An Overview of Bladder Exstrophy
The management of children born with bladder exstrophy has changed dramatically during the past 15 years, allowing many of these patients to live a more "normal" and functional lifestyle (Exstrophy-epispadias complex, n.d.). The Johns Hopkins Children's Center is an international referral center for children born with this disease, having followed over 900 patients with the bladder exstrophy-epispadias complex and treating approximately 15 new or failed closure patients yearly (J.P. Gearhart, personal communication, January 15, 2008). The Pediatric Pain Service is an important component of their care team.
Bladder exstrophy is an abnormality of formation of the bladder and pelvis. It is a developmental defect that is seen in approximately 1 in 40,000 live births. Specifically, bladder exstrophy results from an abnormal anterior rupture of the cloacal membrane early in the embryonic period. This rupture inhibits mesenchymal ingrowth into the abdominal wall. The development of the pelvis also is affected by this failure in migration of mesenchyme. Classic bladder exstrophy presents at birth with a wide pubic separation and an exposed bladder (Figure 1). Cloacal exstrophy also involves intestinal prolapse (Okubadejo et al., 2003, Sponseller et al., 2001).
A, Three-dimensional volumetric models of normal pediatric pelvis. The views are anterior (A), sagittal (B), superior (C), and a second sagittal view with the pelvis rotated 45° (D). B, Three-dimensional volumetric models of an unclosed exstrophied pelvis. The views are anterior (A), sagittal (B), superior (C), and a second sagittal view with the pelvis rotated 45° (D). (Reprinted from Stec et al. . Evaluation of the bony pelvis in classic bladder exstrophy by using 3D-CT: Further insights. Urology, 58, 1030-1035, Elsevier Science Inc., 2001, with permission from Elsevier.)
Diagnosis of bladder exstrophy can be made on prenatal ultrasonography or at the time of delivery. Prenatal diagnosis can allow for prompt evaluation for surgical reconstruction, preparation of the family for surgery, and referral to a specialty center if needed. In the best circumstances, initial closure is performed in the first 48 to 72 hours of life (Exstrophy-epispadias complex, n.d.).
Although some controversies exist concerning timing and techniques of reconstruction, there is consensus that the best chance of success in achieving long-term continence is a successful primary operation (Woodhouse, North & Gearhart, 2006). The best functional results have been obtained with the use of the staged approach to reconstruction. This approach was introduced in the mid 1970s by Dr. Robert Jeffs at Johns Hopkins Hospital. The staged approach entails early bladder and posterior urethral valve closure, followed by epispadias repair at 12 to 18 months of age, and finally bladder neck reconstruction at 4 to 5 years of age (Baker & Gearhart, 1998).
The first stage of the repair, which is typically done during the newborn period, involves approximation of the pelvic ring, closure of the bladder and posterior urethra inside the pelvic ring, and closure of the abdominal wall (Baker and Gearhart, 1998, Okubadejo et al., 2003). Pubic approximation eases abdominal wall closure and decreases the rate of bladder and abdominal wall dehiscence, which ultimately contributes to long-term continence in children with this condition. In a neonate younger than 72 hours of age with a malleable pelvis and a small degree of pelvic diastasis, closure can be achieved with manual approximation without undue tension, rather than osteotomies, followed by postoperative traction. In older patients, those with wide pelvic separation, or those undergoing re-operative closures, osteotomies are used (Baker & Gearhart; Meldrum, Baird, & Gearhart, 2003). There are some institutional differences in the technique for approximation of the pelvic ring, but the combined approach of vertical iliac osteotomy with transverse innominate osteotomy has yielded excellent results (Figure 2) (Baker & Gearhart; Nelson, King, Sponseller, & Gearhart, 2006). Osteotomies are often performed by a separate pediatric orthopedic team.
Osteotomy sites for combined vertical iliac and transverse innominate osteotomies showing the placement of intrafragmentary pins. (Reprinted with kind permission from Springer Science, Business Media, and Baker, L. A., & Gearhart, J. P. . The staged approach to bladder exstrophy closure and the role of osteotomies. World Journal of Urology, 16, 205-211.)
Postoperative pelvic stabilization permits the bladder and abdominal wall reconstruction to heal without tension (Mathews, Gearhart, Bhatnager, & Sponseller, 2006). Effective postoperative immobilization of the pelvis is achieved by the use of 4 to 6 weeks of modified Bryant's traction in children without osteotomies (Figure 3) or an external fixator and 6 to 8 weeks of modified Buck's traction for children requiring osteotomies (Figure 4). Fixation maintains the closure during the critical initial weeks of healing (Figure 5). Leg traction controls the action of the legs and keeps the buttocks and pelvis flat on the bed. Suprapubic tubes and ureteral stents are placed in all cases (Baker and Gearhart, 1998, Mathews et al., 2006, Meldrum et al., 2003). The external fixator and/or traction is removed when there is radiologic evidence that sufficient callous has formed at the site of the osteotomy. At that point, the child is permitted to become more mobile (Meldrum et al.; Sponseller et al., 2001).
Modified Bryant's traction. Child is positioned with weights maintaining the legs in an extended position and the hips in 90 degrees of flexion. This figure is available in color online at www.jpedhc.org
Modified Buck's traction. Child is positioned with weights maintaining the legs in an extended position parallel to the bed. This figure is available in color online at www.jpedhc.org.
An external fixator in position keeps tension off the repair as the pelvis heals. Hardware remains in place for at least 6 weeks. This figure is available in color online at www.jpedhc.org.
If the pelvis prematurely separates, there is significant tension on the bladder and posterior urethra, leading to bladder prolapse or dehiscence (Meldrum et al., 2003). Failure of the initial closure can be caused by several factors. These factors include wound infection, abdominal distention, premature loss of ureteral stents or the suprapubic tube, and the absence of osteotomies in children with a large pelvic diastasis (Meldrum et al.). In addition to pelvic immobilization, other factors that are important to a successful closure include adequate pain control, prevention of abdominal distention, and postoperative antibiotics (Baker & Gearhart, 1998).
When failures occur, it is important to consider referral of these complex cases to an exstrophy center with experience in reoperation for failed primary closures. Failures that occur in otherwise well-performed closures often are the result of excessive movement of the child during the postoperative period and abduction of the lower extremities and pelvis. Lower extremity traction is believed to be the most effective way to avoid this complication (Meldrum et al., 2003, Nelson et al., 2006).
J Pediatr Health Care. 2008;22(6):351-359. © 2008 Mosby, Inc.
Cite this: The Acute Pain Service Nurse Practitioner: A Case Study in the Postoperative Care of the Child With Bladder Exstrophy - Medscape - Nov 01, 2008.