine culture results are reviewed. The radiology film packet is brought into the OR, and the most recent VCUG is hung on the x-ray viewbox for the surgeon to see during the procedure.

If a parent will be accompanying the child into the OR, the perioperative nurse explains to the parent what to expect, what is expected of the parent, and where to stand during the child's anesthesia induction. When the child is anesthetized, the parent is escorted to the surgical waiting area by a nursing assistant.

INTRAOPERATIVE CARE

Approximately 30 minutes before bringing the child into the OR, the perioperative team members prepare the room for the patient's arrival. The OR is warmed to maintain the child's body temperature, and a temperature-regulating blanket is placed on the OR bed to prevent patient heat loss and hypothermia. The patient's temperature is monitored by the anesthesia care provider.

The perioperative nurse ensures that all equipment and supplies needed for the procedure are available and functioning. Two instrument tables are set up, one for the cystoscopy, which is performed first to rule out a ureterocele and other bladder pathology, and another is set up for the ureteral reimplantation. Cystoscopes in the appropriate sizes for the child's age should be available. The surgeon's loupes should be available and in the OR for the open procedure.

The anesthesia care provider and the circulating nurse bring the child into the OR on a stretcher or crib. The child is either assisted to the OR bed or carried, depending on the his or her age, by the anesthesia care provider and positioned on the OR bed by the circulating nurse in preparation for anesthesia induction. The anesthesia care provider and the circulating nurse place the monitoring equipment on the patient (ie, blood pressure cuff, electrocardiogram electrode leads, oxygen saturation monitoring device, temperature probe). The anesthesia care provider establishes an IV line access for administration of fluids and medications. General anesthesia is administered through an endotracheal tube that is secured in place. After general anesthesia is administered, the anesthesia care provider places and secures an epidural catheter. At our institution, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire, bupivacaine is the standard infusion through the epidural catheter. Other agents may be added at the discretion of the anesthesia care provider.

SURGICAL PROCEDURE

The surgeon usually performs a cystoscopy first to rule out a ureterocele and other bladder pathology. The cystoscopy is followed by an open ureteral reimplantation procedure.

Cystoscopy. The circulating nurse and surgeon place the child in the lithotomy position (infants are placed supine) for the cystoscopy. The circulating nurse is careful to pad the stirrups to prevent injury to the child's peroneal nerve. The circulating nurse preps the child's perineum with an antiseptic solution, after which the surgeon drapes the surgical area.

The surgeon performs a cystoscopy with the appropriate-size cystoscope for the child, which varies from a size 9.0-Fr cystoscope for infants and younger children to a size 11.5-Fr cystoscope for older children. The surgeon obtains a urine sample for culture from the bladder through the cystoscope. After the urine sample has been obtained, the anesthesia care provider administers a dose of IV antibiotics, usually cefazolin 25 mg/kg.

Irrigation fluid used for the cystoscopy consists of sterile saline. When the surgeon introduces the cystoscope, he or she evaluates the bladder mucosa for signs of chronic inflammation (ie, cystitis) and verifies the position of the ureteral orifice. He or she examines the bladder to rule out other pathology, such as a ureterocele. At the completion of the cystoscopy, the bladder is filled with sterile normal saline and the cystoscope is removed.

Open procedure. The circulating nurse and the surgeon together lower the child's legs and take the child out of the lithotomy position, maintaining the child's safety, and they reposition the child on the OR bed in a supine position. The circulating nurse places an electrosurgical unit dispersive pad appropriate for the child's size on an area of the skin that is dry, clean, and over a large, well-perfused muscle mass as close to the surgical site as possible. The circulating nurse re-preps the child's abdomen and perineum with the antiseptic prep solution.

The surgeon drapes the surgical area and makes a Pfannenstiel's incision, in a natural skin crease, of 4 cm to 6 cm using a #15 blade and knife handle. He or she dissects the subcutaneous tissue using electro-surgery down to the anterior rectus fascia. The rectus fascia is incised transversely, the length of the incision. The surgeon creates fascial flaps proximally to the umbilicus and distally to the symphysis pubis and enters the prevesical space in the midline area. The peritoneum is gently dissected from the bladder. The surgeon places two full thickness tacking sutures of 3-0 chromic on either side of the midline into the bladder wall and incises the bladder in the midline. To prevent bladder neck injury, the surgeon tacks the proximal aspect of the incision to the rectus fascia with interrupted 3-0 silk sutures. A self-retaining retractor is then positioned to provide surgical accessibility and visibility.

The surgeon identifies and catheterizes the refluxing ureteral orifice with a 5-Fr pediatric feeding tube. A 5-0 polypropylene suture is placed through the ureteral orifice to provide traction. He or she incises the bladder mucosa surrounding the ureteral orifice using the electrosurgical pencil and uses tenotomy scissors to dissect and define the plane between the detrusor urinae muscle and the ureter. The detrusor urinae muscle is a network of longitudinal fibers that form the external layer of the muscular coat of the bladder.(23)

The surgeon dissects the ureter free from the detrusor urinae muscle by a combination of sharp and blunt dissection. Hemostasis is controlled with electrosurgery. When the ureter is fully mobilized, the ureteral hiatus (ie, normal ureteral opening) is narrowed with approximation of the detrusor urinae muscle with 30 polyglactin sutures, and the knots are buried.

At this point in the surgical procedure, a variety of open methods may be used, depending on the child's anatomy and the surgeon's preference. Common open bladder procedures for ureteral reimplantation include the Politano-Leadbetter method (Figure 4), the Glenn-Anderson method (Figure 5), and the Cohen Cross-Trigonal method (Figure 6).

[Figures 5-6 ILLUSTRATION OMITTED]

The Cohen Cross-Trigonal ureteral reimplantation is the procedure most commonly used at our facility for patients with VUR. The procedure involves the creation of a submucosal tunnel that extends from the native ureteral hiatus transversely across the bladder to the contralateral side. This technique is especially useful when the ureter is dilated as it provides a long submucosal tunnel. The surgeon creates the submucosal tunnel with blunt dissection using Metzenbaum scissors. The tunnel is dissected to a width that is a few millimeters wider than the ureteral diameter that ensures that it does not constrict the ureter The length of the submucosal tunnel should be five times the diameter of the ureter.

The ureteral catheter is removed and the ureter is brought through the submucosal tunnel by placing a right angle through the tunnel, grasping the tacking suture that has been placed in the ureter, and drawing the ureter through the tunnel to the contralateral side. The ureteral orifice is secured to the bladder with full-thickness bladder to ureter interrupted sutures of 5-0 poliglecaprone at the apex and interrupted bladder mucosa to full-thickness ureter sutures circumferentially. The ureter is again catheterized to ensure that there is no ureteral kinking. The surgeon removes the ureteral catheter is removed and examines the ureteral orifice for reflux of urine.

At this point in the procedure, the surgeon asks the anesthesia care provider to increase the rate of the IV fluids to increase the urinary output. If the urinary output from the ureteral orifices is poor, despite the additional IV fluid, then a small ureteral catheter (eg, size 4 Fr or 5 Fr) is placed into the ureter and exited through a small perforation made in the contralateral bladder wall that exits along the lateral aspect of the incision. The surgeon sutures the ureteral catheter to the skin with a 4-0 chromic or nylon suture and connects it to a urinary drainage collection system. The bladder mucosa at the native ureteral orifice is reapproximated with running 5-0 chromic or poliglecaprone sutures. If there is urine drainage from the ureteral orifice, the surgeon starts to close the bladder. The surgeon closes the bladder in two layers using 3-0 chromic, approximating the mucosa in the first layer and the submucosa and detrusor urinae muscle in the second layer. The rectus fascia is reapproximated with a running 3-0 polyglactin suture. The surgeon approximates the subcutaneous tissue with interrupted chromic sutures and closes the skin with 4-0 polyglactin or 5-0 poliglecaprone subcuticular sutures. A silastic Foley catheter that is appropriate for the patient's urethra is inserted on completion of the bladder and abdominal wound closure. Self-adhesive wound approximating strips and a sterile gauze dressing are applied to the surgical wound and taped in place. The surgeon gently irrigates the Foley catheter to ensure patency, and the circulating nurse tapes the catheter in place to the child's inner thigh, ensuring there is no tension on the catheter.

Extravesical procedure. An extravesical procedure is a urinary procedure that is performed outside the urinary bladder. The detrusorrhaphy is the most popular extravesical technique (Figure 7).(24) The advantages of not opening the bladder include a decreased incidence of postoperative bladder spasms and hematuria and less risk of urinary tract contamination. This procedure is performed for unilateral VUR and is not usually performed for bilateral VUR because of the 16% risk of transient (eg, approximately four weeks' duration) urinary retention associated with the extensive detrusor urinae muscle dissection that is performed for cases of bilateral VUR.(25)

[Figure 7 ILLUSTRATION OMITTED]

The detrusorrhaphy. The circulating nurse places the patient in a supine position and preps the perineum and abdomen with antiseptic solution. The surgeon drapes the surgical area, performs the cystoscopy, inserts a sterile silastic Foley catheter in the urethra, and fills the bladder with sterile normal saline. The skin incision and bladder exposure are the same as for the open bladder procedure. The surgeon identifies and suture ligates the ipsilateral hypogastric artery and exposes the ureter. The ureter is mobilized distally to the ureterovesical junction. The detrusor urinae muscle is incised cephalad and lateral to the ureterovesical junction for a distance that measures five times the width of the ureter. The detrusor urinae muscle incision is continued circumferentially around the ureterovesical junction. The surgeon dissects the detrusor urinae muscle free from the underlying mucosa to create submucosal flaps. Two anchoring absorbable sutures of 4-0 polyglycolic acid are placed through the apical detrusor urinae muscle flap and proximal ureter to advance the ureter into the bladder. The detrusor urinae muscle flaps are then reapproximated over the ureter with interrupted sutures, creating a submucosal tunnel. The skin closure and dressings are the same as for the open bladder procedure.

POSTOPERATIVE CARE

The anesthesia care provider extubates the child in the OR. The child is usually awake by the time he or she is ready to be transported from the OR to the PACU. If an epidural catheter was inserted before the procedure for postoperative pain control, the child usually experiences minimal postoperative discomfort.

The child is transferred to the PACU by the anesthesia care provider and circulating nurse, who give their surgical reports to the PACU nurse. The surgical report includes the child's age, pertinent medical history, medical allergies, details about the surgical repair, placement of drainage tubes, and type of anesthesia administered. When the PACU nurse establishes that the child and his or her vital signs are stable, the parents are allowed into the PACU and remain with the child until he or she is transferred to a hospital room by the PACU nurse. The child's intake is advanced to clear liquids when he or she is fully awake and alert.

HOSPITAL AND DISCHARGE CARE

Children are given one and one-half times the normal fluid intake during the first 24 to 48 hours postoperatively to maintain a high urinary output. When the surgical procedure involves bilateral ureteral reimplants, potassium is not usually added to the IV fluids during the first 24 hours postoperatively until an adequate urine output has been confirmed. The addition of IV potassium increases the degree of hyperkalemia if the child experiences the complication of postoperative bilateral ureteral obstruction. The increased fluid intake and subsequent urine output helps minimize blood clot formation and helps overcome the obstructive component of the bladder mucosal edema. Often the child's urine will be bloody initially and then clear during the following two to three days. The pediatric unit nurses monitor the urinary output to ensure patency of the Foley catheter. If the urinary output is bloody and appears diminished, standing orders are written for the pediatric unit nurse to gently irrigate the patient's Foley catheter with 10 mL to 20 mL of sterile normal saline to ensure patency of the catheter and remove clots that may be present.

The surgical dressing usually is removed 24 hours postoperatively. The child's diet is advanced as tolerated. The child is maintained on IV antibiotics (ie, cefazolin, 50 mg/kg/day) pending the results of the intraoperative urine culture results. If the intraoperative urine culture is negative and the child is eating well, prophylactic oral antibiotics are resumed (ie, trimethoprim-sulfamethoxazole). If the intraoperative culture is positive, the child is treated with appropriate antibiotic therapy for the organism and resolution of the infection is confirmed with a repeat urine culture. Antibiotic prophylaxis is resumed after treatment of the UTI is completed. Prophylactic antibiotic therapy is continued until a postoperative VCUG demonstrates resolution of the reflux.

If the surgeon inserts a ureteral catheter during the procedure, it is removed at the bedside on the third postoperative day. If two ureteral catheters are inserted during surgery, the second catheter is removed on the fourth postoperative day. The Foley catheter is left in place after the ureteral catheter is removed to ensure that the urinary output from the Foley catheter increases, which reflects drainage from the ureter into the bladder. When this is confirmed, the Foley catheter is removed. If ureteral catheters are not inserted, the Foley catheter is removed on the third postoperative day for an open procedure and on the first postoperative day for an extravesical procedure.

Antispasmotics (eg, oxybutynin, 0.2 mg/kg every six to eight hours) and belladonna and opiate suppositories (ie, one-quarter to one suppository depending on the child's age and size) are used for bladder spasms.(26) Bladder spasms usually resolve when the Foley catheter is removed.

When the Foley catheter balloon is not inflated, a suture is passed through the holes near the tip of the Foley catheter, passed through the bladder, anterior abdominal wall, and skin, and then tied to keep the catheter in place. To remove the Foley catheter, the surgeon cuts the suture at the bedside and removes the Foley catheter, allowing for catheter withdrawal. The rationale for this technique is that it minimizes irritation of the floor of the bladder (ie, trigone) and thus decreases the number of bladder spasms.

The patient is given bladder antispasmotics if urinary frequency is bothersome after the Foley catheter is removed. Incisional pain is usually controlled with medication delivered through the epidural catheter. When the epidural catheter is removed, (eg, usually six to eight hours postoperatively) acetaminophen or acetaminophen with codeine is given to the child for incisional pain.(27)

On the first postoperative day, children usually are able to ambulate and tolerate sitting in a chair. Glycerine and bisacodyl suppositories (eg, dose varies with age) are administered if the child has not had a bowel movement before discharge from the hospital. The parents are warned of the constipating effects of the antispasmotics and codeine, and dietary fiber supplementation is encouraged. The child is discharged from the hospital to go home with his or her parents when the child is able to void spontaneously after the Foley catheter is removed. The child is allowed to take a shower or short tub baths at the time of hospital discharge. School-aged children are told that they have to refrain from gym class and recess for three weeks postoperatively.

FOLLOW-UP CARE

The surgeon prescribes prophylactic antibiotics for the child to take at home. Antispasmotic therapy (eg, oxybutynin 0.2 mg/kg taken every eight hours) is prescribed for the patient to be taken as needed.(28) The surgeon tells the parents and child that the urine may be bloody at first but should be clear, with increased fluid intake, within the first two weeks postoperatively. Parents are instructed to call the surgeon if the child develops a fever greater than 101.5 [degrees] F (38.5 [degrees] C), if the incision is erythematous, or if the child complains of abdominal or back pain. If any of these symptoms are reported, a urine culture is obtained.

Barring any complications, the child usually returns to the urology clinic one month after surgery. At that time, a renal ultrasound is performed to determine the presence or absence of pelvicaliectasis (ie, dilation of the renal pelvis and calyces). The surgeon examines the wound and assesses the child's voiding pattern. If pelvicaliectasis is present on the postoperative ultrasound, an additional follow-up renal ultrasound is obtained. If the pelvicaliectasis persists, then a renal scan or urogram is obtained to rule out obstruction.

The child returns to the urology clinic two to three months postoperatively for a VCUG. If the VCUG shows resolution of the reflux, prophylactic antibiotics may be discontinued two days after the VCUG. The surgeon discusses with the parents the possibility that the child may continue to develop UTIs, but the risk of developing pyelonephritis is markedly decreased. If the child develops recurrent UTIs after surgery, prophylactic antibiotics are restarted. A renal ultrasound is usually performed one year postoperatively to rule out latent obstruction and to evaluate renal growth. Children with significant renal scarring may develop hypertension, and parents are told to have the child's blood pressure monitored at routine pediatric visits.

POSSIBLE COMPLICATIONS

Complications associated with ureteral reimplantation may occur early or late in the postoperative period. Early complications include bleeding, reflux, and ureteral obstruction. Late complications are primarily persistent reflux and ureteral obstruction.

It is important for the surgeon to maintain hemostasis when dissecting the ureter free from the bladder and in the closure of the ureteral hiatus and bladder to minimize the risk of postoperative bleeding. If bleeding occurs retrovesically, it may lead to ureteral obstruction, which requires ureteral stenting until the bleeding is resolved.(29)

Early VUR may be observed in the reimplanted ureter or the contralateral ureter. Early VUR may be the result of postoperative trigonal edema and bladder dysfunction. These cases usually resolve spontaneously. At four months postoperatively, the incidence of VUR in the reimplanted ureter is 3%, and it typically resolves within one year after surgery.(31) Early reflux in the contralateral ureter occurs in approximately 16% of unilateral reimplants; however, in the majority of cases, this resolves spontaneously.(30)

Ureteral obstruction, varying from a partial obstruction in which urine is able to pass at a slower rate than normal into the bladder to complete obstruction in which the urine is unable to pass through the intravesical ureter into the bladder, may occur early after ureteral reimplantation. Contributing factors to partial or complete ureteral obstruction include edema, subtrigonal bleeding, retrovesical bleeding, bladder spasms, and blood clots. Most cases of obstruction are partial and asymptomatic, are typically identified on the postoperative renal ultrasound, and do not require treatment. These typically will resolve without intervention, but they are monitored with periodic renal ultrasounds to ensure complete resolution. Children with significant obstruction usually have flank or abdominal pain and nausea and vomiting. Renal ultrasound in these children usually shows severe pelvicaliectasis. The configuration of the Cohen Cross-Trigonal ureteral reimplantation and the postoperative bladder mucosal edema makes it difficult to gain access in a retrograde approach, and in such cases we ask the interventional radiologists to insert an indwelling ureteral stent using a percutaneous approach. Retrograde studies are performed approximately one month after the percutaneous stent placement, and the stent is removed if contrast is seen passing through the intravesical ureter into the bladder during fluoroscopy.

A late complication that is diagnosed in patients is VUR that persists for longer than one year after surgery. This usually is the result of an inadequate submucosal tunnel or inadequate muscular backing for the ureter within the submucosal tunnel.(31) The incidence of complications varies with the surgical method that is used; it is less than 2% with the Cohen Cross-Trigonal technique and ranges from 1% to 7% with the detrusorrhaphy method.(32)

Persistent reflux commonly occurs with dilated ureters and with more simplified ureteral advancement techniques such as the Glenn-Anderson technique. Voiding dysfunction also may contribute to the persistence of VUR. Children with persistent VUR should have urodynamic studies performed to rule out voiding dysfunction and/or a neurogenic bladder. Neurogenic bladder is a dysfunction of the urinary bladder that is caused by a lesion of the nervous system.(33) Bladder dysfunctions should be treated before a second procedure is performed.

Latent obstruction usually is the result of ureteral ischemia and requires surgical repair. Other causes of persistent obstruction include angulation of the ureter at the new hiatus or inadvertent passage of the ureter through the peritoneum or bowel. Intermittent ureteral obstruction with bladder filling can occur in ureters that are reimplanted in abnormally lateral positions. Surgical revision often is required in these cases.

SUMMARY

The surgical correction of VUR is associated with a high success and low complication rate. Despite this fact, it should not be used as the primary therapy for the treatment of VUR; rather, ureteral reimplantation should be performed in select cases. The use of postoperative epidural catheters for pain control has had a significant impact on the patient's postoperative recovery period. The pediatric urology nurse practitioner, perioperative nurses, and pediatric unit nurses must be capable of interacting with children of all ages as the age at VUR correction may vary significantly. Careful preparation of the child and family members results in minimal discomfort to the child and decreased parental and child anxieties, and it allows for prompt control of postoperative alterations in voiding.

NOTES

(1.) R D Walker, "Vesicoureteral reflux and urinary tract infection in children," in Adult and Pediatric Urology, third ed, J Y Gillenwater et al, eds (St Louis: Mosby, 1996) 2259-2293.

(2.) R Baker et al," Relation of age, sex and infection to reflux: Data indicating high spontaneous cure rate in pediatric patients," Journal of Urology 95 (1966) 27; A Atala, M Keating, "Vesicoureteral reflux and megaureter," in Campbell's Urology, seventh ed, P C Walsh et al, eds (Philadelphia: W B Saunders Co, 1998) 1859-1916.

(3.) Atala, Keating, "Vesicoureteral reflux and megaureter," 1859-1916.

(4.) A D Van den Abbeele et al, "Vesicoureteral reflux in asymptomatic siblings of patients with known reflux: Radionuclide cystography," Pediatrics 79 (January 1987) 147; H N Noe, "The long-term result of prospective sibling reflux screening," Journal of Urology 148 (November 1992) 1739.

(5.) D Lines, "15th century ureteric reflux," Lancet 2 (December 1982) 1473; HC Polk, "Notes on Galenic urology," Urologic Survey 15 (1982) 2.

(6.) J A Sampson," Ascending renal infections: With special reference to the reflux of urine from the bladder into the ureters as an etiological factor in its causation and maintenance," Johns Hopkins Hospital Bulletin 14 (1903) 334.

(7.) G M Gruber, "A comparative study of the intravesical ureter in man and experimental animals," Journal of Urology 21 (1929) 567.

(8.) J A Hutch, "Vesicoureteral reflux in paraplegia: Cause and correction," Journal of Urology 68 (1952) 457.

(9.) L J Cussen, "Dimensions of the normal ureter in childhood," Investigative Urology 5 (1967) 164.

(10.) J Wacksman, "Initial results with the Cohen Cross-trigonal ureteroneocystostomy," Journal of Urology 129 (June 1983) 1198; M F Bellinger, J W Duckett, "Vesicoureteral reflux: A comparison of nonsurgical and surgical management," Contributions to Nephrology 39 (1984) 81-93; E T Gonzalez, J F Glenn, E E Anderson, "Results of distal tunnel ureteral reimplantation," Journal of Urology 107 (1972) 572; J Wacksman, A Gilbert, C A Sheldon, "Results of the renewed extravesical reimplant for surgical correction of vesicoureteral reflux," Journal of Urology 148 (August 1992) 359.

(11.) S K Fernbach, M Maizels, J J Conway, "Ultrasound grading of hydronephrosis: Introduction to the system used by the society for fetal urology," Pediatric Radiology 23 (June 1993) 478.

(12.) K A Burbige et al, "Urinary tract infection in boys," Journal of Urology 132 (June 1984) 541.

(13.) Atala, Keating, "Vesicoureteral reflux and megaureter," 1859-1916.

(14.) J H Paltiel, R C Rupick, H G Kiruluta, "Enhanced detection of vesicoureteral reflux in infants and children with use of cyclic voiding cystourethrography," Radiology 184 (September 1992) 753.

(15.) International Reflux Study Committee, "Medical versus surgical treatment of primary vesicoureteral reflux: Report of the International Reflux Study Committee," Pediatrics 67 (1981) 392.

(16.) M D Blaufox et al, "Radionuclide scintigraphy for detection of vesicoureteral reflux in children," Journal of Pediatrics 79 (August 1971) 239.

(17.) L R King, S O Kazmi, A B Belman, "Natural history of vesicoureteral reflux: Outcome of a trial of nonoperative therapy," Urologic Clinics of North America 1 (October 1974) 441-455; J W Duckett, "Vesicoureteral reflux: A `conservative' analysis," American Journal of Kidney 3 (September 1983) 139.

(18.) Walker, "Vesicoureteral reflux and urinary tract infection in children," 2259-2293.

(19.) G A McLorie et al, "High grade vesicoureteral reflux: Analysis of observational therapy," Journal of Urology 144 (August 1990) 537; Atala, Keating, "Vesicoureteral reflux and megaureter," 1859-1916.

(20.) Atala, Keating, "Vesicoureteral reflux and megaureter," 1859-1916.

(21.) J Elder et al, "Pediatric vesicoureteral reflux guidelines panel summary report on the management of primary vesicoureteral reflux in children," Journal of Urology (May 1997) 1846-1851.

(22.) D E Govan, J M Palmer, "Urinary tract infection in children: The influence of successful antireflux operations on morbidity from infection," Pediatrics 44 (November 1969) 677.

(23.) Mosby's Medical, Nursing, & Allied Health Dictionary, fifth ed, K N Anderson, L E Anderson, W D Glanze, eds (St Louis: Mosby-Year Book, Inc, 1998) 761.

(24.) S L Daines, N B Hodgson, "Management of reflux in total duplication anomalies," Journal of Urology 105 (May 1971) 720; R Lich et al, "The ureterovesical junction of the newborn," Journal of Urology 92 (1964) 436; W Gregoir, G V VanRegermorter, "Le reflux vesico-ureteral congenital," Urology International 18 (1964) 122.

(25.) A M Houle et al, "Extravesical nondismembered ureteroplasty with detrusorrhaphy: A renewed technique to correct vesicoureteral reflux in children," Journal of Urology 148 (August 1992) 704.

(26.) K B Johnson, The Harriet Lane Handbook: A Manual for Pediatric House Officers, 13th ed (St Louis: Mosby, 1993).

(27.) Ibid, 494.

(28.) Ibid.

(29.) P I Ellsworth, M A Barraza, P S Stevens, "Bilateral ureteral obstruction secondary to a retrovesical hematoma following bilateral ureteral reimplantation via the Cohen technique," Journal of Urology 154 (October 1995) 1498-1499.

(30.) M C Willscher et al, "Renal growth and urinary tract infection following antireflux surgery in infants and children," Journal of Urology 115 (June 1976) 722.

(31.) Atala, Keating, "Vesicoureteral reflux and megaureter," 1859-1916.

(32.) M R Zaontz et al, "Detrusorrhaphy: Extravesical ureteral advancement to correct vesicoureteral reflux in children," Journal of Urology 138 (October 1987) 947; Wacksman, Gilbert, Sheldon, "Results of the renewed extravesical reimplant for surgical correction of vesicoureteral reflux," 359.

(33.) Mosby's Medical, Nursing, & Allied Health Dictionary, fifth ed, 1001.

Examination

SURGICAL MANAGEMENT OF VESICOURETERAL REFLUX

AORN, Association of periOperotive Registered Nurses, is accredited as a provider of continuing education in nursing by the American Nurses Credentialing Center's (ANCC's) Commission on Accreditation. AORN recognizes this activity as continuing education for registered nurses. This recognition does not imply that AORN or the ANCC's Commission on Accreditation approves or endorses any product included in the activity. AORN maintains the following state board of nursing provider numbers: Alabama ABNPO075, California BRN00667, Florida FBN 2296, Kansas LT0114-0316. Check with your state board of nursing for acceptability of education activity for relicensure.

Professional nurses ore invited to submit manuscripts for the Home Study Program. Manuscripts or queries should be sent to Editor, AORN Journal, 2170 S Porker Rd, Suite 300, Denver, CO 802315711. As with all manuscripts sent to the Journal, papers submitted for Home Study Programs should not hove been previously published or submitted simultaneously to any other publication.

Answer Sheet

SURGICAL MANAGEMENT OF VESICOURETERAL REFLUX

Please fill out the application and answer form on this page and the evaluation form on the back of this page. Tear the page out of the Journal or make photocopies and mail to:

Or fax with credit card information to (303) 750-3212

A score of 70% correct is required for credit.

Event #00118 Session #5987

Contact hours: 2

Fee: Members $10; Nonmembers $20

Program offered March 2000.

The deadline for this program is April 30, 2001.

1. Record your identification number in the appropriate section below.

2. Completely darken the space that indicates your answer to the examination starting with question one.

3. Record the time required to complete the program

4. Enclose fee if information is mailed.

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[ILLUSTRATION OMITTED]

Learner Evaluation

SURGICAL MANAGEMENT OF VESICOURETERAL REFLUX

The following evaluation is used to determine the extent to which this Home Study Program met your learning needs. Rate the following items on a scale of 1 to 5.

[ILLUSTRATION OMITTED]

OBJECTIVES

To what extent were the following objectives of this Home Study Program achieved?

(1) Identify the cause of vesicoureteral reflux (VUR).

(2) Identify the methods used to diagnose VUR.

(3) Discuss the goal of ureteral reimplantation.

PURPOSE/GOAL

To increase the perioperative nurse's knowledge of the surgical management of VUR.

(4) Did this article increase your knowledge of the subject matter?

(5) Was the content clear and organized?

(6) Did this article facilitate learning?

(7) Were your individual objectives met?

(8) How well did the objectives relate to the overall purpose/goal?

TEST QUESTIONS/ANSWERS

(9) Were they reflective of the content?

(10) Were they easy to understand?

(11) Did they address important points?

What other topics would you like to see addressed in a future Home Study Program? Would you be interested of do you know someone who would be interested in writing an article on this topic?

Topic(s): --

Author names and addresses: --

[ILLUSTRATION OMITTED]

Pamela I. Ellsworth, MD, is the Assistant Professor of Urology at Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire.

Marc Cendron, MD, is the Director of Pediatric Urology at Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire.

Maureen F. McCullough, RN, BSN, is a perioperative nurse III at Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire.

COPYRIGHT 2000 Association of Operating Room Nurses, Inc.
COPYRIGHT 2001 Gale Group

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