Exstrophy of the Bladder

ed family member of 3.6 percent. Of 215 children born to XSTROPHY OF THE BLADDER IS A RARE CONGENITAL parents in this group with exstrophy or epispadias, 3 were u anomaly caused by a deficiency of the anterior abdomiaffected with bladder exstrophy (1 in 70 live births, or 1.4 nal wall involving the genitourinary and musculoskeletal syspercent).3 The male/female ratio for classic bladder exstrophy tems. This anomaly is part of a spectrum of conditions resuitis between 1.5:1 and 2:l.l ing ti-om abnormal development of the cioacal membrane. The spectrum ranges f?om classic EMBRYOLOGY: bladder exstrophy to cloaca1 AJ3sTRAcr DEVELOPMENT OF THE exstrophy (Figure 1). Because Exstrophy of the bladder is a rare congenital defect that BLADDER this anomaly is rare, many neooccurs when the abdominal wall and underlying structures, At the first stage of developnatal nurses are not familiar with including the ventral wall of the bladder, fail to f&e in ment, the cloaca (from the Latin it. This article discusses the inciutero. As a result, the lower urinary tract is exposed, and word for drain or sewer) exists, dence, inheritance, embryology, the everted bladder appears through the abdominal openThis structure drains both the clinical presentation, and maning. Various surgical interventions have been employed with variable success in the hope of achieving complete gastrointestinal and the urinary agement of bladder exstrophy. A dryness, fitll control over delivery of urine, freedom from tract. Growth of mesenchymal case study illustrates some of the catheters and external appliances, and a protected upper tissue between the ectodermal complexities involved in the care urinary tract. The most popular surgical approach is the priand endodermal layers of the of the patient with this condition. mary bladder closure with secondary bladder neck reconcloacal membrane results in forstruction. Comprehensive nursing, medical, and surgical mation of the lower abdominal INCIDENCE AND care are necessary to preserve renal and sexual function. muscles and the pelvic bones. INHERITANCE The many complex problems experienced by these infants Between the fourth and sixth Bladder exstrophy is uncomand their families call for a muhidisciplinary approach. This weeks of gestation, the cloaca is mon; it is estimated to occur in 1 article reviews occurrence, clinical presentation, and manpartitioned into a posterior recin 10,000 to 1 in 50,000 live agement of exstrophy of the bladder. tum and an anterior primitive births.le3 A . multicenter interna.. urogenital sinus by the growth tional retrospective study, which of a coronal partition called the urorectal septum (Figure 2). pooled data from nearly 6.3 million births between 1970 and The primitive urogenital sinus is continuous superiorly with 1975, documented the incidence of bladder exstrophy at 3.3 the allantois (a hindgut diverticulum that extends into the per 100,000 births, or 1 in 30,000.* Although chromosome umbilicus) and is bounded inferiorly by the urogenital memabnormalities do not appear to be a causative factor, some brane. It consists of an expanded superior presumptive bladfamilies are clearly at higher risk than others. Of 2,500 index der, a narrow neck that becomes the pelvic urethra, and an cases in a survey by Shapiro and colleagues, 9 affected siblings inferior expanded definitive urogenital sinus. In males, the were found. This projects to an overall risk of a second affectAccepted for publication February 1998. Revised May 1998.

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FIGURE 1 w Various entities in the exstrophy syndrome. FIGURE 2 H Developmental changes of the cloaca and cloaca1 membrane in an embryo. Arrows show direction of growth of the urorectal septum.

Types of Syndromes _Clo~cerl Exstrophy Supravesical Fissure

Hind@1 orifice

Cecum

A SIZE-4mm

B S I Z E -6mm._

CLassicalExstrophy

And
membrane

C SIZE - 12 mm

SIZE -16mm

P u b i c dxastusis

From: Gearheart JP, and Jeffs RD. 1992. Exstrophy of the bladder, epispadias, and other bladder anomalies. In Campbe//s Urology, Walsh P, et al., eds. Philadelphia: WB Saunders, 1774. Reprinted by permission.

From: Gearheart JP, and jeffs RD. 1992. Exstrophy of the bladder, epispadias, and other bladder anomalies. In Campbells Urology, Walsh P, et al., eds. Philadelphia: WB Saunders, 1773. Reprinted by permission.

pelvic urethra becomes the membranous and prostatic urethra, and the definitive urogenital sinus becomes the penile urethra. In females, the pelvic urethra becomes the membranous urethra, and the definitive urogenital sinus becomes the vestibule of the vagina.j According to the theory of embryonic maldevelopment in exstrophy held by Marshall and Muecke as cited in Jeffs, the basic defect is an abnormal overdevelopment of the cloacal membrane, preventing medial migration of the mesenchymal tissue and proper lower abdominal wall development. The timing of the rupture of this cloacal defect determines the variant of the esstroph!.-epispadias complex that results. Distal perforation results in epispadias; midperforation results in classic exstrophy (Figure 3), and perforation occurring prior to the descent of the urorectal septum results in cloacal exstrophy. Classic bladder exstrophy accounts for 50 to 60 percent of patients born with this complex6 Although epispadias, bladder exstroph!; and cloaca1 esstrophy share a common embryologic origin, they differ \vith respect to severity and extent of involvement. Epispadias, the mildest of the three variants, occurs when the urinary meatus opens onto the dorsal aspect of the penis. Cloaca1 exstrophy is the most extensive of the variants. It includes the features of epispadias and classic bladder exstrophy. In addition, there is exstrophy of the bowel, a poorl!. developed hindgut lisible in

the lower abdominal defect, and a bladder that is separated into two halves. The anus and the anal canal fail to develop because the hindgut is already open. In the male there is usually complete separation of the two halves of the penis. Poor penile development is frequently seen in cloaca1 exstrophy, and the problems of reconstructing the male make assignment to the female sex of rearing desirable in many male patients. In the female, a similar separation of the urethral groove occurs, which may cause two separate vaginas to form. In cloaca1 exstrophy, there may also be an associated omphalocele that may contain small bowel, liver, and spleen. The defect in cloacal exstrophy occurs at approximately the fifth week of gestation; consequently, the causative forces may affect multiple organ systems. Spinal and neural tube defects, hydrocephalus, cardiac anomalies, gastrointestinal obstructions and duplications, renal malformations, and limb defects have been seen in association with cloacal exstrophy.6 CLINICAL PRESEKTATIOK: ANATOMY, PATHOLOGY, AND ASSOCIATED DEFECTS Ultrasonographers can no\l characterize the changes seen in bladder and cloaca1 esstrophy as early as the twentieth u,eek of gestation. j The prenatal sonographic findings that suggest bladder exstrophv include the presence of a soft-tissue mass on the surface of the abdominal wall and the absence of a normal appearing bladder. Infants \vith classic bladder esstrophy have fenrer defects than those with cloacal exstroph!. (Figure 4). The associated

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FIGURE 3

Diagram of events leading to classic bladder exstrophy.

FIGURE 4

Male neonate with classic bladder exstrophy.

Urorectal septum
Future large bowel

Exstrophied bladder

Intact hindgut

From: Gearheart JP, and jeffs RD. 1992. Exstrophy of the bladder, epispadias, and other bladder anomalies. In Ccmpbells Urology, Walsh P, et al., eds. Philadelphia: WB Saunders, 1774. Reprinted by permission.

abnormalities are confined to the development of the abdomen and perineum, the urinary tract, tie genitalia, the spine, and the bony pelvis. The diamond-shaped area bounded by the umbilicus, the anus, and the rectus abdominis muscles houses the defect most apparent in patients with classic bladder esstrophy. Most commonly, the urinary tract in males is open from the dorsal surface the penis through the anterior aspect of the bladder neck and bladder up to the umbilicus. Females with bladder esstrophy may have a duplicated vaginal orifice, nhich may be located just inferior to the distal extent of the exposed urethra. In most patients, it is possible to visualize the urethral groove, the prostatic urethra, and, more proximally, the ureteric orifices. The bladder will often be, or appear to be, much smaller than normal for the size and gestational age of the child. Because the bladder is exposed to air and immediate trauma during delivery and in the nursery, it becomes inflamed. The urothelium often develops a polypoid appearance (Figure 5). Because the bladder

has normal blood supply and normal neuromuscular activity, it should be capable of normal fimction once it is closed.8 The upper urinary tract is usually normal, although anomalies such as horseshoe kidney, pelvic kidney, hypoplastic kidney, solitary kidney, and dysplastic megauFIGURE 5
n

Typical exstrophic bladder in a newborn male with polypoid aooearance of bladder mucosa.

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reter have been seen, Duplication of the ureter does occur and must be recognized prior to closure to avoid injury to one of the ureters. The umbilical-anal distance is short because the umbilicus is more caudally placed than normal and the anus is anterior. Anterior displacement of the anal canal contributes to varying degrees of anal incontinence. Displacement of the rectus abdominis muscles widens the internal inguinal canal and places the internal ring just beneath the external inguinal ring. For this reason, indirect inguinal hernia and intestinal incarceration are common, particularly in male infants.9 Rectal prolapse may occur in the untreated exstrophy patient with a widely separated symphysis. It is usually transient and reduces without difficulty. It is frequently exacerbated by severe straining or crying and virtually always disappears after bladder closure. Gonadal and ductal abnormalities do not appear to be part of this complex in either sex. In male infants, the defect is almost always associated with epispadias, which may or may not involve the full length of the penis. It may also be associated with other problems, such as undescended testes or a penis that appears shortened due to a wide separation of attachment, a prominent dorsal chordee, and a shortened urethral groove. A functional and cosmetically acceptable penis may be achieved if the dorsal chordee is released, the urethral groove is lengthened, and the penis is lengthened by mobilizing the attachment in the midline. A male patient with a very small or dystrophic penis should be considered for sex reassignment. However, the need for sex reassignment in classic bladder exstrophy is rare; it occurs in only 1 in 150 cases.O In female infants, the defect may be associated with a shortened urethra and vagina; the vaginal orifice is frequently stenotic and displaced anteriorly; and the clitoris is sometimes bifid. The uterus, fallopian tubes, and ovaries are usually normal except for the occasional uterine duplication. In female infants, reconstruction is usually less complex than in males. All cases of exstrophy have the characteristic widening of the pubic symphysis (Figure 6). This skeletal defect arises from outward rotation of the innominate bones. In addition, an outward rotation or eversion of the pubic rami occurs at their junction with the ischial and iliac bones. It is these rotational deformities that contribute to a short, pendular penis. These bon! defects do not appear to predispose to dislocation of the hip, regardless of whether osteotomy is used in subsequent treatment. MANAGEMENT Delivery Room and Nursery Immediate treatment after birth consists of protecting the exposed bladder, tissue, and other structures from injury. lo-r2 At birth,, the bladder mucosa is usually smooth, thin, and intact and needs to be protected from clothing, diapers, and other sources of physical injury_.@ the. delivery room, it is important to prevent the umbilical clamp from gouging the surface of the exstrophied bladder. A tie can be substituted and the clamp removed shortly after birth to prevent this problem. The bladder should be covered with a nonadherent sterile dressing, such as salinesoaked sterile Teffi and plastic wrap, to prevent the mucosa fi-om sticking to clothing or diapers. Standard varieties of petrolatum or regular gauze can become dry, adhere to the delicate mucosa, and lift it off when they are removed. For this reason, they should not be used. Early Management Because the bladder is best closed in the first 24 to 72 hours after birth, the infant should be transferred as soon as possible to a Level III nursery where there is a team experienced in newborn exstrophy closure. When transfer requires significant travel, adequate hydration should be provided initially at the referring hospital and then en route. Broad-spectrum antibiotics are initiated prophylactically, both because constant leakage of urine from the exposed urethral orifice makes the area susceptible to infection and to permit the contaminated bladder to be closed as a clean wound.6 Continued protection with a nonadherent sterile dressing is required to prevent trauma to the delicate bladder mucosa. Patients with classic bladder exstrophy have few renal problems; nevertheless, a renal ultrasound should be performed to identify hydronephrosis or other anomalous developments. A DTPL4 scan at 24 to 48 hours of age can indicate that renal vascularity, function, and drainage are satisfactory before proceeding with the initial closure of the bladder. The DTPA scan is a nuclear imaging test that employs fir injections of technetium-tagged diethylenetri-

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FIGURE 6 # Characteristic skeletal changes seen in classic bladder exstrophy: rotational and lateral deformities of the pelvic girdle and an external (outward) rotation of the pubic bones.

;uninepentaacetic acid. This radiopharmaceutical is excreted purely by glomerular filtration, making it possible to obtain images of the collecting systems. The scan provides important functional information about the kidneys that cannot be obtained by other imaging modalities. Operative Procedures The goals of reconstructive surgery for esstrophy of the bladder are (1) preservation of renal function, (2) management of urinary continence, and (3) genital and abdominal wall reconstruction.8 Historically, attempts at reconstruction have almost invariably failed, leaving the problems of incontinence, a deteriorating upper urinary tract, and the need for additional urogenital reconstruction. Internal diversion by anastomoses of the urinary tract to the large bowel frequently solved the incontinence problem but led to deterioration of the upper urinary tract and to problems of electrolyte imbalance. The ureterosigmoidostomy, a procedure involving surgical implantation of the ureters into the sigmoid flexure along with an antiretlus feature, has been more successful in preserving the upper urinary tract by preventing ascending infections, but hyperchloremic acidosis is a common persisting problem. 6~14 External diversion by ileal

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conduit or colon conduit is cftisctive in providing continence by the external bag, but because initialI!. there was not an antireflux mechanism, external diversion did not prevent the slow deterioration of the upper urinarv tract. More recently, ,m Jntirefluxing colonic conduit, which is later undiverted into the sigmoid colon, has shonn positive results, but long-term assessment of renal timction and continence requires tier investigation. Over the last ten years, the staged approach to bladder closure and subsequent continence surgery has made nearly all patients candidates for primary bladder closure. Even a patient with a very small bladder may develop surprisingly good bladder growth following primary bladder closure and epispadias rep&l5 Other operative methods may provide a solution or partial solution for difficult exstrophy cases or cases of cloacal esstrophy when primary bladder closure is not possible. These methods include internal diversion such as ureterosigmoidostomy (reimplantation of the ureters into the sigmoid colon), external diversion by ileal conduit and colon conduit, undiversion through colocolostomy (antirefluxing colonic conduit and later undiversion of the conduit into the sigmoid colon), and, more recently, augmentation cystoplasty (enlarging the bladder using the appendix or a tapered segment of the small bowel) with intermittent catheterization. It is preferable that primary closure be accomplished within the first 18 to 72 hours after birth. This minimizes pain, infection, and trauma to the exposed bladder mucosa. The bladder closure is followed by a period of incontinence or continuous leakage during which the bladder gradually enlarges. Historically, epispadias repair followed the surgical procedure to provide continence. Today, however, the epispadias is repaired before or at the same time as the continence procedure to add slight resistance to a bladder that may be slow to increase in capacity. Surgical correction of bladder incontinence is not attempted until the patient is two and onehalf to five years of age, when bladder neck plasty is considered. At that time, bladder volume is assessed. Bladder neck reconstruction is not attempted until an adequate bladder capacity is obtained. In most cases, a 50-60 ml capacity is adequate. l6 The goals of the primary bladder closure are (1) to close the bladder and displace it to a posi-

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don deep within the pelvis, (2) to provide free urethral and bladder drainage, (3) to perform initial penile lengthening if needed, and (4) to rotate the innominate bones to approximate the pubic symphysis by the use of bilateral iliac osteotomy. This procedure splits the iliac bones and allows for bladder and anterior midline wound closure without tension. The need for bilateral iliac osteotomy to provide improved pelvic mobility at the time of bladder closure remains controversial. In infants under 48 hours of age, the bones and joints are quite malleable, and approximation of the pubic symphysis can usually be obtained without an osteotomy in the primary bladder closure. In older patients, the osteotomy seems to provide additional mobility of the pelvis, which helps to secure a pubic closure with fewer complications and a more permanent pubic symphysis approximation. These observations have led some authors to recommend that an osteotomy be done in all infants. Proponents of osteotomy have suggested that improved continence rates can be expected following bladder neck plasty if osteotomy is used at the initial closure. Patients closed after 48 hours of age without osteotomy have not had as much success in gaining continence after completing the staged reconstruction.*J9 Management After Primary Closure Immobilization of the wound and pelvis following surgical bladder closure is a very important part of healing. The combination of internal and external fixation facilitates fusion of the pubic symphysis and primary healing of the bladder and abdominal wall. Internally, approximation of the pelvic ring is accomplished by bringing together the pubic rami with heavy nylon sutures. The external immobilization of the closed pelvic ring is carried out by using a spica cast, Bryants traction, or an elastic external compression dressing that prevents abduction of the patients legs at all times. Antispasmodics, analgesics, and tranquilizers are all used liberally in an effort to prevent bladder spasms or excessive crying, which may disrupt the closure.6 Postoperatively, urine drains through a suprapubic catheter and right and left ureteral stents.6,10*1s This offers almost complete urinary diversion, promoting healing of the operative site and decreasing the chance of infection and disruption of the suture line. Ureteral sterns provide drainage during the first seven to ten days, when swelling or the pressure of closure of a small bladder may block the ureters and give rise to obstruction. Urethral catheters may or may not be placed; the choice depends on the potential for erosion of the pubic suture into the urethra. Broad-spectrum antibiotics are given prophylactically. If no problems with the ureteral stems occur during healing of the operative site, they are left in place for up to three weeks postoperativei!: Around the third to fourth postoperative week, after the spica cast has been removed, the wound has healed, and the patency of the urethra has been demonstrated by measuring residual urine in the bladder, the suprapubic catheter is removed. 6JoJ5 An intravenous pyelogram (IW) is obtained to ensure free drainage of the upper urinary tract without hydronephrosis or hydroureter. Prophylactic antibiotics to prevent an upper urinary tract infection are continued until reflux has been corrected. Residual urine is estimated by straight catheterization, and cultures are obtained before discharge and at subsequent intervals to detect infection and to ensure adequate drainage.6J0J5 Presuming that the infant has little or no infection and that the bladder closure has been satisfactory, there should be few problems in the first two years of life. At the age of two and one-half to five years, epispadias repair in the male infant (if it was not performed with the initial repa%) .and continence surgery in both sexes are considered. COMPLICATIONS AND LONG-TERM RESULTS In most patients, the bladder can be closed successfully, but prolapse, hydronephrosis, stone formation, and dehiscence can occur. In one review of 75 primary bladder closures, prolapse occurred in 11 patients, and 3 patients developed a complete wound dehiscence.O Even though bilateral osteotomy is thought to make primary closure easier, no study has been able to associate use of osteotomy with wound dehiscence rates following primary closure. Despite the better suture material and the improved techniques that are available today, urethral strictures and urethrocutaneous fistdas are still common following epispadias repair. Some of the small fistulas may close spontaneously. Others can sometimes be closed satisfactorily with a secondary procedure. Although some centers have had good results with staged repair, results at other centers have been less encouraging.20J1 In most centers, overall continence rates following reconstruction of the bladder have been disappointing. This has led some clinicians to take a dif%erent approach to bladder exstrophy-one that uses augmentation with intermittent catheterization or diversion as a means of achieving continence and preserving renal function. 22-Z4 However, augmentation and diversion seem to have a higher rate of complications than staged repair. Complications of augmentation may include stones, hydronephrosis, renal scarring, and the development of rumors. Complications affecting fertility and sexual performance vary. Males with exstrophy and epispadias seem to do well despite what appears to be a severe sexual handicap. Most males with repaired epispadias achieve satisfactory erection for sexual intercourse. However, several long-term follow-up studies reveal significantly decreased fertility in nlales.;-27 The cause is usually an incompetent bladder neck with retrograde ejaculation or scarring of the ampulla of the vas deferens. Females with exstrophy have few problems with sexual intercourse. Many develop uterine prolapse, especially those with poor pelvic approximation, and mam require uterine

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suspension. Fertility problems are less common in reconstructed females th.m in males.. Adenocarcinoma of the bladder occurs in patients with esstrophy approximately 400 times more oken than in the normal population and accounts for about 90 percent of bladder tumors in these patients.28 It is the most commonly reported tumor in untreated cases of bladder exstrophy and occasionally occurs in adults \\,ho have had bladder closure a&r infancy.2y*30 Adenocarcinoma has not yet been reported in a patient whose bladder was successfully closed at birth. The inherent malignant potential of the closed esstrophied bladder has not been determined because long-term followup of a large number of bladder exstrophy cases without urinary tract infection, obstruction, and chronic irritation has not been done. NURSING IMPLICATIONS iiND PARENT COUNSELING One of the most devastating aspects of exstrophy of the bladder is its appearance. Families will continue to require information, emotional support, and reassurance regarding their childs condition. Physical care of the unrepaired defect includes meticulous hygiene of the bladder area to prevent infection and excoriation of the surrounding tissue. It is necessary to maintain a sterile, moist, nonadherent dressing over the exposed bladder area to minimize evaporative fluid and heat losses and aUow for drainage while protecting the bladder. Nursing care should minimize unnecessary manipulation or trauma to the area by avoiding urinary drainage bags, safety pins, ointments, or constrictive clothing.6,11>12 Other aspects of preoperative care are similar to those for any major abdominal surgery (Table 1). Postoperatively, careful monitoring of the suprapubic catheter and right and left ureteral stents is necessary. The nurse should routinely empty and record urine output from each tube at least every two to four hours.y2 Any significant increase or decrease in urine output or any indication of an obstructed suprapubic tube or ureteral stent must be noted. Blood clots and bloody urine are routinely seen for the first three to four days postoperatively. Urine or blood draining f?om the urethral meatus indicates blockage of the tubes or an increased intensity of bladder spasms. If blood or mucus blocks the tubes, &ey may be irrigated with normal saline, using sterile technique to ensure patency.12 Wound care consists of cleansing both the suture line and the area around the urinary drainage tubes with peroxide to prevent any encrustation of dried blood or urine.11T12 It is also vital to cleanse the urethral meatus, to ensure patency and to avoid infection. Patients with iliac osteotomies will have serosanguineous drainage from the surgical site for two to three days postoperatively. Sites may be covered with a sterile surgical film such as Opsite. This film should be removed, and wound care should be initiated after drainage decreases.

TABLE 1

Nursina Care of the Infant with Bladder Exstroohv

Immediately after birth and preoperatively Minimize manipulation or trauma to defect area (use umbilical tie instead of clamp to prevent trauma). Maintain moist, sterile, nonadherent dressing over defect, and cover with plastic wrap to prevent evaporative heat and fluid losses. Provide meticulous hygiene to the bladder area. Monitor for signs and symptoms of infection, and administer prophylactic antibiotics as ordered. Assess hydration status, and maintain parenteral therapy as ordered. Provide family support, and make appropriate referrals. Postoperatively Maintain immobilization of the wound and proper alignment of the pelvis. Assess circulation, and provide good skin care. Monitor intake and output (routinely empty and record urine output from each catheter every two to four hours). Assess urine for presence of blood or blood clots. Provide wound care. Assess for pain, and administer analgesics, antispasmodics, and tranquilizers as required. Continue to monitor for signs and symptoms of infection, and administer prophylactic antibiotics as ordered. Continue to assess hydration status, and maintain parenteral therapy as ordered. Continue to support family, and involve them in infants daily care.

Immobilization of the wound and pelvis following initial surgical bladder closure is essential to healing.12 Nursing care for external immobilization includes maintaining proper alignment of the patient, assessing the circulation of f2et and toes, and providing good skin care. Abduction of the patients legs is prevented at all times. Prompt attention to managing the patients pain, discomfort, hunger, and muscle spasms secondary to prolonged traction is imperative. Analgesics, antispasmodics, and tranquilizers should be liberally employed to ensure a calm postoperative course.612 Parents of bladder exstrophy patients need information and support. Initially, they need reassurance. Parents can generally be told that, with help, renal f&ction, ambulation, and sexual &nction can approach normal. They should be told &at a careMy considered decision concerning the management of urinary storage, drainage, and control will be made by an experienced surgeon as soon as possible. Once they have received this reassurance, they can begin to understand the implications of the problem. This will enable them to help with the medical and social decisions that must be made. Upon arrival, parents should be introduced to the members of the health care team. They should be given realistic information concerning the cosmetic and functional results of surgery because unrealistic expectations may leave them very disappointed. Gender issues should be addressed early. In male infants with poor penile development, the decision for

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reconstruction and sex reassignment should be made as soon as possible after birth. Parents should be counseled that the risk of bladder exstrophy is higher in families where one member is affected than it is in the general population3 Both parents should be involved in the care of the infant as soon as possible; this promotes parent-infant bonding. The social services offered by the hospital can be a great help to parents who are facing the problems that result from the childs prolonged hospitalization. These problems may include the financial strain of the unexpected medical care, family separation, and home-to-hospital travel. CASE PRESENTATION Baby L was a 40-week-gestation, 3,250 gm male infant born by vaginal delivery to a 29-year-old, gravida 2, para 2 Caucasian mother. The pregnancy and family history were unremarkable. Apgar scores were 9 at one minute and 9 at five minutes. Physical exam in the delivery room revealed a midline defect in the abdominal wall just below the umbilicus that had not been detected prenatally. The defect was covered with sterile saline-soaked gauze, and the infant was transported to a Level III nursery. When Baby L was admitted to the NICU, bladder and ureteral openings were noted protruding through the abdominal wall defect, along with urine draining from the ureters. An umbilical cord stump set low on the abdomen, a widened, abnormally formed phallus without a urethral opening, bilaterally descended testes, and an anteriorly displaced anus were also present (see Figures 4 and 5). Other findings on physical exam included abduction and limited range of motion of the lower extremities. A diagnosis of classic bladder exstrophy was suggested. The diagnostic workup included an abdominal flat plate to assess pelvic bony structure; a renal ultrasound to rule out vesicoureteral reflux and any other renal anomalies; and blood work, which included a capillary blood gas, blood culture, creadnine, complete blood count, electrolytes, type and crossmatch, and chromosome studies. The abdominal flat plate showed bowel gas present in the anterior abdominal wall, normal remaining bowel gas pattern, and separation of the pubic rami, all of which are consistent with bladder exstrophy. All blood tests were within normal limits, the blood culture was negative, and the chromosome studies revealed a 46XY male karyotype. The renal ultrasound revealed two kidneys, which were normal in size, contour, and echogenicity, without dilated ureters or hydronephrosis. Intravenous fluids of 10 percent aqueous dextrose solution were started at 110 ml/kg/day, and Baby L was started on ampicillin and cefotaxime for prophylaxis. Consultation with a pediatric urologist confirmed the diagnosis of classic exstrophic bladder and epispadias. An orthopedic specialist was consulted to evaluate pelvic anatomy. It was determined that a pelvic osteotomy would be necessary at the time of

bladder closure. A geneticist evaluated Baby L; his impression was isolated exstrophy of the bladder with epispadias in a phenotypically male infant. A conference was scheduled on the day of admission with the family and the pediatric urologist. At this conference, possible complications and future required surgeries were presented. The family was told that although spontaneous voiding is the goal, most patients do not achieve this goal and that many patients ultimately require the use of a catheter. The family was also told that patients with exstrophy of the bladder may have poor outcomes in terms of erectile ability, penile integrity, and lasting fertility. The family was informed that, despite the formidable obstacles, there is cur-.. rently no reasonable alternative to surgery; andthey agreed to proceed with the primary closure. On day 2, Baby L went to the operating room for closure of the exstrophic bladder, epispadias repair, and penile reconstruction. A pubic rami osteotomy was performed, and suprapubic, bilateral ureteral, and penile catheters were placed. Baby L was placed in a total hip cast for external immobilization of the approximated pelvic ring. A Replogle tube was placed in the stomach with low intermittent suction. Baby L remained intubated postoperatively (secondary to abdominal distention and pressure from surgical repair) and was paralyzed and sedated with vecuronium, fentanyl, and Versed to prevent disruption of the surgical area. The paralyzant and sedative agents were weaned, and Baby L was extubated to room air on postoperative day (POD) 8. Urine output (most of it draining from the ureteral and suprapubic catheters) remained stable until POD 6. Decreased urine output was noted over 24 hours, but output improved after the ureteral stems were irrigated with normal saline. Serial urinalysis, urine cultures, and serum creatinines were obtained and remained relatively normal. Ditropan, a spasmolytic agent that has a direct effect on the urinary tract smooth muscles, was started intravesically through the penile catheter into the bladder on POD 7 and was discontinued on POD 18 prior to discharge. Ureteral stems were capped on POD 8 in preparation for removal. Serial creatinines were obtained; these remained stable, and the ureteral stems were removed on POD 10. The suprapubic and penile catheters remained in place. Distal wound separation was noted in the suprapubic area on POD 12. No signs or symptoms of infection were noted, and the open wound was packed with dry sterile gauze. On exarninadon, a urethrocutaneous fistula was noted. This fistula eventually resolved spontaneously and did not require surgical closure. Intravenous antibiotics continued until POD 10 and then were switched to oral amoxicillin for urinary tract infection prophylaxis. Because he was paralyzed, Baby L was initially maintained on total parenteral nutrition (TPN). Enteral feedings were started on POD 9. Although gavage feedings were advanced slowly, Baby L consistently had emesis with feedings and did not seem interested in nippiing. A barium snallow showed

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Grade 2 gastroesophageal reflus; lack of interest in nippling may have been caused by a secondary esophagitis. The emesis improved after Zantac and cisapride were started. An occupational therapy consult was obtained to evaluate nippling, which improved after the formula was thickened with rice cereal. TPN was gradually weaned as caloric content improved, and Baby L was advanced to full nipple feedings by discharge. Baby L was discharged home on day 23 of tie. The penile catheter was removed prior to discharge, and the suprapubic catheter remained in place until the total hip cast was removed. Parents were instructed to irrigate the suprapubic catheter with 10 to 15 ml of normal saline if the catheter tiled to drain or if output decreased and were taught how to recognize signs and symptoms of inkction. Because the total hip cast made it impossible for Baby L to fit into a regular car seat, a Cosco Dreamrider car seat was obtained by the hospital. Follow-up care included home health visits and appointments with Baby Ls primary care physician, urologist, and orthopedic specialist. Discharge medications included amoxicillin for prevention of urinary tract infection and Zantac and cisapride for gastroesophageal reflux. Two weeks later, Baby L developed hematuria and was brought to the emergency room. Swelling of the left leg and abdomen-probably secondary to the compression of the cast-was noted. The total hip cast was removed, and the infant was sent home with resolution of the hematuria. Approximately one week later, Baby L was diagnosed with a urinary tract infection, and his oral antibiotics were changed. No improvement in symptoms was noted, and Baby L was readmitted to the hospital for increased fussiness, poor appetite, diarrhea, and decreased suprapubic catheter urine output. The white blood cell count was elevated (23,000/mm3) on admission, and a urine culture was positive for Citrobacterfieundii. N fluids for hydration and IV broadspectrum antibiotics were started. A renal ultrasound was performed; it showed only mild bilateral dilation of the renal caiyx. Baby L continued to improve clinically, and the suprapubic tube was removed. After seven days of IV antibiotics, Baby L was discharged home. His planned follow-up care included prophylactic antibiotic therapy until reflux was corrected, serial ultrasonography every three to six months to rule out hydronephrosis, urine cultures to detect infection, &d serum creatinines to assess renal function. SUMMARY Bladder exstrophy is a rare condition-one that poses significant psychosocial and physiologic problems. To preserve renal and ensure huure sexual function requires comprehensive nursing, medical, and surgical care. A multidisciplinary approach to the many complex problems experienced by these infants and their families is necessary to achieve the optimal outcome. @

REFERENCES
1. Rickman Pl? 1960. Vesicointestinal fissure. Archives of Disease in Childhood 35(2): 97-102. Lattimer JR, and Smith IMJK 1966. Exstrophy closure: Follow2. up on 20 cases. Journal ojUroloLgY5!3): 356-359. 3. Shapiro E, Leper H, and Jet% RD. 1984. The inheritance of the exstrophy-epispadias complex. Journal of Urology 132(2): 308-310. 4. Lancaster PAL. 1987. Epidemiology of bladder exstrophy and epispadias: A communication Tom the international clearinghouse for birth defects monitoring systems. Teratology 36(2): 221-227. 5. Larsen WJ. 1993. Human Embryology. New York: Churchill Livingstone, 224-225. 6. Jef& RD. 1987. Exstrophy, epispadias, and cloacai and urogenital sinus abnormalities. Pediatric Clinics of North America 34( 5): 1233-1257. 7. Richards DS, Langham M Jr, and lMaha&y SM. 1992. The prenatal uhrasonographic diagnosis of cloacal exstrophy. Journal of Ultrasound in Medicine ll(9): 507-510. Shapiro E, et al. 1985. Muscarinic cholinergic receptors in blad8. der exstrophy: Insight into surgical management. Journal of urology 134(Z): 308-310. 9. Stringer MD, Du@ PG, and Ransley PG. 1994. Inguinal hernia associated with bladder exstrophy. British Journal of Uro[o~y 73( 3): 308-309. 10. Gearhart JP, and Jet% RD. 1992. Exstrophy of the bladder, epispadias, and other bladder anomalies. In Campbells Urology, Walsh I, et al., eds. Philadelphia: WB Saunders, 1772-1821. 11. Wong DL. 1995. Conditions caused by defects in physical development. In Wbalcy and Won& Nursing Cart of Infants and Children, 5th ed., Wong DL, and Wilson D, eds, St. Louis: Mosby-Year Book, 433-513. 12. Radebaugh LC. 1986. Nursing care of the infant with bladder exstrophy. A UAA Journal 7( 2): 1 l-l 5. 13. Morrison S, and Fletcher BD. 1997. Diagnostic imaging. In Neonatal-P&natal Medicine, vol. 1, 6th ed., Fanaroff & and Martin R, eds. St. Louis: Mosby-Year Book, 639-671. 14. Spence HC, Hof%nan WN, and Pate VA. 1975. Exstrophy of the bladder. Part I: Longer term results in a series of 37 cases treated by ureterosigmoidostomy. Journal of uroio# 114( 7): 133-137. 15. C&g DA, &O HP, and Duckett JW. 1996. Anomalies of the bIadder and doaca. In Adult and Pediahic Uro&y, GiIIenwater JY, et al., eds. St. Louis: Mosby-Year Book, 2445-2488. 16. Lepor J, and Jell? RD. 1983. Primary bladder closure and bladder neck reconsttuction in classical bladder exstrophy. Journal of U~oiigy 130(6): 1142-1145. 17. Allen TD, Husmann DA, and Bucholz RW. 1992. Exstrophy of the bladder: Primary closure after iliac osteotomies without external or internal fixation. Journal of Urology 147(2): 438440. 18. Aadalen RM, et al. 1980. Exstrophy of the bladder: Long-term results of bilateral posterior iliac osteotomies and two-stage anatomic repair. Jouruai of Clinical Ortbopcdics 15 1: 193-200.

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19. Husmann DA, McLorie GA, and Churchii BM. 1989. Closure of the exstrophic bladder: An evaluation of the factors leading to its success and its importance on urinary continence. Journal of Urology 142(2): 522-524. 20. Connor JP, et al. 1989. Long-term follow-up of 207 patients with bladder exstrophy: An evoludon in treatment. Journal of Urology 142( 3): 793-796. 21. Mesrobian HG, Kclalis PP, and Kramer SA. 1988. Long-term follow-up of 103 patients with bladder exstrophy. Journal of UroroBy 139(4): 719-722. 22. de la Hunt MN, and ODonnell B. 1989. Current management of bladder exstrophy: A BAPS collective review Tom eight centers of 81 patients born between 1975 and 1985. Journal of Pediatric Suvgery 24( 6): 584-585. 23. Kajbafzadeh A, Quinn G, and Ransley l? 1995. Radical single stage reconstruction in f%led cxsuophy. Journal of Urology 154(2 part 2): 868-870. 24. Mansi M, and Ahmcd S. 1993. Young-Dees Leadbetter bladder 28. Kandzari SJ, et al. 1974. Exstrophy of the urinary bladder complicated by adenocarcinoma. Urology 3(4): 496498. 29. Davillas N, et al. 1991. Bladder exstrophy complicated by adenocarcinoma. British Journal of Uro&y 68( 1): 107. 30. Facchini V, et al. 1987. Carcinoma developing in bladder exstrophy. British Journal of Obstetrics and Gynecology 94( 8): 795-797.

About the Author

Jeannie Mollohan received her BSN from Southern Illinois Univehq, Edward&h, her MSN(R) in nursing of children from St. Louis University, and her neonatal nurse practitihzer c&i@ate from St. Johns Mercy Medical Center in St. Louis, Missouri. Ms. Molloban has 17 years of NICU/pICU experience at Cardinal Ghnou Childrens Hospital in St. Louis as a &ffnurse/ECMO specialist, ax&ant bead nurse, and bead nurse. she is currently workinp as a neonatal nurse practitioner/cLinicaI nurse specialist in the NICU at Cardinal Glennon. She is a member of NANN and Sigma Theta Tau. The author wishes w thank Carol Trotter, RNC, NNP, PhD (candidate), director of the neonatal nurse practitioner proflarn at St. Johns Mercy Medical Center for her assistance in the preparation of this manuscript. For fi_u-ther information, please contact: Jeannie Mollohan, RNC, MSN(R), NNP Cardinal Glennon Childrens Hospital NICU 1465 South Grand Boulevard St. Louis, MO 63104 (314) 577-5631

neck reconstruction for sphincteric urinary incontinence: The value of augmentation cystoplasty. Scandinavian Journal of

Urology and Nepbro&y27(4): 509-517. 25. Woodhouse C, Ran&y I, and Williams D. 1983. The patient with exstrophy in adult life. British Journal of Urolom 55(6): 632-635. 26. Lattimer JK, et al. 1978. Long-term follow-up after exstrophy closure: Late improvement and good quaIity of life. Journal of Urology 119( 5): 664-666. 27. Stein R, et al. 1994. The fate of the adult exstrophy patient. JournalofUrolqy 152(5): 1413-1416.

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